Hodgkin's Paragranuloma
   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). In current usage the name usually refers to just the cancerous versions rather than all such tumours. Signs and symptoms may include enlar ...
, in which
cancer Cancer is a group of diseases involving abnormal cell growth with the potential to invade or spread to other parts of the body. These contrast with benign tumors, which do not spread. Possible signs and symptoms include a lump, abnormal b ...
originates from a specific type of
white blood cell White blood cells, also called leukocytes or leucocytes, are the cell (biology), cells of the immune system that are involved in protecting the body against both infectious disease and foreign invaders. All white blood cells are produced and de ...
called
lymphocyte A lymphocyte is a type of white blood cell (leukocyte) in the immune system of most vertebrates. Lymphocytes include natural killer cells (which function in cell-mediated, cytotoxic innate immunity), T cells (for cell-mediated, cytotoxic ad ...
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, classically con ...
s (RS cells) are present in the patient's lymph nodes. The condition was named after the English physician Thomas Hodgkin, who first described it in 1832. Symptoms may include
fever Fever, also referred to as pyrexia, is defined as having a body temperature, temperature above the human body temperature, normal range due to an increase in the body's temperature Human body temperature#Fever, set point. There is not a single ...
, night sweats, 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, nonpainful
enlarged lymph nodes 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 cli ...
occur in the neck, under the arm, or in the groin. Those 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), formally called ''Human gammaherpesvirus 4'', is one of the nine known human herpesvirus types in the herpes family, and is one of the most common viruses in humans. EBV is a double-stranded DNA virus. It is b ...
(EBV) and these are generally the classic form. Other risk factors include a family history of the condition and having
HIV/AIDS Human immunodeficiency virus infection and acquired immunodeficiency syndrome (HIV/AIDS) is a spectrum of conditions caused by infection with the human immunodeficiency virus (HIV), a retrovirus. Following initial infection an individual ...
. Diagnosis is conducted by confirming the presence of cancer and identifying RS 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 to chemo and sometimes CTX or CTx) is a type of cancer treatment that uses one or more anti-cancer drugs (chemotherapeutic agents or alkylating agents) as part of a standardized chemotherapy regimen. Chemotherap ...
,
radiation therapy Radiation therapy or radiotherapy, often abbreviated RT, RTx, or XRT, is a therapy using ionizing radiation, generally provided as part of cancer treatment to control or kill malignant cells and normally delivered by a linear accelerator. Radia ...
, and
stem cell transplantation Hematopoietic stem-cell transplantation (HSCT) is the transplantation of multipotent hematopoietic stem cells, usually derived from bone marrow, peripheral blood, or umbilical cord blood in order to replicate inside of a patient and to produce ...
. 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 a class of diseases that involve the heart or blood vessels. CVD includes coronary artery diseases (CAD) such as angina and myocardial infarction (commonly known as a heart attack). Other CVDs include stroke, hea ...
, 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 the following 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 cli ...
: the most common symptom of Hodgkin is the painless enlargement of one or more
lymph nodes 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 ...
. The nodes may also feel rubbery and swollen when examined. The nodes of the neck, armpits and groin (
cervical In anatomy, cervical is an adjective that has two meanings: # of or pertaining to any neck. # of or pertaining to the female cervix: i.e., the ''neck'' of the uterus. *Commonly used medical phrases involving the neck are **cervical collar **cervic ...
and
supraclavicular The supraclavicular nerves (descending branches) arise from the third and fourth cervical nerves. They emerge beneath the posterior border of the sternocleidomastoideus (sternocleidomastoid muscle), and descend in the posterior triangle of the n ...
) 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, called a 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 med ...
. * Systemic symptoms: about one-third of people with Hodgkin disease may also present with systemic symptoms, including: ** Itchy skin ** Night sweats. ** 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, also referred to as pyrexia, is defined as having a body temperature, temperature above the human body temperature, normal range due to an increase in the body's temperature Human body temperature#Fever, set point. There is not a single ...
. **
Fatigue Fatigue describes a state of tiredness that does not resolve with rest or sleep. In general usage, fatigue is synonymous with extreme tiredness or exhaustion that normally follows prolonged physical or mental activity. When it does not resolve ...
(lassitude). ** Systemic symptoms such as fever, night sweats, and weight loss are known as
B symptoms B symptoms are a set of symptoms, namely fever, night sweats, and unintentional weight loss, that can be associated with both Hodgkin lymphoma and non-Hodgkin lymphoma. These symptoms are not specific to lymphomas, especially each one considered ...
; thus, presence of these indicate that the person's stage is, for example, 2B instead of 2A. * Splenomegaly: enlargement of the
spleen The spleen is an organ found in almost all vertebrates. Similar in structure to a large lymph node, it acts primarily as a blood filter. The word spleen comes .
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: enlargement of the
liver The liver is a major Organ (anatomy), organ only found in vertebrates which performs many essential biological functions such as detoxification of the organism, and the Protein biosynthesis, synthesis of proteins and biochemicals necessary for ...
, due to liver involvement, is infrequent in people with Hodgkin Lymphoma. * Hepatosplenomegaly: the enlargement of both the liver and spleen 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 The positive and negative predictive values (PPV and NPV respectively) are the proportions of positive and negative results in statistics and diagnostic tests that are true positive and true negative results, respectively. The PPV and NPV descr ...
is high enough for it to be regarded as a pathognomonic 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: 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 Pel–Ebstein fever is a rarely seen condition noted in patients with Hodgkin's lymphoma Hodgkin lymphoma (HL) is a type of lymphoma, in which cancer originates from a specific type of white blood cell called lymphocytes, where multinucleated R ...
, 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 protein in the urine, low blood albumin levels, high blood lipids, and significant swelling. Other symptoms may include weight gain, feeling tired, and foamy ...
can occur in individuals with Hodgkin lymphoma and is most commonly caused by
minimal change disease Minimal change disease (also known as MCD, minimal change glomerulopathy, and nil disease, among others) is a disease affecting the kidneys which causes a nephrotic syndrome. Nephrotic syndrome leads to the loss of significant amounts of protein ...
. * May present with airway obstruction, pleural/pericardial effusion, hepatocellular dysfunction, bone marrow infiltration.


