2026-05-28 | Allogeneic stem cell transplantation in T-cell prolymphocytic leukemia: final disappointment or a chance for cure?
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2026-04-09 | A case of T-prolymphocytic leukemia harboring RAS mutation.
The text reports the clinical case of a 61-year-old woman with a long history of generalized pruritus who was eventually diagnosed with T-cell prolymphocytic leukemia (T-PLL), a rare and aggressive T-cell lymphoproliferative disorder. At presentation, the patient had fever, skin lesions, lymphocytosis, thrombocytopenia, coagulation abnormalities, and marked splenomegaly without lymphadenopathy. Morphological, immunophenotypic, and histological analyses of peripheral blood and bone marrow revealed diffuse infiltration by mature T lymphocytes with strong TCL1 expression, and T-cell receptor gene rearrangement confirmed clonality, supporting the diagnosis of T-PLL. The patient initially received bendamustine chemotherapy, which failed to induce a response. Second-line treatment with the anti-CD52 monoclonal antibody alemtuzumab was subsequently initiated, but the disease proved refractory, with persistent bone marrow involvement, worsening cytopenias, disease progression, and clinical deterioration. As a result, alemtuzumab was discontinued and the patient was transitioned to palliative care. A particularly notable finding was the detection, by next-generation sequencing, of a pathogenic KRAS G12A mutation, which has not previously been reported in T-PLL. This mutation was identified incidentally using a myeloid-targeted NGS panel. The finding broadens the known mutational spectrum of T-PLL and suggests that activation of the RAS signaling pathway may represent an alternative oncogenic mechanism beyond the canonical TCL1 and JAK–STAT pathways. We suggest that investigating for KRAS mutations in additional T-PLL cases may be beneficial to explore this pathogenetic pathway and could open new avenues for future targeted therapeutic strategies.
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2026-03-30 | Allogeneic transplant outcomes in T-cell prolymphocytic leukemia: a single-center retrospective study.
T-cell prolymphocytic leukemia (T-PLL) is a rare and aggressive malignancy with a poor prognosis. Allogeneic hematopoietic stem cell transplantation (HCT) is often used for treatment, but its effectiveness remains unclear. In this study of 33 adult patients with T-PLL who underwent HCT, 5-year progression-free survival (PFS) was 29% (95% confidence interval [CI], 14-45) and 5-year overall survival (OS) was 41% (95% CI, 24-57). Five-year cumulative incidence of non-relapse mortality was 18%, while 5-year cumulative incidence of relapse was 53%. Univariable analysis revealed that a longer time from diagnosis to HCT was significantly associated with improved PFS (hazard ratio, 0.36; 95% CI, 0.15-0.87). Analysis of immune reconstitution in 22 patients showed a relatively slow recovery of immune cells post-HCT. A higher recovery of CD3 T cells was also associated with increased risk of relapse, which may have prognostic significance and warrants further investigation as a risk factor for disease recurrence.
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