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Home > West Medicine > Pediatrics > Pediatric Internal Med
百科

X-linked lymphoproliferative disease

小儿内科

Common symptoms

疱疹、肉芽肿、肝功能衰竭、钙化、球蛋白异常

The pathology of disease

X-linked lymphoproliferative disease (Duncan disease) is SH2D1A defect; boys; fatal EBV mononucleosis; dysgammaglobulinemia; lymphoma; bone marrow transplant.

Symptoms

Fatal EBV mononucleosis; boys; dysgammaglobulinemia; lymphoma; hemophagocytic lymphohistiocytosis; family history; X-linked

Causes

SH2D1A mutation; X-linked; impaired EBV immunity.

Treatment

EBV-specific therapy; bone marrow transplant before EBV; IVIG; immunology; genetics

Dept

Immunology / pediatrics / hematology / oncology.

Warm reminder: Health education reference only; seek care at regular hospital.