: B 1 = A bs trac t= A cas e of de nov o B - ce ll proly mphoc ytic le ukemia as s oc iated w ith f als e - pos itiv e dire ct antig lobulin

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1 : B 1 = A bs trac t= A cas e of de nov o B ce ll proly mphoc ytic le ukemia as s oc iated w ith f als e pos itiv e dire ct antig lobulin te s t Ji Soo Kim, M.D., Cheol Su Poo, M.D., Sung Min Noh, M.D. Heon Jik Lee, M.D., Yong Seok Yang, M.D., Seung Young Kim, M.D. and Byeung Yub Park, M.D. Department of Internal M edicine, Hae Dong Hospital, Pusan, Korea De novo B cell prolymphocytic leukemia (B PLL) is a distinct clinicopathologic entity that was first described in 1974 by Galton et al. B PLL is characterized by marked lymphocytosis with predominance of prolymphocytes, often massive splenomegaly, minimal lymphadenopathy, often aggressive clinical course and frequently poor prognosis. We experienced a case of B PLL associated with false positive direct antiglobulin test. T he patient was 52 year old man who presented with marked leukocytosis (160.2x103/L) and 60% of characteristic prolymphocytes in the peripheral blood. T he bone marrow aspirate showed dry tap and the PAS stain of peripheral blood smear showed positivity of prolymphocytes. T he immunophenotyping of the leukemic prolymphocytes revealed the positivity of surface immunoglobulin (IgM, lambda type), HLA DR, CD19 and CD5.(Korean J Med 59: , 2000) Key Words : Leukemia, Prolymphocytic B 1974Galton 1, 2),,, 3). 1986Melo 4) B., 55%, 10%, 11% 55% /(CLL/PL). FAB B chronic lymphocytic leukemia/prolymphocytic leukemia (CLL/PL). : : :, 3, ( ) E mail : kjsim@now nuri.net 428

Ji Soo Kim, et al : A case of de novo B cell prolymphocytic leukemia associated with false positive direct antiglobulin test Fig ure 1.

The cytoplasms of prolymphocytes are relatively more abundant than that of small mature lymphocytes.

경험하였기에 문헌고찰과 함께 보고하는 바이다. 증 례 환 자 : 조 철, 남자, 52세 주 소 : 양측 하지 저림 현병력 : 환자는 내원 수 일 전부터 발생한 양측 하지 Figure 3.

2 Ji Soo Kim, et al : A case of de novo B cell prolymphocytic leukemia associated with false positive direct antiglobulin test Fig ure 1. Peripheral blood smear shows markedly increased white blood cells, mainly prolymphocytes. (Wright Giemsa stain, 200) Fig ure 2. Peripheral blood shows 6 prolymphocytes with a large distinct nucleolus. The cytoplasms of prolymphocytes are relatively more abundant than that of small mature lymphocytes. (Wright Giemsa stain, 1000) 최근 저자들은 양측 하지 저림을 주소로 내원한 52세 남자 환자에서 미성숙 림프구가 보여 임상증상, 말초혈 액, 골수 검사 소견 및 면역표현형 검사 소견으로 B형 전림프구성 백혈병으로 진단된 1예에서 용혈성 빈혈이 동반된 것으로 잘못 판단할 수 있는 직접 항글로블린 검 사에 위양성을 보인 경우를 경험하였기에 문헌고찰과 함께 보고하는 바이다. 증 례 환 자 : 조 철, 남자, 52세 주 소 : 양측 하지 저림 현병력 : 환자는 내원 수 일 전부터 발생한 양측 하지 Figure 3. Bone marrow biopsy section reveals diffusely infiltrated leukemic cells(hematoxylin eosin stain, 400). 저림 및 우측 대퇴부 부분 압통을 주소로 응급실에 내원 하여 말초혈액에서 백혈구수가 / μl로 증가되 세포가 관찰되었는데 60%가 중등도 크기의 전림프구였 어 있고 혈색소 6.5 g/μl, 혈소판 / μl로 빈혈 으며, 이는 뚜렷한 핵소체와 비교적 풍부한 세포질을 가 및 혈소판감소증을 보여 혈액학적인 질환에 대한 정밀 지고 있었으며 하나의 세포에서는 핵분절을 보이기도 검사를 위해 입원하였다. 했다. 또한 정색소성의 빈혈 소견과 중등도의 혈소판감 과거력 및 가족력 : 특이한 소견은 없었다. 소증을 보였다(Figure 1, 2,). 이학적 소견 : 환자는 만성 병색이었으며, 결막 및 안 면은 창백하였고 흉부 청진상 거친 호흡음과 함께 나음 직접 항글로블린 시험에서 위양성을 나타내는 전림프 구의 응집 반응을 보였다(Figure 4, 5). 이 들렸으며 복부 촉진상 간이 4횡지 정도 만져졌고, 비 골수 천자 소견상 dry tap 양상을 보였으며 말초혈액 장은 왼쪽 늑골 정중선 하방 7 cm에서 만져졌으며, 양측 도말검사의 PAS염색에서 양성을 나타냈다. 골수 생검 하지에 함몰성 부종이 있었다. 소견에서는 세포 충실도가 100%이었고 수많은 악성 림 검사소견 : 입원 당시 혈액검사상 백혈구수 /μL, 혈색소 6.5 g/μl, 혈소판 /μL이었으며, 프구양 세포가 미만성으로 침윤되어 있었으며 정상 조 혈모세포는 거의 보이지 않았다(Figure 3). 비정상적인 림프구 계통의 세포가 증가되어 있었다. 면역표현형 검사상 세포 표면 면역글로블린은 IgM, 말초혈액 도말검사상 상당히 증가된 림프구 계통의 lambda type이 강양성을 보였고, HLA DR과 CD19가 429

: 59 4 482 2000 Figure 4. Direct Coombs' test reveals several RBC agglutination like clumps which was erroneously read as positive.

