Acquired Amegakaryocytic Thrombocytopenia Misdiagnosed as Immune Thrombocytopenia: A Case Report
Author(s) -
Arya Mariam Roy,
Manojna Konda,
George K Sidarous,
Dinesh Atwal,
Steven A. Schichman,
Anuradha Kunthur
Publication year - 2020
Publication title -
the permanente journal
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.445
H-Index - 30
eISSN - 1552-5775
pISSN - 1552-5767
DOI - 10.7812/tpp/19.203
Subject(s) - medicine , immune thrombocytopenia , aplastic anemia , bone marrow failure , bone marrow , immune system , anemia , myelodysplastic syndromes , biopsy , haematopoiesis , dermatology , surgery , immunology , antibody , stem cell , biology , genetics
Acquired amegakaryocytic thrombocytopenia (AATP) is a rare bleeding disorder that causes severe thrombocytopenia with preserved hematopoiesis of other cell lineages. Many cases are misdiagnosed and treated as immune thrombocytopenia.CASE PRESENTATIONWe report a case of AATP, in a 50-year-old man, that was treated as immune thrombocytopenia for years with no clinical response. The disorder later was diagnosed as AATP after bone marrow biopsy and was successfully treated with cyclosporine.DISCUSSIONThe exact mechanism of AATP remains unclear; it is suspected to be an immune-mediated process. Patients with AATP present with severe bleeding and thrombocytopenia, which is usually unresponsive to high-dose corticosteroids. There are no standard treatment guidelines for AATP. Cyclosporine and antithymocyte globulin are found to be effective in some cases. The prompt diagnosis of AATP is vital because it carries high mortality because of excessive bleeding, and it can progress into aplastic anemia or myelodysplastic syndrome.
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