Idiopathic multicentric Castleman's disease with TAFRO syndrome: a case report

Abstract

Abstract Introduction/Objective Multicentric Castleman's disease(MCD) is a nonneoplastic lymphoproliferative disorder. Individuals affected by MCD exhibit systemic symptoms accompanied by the presence of multiple enlarged lymph nodes. MCD can be associated with human herpesvirus type-8, POEMS syndrome, or idiopathic (iMCD). TAFRO syndrome, a clinical subtype of MCD is a recently recognized disorder with an unclear cause, it comes with a combination of symptoms, namely thrombocytopenia, anasarca, fever, renal dysfunction, and organomegaly. This case report aims to shed light on the diagnostic challenges due to the rarity and nonspecific nature of TAFRO syndrome. Methods/Case Report We report a 30-year-old female who presented with night sweats, fever, fatigue, and cough. She had significant leucocytosis, lymphopenia, thrombocytopenia, anemia requiring transfusion, and elevated creatinine, CT showed ground glass opacities in the lungs and extensive lymphadenopathy. Axillary lymph node excisional biopsy revealed paracortical hyperplasia with increased vascularity, extensive polytypic plasmacytosis, and some follicular hyperplasia. On IHC staining it was positive for CD20, CD79a, PAX 5, and negative cyclin D1 for B lymphocytes. T lymphocytes were positive for CD3, CD5, CD2, CD7, CD4>CD8, Bcl2 and CD43. Plasma cells were composed mainly of IgG cells and some IgG4(IgG4/IgG <20%). HHV8 was negative. Flow cytometry showed 89% lymphocytes with a heterogenous population of T-cells and polytypic B cells. Serology revealed markedly elevated IL- 6, IL-2, VEGF CRP, and ESR while HIV and ANA were negative. Bone marrow biopsy was suggested. A final diagnosis of idiopathic multicentric castle man disease suggestive of TAFRO syndrome was made. The patient received steroids and is currently on siltuximab. Results (if a Case Study enter NA) NA Conclusion Our case fulfills at least four of the five major diagnostic criteria of TAFRO syndrome. It serves as a valuable addition to the scarce literature on TAFRO syndrome, providing insights into its diagnostic challenges