Hodgkin's Lymphoma
   HOME

TheInfoList



OR:

Hodgkin lymphoma (HL) is a type of
lymphoma Lymphoma is a group of blood and lymph tumors that develop from lymphocytes (a type of white blood cell). The name typically refers to just the cancerous versions rather than all such tumours. Signs and symptoms may include enlarged lymph node ...
in which
cancer Cancer is a group of diseases involving Cell growth#Disorders, abnormal cell growth with the potential to Invasion (cancer), invade or Metastasis, spread to other parts of the body. These contrast with benign tumors, which do not spread. Po ...
originates from a specific type of
white blood cell White blood cells (scientific name leukocytes), also called immune cells or immunocytes, are cells of the immune system that are involved in protecting the body against both infectious disease and foreign entities. White blood cells are genera ...
called
lymphocyte A lymphocyte is a type of white blood cell (leukocyte) in the immune system of most vertebrates. Lymphocytes include T cells (for cell-mediated and cytotoxic adaptive immunity), B cells (for humoral, antibody-driven adaptive immunity), an ...
s, where multinucleated
Reed–Sternberg cell Reed–Sternberg cells (also known as lacunar histiocytes for certain types) are distinctive, giant cells found with light microscopy in biopsies from individuals with Hodgkin lymphoma. They are usually derived from B lymphocytes, classicall ...
s (RS cells) are present in the lymph nodes. The condition was named after the English physician Thomas Hodgkin, who first described it in 1832. Symptoms may include
fever Fever or pyrexia in humans is a symptom of an anti-infection defense mechanism that appears with Human body temperature, body temperature exceeding the normal range caused by an increase in the body's temperature Human body temperature#Fever, s ...
,
night sweats Night sweats or nocturnal hyperhydrosis is the repeated occurrence of excessive sweating during sleep. The person may or may not also perspire excessively while awake. One of the most common causes of night sweats in women over 40 is the horm ...
, and
weight loss Weight loss, in the context of medicine, health, or physical fitness, refers to a reduction of the total body mass, by a mean loss of fluid, body fat (adipose tissue), or lean mass (namely bone mineral deposits, muscle, tendon, and other conn ...
. Often, non-painful enlarged lymph nodes occur in the neck, under the arm, or in the
groin In human anatomy, the groin, also known as the inguinal region or iliac region, is the junctional area between the torso and the thigh. The groin is at the front of the body on either side of the pubic tubercle, where the lower part of the abdom ...
. Persons affected may feel tired or be itchy. The two major types of Hodgkin lymphoma are classic Hodgkin lymphoma and nodular lymphocyte-predominant Hodgkin lymphoma. About half of cases of Hodgkin lymphoma are due to
Epstein–Barr virus The Epstein–Barr virus (EBV), also known as human herpesvirus 4 (HHV-4), is one of the nine known Herpesviridae#Human herpesvirus types, human herpesvirus types in the Herpesviridae, herpes family, and is one of the most common viruses in ...
(EBV) and these are generally the classic form. Other risk factors include a family history of the condition and having
HIV/AIDS The HIV, human immunodeficiency virus (HIV) is a retrovirus that attacks the immune system. Without treatment, it can lead to a spectrum of conditions including acquired immunodeficiency syndrome (AIDS). It is a Preventive healthcare, pr ...
. Diagnosis is conducted by confirming the presence of cancer and identifying Reed–Sternberg cells in lymph node biopsies. The virus-positive cases are classified as a form of the Epstein–Barr virus-associated lymphoproliferative diseases. Hodgkin lymphoma may be treated with
chemotherapy Chemotherapy (often abbreviated chemo, sometimes CTX and CTx) is the type of cancer treatment that uses one or more anti-cancer drugs (list of chemotherapeutic agents, chemotherapeutic agents or alkylating agents) in a standard chemotherapy re ...
,
radiation therapy Radiation therapy or radiotherapy (RT, RTx, or XRT) is a therapy, treatment using ionizing radiation, generally provided as part of treatment of cancer, cancer therapy to either kill or control the growth of malignancy, malignant cell (biology), ...
, and stem-cell transplantation. The choice of treatment often depends on how advanced the cancer has become and whether or not it has favorable features. If the disease is detected early, a cure is often possible. In the United States, 88% of people diagnosed with Hodgkin lymphoma survive for five years or longer. For those under the age of 20, rates of survival are 97%. Radiation and some chemotherapy drugs, however, increase the risk of other cancers,
heart disease Cardiovascular disease (CVD) is any disease involving the heart or blood vessels. CVDs constitute a class of diseases that includes: coronary artery diseases (e.g. angina pectoris, angina, myocardial infarction, heart attack), heart failure, ...
, or lung disease over the subsequent decades. In 2015, about 574,000 people globally had Hodgkin lymphoma, and 23,900 (4.2%) died. In the United States, 0.2% of people are affected at some point in their life. Most people are diagnosed with the disease between the ages of 20 and 40.