Diagnosis

Hodgkin lymphoma must be distinguished from non-cancerous 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, or an interventional cardiologist. The process involves extraction of sample cells or tissues for examination to determine the presence or extent of a diseas ...
(usually excisional biopsy with microscopic examination).
Blood test A blood test is a 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 test or a cholester ...
s are also performed to assess function of major organs and to assess safety for
chemotherapy Chemotherapy (often abbreviated to chemo and sometimes CTX or CTx) is a type of cancer treatment that uses one or more anti-cancer drugs (chemotherapeutic agents or alkylating agents) as part of a standardized chemotherapy regimen. Chemotherap ...
.
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 Metabolism, metabolic processes, and in other physiological activities including bl ...
(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

There are two main types of Hodgkin lymphoma: classic Hodgkin lymphoma and nodular lymphocyte predominant Hodgkin lymphoma. The prevalence of classic Hodgkin lymphoma and nodular lymphocyte Hodgkin lymphoma are approximately 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 inclu ...
biopsy A biopsy is a medical test commonly performed by a surgeon, interventional radiologist, or an interventional cardiologist. The process involves extraction of sample cells or tissues for examination to determine the presence or extent of a diseas ...
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 B cells, also known as B lymphocytes, are a type of white blood cell of the lymphocyte subtype. They function in the humoral immunity component of the adaptive immune system. B cells produce antibody molecules which may be either secreted o ...
markers (such as CD20) are not expressed on all cells, Reed–Sternberg cells are usually of B cell origin. Although Hodgkin's 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 T helper cells, monocytes, macrophages, and dendritic ...
) are occasionally expressed. However, this may be an artifact of the ambiguity inherent in the diagnosis. Hodgkin cells produce
interleukin-21 Interleukin 21 (IL-21) is a protein that in humans is encoded by the ''IL21'' gene. Interleukin-21 is a cytokine that has potent regulatory effects on cells of the immune system, including natural killer (NK) cells and cytotoxic T cells that can ...
(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. Due to these differences, among others, NLPHL is often treated differently from classic Hodgkin lymphoma, including using rituximab 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 ''Staged'' is a British television comedy series, set during the COVID-19 pandemic in the United Kingdom and primarily filmed using video-conferencing technology. The first series premiered on 10 June 2020 on BBC One, and the second series prem ...
'': that is, they will undergo a series of tests and procedures that will determine what areas of the body are affected. These procedures may include documentation of their histology, a physical examination, blood tests, chest X-ray radiographs,
computed tomography A computed tomography scan (CT scan; formerly called computed axial tomography scan or 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 ...
(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 Metabolism, metabolic processes, and in other physiological activities including bl ...
(PET)/
magnetic resonance imaging Magnetic resonance imaging (MRI) is a medical imaging technique used in radiology to form pictures of the anatomy and the physiological processes of the body. MRI scanners use strong magnetic fields, magnetic field gradients, and radio wave ...
(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 Lymphography is a medical imaging technique in which a radiocontrast agent is injected, and then an X-ray picture is taken to visualise structures of the lymphatic system, including lymph nodes, lymph ducts, lymphatic tissues, lymph capillarie ...
