Last Resort

^1,4,7-9 TAFRO syndrome occurs primarily in the fourth and fifth decades of life, with case reports ranging from 14 to 78 years of age.^1,3,10,11 Gender distribution varies but is likely equal for males and females.³ Mortality in TAFRO syndrome is estimated at 11%-12%.^1,3 Over the past several years, a North American and European patient registry and natural history study for CD, ACCELERATE, has been initiated.⁴ In addition, the international Castleman Disease Collaborative Network, a Japanese multicenter retrospective study for MCD, and a nationwide Japanese research team for CD have been created.^3,4 Previously, CD did not have an International Classification of Diseases (ICD) code and was likely under-recognized. An ICD-10 for CD was added, making CD and its variants easier to research for prevalence, characterization, mortality, and treatment.

After prolonged hospitalizations and extensive workup with no diagnosis, the patient’s clinical picture was most consistent with the lymphoproliferative disorder iMCD. However, iMCD is notable for polyclonal hypergammaglobulinemia, thrombocytosis, and mild anasarca. This patient had normal gammaglobulins, significant thrombocyotopenia, and profound, difficult-to-treat anasarca and ascites. Recognizing that the patient’s presentation did not fit neatly into a known clinical syndrome, an internet search was conducted based on his clinical features. This revealed TAFRO syndrome, which was at the time a newly described clinical syndrome with only a few published case reports. It was an internet search undertaken as a last resort that ultimately led to the patient’s diagnosis and successful treatment.

• Key clinical and pathologic features of TAFRO syndrome include thrombocytopenia, anasarca, fever, reticulin fibrosis and/or renal insufficiency, and organomegaly.
• TAFRO syndrome may be under-recognized due to very recent characterization and no previous ICD code for CD.
• TAFRO syndrome experts recommend immunosuppression for treatment of TAFRO syndrome, including glucocorticoids as first-line treatment.
• Internet searches can be helpful in the diagnosis of challenging cases, particularly with rare, unusual, and emerging diseases that have not yet been described in reference texts and only infrequently reported in the medical literature.

Disclosures

Jonathan S. Zipursky, Keri T. Holmes-Maybank, Steven L. Shumak, and Ashley A. Ducketthave none to declare.