Castleman-Kojima disease, also known as idiopathic multicentric Castleman 
disease with TAFRO syndrome (iMCD-TAFRO), is a recently recognized systemic 
inflammatory disorder with a characteristic series of clinical symptoms, 
including thrombocytopenia (T), anasarca (A), fever (F), reticulin fibrosis (R), 
and organomegaly (O). Patients with iMCD-TAFRO often develop severe abdominal 
pain, elevated alkaline phosphatase levels, and systemic inflammation, but the 
etiological factors are unknown. To investigate the potential role of bacterial 
infection in the pathogenesis of iMCD-TAFRO, we performed polymerase chain 
reaction (PCR) for the bacterial 16S rRNA gene with DNA extracted from liver 
specimens of three patients with iMCD-TAFRO, four patients with amyotrophic 
lateral sclerosis, and seven patients with inflammatory conditions. Sequencing 
of the PCR product showed 99% DNA sequence identity with Campylobacter jejuni in 
all three patients with iMCD-TAFRO and in two patients with inflammatory 
conditions. Immunohistochemical and electron microscopy analyses could not 
identify C. jejuni in patients with iMCD-TAFRO. The findings indicated that C. 
jejuni infection is not the pathological cause of iMCD-TAFRO; however, this 
ubiquitous bacterium may play a role in uncontrolled systemic hypercytokinemia, 
possibly through the development of cross-reactive autoantibodies.