AI Article Synopsis
- Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare hematologic cancer affecting subcutaneous fat, often accompanied by systemic symptoms.
- Typical treatments, like CHOP and related regimens, may not be effective, as seen in four cases that were resistant to standard therapies but showed significant improvement after starting oral cyclosporin.
- The study recommends considering cyclosporin as a potential treatment option for SPTCL, with some patients achieving complete remission for 8 to 9 months following its use.
Article Abstract
Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare form of hematologic malignancy characterized by lesions in subcutaneous fat associated with systemic symptoms. The standard treatment of the disease, currently, is not established, but CHOP or CHOP-like regimens are usually given. We report, herein, 4 cases of SPTCL diagnosed by histopathology and immunohistochemistry who were refractory to CHOP and/or ESHAP and/or fludarabine-based regimen, but showed rapid improvement within weeks after oral cyclosporin 4 mg/kg/day. Three sustained complete remission for the durations of 8 - 9 months off-treatments. T-cell receptor gene rearrangement revealed polyclonality in 3 cases and monoclonality in 1 case. Our data suggest the benefit of incorporating cyclosporin into the treatment regimen for SPTCL.
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| http://dx.doi.org/10.1080/10428190601078456 | DOI Listing |
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Article Synopsis
- Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare type of T-cell lymphoma that primarily affects the skin and has a high five-year survival rate of over 80%.
- A study involving whole-exome sequencing of 60 samples from 36 SPTCL patients identified 138 mutations in skin tumors and recurring HAVCR2 germline mutations in many patients, particularly in Japanese individuals.
- Despite treatment with multiple chemotherapy agents, some patients showed only partial responses, but their conditions remained stable with further treatments, highlighting the importance of long-term monitoring due to potential complications.
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