Introduction
Idiopathic multicentric Castleman disease (iMCD) cases with thrombocytopenia (T), anasarca (A), Fever (F), reticulin fibrosis (R), and organomegaly (O) are considered a subtype of iMCS called TAFRO syndrome. It is a rare syndrome characterized by an acute clinical course and a poor prognosis. The optimal management of TAFRO syndrome is unclear. Treatment is predicated on case reports, which have used an assortment of agents including corticosteroids, cyclosporin, rituximab, and/or anti-IL-6 therapies such as tocilizumab or siltuximab.
Case Report
A 73-year-old Japanese man recently diagnosed with iMCD but not currently on treatment presented with a 3-week history of worsening lower extremity edema and abdominal distension. On exam, he had anasarca and was also found to be anuric. Diagnostic testing was notable for hemoglobin 10.9 g/dL, platelets 42 K/uL, potassium 5.7 mmol/L, creatinine 3.7, albumin 2.4 g/dL, troponin 0.1, BNP 805, and Echocardiogram demonstrating an ejection fraction of 40% to 50%. Imaging revealed pleural effusion, splenomegaly and large volume ascites while paracentesis confirmed transudative ascitic fluid. He failed a trial of IV diuresis and was started on dialysis for refractory hyperkalemia. Upon review, his prior bone marrow had mild reticulin fibrosis, and additional lab testing showed an elevated hs-CRP 101 mg/dL and IL-6 of 4.8 pg/mL. His presentation fit multiple criteria for TAFRO including thrombocytopenia, anasarca, fibrosis in bone marrow, renal failure and organomegaly. He was started on weekly rituximab for 4 doses, as well as daily dexamethasone 40 mg for 4 days followed by a planned 6-week prednisone taper. Within a week his urine output began to improve, creatinine improved to 2 and he no longer needed dialysis.
Despite improvement in his renal function, he had progressive anasarca, fatigue and appetite loss over the next three weeks. He was given one dose of siltuximab as salvage therapy, but due to worsening quality of life, he transitioned to comfort care shortly thereafter with eventual demise.
Discussion
Determination of iMCD-TAFRO syndrome requires high clinical suspicion from clinicians to enable early treatment. This case report illustrates the need for early recognition and aggressive treatment to improve outcomes in patients with this deadly disease.
