Learn More About X-Linked Lymphoproliferative Disease

Overview

Learn More About X-Linked Lymphoproliferative Disease

One of the first steps after an X-linked lymphoproliferative (XLP) disease diagnosis is understanding the condition. Diplomat is here to help.

But remember: The information here — and on linked sites — is not meant to replace advice, care, or a diagnosis from your physician. If you have questions about your condition, please ask your provider.

Overview

X-linked lymphoproliferative disease is a genetic condition characterized by a defective immune system. It occurs in about one in a million males worldwide. Although XLP is genetic, it might not be diagnosed until well into childhood. Symptoms typically occur in response to an episode of Epstein-Barr virus (EBV), a common disease in the herpesvirus family that will affect most people in their lifetime.

As a patient responds to EBV, their body will create an overabundance of immune cells. This potentially fatal reaction can trigger hemophagocytic lymphohistiocytosis (HLH), a life-threatening condition. The HLH reaction puts the patient at risk for hepatitis and lymphoma after exposure to EBV.

XLP causes no symptoms until a patient is infected with EBV, though blood tests might show it. Treatment with immunoglobulin G (IgG), with or without antibodies to EBV, can help prevent or treat the infection.

Sources

National Institute of Allergy and Infectious Diseases

National Organization for Rare Disorders

The information contained herein may not be construed as medical advice. It is for educational purposes only. Diplomat Pharmacy Inc. takes no responsibility for the accuracy or validity of the information contained herein, nor the claims or statements of any manufacturer.