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Encyclopedia > Essential thrombocytosis
Essential thrombocytosis
Classification & external resources
ICD-10 D75.2, D47.3
ICD-9 289.9
ICD-O: 9962/3
OMIM 187950
DiseasesDB 4522
MedlinePlus 000543
eMedicine med/2266 
MeSH D013920

Essential thrombocytosis (ET, also known as essential thrombocythemia) is a rare chronic blood disorder characterized by the overproduction of platelets by megakaryocytes in the bone marrow in the absence of an alternative cause. In some cases this disorder may be progressive, and rarely may evolve into acute myeloid leukemia or myelofibrosis. It is one of four myeloproliferative disorders. The International Statistical Classification of Diseases and Related Health Problems (most commonly known by the abbreviation ICD) provides codes to classify diseases and a wide variety of signs, symptoms, abnormal findings, complaints, social circumstances and external causes of injury or disease. ... The International Statistical Classification of Diseases and Related Health Problems 10th Revision (ICD-10) is a coding of diseases and signs, symptoms, abnormal findings, complaints, social circumstances and external causes of injury or diseases, as classified by the World Health Organization (WHO). ... // C00-D48 - Neoplasms (C00-C14) Malignant neoplasms, lip, oral cavity and pharynx (C00) Malignant neoplasm of lip (C01) Malignant neoplasm of base of tongue (C02) Malignant neoplasm of other and unspecified parts of tongue (C03) Malignant neoplasm of gum (C04) Malignant neoplasm of floor of mouth (C05) Malignant neoplasm of... // C00-D48 - Neoplasms (C00-C14) Malignant neoplasms, lip, oral cavity and pharynx (C00) Malignant neoplasm of lip (C01) Malignant neoplasm of base of tongue (C02) Malignant neoplasm of other and unspecified parts of tongue (C03) Malignant neoplasm of gum (C04) Malignant neoplasm of floor of mouth (C05) Malignant neoplasm of... The International Statistical Classification of Diseases and Related Health Problems (most commonly known by the abbreviation ICD) provides codes to classify diseases and a wide variety of signs, symptoms, abnormal findings, complaints, social circumstances and external causes of injury or disease. ... The following is a list of codes for International Statistical Classification of Diseases and Related Health Problems. ... The International Classification of Diseases for Oncology (ICD-O) is a domain specific extension of the International Statistical Classification of Diseases and Related Health Problems for tumor diseases. ... The Mendelian Inheritance in Man project is a database that catalogues all the known diseases with a genetic component, and - when possible - links them to the relevant genes in the human genome. ... The Disease Bold textDatabase is a free website that provides information about the relationships between medical conditions, symptoms, and medications. ... MedlinePlus (medlineplus. ... eMedicine is an online clinical medical knowledge base that was founded in 1996. ... Medical Subject Headings (MeSH) is a huge controlled vocabulary (or metadata system) for the purpose of indexing journal articles and books in the life sciences. ... The myeloproliferative diseases (MPDs) are a group of diseases of the bone marrow where excess cells are produced. ... A 250 ml bag of newly collected platelets. ... The megakaryocyte is a bone marrow cell responsible for the production of blood platelets when its cytoplasm becomes fragmented. ... This article does not cite any references or sources. ... Acute myeloid leukemia (AML), also known as acute myelogenous leukemia, is a cancer of the myeloid line of white blood cells, characterized by the rapid proliferation of abnormal cells which accumulate in the bone marrow and interfere with the production of normal blood cells. ... Myelofibrosis with myeloid metaplasia, also known as agnogenic myeloid metaplasia, chronic idiopathic myelofibrosis, and primary myelofibrosis,[1] was first described in 1879 and is currently classified as a myeloproliferative disease caused by the growth and proliferation of an abnormal bone marrow stem cell, resulting in the replacement of the bone... The myeloproliferative diseases (MPDs) are a group of diseases of the bone marrow where excess cells are produced. ...

