BMSEHA15
Published online 30 November 2009
Haematologica, Vol 95, Issue 3, 449-455 doi:10.3324/haematol.2009.015024
Copyright © 2010 by Ferrata Storti Foundation
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Malignant Lymphomas

Cutaneous presentation of ALK-positive anaplastic large cell lymphoma following insect bites: evidence for an association in five cases

Laurence Lamant1, Stefano Pileri2, Elena Sabattini2, Laurence Brugières3, Elaine S. Jaffe4, Georges Delsol1

1 INSERM, U.563, Centre de Physiopathologie de Toulouse-Purpan, Toulouse, France, Université Paul-Sabatier, Toulouse, France; Laboratoire d’Anatomie Pathologique, CHU Purpan, Place Baylac, Toulouse, France;
2 Unit of Haematopathology, Department of Haematology and Oncological Sciences "L. & A. Seràgnoli", Bologna University, Policlinico S. Orsola, Bologna, Italy;
3 Département d’Oncologie Pédiatrique, Institut Gustave-Roussy, Villejuif Cedex, France and
4 Laboratory of Pathology, Center for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, MD, USA

Correspondence: Elaine S. Jaffe, Chief, Hematopathology Section, Laboratory of Pathology, Center for Cancer Research, National Cancer Institute Building 10/Room 2B 42 10 Center Drive MSC-1500 Bethesda, MD 20892-1500 USA. E-mail: elainejaffe{at}nih.gov

Background: Skin involvement is frequent in ALK-positive anaplastic large cell lymphomas. The role of an insect bite as a triggering event has been postulated but not well documented.

Design and Methods: We retrospectively investigated five cases of ALK-positive anaplastic large cell lymphoma who presented with skin lesions occurring after an insect bite. Biopsies were immunostained with antibodies against CD30, ALK, T- and B-cell antigens.

Results: Persistent skin lesions developed after solitary insect bites in three patients and after multiple bites in two. Regional lymphadenopathy developed within weeks after the bite in three cases. In four cases the correct diagnosis was delayed due to misinterpretation of the findings as a reactive infiltrate in the skin (n=2) or lymph nodes (n=2); all cases subsequently showed small numbers of cells with nuclear and cytoplasmic staining for ALK. The final diagnoses were lymphohistiocytic variant (n=3) and composite common/small cell type (n=2) anaplastic large cell lymphoma. The patients were treated and three were alive at the last follow-up. Two patients died, one of pneumonia and the other of disseminated disease.

Conclusions: In these cases the sequence of events between the insect bites and the occurrence of both skin lesions and satellite lymphadenopathy suggest a direct relationship between the bite and the presentation with anaplastic large cell lymphoma. We postulate that insect bite-associated antigens could result in an influx of T lymphocytes, some bearing the t(2;5). The subsequent release of cytokines at the site of the bite could act as a ‘second hit’, eliciting activation of the latter cells, which would then express the oncogenic NPM-ALK protein and undergo uncontrolled proliferation.

Key words: ALK-positive ALCL, NPM-ALK, skin involvement.