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Autoimmune thrombocytopenic purpura(ITP)

MedGen UID:
584986
Concept ID:
C0398650
Disease or Syndrome
Synonyms: Idiopathic thrombocytopenic purpura; IMMUNE THROMBOCYTOPENIA; ITP
SNOMED CT: Werlhof's disease (13172003); Autoimmune thrombocytopenic purpura (13172003)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
Monarch Initiative: MONDO:0008558
OMIM®: 188030
Orphanet: ORPHA3002

Definition

Immune thrombocytopenic purpura is characterized by a low platelet count, normal bone marrow, and the absence of other causes of thrombocytopenia. It is principally a disorder of increased platelet destruction mediated by autoantibodies to platelet-membrane antigens (George et al., 1994). [from OMIM]

Additional description

From MedlinePlus Genetics
Immune thrombocytopenia is a disorder characterized by a blood abnormality called thrombocytopenia, which is a shortage of blood cells called platelets that are needed for normal blood clotting.

Affected individuals can develop red or purple spots on the skin caused by bleeding just under the skin's surface. Small spots of bleeding under the skin are called purpura and larger spots are called ecchymoses. People with immune thrombocytopenia can have significant bleeding episodes, such as nose bleeds (epistaxis) or bleeding in the moist lining (mucosae) of the mouth. In severe cases, individuals may have gastrointestinal bleeding or blood in the urine or stool, or heavy and prolonged menstrual bleeding (menorrhagia). In very rare instances, bleeding inside the skull (intracranial hemorrhage) can occur, which can be life-threatening. A greater reduction in platelet numbers is often associated with more frequent bleeding episodes and an increased risk of severe bleeding.

While immune thrombocytopenia can be diagnosed at any age, there are two periods when the condition is most likely to develop: early childhood and late adulthood. In children, the reduction in platelets is often sudden, but platelet levels usually return to normal levels within weeks to months. Immune thrombocytopenia in children is often preceded by a minor infection, such as an upper respiratory infection, but the relationship between the infection and immune thrombocytopenia is not clear. In adults, the development of immune thrombocytopenia is usually gradual and the condition tends to persist throughout life.  https://medlineplus.gov/genetics/condition/immune-thrombocytopenia

Clinical features

From HPO
Thrombocytopenia
MedGen UID:
52737
Concept ID:
C0040034
Disease or Syndrome
A reduction in the number of circulating thrombocytes.
Abnormal bleeding
MedGen UID:
264316
Concept ID:
C1458140
Pathologic Function
An abnormal susceptibility to bleeding, often referred to as a bleeding diathesis. A bleeding diathesis may be related to vascular, platelet and coagulation defects.
Platelet antibody positive
MedGen UID:
349070
Concept ID:
C1858980
Laboratory or Test Result
The presence in the serum of autoantibodies directed against thrombocytes.

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
Follow this link to review classifications for Autoimmune thrombocytopenic purpura in Orphanet.

Professional guidelines

PubMed

Balaraman R
Lancet 2002 Jun 29;359(9325):2279; author reply 2279-80. doi: 10.1016/S0140-6736(02)09281-4. PMID: 12103322
Kaplan C
Transfus Clin Biol 2001 Jun;8(3):311-4. doi: 10.1016/s1246-7820(01)00114-8. PMID: 11499984
N Engl J Med 1989 Sep 14;321(11):760-1. doi: 10.1056/NEJM198909143211113. PMID: 2597267

Recent clinical studies

Etiology

Michel M, Chanet V, Galicier L, Ruivard M, Levy Y, Hermine O, Oksenhendler E, Schaeffer A, Bierling P, Godeau B
Medicine (Baltimore) 2004 Jul;83(4):254-263. doi: 10.1097/01.md.0000133624.65946.40. PMID: 15232313
Kurtzberg J, Stockman JA 3rd
Adv Pediatr 1994;41:111-34. PMID: 7992681
Lim SH, Ifthikharuddin JJ
Leuk Lymphoma 1994 Sep;15(1-2):61-4. doi: 10.3109/10428199409051678. PMID: 7858503
Pachì A, Carapella E, Mazzucconi MG, Gandolfo GM, Paesano R, Petrelli V, De Sanctis V, Mandelli F
Early Hum Dev 1992 Jun-Jul;29(1-3):143-7. doi: 10.1016/0378-3782(92)90129-5. PMID: 1396227
Karpatkin S
Blood Rev 1987 Jun;1(2):119-25. doi: 10.1016/0268-960x(87)90006-3. PMID: 3332092

