T-Cell Lymphoma With Sarcoid-Like Reaction and Secondary Myelofibrosis: A Unique Case Report and Literature Review.

Abstract

Introduction: T-cell lymphomas are rare, comprising 10%-15% of non-Hodgkin lymphomas (NHLs). Their etiology remains unclear, and pathophysiology varies widely among subtypes. Sarcoid-like reactions (SLRs) have been reported in approximately 7.3% of NHLs, including T-cell lymphomas, and can closely resemble sarcoidosis clinically/histologically. This overlap complicates diagnosis, particularly when secondary myelofibrosis-a rare and poorly understood complication-arises. Myelofibrosis has also been observed in sarcoidosis, further blurring the distinction between these entities. Treatment for T-cell lymphoma-associated secondary myelofibrosis is not standardized, with predominantly CHOP-based regimens showing variable efficacy. This warrants further research to guide diagnostic and management frameworks.

Case description: We report the case of a 57-year-old male with known systemic sarcoidosis who developed recurrent symptomatic hypercalcemia and new-onset transfusion-dependent pancytopenia. Initial evaluation revealed bone marrow fibrosis with noncaseating granulomas, raising concern for sarcoidosis-driven secondary myelofibrosis. However, repeat bone marrow biopsy demonstrated T-cell lymphoma with a SLR. Primary myelofibrosis was excluded, supporting a secondary lymphoma-driven process. The patient is currently undergoing treatment with BV-CHP with close hematologic monitoring.

Conclusion: T-cell lymphoma-associated SLR can closely mimic sarcoidosis and should be considered in the differential, particularly in patients with atypical presentations or cytopenias. Despite similar clinical and histological features, the underlying pathophysiology differs, necessitating thorough molecular and immunophenotypic evaluation. Though rare, this entity can lead to secondary myelofibrosis, underscoring the importance of excluding a primary myeloproliferative process. Further research is needed to define optimal therapy, with BV-CHP representing a promising option in the evolving landscape of T-cell lymphoma management.