Pancytopenia in mannosidosis.
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 and 111InCl3 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.
D000328:Adult; D001792:Blood Platelets / Q000276:immunology; D001856:Bone Marrow Examination; D002239:Carbohydrate Metabolism, Inborn Errors / Q000150:complications*; D006242:Haptoglobins / Q000032:analysis; D006801:Humans; D008297:Male; D008358:Mannose / Q000378:metabolism*; D008361:Mannosidases / Q000172:deficiency; D009504:Neutrophils / Q000276:immunology; D010198:Pancytopenia / Q000209:etiology* / Q000276:immunology / Q000473:pathology
D006242:Haptoglobins; D008361:Mannosidases; D008358:Mannose