|
Lymphoproliferative Disorders
Lymphoproliferative disorders (LPDs) refer to a specific class of diagnoses, comprising a group of several conditions, in which lymphocytes are produced in excessive quantities. These disorders primarily present in patients who have a compromised immune system. Due to this factor, there are instances of these conditions being equated with " immunoproliferative disorders"; although, in terms of nomenclature, lymphoproliferative disorders are a subclass of immunoproliferative disorders—along with hypergammaglobulinemia and paraproteinemias. Lymphoproliferative disorders (examples) * Follicular lymphoma * Chronic lymphocytic leukemia * Acute lymphoblastic leukemia * Hairy cell leukemia * Hemophagocytic lymphohistiocytosis (HLH) * B-cell lymphomas * T-cell lymphomas * Multiple myeloma * Waldenström's macroglobulinemia * Wiskott–Aldrich syndrome * Langerhans cell histiocytosis (LCH) * Lymphocyte-variant hypereosinophilia * Pityriasis lichenoides (PL, PLC, PLVA) * Po ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Hematology
Hematology (American and British English spelling differences#ae and oe, spelled haematology in British English) is the branch of medicine concerned with the study of the cause, prognosis, treatment, and prevention of diseases related to blood. It involves treating diseases that affect the production of blood and its components, such as blood cells, hemoglobin, blood proteins, bone marrow, platelets, blood vessels, spleen, and the mechanism of coagulation. Such diseases might include hemophilia, sickle cell anemia, blood clots (thrombus), other bleeding disorders, and blood cancers such as leukemia, multiple myeloma, and lymphoma. The laboratory analysis of blood is frequently performed by a medical technologist or medical laboratory scientist. Specialization Physicians specialized in hematology are known as hematologists or haematologists. Their routine work mainly includes the care and treatment of patients with hematological diseases, although some may also work at the hema ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Multiple Myeloma
Multiple myeloma (MM), also known as plasma cell myeloma and simply myeloma, is a cancer of plasma cells, a type of white blood cell that normally produces antibody, antibodies. Often, no symptoms are noticed initially. As it progresses, bone pain, anemia, Kidney failure, renal insufficiency, and infections may occur. Complications may include hypercalcemia and amyloidosis. The cause of multiple myeloma is unknown. Risk factors include obesity, radiation exposure, family history, age and certain chemicals. There is an increased risk of multiple myeloma in certain occupations. This is due to the occupational exposure to aromatic hydrocarbon solvents having a role in causation of multiple myeloma. Multiple myeloma is the result of a multi-step malignant transformation, and almost universally originates from the pre-malignant stage monoclonal gammopathy of undetermined significance (MGUS). As MGUS evolves into MM, another pre-stage of the disease is reached, known as Smouldering ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
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), and innate lymphoid cells (ILCs; "innate T cell-like" cells involved in mucosal immunity and homeostasis), of which natural killer cells are an important subtype (which functions in cell-mediated, cytotoxic innate immunity). They are the main type of cell found in lymph, which prompted the name "lymphocyte" (with ''cyte'' meaning cell). Lymphocytes make up between 18% and 42% of circulating white blood cells. Types The three major types of lymphocyte are T cells, B cells and natural killer (NK) cells. They can also be classified as small lymphocytes and large lymphocytes based on their size and appearance. Lymphocytes can be identified by their large nucleus. T cells and B cells T cells (thymus cells) and B cells ( bone marrow- ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Primary Cutaneous Acral CD8 Positive T Cell Lymphoproliferative Disorder
Primary cutaneous acral CD8 positive T cell lymphoproliferative disorder (CD8+ TLPD) is one type of the lymphoproliferative disorders, subclass cutaneous T-cell lymphoma, in which a slow-growing nodule or papule develops on the ear or, less commonly, other acral sites. CD8 positive T-cells (i.e., CD8+ T-cells) are a subtype of the lymphocytes and acral sites are peripheral parts of the body, i.e., the hands, arms, feet, legs, ears, nose, fingernails, and toenails. In 2007, Petrella et al.,7 reported four patients with tumors composed of CD8+ T-cells and termed the disorder indolent CD8+ lymphoid proliferation of the ear. In 2018, the World Health Organization and European Organisation for Research and Treatment of Cancer provisionally classified this disorder as a lymphoma and termed it primary cutaneous acral CD8 positive T cell lymphoma. (The "primary" used to designate cutaneous lymphomas indicates that the lymphoma was first diagnosed as limited to the skin and there was no evide ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
X-linked Lymphoproliferative Disease
X-linked lymphoproliferative disease (also known as Duncan disease or Purtilo syndrome and abbreviated as XLP) is a lymphoproliferative disorder, usually caused by SH2DIA gene mutations in males. XLP-positive individuals experience immune system deficiencies that render them unable to effectively respond to the Epstein-Barr virus (EBV), a common virus in humans that typically induces mild symptoms or infectious mononucleosis (IM) in patients. There are two currently known variations of the disorder, known as XLP1 (XLP Type 1) and XLP2. XLP1 is estimated to occur in approximately one in every million males, while XLP2 is rarer, estimated to occur in one of every five million males. Due to therapies such as chemotherapy and stem cell transplants, the survival rate of XLP1 has increased dramatically since its discovery in the 1970s. Presentation In boys with X-linked lymphoproliferative disorder, the inability to mount an immune response to EBV may lead to death via hemophagocytic ly ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Castleman Disease
