Subcutaneous panniculitis-like T-cell lymphoma in children: a detailed clinicopathological description of 11 multifocal cases with a high frequency of haemophagocytic syndrome

Oschlies, Ilske; Simonitsch‐Klupp, Ingrid; Małdyk, Jadwiga; Konovalov, D.M.; Abramov, D. S.; Myakova, Natalia; Lisfeld, Jasmin; Attarbaschi, Andishe; Kontny, Udo; Woessmann, Wilhelm; Klapper, Wolfram

doi:10.1111/bjd.13440

Cited by 35 publications

(32 citation statements)

References 10 publications

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“…Cytotoxic T cell lymphomas or lymphoproliferation within the skin and subcutaneous tissues are divided in two different entities in the present 2008 WHO classification; SP TCL, restricted to cases with αβ-phenotype and primary cutaneous gamma-delta T cell lymphoma (PCGD-TCL) with γδ-phenotype. Patients with SP TCL had the best outcome in our series, with a pOS at 5 years of 0.78, and this is in accordance to earlier reports [13,14]. Only one patient in our series was diagnosed with PCGD TCL, and that patient died from disease, in accordance to what is known from the literature [15].…”

Section: Discussionsupporting

confidence: 92%

Non-anaplastic peripheral T cell lymphoma in children and adolescents—an international review of 143 cases

et al. 2016

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Peripheral T cell lymphomas (PTCL) are rare in children and adolescents, and data about outcome and treatment results are scarce. The present study is a joint, international, retrospective analysis of 143 reported cases of non-anaplastic PTCL in patients <19 years of age, with a focus on treatment and outcome features. One hundred forty-three patients, between 0.3 and 18.7 years old, diagnosed between 2000 and 2015 were included in the study. PTCL not otherwise specified was the largest subgroup, followed by extranodal NK/T cell lymphoma, hepatosplenic T cell lymphoma (HS TCL), and subcutaneous panniculitis-like T cell lymphoma (SP TCL). Probability of overall survival (pOS) at 5 years for the whole group was 0.56 ± 0.05, and probability of event-free survival was (pEFS) 0.45 ± 0.05. Patients with SP TCL had a good outcome with 5-year pOS of 0.78 ± 0.1 while patients with HS TCL were reported with 5-year pOS of only 0.13 ± 0.12. Twenty-five percent of the patients were reported to have a pre-existing condition, and this group had a dismal outcome with 5-year pOS of 0.29 ± 0.09. The distribution of non-anaplastic PTCL subtypes in pediatric and adolescent patients differs from what is reported in adult patients. Overall outcome depends on the subtype with some doing better than others. Pre-existing conditions are frequent and associated with poor outcomes. There is a clear need for subtype-based treatment recommendations for children and adolescents with PTCL.

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Section: Discussionsupporting

confidence: 92%

Non-anaplastic peripheral T cell lymphoma in children and adolescents—an international review of 143 cases

et al. 2016

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“…CNS involvement was not detected in any of them. The more detailed clinical and histopathological features of these five patients with this extremely rare skin/subcutis‐restricted lymphoma are described in a series of 11 paediatric patients in a separate report (Oschlies et al , in press). The five patients are alive in CR1 with a median follow‐up of 2·2 years.…”

Section: Resultsmentioning

confidence: 99%

Non‐anaplastic peripheral T‐cell lymphoma in children and adolescents – a retrospective analysis of the NHL‐BFM study group

et al. 2014

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SummaryMature (peripheral) T-cell lymphoma (PTCL) other than anaplastic large cell lymphoma is a heterogeneous group of diseases and exceedingly rare in children and adolescents. Survival rates range between 46% and 85%. This study reports the disease characteristics, treatment and outcome of all patients with the diagnosis of mature TCL registered in the Berlin-Frankfurt-Munster non-Hodgkin lymphoma database between 1986 and 2012. All diagnoses were centrally reviewed and revised by clinico-pathological correlation according to the criteria of the current World Health Organization classification. Of the 69 patients originally registered as having PTCL, the diagnosis was confirmed in 38 of them. Most patients were treated with an anaplastic large cell lymphoma (ALCL)-like therapy regimen. Patients with PTCL-not otherwise specified comprised the largest group and showed a 5-year event-free survival rate of 61 AE 11%. Patients suffering from Natural Killer/T-cell-and hepatosplenic TCL had the poorest outcome. Our results suggest that the outcomes of children with mature TCL other than ALCL depend on the subtype and are worse than in all other paediatric lymphomas. The clinical experience presented in this largest study on paediatric mature TCL may serve as basis for future collaborative international prospective clinical trials.

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“…[13] The presence of MAS or hemophagocytosis (HPS) might change the management approach and outcome in SPTCL. [14] One study reported 5-year overall survival rates of 91% and 46% in SPTCL patients without and with an HPS, respectively. [1] In SPTCL without associated HPS, careful observation, systemic steroids or other immunosuppressive agents could be considered first, whereas in cases of solitary or localized skin lesions, radiotherapy with electrons is advised.…”

Section: Discussionmentioning

confidence: 99%

Subcutaneous panniculitis-like T-cell lymphoma with macrophage activation syndrome treated by cyclosporine and prednisolone

Asati

Ingle

Joshi

et al. 2016

Indian Dermatol Online J

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Subcutaneous panniculitis-like T-cell lymphoma (SPTCL; α/β T-cell subtype) is a distinct variantof cutaneous T-cell lymphomas, which presents as inflammatory subcutaneous nodules. A 17-year-old male presented with recurrent fever with concomitant facial swelling, pedal edema, hepatosplenomegaly, and mildly tender subcutaneous plaques in generalized distribution along with patches of scarring alopecia on scalp. There were features of macrophage activation syndrome in the form of hemophagocytosis in the bone marrow, pancytopenia, high serum lactate dehydrogenase levels, low fibrinogen clotting activity, prolonged activated prothrombine time (aPTT), increased serum ferritin, hypoalbuminemia, and hypertriglyceridemia. Histopathology showed lobular panniculitis-like infiltration by atypical lymphocytes rimming the adipocytes. Immunohistochemistry revealed positive CD3 and CD8 markers, whereas CD4, CD56, and CD20 were negative, consistent with the diagnosis of α/β type of SPTCL. Treatment with oral prednisolone (1mg/kg/day) and cyclosporine (2mg/kg/day; 100 mg) led to rapid subsidence of fever, plaques, and abnormal hematological parameters within a few weeks.

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Subcutaneous panniculitis-like T-cell lymphoma in children: a detailed clinicopathological description of 11 multifocal cases with a high frequency of haemophagocytic syndrome

Abstract: Subcutaneous panniculitis-like T-cell lymphoma in children -a detailed clinico-pathological description of 11 multifocal cases: high frequence of hemophagocytic syndrom

Cited by 35 publications

References 10 publications

Non-anaplastic peripheral T cell lymphoma in children and adolescents—an international review of 143 cases

Non-anaplastic peripheral T cell lymphoma in children and adolescents—an international review of 143 cases

Non‐anaplastic peripheral T‐cell lymphoma in children and adolescents – a retrospective analysis of the NHL‐BFM study group

Subcutaneous panniculitis-like T-cell lymphoma with macrophage activation syndrome treated by cyclosporine and prednisolone

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