or surgical laparotomy (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 Diaphragm may refer to: Anatomy * Thoracic diaphragm, a thin sheet of muscle between the thorax and the abdomen * Pelvic diaphragm or pelvic floor, a pelvic structure * Urogenital diaphragm or triangular ligament, a pelvic structure Other * Diap ...
(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 is an organ found in almost all vertebrates. Similar in structure to a large lymph node, it acts primarily as a blood filter. The word spleen comes .
(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 localised 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 A fibrin ring granuloma, also known as doughnut granuloma, is a histopathological finding that is characteristic of Q fever. On hematoxylin-eosin staining, the fibrin ring granuloma consists of a central lipid vacuole (usually washed-out during ...
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 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: the treatment and prognosis in classic Hodgkin lymphoma usually depends on the stage of disease rather than the histotype.


Management

The current 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) are effectively treated with
radiation therapy Radiation therapy or radiotherapy, often abbreviated RT, RTx, or XRT, is a therapy using ionizing radiation, generally provided as part of cancer treatment to control or kill malignant cells and normally delivered by a linear accelerator. Radia ...
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 (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 recently reviewed. The evidence is very uncertain about the effect of Nivolumab for patients with a Hodgkin's 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. Recently, 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 A mantle is a piece of clothing, a type of cloak. Several other meanings are derived from that. Mantle may refer to: *Mantle (clothing), a cloak-like garment worn mainly by women as fashionable outerwear **Mantle (vesture), an Eastern Orthodox ve ...
. 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 leukemias, 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 agents 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's disease has been improving over the past few decades. Recent trials that have made use of new types of chemotherapy have indicated higher survival rates than have previously been seen. In one recent 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 < 10.5 g/dl *
Lymphocyte count A lymphocyte is a type of white blood cell (leukocyte) in the immune system of most vertebrates. Lymphocytes include natural killer cells (which function in cell-mediated, cytotoxic innate immunity), T cells (for cell-mediated, cytotoxic adapt ...
< 600/µl or < 8% * Male * Albumin < 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 ) is the central compartment of the thoracic cavity. Surrounded by loose connective tissue, it is an undelineated region that contains a group of structures within the thorax, namely the heart and its vessels, the esophagu ...
by more than one third, or the presence of a nodal mass measuring more than 10 cm in any dimension.) More recently, 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 very uncertain about the effect of negative (= good prognosis) or positive (= bad prognosis) interim PET scan results for patients with a Hodgkin's 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 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 old 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
HIV The human immunodeficiency viruses (HIV) are two species of '' Lentivirus'' (a subgroup of retrovirus) that infect humans. Over time, they cause acquired immunodeficiency syndrome (AIDS), a condition in which progressive failure of the immu ...
infection. In contrast to many other lymphomas associated with HIV infection it occurs most commonly in people with higher
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 T helper cells, monocytes, macrophages, and dendritic ...
T cell counts.


Canada

Hodgkin lymphoma accounts for 0.6% of all male cancer cases, and 0.4% of all female cancer cases in Canada. In 2017, approximately 990 Canadians will be diagnosed with Hodgkin lymphoma, and 140 will die of the disease.


