RESUMO
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 x 103 sub/ml, Hb 8.5 gm/dl, Platelet 25 x 103 sub/microl) 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 CD 7. 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.
Assuntos
Idoso , Células da Medula Óssea/patologia , Diagnóstico Diferencial , Humanos , Leucemia Prolinfocítica/sangue , Masculino , Redução de PesoRESUMO
Prolymphocytic leukaemia is a rare subtype of chronic lymphocytic leukaemia. Three such cases were reported here along with clinical details. All these cases were seen in males above 5th decade. These patients showed moderate to massive splenomegaly, inconspicuous lymphadenoapthy in two cases and one with minimal lymphadenopathy. Peripheral smear showed high leukocyte count with more than 55% of prolymphocytes. Bone marrow aspiration showed diffuse involvement and in one with minimal lymphadenopathy, lymphnode aspiration showed prolymphocytes. All the three patients died within a year after diagnosis.