Clinically silent indolent T‐cell leukemia

Janot, Clément; Feriel, Joffrey; Borie, Claire; Lefèvre, Édouard; Bennaceur‐Griscelli, Annelise; Turhan, Ali G; Aumont, Cédric

doi:10.1002/ccr3.1528

Cited by 3 publications

(7 citation statements)

References 8 publications

(8 reference statements)

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“…However, some patients have been noted to remain stable without progression for more than 5 years. 8 Our median time to first treatment in the inactive group was 422 days (range: 28 days to 4 years). Our study also suggests that therapy using alemtuzumab as first-line treatment and consolidation with alloSCT are strongly recommended for both active and inactive disease at the time of progression in order to achieve long-term remission.…”

Section: Discussionmentioning

confidence: 84%

“…5 Typically, T-PLL is associated with a poor prognosis; however, growing evidence suggests that patients with T-PLL show a heterogeneous clinical course ranging from indolent to aggressive disease. [5][6][7][8][9] Due to the rarity of the disease, the largest observational studies that describe clinicopathologic characteristics range from 38 to 119 cases. [10][11][12][13][14] The typical clinical presentation consists of B-symptoms, hepatosplenomegaly, lymphadenopathy, and lymphocytosis.…”

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confidence: 99%

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Delineation of clinical course, outcomes, and prognostic factors in patients with T‐cell prolymphocytic leukemia

et al. 2023

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T‐cell prolymphocytic leukemia (T‐PLL) is a rare, post‐thymic T‐cell neoplasm with a diverse clinical course. T‐PLL is typically associated with a poor prognosis; however, a subset of patients have inactive disease on initial presentation. There is a lack of accurate delineation of the disease based on initial clinical presentation and pathological assessment, hindering clinical decision‐making. To characterize and delineate disease subtypes based on initial clinical presentation and pathologic assessment, we retrospectively reviewed 81 patients with T‐PLL treated at our institution. We compared patients with T‐PLL who initially presented with a relatively indolent or stable disease course to those with an aggressive disease course. Clinicopathologic characteristics, overall survival (OS), and prognostic factors were analyzed. Patients with inactive disease had a significantly longer OS than patients with active disease. At diagnosis, presence of B symptoms, low hemoglobin, low platelet count, lymphocyte doubling time of fewer than 3 months, and abnormal cytogenetics were associated with shorter OS. Cell morphology, immunophenotype, absolute lymphocyte count, lactate dehydrogenase levels, involvement of liver, spleen, skin or central nervous system, presence of TCL1 rearrangement or inv (14)/t(14;14), presence of chromosome 8 abnormalities, and presence of deletion of 11q were not associated with significant OS difference among the patients. Receiving alemtuzumab as first‐line treatment and consolidation with allogeneic hematopoietic stem cell transplant were associated with better outcomes. T‐PLL inactive and active disease subtypes can exhibit overlapping yet different clinical and pathological features. We describe several prognostic factors at diagnosis that can be used for risk stratification and aid in guiding treatment decisions.

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Section: Discussionmentioning

confidence: 84%

mentioning

confidence: 99%

Delineation of clinical course, outcomes, and prognostic factors in patients with T‐cell prolymphocytic leukemia

et al. 2023

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“…The indolent phase of T-PLL has also been described in which the patient remains clinically silent with the background of peripheral blood lymphocytosis. The average period for this phase is 33 months (6-103); after that, unfortunately, it transforms into a rapidly fatal aggressive disease [3][4][5]. However, there is a case report that the patient remained in the indolent phase for 18 years, which is strikingly exceptional [6].…”

Section: Discussionmentioning

confidence: 99%

“…Chromosomal microarray analysis revealed a loss of chromosome segment 6q, a gain of 6p in a mosaic state, and heterozygous deletion of the T-cell receptor alpha variable region on chromosome 14 (Figure 3). Conventional cytogenetics showed an abnormal mosaic male karyotype: 46,XY,inv(1)(q25q42),add(6)t(6;6)(q13::p11.2->pter),inv(14) (q11q32)[cp17]/46,XY [5] (Figure 4). Fluorescence In situ hybridization (FISH) identified the T-cell receptor alpha/delta gene rearrangement at 14q11.2 (Figure 5).…”

Section: Case Presentationmentioning

confidence: 99%

“…It is an aggressive disease that commonly presents with splenomegaly, lymphadenopathy, hepatomegaly, and peripheral blood lymphocytosis. It can present as an 'indolent' disease as well [1][2][3][4][5][6][7]. Autoimmune hemolytic anemia (AIHA) is characterized by the production of autoantibodies against blood cell antigens destroying red blood cells.…”

Section: Introductionmentioning

confidence: 99%

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A Rare Association: Autoimmune Hemolytic Anemia With Indolent T-Cell Prolymphocytic Leukemia

Yahya

Khan

Barta

et al. 2020

Cureus

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The association of warm autoimmune hemolytic anemia (wAIHA) with various lymphoproliferative disorders is well reported in the literature. But the association of wAIHA with T-cell prolymphocytic leukemia (T-PLL), a very rare lymphoproliferative disorder, has never been reported. A 71-year-old man was in his usual state of health until three years ago when he developed intermittent bouts of worsening anemia associated with mild peripheral blood lymphocytosis. He was diagnosed with wAIHA and steroid therapy was initiated, resulting in an improvement in the hemoglobin level of the patient. His lymphocyte count remained persistently elevated but he did not develop any malignancy-related signs or symptoms. A diagnosis of 'indolent' T-cell prolymphocytic leukemia (small cell variant) was made by combining distinctive clinical, morphologic, immunophenotypic, and cytogenetic analysis. His wAIHA went into complete remission and steroid therapy was successfully tapered off. He has not required any treatment for his T-PLL during the last two years' follow-up.

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Long-Smoldering T-prolymphocytic Leukemia: A Case Report and a Review of the Literature

Gjelberg,

Helgeland,

Liseth

et al. 2023

Current Oncology

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T-prolymphocytic leukemia (T-PLL) is a rare malignancy of mature T-cells with distinct clinical, cytomorphological, and molecular genetic features. The disease typically presents at an advanced stage, with marked leukocytosis, B symptoms, hepatosplenomegaly, and bone marrow failure. It usually follows an aggressive course from presentation, and the prognosis is often considered dismal; the median overall survival is less than one year with conventional chemotherapy. This case report describes a patient with T-PLL who, after an unusually protracted inactive phase, ultimately progressed to a highly invasive, organ-involving disease. After initial treatments failed, a novel treatment approach resulted in a significant response.

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Clinically silent indolent T‐cell leukemia

Cited by 3 publications

References 8 publications

Delineation of clinical course, outcomes, and prognostic factors in patients with T‐cell prolymphocytic leukemia

Delineation of clinical course, outcomes, and prognostic factors in patients with T‐cell prolymphocytic leukemia

A Rare Association: Autoimmune Hemolytic Anemia With Indolent T-Cell Prolymphocytic Leukemia

Long-Smoldering T-prolymphocytic Leukemia: A Case Report and a Review of the Literature

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