An Autopsied Case of Chronic Myelogenous Leukemia (CML) with Atypical Blastic Crisis, Initiated with Tumor Formation (Myeloblastoma) at the Left Knee Joint

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  • 慢性骨髄性白血病の経過中に左膝関節腫瘤形成で初発した非定型的急性転化の一剖検例
  • 慢性骨髄性白血病の経過中に左膝関節腫瘤形成で初発した非定型的急性転化の1剖検例--本邦での腫瘤形成性慢性骨髄性白血病の文献的考察
  • マンセイ コツズイセイ ハッケツビョウ ノ ケイカチュウ ニ サシツカンセツ
  • —本邦での腫瘤形成性慢性骨髄性白血病の文献的考察—

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Abstract

Most cases with chronic myelogenous leukemia (CML) usually terminate in blastic crisis. But, there are some cases with CML, in whom tumor-formation has appeared without or before blastic crisis.<br>We presented an autopsied case of CML, who showed a painful tumor formation at the left knee joint with atypical blastic crisis. A 31-year-old housewife admitted to our department of Tokyo University on Jan. 13, 1975, with a chief complaint of visual disturbance and splenomegaly. She was diagnosed as CML from the peripheral blood and bone marrow findings, a low value of NAP score and rate, and positive Ph1. During four years, administration of busulfan has brought about clinical improvement and good control.<br>On Feb. 1, 1979, she complained of severe pain in the left knee without roentgenographic abnormality. On Feb. 26, local tenderness and swelling was disclosed, and hematological finding of peripheral blood (myeloblast 9.5%, promyelocyte 19.0%), increasing splenomegaly and refractory leukocytosis were suggestive of atypical blastic crisis. Immediately combination therapy (VP and VEP) were tried, and brought about good result.<br>On March 20, the similar in the right knee, where tumor formation in the soft tissue was disclosed 6 weeks later, and thereafter an osteolytic lesion of right femur was revealed by roentgenography and CT scanning. Local irradiation of X-ray with total dosis of 2500 rad. and intravenous administration of cytosine arabinoside with total dosis of 800 mg for a period 10 days, brought about clinical improvement with diminished size of lesion.<br>One month later, multiple painful tumors developed at the knee, elbow and shoulder, followed by hematological blastic crisis. Though intensive combination therapy (DCP) was instituted, no effect was obtained, and on July 26, 1979, she was died with bronchopneumonia.<br>Pathological diagnosis in autopsy was CML with blastic crisis and tumor formation of the right knee joint, gum and right kidney.<br>Eighty-two cases of CML with tumor formation reported in Japanese literature were analysed.

Journal

  • Rinsho Ketsueki

    Rinsho Ketsueki 22 (11), 1781-1787, 1981

    The Japanese Society of Hematology

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