X-linked Lymphoproliferative Syndrome Questions & Answers

Updated: Jan 27, 2019
  • Author: Karen Seiter, MD; Chief Editor: Emmanuel C Besa, MD  more...
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Questions & Answers

Overview

What is X-linked lymphoproliferative (XLP) syndrome?

What is the pathophysiology of X-linked lymphoproliferative (XLP) syndrome?

What causes X-linked lymphoproliferative (XLP) syndrome?

What is the prevalence X-linked lymphoproliferative (XLP) syndrome?

Which patient groups have the highest prevalence of X-linked lymphoproliferative (XLP) syndrome?

What is the prognosis of X-linked lymphoproliferative (XLP) syndrome?

Presentation

Which clinical history findings are characteristic of X-linked lymphoproliferative syndrome type 1 (XLP1)?

Which clinical history findings are characteristic of X-linked lymphoproliferative syndrome type 2 (XLP2)?

Which physical findings are characteristic of X-linked lymphoproliferative (XLP) syndrome?

How is lymphoma characterized in X-linked lymphoproliferative (XLP) syndrome?

DDX

What are the differential diagnoses for X-linked Lymphoproliferative Syndrome?

Workup

What is the role of lab testing in the workup of X-linked lymphoproliferative (XLP) syndrome?

Which histologic findings are characteristics of X-linked lymphoproliferative (XLP) syndrome?

Treatment

What is the role of gene therapy in the treatment of X-linked lymphoproliferative (XLP) syndrome?

How is X-linked lymphoproliferative (XLP) syndrome treated?

How is Epstein-Barr infection treated in X-linked lymphoproliferative (XLP) syndrome?

How is hemophagocytic lymphohistiocytosis (HLH) treated in X-linked lymphoproliferative (XLP) syndrome?

How is lymphoma treated in X-linked lymphoproliferative (XLP) syndrome?

What is the role of hematopoietic stem cell transplantation (HSCT) in the treatment of X-linked lymphoproliferative (XLP) syndrome?

What is included in the long-term monitoring of X-linked lymphoproliferative (XLP) syndrome?

Medications

What is the role of medications in the treatment of X-linked lymphoproliferative (XLP) syndrome?

Which medications in the drug class Blood Products are used in the treatment of X-linked Lymphoproliferative Syndrome?

Which medications in the drug class Monoclonal Antibody are used in the treatment of X-linked Lymphoproliferative Syndrome?

Which medications in the drug class Antineoplastic Agents are used in the treatment of X-linked Lymphoproliferative Syndrome?