Abstract
Multicentric Castleman’s disease is a polyclonal lymphoproliferative disorder. Recently, a new variant of the disease was reported and named TAFRO syndrome, an acronym for thrombocytopenia, ascites, myelofibrosis, renal dysfunction, and organomegaly. A 55-year-old woman presented to our hospital with dyspnea on exertion and high fever. Laboratory tests revealed anemia, thrombocytopenia, and proteinuria. Computed tomography (CT) revealed a large anterior mediastinal mass, mild splenomegaly, bilateral pleural effusion, pericardial effusion, and mild systemic lymphadenopathy. A CT-guided biopsy was unable to establish a definitive diagnosis, so we resected the mediastinal mass for diagnostic and therapeutic purposes. Pathological findings were consistent with the hyaline vascular type of Castleman’s disease (CD), and she was diagnosed with TAFRO syndrome. There has been no description of a patient with TAFRO syndrome with a large mass, and this is the first case of TAFRO syndrome treated with debulking surgery.
Highlights
Castleman’s disease (CD) is a rare, non-neoplastic lymphoproliferative disorder first reported in 1956 [1]
The human herpesvirus-8 (HHV-8) and human immunodeficiency virus (HIV) infections are a well-established cause of hypercytokinemia in Multicentric Castleman’s disease (MCD) patients with plasma cell histology [6]
The histopathological and systemic features in idiopathic MCD are thought to be secondary to hypercytokinemia caused by these diseases, and the new disease concept of TAFRO syndrome was recently proposed in Japan as a unique clinicopathological variant of idiopathic MCD
Summary
Castleman’s disease (CD) is a rare, non-neoplastic lymphoproliferative disorder first reported in 1956 [1]. Multicentric Castleman’s disease (MCD) is a CD subtype with multiple lesions and systemic symptoms that includes a heterogeneous group of disorders with various etiologies. Computed tomography (CT) revealed a large anterior mediastinal mass, mild splenomegaly, bilateral pleural effusion, pericardial effusion, and mild systemic lymphadenopathy (Fig. 1). A CTguided biopsy of the mediastinal mass was unable to establish a definitive diagnosis, showing only severe fibrosis with a few spindle cells.
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