Castleman disease (CD) describes a group of rare lymphoproliferative disorders that involve enlarged lymph nodes, and a broad range of inflammatory symptoms and laboratory abnormalities. Whether Castleman disease should be considered an autoimmune disease, cancer, or infectious disease is currently unknown. Castleman disease includes at least three distinct subtypes: unicentric Castleman disease (UCD), human herpesvirus 8 associated multicentric Castleman disease (HHV-8-associated MCD), and idiopathic multicentric Castleman disease (iMCD). These are differentiated by the number and location of affected lymph nodes and the presence of human herpesvirus 8, a known causative agent in a portion of cases. Correctly classifying the Castleman disease subtype is important, as the three subtypes vary significantly in symptoms, clinical findings, disease mechanism, treatment approach, and prognosis. All forms involve overproduction of cytokines and other inflammatory proteins by the body ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Lymphoid Interstitial Pneumonia
Lymphocytic interstitial pneumonia (LIP) is a syndrome secondary to autoimmune and other lymphoproliferative disorders. Symptoms include fever, cough, and shortness of breath. Lymphocytic interstitial pneumonia applies to disorders associated with both monoclonal or polyclonal gammopathy. Signs and symptoms Patients with lymphocytic interstitial pneumonia may present with lymphadenopathy, enlarged liver, enlarged spleen, enlarged salivary gland, thickening and widening of the extremities of the fingers and toes ( clubbing), and breathing symptoms such as shortness of breath and wheezing. Causes Possible causes of lymphocytic interstitial pneumonia include the 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 ..., auto-immune, and HIV. Diagnosis Arte ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Autoimmune Lymphoproliferative Syndrome
Autoimmune lymphoproliferative syndrome (ALPS) is a form of lymphoproliferative disorder (LPDs). It affects lymphocyte apoptosis. It is a rare genetic disorder of abnormal lymphocyte survival caused by defective Fas mediated apoptosis. Normally, after infectious insult, the immune system down-regulates by increasing Fas expression on activated B and T lymphocytes and Fas-ligand on activated T lymphocytes. Fas and Fas-ligand interact to trigger the caspase cascade, leading to cell apoptosis. Patients with ALPS have a defect in this apoptotic pathway, leading to chronic non-malignant lymphoproliferation, autoimmune disease, and secondary cancers. Signs and symptoms All people with ALPS have signs of lymphoproliferation, which makes it the most common clinical manifestation of the disease. The increased proliferation of lymphoid cells can cause the size of lymphoid organs such as the lymph nodes and spleen to increase (lymphadenopathy and splenomegaly, present in respectively over 9 ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Post-transplant Lymphoproliferative Disorder
Post-transplant lymphoproliferative disorder (PTLD) is the name given to a B cell proliferation due to therapeutic immunosuppression after organ transplantation. These patients may develop infectious mononucleosis-like lesions or polyclonal polymorphic B-cell hyperplasia. Some of these B cells may undergo mutations which will render them malignant, giving rise to a lymphoma. In some patients, the malignant cell clone can become the dominant proliferating cell type, leading to frank lymphoma, a group of B cell lymphomas occurring in immunosuppressed patients following organ transplant. Signs and symptoms Symptoms of PTLD are highly variable and nonspecific, and may include fever, weight loss, night sweats, and fatigue. Symptoms may be similar to those seen in infectious mononucleosis (caused by EBV). Pain or discomfort may result from lymphadenopathy or mass effect from growing tumors. Dysfunction may occur in organs affected by PTLD. Lung or heart involvement may result in shortne ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Pityriasis Lichenoides
Pityriasis lichenoides represents a distinct subset of inflammatory skin disorders that includes pityriasis lichenoides chronica, febrile ulceronecrotic Mucha-Habermann disease, and pityriasis lichenoides et varioliformis acuta (PLEVA). PLEVA typically manifests as an acute to subacute skin eruption of several tiny red papules that grow into polymorphic lesions. It may also leave behind varicella-like scars and hyper- or hypopigmentation sequelae. Pityriasis lichenoides chronica (PLC) has very small reddish-brown flat maculopapules with a mica-like scale that appear more gradually; it also has long remission intervals between episodes of relapse. Febrile ulceronecrotic Mucha-Habermann disease (FUMHD) is best treated as a dermatological emergency because it is an acute, severe, widespread eruption of purpuric and ulceronecrotic plaques that can have a 25% fatality rate and accompanying systemic involvement. Signs and symptoms The characteristic feature of PLEVA is the rapid evol ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Lymphocyte-variant Hypereosinophilia
Lymphocyte-variant hypereosinophilia is a rare disorder in which eosinophilia or hypereosinophilia (i.e. a large or extremely large increase in the number of eosinophils in the blood circulation) is caused by an aberrant population of lymphocytes. These aberrant lymphocytes function abnormally by stimulating the proliferation and maturation of Eosinophil#Development, bone marrow eosinophil-precursor cells termed CFU-Eos, colony forming unit-eosinophils or CFU-Eos. The overly stimulated CFU-Eos cells mature to apparently normal appearing but possibly overactive eosinophils which enter the circulation and may accumulate in and damage various tissues. The disorder is usually indolent or slowly progressive but may proceed to a leukemic phase sometimes classified as acute eosinophilic leukemia. Lymphocyte-variant hypereosinophilia can therefore be regarded as a Precancerous condition, precancerous disorder. The disorder merits therapeutic intervention to avoid or reduce eosinophil-induc ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
