An autopsy case of pure red cell aplasia with thymoma

Abstract

A 76 year-old woman was hospitalized on Oct. 22, 1973, because of fatigue, anorexia, pallor of the face for the past 2 months.Laboratory data: Red cell count was extremely reduced, being 77×104 per cu mm, reticulocytes 1.0‰, Hb. 3.0g/dl, Ht. 7%. Bone marrow aspiration disclosed erythroblast 1.0%.Hematologic examination disclosed normocytichyperchromic anemia without granulocytopenia and thrombocytopenia.Serum examination revealed hypo-γ-globulinemia (9.8%), increased serum iron value (231-282γ/dl), negative for Coombs' and LE test.There was no evidence of pathologic shadow on chest X-ray examination.Clinical diagnosis was pure red cell aplasia without thymoma.Treatment was instituted with Methycobal injection, blood transfusion (total 4, 600ml) and steroids. The therapeutic effects appeared to enhance remission with further steroid therapy.The patient died on June 22, 1974, 8 months after admission due to bronchopneumonia.At autopsy, there was encapsulated solid tumor on anterior upper mediastinum -70g weight of thymoma. Histologically, tumor consisted predominantly made of spindle-shaped cells and small number of lymphocytes scattered among them separated by fibrous septa.Bone marrow was in normal hematopoeisis including erythropoeisis.The lungs showed bilateral bronchopneumonia associated with many cytomegalic inclusions and fungus infection. The similar cytomegalic infected cells were found in thymus, salivary glands, liver, tongue, esophagus, stomach, pancreas and genital organ.Electron microscopically, herpes type virus showed in squamous cells of tongue.The association of pure red cell aplasia, hypo-γ-globulinemia and thymoma in our case suggests the presence of a basic inmunologic disorders, but not any definitive evidence as autoinmune disease was obtained.Eighteen cases out of 88 cases of pure red cell aplasia have been autopsied in Japan.