A 61-year-old clerk was first hospitalized in April 1968 because of fever, rash and general lymphadenopathy of one month's duration. He had been hospitalized under the diagnosis of pulmonary tuberculosis when he was 34 years old.
Examination revealed moderate pallor, non-tender general lymphadenopathy and measles-like rash on the lower abdomen and extremities, but no other abnormal physical findings. Initial laboratory data: Hemoglobin was 8.4 gm/100 m
l, PCV 28.5%, reticulocytes 9.9%, leukocytes 12,500 with slight lymphocytosis, and platelets 144,000. A sternal marrow aspiation yielded normocellular material with active erythropoiesis. Urobilinogen was strongly positive in the urine. Serum total protein was 9.6 gm/100 m
l with hypergammaglobulinemia; an immunoglobulin determination revealed a simultaneous increase in γG, γM and γA. Direct Coombs' test was positive on repeated occasions. Cold agglutinin titer was always very high, exceeding 1: 2,048. A diagnosis of Hodgkin's disease was made on the finding of the histology of the inguinal lymph node.
Administration of prednisolone resulted in a good response and he left the hospital. He had to return three months later because of right mediastinal pleurisy. Oral administration of Endoxan 100 mg. daily resulted in a good response and he left the hospital on September 28, 1968. Afterwards he was on 50 mg. of Endoxan and 15 mg. of prednisolone daily until January 24, 1969. Fever and lymphadenopathy relapsed at the end of 1968. At the beginning of 1969, anemia progressed and on January 24, 1969 hemoglobin fell to 2.8 gm/100 m
l and reticulocytes 0.2% with normal leukocytes and platelets. A sternal marrow aspirate revealed “pure red cell aplasia.” Endoxan was stopped and after two weeks hemoglobin rose up to 8.2 gm/100 m
l. He expired on February 17, 1970, after repeated vomiting. The total dose of Endoxan was 9,050 mg.
The clinical diagnosis was confirmed by autopsy. Extensive increase in erythroblasts with maturation arrest was noted in all bone marrow specimens examined.
抄録全体を表示