TAFRO syndrome

TAFRO syndrome is a rare and serious inflammatory disorder. Its name comes from the five main features of the disease: Thrombocytopenia (low platelet count), Anasarca (severe fluid buildup throughout the body), Fever, Reticulin fibrosis (scarring in the bone marrow), and Organomegaly (enlarged organs, especially the spleen and liver). TAFRO syndrome is considered a subtype or variant of idiopathic multicentric Castleman disease (iMCD), a group of conditions involving overgrowth of cells in the lymph nodes and widespread inflammation. The disease causes the immune system to become overactive, leading to a dangerous inflammatory response. Patients often experience sudden onset of high fevers, rapid fluid retention causing swelling in the abdomen and legs, very low platelet counts that can lead to bleeding, and kidney problems. The bone marrow shows a specific type of scarring called reticulin fibrosis. Blood tests typically show elevated inflammatory markers and low platelet counts, which helps distinguish TAFRO from other forms of Castleman disease where platelets are usually high. Treatment focuses on calming the overactive immune system and managing symptoms. Doctors commonly use corticosteroids (like dexamethasone or methylprednisolone) as a first step, often combined with other immunosuppressive drugs. Tocilizumab, a drug that blocks the inflammatory protein interleukin-6, and rituximab, which targets certain immune cells, have shown benefit in many patients. Some patients require intensive care during severe flares. The disease can follow a relapsing course, meaning it may come back after treatment, and some cases can be life-threatening if not treated promptly.

Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.

Bring these to your next appointment

• Q1.How severe is my case of TAFRO syndrome, and which organs are currently affected?,What treatment plan do you recommend, and what are the potential side effects?,How will we monitor for disease relapses, and what warning signs should I watch for at home?,Are my kidneys affected, and if so, what can be done to protect them?,Should I see additional specialists, such as a nephrologist or rheumatologist?,Are there any clinical trials or newer treatments available for TAFRO syndrome?,What precautions should I take to prevent infections while on immunosuppressive therapy?

Common questions about TAFRO syndrome

Related conditions

Other rare diseases commonly researched alongside TAFRO syndrome.

• POEMS syndrome→
• Unicentric Castleman disease→