[Autoimmune hemolytic anemia with medullary erythroplasia and black serum with methemalbuminemia].
A patient with idiopathic autoimmune hemolytic anemia is described. On day 5 of therapy with corticosteroids, immunoglobulin and several plasmaphereses, a high level of methemalbumin was found while RBC lysis had ceased and bilirubin and haptoglobin had normalized. The hemoglobin and the reticulocyte count were still very low and a bone marrow aspirate showed erythroaplasia. Since the LDH was consistently elevated during the entire period of erythroaplasia, ongoing intramedullary hemolysis due to antibodies (IgG) was assumed. The patient received cyclophosphamide. After 15 days all symptoms, signs of hemolysis and aplasia resolved. The patient has not had any other hemolytic crisis without treatment (follow-up 18 months). The presence of methemalbumin is observed in intravascular hemolysis and also in association with hemorrhagic pancreatitis. Its diagnostic and prognostic value is discussed.
Betticher
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Service d'hématologie, Hôpital cantonal, Fribourg.
Pugin
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D000328:Adult; D000744:Anemia, Hemolytic, Autoimmune / Q000097:blood* / Q000628:therapy; D003131:Combined Modality Therapy; D003520:Cyclophosphamide / Q000627:therapeutic use; D004913:Erythrocytes, Abnormal; D005260:Female; D006801:Humans; D007770:L-Lactate Dehydrogenase / Q000097:blood; D008705:Methemalbumin / Q000032:analysis*; D008775:Methylprednisolone / Q000627:therapeutic use; D010956:Plasmapheresis
D008705:Methemalbumin; D003520:Cyclophosphamide; D007770:L-Lactate Dehydrogenase; D008775:Methylprednisolone