Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) with probable mesentery involvement with associated hemophagocytic syndrome (HPS) – how to treat it?
| العنوان: | Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) with probable mesentery involvement with associated hemophagocytic syndrome (HPS) – how to treat it? |
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| المؤلفون: | Krystyna Gałązka, Dagmara Zimowska-Curyło, Beata Piątkowska-Jakubas, Małgorzata Raźny, Sarah Goldman-Mazur, Marcin Jońca, Mateusz Wilk, Tomasz Sacha, Agnieszka Giza |
| المصدر: | Journal of Dermatological Treatment. 33:2674-2676 |
| بيانات النشر: | Informa UK Limited, 2022. |
| سنة النشر: | 2022 |
| مصطلحات موضوعية: | Adult, Male, medicine.medical_specialty, Panniculitis, Hepatosplenomegaly, Dermatology, Lymphoma, T-Cell, Gastroenterology, Lymphohistiocytosis, Hemophagocytic, Cutaneous lymphoma, 030207 dermatology & venereal diseases, 03 medical and health sciences, 0302 clinical medicine, Subcutaneous Panniculitis-Like T-Cell Lymphoma, hemic and lymphatic diseases, Internal medicine, medicine, Humans, Mesentery, 030203 arthritis & rheumatology, business.industry, Cutaneous T-cell lymphoma, medicine.disease, Pancytopenia, Transplantation, medicine.anatomical_structure, B symptoms, medicine.symptom, business, Subcutaneous tissue |
| الوصف: | Subcutaneous panniculitis-like T-cell lymphoma (SPTL) is a rare, cutaneous lymphoma involving subcutaneous adipose tissue. SPTL is associated in less than 20% with hemophagocytic syndrome (HPS). A 5-year overall survival rate is inferior in patients with SPTL and HPS (46%) as compared with 91% in patients without HPS. No standardized therapy for SPTCL has yet been established. This is a case of 35-year-old Caucasian man with a one-month history of B symptoms with the suspicion of Still's disease, at admission with leucopenia, high LDH, ferritin, sIl-R2, and triglycerides levels, hepatosplenomegaly, small right supraclavicular nodule, and irregular thickening of trunk subcutaneous tissue. The abdomen MRI showed generalized thickening of mesentery and colonic mucosa. In the patient, diagnosis of SPTCL was established with secondary HPS. CHOEP chemotherapy and modified HLH 2014 protocol were applied with subsequent high dose chemotherapy (BEAM) supported by autologous stem cells transplantation. Treatment was complicated by pancytopenia and pneumonia. The outcome of the disease treated by intensive protocol seems to be good. |
| تدمد: | 1471-1753 0954-6634 |
| الوصول الحر: | https://explore.openaire.eu/search/publication?articleId=doi_dedup___::71ca38264b92699929b1a7019291fd98Test https://doi.org/10.1080/09546634.2020.1809624Test |
| حقوق: | OPEN |
| رقم الانضمام: | edsair.doi.dedup.....71ca38264b92699929b1a7019291fd98 |
| قاعدة البيانات: | OpenAIRE |
| تدمد: | 14711753 09546634 |
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