1986 Volume 27 Issue 3 Pages 407-411
A 19-year-old male repeatedly suffered nasal bleeding and multiple phlegmones of the skin since his childhood. Hematological study on admission (March, 1984) included RBC of 490×104/cmm, Platelet of 3.9×104/cmm and WBC of 2,200/cmm with neutropenia (19%). A biopsy specimen from the bone marrow showed hypocellularity with increasing adipose cells.
CFU-C formation of the bone marrow cells from the patient was impaired. The clinical picture, therefore, corresponds to the rare disorder, called Chronic Hypoplastic Neutropenia which is characterized according to Dameshek et al by an increased susceptibility to infections especially of the skin, bleeding tendency, hypoplasia of the granulocytic elements and megakaryocytes of the marrow and its prolonged and relatively benign clinical course. Familial survey revealed that his mother also had pancytopenia. By hematological study, scintigram and biopsy of the bone marrow, CFU-C assay, etc, she was diagnosed to have a typical Aplastic Anemia. This is the first case report of familial occurrence of Chronic Hypoplastic Neutropenia and Aplastic Anemia.