Contents

Epidemiology

Essential thrombocytosis is diagnosed at a rate of about 2 to 3 per 100,000 individuals annually.[1][2] The disease usually affects middle aged to elderly individuals, with an average age at diagnosis of 50-60 years, although it can affect children and young adults as well.[3]


Pathophysiology

The pathologic basis for this disease is unknown. However, essential thrombosis resembles polycythemia vera in that cells of the megakaryocytic series are more sensitive to growth factors. Platelets derived from the abnormal megakaryocytes do not function properly, which contributes to the clinical features of bleeding and thrombosis. Polycythemia is a condition in which there is a net increase in the total circulating erythrocyte (red blood cell) mass of the body. ... The megakaryocyte is a bone marrow cell responsible for the production of blood platelets when its cytoplasm becomes fragmented. ... Growth factor is any of about twenty small proteins that attach to specific receptors on the surface of stem cells in bone marrow and promote differentiation and maturation of these cells into morphotic constituents of blood. ...


In 2005, a mutation in the JAK2 kinase (V617F) was found by multiple research groups [4][5] [6] to be associated with essential thrombocytosis in around 30% of cases. JAK2 is a member of the Janus kinase family. This mutation may be helpful in making a diagnosis or as a target for future therapy. JAK2 (Janus kinase 2 (a protein tyrosine kinase)) is a human gene that encodes for a protein called Janus kinase 2, a tyrosine kinase of the non-receptor type, involved in interleukin 3 signal transduction. ... Janus kinase< (JAK) is a family of intracellular non-receptor tyrosine kinases, ranging from 120-140 kDa, that transduce cytokine-mediated signals via the JAK-STAT pathway. ...


Clinical features

The major symptoms are bleeding and thrombosis. Other symptoms include epistaxis (nosebleeds) and bleeding from gums and gastrointestinal tract. One characteristic symptom is throbbing and burning of the hands and feet due to the occlusion of small arterioles by platelets (erythromelalgia). An enlarged spleen (splenomegaly) may be found on examination. Nosebleed as a result of fracture through a rugby impact. ... To meet Wikipedias quality standards, this article or section may require cleanup. ... Erythromelalgia, also known as Mitchells disease (after Silas Weir Mitchell) and red neuralgia(also referred to as man on fire), is a rare disorder in which blood vessels, usually in the extremities and especially in the feet, are blocked and inflamed, causing a painful burning and throbbing sensation and... Splenomegaly is an enlargement of the spleen, which usually lies in the left upper quadrant (LUQ) of the human abdomen. ...


Diagnostic criteria

The diagnosis of essential thrombocytosis requires the presence of a persistent thrombocytosis of greater than 600 x109/L in the absence of an alternative cause.


The following revised diagnostic criteria for essential thrombocytosis were proposed in 2005 [7]. The diagnosis requires the presence of both A criteria together with B3 to B6, or of criterion A1 together with B1 to B6.

  • A1. Platelet count > 600 x 109/L for at least 2 months
  • A2. Acquired V617F JAK2 mutation present
  • B1. No cause for a reactive thrombocytosis
    • normal inflammatory indices
  • B2. No evidence of iron deficiency
    • stainable iron in the bone marrow or normal red cell mean corpuscular volume
  • B3. No evidence of polycythemia vera
    • hematocrit < midpoint of normal range or normal red cell mass in presence of normal iron stores
  • B4. No evidence of chronic myeloid leukemia

But the Philadelphia chromosome may be present in up to 10% of cases. Patients withe the Philadelphia chromosome have a potential for the development of acute leukemia, especially acute lymphocytic leukemia. Polycythemia is a condition in which there is a net increase in the total circulating erythrocyte (red blood cell) mass of the body. ...

  • B5. No evidence of myelofibrosis
    • no collagen fibrosis and ≤ grade 2 reticulin fibrosis (using 0–4 scale)
  • B6. No evidence of a myelodysplastic syndrome
    • no significant dysplasia
    • no cytogenetic abnormalities suggestive of myelodysplasia

Myelofibrosis with myeloid metaplasia, also known as agnogenic myeloid metaplasia, chronic idiopathic myelofibrosis, and primary myelofibrosis,[1] was first described in 1879 and is currently classified as a myeloproliferative disease caused by the growth and proliferation of an abnormal bone marrow stem cell, resulting in the replacement of the bone...

