CASE PRESENTATION

A case of adult T-cell leukemia/lymphoma (ATLL) with atypical morphology and immunophenotypic profile

Un caz de leucemie/limfom cu celule T al adultului (ATLL), cu morfologie şi profil de imunofenotipare atipice

Abstract

A 23-year-old female patient presented with abdominal lymphadenopathy, severe hypercalcemia, leukocytosis (54.9x109/L), mild anemia, and normal platelet count. 

Keywords
T-cellleukemialymphoma

Rezumat

Cuvinte Cheie

A 23-year-old female patient presented with abdominal lymphadenopathy, severe hypercalcemia, leukocytosis (54.9x109/L), mild anemia, and normal platelet count. Peripheral blood smear showed 40% atypical lymphoid cells with convoluted nuclei (Figure 1). Flow cytometry analysis performed in the peripheral blood revealed an abnormal population of memory CD4+ T cells (CD5+/-, CD3+/-, CD7-, CD45RO-, CD25-), compatible with Sézary cells. However, serology for human T-cell lymphotropic virus type 1 (HTLV-1) was positive, the diagnosis of CD25-negative ATLL being established.
 

Figure 1. Representative images of peripheral blood smear (100x mag­ni­fi­ca­tion) showing a hete­ro­genous population of aty­pical lymphoid cells with convoluted nuclei, mo­derately condensed chromatin, and basophilic agranular cytoplasm. A few giant cells displaying “Burkitt-like” features (cytoplasmic and nuclear vacuolization) are observed
Figure 1. Representative images of peripheral blood smear (100x mag­ni­fi­ca­tion) showing a hete­ro­genous population of aty­pical lymphoid cells with convoluted nuclei, mo­derately condensed chromatin, and basophilic agranular cytoplasm. A few giant cells displaying “Burkitt-like” features (cytoplasmic and nuclear vacuolization) are observed

ATLL is a rare peripheral T-cell lymphoma that occurs in about 5% of HTLV-1 carriers from endemic regions, after a long latency period(1). Four clinical subtypes of ATLL with different clinical courses are described: smoldering, chronic, acute leukemic, and lymphomatous(1). Morphologically, typical ATL cells display polylobulated nuclei, being recognized on smears as “flower cells”(2). ATLL neoplastic lymphocytes originate in CD4+ helper T cells that share the CD25+ phenotype with regulatory T cells(3). Nevertheless, a lack of CD25 expression was reported in a subset of ATLL cases(4).

We report atypical morphological features of lymphoid cells (“Burkitt-like”) in our patient, as well as an unusual immunophenotypic profile.

 

 

 

Autori pentru corespondenţă: Cristina Mambet E-mail: cristina.mambet@gmail.com

CONFLICT OF INTEREST: none declared.

FINANCIAL SUPPORT: none declared.

This work is permanently accessible online free of charge and published under the CC-BY.

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Bibliografie


  1. Taylor GP, Matsuoka M. Natural history of adult T-cell leukemia/lymphoma and approaches to therapy. Oncogene. 2005;24(39):6047-57.

  2. Tsukasaki K, Marçais A, Nasr R, Kato K, Fukuda T, Hermine O, Bazarbachi A. Diagnostic Approaches and Established Treatments for Adult T Cell Leukemia Lymphoma. Front Microbiol. 2020;11:1207.

  3. Ong SY, Zain JM. Aggressive T-cell lymphomas: 2024: Updates on diagnosis, risk stratification, and management. Am J Hematol. 2024;99(3):439-456.

  4. Khanlari M, Ramos JC, Sanchez SP, Cho-Vega JH, Amador A, Campuzano-Zuluaga G, Vega F, Chapman JR. Adult T-cell leukemia/lymphoma can be indistinguishable from other more common T-cell lymphomas. The University of Miami experience with a large cohort of cases. Mod Pathol. 2018;31(7):1046-1063.

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