UK

Hodgkin lymphoma accounts for less than 1% of all cancer cases and deaths in the UK. 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 1, 2016, the 5-year limited duration prevalence of Hodgkin Lymphoma was 37,513 representing 0.71% of all diagnosed cancers in the U.S.


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 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 Robert Carswell may refer to: * Robert Carswell, Baron Carswell (1934–2023), British law lord * Robert Carswell (cricketer) (born 1936), New Zealand cricketer * Robert Carswell (MP) for Wallingford (UK Parliament constituency) * 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, tuberculosis, and
syphilis Syphilis () is a sexually transmitted infection caused by the bacterium ''Treponema pallidum'' subspecies ''pallidum''. The signs and symptoms of syphilis vary depending in which of the four stages it presents (primary, secondary, latent, an ...
. Hodgkin lymphoma was one of the first cancers to be treated successfully with
radiation therapy Radiation therapy or radiotherapy, often abbreviated RT, RTx, or XRT, is a therapy using ionizing radiation, generally provided as part of cancer treatment to control or kill malignant cells and normally delivered by a linear accelerator. Radia ...
and, later, it was one of the first to be treated by combination chemotherapy.


Notable cases

* Paul Allen, co-founder of Microsoft was diagnosed with Hodgkin lymphoma in 1982. He later died from non-Hodgkin lymphoma, on October 15, 2018. * Eric Berry, All-Pro
strong safety Safety is a position in gridiron football on the defense. The safeties are defensive backs who line up ten to fifteen yards from the line of scrimmage. There are two variations of the position: the free safety and the strong safety. Their dut ...
for the Kansas City Chiefs of the National Football League, diagnosed in 2014. *
Howard Carter Howard Carter (9 May 18742 March 1939) was a British archaeologist and Egyptologist who discovered the intact tomb of the 18th Dynasty Pharaoh Tutankhamun in November 1922, the best-preserved pharaonic tomb ever found in the Valley of the K ...
, Egyptologist and discoverer of the Tomb of Tutankhamun, died in 1939 from Hodgkin's disease. *
Starchild Abraham Cherrix Virginia v. Cherrix is a 2006 court case in which the Commonwealth of Virginia sued to force Starchild Abraham ("Wolf") Cherrix (born June 1990), aged 16 at the time of the court case, to undergo further conventional medical treatment for a highly ...
, 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 and 2014 ACC Player of the Year for the
Pittsburgh Steelers The Pittsburgh Steelers are a professional American football team based in Pittsburgh. The Steelers compete in the National Football League (NFL) as a member club of the American Football Conference (AFC) North division. Founded in , the Steel ...
. * 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 Victoria Duval (born 30 November 1995) is an American professional tennis player. Duval has won one singles title and two doubles titles on the ITF Women's Circuit. In August 2014, she reached her best singles ranking of No. 87. In 2012, she w ...
, American tennis player, was diagnosed in 2014. *
Gerald Finzi Gerald Raphael Finzi (14 July 1901 – 27 September 1956) was a British composer. Finzi is best known as a choral composer, but also wrote in other genres. Large-scale compositions by Finzi include the cantata '' Dies natalis'' for solo voice and ...
, British composer, was diagnosed in 1951 and died in 1956. *
Mist Edvardsdóttir Mist Edvardsdóttir (born 17 October 1990) is an Icelandic footballer who plays as a defender for Valur in the Icelandic top-tier Úrvalsdeild kvenna. She was a member of the Icelandic national team, appearing in 13 matches from 2010 to 2015. ...
, 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 AM (born November 9, 1984) is an Australian singer, songwriter, and actress. Goodrem signed to Sony Music at the age of 15. Her debut album, '' Innocent Eyes'' (2003), topped the ARIA Albums Chart for 29 non-consecutive week ...
, Australian singer, songwriter, and actress. She was diagnosed in July 2003 at the age of 18. * Jiří Grossmann, Czechoslovak theatre actor, poet, and composer * Michael C. Hall (born February 1, 1971), American actor, best known for his lead role as Dexter Morgan, in Showtime's crime series ''
Dexter Dexter may refer to: Arts and entertainment * Dexter, the main character of the American animated series ''Dexter's Laboratory'' that aired from 1996 to 2003 * Dexter, a fictional character in the British Diary of a Bad Man#Main, web series ''Diar ...
