Giant platelet disorder

Giant platelet disorder
Classification and external resources
Two giant platelets (stained purple) are visible in this image from a light microscope (40×) from a peripheral blood smear surrounded by red blood cells. One normal platelet can be seen in the upper left side of the image (purple) and is significantly smaller in size than the red blood cells (stained pink).
ICD-10	D 69.1

Giant platelet disorders are rare disorders featuring abnormally large platelets, thrombocytopenia and a tendency to bleeding. Giant platelet disorder occurs for inherited diseases like Bernard-Soulier syndrome, gray platelet syndrome and May-Hegglin anomaly.

Giant platelet disorders can be further categorized:^[1]

Caused by glycoprotein abnormalities: Bernard-Soulier syndrome, Velocardiofacial syndrome
Caused by calpain defect: Montreal platelet syndrome
Caused by alpha granules defect: Gray platelet syndrome
Characterized by abnormal neutrophil inclusions: May-Hegglin anomaly, Sebastian syndrome
With systemic manifestations: Hereditary macrothrombocytopenia with hearing loss, Epstein syndrome, Fechtner syndrome
With no specific abnormalities: Mediterranean macrothrombocytopenia

References

^ Mhawech, P; Saleem, A (2000 Feb). "Inherited giant platelet disorders. Classification and literature review.". American journal of clinical pathology 113 (2): 176–90. PMID 10664620.

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Poly- cythemia	Polycythemia vera

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Hereditary	enzymopathy: G6PD glycolysis PK TI HK hemoglobinopathy: Thalassemia alpha beta delta Sickle-cell disease/trait HPFH membrane: Hereditary spherocytosis Minkowski-Chauffard syndrome Hereditary elliptocytosis Southeast Asian ovalocytosis Hereditary stomatocytosis

Acquired	Autoimmune WAHA CAD PCH membrane PNH MAHA TM HUS Drug-induced autoimmune Drug-induced nonautoimmune Hemolytic disease of the newborn

Other

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Hyper- coagulability	primary: Antithrombin III deficiency Protein C deficiency/Activated protein C resistance/Protein S deficiency/Factor V Leiden Prothrombin G20210A acquired:Thrombocytosis essential DIC Congenital afibrinogenemia Purpura fulminans autoimmune Antiphospholipid

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Thrombocytopenia	Thrombocytopenic purpura: ITP Evans syndrome TM TTP Heparin-induced thrombocytopenia May-Hegglin anomaly

Platelet function	adhesion Bernard–Soulier syndrome aggregation Glanzmann's thrombasthenia platelet storage pool deficiency Hermansky–Pudlak syndrome Gray platelet syndrome

Clotting factor	Hemophilia A/VIII B/IX C/XI von Willebrand disease Hypoprothrombinemia/II XIII Dysfibrinogenemia

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