Current treatment options in prolymphocytic leukemia.
ABSTRACT Prolymphocytic leukemia (PLL) is a rare lymphoproliferative disorder characterized by marked leukocytosis and splenomegaly. PLL accounts for approximately 2% of chronic lymphoid leukemias. The clinical course is progressive in the majority of cases due to the resistance of the disease to conventional chemotherapy. The disease is divided according to the cell of origin into the B- (B-PLL) and T-cell (T-PLL) types. T-PLL and B-PLL are morphologically identical, but lymphadenopathy and skin involvement are more common in T-PLL than in B-PLL. Approximately 80% of cases are of B-cell phenotype. T-PLL has a more aggressive course, poorer response to chemotherapy, and shorter median survival than B-PLL. PLL has poorer prognosis than chronic lymphocytic leukemia (CLL), and the patients with static disease for a longer period of time are rare. In general, B-PLL patients have better prognosis than T-PLL patients. PLL is still considered an incurable disease. Similarly to CLL, treatment is not indicated in asymptomatic patients. In previous decades, splenectomy, splenic irradiation, leucapheresis, and alkylating agents used alone or in combination with other cytotoxic agents have been used for the treatment of PLL. Subsequently, purine nucleoside analogs (fludarabine, cladribine, and pentostatin) have been introduced for the therapy of these disorders. More recently, monoclonal antibodies, especially alemtuzumab, have been found more effective, especially in T-PLL. Finally, high-dose chemotherapy followed by allogenic or autologous stem cell transplantation seems to be an effective, probably curative, strategy for the treatment of selected patients with PLL. In this review, current therapeutic strategies in PLL are presented.
- SourceAvailable from: Monica Else
British Journal of Haematology 03/2010; 149(6):907-10. DOI:10.1111/j.1365-2141.2010.08134.x · 4.96 Impact Factor
- "Stem cell transplantation after alemtuzumab in T-cell prolymphocytic leukaemia results in longer survival than after alemtuzumab alone: a multicentre retrospective study T-cell prolymphocytic leukaemia (T-PLL) is a rare malignancy with an aggressive course and limited treatment options (Robak & Robak, 2007; Dungarwalla et al, 2008). The median survival in historical series is <1 year (Matutes et al, 1991). "
- [Show abstract] [Hide abstract]
ABSTRACT: B-cell prolymphocytic leukemia (B-PLL) comprises 1% of chronic lymphocytic leukemias. CD5 positivity is seen in 1/3rd of cases which generally arise from pre existing CLL. They have longer median survival compared with de novo B-PLL which are commonly CD5 negative and are more aggressive with an older age of presentation. Herewith, we describe a 48-year-old male of de-novo CD5+ B-PLL presenting with minimal lymphadenopathy and massive splenomegaly with 90% atypical lymphoid cells in the peripheral smear and bone marrow. Immunophenotyping was strongly positive for CD5, CD45, CD19, CD22, FMC-7, S-Ig and CD38, moderately positive for CD 11c, weakly positive for CD23 and negative for CD-103 and ZAP 70. The patient responded well to fludarabine and cyclophosphamide and had an uneventful hospital course. Our case illustrates a de-novo B-PLL with aberrant CD5 positivity who had a short duration of illness, younger age at presentation and favourable treatment outcome.
- [Show abstract] [Hide abstract]
ABSTRACT: Rarer indolent lymphoid leukemias include well defined mature B-cell and T-cell neoplasm with widely varying natural history and specific morphological, immunophenotypic and molecular characteristics. Among these are prolymphocytic leukemia (PLL), hairy cell leukemia (HCL) and its variants, large granular lymphocyte leukemia (LGLL) and adult T-cell leukemia/lymphoma (ATLL). To present current therapies and emerging drugs potentially useful in the treatment of rarer chronic lymphoid leukemias. After searching MEDLINE, PubMed and the Current Contents database, and conference proceedings from the previous 3 years of the American Society of Hematology (ASH), the European Society of Hematology (EHA) and the American Society of Clinical Oncology (ASCO) were searched manually; articles written in English and additional relevant publications were then selected. New drugs including monoclonal antibodies (mAbs), new purine analogs, small molecules targeting specific molecular targets and other agents are included. Future research should focus on the novel therapeutic strategies based on the molecular pathogenic mechanisms and the development of new targeted therapies for each distinct chronic lymphoid leukemia.Expert Opinion on Emerging Drugs 04/2008; 13(1):95-118. DOI:10.1517/14728188.8.131.52 · 3.28 Impact Factor