SHM Converge Scientific Abstract ePoster Gallery-Tafro Syndrome, a Difficult Diagnosis and Its Treatment

Pdf Summary

This case report describes a 59-year-old man with a history of Castleman disease, anemia, hypothyroidism, hypertension, chronic kidney disease, and neurogenic bladder who presented with thrombocytopenia and acute kidney injury. In the emergency department, he was febrile, hypotensive, and later became hypoxic after fluids, prompting ICU admission for possible dialysis and plasma exchange. Labs showed severe thrombocytopenia, renal failure, hyperkalemia, metabolic acidosis, and schistocytes on peripheral smear, raising concern for microangiopathic hemolysis. ADAMTS13 was only mildly reduced, IL-6 was elevated, and complement levels were not diagnostic for classic TTP.<br /><br />A bone marrow biopsy showed grade 2 myelofibrosis with negative JAK2, CALR, and MPL testing, supporting a cytokine-driven process consistent with Castleman-associated disease or TAFRO syndrome. He received meningococcal vaccination and eculizumab, but had minimal improvement in renal function and platelets. He was then treated with siltuximab with only marginal benefit, and ultimately switched to tocilizumab, an IL-6 inhibitor, after which his kidney function recovered and platelet count improved to 70,000/uL. He was discharged to acute rehab with hematology follow-up.<br /><br />The discussion explains that TAFRO syndrome is a rare subtype of idiopathic multicentric Castleman disease characterized by thrombocytopenia, anasarca, fever, reticulin fibrosis, and organomegaly. It is difficult to distinguish from iMCD and often leads to delayed diagnosis. Treatment is not well established, but may include corticosteroids, anti-IL-6 therapy, anti-CD-20 therapy, and chemotherapy. Prompt recognition and treatment are important because prognosis is significantly better when therapy is started early.

Asset Subtitle

Alexander Shaffer

Meta Tag

Author List Alexander Shaffer, John K. Smith, Nicholas Condiles

Category Clinical Vignettes

Concept TAFRO Syndrome

Concept Thrombocytopenia

Concept Anasarca

Concept Fever

Concept Reticulin fibrosis

Distinguished Non-Finalist

Presenter Organization Northwell/ NSLIJ

Presenting Author Alexander Shaffer

Track Adult

Keywords

Castleman disease

TAFRO syndrome

thrombocytopenia

acute kidney injury

myelofibrosis

IL-6 inhibitor

tocilizumab

eculizumab

microangiopathic hemolysis

idiopathic multicentric Castleman disease

TAFRO Syndrome

Thrombocytopenia

Anasarca

Fever

Reticulin fibrosis