Hemophagocytic lymphohistiocytosis in a patient with x-linked lymphoproliferative disease.

J. Andrew Bird; Kenneth L. McClain; Howard M. Rosenblatt; Stuart L. Abramson; Imelda C. Hanson

doi:10.2500/aap.2009.30.3259

Hemophagocytic lymphohistiocytosis in a patient with x-linked lymphoproliferative disease.

J. Andrew Bird, Kenneth L. McClain, Howard M. Rosenblatt, Stuart L. Abramson, Imelda C. Hanson

Research output: Contribution to journal › Article › peer-review

5 Scopus citations

Abstract

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.

Original language	English (US)
Pages (from-to)	458-462
Number of pages	5
Journal	Allergy and asthma proceedings : the official journal of regional and state allergy societies
Volume	30
Issue number	4
DOIs	https://doi.org/10.2500/aap.2009.30.3259
State	Published - Jan 1 2009

ASJC Scopus subject areas

Immunology and Allergy
Pulmonary and Respiratory Medicine

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abstract = "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.",

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AU - Hanson, Imelda C.

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Hemophagocytic lymphohistiocytosis in a patient with x-linked lymphoproliferative disease.

Abstract

ASJC Scopus subject areas

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