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 .