2020
DOI: 10.1111/ijlh.13180
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T‐cell prolymphocytic leukemia: Review of an entity and its differential diagnostic considerations

Abstract: T-cell prolymphocytic leukemia (T-PLL) is a rare T-cell leukemia characterized in many patients by marked peripheral lymphocytosis, prominent splenomegaly, and skin lesions. The differential diagnosis is broad and includes other T-cell disorders presenting with similar clinical findings. This review addresses (a) the natural history, demographics, and genetic features of T-PLL; (b) clinical and pathologic differential diagnostic considerations; and (c) recent developments in the T-PLL literature relevant to la… Show more

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Cited by 5 publications
(10 citation statements)
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“…Evaluation of the bone marrow and skin biopsy specimens reveal cells with a similar morphology to those seen in the peripheral blood. Flow cytometry confirms a post‐thymic mature T‐cell population with negative expression of TDT/CD1A and positive expression of CD5, CD2, and CD7 17 . Most cases are CD52+ and CD4+/CD8— though nearly 25% of cases coexpress CD4 and CD8 17 .…”
Section: Discussionmentioning
confidence: 70%
See 2 more Smart Citations
“…Evaluation of the bone marrow and skin biopsy specimens reveal cells with a similar morphology to those seen in the peripheral blood. Flow cytometry confirms a post‐thymic mature T‐cell population with negative expression of TDT/CD1A and positive expression of CD5, CD2, and CD7 17 . Most cases are CD52+ and CD4+/CD8— though nearly 25% of cases coexpress CD4 and CD8 17 .…”
Section: Discussionmentioning
confidence: 70%
“…In addition, negative serology for HTLV‐I/II ruled out the diagnosis of ATLL (most cases of ATLL are CD25+, whereas this is negative in T‐PLL) 15 . The morphology was also distinct from the classic granular cytoplasm of large granular cell leukemia and the gyrate cerebriform nuclei of Sézary syndrome and ATLL 17 . An immature T‐cell neoplasm like lymphoblastic lymphoma could be ruled out on the basis of the negative expression of immature markers 18 .…”
Section: Discussionmentioning
confidence: 99%
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“…T‐PLL is a rare aggressive, mature T‐cell neoplasm affecting mainly elderly adults or younger individuals with ataxia‐telangiectasia 1,2 . The disease commonly presents with a very high white blood cell count and involvement of BM, spleen, liver, and lymph nodes 1,2 .…”
Section: Figurementioning
confidence: 99%
“…T‐PLL is a rare aggressive, mature T‐cell neoplasm affecting mainly elderly adults or younger individuals with ataxia‐telangiectasia 1,2 . The disease commonly presents with a very high white blood cell count and involvement of BM, spleen, liver, and lymph nodes 1,2 . Cutaneous involvement is described in almost 30% of patients, with purpuric maculopapular or nodular lesions and rather common prominent facial involvement 3,4 .…”
Section: Figurementioning
confidence: 99%