Treatment

Not all patients will require treatment at presentation. In those who are at increased risk of thrombosis or bleeding (older age, prior history of bleeding or thrombosis, or very high platelet count), reduction of the platelet count to the normal range can be achieved using hydroxyurea (also known as hydroxycarbamide), interferon-α or anagrelide. Low-dose aspirin is widely used to reduce the risk of thrombosis, but there may be an increased risk of bleeding if aspirin is initiated whilst the platelet count is very high. Hydroxyurea chemical structure Hydroxyurea or hydroxycarbamide (rINN), (brand names include Hydrea®) is an antineoplastic drug used in hematological malignancies. ... Anagrelide (Agrylin®/Xagrid®, Shire) is a drug used for the treatment of essential thrombocytosis (ET; essential thrombocythemia). ...


The PT1 study [8] compared hydroxyurea in combination with aspirin to anagrelide in combination with Aspirin as initial therapy for essential thrombocytosis. Hydroxyurea was superior, with lower risk of arterial thrombosis, lower risk of severe bleeding and lower risk of transformation to myelofibrosis (although the rate of venous thrombosis was higher with hydroxycarbamide than with anagrelide).


In rare cases where patients have life-threatening complications, the platelet count can be reduced rapidly using platelet apheresis (a procedure that removes platelets from the blood directly). Whole blood enters the centrifuge on the left and separates into layers so that selected components can be drawn off on the right. ...


Prognosis

Essential thrombocytosis is a slowly progressive disorder with long asymptomatic periods punctuated by thrombotic or hemorrhagic events.


Special care related to pregnancy

Hydroxyrea and anagrelide are counter-indicated during pregnancy and nursing. There is current debate as to the safety of interferon during pregnancy and nursing. Essential thrombocytosis can be linked with increased risk of spontaeous abortion or miscarriage in the first trimester of pregnancy. Throughout pregnancy, close monitoring of the mother for thrombosis and placenta is recommended to ensure blood clots are caught. Post partum, often daily injections of low dose low molecular weight heparin (e.g. enoxaparin) are prescribed for several weeks as this is a period where the mother is at higher risk of developing a blood clot. Anagrelide (Agrylin®/Xagrid®, Shire) is a drug used for the treatment of essential thrombocytosis (ET; essential thrombocythemia). ... This article needs additional references or sources for verification. ... Nursing is a profession focused on assisting individuals, families, and communities in attaining, re-attaining, and maintaining optimal health and functioning. ... Interferons (IFNs) are natural proteins produced by the cells of the immune system of most vertebrates in response to challenges by foreign agents such as viruses, bacteria, parasites and tumor cells. ... In medicine, low molecular weight heparin (LMWH) is a class of medication used as an anticoagulant in diseases that feature thrombosis, as well as for prophylaxis in situations that lead to a high risk of thrombosis. ... Enoxaparin is a low molecular weight heparin manufactured by Sanofi-Aventis. ...


References

  1. ^ Mesa R, Silverstein M, Jacobsen S, Wollan P, Tefferi A (1999). "Population-based incidence and survival figures in essential thrombocythemia and agnogenic myeloid metaplasia: an Olmsted County Study, 1976-1995.". Am J Hematol 61 (1): 10-5. PMID 10331505. 
  2. ^ Kutti J, Ridell B (2001). "Epidemiology of the myeloproliferative disorders: essential thrombocythaemia, polycythaemia vera and idiopathic myelofibrosis.". Pathol Biol (Paris) 49 (2): 164-6. PMID 11317963. 
  3. ^ Hoffman: Hematology: Basic Principles and Practice, 4th ed., 2005 Churchill Livingstone, Chapter 71.
  4. ^ Kralovics R, Passamonti F, Buser AS, Teo SS, et al (2005 Apr 28). "A gain-of-function mutation of JAK2 in myeloproliferative disorders". N Engl J Med 352 (17): 1779-90. 
  5. ^ Baxter EJ et al. Acquired mutation of the tyrosine kinase JAK2 in human myeloproliferative disorders. Lancet. 2005;365:1054-61. PMID 15781101
  6. ^ Levine RL et al. Activating mutation in the tyrosine kinase JAK2 in polycythemia vera, essential thrombocythemia, and myeloid metaplasia with myelofibrosis. Cancer Cell. 2005;7:387-97. PMID 15837627
  7. ^ Campbell PJ, Green AR. Management of Polycythemia Vera and Essential Thrombocythemia. Hematology (Am Soc Hematol Educ Program). 2005;:201-8. PMID 16304381
  8. ^ Harrison CN et al. Hydroxyurea compared with anagrelide in high-risk essential thrombocythemia. N Engl J Med. 2005;7:33-45. PMID 16000354.

