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Platelet-type bleeding disorder 16(BDPLT16)

MedGen UID:
1781222
Concept ID:
C5442010
Disease or Syndrome
Synonyms: BDPLT16; Bleeding disorder, platelet-type, 16, autosomal dominant
 
Gene (location): ITGA2B (17q21.31)
 
Monarch Initiative: MONDO:0008552
OMIM®: 187800

Definition

Platelet-type bleeding disorder-16 (BDPLT16) is an autosomal dominant form of congenital macrothrombocytopenia associated with platelet anisocytosis. It is a disorder of platelet production. Affected individuals may have no or only mildly increased bleeding tendency. In vitro studies show mild platelet functional abnormalities (summary by Kunishima et al., 2011 and Nurden et al., 2011). Genetic Heterogeneity of Glanzmann Thrombasthenia-like with Macrothromocytopenia See BDPLT24 (619271), caused by mutation in the ITGB3 gene (173470) on chromosome 17q21.32. Together the ITGB2B and ITBG3 genes form an integrin, known as platelet glycoprotein GPIIb/III, that is expressed on platelets. [from OMIM]

Clinical features

From HPO
Anemia
MedGen UID:
1526
Concept ID:
C0002871
Disease or Syndrome
A reduction in erythrocytes volume or hemoglobin concentration.
Thrombocytopenia
MedGen UID:
52737
Concept ID:
C0040034
Disease or Syndrome
A reduction in the number of circulating thrombocytes.
Giant platelets
MedGen UID:
137700
Concept ID:
C0333864
Finding
Giant platelets are larger than 7 micrometers and usually 10 to 20 micrometers. The term giant platelet is used when the platelet is larger than the size of the average red cell in the field. (Description adapted from College of American Pathologists, Hematology Manual, 1998).
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 anisocytosis
MedGen UID:
810926
Concept ID:
C1739105
Pathologic Function
Abnormally increased variability in the size of platelets.
Impaired platelet aggregation
MedGen UID:
383786
Concept ID:
C1855853
Finding
An impairment in the rate and degree to which platelets aggregate after the addition of an agonist that stimulates platelet clumping. Platelet aggregation is measured using aggregometer to measure the optical density of platelet-rich plasma, whereby platelet aggregation causes the plasma to become more transparent.
Macrothrombocytopenia
MedGen UID:
414334
Concept ID:
C2751260
Disease or Syndrome
Petechiae
MedGen UID:
10680
Concept ID:
C0031256
Disease or Syndrome
Petechiae are pinpoint-sized reddish/purple spots, resembling a rash, that appear just under the skin or a mucous membrane when capillaries have ruptured and some superficial bleeding into the skin has happened. This term refers to an abnormally increased susceptibility to developing petechiae.

Recent clinical studies

Diagnosis

Grover N, Boama V, Chou MR
BMC Pregnancy Childbirth 2013 Jan 17;13:16. doi: 10.1186/1471-2393-13-16. PMID: 23327637Free PMC Article

Therapy

Grover N, Boama V, Chou MR
BMC Pregnancy Childbirth 2013 Jan 17;13:16. doi: 10.1186/1471-2393-13-16. PMID: 23327637Free PMC Article

Clinical prediction guides

Montague SJ, Price J, Pennycott K, Pavey NJ, Martin EM, Thirlwell I, Kemble S, Monteiro C, Redmond-Motteram L, Lawson N, Reynolds K, Fratter C, Bignell P, Groenheide A, Huskens D, de Laat B, Pike JA, Poulter NS, Thomas SG, Lowe GC, Lancashire J, Harrison P, Morgan NV
J Thromb Haemost 2024 Aug;22(8):2281-2293. Epub 2024 Mar 15 doi: 10.1016/j.jtha.2024.02.021. PMID: 38492852
Guerrero JA, Kyei M, Russell S, Liu J, Gartner TK, Storrie B, Ware J
Blood 2009 Dec 24;114(27):5541-6. Epub 2009 Oct 6 doi: 10.1182/blood-2009-03-210823. PMID: 19808696Free PMC Article

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