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Title   피부병변 및 전신 림프절 종대로 발현된 Ph 염색체 음성 , bcr / abl 유전자 재배열 양성인 만성 골수성 백혈병 1 예 ( A Case of Ph chromosome - Negative , bcr / abl Rearrangement - Positive Chronic Myelogenous Leukemia Prasenting with Dermopathy and Lymphadenopathy )
Publicationinfo   1996 Jan; 028(05): 927-936.
Key_word   CML, Dermopathy, Lymphadenopathy, Philadelphia chromosome, bcr/abl rearrangement, PCR
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Abstract   Chronic myelogenous leukemia(CML) is a clonal stem cell disorder, characterized by markedly increased myelopoiesis and the presence of the Philadelphia(Ph) chromosome. Ph chromosome, the result of a translocation between the abl proto-oncogene on chromosome 9 and the bcr gene on chromosome 22, is found in more than 95% of CML patients. The remaining 5% of patients are classified as Ph chromosome-negative CML and the bcr/abl gene rearrangement is detectable in approximately 50% of these patients. These Ph chromosome-negative, bcr/abl rearrangement-positive patients have clincal course and prognosis very similar to those of Ph chromosome-positive CML patients. We experienced a case of Ph chromosome-negative, bcr/abl rearrangement-positive CML presenting with multiple skin lesions and lymphadenopathy in a 59-years-old man. Bone marrow aspiration and biopsy showed typical features of CML in chronic phase. Skin and lymph node biopsies showed extramedullary leukemic cell infiltration, suggesting aggressive phase of CML. While the chramosome study revealed normal karyotype, RT-PCR analysis revealed bcr/abl fusion transcripts. In spite of chemotherapy, he expired 13 months after diagnosis.
저 자   이영우(Young Wo Lee),권교선(Gyo Seon Kwun),김경태(kyung Tae Kim),김용조(Young Cho Kim),성주병(Ju Byeung Sung),장은정(Eun Jung Jang),황춘홍(Choon Hong Hwnag),류백렬(Baek Yeol Ryoo),김태유(Tae You Kim),임영혁(Young Hyuck Im),강윤구(Yoon Koo Kang),이진오(Jhin Oh Lee),강태웅(Tae Woong Kagn)