Primary Cutaneous Gamma-Delta T-Cell Lymphoma With Long-Term Indolent Clinical Course Initially Mimicking Lupus Erythematosus Profundus
| Autor: | Markus Thieme, Patrick Terheyden, Mareike Witte, Mariella Fleischer, Christian D. Sadik, Laura von Dücker, Nathalie Stutz, Detlef Zillikens |
|---|---|
| Jazyk: | angličtina |
| Rok vydání: | 2020 |
| Předmět: |
0301 basic medicine
Cancer Research Pathology medicine.medical_specialty T-cell lymphoma cutaneous Case Report cutaneous gamma-delta T-cell lymphoma Disease Immunofluorescence Microscopy lcsh:RC254-282 lupus erythematosus profundus/panniculitis 03 medical and health sciences 0302 clinical medicine hemophagocytic syndrome (HPS) immune system diseases Primary cutaneous gamma-delta T-cell lymphoma medicine Clinical phenotype business.industry Lupus erythematosus profundus Clinical course lcsh:Neoplasms. Tumors. Oncology. Including cancer and carcinogens medicine.disease Lymphoma 030104 developmental biology Oncology 030220 oncology & carcinogenesis immunohistochemistry Immunohistochemistry business |
| Zdroj: | Frontiers in Oncology Frontiers in Oncology, Vol 10 (2020) |
| ISSN: | 2234-943X |
| Popis: | Primary Cutaneous Gamma-Delta (γδ) T-Cell Lymphoma (PCGDTCL) is a rare primary cutaneous lymphoma of aggressive nature. Only a few cases with an initially indolent course over years have been published. PCGDTCL can mimic diseases with benign behavior in their clinical and histopathological presentation, such as lupus erythematosus profundus, but also other lymphomas, for example subcutaneous panniculitis-like T-cell lymphoma. In our patient, the results of histopathological, immunofluorescence microscopy, and clinical examinations of early lesions first led to the diagnosis of lupus erythematosus profundus. Two years after this diagnosis and 6 years after the first clinical symptoms appeared, the disease progressed with erosive and ulcerating plaques and a PCGDTCL with hemophagocytic syndrome with an aggressive course was diagnosed. A distinct correlation of clinical, histopathological, immunohistochemical, and molecular-pathological examinations is needed to differentiate between the potentially malignant and benign diseases. Re-biopsies of different skin lesions in uncertain cases are strongly indicated. This case demonstrates that an indolent clinical phenotype can precede an aggressive clinical course in PCGDTCL. |
| Databáze: | OpenAIRE |
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