Signs and symptoms

People with Hodgkin lymphoma may present with these symptoms: *
Lymphadenopathy Lymphadenopathy or adenopathy is a disease of the lymph nodes, in which they are abnormal in size or consistency. Lymphadenopathy of an inflammatory type (the most common type) is lymphadenitis, producing swollen or enlarged lymph nodes. In c ...
: The most common symptom of Hodgkin lymphoma is the painless enlargement of one or more
lymph nodes A lymph node, or lymph gland, is a kidney-shaped Organ (anatomy), organ of the lymphatic system and the adaptive immune system. A large number of lymph nodes are linked throughout the body by the lymphatic vessels. They are major sites of lymphoc ...
. The nodes may also feel rubbery and swollen when examined. The nodes of the neck, armpits and groin ( cervical and supraclavicular) are most frequently involved (80–90% of the time, on average). The lymph nodes of the chest are often affected, and these may be noticed on a
chest radiograph A chest radiograph, chest X-ray (CXR), or chest film is a projection radiograph of the chest used to diagnose conditions affecting the chest, its contents, and nearby structures. Chest radiographs are the most common film taken in medicine. L ...
. * Systemic symptoms: About one-third of people with Hodgkin lymphoma may also present with systemic symptoms, including: ** Itchy skin **
Night sweats Night sweats or nocturnal hyperhydrosis is the repeated occurrence of excessive sweating during sleep. The person may or may not also perspire excessively while awake. One of the most common causes of night sweats in women over 40 is the horm ...
** Unexplained
weight loss Weight loss, in the context of medicine, health, or physical fitness, refers to a reduction of the total body mass, by a mean loss of fluid, body fat (adipose tissue), or lean mass (namely bone mineral deposits, muscle, tendon, and other conn ...
of at least 10% of the person's total body mass in six months or less ** Low-grade
fever Fever or pyrexia in humans is a symptom of an anti-infection defense mechanism that appears with Human body temperature, body temperature exceeding the normal range caused by an increase in the body's temperature Human body temperature#Fever, s ...
. **
Fatigue Fatigue is a state of tiredness (which is not sleepiness), exhaustion or loss of energy. It is a signs and symptoms, symptom of any of various diseases; it is not a disease in itself. Fatigue (in the medical sense) is sometimes associated wit ...
(lassitude) ** Systemic symptoms such as fever, night sweats, and weight loss are known as B symptoms; thus, presence of these indicate that the person's stage is, for example, 2B instead of 2A. *
Splenomegaly Splenomegaly is an enlargement of the spleen. The spleen usually lies in the left upper quadrant (LUQ) of the human abdomen. Splenomegaly is one of the four cardinal signs of ''hypersplenism'' which include: some reduction in number of circulat ...
: Enlargement of the
spleen The spleen (, from Ancient Greek '' σπλήν'', splḗn) is an organ (biology), organ found in almost all vertebrates. Similar in structure to a large lymph node, it acts primarily as a blood filter. The spleen plays important roles in reg ...
is often present in people with Hodgkin lymphoma. The enlargement is seldom massive, and the size of the spleen may fluctuate during the course of treatment. *
Hepatomegaly Hepatomegaly is enlargement of the liver. It is a non-specific sign (medicine), medical sign, having many causes, which can broadly be broken down into infection, hepatic tumours, and metabolic disorder. Often, hepatomegaly presents as an abdomin ...
: Enlargement of the
liver The liver is a major metabolic organ (anatomy), organ exclusively found in vertebrates, which performs many essential biological Function (biology), functions such as detoxification of the organism, and the Protein biosynthesis, synthesis of var ...
, due to liver involvement, is infrequent in people with Hodgkin lymphoma. * Hepatosplenomegaly: The enlargement of both the liver and spleen can be caused by the same disease. * Pain following alcohol consumption: Classically, involved nodes are painful after alcohol consumption, though this phenomenon is very uncommon, occurring in only two to three percent of people with Hodgkin lymphoma,Page 242
in:
thus having a low sensitivity. On the other hand, its positive predictive value is high enough for it to be regarded as a
pathognomonic Pathognomonic (synonym ''pathognomic'') is a term, often used in medicine, that means "characteristic for a particular disease". A pathognomonic sign is a particular sign whose presence means that a particular disease is present beyond any doubt. ...
sign of Hodgkin lymphoma. The pain typically has an onset within minutes after ingesting alcohol, and is usually felt as coming from the vicinity where there is an involved lymph node. The pain has been described as either sharp and stabbing or dull and aching. *
Back pain Back pain (Latin: ''dorsalgia'') is pain felt in the back. It may be classified as neck pain (cervical), middle back pain (thoracic), lower back pain (lumbar) or coccydynia (tailbone or sacral pain) based on the segment affected. The lumbar area ...
: Nonspecific back pain (pain that cannot be localised or its cause determined by examination or scanning techniques) has been reported in some cases of Hodgkin lymphoma. The lower back is most often affected. * Cyclical fever: People may also present with a cyclical high-grade fever known as the Pel–Ebstein fever, or more simply "P-E fever". However, there is debate as to whether the P-E fever truly exists. *
Nephrotic syndrome Nephrotic syndrome is a collection of symptoms due to kidney damage. This includes proteinuria, protein in the urine, hypoalbuminemia, low blood albumin levels, hyperlipidemia, high blood lipids, and significant edema, swelling. Other symptoms ...
can occur in individuals with Hodgkin lymphoma and is most commonly caused by minimal change disease. * May present with airway obstruction, pleural/pericardial effusion, hepatocellular dysfunction, or bone-marrow infiltration.


Diagnosis

Hodgkin lymphoma must be distinguished from noncancerous causes of lymph node swelling (such as various infections) and from other types of cancer. Definitive diagnosis is by lymph node
biopsy A biopsy is a medical test commonly performed by a surgeon, interventional radiologist, an interventional radiologist, or an interventional cardiology, interventional cardiologist. The process involves the extraction of sampling (medicine), sample ...
(usually excisional biopsy with microscopic examination).
Blood test A blood test is a medical laboratory, laboratory analysis performed on a blood sample that is usually extracted from a vein in the arm using a hypodermic needle, or via fingerprick. Multiple tests for specific blood components, such as a glucose ...
s are also performed to assess function of major organs and safety for chemotherapy.
Positron emission tomography Positron emission tomography (PET) is a functional imaging technique that uses radioactive substances known as radiotracers to visualize and measure changes in metabolic processes, and in other physiological activities including blood flow, r ...
(PET) is used to detect small deposits that do not show on CT scanning. PET scans are also useful in functional imaging (by using a radiolabeled glucose to image tissues of high metabolism). In some cases, a gallium scan may be used instead of a PET scan.


