PDF
ePub
Citation
Print
Abstract
T-cell prolymphocytic leukemia (T-PLL) is a post-thymic T-cell malignancy that has an aggressive clinical course and it is a distinct clinico-biological entity from other T-cell disorders. It is now apparent that this disease represents a separate entity from CLL. Clinically, T-PLL presents with hepatosplenomegaly, lymphadenopathy, skin lesion, and marked lymphocytosis exceeding 100×109/L. Because its clinical course is aggressive, the treatment is difficult. We report a case of small cell variant of T-cell with a review of literatures.
REFERENCES
1). Galton DAG, Goldman JM, Wiltshaw E, Catovsky D, Henry K, Goldenberg GJ. Prolymphocytic leukemia. Br J Haematol. 1974; 27:7–23.
2). Bartlett NL, Longo DL. T-small lymphocyte disorders. Semin Hematol. 1999; 36:164–70.
3). Matutes E, Catovsky D. Similarities between T-cell chronic lymphocytic leukemia and the small-cell variant of T-prolymphocytic leukemia. Blood. 1996; 87:3520–1.
4). Foon KA, Gale RP. Is there a T-cell form of chronic lymphocytic leukemia? Leukemia. 1992; 6:867–8.
5). Matutes E, Brito-Babapulle V, Swansbury J, et al. Clinical and laboratory features of 78 cases of T-prolymphocytic leukemia. Blood. 1991; 78:3269–74.
6). Harris NL, Jaffe ES, Stein H, et al. A revised European-American classification of lymphoid neoplasms: a proposal from the International Lymphoma Study Group. Blood. 1994; 84:1361–92.
7). Jaffe ES, Harris NL, Stein H, Vardiman JW. Pathology and genetic of tumors of hematopoietic and lymphoid tissues. Kleihues P, Sobin L, editors. World Health Organization classification of tumors, vol 3. Lyon: IARC Press;2001.
8). Ryu KC, Moon GH, Jang JG, Kim HG, Kim HK, Ok JH. A case of T-cell prolymphocytic leukemia with a phenotype CD4+. Kor J Med. 1995; 48:273–8.
9). Matutes E, Catovsky D. Mature T-cell leukemias and leukemia/lymphoma syndromes. Review of our experience in 175 cases. Leuk Lymphoma. 1991; 4:81–91.
10). Cogliatti SB, Schmid U. Who is WHO and what was REAL? Swiss Med Wkly. 2002; 132:607–17.
11). Stone RM. Prolymphocytic leukemia. Hematol Oncol Clin North Am. 1990; 4:457–71.
12). Keating MJ, Cazin B, Coutre S, et al. Campath-1H treatment of T-cell prolymphocytic leukemia in patients for whom at least one prior chemotherapy regimen has failed. J Clin Oncol. 2002; 20:205–13.
13). Dearden CE, Matutes E, Cazin B, et al. High remission rate in T-cell prolymphocytic leukemia with CAMPATH-1H. Blood. 2001; 98:1721–6.
14). Collins RH, Pineiro LA, Agura ED. Treatment of T prolymphcytic leukemia with allogeneic bone marrow transplantation. Bone Marrow Transplant. 1998; 2:627–8.
Fig. 1.
(A) Photography of skin lesion. (B), (C) and (D) Dense band-like infiltration of small sized atypical lymphocytes with slightly irregular nuclear contour in papillary dermis and perivascular space (B: H-E stain, × 100, C: H-E stain, × 400, D: Immunohistochemical stain (C D45RO, Zymed, 1:100 dilution, PAP method), × 400).
Fig. 2.
(A) Mature lymphocytes with condensed chromatin and inconspicuous nucleoli mimicking CLL in peripheral blood (Wright stain, × 400). (B) Same lymphocytes in bone marrow aspiration (Wright stain, × 400). (C) Bone marrow biopsy shows hypercellular marrow with extensive diffuse infiltration of lymphoid cells found at the skin lesion (H-E stain, × 100).
XML Download