A recent study has described the clinical features associated with subcutaneous panniculitis-like T-cell lymphoma (SPTCL), a rare disease which is difficult to diagnose. The findings were reported in the journal Dermatology.
SPTCL is a rare condition with very few studies making diagnosing it difficult. A group of pathologists, dermatologists and oncologists have joined hands to evaluate clinical data retrospectively to address this disease’s knowledge gap.
Study design
The retrospective clinical study included cutaneous lymphoma patients from six large US academic centres between 1998 and 2018. Among the cohort of 95 cases, 75 cases were of SPTCL, and the remaining was of adipotropic lymphoproliferative disorder (ALPD; n = 20). The median age of the participants had a median age of 38 years (range, 2-81 years) and the female to male ratio was 2:7.
Main findings
The clinical presentation was similar in both groups, where multiple and single tender nodules were seen in 85% (61/72) and 15% (11/72) of the cases, respectively. However, hemophagocytic lymphohistiocytosis (HLH) was seen only in SPTCL cases. During a follow-up period of 56 months, complete remission was observed in 67% of the patients. Importantly, despite remission being commonly observed, no death due to disease or progression to HLH was seen. Two patients progressed from ALPD to SPTCL despite a lack of associated systemic symptoms or HLH.
Conclusion
Based on these findings, Joan Guitart, MD, of the department of dermatology and pathology at Northwestern University, said, “Our experience shows that SPTCL does not behave like other lymphomas. Unlike other malignancies, SPTCL morbidity and mortality are purely related to hemophagocytic syndrome — a cytokine storm — rather than metastatic progression, which was not observed in any of the cases.”
Reference
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