Types

The two main types of Hodgkin lymphoma are classic Hodgkin lymphoma and nodular lymphocyte-predominant Hodgkin lymphoma. The prevalence of classic Hodgkin lymphoma and nodular-lymphocyte Hodgkin lymphoma are about 90% and 10%, respectively. The morphology, phenotype, molecular features, and, therefore, the clinical behaviour and presentation of the two types differ.


Classic

Classic Hodgkin lymphoma (excluding nodular lymphocyte predominant Hodgkin lymphoma) can be subclassified into four pathologic subtypes based upon Reed–Sternberg cell morphology and the composition of the reactive cell infiltrate seen in the
lymph node A lymph node, or lymph gland, is a kidney-shaped organ of the lymphatic system and the adaptive immune system. A large number of lymph nodes are linked throughout the body by the lymphatic vessels. They are major sites of lymphocytes that includ ...
biopsy A biopsy is a medical test commonly performed by a surgeon, interventional radiologist, an interventional radiologist, or an interventional cardiology, interventional cardiologist. The process involves the extraction of sampling (medicine), sample ...
specimen (the cell composition around the Reed–Sternberg cell(s)). Presence of EBV in Reed-Sternberg cells is most commonly found in the subtypes lymphocyte depleted HL (>70%) and mixed cellularity HL (70%), whilst being less prevalent in lymphocyte-rich HL (40%) and relatively uncommon by comparison in nodular sclerosing HL. For the other forms, although the traditional B-cell markers (such as
CD20 B-lymphocyte antigen CD20 or CD20 is B lymphocyte cell-surface molecule. It is a 33-37 kDa non-glycosylated protein. CD20 is expressed on the surface of B-cells from the pre-B phase, the expression is lost in terminally differentiated plasm ...
) are not expressed on all cells, Reed–Sternberg cells are usually of B cell origin. Although Hodgkin lymphoma is now frequently grouped with other B-cell malignancies, some T-cell markers (such as CD2 and
CD4 In molecular biology, CD4 (cluster of differentiation 4) is a glycoprotein that serves as a co-receptor for the T-cell receptor (TCR). CD4 is found on the surface of immune cells such as helper T cells, monocytes, macrophages, and dendritic c ...
) are occasionally expressed. Hodgkin cells produce interleukin-21 (IL-21), which was once thought to be exclusive to T-cells. This feature may explain the behavior of classic Hodgkin lymphoma, including clusters of other immune cells gathered around HL cells (infiltrate) in cultures.


Nodular lymphocyte predominant

Nodular lymphocyte predominant Hodgkin lymphoma (NLPHL) is another subtype of Hodgkin lymphoma distinct from classic Hodgkin lymphoma and is characterized by the presence of popcorn cells which express
CD20 B-lymphocyte antigen CD20 or CD20 is B lymphocyte cell-surface molecule. It is a 33-37 kDa non-glycosylated protein. CD20 is expressed on the surface of B-cells from the pre-B phase, the expression is lost in terminally differentiated plasm ...
. Due to these differences, among others, NLPHL is often treated differently from classic Hodgkin lymphoma, including using
rituximab Rituximab, sold under the brand name Rituxan among others, is a monoclonal antibody medication used to treat certain autoimmune diseases and types of cancer. It is used for non-Hodgkin lymphoma, chronic lymphocytic leukemia (in children and ad ...
in combination with AVBD chemotherapy, though individual cases vary and clinical trials are ongoing.


Staging

The staging is the same for both Hodgkin and non-Hodgkin lymphomas. After Hodgkin lymphoma is diagnosed, a person will be staged by undergoing a series of tests and procedures that will determine what areas of the body are affected. These procedures may include documentation of their
histology Histology, also known as microscopic anatomy or microanatomy, is the branch of biology that studies the microscopic anatomy of biological tissue (biology), tissues. Histology is the microscopic counterpart to gross anatomy, which looks at large ...
, a physical examination, blood tests, chest
X-ray An X-ray (also known in many languages as Röntgen radiation) is a form of high-energy electromagnetic radiation with a wavelength shorter than those of ultraviolet rays and longer than those of gamma rays. Roughly, X-rays have a wavelength ran ...
radiographs,
computed tomography A computed tomography scan (CT scan), formerly called computed axial tomography scan (CAT scan), is a medical imaging technique used to obtain detailed internal images of the body. The personnel that perform CT scans are called radiographers or ...
(CT)/
positron emission tomography Positron emission tomography (PET) is a functional imaging technique that uses radioactive substances known as radiotracers to visualize and measure changes in metabolic processes, and in other physiological activities including blood flow, r ...
(PET)/
magnetic resonance imaging Magnetic resonance imaging (MRI) is a medical imaging technique used in radiology to generate pictures of the anatomy and the physiological processes inside the body. MRI scanners use strong magnetic fields, magnetic field gradients, and ...
(MRI) scans of the chest, abdomen and pelvis, and usually a bone marrow biopsy. PET scan is now used instead of the gallium scan for staging. On the PET scan, sites involved with lymphoma light up very brightly enabling accurate and reproducible imaging. In the past, a lymphangiogram or surgical
laparotomy A laparotomy is a surgical procedure involving a surgical incision through the abdominal wall to gain access into the abdominal cavity. It is also known as a celiotomy. Origins and history The first successful laparotomy was performed without ...
(which involves opening the abdominal cavity and visually inspecting for tumors) were performed. Lymphangiograms or laparotomies are very rarely performed, having been supplanted by improvements in imaging with the CT scan and PET scan. On the basis of this staging, the person will be classified according to a staging classification (the Ann Arbor staging classification scheme is a common one): * Stage I is involvement of a single lymph node region (I) (mostly the cervical region) or single extralymphatic site (Ie); * Stage II is involvement of two or more lymph node regions on the same side of the diaphragm (II) or of one lymph node region and a contiguous extralymphatic site (IIe); * Stage III is involvement of lymph node regions on both sides of the diaphragm, which may include the
spleen The spleen (, from Ancient Greek '' σπλήν'', splḗn) is an organ (biology), organ found in almost all vertebrates. Similar in structure to a large lymph node, it acts primarily as a blood filter. The spleen plays important roles in reg ...
(IIIs) or limited contiguous extralymphatic organ or site (IIIe, IIIes); * Stage IV is disseminated involvement of one or more extralymphatic organs. The absence of systemic symptoms is signified by adding "A" to the stage; the presence of systemic symptoms is signified by adding "B" to the stage. For localized extranodal extension from mass of nodes that does not advance the stage, subscript "E" is added. Splenic involvement is signified by adding "S" to the stage. The inclusion of "bulky disease" is signified by "X". File:Diagram showing stage 1 Hogkin's lymphoma CRUK 191.svg, Stage 1 Hodgkin lymphoma File:Diagram showing stage 2 Hodgkin's lymphoma CRUK 208.svg, Stage 2 Hodgkin lymphoma File:Diagram showing stage 3 Hodgkin's lymphoma CRUK 221.svg, Stage 3 Hodgkin lymphoma File:Diagram showing stage 4 Hodgkin's lymphoma CRUK 230.svg, Stage 4 Hodgkin lymphoma