After Wright staining of dried direct Coombs' testing slide many well marked clumping of prolymphocytes are identified. (Wright stain, 200),, 3). 2:1, 70. 50.,.,,, 18), Table 1.

3 : Figure 4. Direct Coombs' test reveals several RBC agglutination like clumps which was erroneously read as positive. (Direct wet smear, phase contrast microscopy, 100) CD5 (T able 1). :,, CHOP(cyclophosphamide, doxorubicin, vincristine, prednisolone),,. 80% 10% 3,5) ), ). Figure 5. After Wright staining of dried direct Coombs' testing slide many well marked clumping of prolymphocytes are identified. (Wright stain, 200),, 3). 2:1, ,.,,, 18), Table 1. Immunophenotyping results of the peripheral blood CD45 CD19 CD7 CD5 CD2 CD10 Kappa Lambda cigm HLA DR panleukocytes pan Bcells Common ALL surface Ig surface Ig pre B cells hematopoietic cells T dt immature lymphoid cells (20%) TdT (10%) % 88% 19% 97% 13% 4% 2% 96% 31% 93% 0.1%

4 6: B1.. ( ) 10% 20%,., anti human Ig(Coombs' reagent, polyvalent) 11). (Lambda 96%) anti human Ig.. Wright stain,., buffy coat. B 8). FAB 11% 55%, B 55% 30% B 8). de novo % CD22 9,10, 12). B CD5, CD5 13). CD5. FMC7B 5), 100%. HLA DR93%, CD1988% FMC7., B cyclophosphamide chlorambucil 20% 1,14). CHOP 15). purine fludarabine2' deoxycoformycin B 35 50% 15,16). purine clad ribine(2 chlorodeoxyadenosine [2 CdA], Leustatin ) 17).,,. B 431

5 Korean Journal of Medicine : Vol. 59, No. 4, B 1974Galton,,,, /L 60% dry tap PAS, (IgM, L ambda type), HLA DR, CD19, CD5 B, B1. R E F E R E N C E S 1) Galton DAG, Goldman JM, wiltshaw E, Catovsky D, Henry K, Goldenberg J. Prolymphocytic leukemia. Br J Haematol 27:7 23, ) Bennett JM, Catovsky D, Daniel MT, Flandrin G, Galton DAG, Gralnick HR, Sultan C. Proposals for the classification of chronic (mature) B and T lymphoid leukaemias. J Clin Pathol 42: , ) Stone RM. Prolymphocytic leukemia. Hematol Oncol Clin N orth A m 4: , ) Melo JV, Catovsky D, Galton DG. T he relationship leukaemia. I. Clinical and lavoratory features of 300 patients and characterixation of an intermediate group. Br J Haematol 63: , ) Jandl JH. Blood: T extbook of Hematology: Prolymphocytic and Hairy cell leukemia. 2nd ed. p. 1019, Boston, Little B rown and Company, ),,,,. Prolymphocytic leukemia 1. 16: , ),,,,. B 1. 32: , ) Scott C, Stark A, Head C, Roberts BE. Diagnostic features and survival in typical and prolymphocytoid variants of chronic lymphocytic leukemia. Hematol Oncol 7: , ) Catovosky D, Galetto J, Galtone DAG, Wiltshow E, Stathopoulos G. Prolymphocytic leukemiaof B and T cell type. Lancet 2: , ) Bearman RM, Pangalis GA, Rappaport H. Prolymphocytic leukemia: Clinical, histop athological, and cytochemical observations. Cancer 42: , ) Lee GR, Bithell T C, Foerster J, Athens JW, Lukens JN. W introbe's Clinical Hematology. 9th ed. p , Pennsylvania, Lea & Febigen, ) Melo JV,Catovsky D, Galton DG. The relationship leukaemia. IV. Analysis of survival and prognostic features. B r J Haematol 65:23 29, ) Melo JV, Catovsky D, Galton DG. T he relationship leukaemia. II. Patterns of evolution of "prolymphocytoid" transformation. Br J Haematol 64:77 86, ) Hollister D, Coleman MS. Treatment of prolymphocytic leukemia. Cancer 50: , ) Taylor HG, Butler WM, Rhoads J, Karcher DS, Detrick HB. Prolymphocytic leukemia: T reatment with combination chemotherapy to include doxorubicin. Cancer 49: , ) Kantarjian HM, Childs C, O`Brien S, Huh Y, Beran M, Schachner J, Joller C, Keating MT. Efficacy of fludarabine, a new adenosine nucleoside analogue, in patients with prolymphocytic leukemia and the prolymphocytoid variant of chronic lymphocytic leukemia. A m J M ed 90: , ) Saven A, Lee T, Schultz M, Jacobs A, Ellison D, Longmire R, Piro L. M ajor activity of cladribine in patient with de novo B cell prolymphocytic leukemia. J Clin Oncol 15:37 43, ) Bennett JM, Catovky D, Daniel MT, Flandrin G, Galton DAG, Granick HR, Sultan C. Proposals for the classification of chronic(mature) B and T lymphoid leukaemias. J Clin Pathol 42: ,

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<32352EBDC5BCBAC8C62E687770> 대한내과학회지 : 제 74 권부록 1 호 2008 Campath-1H 로치료한 T 세포전림프구성백혈병 1 예 고신대학교의과대학내과학교실 1, 진단검사학교실 2, 병리학교실 신성훈 1 김양수 1 김미향 2 안지현 A case of T-cell prolymphocytic leukemia with cutaneous involvement Seong Hoon Shin,