X-linked Immunodeficiency With Hyper IgM Questions & Answers

Updated: Oct 23, 2019
  • Author: C Lucy Park, MD; Chief Editor: Harumi Jyonouchi, MD  more...
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Questions & Answers

Overview

What is X-linked immunodeficiency with hyper–immunoglobulin M (XHIGM)?

What is the pathophysiology of X-linked immunodeficiency with hyper–immunoglobulin M (XHIGM)?

What is the prevalence of X-linked immunodeficiency with hyper–immunoglobulin M (XHIGM) in the US?

What is the global prevalence of X-linked agammaglobulinemia (XLA)?

What is the mortality and morbidity associated with X-linked immunodeficiency with hyper–immunoglobulin M (XHIGM)?

What are the racial predilections of X-linked immunodeficiency with hyper–immunoglobulin M (XHIGM)?

What are the sexual predilections of X-linked immunodeficiency with hyper–immunoglobulin M (XHIGM)?

At what age is X-linked immunodeficiency with hyper–immunoglobulin M (XHIGM) typically diagnosed?

Presentation

Which clinical history findings are characteristic of X-linked immunodeficiency with hyper–immunoglobulin M (XHIGM)?

Which physical findings are characteristic of X-linked immunodeficiency with hyper–immunoglobulin M (XHIGM)?

What causes X-linked immunodeficiency with hyper–immunoglobulin M (XHIGM)?

DDX

How is X-linked immunodeficiency with hyper–immunoglobulin M (XHIGM) differentiated from other types of hyper-IgM syndrome (HIGM)?

What are the differential diagnoses for X-linked Immunodeficiency With Hyper IgM?

Workup

How is X-linked immunodeficiency with hyper–immunoglobulin M (XHIGM) diagnosed?

What is the role of imaging studies in the workup of X-linked immunodeficiency with hyper–immunoglobulin M (XHIGM)?

What is the role of pulmonary function testing (PFT) in the workup of X-linked immunodeficiency with hyper–immunoglobulin M (XHIGM)?

What is the role of biopsy in the workup of X-linked immunodeficiency with hyper–immunoglobulin M (XHIGM)?

What is the role of bronchoscopy in the workup of X-linked immunodeficiency with hyper–immunoglobulin M (XHIGM)?

Which histologic findings are characteristics of X-linked immunodeficiency with hyper–immunoglobulin M (XHIGM)?

Treatment

What is the role of IVIG therapy in the treatment of X-linked immunodeficiency with hyper–immunoglobulin M (XHIGM)?

What are the possible adverse reactions to IVIG therapy for the treatment of X-linked immunodeficiency with hyper–immunoglobulin M (XHIGM)?

What is the role of SCIG therapy in the treatment of X-linked immunodeficiency with hyper–immunoglobulin M (XHIGM)?

How is X-linked immunodeficiency with hyper–immunoglobulin M (XHIGM) treated?

When is P jiroveci prophylaxis indicated in the treatment of X-linked immunodeficiency with hyper–immunoglobulin M (XHIGM)?

How is severe neutropenia treated in X-linked immunodeficiency with hyper–immunoglobulin M (XHIGM)?

What is the role of antimicrobial medications in the treatment of X-linked immunodeficiency with hyper–immunoglobulin M (XHIGM)?

What is the role of immunosuppressants in the treatment of X-linked immunodeficiency with hyper–immunoglobulin M (XHIGM)?

What is the role of bone marrow transplantation (BMT) in the treatment of X-linked immunodeficiency with hyper–immunoglobulin M (XHIGM)?

What is the role of recombinant CD40L in the treatment of X-linked immunodeficiency with hyper–immunoglobulin M (XHIGM)?

What is the role of surgery in the treatment of X-linked immunodeficiency with hyper–immunoglobulin M (XHIGM)?

Which specialist consultations are beneficial to patients with X-linked immunodeficiency with hyper–immunoglobulin M (XHIGM)?

Which dietary modifications are used in the treatment of X-linked immunodeficiency with hyper–immunoglobulin M (XHIGM)?

Which activity modifications are used in the treatment of X-linked immunodeficiency with hyper–immunoglobulin M (XHIGM)?

Medications

What is the role of medications in the treatment of X-linked immunodeficiency with hyper–immunoglobulin M (XHIGM)?

Which medications in the drug class Granulocyte-Colony Stimulating Factors (G-CSF) are used in the treatment of X-linked Immunodeficiency With Hyper IgM?

Which medications in the drug class Antibiotics are used in the treatment of X-linked Immunodeficiency With Hyper IgM?

Which medications in the drug class Antiprotozoal agents are used in the treatment of X-linked Immunodeficiency With Hyper IgM?

Which medications in the drug class Immune Globulins are used in the treatment of X-linked Immunodeficiency With Hyper IgM?

Follow-up

What is included in the long-term monitoring of X-linked immunodeficiency with hyper–immunoglobulin M (XHIGM)?

When is bone marrow transplantation (BMT) indicated for the treatment of X-linked immunodeficiency with hyper–immunoglobulin M (XHIGM)?

When is inpatient care indicated for the treatment of X-linked immunodeficiency with hyper–immunoglobulin M (XHIGM)?

When is patient transfer needed for the treatment of X-linked immunodeficiency with hyper–immunoglobulin M (XHIGM)?

How is X-linked immunodeficiency with hyper–immunoglobulin M (XHIGM) diagnosed prenatally?

How are infections prevented in patients with X-linked immunodeficiency with hyper–immunoglobulin M (XHIGM)?

What are the possible complications of X-linked immunodeficiency with hyper–immunoglobulin M (XHIGM)?

What is the prognosis of X-linked immunodeficiency with hyper–immunoglobulin M (XHIGM)?

Where can patient education resources about X-linked immunodeficiency with hyper–immunoglobulin M (XHIGM) be found?