Anasarca, Fever, Thrombocytopenia, Organomegaly, and Multiorgan Failure in a 24-Year-Old Pregnant Woman

Abstract

TAFRO syndrome is a distinct idiopathic multicentric Castleman disease characterized by the association of thrombocytopenia, anasarca, fever, reticulin fibrosis, and organomegaly. We report the first case occurring in a Caucasian pregnant woman. At 34 weeks of gestation, our patient presented with all clinical and biological symptoms compatible with a TAFRO syndrome. Tough quick cesarean section was performed as symptoms got worse with onset of multiorgan failure requiring mechanical ventilation for acute respiratory distress, continuous renal replacement, and vasopressors. Nine days after ICU admission, steroid boluses were started and allowed spectacular clinical and biological improvement. As systemic inflammatory manifestations are important, TAFRO syndrome can be mistaken with severe autoimmune diseases, systemic infections, hematological malignancies, or hemophagocytic lymphohistiocytosis.

Severe refractory TAFRO syndrome requiring continuous renal replacement therapy complicated with Trichosporon asahii infection in the lungs and myocardial infarction: an autopsy case report and literature review

“…Some severe cases might require temporary hemodialysis (HD). We reviewed previously reported cases of TAFRO syndrome requiring HD during the clinical course in the English literature and identified 19 cases (16 Japanese cases [17,19,20,28,33,[43][44][45][46][47][48][49][50][51]] and 3 non-Japanese cases [42,52,53]). Japanese patients were older than non-Japanese patients (mean age, 57.6 vs. 35.7 years).…”

“…Steroid was used in 18 cases (94.7%), and mPSL pulse therapy was used in 17 cases (89.5%). Only 5 cases (26.3%) were successfully treated with steroid therapy alone [28,43,49,52]. It should be noted that almost all cases (84.2%) required HD within three weeks of admission.…”

International definition of iMCD-TAFRO: future perspectives

“…However, since the late 2010s, there has been an increasing number of case reports of TAFRO syndrome worldwide. [31][32][33][34][35][36][37][38][39][40][41] Idiopathic multicentric Castleman disease (iMCD) is one of the primary causes of TAFRO syndrome. MCD is a rare disorder with systemic inflammation, diffuse lymphadenopathy with characteristic lymph node histopathology, and multi-organ dysfunction.…”