Prolymphocytic Leukemia: New Insights in Diagnosis and in Treatment

Abstract

B cell prolymphocytic leukemia (B-PLL) diagnosis remains challenging in the absence of clear immunophenotypic or cytogenetic signature and overlap with mantle cell lymphoma. New molecular defects have been identified in T cell prolymphocytic leukemia (T-PLL), especially in the JAK STAT pathway. Like in chronic lymphocytic leukemia (CLL), B-PLL treatment depends on the presence of TP53 dysfunction. In T-PLL, alemtuzumab still remains the standard of care. Allogeneic transplantation is the only curable option. Thanks to reduced intensity conditioning regimens, it has become accessible to a larger number of patients. PLL prognosis remains poor with conventional therapies. However, great advances in the understanding of both T- and B-PLL pathogenesis lead to promising new therapeutic agents.

Papuloerythroderma‐like cutaneous involvement of a CD62L− subclone of T‐cell prolymphocytic leukemia

“…Recent advances in molecular analyses have provided new therapeutic approaches to a mutational activation of the IL2RG‐JAK1/JAK3‐STAT5B axis and AKT pathway . In our patient's case, no significant STAT3 / STAT5b mutations were detected.…”

“…T-cell prolymphocytic leukemia is a rare, aggressive, mature lymphoid neoplasm. 1,3 Generally, it shows a rapid clinical course with resistance to conventional chemotherapy. 2,4 Because of the strong expression of CD52 on tumor cells, alemtuzumab-based chemotherapy has shown good efficacy in T-PLL patients.…”

“…T‐cell prolymphocytic leukemia is a rare, aggressive, mature lymphoid neoplasm . Generally, it shows a rapid clinical course with resistance to conventional chemotherapy .…”

Persistent Mixed Donor Chimerism following Double Umbilical Cord Transplantation in a Patient with T-Cell Prolymphocytic Leukemia

“…T-cell prolymphocytic leukemia (T-PLL) is a rare postthymic T-cell neoplasm, accounting for approximately 2% of all leukemia cases, with a greater incidence in males beginning in their sixth decade [1]. Typically, untreated T-PLL is associated with a survival of less than 1 year [2].…”

“…The aggressive nature of this disease, and its rarity, presents both diagnostic and therapeutic challenges to physicians [2]. Current standard of care for T-PLL includes the use of alemtuzumab (Campath-1H), a humanized IgG1 antibody specific for the CD52 antigen which is highly expressed on normal and malignant lymphocytes [1, 3]. Thereafter, postremission therapy with either autologous or allogeneic stem cell transplant (SCT) has been associated with disease-free and long-term survival, with a median overall survival of 48 months for both allogeneic and autologous SCT, compared to 20 months for those patients who received alemtuzumab alone [1].…”