Pathology


Macroscopy

Affected lymph nodes (most often, laterocervical lymph nodes) are enlarged, but their shape is preserved because the capsule is not invaded. Usually, the cut surface is white-grey and uniform; in some histological subtypes (e.g. nodular sclerosis) a nodular aspect may appear. A fibrin ring granuloma may be seen.


Microscopy

Microscopic examination of the lymph node biopsy reveals complete or partial effacement of the lymph node architecture by scattered large malignant cells known as Reed-Sternberg cells (RSC) (typical and variants) admixed within a reactive cell infiltrate composed of variable proportions of lymphocytes, histiocytes, eosinophils, and plasma cells. The Reed–Sternberg cells are identified as large often bi-nucleated cells with prominent nucleoli and an unusual CD45−, CD30+, CD15+/− immunophenotype. In approximately 50% of cases, the Reed–Sternberg cells are infected by the Epstein–Barr virus. Characteristics of classic Reed–Sternberg cells include large size (20–50 micrometres), abundant, amphophilic, finely granular/homogeneous cytoplasm; two mirror-image nuclei (owl eyes) each with an eosinophilic nucleolus and a thick nuclear membrane (
chromatin Chromatin is a complex of DNA and protein found in eukaryote, eukaryotic cells. The primary function is to package long DNA molecules into more compact, denser structures. This prevents the strands from becoming tangled and also plays important r ...
is distributed close to the nuclear membrane). Almost all of these cells have an increased copy number of chromosome 9p/9p24.1. Variants: * Hodgkin cell (atypical mononuclear RSC) is a variant of RS cell, which has the same characteristics but is mononucleated. * Lacunar RSC is large, with a single hyperlobulated nucleus, multiple, small nucleoli and eosinophilic cytoplasm which is retracted around the nucleus, creating an empty space ("lacunae"). * Pleomorphic RSC has multiple irregular nuclei. * "Popcorn" RSC (lympho-histiocytic variant) is a small cell, with a very lobulated nucleus, small nucleoli. * "Mummy" RSC has a compact nucleus with no nucleolus and basophilic cytoplasm. Hodgkin lymphoma can be sub-classified by histological type. The cell histology in Hodgkin lymphoma is not as important as it is in
non-Hodgkin lymphoma Non-Hodgkin lymphoma (NHL), also known as non-Hodgkin's lymphoma, is a group of blood cancers that includes all types of lymphomas except Hodgkin lymphomas. Symptoms include enlarged lymph nodes, fever, night sweats, weight loss, and tiredn ...
: the treatment and prognosis in classic Hodgkin lymphoma usually depends on the stage of disease rather than the histotype.


Management

The approach for treatment aims to reduce the acute and long-term toxicities associated with Hodgkin lymphoma (e.g. cardiac damage and secondary cancers) and increase overall survival. People with early stage disease (IA or IIA) can be treated effectively with
radiation therapy Radiation therapy or radiotherapy (RT, RTx, or XRT) is a therapy, treatment using ionizing radiation, generally provided as part of treatment of cancer, cancer therapy to either kill or control the growth of malignancy, malignant cell (biology), ...
or chemotherapy. The choice of treatment depends on the age, sex, bulk and the histological subtype of the disease. Adding localised radiation therapy after the chemotherapy regimen may provide a longer progression-free survival compared with chemotherapy treatment alone. People with later disease (III, IVA, or IVB) are treated with combination chemotherapy alone. People of any stage with a large mass in the chest are usually treated with combined chemotherapy and radiation therapy. The common non-Hodgkin treatment,
rituximab Rituximab, sold under the brand name Rituxan among others, is a monoclonal antibody medication used to treat certain autoimmune diseases and types of cancer. It is used for non-Hodgkin lymphoma, chronic lymphocytic leukemia (in children and ad ...
(which is a monoclonal antibody against CD20) is not routinely used to treat Hodgkin lymphoma due to the lack of CD20 surface antigens in most cases. The use of rituximab in Hodgkin lymphoma, including the lymphocyte predominant subtype has been reviewed. The evidence is uncertain about the effect of nivolumab for people with a Hodgkin lymphoma e.g. on the overall survival. Increased age is an adverse risk factor for Hodgkin lymphoma, but in general elderly people (≥ 60 years of age) without major comorbidities are sufficiently fit to tolerate therapy with curative intent. Despite this, treatment outcome in the elderly patient is not comparable to that of younger people and the disease is a different entity in older people where different considerations enter into treatment decisions. Two novel targeted drugs have been developed for relapsing and refractory HL patients; brentuximab vedotin, a CD30 antibody conjugated with a cytotoxic component MMAE, and the checkpoint inhibitors, nivolumab and pembrolizumab. This has been an important step in the treatment for the few, but still existing refractory patients. For Hodgkin lymphomas, radiation oncologists typically use external beam radiation therapy (sometimes shortened to EBRT or XRT). Radiation oncologists deliver external beam radiation therapy to the lymphoma from a machine called a linear accelerator which produces high energy X-rays and electrons. People usually describe treatments as painless and similar to getting an X-ray. Treatments last less than 30 minutes each. For lymphomas, there are a few different ways radiation oncologists target the cancer cells. Involved site radiation is when the radiation oncologists give radiation only to those parts of the person's body known to have the cancer. Very often, this is combined with chemotherapy. Radiation therapy directed above the diaphragm to the neck, chest or underarms is called mantle field radiation. Radiation to below the diaphragm to the abdomen, spleen or pelvis is called inverted-Y field radiation. Total nodal irradiation is when the therapist gives radiation to all the lymph nodes in the body to destroy cells that may have spread.


