358 CLINICAL REPORT
ActaDV ActaDV Advances in dermatology and venereology Acta Dermato-Venereologica
Subcutaneous Panniculitis-like T-cell Lymphoma: Immunosuppressive Drugs Induce Better Response than Polychemotherapy
David MICHONNEAU 1, 2, Tony PETRELLA 3, Nicolas ORTONNE 4, Saskia INGEN-HOUSZ-ORO 5, Nathalie FRANCK 6, Stéphane BARETE 7, Maxime BATTISTELLA 8, Marie BEYLOT-BARRY 9, Beatrice VERGIER 10, Marc MAYNADIÉ 11, Christine BODEMER 12, Olivier HERMINE 2, 13, Martine BAGOT 14, 15, Nicole BROUSSE 1, 13 and Sylvie FRAITAG 1
1
Service d’ anatomie pathologique, 2 Service d’ hématologie adulte and 12 Service de dermatologie, Hôpital Necker-Enfants Malade, APHP, Université Paris Descartes, Paris, 3 Service d’ anatomie pathologique, Hôpital Maisonneuve-Rosemont, Montréal, Canada, 4 Service d’ anatomie pathologique and 5 Service de dermatologie, Hôpital Henri Mondor, APHP, Université Paris Est, Créteil, 6 Service de dermatologie, Hôpital Cochin, APHP, Université Paris Descartes, 7 UF de dermatologie, Hôpital La Pitié-Salpétrière, APHP, Université Pierre et Marie Curie, 8 Service d’ anatomie pathologique, 14 Service de dermatologie, Hôpital Saint Louis, APHP, Université Paris Diderot, Paris, 9 Service de dermatologie, CHU de Bordeaux, Université de Bordeaux, Bordeaux, 10 Service d’ anatomie pathologique, CHU de Bordeaux, Bordeaux, 11 Registre des hémopathies malignes de Côte d’ Or, Université de Bourgogne, CHU de Dijon, Dijon, 13 INSERM U1163 and CNRS ERL8654, Imagine Institute, Paris, and
15
INSERM U976, Hôpital Saint-Louis, APHP, Paris, France
Subcutaneous panniculitis-like T-cell lymphoma( SPT- CL) is a rare condition usually considered to have a favourable prognosis. However, it is not known whether polychemotherapy or immunosuppressive-based therapy is the best approach for treating SPTCL. Using data collected between 2000 and 2012 in France, we analysed clinical, biological and pathological data of 27 patients with SPTCL. Medical history revealed that 40 % of patients had been previously diagnosed with an autoimmune disorder and 22 % with inflammatory panniculitis. Haemophagocytic syndrome was present in 37 % of cases. Autoantibodies were positive in 65 % of cases. Complete remission( CR) was reached in 74 % of cases. Immunosuppressive drug treatment was given in 69.5 % of patients( group 1) and polychemotherapy in 30.5 %( group 2). CR was 81.2 % and 28.5 %( p = 0.025), respectively. Progression rate was 6.2 % and 42.8 %( p = 0.067), respectively. This study suggests that immunosuppressive drugs should be considered as the first-line treatment for SPTCL.
Key words: subcutaneous panniculitis-like T-cell lymphoma; chemotherapy; immunosuppresive agents.
Accepted Oct 6, 2016; Epub ahead of print Oct 10, 2016 Acta Derm Venereol 2017; 97: 358 – 364.
Corr: Sylvie Fraitag, Service d’ anatomie pathologique, Hôpital Necker- Enfants Malades, 149 rue de Sèvres, FR-75015 Paris, France. E-mail: sylvie. fraitag @ aphp. fr
Subcutaneous panniculitis-like T-cell lymphoma
( SPTCL) is a rare condition, defined as a cytotoxic T cell-mediated lymphoma of αβ-T-cell origin( SPTCL- AB)( 1). Diagnostic criteria have been described in the World Health Organization( WHO) classification and γδ- T-cell lymphomas are now considered as a distinct entity( 2, 3). In 2008, the European Organisation for Research and Treatment of Cancer( EORTC) conducted a major study, which highlighted the good prognosis of SPTCL and the adverse impact of haemophagocytic syndrome( HPS) on survival( 4). In this study, most patients were treated with polychemotherapy, while approximately one-third received immunosuppressive drugs. However, the respective efficacy of these treatments was not evaluated. Most other cohorts have reported patients treated with polychemotherapy, where complete response( CR) was reached in 0 – 67 % of patients( 5 – 7). By contrast, numerous case reports or small series suggest that immunosuppressive drugs could also be effective in some cases( 8 – 14). It should be emphasized that interpretation of data can be sometimes hampered by the possibility of misdiagnoses. Indeed, one of the main alternative diagnoses is lupus erythematosus profundus( 15 – 17), resulting in some lupus panniculitis being misdiagnosed as SPTCL. The possible association of SPTCL with autoimmunity further increases the difficulty of diagnosis( 18 – 21), as oligo- or monoclonal T-cell receptor rearrangement can occasionally be detected in lupus panniculitis( 22). New criteria have been proposed to distinguish these 2 disorders, based on the expression of human myxovirus resistance protein 1( MxA)( 23) or the presence of plasmacytoid dendritic cell clusters( 24) in lupus panniculitis, but not in SPTCL. Now that the SPTCL diagnosis criteria have been better described and clearly defined, the French Group for Study of Cutaneous Lymphoma( GFELC) favours a conservative approach, with immunosuppressive drugs as first-line treatment, as long as the patient’ s health condition allows it. The GFELC has systematically reviewed all cases of SPTCL that were registered in its database according to WHO classification criteria. The aims were to better characterize the clinical and biological features of SPTCL; to review all biopsies in order to homogenize and confirm histological analysis; and to compare different therapeutic strategies in terms of response and survival. This study reports the analysis of 27 patients who were diagnosed with an SPTCL in France.
PATIENTS AND METHODS Patients
A retrospective multicentre analysis of patients with an SPTCL diagnosed between 2000 and 2012 in France was conducted. Two databases were used to recruit patients. The GFELC register doi: 10.2340 / 00015555-2543 Acta Derm Venereol 2017; 97: 358 – 364
This is an open access article under the CC BY-NC license. www. medicaljournals. se / acta Journal Compilation © 2017 Acta Dermato-Venereologica.