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June 1983

Pancytopenia in Mannosidosis

Author Affiliations

From the Division of Hematology-Oncology (Dr Fingert), the Department of Medicine (Drs Press and Fingert), and the Departments of Neurology (Dr Lott) and Pathology (Dr Dickersin), Massachusetts General Hospital, Boston; and the Eunice Shriver Kennedy Center for Mental Retardation, Waltham, Mass (Dr Lott). Dr Press is now with the Department of Medicine, University of Washington, Seattle.

Arch Intern Med. 1983;143(6):1266-1268. doi:10.1001/archinte.1983.00350060198036

• A case of autoimmune pancytopenia is described in a patient with mannosidosis who developed anti-platelet and anti-neutrophil antibodies and a low haptoglobin level. Bone marrow biopsy and111InCl3 bone marrow scanning demonstrated hypoplasia of the marrow with a paucity of "storage cells." Pathogenic mechanisms and implications for other inborn errors of metabolism are discussed.

(Arch Intern Med 1983;143:1266-1268)