(b asophilic crisis ) 1

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1 : (b asophilic crisis ) 1, * * =A b s t r a c t = A c a s e of b a s op h ilic c ri s i s in a p atie n t w ith c h ro n ic m y e l o g e n o u s le u k e m ia Myung Sook Kim, M.D., Kee Won Kim, M.D., Hee Jung Kim, M.D. *, Suk Young Park, M.D. and Kyung Shick Lee, M.D. Departments of Internal M edicine and Clinical Pathology *, College of M edicine, The Catholic University of K orea, Seoul, K orea Chronic myelogenous leukemia (CML) is a clonal disorder of pluripotent hematopoietic stem cell. T ransformation of CML can take place at different stages of stem cell development. The common terminal event in CML is blastic crisis in the majority of cases. Basophilic crisis of CML is very rare event and we experienced a case in 48- year- old patient with philadelphia chromosome positive CML. He had received conservative treatment for 3 years. In the basophilic crisis phase, the WBC count was 64,800 / mm 3 with 70 % basophils in the peripheral blood and 43.6% in bone marrow. T hese basophils had left- shifted maturation. Cytogenetic study revealed the philadelphia chromosome without other abnormalities.(korean J Med 61: , 2001) Key W ords : Leukemia; Myeloid; Chronic; Basophils (pluripotent hematopoietic stem cell) (blastic transformation) (blast crisis). (blastic transformation), 2/3 (myeloid blastic crisis), 1/3 (lymphoid blast crisis), (erythroblastic), (monocytic), (basophilic).., Shigeta (1) Philadelphia, (2), (3), (4), (5) Philadelphia 1). : : :, 520-2, ( ) E - m ail : kkw 1965@chollian.net

2 Korean Journal of Medicine : Vol. 61, No. 3, 2001 Fig ure 1. Peripheral blood shows A: myeloblast and basophil with basophilic granule (Wright- Giemza stain. 1,000) and B: immature basophils with metachromatically positive stained basophilic granules. (toluidine blue stain, 1,000). Shigeta 1. :, 48, :, :,,. : 19961, 6. 7 cm, 139,500 / mm 3 ( 33%, 2%, 7%, 11%, 1%, 3%, 3%, 13%, 12%, 15%), 6.4 g/ dl, 1,472,000 / mm XY, t (9,22), LAP score0. hydroxyuria 4 70%. :, 130/ 90 mmhg, 90/, 20/, 37.5.,, 7 cm Fig ure 2. Bone marrow smear shows immature basophils and eosinophil (Wright Giemsa stain, 1,000).. : 64,800/ ( 9%, 0%, 70%, 16%, 2%, 2%, 1%), 7.4 g/ dl, 15,000 /.. : (aspiration smear) (biopsy) 100%, ( 1, 2) % toluidine blue (metachromatically positive). 43.6%. 25 %.. 38(metaphase) 9 22(translocation) 46, XY, t (9;22) (q34;q11.2),. CD5- CD10- CD13+ CD14- CD19- CD33+ HLA- DR-. :.

3 Myung Sook Kim, et al : A case of basophilic crisis in a patient with chronic myelogenous leukemia (myelopoiesis)philadelphia (a clonal stem cell disorder), 2-4,,, (blast transformation) (accelerated phase). Philadelphia 2, 3 ), Parkin Philadelphia,,,,, Philadelphia, 4 ).,,,,,,. Williams ), Rosenthal ), Ekblom ) ). (basophilic crisis) 9 ), 10, 11),,.,, 70% 43.6%, 25%, 10.2%. 30%., 12 ), 17% 3 70% 4. Shigeta,. 13 ), 9), 14)., t (9;22) (q34;q11.2) t (6;9) (p23;q34), 9q34 15 )., (immature basophil- like blasts), PAS+ MPO-, CD4+ CD7+ CD13+ CD33+ HLA- DR+, IL- 1, IL- 3, IL- 4, GM- CSF cytokines, IL ), GM- CSF IL- 1 17, 18 ). Yamauchi 12 11, 5, 3, 1 H2 9 )., 9, 12 )

