Prolymphocytic Leukaemia: an Update on Biology and Treatment.

PURPOSE OF REVIEW: This review summarises the recent advances in knowledge regarding the biology and treatment of prolymphocytic leukaemias. RECENT FINDINGS: Both B-PLL and T-PLL are genetically complex, and the molecular landscape of these diseases has been well characterised recently. Diagnostic criteria for T-PLL have been refined with the publication of the first international consensus criteria, whereas the diagnosis of B-PLL has been thrown into question by the most recent WHO classification. Treatment advances in B-PLL have relied heavily on the advances seen in CLL that have then been extrapolated to B-PLL with just a few case reports to support the use of these targeted inhibitors. Despite increased knowledge of the biology of T-PLL and some elegant pre-clinical models to identify potential treatments, unfortunately, no improvements have been made in the treatment of T-PLL. Unmet need is a term oft used for many diseases, but this is particularly true for patients with prolymphocytic leukaemias. Ongoing improvements in our understanding of these diseases will hopefully lead to improved therapies in the future.

De novo chronic lymphocytic leukemia/prolymphocytic leukemia or B-cell prolymphocytic leukemia? The importance of integrating clinico-morphological and immunophenotypic findings in distinguishing chronic lymphoproliferative diseases with circulating phase

B-cell prolymphocytic leukemia (B-PLL) is an extremely rare disease, accounting for approximately 1% of the lymphocytic leukemias. B-PLL generally occurs in older people. It is characterized by the presence of more than 55% prolymphocytes in the peripheral blood (PB), no or minimal lymphadenopathy, massive splenomegaly, and very high white blood cell counts. The prognosis of B-PLL patients is generally poor, with a median survival of 3 years, although a subset of patients may show a prolonged survival. Herein, we report a case of a 70-year-old male with weakness, generalized lymphadenopathy, and moderate splenomegaly at the initial presentation. Hematologic examination revealed lymphocytic leukocytosis, favoring a chronic lymphoproliferative disorder (CLPD). The key to decoding the precise CLPD was a combination of the clinical profile, morphologic findings on the peripheral blood and the bone marrow, immunophenotypic analysis, and cytogenetic study. The best diagnosis proffered was a de novo chronic lymphocytic leukemia/prolymphocytic leukemia. There was no prior history of lymphoproliferative disorder or lymphocytic leukocytosis. Discriminating this entity from other lymphoproliferative disorders is crucial as the treatment and prognosis are varied compared to the other lymphoproliferative disorders. The diagnostic conundrum encountered and the incredible utility of ancillary studies in such a scenario are highlighted in this study.

Erupción cutánea eritematosa como primera manifestación de leucemia linfática prolinfocítica de células T / Cutaneous involvement as the first manifestation of T-cell prolymphocytic leukaemia

Se presenta el caso de un varón de 84 años que debutó con una erupción eritematosa y en el cual se efectuó el diagnóstico de leucemia prolinfocítica T. Con este motivo, se revisan diversos aspectos actuales concernientes a la biología, diagnóstico, clasificación pronóstico y tratamiento de las leucemias de células T maduras. (AU)

Leucemia prolinfocítica, descripción de dos casos / Prolymphocytic leukemia, description of 2 cases

We present the clinical and laboratory features of 2 patients with B prolymphocitic leukemia. Both are females of the fith and seventh decade of life. One had the classical clinical picture of massive splenomegaly and a high white cell count, with characteristic prolymphocytes and the other was asymptomatic, with a low white cell count. The cells were positive to B cell lineage reagents with strong surface immunoglobuline (Ig) and unreactive to T cell antibodies. Analysis of Ig genes at the DNA level demonstrated that both cases had heavy-chain gene rearrangements, confirming the B-cell origin. These are the first patients of prolymphocytic leukemia described in Chile

Trastornos de los linfocitos. Leucemia linfática crónica y otros síndromes linfoproliferativos crónicos

A lo largo de estos años se han realizado diversas clasificaciones de los síndromes linfoproliferativos. Sin embargo, la más aceptada hoy día es la clasificación REAL. Mencionamos en primer lugar la leucemia linfática crónica tipo B que es la forma más frecuente de todas las leucemias; los pacientes suelen tener una edad media de 65 años. Su frecuencia ha aumentado hoy día, debido al alargamiento de la edad media. Recientemente se han alcanzado importantes avances terapéuticos, sobre todo con la aparición de los análogos de las purinas. Comentaremos también avances terapéuticos en otros síndromes linfoproliferativos como leucemia prolifoncítica, tricoleucemia y síndrome de Sézary (AU)