TAFRO Syndrome in Caucasians: A Case Report and Review of the Literature

Abstract

BackgroundTAFRO syndrome has been reported in Japan among human herpesvirus 8 (HHV-8)-negative/idiopathic multicentric Castleman’s disease (iMCD) patients. To date, the majority of iMCD patients with TAFRO syndrome originate from Japan.Case presentationHerein, we report a 67-year-old HIV/HHV-8-negative Caucasian iMCD patient diagnosed with TAFRO. He presented with marked systemic inflammation, bicytopenia, terminal renal insufficiency, diffuse lymphadenopathies, and anasarca. Lymph node and bone marrow biopsies revealed atrophic germinal centers variably hyalinized and megakaryocytic hyperplasia with mild myelofibrosis. Several other biopsies performed in kidneys, liver, gastrointestinal tract, prostate, and lungs revealed unspecific chronic inflammation. The patient had a complete response to corticosteroids, tocilizumab, and rituximab. He relapsed twice following discontinuation of rituximab. When reviewing the literature, we found seven other Caucasian cases with TAFRO syndrome. There were no significant differences with those described by the Japanese cohort except for the higher frequency of kidney failure and auto-antibodies in Western patients.ConclusionThis case illustrates that patients with TAFRO syndrome can develop non-specific inflammation in several tissue sites. Furthermore, this case and our review of the literature demonstrate that TAFRO syndrome can affect Caucasian and Japanese patients highlighting the importance of evaluating for this syndrome independently of ethnic background.

TAFRO syndrome with skin manifestations treated with bortezomib and tocilizumab; a case report

“…Bortezomib decreases IL6 levels, inhibits the nuclear factor kappa B (NF-κB) and induces the remission in idiopathic MCD (7). Methylprednisolone and tocilizumab combination can also rapidly stop IL6 mediated inflammation (8). Our case, similar to the previously reported cases, responded well to tocilizumab (9)(10)(11).…”

Comparison of the clinical characteristics of TAFRO syndrome and idiopathic multicentric Castleman disease in general internal medicine: a 6‐year retrospective study

“…8 Previously, MCD was considered to occur mainly in association with a human herpes virus 8 (HHV-8) infection in immunocompromised patients. [10][11][12][13] Although the clinical characteristics are similar in these two disorders, TAFRO syndrome has been described as a clinical entity distinct from iMCD because TAFRO syndrome is usually associated with hypogammaglobulinaemia, which is in stark contrast to iMCD, and with poor treatment response. 7 iMCD was thus further defined by HIV-and HHV-8-negative MCD subtypes.…”

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

“…However, the association between SjS syndrome or anti-SS-A/SS-B antibodies with TAFRO syndrome remains unclear. Non-Japanese cases of TAFRO syndrome with positive autoantibodies, including anti-thyroid peroxidase antibody and anti-cardiolipin antibody, have been reported [12]. We could not identify a case of TAFRO syndrome involving significantly elevated IgG4 levels (≥135 mg/dL), displaying an association with IgG4-related disease (IgG4-RD).…”

“…TAFRO syndrome was initially reported mainly from Japan, but recently, cases of TAFRO syndrome are being reported worldwide [12,13]. As severe cases of iMCD-NOS might show TAFRO syndrome-like clinical manifestations, some cases of TAFRO syndrome might be diagnosed as severe iMCD-NOS [11].…”