4 : ,. Coleman 2, 6 19 ), 6 10., 3.3 ( ) 4 ( ) 9 ). 5.,,,,,,.. 3. R E F E R E N C E S 1) Shigeta H. Basophilic crisis. M edicina 14: , ) Janossy G, Woodruff RK, Paxton A. M embrane marker and cell separation studies in Ph 1 -p ositive leukemia. B lood 51: , ) Boggs DR. H ematop oietic stem cell theory in relation to p ossible lymphoblastic conversion of chronic myeloid leukemia. B lood 44: , ) Parkin JL, Mckenna RW, Brunning RD. Philadelphia chromosome-p ositive blast leukemia: ultrastructural and ultracytochemical evidence of basophil and mast cell diff erentiation. B r J H ematol 52: , ) Williams WC, Weiss GB. M egakaryoblastic transformation of chronic myelogenous leukemia. Cancer 49: , ) Rosenthal S, Canellos GP, Gralnick HR. E rythroblastic transformation of chronic granulocytic leukemias. A m J M ed 63: , ) Ekblom M, Borgstrom G, von Wiilebrand E, Gahmberg CG, Vuopio P, Anderson LG. E rythroid blast crisis in chronic myelogenous leukemia. B lood 62: , ),,,.. 11: , ) Yamauchi K, Arimori S. Basophilic crisis in chronic myelogenous leukemia: case rep ort and literature review in Japan. Jpn J M ed 29: , ),,, : , ),, : , ) Denburg JA, Wilson WEC, Bienenstock J. Basophil p roduction in myelop rolif erative disorders: increases during acute blastic transformation of chronic myeloid leukemia. B lood 60: , ) Kitano K, Sagara T, Koike S, Kawakami Y, Iijima Y, Kaji R, Imai S, Saitoo H, Furuta S, Ando O. A case of CM L blast crisis with numerous megakaryocyte and basophil colony f ormations. Jpn J Clin H ematol 26: , ) Fujii H, Maekawa T, Misawa S, Yokota S, Urata Y. P romyelocytic crisis in chronic myelogenous leukemia transf ormed to basophilic crisis with clonal evolution during a short p eriod. A cta Haematol Jpn 51: , ) Bodger MP, Morris CM, Kennedy MA, Bowen JA, Hilton JM, Fitzgerald PH. Basophils(B sp -1 + ) derive f rom the leukemic clone in human myeloid leukem ias involving the chromosome breakpoint 9q34. B lood 73: , ) T akanori T, Seiji K, Mine H, T sunefumi S, T akashi O, Yuichi T, Nobuhiro K, Koichi A, Seiichi O, Yoshiyuki N. Characterization of leukaemic basophil p rogenitors from chronic myelogenous leukaemia. B r J Haematol 78:55-59, ) Haak- Frendscho M, Arai N, Arai K, Baeza ML, Finn A, Kaplan AP. H uman recombinant granulocytemacrophage colony - stim ulating factor and interleukin 3 cause basophil histamine release. J Clin Invest 82:17, ) Alam R, Welter JB, Forsythe PA, Lett-Brown MA, Grant JA. Comparative effect of recombinant IL -1, 2, 3, 4, and 6, IFN -, granulocyte- macrophagecolony - stimulating factor, tumor necrosis factor-a,

5 4: (basophilic crisis)1 and histam ine- release factors on the secretion of histam ine f rom basophils. J Imm unol 142:3431, ) Coleman M, Silver RT, Pajak T F, Cavalli F, Rai KR, Kostinas JE, Glidewell O, Holland JF. Combination chemotherapy for terminal phase chronic myelocytic leukemia: Cancer and leukemia group B studies. B lood 55:29-36,