非ホジキンリンパ腫を合併したBloom症候群の1例

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  • Bloom's Syndrome Associated with Non-Hodgkin's Lymphoma

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We report a case of Bloom's syndrome associated with non-Hodgkin's lymphoma. A 6-year-old girl was referred to this hospital because of lumbago in August 1985. Skeletal survey showed compression fractures of thoracolumbar vertebrae and multiple osteolytic lesions of skull, and bone marrow smear revealed blasts of 76%. The blasts were positive for PAS stain and negative for peroxidase stain. Surface marker analysis revealed that the blasts were positive for OKIa 1 and OKB2, negative for SmIg and OKT11 and did not form E rosette. By chromosome analysis, karyotype was 46, XX, i (9 q). A diagnosis of primary non-Hodgkin's lymphoma of bone was made. She was treated with chemotherapy and irradiation, and bone marrow remission was attained. Cytological study revealed very high frequency in sister chromatid exchanges, and she was diagnosed as Bloom's syndrome. In March 1987, she relapsed in bone marrow, complicated with disseminated intravascular coagulation and hypercalcemia. Despite chemotherapy and supportive therapy, she died 49 months after the onset of lymphoma.

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