Adverse effects

The high cure rates and long survival of many people with Hodgkin lymphoma has led to a high concern with late adverse effects of treatment, including cardiovascular disease and second malignancies such as acute
leukemia Leukemia ( also spelled leukaemia; pronounced ) is a group of blood cancers that usually begin in the bone marrow and produce high numbers of abnormal blood cells. These blood cells are not fully developed and are called ''blasts'' or '' ...
s, lymphomas, and solid tumors within the radiation therapy field. Most people with early-stage disease are now treated with abbreviated chemotherapy and involved site radiation therapy rather than with radiation therapy alone. Clinical research strategies are exploring reduction of the duration of chemotherapy and dose and volume of radiation therapy in an attempt to reduce late morbidity and mortality of treatment while maintaining high cure rates. Hospitals are also treating those who respond quickly to chemotherapy with no radiation. In childhood cases of Hodgkin lymphoma, long-term endocrine adverse effects are a major concern, mainly gonadal dysfunction and growth retardation. Gonadal dysfunction seems to be the most severe endocrine long-term effect, especially after treatment with
alkylating agent Alkylation is a chemical reaction that entails transfer of an alkyl group. The alkyl group may be transferred as an alkyl carbocation, a free radical, a carbanion, or a carbene (or their equivalents). Alkylating agents are reagents for effecting ...
s or pelvic radiotherapy. It is possible that patients undergoing a chemotherapy need a platelet transfusion. If a stem cell transplantation is necessary for the treatment of a relapse, graft-versus-host diseases might occur.


Supportive treatment

Adding physical exercises to the standard treatment for adult patients with haematological malignancies like Hodgkin lymphoma may result in little to no difference in the mortality, the quality of life and the physical functioning. These exercises may result in a slight reduction in depression. Furthermore, aerobic physical exercises probably reduce fatigue. The evidence is very uncertain about the effect on anxiety and serious adverse events. 


Prognosis

Treatment of Hodgkin lymphoma has been improving. Types of chemotherapy have resulted in higher survival rates than have previously been seen. In a 2007 European trial, the five-year survival rate for those people with a favorable prognosis (FFP) was 98%, while that for people with worse outlooks was at least 85%. In 1998, an international effort identified seven prognostic factors that accurately predict the success rate of conventional treatment in people with locally extensive or advanced-stage Hodgkin lymphoma. Freedom from progression (FFP) at five years was directly related to the number of factors present in a person. The five-year FFP for people with zero factors is 84%. Each additional factor lowers the five-year FFP rate by 7%, such that the five-year FFP for a person with five or more factors is 42%. The adverse prognostic factors identified in the international study are: * Age ≥ 45 years * Stage IV disease *
Hemoglobin Hemoglobin (haemoglobin, Hb or Hgb) is a protein containing iron that facilitates the transportation of oxygen in red blood cells. Almost all vertebrates contain hemoglobin, with the sole exception of the fish family Channichthyidae. Hemoglobin ...
< 10.5 g/dl * Lymphocyte count < 600/μL or < 8% *
Male Male (Planet symbols, symbol: ♂) is the sex of an organism that produces the gamete (sex cell) known as sperm, which fuses with the larger female gamete, or Egg cell, ovum, in the process of fertilisation. A male organism cannot sexual repro ...
*
Albumin Albumin is a family of globular proteins, the most common of which are the serum albumins. All of the proteins of the albumin family are water- soluble, moderately soluble in concentrated salt solutions, and experience heat denaturation. Alb ...
< 4.0 g/dl * White blood count ≥ 15,000/μL Other studies have reported the following to be the most important adverse prognostic factors: mixed-cellularity or lymphocyte-depleted histologies, male sex, large number of involved nodal sites, advanced stage, age of 40 years or more, the presence of B symptoms, high erythrocyte sedimentation rate, and bulky disease (widening of the
mediastinum The mediastinum (from ;: mediastina) is the central compartment of the thoracic cavity. Surrounded by loose connective tissue, it is a region that contains vital organs and structures within the thorax, mainly the heart and its vessels, the eso ...
by more than one third, or the presence of a nodal mass measuring more than 10 cm in any dimension.) The use of positron emission tomography (PET) early after commencing chemotherapy has demonstrated to have powerful prognostic ability. This enables assessment of an individual's response to chemotherapy as the PET activity switches off rapidly in people who are responding. In this study, after two cycles of ABVD chemotherapy, 83% of people were free of disease at 3 years if they had a negative PET versus only 28% in those with positive PET scans. This prognostic method improves on FFP estimates based on the seven conventional factors. Several trials are underway to see if PET-based risk adapted response can be used to improve a person's outcomes by changing chemotherapy early in people who are not responding. The evidence is uncertain about the effect of negative (= good prognosis) or positive (= bad prognosis) interim PET scan results for people with a Hodgkin lymphoma on the progression-free survival. Negative interim PET scan results may result in an increase in progression-free survival compared if the adjusted result was measured. Negative interim PET scan results probably result in a large increase in the overall survival compared to those with a positive interim PET scan result,


