Allergy and asthma proceedings · 2009
Hemophagocytic lymphohistiocytosis in a patient with x-linked lymphoproliferative disease.
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Bird J Andrew, McClain Kenneth L, Rosenblatt Howard M, Abramson Stuart L, Hanson Imelda C
Аннотация
X-linked lymphoproliferative disease (XLP) is a primary immunodeficiency affecting approximately 1 to 3 per million live male births. Patients are generally healthy until facing a viral infection such as Epstein-Barr Virus and then may develop fulminant infectious mononucleosis and die. XLP patients are also at increased risk of hemophagocytic lymphohistiocytosis (HLH), which may be triggered by assorted viruses. Here we report a novel case of HLH in a patient with XLP. Significant to his presentation is a paradoxical increase in natural killer (NK) cell activity. We hypothesize that this indicates that Parvovirus B19 activates NK cells via a signaling lymphocytic activation molecule-associated protein (SAP)-independent mechanism. Our case demonstrates an important etiology to consider in the differential diagnosis of XLP patients with nonfocal findings and febrile illnesses.
Предметные рубрики MeSH
Bacterial InfectionsChild, PreschoolDNA Mutational AnalysisFeverHumansIntracellular Signaling Peptides and ProteinsKiller Cells, NaturalLymphohistiocytosis, HemophagocyticLymphoproliferative DisordersMaleMultiple Organ FailureParvoviridae InfectionsParvovirus B19, HumanPedigreeSepsisSignaling Lymphocytic Activation Molecule Associated Protein