American Journal of Dermatopathology:
Letters to the Editor
Kim, Nancy H MD*; Torchia, Daniele MD*; Miteva, Maria MD*; Rongioletti, Franco MD†; MD, Paolo Romanelli*
*Department of Dermatology and Cutaneous Surgery, University of Miami Miller School of Medicine, Miami, FL; and †Section of Dermatology, DISEM, University of Genoa, Genoa, Italy
To the Editor:
Adult T-cell leukemia/lymphoma (ATLL) is a T-cell neoplasm etiologically linked to the human T-cell leukemia virus 1 (HTLV-1) and therefore endemic in areas such as southwest Japan and the Caribbean. Skin lesions are generally a manifestation of widely disseminated disease. However, a slowly progressive form strictly confined to the skin, clinically and histologically indistinguishable from mycosis fungoides (MF), has been described as well.1
A 34-year-old Haitian woman was referred for a recent history of an asymptomatic nodular lesion. A well-demarcated oval, 3 by 2.5-cm, raised, eroded nodule was observed on the left shoulder (Fig. 1A). Histopathological examination of a punch biopsy showed a dense monomorphous infiltrate of atypical lymphocytes in the superficial and deep dermis. Intense epidermotropism and formation of Pautrier microabscesses were also detected (Fig. 1B). Notably, apoptotic cells and fragments in the dermis and apoptotic lymphocytes within Pautrier microabscesses were found (Fig. 2). Immunophenotyping revealed strong positivity for CD3, CD4 (Fig. 1C), and CD25 in most cells, whereas CD8 staining proved negative. Infection with HTLV-1 had been confirmed elsewhere by serology and polymerase chain reaction. A diagnosis of HTLV-1-associated ATLL was therefore made. No information about extracutaneous involvement was made available, and the patient was lost to follow-up.
It is commonly believed that ATLL and MF are almost indistinguishable at routine histological examination.2 Differential diagnosis clues include demonstration of HTLV-1 infection by polymerase chain reaction and/or serology and FoxP3 immunostaining, which proves positive in MF but negative in ATLL.2 However, it has been suggested that different from MF, in ATLL, prominent apoptotic debris are present within Pautrier microabscesses.3 This finding is in line with what observed in our case. Because HTLV-1-infected cells are known to feature a decreased apoptotic rate,4 we hypothesize that the increase of apoptotic material in ATLL skin lesions may be ascribed to faster replication and turnover of neoplastic cells in comparison with slower growing tumors such as MF. It is hoped that future studies will address the biological significance of prominent neoplastic cell apoptosis within ATLL lesions and whether this phenomenon warrants diagnostic and/or prognostic significance.
Nancy H. Kim, MD*
Daniele Torchia, MD*
Maria Miteva, MD*
Franco Rongioletti, MD†
Paolo Romanelli MD*
*Department of Dermatology and Cutaneous Surgery, University of Miami Miller School of Medicine, Miami, FL; †Section of Dermatology, DISEM, University of Genoa, Genoa, Italy
1. Johno M, Ohishi M, Kojo Y, et al. Cutaneous manifestations of adult T-cell leukaemia/lymphoma. Gann Monograph Cancer Res
2. Cerroni L, Gatter K, Kerl H. Cutaneous adult T-cell leukemia/lymphoma. In: Cerroni L, Gatter K, Kerl H, eds. Skin Lymphoma: The Illustrated Guide
. 3rd ed. Oxford, United Kingdom: Blackwell Publishing; 2009:114-116.
3. Strutton G. Adult T-cell leukemia/lymphoma (HTLV-1+). In: Weedon D, ed. Skin Pathology
. 2nd ed. London, United Kingdom: Churchill Livingstone; 2002:1116-1117.
4. Tomita M, Tanaka Y, Mori N. Aurora kinase inhibitor AZD1152 negatively affects the growth and survival of HTLV-1-infected T lymphocytes in vitro. Int J Cancer
© 2011 by Lippincott Williams & Wilkins.