ABSTRACT
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.
Subject(s)
Ascites/drug therapy , Glucocorticoids/therapeutic use , Immunologic Factors/therapeutic use , Rituximab/therapeutic use , Drug Therapy, Combination , Edema/diagnosis , Edema/drug therapy , Female , Fever/diagnosis , Humans , Lymphadenopathy/diagnosis , Lymphadenopathy/drug therapy , Middle Aged , Primary Myelofibrosis/diagnosis , Primary Myelofibrosis/drug therapy , Remission Induction , Renal Dialysis , Renal Insufficiency/diagnosis , Renal Insufficiency/therapy , Syndrome , Thrombocytopenia/diagnosis , Thrombocytopenia/drug therapy , Withholding TreatmentABSTRACT
A 26-year-old man highly suspected of having antiglomerular basement membrane (GBM) disease was treated with corticosteroid pulse therapy 9 days after initial infection-like symptoms with high procalcitonin value. The patient required hemodialysis the next day of the treatment due to oliguria. In addition to corticosteroid therapy, plasmapheresis was introduced and the patient could discontinue hemodialysis 43 days after the treatment. Kidney biopsy after initiation of hemodialysis confirmed anti-GBM disease with 86.3% crescent formation. Physician should keep in mind that active anti-GBM disease shows even high procalcitonin value in the absence of infection. To pursue recovery of renal function, the challenge of the immediate and persistent treatment with high-dose corticosteroids plus plasmapheresis for highly suspected anti-GBM disease is vitally important despite the presence of reported predictors for dialysis-dependence including oliguria and requiring hemodialysis at presentation.