External links

v • d • e
Pathology: hematology/hematological malignancy (primarily C81-C96/200-208, D45-D47, D50-D77/280-289)
WBCs Lymphoma and leukemia
-cytosis (Agranulocytosis, Leukocytosis, Lymphocytosis, Monocytosis) • -penia (Lymphopenia, Neutropenia)
RBCs/anemia/hemoglobinopathy Nutritional anemia: Iron deficiency anemia, Plummer-Vinson syndrome, Megaloblastic anemia (Pernicious anemia)
Hereditary hemolytic anemia: G6PD Deficiency, Thalassemia, Sickle-cell disease/trait, Hereditary spherocytosis, Hereditary elliptocytosis, Hereditary stomatocytosis
Acquired hemolytic anemia: Warm autoimmune hemolytic anemia, HUS, MAHA, PNH
Aplastic anemia: Acquired PRCA, Diamond-Blackfan anemia, Fanconi anemia • Sideroblastic anemia • Hemochromatosis
Coagulation/platelets Coagulopathy: DIC • Hemophilia (A, B, C, XIII) • Von Willebrand disease
Purpura: Henoch-Schönlein, ITP, TTP
Other hemorrhagic conditions: Bernard-Soulier syndrome - Glanzmann's thrombasthenia - Grey platelet syndrome
Histiocytosis Acute monocytic leukemia - Malignant histiocytosis - Erdheim-Chester disease - Langerhans cell histiocytosis - Juvenile xanthogranuloma - Hemophagocytic lymphohistiocytosis
Other Asplenia/hyposplenism - Methemoglobinemia
v • d • e
Hematological malignancy histology (ICD-O 9590-9989)
(9590-9799) Hematologic (Leukemias, Lymphomas and related disorders) Hodgkin's lymphoma vs. Non-Hodgkin lymphoma