Epidemiology

Unlike some other lymphomas, whose number of new cases per year increases with age, Hodgkin lymphoma has a bimodal distribution curve for the number of cases; that is, it occurs most frequently in two separate age groups, the first being young adulthood (age 15–35) and the second being in those over 55 years of age although these peaks may vary slightly with nationality. Overall, it is more common in males, except for the nodular sclerosis variant, which is slightly more common in females. The annual number of cases of Hodgkin lymphoma is 2.7 per 100,000 per persons per year, and the disease accounts for slightly less than 1% of all cancers worldwide. In 2010, globally it resulted in about 18,000 deaths down from 19,000 in 1990. In 2012, there were an estimated 65,950 cases and 25,469 deaths from Hodgkin lymphoma worldwide, with 28,852 and 37,098 cases occurring in developed and developing countries, respectively. However, the age-standardized rates were higher in developed regions, with the greatest rates in the Americas (1.5 per 100,000), East Mediterranean Region (1.5 per 100,000), and Europe (2.0 per 100,000). The East Mediterranean Region also has the highest age-standardized mortality rate of 1.0 per 100,000, which is mainly attributed to lifestyle and environmental risk factors associated with transitional economies such as smoking, obesity, physical inactivity, and reproductive behaviors, as well as availability of diagnostic practices and awareness of the disease. The number of cases of Hodgkin lymphoma is increased in people with infected with HIV.


United Kingdom

Hodgkin lymphoma accounts for less than 1% of all cancer cases and deaths in the United Kingdom. Around 1,800 people were diagnosed with the disease in 2011, and around 330 people died in 2012.


United States

In 2016, there were 8,389 new cases and 1,000 mortalities attributed to Hodgkin lymphoma, a decrease from the 8,625 new cases and 1,120 mortalities in 2015. As of January 2016, the five-year limited duration prevalence of Hodgkin lymphoma was 37,513 representing 0.71% of all diagnosed cancers in the United States.


History

Hodgkin lymphoma was first described in an 1832 report by Thomas Hodgkin, although Hodgkin noted that perhaps an earlier reference to the condition was provided by
Marcello Malpighi Marcello Malpighi (10 March 1628 – 30 November 1694) was an Italians, Italian biologist and physician, who is referred to as the "founder of microscopical anatomy, histology and father of physiology and embryology". Malpighi's name is borne by ...
in 1666. While occupied as museum curator at Guy's Hospital, London, Hodgkin studied seven people with painless lymph node enlargement. Of the seven cases, two were under the care of Richard Bright, one was of Thomas Addison, and one was of Robert Carswell. Carswell's report of the seventh case was accompanied by numerous illustrations that aided early descriptions of the disease. Hodgkin's report on the seven cases, entitled "On some morbid appearances of the absorbent glands and spleen", was presented to the Medical and Chirurgical Society of London in January 1832 and was subsequently published in the society's journal, ''Medical-Chirurgical Society Transactions''. Hodgkin's paper went largely unnoticed, however, even though Bright highlighted it in an 1838 publication. Indeed, Hodgkin himself did not view his contribution as particularly significant. In 1856, Samuel Wilks independently reported on a series of patients with the same disease that Hodgkin had previously described. Wilks, a successor to Hodgkin at Guy's Hospital, was unaware of Hodgkin's prior work on the subject. Bright informed Wilks of Hodgkin's contribution and in 1865, Wilks published a second paper, entitled "Cases of enlargement of the lymphatic glands and spleen", in which he named the illness "Hodgkin's disease" in honor of his predecessor. Theodor Langhans and WS Greenfield first described the microscopic characteristics of Hodgkin lymphoma in 1872 and 1878, respectively. In 1898 and 1902, respectively, Carl Sternberg and Dorothy Reed independently described the cytogenetic features of the malignant cells of Hodgkin lymphoma, now called Reed–Sternberg cells. Tissue specimens from Hodgkin's seven cases were preserved at Guy's Hospital. Nearly 100 years after Hodgkin's initial publication, histopathologic reexamination confirmed Hodgkin lymphoma in only three of seven of these people. The remaining cases included
non-Hodgkin lymphoma Non-Hodgkin lymphoma (NHL), also known as non-Hodgkin's lymphoma, is a group of blood cancers that includes all types of lymphomas except Hodgkin lymphomas. Symptoms include enlarged lymph nodes, fever, night sweats, weight loss, and tiredn ...
,
tuberculosis Tuberculosis (TB), also known colloquially as the "white death", or historically as consumption, is a contagious disease usually caused by ''Mycobacterium tuberculosis'' (MTB) bacteria. Tuberculosis generally affects the lungs, but it can al ...
, and
syphilis Syphilis () is a sexually transmitted infection caused by the bacterium ''Treponema pallidum'' subspecies ''pallidum''. The signs and symptoms depend on the stage it presents: primary, secondary, latent syphilis, latent or tertiary. The prim ...
.