Diagnosis

Lim SH, Ifthikharuddin JJ
Leuk Lymphoma 1994 Sep;15(1-2):61-4. doi: 10.3109/10428199409051678. PMID: 7858503
Bussel JB
Hematol Oncol Clin North Am 1990 Feb;4(1):179-91. PMID: 1690204
Martin JN Jr, Morrison JC, Files JC
Am J Obstet Gynecol 1984 Sep 1;150(1):86-96. doi: 10.1016/s0002-9378(84)80115-5. PMID: 6383046
Karpatkin S
Blood 1980 Sep;56(3):329-43. PMID: 6157441
Karpatkin S
Am J Med Sci 1971 Mar;261(3):127-38. doi: 10.1097/00000441-197103000-00001. PMID: 4933874

Therapy

Ikehara S, Mizutani H, Kurata Y
Crit Rev Oncol Hematol 1995 Apr;19(1):33-45. doi: 10.1016/1040-8428(94)00128-g. PMID: 7741979
Bussel JB
Hematol Oncol Clin North Am 1990 Feb;4(1):179-91. PMID: 1690204
Karpatkin S
Semin Hematol 1985 Oct;22(4):260-88. PMID: 2413550
Martin JN Jr, Morrison JC, Files JC
Am J Obstet Gynecol 1984 Sep 1;150(1):86-96. doi: 10.1016/s0002-9378(84)80115-5. PMID: 6383046
Karpatkin S
Am J Med Sci 1971 Mar;261(3):127-38. doi: 10.1097/00000441-197103000-00001. PMID: 4933874

Prognosis

Michel M, Chanet V, Galicier L, Ruivard M, Levy Y, Hermine O, Oksenhendler E, Schaeffer A, Bierling P, Godeau B
Medicine (Baltimore) 2004 Jul;83(4):254-263. doi: 10.1097/01.md.0000133624.65946.40. PMID: 15232313
Lim SH, Ifthikharuddin JJ
Leuk Lymphoma 1994 Sep;15(1-2):61-4. doi: 10.3109/10428199409051678. PMID: 7858503
Bussel JB
Hematol Oncol Clin North Am 1990 Feb;4(1):179-91. PMID: 1690204
Stratton JR, Ballem PJ, Gernsheimer T, Cerqueira M, Slichter SJ
J Nucl Med 1989 May;30(5):629-37. PMID: 2497234
Freedman J, Musclow E, Garvey B, Abbott D
Am J Hematol 1986 Apr;21(4):397-407. doi: 10.1002/ajh.2830210408. PMID: 3953558

Clinical prediction guides

Franchini M, Lippi G, Manzato F, Vescovi PP
J Thromb Thrombolysis 2010 Jan;29(1):87-91. Epub 2009 Mar 10 doi: 10.1007/s11239-009-0327-1. PMID: 19277469
Semple JW
Blood Rev 2002 Mar;16(1):9-12. doi: 10.1054/blre.2001.0172. PMID: 11913985
Stratton JR, Ballem PJ, Gernsheimer T, Cerqueira M, Slichter SJ
J Nucl Med 1989 May;30(5):629-37. PMID: 2497234
Freedman J, Musclow E, Garvey B, Abbott D
Am J Hematol 1986 Apr;21(4):397-407. doi: 10.1002/ajh.2830210408. PMID: 3953558
Zucker-Franklin D, Karpatkin S
N Engl J Med 1977 Sep 8;297(10):517-23. doi: 10.1056/NEJM197709082971001. PMID: 560627

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