Abstract
Early detection and prophylaxis of blastic crisis in chronic myelogenous leukemia (CML) is one of the most important subjects to prolong the survival of the patients. We presented a case of CML, whose initial sign of blastic crisis had appeared as a painful bone lesion before hematological change became manifest.
A 30-year-old male visited Hiroshima University Hospital on Feb. 27, 1974, with a chief complaint of abdominal distension. A diagnosis of CML was made from the hematological findings of peripheral blood and bone marrow, increased WBC without hiatus, a low score of neutrophil alkaline phosphatase and positive Philadelphia chromosome (Ph1). In May, 1974, he complained of severe pain in the upper part of the right tibia, where an osteolytic lesion was disclosed by roentgenography on June 29, 1974. On July 31, 1974, histopathology of a biopsy specimen from the osteolytic lesion revealed myeloblastoma. 99Tc scintigram proved specific high concentration of radioactivity in the lesion. Local irradiation of X-ray, with total dosis of 4,000 R for a period of 3 weeks, brought about clinical improvement with diminished uptake of 99Tc-ppi and size of lesion on an X-ray film. On September 2, 1974 the patient got high fever and large atypical cells in the peripheral smear were recognized. On September 13, 1974, the tumor was enucleated and a bone marrow homograft was transplanted into the enucleated lesion, and chemotherapy was promptly instituted. The following symptoms such as markedly enlarged spleen, increase of WBC, dry tap of the bone marrow and a hyperdiploid clone with Ph1 chromosome in the tumor were highly suggestive of blastic crisis of CML. Immediately intensive combination chemotherapies were performed. However these treatment brought about irreversible bone marrow aplasia.
Fifty-four cases of CML with tumor formation appeared in the literature were summarized of their clinical characterisitics and therapy administered. In this case, the tumor developed 3 months after the clinical onset of CML and lasted 6 months ending in blastic crisis and fatal cerebral bleeding. We evaluated clinical usefulness of the 99Tc bone marrow scintigram from the standpoint of therapeutic effects of local irradiation of X-rays to eliminate the primary focus of blastic crisis of CML.