[Association of autoimmune chronic active hepatitis and acquired erythroblastopenia cured by cyclophosphamide]. 1986

J C Trinchet, and C Lacombe, and S Islam, and N Casadevall, and B Varet, and R Poupon

To the best of our knowledge this is the reported first case of the successive occurrence in the same patient of chronic active hepatitis and acquired pure red cell aplasia, both probably of autoimmune origin. The diagnosis of autoimmune hepatitis was based on the presence of characteristic lesions at the examination of the liver biopsy specimen, high titer of anti-smooth muscle antibodies in the serum, and remission obtained by steroid therapy. Erythroid aplasia, which appeared during the course of this treatment, was revealed by a regenerative anemia (4.4 g Hb/100 ml) and proved by bone marrow aspiration and biopsy. In vitro bone marrow culture was normal, suggesting the in vivo presence of an inhibitor of erythroblastic differentiation. Red cell aplasia was cured by cyclophosphamide (100 mg/day during 56 days). No recurrence was noted until the death of the patient, which occurred one year later, due to hepatic cholangiocarcinoma. Action of cyclophosphamide on the pure red cell aplasia suggested the immune origin of this disease. The liver and the bone marrow erythroid lineage have probably been the successive targets of immunologic dyscrasia.

UI MeSH Term Description Entries
D008875 Middle Aged An adult aged 45 - 64 years. Middle Age
D012010 Red-Cell Aplasia, Pure Suppression of erythropoiesis with little or no abnormality of leukocyte or platelet production. Aplasia Pure Red Cell,Erythrocyte Aplasia,Pure Red-Cell Aplasia,Aplasia, Erythrocyte,Aplasia, Pure Red-Cell,Aplasias, Erythrocyte,Erythrocyte Aplasias,Pure Red Cell Aplasia,Pure Red-Cell Aplasias,Red Cell Aplasia, Pure,Red-Cell Aplasias, Pure
D003520 Cyclophosphamide Precursor of an alkylating nitrogen mustard antineoplastic and immunosuppressive agent that must be activated in the LIVER to form the active aldophosphamide. It has been used in the treatment of LYMPHOMA and LEUKEMIA. Its side effect, ALOPECIA, has been used for defleecing sheep. Cyclophosphamide may also cause sterility, birth defects, mutations, and cancer. (+,-)-2-(bis(2-Chloroethyl)amino)tetrahydro-2H-1,3,2-oxazaphosphorine 2-Oxide Monohydrate,B-518,Cyclophosphamide Anhydrous,Cyclophosphamide Monohydrate,Cyclophosphamide, (R)-Isomer,Cyclophosphamide, (S)-Isomer,Cyclophosphane,Cytophosphan,Cytophosphane,Cytoxan,Endoxan,NSC-26271,Neosar,Procytox,Sendoxan,B 518,B518,NSC 26271,NSC26271
D005260 Female Females
D006521 Hepatitis, Chronic INFLAMMATION of the LIVER with ongoing hepatocellular injury for 6 months or more, characterized by NECROSIS of HEPATOCYTES and inflammatory cell (LEUKOCYTES) infiltration. Chronic hepatitis can be caused by viruses, medications, autoimmune diseases, and other unknown factors. Chronic Hepatitis,Cryptogenic Chronic Hepatitis,Hepatitis, Chronic, Cryptogenic,Hepatitis, Chronic Active,Hepatitis, Chronic Persistent,Chronic Active Hepatitis,Chronic Hepatitis, Cryptogenic,Chronic Persistent Hepatitides,Chronic Persistent Hepatitis,Hepatitis, Cryptogenic Chronic
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D001327 Autoimmune Diseases Disorders that are characterized by the production of antibodies that react with host tissues or immune effector cells that are autoreactive to endogenous peptides. Autoimmune Disease,Disease, Autoimmune,Diseases, Autoimmune

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