Notable cases

*
Paul Allen Paul Gardner Allen (January 21, 1953 – October 15, 2018) was an American businessman, computer programmer, and investor. He co-founded Microsoft, Microsoft Corporation with his childhood friend Bill Gates in 1975, which was followed by the ...
, co-founder of Microsoft was diagnosed with Hodgkin lymphoma in 1982. He later died from non-Hodgkin lymphoma, in October 2018. *
Eric Berry James Eric Berry (born December 29, 1988) is an American former professional American football, football player who was a Safety (gridiron football position), safety for the Kansas City Chiefs of the National Football League (NFL). He played co ...
, All-Pro strong safety for the
Kansas City Chiefs The Kansas City Chiefs are a professional American football team based in Kansas City, Missouri. The Chiefs compete in the National Football League (NFL) as a member of the American Football Conference (AFC) West division. Established in 1959 ...
of the
National Football League The National Football League (NFL) is a Professional gridiron football, professional American football league in the United States. Composed of 32 teams, it is divided equally between the American Football Conference (AFC) and the National ...
, diagnosed in 2014. * David Brooks, Welsh professional footballer, diagnosed in 2021, while playing for
AFC Bournemouth AFC Bournemouth ( ) is a professional association football club based in Kings Park, Boscombe, a suburb of Bournemouth, Dorset, England. The club compete in the Premier League, the top tier of English football league system, English football. ...
. *
Dale Carnegie Dale Carnegie ( ; spelled Carnagey until c. 1922; November 24, 1888 – November 1, 1955) was an American writer and teacher of courses in self-improvement, salesmanship, corporate training, public speaking, and interpersonal skills. Born into ...
, public speaker and author of '' How to Win Friends and Influence People''. * Howard Carter, Egyptologist and discoverer of the Tomb of
Tutankhamun Tutankhamun or Tutankhamen, (; ), was an Egyptian pharaoh who ruled during the late Eighteenth Dynasty of Egypt, Eighteenth Dynasty of ancient Egypt. Born Tutankhaten, he instituted the restoration of the traditional polytheistic form of an ...
, died in 1939 from Hodgkin's disease. * Starchild Abraham Cherrix, a teenager whose refusal to undergo further conventional treatment after relapsing in 2006 resulted in a court battle and a change to Virginia laws about medical neglect. * James Conner,
running back A running back (RB) is a member of the offensive backfield in gridiron football. The primary roles of a running back are to receive American football plays#Offensive terminology, handoffs from the quarterback to Rush (American football)#Offense ...
and 2014 ACC Player of the Year for the
Arizona Cardinals The Arizona Cardinals are a professional American football team based in the Phoenix metropolitan area. The Cardinals compete in the National Football League (NFL) as a member of the National Football Conference (NFC) NFC West, West division. The ...
. * Michael Cuccione, Canadian child actor, was diagnosed in 1994 at age 9. Treatments that rendered him cancer-free, including chemotherapy, a bone marrow transplant, and radiation, left him with permanent lung and respiratory problems and he died in 2001 just after turning 16. * Victoria Duval, American tennis player, was diagnosed in 2014. * Gerald Finzi, British composer, was diagnosed in 1951 and died in 1956. * Mist Edvardsdóttir, Icelandic football player and member of the Icelandic women's national team. Diagnosed in June 2014 at the age of 23. Continued to play until becoming too ill due to chemotherapy. Made recovery in early 2015. *
Delta Goodrem Delta Lea Goodrem Order of Australia, AM (born 9 November 1984) is an Australian singer, songwriter, television personality and actress based in Sydney. Goodrem signed a recording contract with Sony Music at the age of 15. Her debut studio album ...
, Australian singer, songwriter, and actress. She was diagnosed in July 2003 at the age of 18. *
Hank Green William Henry Green II (born May 5, 1980) is an American YouTuber, science communicator, novelist, Stand-up comedy, stand-up comedian, and entrepreneur. He produces the YouTube channel Vlogbrothers with his older brother, author John Green, a ...
, one of the cofounders of the Vlogbrothers, VidCon and production company Complexly, announced that he was diagnosed with Hodgkin lymphoma in a video he released on 19 May 2023. Green would announce his entrance into remission on 25 August 2023. * Jiří Grossmann, Czechoslovak theatre actor, poet, and composer *
Michael C. Hall Michael Carlyle Hall (born February 1, 1971) is an American actor and musician. He is known for playing Dexter Morgan, the titular character in the Showtime (TV network), Showtime series ''Dexter (TV series), Dexter'' and David Fisher (Six Feet ...
(born 1 February 1971), American actor, best known for his lead role as
Dexter Morgan Dexter Morgan (born Dexter Moser), also known as The Bay Harbor Butcher, is a fictional serial killer and the antihero protagonist of the ''Dexter'' book series written by Jeff Lindsay (writer), Jeff Lindsay, as well as the Dexter (TV series), tel ...
, in Showtime's crime series '' Dexter''. In 2010, aged 38, Hall announced he was undergoing treatment for Hodgkin lymphoma; within two years, the disease was in full remission. *
Richard Harris Richard St John Francis Harris (1 October 1930 – 25 October 2002) was an Irish actor and singer. Having studied at the London Academy of Music and Dramatic Art, he rose to prominence as an icon of the British New Wave. He received numerous a ...
, Irish actor who portrayed Albus Dumbledore in the first two Harry Potter movies, died on 25 October 2002, after being diagnosed earlier that year. * Daniel Hauser, whose mother fled with him in 2009 in order to prevent him from undergoing chemotherapy. * Tessa James, Australian actress, was diagnosed in 2014. * Sean Kent, American
stand up comedian Stand-up comedy is a performance directed to a live audience, where the performer stands on a stage and delivers humorous and satirical monologues sometimes incorporating physical acts. These performances are typically composed of rehea ...
and actor. Was diagnosed in 2002 while writing on '' The Best Damn Sports Show Period''. After three months of chemotherapy and one month of radiation, the cancer went into remission. *
Mario Lemieux Mario Lemieux (; ; born October 5, 1965) is a Canadian former professional ice hockey player. He played parts of 17 seasons in the National Hockey League (NHL) for the Pittsburgh Penguins between 1984–85 NHL season, 1984 and 2005–06 NHL se ...
