monomac syndrome bone marrow |
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navigate by keyword : syndrome autosomal dominant gata2 monocytopenia lymphocytopenia bone marrow hypocellularity fibrosis dysplasia monomac hematology oncology cytology pathology laboratory clinical medical rare diseases aplasia myelodisplastic infectious mycobacterium genetics multilineage medullary capillaries deficiency |
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MonoMAC syndrome is a rare autosomal dominant syndrome associated with GATA2 deficiency. There is profound monocytopenia, B lymphocytopenia and NK lymphocytopenia. The bone marrow characterized by hypocellularity, fibrosis, and multilineage dysplasia. Note the medullary capillaries. Bone marrow, HEx100. |
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