''. In 2010, aged 38, Hall announced he was undergoing treatment for Hodgkin lymphoma; within two years, the disease was in full remission. * Richard Harris, Irish actor who portrayed Albus Dumbledore in the first two Harry Potter movies, died on October 25, 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 and actor. Was diagnosed in 2002 while writing on a TV show. After three months in remission the cancer returned and he was given an autologous stem cell transplant at City of Hope Cancer Hospital in Los Angeles. Currently lives in Texas with his wife and family and still performs many weeks a year. * Mario Lemieux, Hall of Fame NHL player, co-owner of the Pittsburgh Penguins and founder of the Mario Lemieux Foundation, diagnosed in 1993. * Dinu Lipatti (1917–1950), Romanian classical pianist and composer. Diagnosed in 1947, received cortisone treatment in 1949; died from a burst abscess on his one lung. * Jack Lisowski, 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 The 2020 Democratic National Convention was a presidential nominating convention that was held from August 17 to 20, 2020, at the Wisconsin Center in Milwaukee, Wisconsin, and virtually across the United States. At the convention, delegates of ...
. * Nikola Pokrivač, Croatian soccer midfielder, diagnosed in 2015. * Anthony Rizzo, MLB All-Star first baseman for the New York Yankees, diagnosed in May 2008 while signed as a minor league player for the Boston Red Sox. *
Dave Roberts David or Dave Roberts may refer to: Arts and literature * David Roberts (painter) (1796–1864), Scottish painter * David Roberts (art collector), Scottish contemporary art collector * David Roberts (novelist), English editor and mystery writer ...
, MLB outfielder and manager of the Los Angeles Dodgers. Diagnosed in March 2010 while he was a coach for the San Diego Padres. * Chip Roy, Texas congressman. * Flip Saunders, head coach of the
NBA The National Basketball Association (NBA) is a professional basketball league in North America. The league is composed of 30 teams (29 in the United States and 1 in Canada) and is one of the major professional sports leagues in the United St ...
team Minnesota Timberwolves, announced in August 2015 that he was diagnosed with Hodgkin's disease. He died of the disease in October 2015. * Brandon Tartikoff, American television executive, diagnosed around 1974, died in 1997. *
Ethan Zohn Ethan Zohn (born November 12, 1973) is an American motivational speaker, former professional soccer player, and reality television series contestant who won '' Survivor: Africa'', the third season of the reality TV series ''Survivor''. He went o ...
, American professional soccer player and a winner of the ''Survivor'' reality television series. Zohn was diagnosed twice (in 2009 and 2011). * Arlen Specter, United States Senator from Pennsylvania (1981 - 2011), diagnosed in 2005. He later died from non-Hodgkin lymphoma in 2012. * 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 competes in the Premier League, the highest division of English club football. Formed in 1899 as B ...
. *
Bernardo Tengarrinha Bernardo David Mendes Salgueiro Campos Tengarrinha (17 February 1989 – 30 October 2021) was a Portuguese professional footballer who played as a centre-back or a defensive midfielder. He made 106 Primeira Liga appearances for Estrela da Amador ...
, Portuguese professional footballer, diagnosed in 2017 Tengarrinha died on October 30, 2021, at the age of 32. 1Hours 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, MHIH, 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 football team playing in the Primeira Liga, the top flight of Portu ...
.


References


Further reading

* Charlotte DeCroes Jacobs. ''Henry Kaplan and the Story of Hodgkin's Disease'' (
Stanford University Press Stanford University Press (SUP) is the publishing house of Stanford University. It is one of the oldest academic presses in the United States and the first university press to be established on the West Coast. It was among the presses officially ...
; 2010) 456 pages; 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 voluntary health organization dedicated to eliminating cancer. Established in 1913, the society is organized into six geographical regions of both medical and lay volunteers operating in more than ...

Hodgkin Lymphoma
at the American National Cancer Institute {{DEFAULTSORT:Hodgkin Lymphoma Infectious causes of cancer Rare cancers Wikipedia medicine articles ready to translate