, Hall of Fame NHL player, co-owner of the
Pittsburgh Penguins The Pittsburgh Penguins (colloquially known as the Pens) are a professional ice hockey team based in Pittsburgh. The Penguins compete in the National Hockey League (NHL) as a member of the Metropolitan Division in the Eastern Conference (NHL), E ...
and founder of the Mario Lemieux Foundation, diagnosed in 1993. * Dinu Lipatti (1917–1950), Romanian classical pianist and composer. Diagnosed in 1947, received
cortisone Cortisone is a pregnene (21-carbon) steroid hormone. It is a naturally-occurring corticosteroid metabolite that is also used as a pharmaceutical prodrug. Cortisol is converted by the action of the enzyme corticosteroid 11-beta-dehydrogenase ...
treatment in 1949; died from a burst abscess on his one lung. *
Jack Lisowski Jack Lisowski (born 25 June 1991) is an English professional snooker player from Churchdown, Gloucestershire. He turned professional in 2010 by finishing first in the 2009/2010 International Open Series, PIOS rankings. A left-handed player, he ...
, English snooker player, diagnosed in 2008 at the age of 16. * Mamta Mohandas, Indian film actress and producer, diagnosed in 2010. * Nanni Moretti, Italian actor and director. * Laura Packard, health care activist diagnosed in 2017, spoke at the 2020 Democratic National Convention. * Nikola Pokrivač, Croatian soccer
midfielder In the sport of association football, a midfielder takes an Glossary of association football terms#O, outfield position primarily in the middle of the pitch. Midfielders may play an exclusively defensive role, breaking up attacks, and are in t ...
, diagnosed in 2015. * Anthony Rizzo, MLB All-Star
first baseman A first baseman, abbreviated 1B, is the player on a baseball or softball team who fields the area nearest first base, the first of four bases a baserunner must touch in succession to score a run. The first baseman is responsible for the majori ...
for the
New York Yankees The New York Yankees are an American professional baseball team based in the Boroughs of New York City, New York City borough of the Bronx. The Yankees compete in Major League Baseball (MLB) as a member club of the American League (AL) Am ...
, diagnosed in May 2008 while signed as a minor league player for the
Boston Red Sox The Boston Red Sox are an American professional baseball team based in Boston. The Red Sox compete in Major League Baseball (MLB) as a member club of the American League (AL) American League East, East Division. Founded in as one of the Ameri ...
. * Dave Roberts, MLB outfielder and manager of the
Los Angeles Dodgers The Los Angeles Dodgers are an American professional baseball team based in Los Angeles. The Dodgers compete in Major League Baseball (MLB) as a member club of the National League (baseball), National League (NL) National League West, West Div ...
. Diagnosed in March 2010 while he was a coach for the
San Diego Padres The San Diego Padres are an American professional baseball team based in San Diego. The Padres compete in Major League Baseball (MLB) as a member club of the National League (baseball), National League (NL) National League West, West Division. ...
. * Chip Roy, Texas congressman. *
Flip Saunders Philip Daniel "Flip" Saunders (February 23, 1955 – October 25, 2015) was an American basketball player and coach. During his career, he coached the La Crosse Catbirds, Minnesota Timberwolves, Detroit Pistons, and Washington Wizards. High schoo ...
,
head coach A head coach, senior coach, or manager is a professional responsible for training and developing athletes within a sports team. This role often has a higher public profile and salary than other coaching positions. In some sports, such as associat ...
of the NBA team
Minnesota Timberwolves The Minnesota Timberwolves (often referred to as the Wolves or T-wolves) are an American professional basketball team based in Minneapolis. The Timberwolves compete in the National Basketball Association (NBA) as a member of the Northwest Divisio ...
, announced in August 2015 that he was diagnosed with Hodgkin's disease. He died of the disease in October 2015. *
Arlen Specter Arlen Specter (February 12, 1930 – October 14, 2012) was an American lawyer, author and politician who served as a United States Senator from Pennsylvania from 1981 to 2011. Specter was a Democrat from 1951 to 1965, then a Republican fr ...
, United States Senator from Pennsylvania (1981–2011), diagnosed in 2005. He later died from non-Hodgkin lymphoma in 2012. *
Brandon Tartikoff Brandon Tartikoff (January 13, 1949 – August 27, 1997) was an American television executive who was head of the entertainment division of NBC from 1981 to 1991. He was credited with turning around NBC's low prime time reputation with several ...
, American television executive, diagnosed around 1974, died in 1997. * Bernardo Tengarrinha, Portuguese professional footballer, diagnosed in 2017 Tengarrinha died on 30 October 2021, at the age of 32. Hours later, his former teams FC Porto and Boavista FC paid tribute to him before the local derby. playing for
FC Porto Futebol Clube do Porto, Order of Prince Henry, MHIH, Order of Merit (Portugal), OM (), commonly known as FC Porto or simply Porto, is a Portuguese professional sports club based in Porto. It is best known for the professional association footbal ...
. * Ethan Zohn, American professional soccer player and a winner of the ''Survivor'' reality television series. Zohn was diagnosed twice (in 2009 and 2011). * Richard Holliday, American professional wrestler, reported to be ill with diagnosis from June 2022, completed treatment 17 February 2023


References


Further reading

* combines a biography of the American radiation oncologist (1918–84) with a history of the lymphatic cancer whose treatment he helped to transform.


External links


Hodgkin Lymphoma
at
American Cancer Society The American Cancer Society (ACS) is a nationwide non-profit organization dedicated to eliminating cancer. The ACS publishes the journals ''Cancer'', '' CA: A Cancer Journal for Clinicians'' and '' Cancer Cytopathology''. History The society w ...

Hodgkin Lymphoma
at the American
National Cancer Institute The National Cancer Institute (NCI) coordinates the United States National Cancer Program and is part of the National Institutes of Health (NIH), which is one of eleven agencies that are part of the U.S. Department of Health and Human Services. ...
{{DEFAULTSORT:Hodgkin Lymphoma Infectious causes of cancer Rare cancers Wikipedia medicine articles ready to translate Diseases named after discoverers