PROGRAMM JAHRESTAGUNG 2012 30. Nov. – 2. Dez ... - ÖGDV

Poster Tumore der Haut
P 46
Subcutaneous panniculitis-like T-cell Lymphoma with α/β T-cell phenotype
associated with a hemophagozytic syndrome and maligne ascites
Bettina Kerbler 1
Michael Steurer 2
Matthias Schmuth 1
Elisabeth Muglach 1
Bernhard Zelger 1
Van Anh Nguyen 1
1 Department of Dermatology, Innsbruck Medical University, Innsbruck, Austria
2 Hematology and Oncology, Innsbruck Medical University, Innsbruck, Austria
Introduction: Subcutaneous panniculitis-like T cell lymphoma (SPTL) is a rare skin
T-cell lymphoma characterized by infiltration of the subcutaneous tissue with
pleomorphic T-cells that mimic lobular panniculitis. Patients with SPTL present with
typical nodular lesions or plaques that occur preferentially on the limbs and trunk and
often are misdiagnosed as benign as lupus panniculitis. Distinction should be made
between SPTL with an α/β T-cell phenotype (SPTL-AB) and SPTL with a γ/δ T-cell
phenotype (SPTL-GD). SPTL-ABs are generally confined to the subcutis, have a CD4-,
CD8+, CD56- phenotype, are uncommonly associated with a hemophagocytic
syndrome (HPS, 17 %) and have a favorable prognosis (5-year overall survival 82 %). On
the other hand, SPTL-GDs display epidermal involvement and/or ulceration, a CD4-,
CD8-, CD56+ phenotype and poor prognosis (5-year overall survival 11%). Based on
these observations, the term SPTL is used only for SPTL-ABs, whereas SPTL-GDs are
included into the group of cutaneous γ/δ T-cell lymphoma.
Methods and Results: We report a 34- year- old caucasian woman who noticed
subcutaneous nodules on her left leg and gluteal area. Two months later, she developed
persistently high-grade fevers, night sweats, chills and myalgias. Her abdomen was
distended due to ascites that was confirmed by ultrasound. Laboratory analysis
disclosed panzytopenia (hemoglobulin 100 g/l, WBC count 1,9 G/l, platelets 119 G/l),
elevated transaminases (ASAT 463 U/l, ALAT 197 U/l, γGT 80 U/l) and high levels of
lactatdehydrogenase (LDH) (1642 U/l). Antinuclear antibodies (1:160 speckled pattern)
and dsDNA were initially detected. Positron emission tomography (PET)/computed
tomography (CT) revealed areas of hypermetabolic activity in the subcutaneous
adipose tissue of the left leg and gluteal area as well as in the bones. Furthermore, the
liver and the spleen were markedly enlarged. A deep biopsy from the left leg
demonstrated a dense lobular proliferation of atypical lymphocytes with irregular and
hyperchromatic nuclei that infiltrated the subcutaneous fat with rimming around
adipocytes. No infiltration of the superficial dermis and epidermis was seen. The atypical
lymphocytic infiltrate was stained positive for CD3, CD2, CD7, CD5, CD8, T-cellintracytoplasmic
antigen1 and Granzyme B, but negative for CD 30, CD 56 and EBER.
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