Bacteremia and skin/bone infections in two patients with X-linked agammaglobulinemia caused by an unusual organism related to Flexispira/Helicobacterspecies

Two patients with Bruton's X-linked agammaglobulinemia are described with b acteremia and skin/bone infection due to an organism which by 16S rRNA gene sequence analysis was most closely related to "Flexispira" rappini land th us designated a Flexispira-like organism, FLO) and more distantly related t o the Helicobacter species, The organism required microaerobic conditions a nd, supplemental H-2 gas for growth and was reliably stained with acridine orange. In common with Helicobacter cinaedi infections, the focus of the FL O infection was in one case in the blood vessels or lymphatics of an extrem ity and in the other case in the skin and adjacent bone of an extremity. In both cases, prolonged TV antibiotic therapy was necessary to clear the inf ection. The susceptibility of XLA patients to FLO infection appears to be r elated to the fact that XLA is associated with severe B cell (humoral) immu nodeficiency and thus these patients have difficulty with intravascular or intralymphatic infection. These findings elucidate the nature of FLO infect ions in humans and point the way to their detection and treatment, (C) 2000 Academic Press.