Hypogammaglobulinemia Questions & Answers

Updated: Dec 26, 2018
  • Author: Elizabeth A Secord, MD; Chief Editor: Michael A Kaliner, MD  more...
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Questions & Answers

Overview

How is hypogammaglobulinemia characterized?

What are the signs and symptoms of hypogammaglobulinemia?

Which physical findings are characteristic of primary hypogammaglobulinemia?

What lab tests are performed in the workup of hypogammaglobulinemia?

Which imaging studies are performed in the workup of hypogammaglobulinemia?

Which lab tests may be considered in the workup of hypogammaglobulinemia?

When is biopsy indicated in the workup of hypogammaglobulinemia?

When is watchful waiting indicated in the treatment of hypogammaglobulinemia?

When is replacement IgG indicated in the treatment of hypogammaglobulinemia?

When is IgG replacement indicated in the treatment of secondary hypogammaglobulinemia?

What is hypogammaglobulinemia?

What is the pathophysiology of hypogammaglobulinemia?

What is the prevalence of hypogammaglobulinemia?

What is the mortality and morbidity associated with hypogammaglobulinemia?

Which patient groups have the highest prevalence of hypogammaglobulinemia?

Presentation

Which clinical history findings are characteristic of hypogammaglobulinemia?

Which family history findings are characteristic of hypogammaglobulinemia?

What is the typical age of onset for hypogammaglobulinemia?

Which microorganisms are associated with hypogammaglobulinemia?

What is the role of blood product reactions in the etiology of hypogammaglobulinemia?

Which recurrent infection history findings are characteristic of hypogammaglobulinemia?

What are the noninfectious GI signs and symptoms of hypogammaglobulinemia?

What are the musculoskeletal signs and symptoms of hypogammaglobulinemia?

Which autoimmune and collagen vascular diseases are associated with hypogammaglobulinemia?

Which growth findings are characteristic of hypogammaglobulinemia?

Which lymphoid tissue and organ findings are characteristic of hypogammaglobulinemia?

Which developmental abnormalities are characteristic of hypogammaglobulinemia?

Which skin and mucous membrane findings are characteristic of hypogammaglobulinemia?

Which ENT findings are characteristic of hypogammaglobulinemia?

Which pulmonary findings are characteristic of hypogammaglobulinemia?

Which cardiovascular findings are characteristic of hypogammaglobulinemia?

Which neurologic findings are characteristic of hypogammaglobulinemia?

What causes hypogammaglobulinemia?

Which genetic B-cell disorders cause hypogammaglobulinemia?

What are isolated non-IgG immunoglobulin deficiencies associated with hypogammaglobulinemia?

What is IgG subclass deficiency associated with hypogammaglobulinemia?

What is specific antibody deficiency (SAD) or specific polysaccharide antibody deficiency (SPAD) associated with hypogammaglobulinemia?

What is common variable immunodeficiency (CVID) associated with hypogammaglobulinemia?

What causes transient hypogammaglobulinemia of infancy?

What is immunodeficiency with thymoma (Good syndrome) associated with hypogammaglobulinemia?

What is SCID associated with hypogammaglobulinemia?

What is Wiskott-Aldrich syndrome associated with hypogammaglobulinemia?

What is ataxia-telangiectasia (A-T) associated with hypogammaglobulinemia?

What is nephrotic syndrome associated with hypogammaglobulinemia?

What is protein-losing enteropathy associated with hypogammaglobulinemia?

Which catabolic disorders cause hypogammaglobulinemia?

What is the role of immunosuppressive therapy in the etiology of hypogammaglobulinemia?

Which lymphoproliferative malignancies cause hypogammaglobulinemia?

What is the role of prematurity in the etiology of hypogammaglobulinemia?

What are the drug-related causes of hypogammaglobulinemia?

DDX

What are the differential diagnoses for Hypogammaglobulinemia?

Workup

What is the role of serum immunoglobulin measurement in the workup of hypogammaglobulinemia?

What is the role of antibody response after immunization in the workup of hypogammaglobulinemia?

What is the role of isohemagglutinins testing in the workup of hypogammaglobulinemia?

What is the role of peripheral blood lymphocyte immunophenotyping in the workup of hypogammaglobulinemia?

What is the role of delayed-type hypersensitivity testing the workup of hypogammaglobulinemia?

What is the role of a CBC panel in the workup of hypogammaglobulinemia?

What is the role of renal studies in the workup of hypogammaglobulinemia?

What is the role of GI studies in the workup of hypogammaglobulinemia?

What is the role of chest radiography in the workup of hypogammaglobulinemia?

What is the role of HRCT in the workup of hypogammaglobulinemia?

How is ADA deficiency diagnosed in hypogammaglobulinemia?

How is Wiskott-Aldrich syndrome diagnosed in hypogammaglobulinemia?

What is the role of prenatal testing in the workup of hypogammaglobulinemia?

Which histologic findings are characteristic of the workup of hypogammaglobulinemia?

Treatment

How is hypogammaglobulinemia treated?

How is agammaglobulinemia due to absence of B cells treated?

How is hypogammaglobulinemia in primary immunodeficiencies treated?

How is hypogammaglobulinemia with impaired specific antibody production treated?

How is hypogammaglobulinemia with selective antibody deficiency treated?

How is hypogammaglobulinemia with normal-quality antibody response treated?

How is secondary hypogammaglobulinemia treated?

What is the role of IgG replacement therapy in the treatment of primary hypogammaglobulinemia syndromes?

What is the focus of treatment for secondary hypogammaglobulinemia?

What is the role of live vaccines in the treatment of hypogammaglobulinemia?

What is the role of high dose IVIG in the treatment of hypogammaglobulinemia?

How is ADA deficiency treated in hypogammaglobulinemia?

What is the role of TNF in the treatment of hypogammaglobulinemia?

What is the role of gene therapy in the treatment of hypogammaglobulinemia?

What is the role of PCV13 and PPSV23 vaccines in the treatment of hypogammaglobulinemia?

Which activity modifications are used in the treatment of hypogammaglobulinemia?

Medications

What is the role of medications in the treatment of hypogammaglobulinemia?

Which medications in the drug class Blood Product Derivatives are used in the treatment of Hypogammaglobulinemia?

Which medications in the drug class Vaccines are used in the treatment of Hypogammaglobulinemia?

Follow-up

What is included in long-term monitoring of hypogammaglobulinemia?

Which medications are used in the treatment of hypogammaglobulinemia?

Which specialist consultations are beneficial to patients with hypogammaglobulinemia?

What is the role of antibiotics in the management of hypogammaglobulinemia?

What are the possible complications of hypogammaglobulinemia?

What is the prognosis of hypogammaglobulinemia?

What is included in the patient education about hypogammaglobulinemia?