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

Abstract

Background: TAFRO (thrombocytopenia, anasarca, fever, reticulin myelofibrosis/renal failure, and organomegaly) syndrome is a systemic inflammatory disorder and unique clinicopathological variant of idiopathic multicentric Castleman disease that was proposed in Japan. Prompt diagnosis is critical because TAFRO syndrome is a progressive and life threating disease. Some cases are refractory to immunosuppressive treatments. Renal impairment is frequently observed in patients with TAFRO syndrome, and some severe cases require hemodialysis. Histological evaluation is important to understand the pathophysiology of TAFRO syndrome. However, systemic histopathological evaluation through autopsy in TAFRO syndrome has been rarely reported previously. Case presentation: A 46-year-old Japanese man with chief complaints of fever and abdominal distension was diagnosed with TAFRO syndrome through imaging studies, laboratory findings, and pathological findings on cervical lymph node and bone marrow biopsies. Interleukin (IL)-6 and vascular endothelial growth factor (VEGF) levels were remarkably elevated in both blood and ascites. Methylprednisolone (mPSL) pulse therapy was initiated on day 10, followed by combination therapy with PSL and cyclosporine A. However, the amount of ascites did not respond to the treatment. The patient became anuric, and continuous renal replacement therapy was initiated from day 50. However, the patient suddenly experienced cardiac arrest associated with myocardial infarction (MI) on the same day. Although the emergent percutaneous coronary intervention was successfully performed, the patient died on day 52, despite intensive care. Autopsy was performed to ascertain the cause of MI and to identify the histopathological characteristics of TAFRO syndrome.(Continued on next page)

Hearts of stone: Rapidly progressive left ventricular myocardial calcifications in severe sepsis: Case series

“…Proposed mechanisms include the release of cardio-suppressing mediators and alteration of calcium-phosphate metabolism in inflammation and acute kidney injury. 2,3 In severe sepsis, transmural ischaemia from increased oxygen demand can lead to the hydrolysis of calcium-containing vesicles and calcium influx to myocardial fibres, causing RPLVMC. 2,4,5 This likely explains the electrocardiogram changes and troponin rise in our patients in the absence of obstructive coronary artery disease.…”

TAFRO syndrome with a fatal clinical course following BNT162b2 mRNA (Pfizer-BioNTech) COVID-19 vaccination: A case report

“…The mechanism of SCLS is thought to be the extravasation of fluid and protein from the intravascular to the interstitial space [ 10 ]. Some studies have reported that hypercytokinemia and increased intra-abdominal pressure due to massive ascites caused SCLS in patients with TAFRO syndrome [ 11 ]. Third, PAIgG was positive in this case.…”

TAFRO Syndrome That Responded to Prednisolone-only Treatment: Evaluating Changes in IL-6

“…In this report, the patient had a history of alcohol consumption, was diagnosed with intractable ascites associated with liver cirrhosis, and underwent repeated abdominal paracentesis and CART. In a previous study, CART and hemodialysis were reported to be partially effective for TAFRO syndrome, and more than 80% (16/19) of patients with TAFRO syndrome required hemodialysis within 3 weeks of admission ( 10 ). Even if TAFRO syndrome has not been diagnosed, CART and hemodialysis may improve the symptoms.…”