Hemophagocytic Lymphohistiocytosis as the Initial Presentation of Subcutaneous Panniculitis-Like T-Cell Lymphoma: A Rare Case Responding to Cyclosporine A and Steroids
| Autor: | Arya Loghmani, Katharine Thomas, Caitlin Sullivan, Rajasree Pia Chowdry, Rachna Jetly-Shridhar |
|---|---|
| Jazyk: | angličtina |
| Rok vydání: | 2020 |
| Předmět: |
Adult
Male medicine.medical_specialty Panniculitis Epidemiology Case Report Malignancy Lymphoma T-Cell Lymphohistiocytosis Hemophagocytic corticosteroids 030207 dermatology & venereal diseases 03 medical and health sciences 0302 clinical medicine Subcutaneous Panniculitis-Like T-Cell Lymphoma medicine lcsh:Pathology Humans Safety Risk Reliability and Quality subcutaneous panniculitis-like T-cell lymphoma Hemophagocytic lymphohistiocytosis lcsh:R5-920 medicine.diagnostic_test business.industry medicine.disease Dermatology Mucinosis Lymphoma hemophagocytic lymphohistiocytosis 030220 oncology & carcinogenesis Skin biopsy Cyclosporine Steroids business lcsh:Medicine (General) Safety Research Progressive disease cyclosporine A lcsh:RB1-214 |
| Zdroj: | Journal of Investigative Medicine High Impact Case Reports, Vol 8 (2020) Journal of Investigative Medicine High Impact Case Reports |
| ISSN: | 2324-7096 |
| Popis: | Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare peripheral cytotoxic T-cell lymphoma, clinically resembling panniculitis. Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening syndrome of immune overactivation, triggered by underlying conditions. SPTCL presenting with HLH may represent a severe and rapidly progressive disease course. Currently, there is no standardized approach to treatment of HLH secondary to underlying SPTCL. A 34-year-old Asian male presented with a several months history of high fevers, weight loss, and nonpruritic purple discoloration of the skin. He had a skin biopsy showing atypical lymphohistiocytic panniculitis with dermal mucinosis and erythrophagocytosis consistent with SPTCL. The patient was initiated on treatment with dexamethasone and cyclosporine A. Almost immediate improvement of his skin lesions was noted and laboratory abnormalities trended toward baseline within 2 weeks. He noted complete symptom resolution after 3 months on therapy. SPTCL may be treated effectively with cyclosporine A and steroids to achieve rapid clinical and symptom management of this rare malignancy. |
| Databáze: | OpenAIRE |
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