The distinctive cytological features of T‐cell prolymphocytic leukemia

Jayakar, Vishal; Cheung, Kan Luk; Yebra-Fernandez, Eva; Bain, Barbara J.

doi:10.1002/ajh.24659

Cited by 3 publications

(1 citation statement)

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“…The second morphologic variant (20-25%) is characterized by atypical lymphocytes with convoluted nuclear contours that resemble the cells seen in Sézary Syndrome. The third morphological variant features heterogeneous populations of atypical lymphocytes that comprise small lymphocytes that are similar to those identified in chronic lymphocytic leukemia (CLL) and, to a lesser extent, medium-sized forms that are similar to those identified in the most common morphological pattern of T-PLL [1,3,6,7,[11][12][13].…”

Section: Diagnosismentioning

confidence: 90%

T-Cell Prolymphocytic Leukemia: Diagnosis, Pathogenesis, and Treatment

Gutierrez,

Bladek,

Goksu

et al. 2023

IJMS

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T-cell prolymphocytic leukemia (T-PLL) is a rare and aggressive neoplasm of mature T-cells. Most patients with T-PLL present with lymphocytosis, anemia, thrombocytopenia, and hepatosplenomegaly. Correct identification of T-PLL is essential because treatment for this disease is distinct from that of other T-cell neoplasms. In 2019, the T-PLL International Study Group (TPLL-ISG) established criteria for the diagnosis, staging, and assessment of response to treatment of T-PLL with the goal of harmonizing research efforts and supporting clinical decision-making. T-PLL pathogenesis is commonly driven by T-cell leukemia 1 (TCL1) overexpression and ATM loss, genetic alterations that are incorporated into the TPLL-ISG diagnostic criteria. The cooperativity between TCL1 family members and ATM is seemingly unique to T-PLL across the spectrum of T-cell neoplasms. The role of the T-cell receptor, its downstream kinases, and JAK/STAT signaling are also emerging themes in disease pathogenesis and have obvious therapeutic implications. Despite improved understanding of disease pathogenesis, alemtuzumab remains the frontline therapy in the treatment of naïve patients with indications for treatment given its high response rate. Unfortunately, the responses achieved are rarely durable, and the majority of patients are not candidates for consolidation with hematopoietic stem cell transplantation. Improved understanding of T-PLL pathogenesis has unveiled novel therapeutic vulnerabilities that may change the natural history of this lymphoproliferative neoplasm and will be the focus of this concise review.

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Section: Diagnosismentioning

confidence: 90%

T-Cell Prolymphocytic Leukemia: Diagnosis, Pathogenesis, and Treatment

Gutierrez,

Bladek,

Goksu

et al. 2023

IJMS

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show abstract

References

2021

Flow Cytometry of Hematological Malignancies

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Advances in the understanding and management of T-cell prolymphocytic leukemia

et al. 2017

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T-prolymphocytic leukemia (T-PLL) is a rare T-cell neoplasm with an aggressive clinical course. Leukemic T-cells exhibit a post-thymic T-cell phenotype (Tdt−, CD1a−, CD5+, CD2+ and CD7+) and are generally CD4+/CD8−, but CD4+/CD8+ or CD8+/CD4− T-PLL have also been reported. The hallmark of T-PLL is the rearrangement of chromosome 14 involving genes for the subunits of the T-cell receptor (TCR) complex, leading to overexpression of the proto-oncogene TCL1. In addition, molecular analysis shows that T-PLL exhibits substantial mutational activation of the IL2RG-JAK1-JAK3-, STAT5B axis. T-PLL patients have a poor prognosis, due to a poor response to conventional chemotherapy. Monoclonal antibody therapy with antiCD52-alemtuzumab has considerably improved outcomes, but the responses to treatment are transient; hence, patients who achieve a response to therapy are considered for stem cell transplantation (SCT). This combined approach has extended the median survival to four years or more. Nevertheless, new approaches using well-tolerated therapies that target growth and survival signals are needed for most patients unable to receive intensive chemotherapy.

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The distinctive cytological features of T‐cell prolymphocytic leukemia

Abstract: FIG URE 1 Peripheral blood (PB) film, case 1 FIG URE 2 Karyogram, case 1

Cited by 3 publications

References 1 publication

T-Cell Prolymphocytic Leukemia: Diagnosis, Pathogenesis, and Treatment

T-Cell Prolymphocytic Leukemia: Diagnosis, Pathogenesis, and Treatment

References

Advances in the understanding and management of T-cell prolymphocytic leukemia

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