“Almost Bleeding to Death”: The Conundrum of Acquired Amegakaryocytic Thrombocytopenia

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“Almost Bleeding to Death”: The Conundrum of Acquired Amegakaryocytic Thrombocytopenia

Acquired amegakaryocytic thrombocytopenia (AAT) is a rare hematological disorder causing severe thrombocytopenia and bleeding. Previous in vitro studies postulated both cell-mediated suppression of megakaryocytopoiesis in early megakaryocytic progenitor cells and humoral-mediated suppression by anti-thrombopoietin antibodies as possible etiologies of AAT. Patients with AAT usually present with ...

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Acquired Amegakaryocytic Thrombocytopenia in a Child: A Very Rare Case

Acquired amegakaryocytic thrombocytopenic purpuria (AATP) is an unusual hematologic disorder characterized by thrombocytopenia resulting from an unexplained reduction in the number of bone marrow megakaryocytes in the presence of otherwise normal hematopoiesis in the bone marrow [1]. The exact prevalence of acquired amegakaryocytic thrombocytopenia is unknown due to its very rare incidence. Aro...

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Congenital Amegakaryocytic Thrombocytopenia: A Brief Review of the Literature

Congenital amegakaryocytic thrombocytopenia (CAMT) is a rare inherited autosomal recessive disorder that presents with thrombocytopenia and absence of megakaryocytes. It presents with bleeding recognized on day 1 of life or at least within the first month. The cause for this disorder appears to be a mutation in the gene for the thrombopoeitin (TPO) receptor, c-Mpl, despite high levels of serum ...

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Acquired amegakaryocytic thrombocytopenic purpura - An underdiagnosed entity

Acquired Amegakaryocytic Thrombocytopenic Purpura (AATP) is a rare cause of thrombocytopenia presenting over a wide age group with symptoms of bleeding and bone marrow showing isolated absence of megakaryocytes in an otherwise normal marrow. Here, we report a case of AATP in a three year old female child who was then treated with anti thymocyte globulin successfully. We report this case because...

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c-mpl mutations are the cause of congenital amegakaryocytic thrombocytopenia.

Congenital amegakaryocytic thrombocytopenia (CAMT) is a rare disease presenting with isolated thrombocytopenia in infancy and developing into a pancytopenia in later childhood. Thrombopoietin (TPO) is the main regulator of thrombocytopoiesis and has also been demonstrated to be an important factor in early hematopoiesis. We analyzed 9 patients with CAMT for defects in TPO production and reactiv...

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ژورنال

عنوان ژورنال: Case Reports in Hematology

سال: 2014

ISSN: 2090-6560,2090-6579

DOI: 10.1155/2014/806541