From the archives of MD Anderson Cancer Center: Aleukemic T-prolymphocytic leukemia, a rare presentation and review of the literature

Karen A. Nahmod, Beenu Thakral, F. N.U. Aakash, Swaminathan P. Iyer, L. Jeffrey Medeiros, Andres E. Quesada

Research output: Contribution to journalReview articlepeer-review

3 Scopus citations

Abstract

T-prolymphocytic leukemia (T-PLL) is a rare, aggressive T-cell leukemia, and patients typically present with marked peripheral blood lymphocytosis. Approximately 15–20 % of patients may present with moderate and relative stable lymphocytosis and an indolent clinical course that can persist for a few years. However, eventually these patients go on to develop marked lymphocytosis and rapidly progressive disease. We report a 72-year-old man who presented with multicompartmental lymphadenopathy and a normal complete blood count. Excision of left and right cervical lymph nodes showed replacement of the lymph node architecture by a small T-cell neoplasm positive for CD3, CD4, CD5, CD7 and TCL-1, and negative for CD8, CD20, CD30 and ALK. Subsequent bone marrow evaluation showed minimal bone marrow involvement by a T-cell neoplasm associated with TCL1A rearrangement in 11 % of cells supporting the diagnosis of T-PLL. Despite treatment, he showed progressive lymphadenopathy while remarkably maintaining normal white blood cell counts until he eventually developed leukocytosis of 110.9 × 103/uL 26 months later. Review of the literature identified only a single abstract reporting a patient with a similar lymphoma-like presentation and normal white blood cell count; however, that case showed significant bone marrow involvement in stark contrast to the current case. In summary, we report a highly unusual case of T-PLL can initially presenting with an aleukemic or lymphoma-like clinical picture, which can make establishing the diagnosis challenging.

Original languageEnglish (US)
Article number152077
JournalAnnals of Diagnostic Pathology
Volume62
DOIs
StatePublished - Feb 2023
Externally publishedYes

Keywords

  • Aleukemic
  • Differentiation state
  • Indolent
  • Lymph node
  • Lymphomatous presentation
  • T-PLL

ASJC Scopus subject areas

  • Pathology and Forensic Medicine

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