Rare T-Cell Subtypes

Pediatric Subcutaneous Panniculitis-like T-cell Lymphoma of the Orbit

“…3). 3,4 Flow cytometry confirmed the tumor was of the alpha/beta type and the cytotoxic T-cell lineage.…”

“…There is often overlying induration without ulceration and residual fat atrophy. 3 Orbital involvement is rare and eyelid lesions can present with localized edema, discoloration, or erythema, which can masquerade as nonresolving preseptal cellulitis. The typical demographic of SPTCL is a young adult with a median age of 36 years and female predominance (female to male ratio, 2:1).…”

“…1 The lace-like rimming of neoplastic T cells around individual adipose cells is another supportive histological feature. 3,4 Furthermore, admixed reactive histiocytes in the absence of inflammatory plasma cells and plasmacytoid dendritic cells helps to distinguish SPTCL from lupus panniculitis. Our case is consistent with SPTCL based on the following: T-cell lymphoma with a subcutaneous panniculitis-like pattern, CD3+, CD4–, CD8+, CD56–, EBV–, and compatible flow cytometry.…”

“…1,2 Approximately 20% of cases are associated with haemophagocytic syndrome (HPS), an uninhibited systemic inflammatory response characterized by fever, hepatosplenomegaly, and pancytopenia. 1–4 We report the youngest known case of primary orbital SPTCL, highlight the clinicopathological features and provide a review of the literature. This report was approved by the institutional Human Research Ethics Committee and compliant with tenets of the Declaration of Helsinki.…”