Subcutaneous Panniculitis-Like T-Cell Lymphoma With Hemophagocytic Lymphohistiocytosis.
J Investig Med High Impact Case Rep
; 12: 23247096241253337, 2024.
Article
in En
| MEDLINE
| ID: mdl-38742532
ABSTRACT
Subcutaneous panniculitis-like T-cell lymphoma (SPTLP), a unique variant of primary cutaneous T-cell lymphomas, clinically mimics subcutaneous panniculitis. It is typified by the development of multiple plaques or subcutaneous erythematous nodules, predominantly on the extremities and trunk. Epidemiological findings reveal a greater incidence in females than males, affecting a wide demographic, including pediatric and adult cohorts, with a median onset age of around 30 years. Diagnosis of SPTLP is complex, hinging on skin biopsy analyses and the identification of T-cell lineage-specific immunohistochemical markers. Treatment modalities for SPTLP are varied; while corticosteroids may be beneficial initially for many patients, a substantial number require chemotherapy, especially in cases of poor response or relapse. Generally, SPTLP progresses slowly, yet approximately 20% of cases advance to hemophagocytic lymphohistiocytosis (HLH), often correlating with a negative prognosis. We report a case of a young male patient presenting with prolonged fever, multiple skin lesions accompanied by HLH, a poor clinical course, and eventual death, diagnosed postmortem with SPTLP. In addition, we also present a literature review of the current evidence of some updates related to SPTLP.
Key words
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Main subject:
Panniculitis
/
Lymphoma, T-Cell
/
Lymphohistiocytosis, Hemophagocytic
Limits:
Adult
/
Humans
/
Male
Language:
En
Journal:
J Investig Med High Impact Case Rep
/
J. Investig. Med. High Impact Case Rep
/
Journal of investigative medicine high impact case reports
Year:
2024
Document type:
Article
Affiliation country:
Vietnam
Country of publication:
Estados Unidos