| A case of T-cell prolymphocytic leukemia with cutaneous involvement |
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Seong Hoon Shin, Yang Soo Kim, Mi Hyang Kim, Ji Hyun Ahn |
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성균관 의과대학교 삼성서울병원 심혈관센터 순환기내과 |
| 증례 : Campath-1H로 치료한 T 세포 전림프구성 백혈병 1예 |
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신성훈&#;김양수&#;김미향&#;안지현, Yang Soo Kim, Mi Hyang Kim, Ji Hyun Ahn |
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| Abstract |
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Prolymphocytic leukemia (PLL) is one of the chronic lymphoproliferative disorders characterized by the neoplastic proliferation of lymphoid cells that are capable of maturation. On immunophenotyping, the majority (80%) of the cases express B cell markers, and approximately 20% express post-thymic T-cell types. PLL is characterized by leukocytosis, massive splenomegaly with little or no lymphadenopathy, anemia, thrombocytopenia, and male prevalence. T-cell PLL is especially rare and aggressive. It is known that T-PLL may be accompanied by leukemia cutis. The patient presented in this report was a 69-year-old man who presented with marked leukocytosis and peripheral prolymphocytosis with leukemic skin involvement. His T-PLL showed resistance to first line treatment with the alkylating agent chlorambucil and did not exhibit sustained response with fludarabine, cyclophosphamide, vincristine, and prednisone combination regimen (R-CVP) as a second line treatment. Finally, complete response was achieved with Campath-1H, so we report this case with a review of the literature. |
| Key Words:
T-cell prolymphocytic leukemia; leukemia cutis; Campath-1H |
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