Diffuse lymphoma vs. Follicular lymphoma
B-cell lymphoma (Small cell lymphoma, Primary effusion lymphoma, Burkitt's lymphoma, Splenic marginal zone lymphoma, MALT lymphoma)
T-cell lymphoma (Cutaneous T cell lymphoma, Mycosis fungoides, Sézary's disease, Angioimmunoblastic T-cell lymphoma, Anaplastic large cell lymphoma, Hepatosplenic T-cell lymphoma)
plasma cell (Plasmacytoma, Multiple myeloma)
mast cell (Langerhans cell histiocytosis, Mast cell tumor, Mast-cell sarcoma)
Pathology (from Greek pathos, feeling, pain, suffering; and logos, study of; see also -ology) is the study of the processes underlying disease and other forms of illness, harmful abnormality, or dysfunction. ... Hematology is the branch of medicine that is concerned with blood and its disorders. ... Although hematological malignancies are a form of cancer, they are generally treated by specialists in hematology, although in many hospitals oncology specialists also manage these diseases. ... A scanning electron microscope image of normal circulating human blood. ... This article is about lymphoma in humans. ... Leukemia or leukaemia (see spelling differences) is a cancer of the blood or bone marrow and is characterized by an abnormal proliferation (production by multiplication) of blood cells, usually white blood cells (leukocytes). ... Leukocytosis is an elevation of the white blood cell count (the leukocyte count) above the normal range. ... Lymphocytosis is an increase in the number of lymphocytes in the blood. ... Monocytosis is an increase in the number of circulating monocytes. ... Lymphopenia is the condition in which there exists an abnormally low number of lymphocytes in the blood. ... Neutropenia (or neutropaenia, adjective neutrop(a)enic) is a hematological disorder characterized by an abnormally low number of neutrophil granulocytes (a type of white blood cell). ... Human red blood cells Red blood cells are the most common type of blood cell and the vertebrate bodys principal means of delivering oxygen from the lungs or gills to body tissues via the blood. ... Anemia (AmE) or anæmia (BrE), from the Greek () meaning without blood, is a deficiency of red blood cells (RBCs) and/or hemoglobin. ... Hemoglobinopathy is a kind of genetic defect that results in abnormal structure of one of the globin chains of the hemoglobin molecule. ... Iron deficiency anemia is the most common type of anemia, and the most common cause of microcytic anemia. ... The Plummer-Vinson syndrome, also called Paterson-Kelly syndrome or sideropenic dysphagia is a disorder linked to severe, long-term iron deficiency anemia, which causes swallowing difficulty (dysphagia) due to web-like membranes of tissue growing in the throat (esophageal webs). ... Megaloblastic anemia is an anemia (of macrocytic classification) which results from a deficiency of vitamin B12 and folic acid. ... Pernicious anemia (also known as Biermers anaemia or Addisons anaemia or Addison-Biermer anaemia) is a form of megaloblastic anaemia due to vitamin B12 deficiency dependent on impaired absorption of vitamin B12 in the setting of atrophic gastritis, and more specifically of loss of gastric parietal cells. ... Hemolytic anemia is anemia due to hemolysis, the abnormal breakdown of red blood cells either in the blood vessels (intravascular hemolysis) or elsewhere in the body (extravascular). ... Glucose-6-phosphate dehydrogenase (G6PD) deficiency is an X-linked recessive hereditary disease featuring nonimmune hemolytic anemia in response to a number of causes. ... Thalassemia (American English) or thalassaemia (British English) is a recessive trait inherited disease of the red blood cells. ... Sickle-cell disease is a group of genetic disorders caused by sickle hemoglobin (Hgb S or Hb S). ... Sickle cell trait describes the way a person can inherit some of the genes of sickle cell disease, but not develop symptoms. ... Hereditary spherocytosis is a genetic disorder of the red blood cells that makes them prone to hemolysis. ... Hereditary elliptocytosis is a blood disorder in which 50-90% of the red blood cells consist of rod forms and elliptocytes (that is, elliptical erythrocytes); often associated with a hemolytic anemia. ... Hereditary stomatocytosis describes a number of inherited autosomal dominant human conditions which affect the red blood cell, in which the membrane or outer coating of the cell leaks sodium and potassium ions. ... Warm Autoimmune Hemolytic Anemia (warm AIHA) is the most common of the autoimmune hemolytic diseases. ... In medicine, Hemolytic-uremic syndrome (or haemolytic-uraemic syndrome, abbreviated HUS) is a disease characterised by microangiopathic hemolytic anemia, acute renal failure and a low platelet count (thrombocytopenia). ... In medicine (hematology) microangiopathic hemolytic anemia (MAHA) is a subgroup of hemolytic anemia (anemia, loss of red blood cells through destruction) caused by factors in the small blood vessels. ... Paroxysmal nocturnal hemoglobinuria (PNH) is a rare acquired life-threatening disease of the blood characterised by hemolytic anemia, thrombosis and red urine due to breakdown of red blood cells. ... Aplastic anemia is a condition where bone marrow does not produce sufficient new cells to replenish blood cells. ... Acquired pure red cell aplasia (or PRCA) refers to a type of anemia affecting the precursors to red blood cells but not to white blood cells. ... Diamond-Blackfan anemia (DBA) is a congenital erythroid aplasia that usually presents in infancy. ... Fanconi anemia (FA) is a genetic disease that affects children and adults from all ethnic backgrounds. ... Sideroblastic anemia is caused by the abnormal production of red blood cells as part of myelodysplastic syndrome, which can evolve into hematological malignancies (especially acute myelogenous leukemia). ... Haemochromatosis, also spelled hemochromatosis, is a hereditary disease characterized by improper processing by the body of dietary iron which causes iron to accumulate in a number of body tissues, eventually causing organ dysfunction. ... Coagulation is a complex process by which blood forms solid clots. ... A 250 ml bag of newly collected platelets. ... This page is a candidate to be moved to Wiktionary. ... Disseminated intravascular coagulation (DIC) is a pathological process in the body where the blood starts to coagulate throughout the whole body. ... This article does not cite any references or sources. ... Haemophilia A (also spelt Hemophilia A or Hæmophilia A) is a blood clotting disorder caused by a mutation of the factor VIII gene, leading to a deficiency in Factor VIII. It is the most common hemophilia. ... Haemophilia B (also spelled Hemophilia B or Hæmophilia B) is a blood clotting disorder caused by a mutation of the Factor IX gene. ... A mild form of hemophilia that mainly occurs in Jews of Ashkenazi