Track: Case Reports

Abstract:

Nodal T-follicular helper cell lymphoma (T-FHCL) is a subtype of non-Hodgkin lymphoma encompassing three aggressive variants: angioimmunoblastic-type, follicular-type, and not otherwise specified (NOS). All three subtypes are treated similarly, despite biological variations. Several reports describe the angioimmunoblastic T-cell lymphoma masquerading as Drug-Induced Hypersensitivity Syndrome (DIHS)/Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS). These cases underscore the diagnostic complexity due to overlapping clinical features including rash, lymphadenopathy, eosinophilia, and multiorgan involvement.

We present the case of a 78-year-old woman who presented with acute encephalopathy, prominent lymphadenopathy, and pruritic erythroderma. Initial evaluation favored possible DIHS/DRESS, with a RegiSCAR score of 5, and a punch biopsy of the left arm showing superficial perivascular lymphocytic infiltrate, epidermal acanthosis, and many eosinophils. Atypical features, including persistent lymphadenopathy despite treatment, prompted further investigation. A second punch biopsy of the left arm, performed with concern for infection, showed superficial and deep perivascular lymphocytic infiltrate with eosinophils and subcutaneous abscess. Lymph node biopsy was also completed; molecular studies revealed clonal T-cell receptor (TCR) gamma and beta gene rearrangements in this sample and the initial punch biopsy with similar neoplastic clone. A final diagnosis of CD4+ nodal T-FHCL, NOS was made, and patient was referred to oncology.

This case underscores the diagnostic challenge of T-FHCL presenting with features mimicking DIHS/DRESS. The overlap in clinical and histopathologic findings can obscure the correct diagnosis. In patients with atypical presentations of DIHS/DRESS, or in patients who fail standard therapies, it is essential to consider the possibility of an underlying T-FHCL.

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