The perfect storm: hemophagocytic lymphohistiocytosis complicating disseminated yersiniosis.
Hemophagocytic lymphohistio-cytosis (HLH) is a rare but serious medical condition with variable clinical outcome. HLH presents real diagnostic and therapeutic challenges to the treating physician despite the major advances in molecular laboratory testing and the immune-chemotherapeutic interventions. Here we present a unique case ofHLH in the setting of hemoglobin H disease provoked by disseminated Yersinia enterocolitica infection. The hemoglobinopathy led to an iron overload state, which provided an optimal milieu for the siderophilic bacterium, Y. enterocolitica, to disseminate. This over whelming infection triggered an uncontrolled and dysregulated inflammatory cytokine cascade with resulting clinical sequelae. The patient was treated conservatively without immunosuppressive therapy and recovered quickly. The long-term outcome of such cases needs further definition.
Michel
M
Bidros
Lisa M
LM
Chirch
Maria T
MT
Santos
Susan H
SH
Tannenbaum
D000900:Anti-Bacterial Agents / Q000627:therapeutic use; D006801:Humans; D051359:Lymphohistiocytosis, Hemophagocytic / Q000209:etiology* / Q000503:physiopathology*; D008297:Male; D008875:Middle Aged; D015009:Yersinia Infections / Q000150:complications* / Q000188:drug therapy; D015008:Yersinia enterocolitica
D000900:Anti-Bacterial Agents