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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

Kaplan C
Transfus Clin Biol 2001 Jun;8(3):311-4. doi: 10.1016/s1246-7820(01)00114-8. PMID: 11499984
Berchtold P, Wenger M
Blood 1993 Mar 1;81(5):1246-50. PMID: 8443385
Macey MG, Newland AC
Br J Haematol 1990 Dec;76(4):513-20. doi: 10.1111/j.1365-2141.1990.tb07909.x. PMID: 1979913

Recent clinical studies

Etiology

Jaime-Pérez JC, Aguilar-Calderón P, Jiménez-Castillo RA, Ramos-Dávila EM, Salazar-Cavazos L, Gómez-Almaguer D
Ann Hematol 2020 Nov;99(11):2513-2520. Epub 2020 Sep 18 doi: 10.1007/s00277-020-04257-2. PMID: 32945941
Badura R, Foxall RB, Ligeiro D, Rocha M, Godinho-Santos A, Trombetta AC, Sousa AE
Front Cell Infect Microbiol 2020;10:347. Epub 2020 Jul 16 doi: 10.3389/fcimb.2020.00347. PMID: 32766164Free PMC Article
Ramírez-Vargas N, Arablin-Oropeza SE, Mojica-Martínez D, Yamazaki-Nakashimada MA, de la Luz García-Cruz M, Terán-Juárez LM, Cortés-Grimaldo RM, Torres-Lozano C, Madrigal-Beas I, Ortega-Cisneros M, Vargas-Camaño ME, Staines-Boone T, Pietropaolo-Cienfuegos D, Berrón-Ruiz L, Espinosa-Rosales FJ, Guevara-Cruz M, Blancas-Galicia L
Allergol Immunopathol (Madr) 2014 May-Jun;42(3):235-40. Epub 2013 Apr 8 doi: 10.1016/j.aller.2013.01.007. PMID: 23578780
Bader-Meunier B, Proulle V, Trichet C, Debray D, Gabolde M, Yvart J, Dreyfus M
J Pediatr Hematol Oncol 2003 Jul;25(7):548-52. doi: 10.1097/00043426-200307000-00010. PMID: 12847322
Berchtold P, Wenger M
Blood 1993 Mar 1;81(5):1246-50. PMID: 8443385

Diagnosis

Jaime-Pérez JC, Aguilar-Calderón P, Jiménez-Castillo RA, Ramos-Dávila EM, Salazar-Cavazos L, Gómez-Almaguer D
Ann Hematol 2020 Nov;99(11):2513-2520. Epub 2020 Sep 18 doi: 10.1007/s00277-020-04257-2. PMID: 32945941
Bader-Meunier B, Proulle V, Trichet C, Debray D, Gabolde M, Yvart J, Dreyfus M
J Pediatr Hematol Oncol 2003 Jul;25(7):548-52. doi: 10.1097/00043426-200307000-00010. PMID: 12847322
Karpatkin S
Blood Rev 1987 Jun;1(2):119-25. doi: 10.1016/0268-960x(87)90006-3. PMID: 3332092
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

Jaime-Pérez JC, Aguilar-Calderón P, Jiménez-Castillo RA, Ramos-Dávila EM, Salazar-Cavazos L, Gómez-Almaguer D
Ann Hematol 2020 Nov;99(11):2513-2520. Epub 2020 Sep 18 doi: 10.1007/s00277-020-04257-2. PMID: 32945941
Ramírez-Vargas N, Arablin-Oropeza SE, Mojica-Martínez D, Yamazaki-Nakashimada MA, de la Luz García-Cruz M, Terán-Juárez LM, Cortés-Grimaldo RM, Torres-Lozano C, Madrigal-Beas I, Ortega-Cisneros M, Vargas-Camaño ME, Staines-Boone T, Pietropaolo-Cienfuegos D, Berrón-Ruiz L, Espinosa-Rosales FJ, Guevara-Cruz M, Blancas-Galicia L
Allergol Immunopathol (Madr) 2014 May-Jun;42(3):235-40. Epub 2013 Apr 8 doi: 10.1016/j.aller.2013.01.007. PMID: 23578780
Berchtold P, Wenger M
Blood 1993 Mar 1;81(5):1246-50. PMID: 8443385
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

Jaime-Pérez JC, Aguilar-Calderón P, Jiménez-Castillo RA, Ramos-Dávila EM, Salazar-Cavazos L, Gómez-Almaguer D
Ann Hematol 2020 Nov;99(11):2513-2520. Epub 2020 Sep 18 doi: 10.1007/s00277-020-04257-2. PMID: 32945941
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
von Baeyer H
Ther Apher Dial 2003 Feb;7(1):127-40. doi: 10.1046/j.1526-0968.2003.00004.x. PMID: 12921129
Bader-Meunier B, Proulle V, Trichet C, Debray D, Gabolde M, Yvart J, Dreyfus M
J Pediatr Hematol Oncol 2003 Jul;25(7):548-52. doi: 10.1097/00043426-200307000-00010. PMID: 12847322
Bussel JB
Hematol Oncol Clin North Am 1990 Feb;4(1):179-91. PMID: 1690204

Clinical prediction guides

Jaime-Pérez JC, Aguilar-Calderón P, Jiménez-Castillo RA, Ramos-Dávila EM, Salazar-Cavazos L, Gómez-Almaguer D
Ann Hematol 2020 Nov;99(11):2513-2520. Epub 2020 Sep 18 doi: 10.1007/s00277-020-04257-2. PMID: 32945941
Badura R, Foxall RB, Ligeiro D, Rocha M, Godinho-Santos A, Trombetta AC, Sousa AE
Front Cell Infect Microbiol 2020;10:347. Epub 2020 Jul 16 doi: 10.3389/fcimb.2020.00347. PMID: 32766164Free PMC Article
Kamhieh-Milz J, Bal G, Sterzer V, Kamhieh-Milz S, Arbach O, Salama A
Platelets 2012;23(3):184-94. Epub 2011 Sep 13 doi: 10.3109/09537104.2011.610909. PMID: 21913810
Garcia-Suarez J, Prieto A, Reyes E, Manzano L, Merino JL, Alvarez-Mon M
Blood 1993 Sep 1;82(5):1538-45. PMID: 7689873
Taaning E
Dan Med Bull 1992 Aug;39(4):343-54. PMID: 1382006

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