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- W1964740909 abstract "Chronic thrombocytopenia was noted in two siblings and a first cousin. The initial impression was of immune thrombocytopenic purpura (ITP) with decreased megakaryocytes. One patient had splenectomy for presumed chronic ITP but showed no improvement. Bone marrow buffy coat slides were examined in the three children with thrombocytopenia, four normal controls, and five children with classic acute ITP. Megakaryocyte size, maturation, and ploidy were determined with Wright-Giemsa and Feulgen-stained material. Mean megakaryocyte diameters were 23.1 microns in the three related patients, 30.8 microns in normal controls, and 63.1 microns in children with classic acute ITP. Many micromegakaryocytes were noted in the three related children with chronic thrombocytopenia. An exhaustive family history was obtained, which showed multiple points of consanguinity. These patients represent an apparently new autosomal recessive disorder of megakaryocytopoiesis, characterized by disturbed megakaryocyte ploidization and maturation. More sensitive recognition of micromegakaryocytes should be attempted in children with atypical chronic thrombocytopenia, familial history of thrombocytopenia, or patients who have ITP and who have not responded to initial therapy." @default.
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- W1964740909 date "1992-08-01" @default.
- W1964740909 modified "2023-09-24" @default.
- W1964740909 title "Familial Thrombocytopenia with Micromegakaryocytes" @default.
- W1964740909 doi "https://doi.org/10.1097/00043426-199208000-00012" @default.
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