Cutaneous presentation of adult T-cell leukemia/lymphoma (ATLL). Single-center study on 37 patients in metropolitan France between 1996 and 2016

C Hurabielle; M Battistella; C Ram-Wolff; C Fite; V Meignin; J Rivet; M-D Vignon-Pennamen; T de Risi-Pugliese; E Raffoux; B Arnulf; E Oksenhendler; F Sicre de Fontbrune; R Peffault de Latour; G Socié; J-D Bouaziz; C Lebbé; A Bensussan; M Bagot

doi:10.1016/j.annder.2018.01.049

Cutaneous presentation of adult T-cell leukemia/lymphoma (ATLL). Single-center study on 37 patients in metropolitan France between 1996 and 2016

Ann Dermatol Venereol. 2018 Jun-Jul;145(6-7):405-412. doi: 10.1016/j.annder.2018.01.049. Epub 2018 Apr 14.

Authors

C Hurabielle¹, M Battistella², C Ram-Wolff³, C Fite⁴, V Meignin⁵, J Rivet⁵, M-D Vignon-Pennamen⁵, T de Risi-Pugliese³, E Raffoux⁶, B Arnulf⁷, E Oksenhendler⁷, F Sicre de Fontbrune⁸, R Peffault de Latour⁸, G Socié⁸, J-D Bouaziz⁹, C Lebbé⁹, A Bensussan¹⁰, M Bagot⁹

Affiliations

¹ Dermatology department, hôpital Saint-Louis, AP-HP, 1, avenue Claude-Vellefaux, 75010 Paris, France; Inserm Unité 976, hôpital Saint-Louis, 1, avenue Claude-Vellefaux, 75010 Paris, France.
² Histopathology department, hôpital Saint-Louis, France Inserm Unit 976, AP-HP, 1, avenue Claude-Vellefaux, 75010 Paris, France; Université Paris-Diderot, Sorbonne-Paris-Cité, 75000 Paris, France. Electronic address: maxime.battistella@aphp.fr.
³ Dermatology department, hôpital Saint-Louis, AP-HP, 1, avenue Claude-Vellefaux, 75010 Paris, France.
⁴ Dermatology department, hôpital Bichat, AP-HP, 46, rue Henri-Huchard, 75018 Paris, France.
⁵ Histopathology department, hôpital Saint-Louis, France Inserm Unit 976, AP-HP, 1, avenue Claude-Vellefaux, 75010 Paris, France.
⁶ Haematology department, hôpital Saint-Louis, AP-HP, 1, avenue Claude-Vellefaux, 75010 Paris, France.
⁷ Immunology-Haematology department, hôpital Saint-Louis, AP-HP, 1, avenue Claude-Vellefaux, 75010 Paris, France.
⁸ Haematology-transplantations department, hôpital Saint-Louis, AP-HP, 1, avenue Claude-Vellefaux, 75010 Paris, France.
⁹ Dermatology department, hôpital Saint-Louis, AP-HP, 1, avenue Claude-Vellefaux, 75010 Paris, France; Inserm Unité 976, hôpital Saint-Louis, 1, avenue Claude-Vellefaux, 75010 Paris, France; Université Paris-Diderot, Sorbonne-Paris-Cité, 75000 Paris, France.
¹⁰ Inserm Unité 976, hôpital Saint-Louis, 1, avenue Claude-Vellefaux, 75010 Paris, France; Université Paris-Diderot, Sorbonne-Paris-Cité, 75000 Paris, France.

PMID: 29661476
DOI: 10.1016/j.annder.2018.01.049

Abstract

Introduction: Adult T-cell leukemia/lymphoma (ATLL) is a hematological malignancy associated with chronic HTLV-1 infection.

Aim: To describe skin lesions in ATLL.

Methods: A descriptive, retrospective study between 1996 and 2016, including all patients diagnosed with ATLL at Saint-Louis Hospital (Paris, France).

Results: Thirty-seven ATLL patients were included. Fifteen patients (41%) had a cutaneous localization of the disease, which was present from the beginning of the disease for two thirds of them. ATLL types in patients with cutaneous localization of the disease were as follows: lymphoma, n=5, chronic, n=4, smoldering, n=4, acute, n=2. Half the patients had 2 or more cutaneous manifestations. The cutaneous localizations observed were as follows: nodulotumoral (n=8), plaques (n=7), multipapular (n=6), macular (n=4), purpuric (n=2). Among the 15 patients with cutaneous localization, median overall survival was significantly shorter in the acute and lymphoma types compared to the smoldering and chronic types (8.7 months vs. 79 months, P=0.003).

Discussion: ATLL is a hematologic malignancy with variable expression that is diagnosed only very rarely in metropolitan France, but that should be sought in patients from countries with high HTLV-1 prevalence in the event of a chronic eruption with patches, papules, plaques and/or tumors. The chronic and smoldering types are relatively indolent, whereas the acute and lymphoma forms have a poor prognosis.

Keywords: ATLL; Adult T-cell leukemia/lymphoma; HTLV-1; HTLV1; Hématodermie; Leucémie/lymphome T de l’adulte.

MeSH terms

Adult
Female
Humans
Leukemia-Lymphoma, Adult T-Cell / complications*
Leukemia-Lymphoma, Adult T-Cell / pathology
Male
Paris
Retrospective Studies
Skin Neoplasms / etiology*
Skin Neoplasms / pathology
Time Factors