Subcutaneous panniculitis-like T-cell lymphoma with hemophagocytic syndrome: A case report.
- Author:
Ranillo V. Gamutan
1
Author Information
- Publication Type:Journal Article
- Keywords: Subcutaneous panniculitis-like T-cell lymphoma; lupus erythematosus panniculitis
- From: Journal of the Philippine Medical Association 2021;100(1):58-65
- CountryPhilippines
- Language:English
-
Abstract:
INTRODUCTION:Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare disease, accounting for less than 1 % of non-Hodgkin's lymphomas that is
characterized by infiltration of T-cells in the
subcutaneous adipose tissue.
CASE PRESENTATION:A 21-year-old Filipino female presented with intermittent fever which eventually was associated with multiple eruption of cutaneous lesions. The serum antinuclear antibody (ANA) titer and pattern revealed negative results. Further clinical investigation prompted a skin lesion punch biopsy revealing atypical lymphoid infiltrates with lobular panniculitis consistent with SPTCL. lmmunohistochemical studies stained strongly posItIve for CD3, CDS, granzyme B and negative for CD20, CD4, and CD56. The case was also compounded with the existence of hemophagocytic syndrome having fulfilled five of the eight criteria . She was given prednisone (1 mg/kg/day) with gradual resolution of cutaneous lesions leaving marked hyperpigmentation and lipoatrophy.
DISCUSSION:SPTCL presents with a myriad of systemic symptoms but will always present with skin lesions. The biopsy for histopathological interpretation is commonly performed as a first diagnostic step followed by immunohistochemical staining. Clinical presentation, histo-pathological findings, and 1mmunohistochemical results together clinched the diagnosis of SPTCL.
CONCLUSION:Patients with history of intermittent fever associated with cutaneous lesions and systemic clinical findings, SPTCL should be a differential diagnosis. However, lupus erythematosus panniculitis (LEP) must be ruled out first as its closest mimicker. This case report along with other large case series provides further evidence that monotherapy with oral prednisone can be an initial choice of therapy. - Full text:subcutaneous.pdf
