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Tumori. 2019 May 1:300891619847756. doi: 10.1177/0300891619847756. [Epub ahead of print]

Flower cells of tropical descent: a challenging case of adult T-cell leukemia/lymphoma.

Author information

1
1 Hematology and Clinical Immunology, University of Perugia, Perugia, Italy.
2
2 Pathology Unit, Azienda Unità Sanitaria Locale/IRCCS Reggio Emilia, Italy.
3
3 Surgical Oncology Unit, Azienda Unità Sanitaria Locale/IRCCS Reggio Emilia, Italy.
4
5 Clinical and Experimental Medicine PhD Program, University of Modena and Reggio Emilia, Modena, Italy.
5
4 Department of Experimental Medicine, Section of Pathologic Anatomy and Histology Medical School, University of Perugia, Italy.

Abstract

BACKGROUND:

Adult T-cell leukemia/lymphoma (ATLL) is an aggressive disease associated with human T-lymphotropic virus type 1 infection, with a very high prevalence in tropical areas but exceptionally rare in Europe and Western countries.

CASE PRESENTATION:

We describe a challenging case of ATLL in a young male patient with Brazilian origin and adopted as a child by an Italian family, presenting to our clinic with atypical T-lymphocytosis and life-threatening lung infections.

CONCLUSIONS:

Diagnosis of ATLL outside of endemic areas can be difficult, requiring a high index of clinical suspicion with careful evaluation of the patient's clinical history. Prognosis is affected by disease stage at presentation and degree of immunosuppression. Few effective treatments are available, although new molecular insights have highlighted the role of host immune response and immune checkpoint blockade inhibitors, given the overexpression of PD-L1 on lymphoma cells and on microenvironment cells.

KEYWORDS:

Adult T-cell leukemia; HTLV-1; PD-L1; T-cell lymphoma; immunosurveillance; leukemia

PMID:
31041884
DOI:
10.1177/0300891619847756

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