Primary Cutaneous Peripheral T-Cell Lymphoma With Follicular Helper T-Cell Phenotype: Report of 2 Epstein-Barr Virus-Positive Cases.

ABSTRACT: Primary cutaneous T-cell lymphoma is distinct from nodal T-cell lymphoma clinically and pathologically. Recently, primary cutaneous follicular helper T-cell lymphoma (PC-TFHL) has been described as a peripheral T-cell lymphoma with T-follicular helper (TFH) cell phenotype. PC-TFHL usually presents as multiple plaques and nodules of skin with an indolent clinical course, but without association with Epstein-Barr virus. In this article, we report 2 rare cases of PC-TFHL which are Epstein-Barr virus-positive and with an aggressive clinical course. We discuss the challenges in the differential diagnoses, particularly with primary cutaneous extranodal NK/T-cell lymphoma, and nodal T-cell lymphoma of TFH origin with secondary cutaneous involvement.

Primary cutaneous gamma/delta T-cell lymphoma in Taiwan: A series of six cases with frequent solitary presentation and relatively indolent behavior.

BACKGROUND: Primary cutaneous gamma/delta T-cell lymphoma (PCDG-TCL) is aggressive, frequently presenting as multiple plaques, tumors, and/or subcutaneous nodules. METHODS: In this study, we conducted a retrospective study in a tertiary center in Taiwan to characterize this rare tumor. RESULTS: We identified six patients. Five presented with a solitary lesion, including two with clinical impression of epidermal inclusion cyst or lipoma. Two of four evaluable cases exhibited epidermotropism, with one mimicking Pautrier microabscess. The neoplastic cells were pleomorphic and mostly medium- to large-sized. In all cases, the neoplastic cells expressed T-cell receptor (TCR)-γ and/or TCR-δ, with four co-expressing ßF1. Two of these ßF1+ cases co-expressed TCR-γ but not TCR-δ (two different clones). All were negative for Epstein-Barr virus (EBV), low stage, and treated with radiotherapy alone or combined chemotherapy and radiotherapy. In two patients, lymphoma relapsed in 3 and 7 months, respectively, and one patient died of the disease in 7 months. Four other patients were free of disease for 6 to 126 months. CONCLUSION: PCGD-TCL cases in Taiwan are more commonly solitary, frequently with indolent courses. The two currently available TCR-δ clones alone might be insufficient to detect all tumors.

A Case of Fabry Disease Presenting with Young Stroke and Fever.

Although it is known that Fabry disease should be included in the differential diagnosis of young stroke and fever of unknown origin, it has not been reported in the literature of stroke with fever as the presentation of Fabry disease. This is relevant because stroke with fever may misguide the differential diagnosis. Here we present a young stroke patient with fever. DWI of brain MRI revealed acute multiple infarctions. Due to the fever, infectious and inflammatory origins such as infective endocarditis and autoimmune diseases were examined first. However, we could not identify the source of fever after fever workup. The fever did not respond to one week of acetaminophen and antibiotics, but responded promptly to steroid. Our patient is also a case of de novo mutation rather than being inherited that further complicates the diagnosis of this patient. Because of the rare combined presentation of stroke with fever, Fabry disease should also be considered in stroke with fever, even without family history of Fabry disease.

Central granular cell odontogenic tumor: the first reported case in Oriental people and literature review.

The central granular cell odontogenic tumor (CGCOT) is a rare odontogenic neoplasm, usually occurring in the mandible of middle-aged women. Previous studies have reported only 34 cases, all of whom were white or black individuals. The present study reports an additional case of CGCOT, occurring in the posterior mandible of a 69-year-old Taiwanese man. To the authors' knowledge, this is the first reported case in Oriental people. The odontogenic epithelium exhibited strong positive immunoreactivity for pan-cytokeratin, and focal weak staining for bcl-2. The granular cells showed strong positivity for vimentin and α1-antichymotrypsin, and focal weak staining for carcinoembryonic antigen (CEA), neuron-specific enolase (NSE), and CD68. These features indicated a mesenchymal origin and possible histiocytic lineage for the granular cells. This study also presents a literature review and describes immunohistochemical features of the tumor.