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Giant platelet disorders, also known as macrothrombocytopenia, are rare disorders featuring abnormally large platelets, thrombocytopenia and a tendency to bleeding. Giant platelets cannot stick adequately to injured blood vessel walls, resulting in abnormal bleeding when injured. Giant platelet disorder occurs for inherited diseases like ...
Harris platelet syndrome, previously known as asymptomatic constitutional macrothrombocytopenia, is the most common inherited giant platelet disorder in the Indian subcontinent. It is characterized by a functional thrombocytopenia due to the presence of giant platelet cells.
The disease is characterised by large lysosome vesicles in phagocytes (neutrophils), which thus have poor bactericidal function, leading to susceptibility to infections, abnormalities in nuclear structure of leukocytes, anemia, and hepatomegaly. Döhle bodies (remnants of endoplasmic reticulum) in the neutrophil are also seen. [citation needed]
Immune thrombocytopenic purpura (ITP), also known as idiopathic thrombocytopenic purpura or immune thrombocytopenia, is an autoimmune primary disorder of hemostasis characterized by a low platelet count in the absence of other causes. [1][2] ITP often results in an increased risk of bleeding from mucosal surfaces (such as the nose or gums) or ...
[citation needed] The disease can cause dysfunctions of the lungs, intestine, kidneys, and heart. The major complication of most forms of the disorder is pulmonary fibrosis, which typically exhibits in patients ages 40–50 years. [15] This is a fatal complication seen in many forms of HPS, and is the usual cause of death from the disorder. [16]
Glanzmann's thrombasthenia is an abnormality of the platelets. [ 2 ] It is an extremely rare coagulopathy (bleeding disorder due to a blood abnormality), in which the platelets contain defective or low levels of glycoprotein IIb/IIIa (GpIIb/IIIa), which is a receptor for fibrinogen. As a result, no fibrinogen bridging of platelets to other ...
May–Hegglin anomaly. Other names. Döhle leukocyte inclusions with giant platelets and Macrothrombocytopenia with leukocyte inclusions[1] Specialty. Hematology. May–Hegglin anomaly (MHA), is a rare genetic disorder of the blood platelets that causes them to be abnormally large.
Quebec platelet disorder (QPD) is a rare autosomal dominant bleeding disorder first described in a family from the province of Quebec, Canada. [1][2] The disorder is characterized by large amounts of the fibrinolytic enzyme urokinase -type plasminogen activator (uPA) in platelets. [3] This causes accelerated fibrinolysis (blood clot breakdown ...