Thrombocytopenia, ascites, myelofibrosis, renal dysfunction, and organomegaly (TAFRO) syndrome is a newly recognized but rare disease, and its treatment has not yet been established. We reported a 50-year-old woman with TAFRO syndrome diagnosed 2 years after the initial symptoms of a fever, fatigue, epigastric pain, edema, ascites, lymphadenopathy, thrombocytopenia and renal insufficiency. The patient showed refractory ascites and required hemodialysis under corticosteroid mono-therapy for suspected immune-mediated disease but was successfully treated with additive rituximab, resulting in improvement in her laboratory data, the withdrawal of hemodialysis and the disappearance of ascites. This case underscores the therapeutic utility of rituximab in patients with corticosteroid-resistant TAFRO syndrome, even long after the onset of the disease.
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http://dx.doi.org/10.2169/internalmedicine.0116-17 | DOI Listing |
Ann Thorac Surg Short Rep
September 2024
Department of Thoracic & Cardiovascular Surgery, Louis Stokes Cleveland Veterans Affairs Medical Center, Cleveland, Ohio.
We present the case of a 41-year-old man with an anterior mediastinal mass and constellation of clinical symptoms, including dyspnea, pleural effusions, pericardial effusions, renal insufficiency, and pancytopenia. After inconclusive results on several laboratory tests and a nondiagnostic surgical biopsy specimen, a specimen from a second surgical biopsy identified the patient's condition as Castleman disease associated with TAFRO (thrombocytopenia, anasarca, fevers, reticulin myelofibrosis, organomegaly) syndrome. This case highlights the importance of obtaining large tissue biopsy samples, interval follow-up, and acknowledging cognitive biases.
View Article and Find Full Text PDFJ Clin Exp Hematop
December 2024
Department of Radiology, Faculty of Medicine, Dentistry and Pharmaceutical Sciences, Okayama University, Okayama, Japan.
This study retrospectively evaluated the computed tomography (CT) findings of idiopathic multicentric Castleman disease (iMCD) at a single center and compared the CT findings of iMCD-TAFRO with those of iMCD-non-TAFRO. CT images obtained within 30 days before diagnostic confirmation were reviewed for 20 patients with iMCD (8 men and 12 women, mean age 52.8 ± 12.
View Article and Find Full Text PDFFront Med (Lausanne)
December 2024
Department of Hematology, Xuanwu Hospital, Capital Medical University, Beijing, China.
POEMS syndrome is a complex and rare hematological disease involving multiple physiological systems. According to the currently accepted diagnostic criteria for POEMS syndrome, polyneuropathy is one of the primary mandatory criteria. A patient presented with M protein, Castleman disease (CD), elevated vascular endothelial growth factor (VEGF), extravascular volume overload, and endocrinopathy.
View Article and Find Full Text PDFClin Exp Nephrol
November 2024
Nephrology Center and Okinaka Memorial Institute for Medical Research, Toranomon Hospital, 2-2-2, Toranomon, Minato, Tokyo, Japan.
Renal involvement in TAFRO syndrome is characterized clinically by general edema with ascites and pleural effusions and a rapidly progressive decline in renal function, with urinary protein levels of usually less than 1 g/day. The histologic features of the kidneys can be described as glomerular microangiopathy characterized by mesangiolysis or mesangial loosening, endothelial cell proliferation, edematous opening in the subendothelial space, and glomerular basement membrane (GBM) doubling due to newly formed basement membrane. Findings such as rupture of the GBM, foot-process effacement or fusion, and epithelial cell loss are rare, and thrombus formation is difficult to identify in the glomerulus.
View Article and Find Full Text PDFJ Clin Exp Hematop
December 2024
Department of Molecular Hematopathology, Okayama University Graduate School of Health Sciences, Okayama, Japan.
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