descent. ... Factor XIII or fibrin stabilizing factor is an enzyme (EC 2. ... Von Willebrands disease (vWD) is the most common hereditary coagulation abnormality described in humans. ... Purple discolorations on the skin caused by bleeding underneath the skin. ... In medicine (rheumatology and pediatrics) Henoch-Schönlein purpura (HSP, also known as allergic purpura) is a systemic vasculitis characterized by prominent tissue deposition of IgA-containing immune complexes, especially in the skin and kidney. ... Idiopathic thrombocytopenic purpura (ITP) is the condition of having a low platelet count (thrombocytopenia) of no known cause (idiopathic). ... Thrombotic thrombocytopenic purpura (TTP or Moschcowitz disease) is a rare disorder of the blood coagulation system that in most cases arises from the deficiency or inhibition of the enzyme ADAMTS13, which is responsible for cleaving large multimers of von Willebrand factor. ... Bernard-Soulier syndrome (BSS, named after Jean Bernard and Jean Pierre Soulier) is a rare congenital bleeding disorder characterized by thrombocytopenia and large platelets and was first described in 1948. ... Glanzmanns thrombasthenia is an extremely rare, autosomal recessive disorder of the blood, in which the platelets lack GPIIb/IIIa. ... Grey platelet syndrome is a rare condition caused by a reduction or absence of the platelet alpha-granules in blood platelet, or of the proteins contained in these granules. ... Though histiocytosis can refer to any of several specific diseases, the term is generally used to refer to a rare blood disease that is caused by an excess of white blood cells called histiocytes. ... Acute monocytic leukemia (AMoL, or AML-M5) is considered a type of acute myeloid leukemia. ... Malignant histiocytosis is a hereditary disease found in the Bernese Mountain Dog characterized by histiocytic infiltration of the lungs and lymph nodes. ... Erdheim-Chester disease (also known as Erdheim-Chester syndrome) is a rare form of non-Langerhans-cell histiocytosis. ... This article or section is in need of attention from an expert on the subject. ... Juvenile xanthogranuloma is a form of histiocytosis, classified as non-Langerhans cell histiocytosis,[1] or more specifically, type 2.[2] ^ Nakasu S, Tsuji A, Fuse I, Hirai H. Intracranial solitary juvenile xanthogranuloma successfully treated with stereotactic radiosurgery. J Neurooncol. ... Hemophagocytic lymphohistiocytosis is a form of histiocytosis where there is an excess of both histiocytes and lymphocytes. ... Asplenia refers to the absence (a-) of normal spleen function and is associated with some risks. ... // Methemoglobinemia, also known as met-Hb, is a disorder characterized by the presence of a higher than normal level of methemoglobin in the blood. ... Although hematological malignancies are a form of cancer, they are generally treated by specialists in hematology, although in many hospitals oncology specialists also manage these diseases. ... The International Classification of Diseases for Oncology (ICD-O) is a domain specific extension of the International Statistical Classification of Diseases and Related Health Problems for tumor diseases. ... Although hematological malignancies are a form of cancer, they are generally treated by specialists in hematology, although in many hospitals oncology specialists also manage these diseases. ... Leukemia or leukaemia (see spelling differences) is a cancer of the blood or bone marrow and is characterized by an abnormal proliferation (production by multiplication) of blood cells, usually white blood cells (leukocytes). ... This article is about lymphoma in humans. ... Hodgkins lymphoma, also known as Hodgkins disease, is a type of lymphoma first described by Thomas Hodgkin in 1832. ... Non-Hodgkin lymphoma is a cancer arising from lymphocytes, a type of white blood cells. ... Diffuse lymphoma is a type of Non-Hodgkin lymphoma where there is not a detectable pattern to the tumors progression through the lymph node (in contrast to follicular lymphoma. ... Follicular lymphoma (FL) is the most common of the indolent non-Hodgkins lymphomas. ... B-cell lymphoma is a type of non-Hodgkin lymphoma affecting B cells. ... Small cell lymphoma (or small lymphocytic lymphoma) is a type of follicular B-cell lymphoma. ... Primary effusion lymphoma (PEL) is a malignancy of B cells that is caused by Kaposis sarcoma-associated herpesvirus (KSHV). ... Burkitts lymphoma (or Burkitts tumor, or Malignant lymphoma, Burkitts type) is a cancer of the lymphatic system (in particular, B lymphocytes). ... Splenic marginal zone lymphoma is an indolent lymphoma recognized by the World Health Organization. ... MALT lymphoma is a form of non-Hodgkin lymphoma (NHL) involving the mucosa-associated lymphoid tissue, usually of the stomach. ... T-cell lymphoma (in contrast to B-cell lymphoma) can refer to: Adult T-cell leukemia Cutaneous T Cell lymphoma Category: ... Cutaneous T-Cell lymphoma (CTCL) is a class of non-Hodgkins lymphoma, which is a type of cancer of the immune system. ... Mycosis Fungoides, also known as Alibert-Bazin syndrome or granuloma fungoides, is a rare form of non-Hodgkins lymphoma. ... Sézarys disease (or Sézary syndrome) is a type of cutaneous lymphoma characterized by Albert Sézary. ... Angioimmunoblastic T-cell lymphoma (AILT) is a mature T-cell lymphoma with systemic characterized by a polymorphous lymph node infiltrate showing a marked increase in follicular dendritic cells (FDCs) and high endothelial venules (HEVs) and systemic involvement. ... Anaplastic large cell lymphoma (ALCL) is a type of non_Hodgkin lymphoma that features in the World Health Organisation (WHO) classification of lymphomas. ... // Hepatosplenic γδ T-cell lymphoma[1] 9716/3[1] Hepatosplenic T-cell lymphoma is a systemic neoplasm comprised of medium-sized cytotoxic T-cells that show a significant sinusoidal infiltration in the liver, spleen, and bone marrow. ... Plasma cells (also called plasma B cells or plasmocytes) are cells of the immune system that secrete large amounts of antibodies. ... Plasmacytoma refers to a malignant monoclonal plasma cell tumor growing either in bone or soft tissue. ... Multiple myeloma (also known as MM, myeloma, plasma cell myeloma, or as Kahlers disease after Otto Kahler) is a type of cancer of plasma cells which are immune system cells in bone marrow that produce antibodies. ... Mast cells A mast cell (or mastocyte) is a resident cell of areolar connective tissue (loose connective tissue) that contains many granules rich in histamine and heparin. ... This article or section is in need of attention from an expert on the subject. ... A mast cell tumor is a type of tumor consisting of mast cells that is found in many species of animals. ... Mast-cell sarcoma is an aggressive[1] form of sarcoma of the mast cells. ...

