T- cell prolymphocytic leukemia - A rare case

Abstract

T-cell Prolymhocytic leukemia (T-PLL) is a rare mature post-thymic T-cell malignancy that is usually reported in the elderly and follows an aggressive course. A 68 year old male presented with a history of weakness and weight loss of two months duration. Clinical examination revealed pallor, enlarged cervical and axillary lymph nodes and splenomegaly. He also had a maculo-papular skin rash. There was marked leucocytosis, anemia and thrombocytopenia (WBC 445 x10 3 /ml, Hb 8.5gm/dl, Platelet 25x10 3 /µl) with 60% prolymphocytes in the peripheral blood. Bone marrow was hypercellular with an excess of prolymphocytes. Flow cytometric analysis of the bone marrow showed positivity for CD2, CD3, CD4, CD5 and CD7.T-PLL is a rare T cell disorder with characteristic clinical and laboratory features.Currently, no optimal treatment exists although there has been some success with 2'-deoxycoformycin or Campath-1H

T‐cell prolymphocytic leukemia: A rare disease in an elderly female

“…Interventions such as splenectomy, splenic irradiation, leucapheresis, and alkylating agents have been used to treat PLL but with disappointing results. CHOP chemotherapy (cyclophosphamide, doxorubicin, vincristine, prednisone) has shown a 30% response rate in patients studied, but eventually the disease reoccurred in all of those who had a favorable response (Ghosh & Advani, 2005). Purine nucleoside analogues (PNA) and deoxycoformycin (DCF) has been more successful in the treatment of these disorders, but most recently, monoclonal antibodies (MoAbs) have been found to be highly effective for the treatment of PLL (Ghosh & Advani, 2005).…”

“…Morphologically, the T‐prolymphocytes are smaller than the B‐prolymphocytes and have more cytoplasmic basophilia with occasional blebs or projections. The nucleus is irregular or missing in half of the cases of T‐PLL and serum antibodies to the human T‐cell leukemia virus‐I and II are not detected in T‐PLL (Ghosh & Advani, 2005). A small cell variant comprises 20% of all T‐PLL cases, and the Sezary cell‐like (cerebriform) variant is seen in 5% of cases.…”

T-cell prolymphocytic leukemia with cutaneous involvement as the presenting feature

“…[1] In contrast to B-cell lymphoproliferative disorders, T-cell malignancies show a tendency to specific cutaneous involvement. [2] The clinical course of T-PLL is typically aggressive with poor response to conventional chemotherapy.…”

“…[6] Although Campath-1H, an anti-CD52 humanized monoclonal antibody has been found to be effective in T-PLL,[1] allogeneic stem cell transplantation is still the only curative option for T-PLL[3] and should be considered the initial treatment of choice.…”