“Hairiness” is a Facsimile of Reorganized Cytoskeletons: A Cytopathic Effect of Coxiella burnetii

In 1993, I reported that Coxiella burnetii transforms human B cells into hairy cells (cbHCs), the first hairy cell reported outside of hairy cell leukemia (HCL). Over last few decades, advances in molecular biology have provided evidence supporting that C. burnetii induces hairiness and inhibits the apoptosis of host cells. The present review summarizes new information in support of cbHC. C. burnetii was shown to induce reorganization of the cytoskeleton and to inhibit apoptosis in host cells. Peritoneal B1a cells were found to be permissive for virulent C. burnetii Nine Mile phase I (NMI) strains in mice. C. burnetii severely impaired E-cad expression in circulating cells of Q fever patients. B-cell non-Hodgkin lymphoma was linked to C. burnetii. Mutation of BRAF V600E was pronounced in HCL, but “hairiness” was not linked to the mutation. Risk factors shared among coxiellosis and HCL in humans and animals were reported in patients with Q-fever. Accordingly, I propose that C. burnetii induces reorganization of the cytoskeleton and inhibits apoptosis as cytopathic effects that are not target cell specific. The observed hairiness in cbHC appears to be a fixed image of dynamic nature, and hairy cells in HCL are distinct among lymphoid cells in circulation. As the cytoskeleton plays key roles in maintaining cell structural integrity in health and disease, the pathophysiology of similar cytopathic effects should be addressed in other diseases, such as myopathies, B-cell dyscrasias, and autoimmune syndromes.

Seroprevalence to Coxiella burnetii in Patients with Acute Febrile Episodes during 1993

Coxiella burnetii is the etiological agent of Q fever, that may occur either acutely or the chronically. To understand the seroepidemiological patterns of C. burnetii infection in Korea, we examined a total of 3,178 sera from patients with acute febrile episodes by using indirect immunofluorescence assay (IFA) for detectable antibodies to C. burnetii and other eight rickettsial antigens. The IFA seropositivity>or=1:20 for C. burnetii phase II was 11.5% (368 out of 3,178 sera). The co-existence of antibodies to other rickettsial antigens was found in 216 out of the 368 positive sera. Thirty-seven point five percent (n=138) had antibodies to R. tsutsugamushi (cutoff>or=1:20), 16% (n=59) to Ehrlichia sennetsu, 14.9% (n=55) to Rickettsia typhi, 13.5% (n=50) to R. akari, 11.4% (n=42) to R. japonica, 8.9% (n=33) to R. prowazekii, 7.6% (n=28) to R. sibirica, and 6.7% (n=25) to R. conorii by IFA, respectively. These results are consistent with previous reports documenting diverse serum cross-reactivity in chronic Q fever. Therefore we excluded the samples that reacted to other rickettsial antigens at same or higher titers than to C. burnetii, resulting in the seropositive rate of 4.1%. The serological prevalence was 2% (n=64) when the conventional cut-off titer of 1:80 was used. Our results suggest that infections with C. burnetii are more prevalent than expected previously and should be differentially diagnosised for febrile illness occurring after exposure to ticks or other vectors.