immunoproliferative (Waldenström macroglobulinemia, Lymphomatoid granulomatosis)
(9800-9839) Lymphoid leukemias, and related conditions Acute lymphoblastic leukemia - Chronic lymphocytic leukemia - T-cell leukemia (Adult T-cell leukemia, T-cell large granular lymphocyte leukemia, T-cell prolymphocytic leukemia, T-cell acute lymphoblastic leukemia) - B-cell leukemia
(9840-9939) Myeloid leukemias, and related conditions Erythroleukemia - Myeloid leukemia (Acute myeloid leukemia, Chronic myelogenous leukemia, Acute promyelocytic leukemia, Philadelphia chromosome) - Monocytic leukemia (Acute) - Granulocytic sarcoma
(9940-9989) Other Hairy cell leukemia - Aggressive NK-cell leukemia - Myeloproliferative disease (Polycythemia vera, Essential thrombocytosis, Myelofibrosis) - Chronic neutrophilic leukemia - Hypereosinophilic syndrome - Post-transplant lymphoproliferative disorder - Myelodysplastic syndrome

  Results from FactBites:
 
eMedicine - Thrombocytosis : Article by Susumu Inoue (4632 words)
Primary thrombocytosis is a component of a myeloproliferative disorder (eg, essential thrombocythemia, myelofibrosis with myeloid metaplasia, polycythemia vera, chronic myelocytic leukemia [rare]) or, in rare cases, of acute myelocytic leukemia.
In pediatrics, primary thrombocytosis is exceedingly rare, whereas secondary, or reactive, thrombocytosis is common, particularly in infants.
Older children with thrombocytosis with thrombosis (suspected or demonstrated) or a history of thrombosis, increased bleeding tendency despite thrombocytosis, or splenomegaly must promptly be referred to a hematologist.
  More results at FactBites »


 

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