What are the possible complications of hypogammaglobulinemia?

Updated: Dec 26, 2018
  • Author: Elizabeth A Secord, MD; Chief Editor: Michael A Kaliner, MD  more...
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See the list below:

  • Spruelike syndrome with malabsorption is observed in 10% of patients with common variable immunodeficiency (CVID). Upon small bowel biopsy, this syndrome resembles gluten-sensitive enteropathy, except for the absence of plasma cells. Infectious enteritis can be mistaken for ulcerative colitis or Crohn disease; both seem to occur with increased frequency in patients with CVID. Children with CVID frequently have lymphoid hyperplasia in the intestines, which may be comprised of plasmacytoid cells of B-cell lineage.

  • Vaccine-associated poliomyelitis may occur in patients with X-linked agammaglobulinemia (XLA) who receive the attenuated live poliovirus vaccine (no longer commonly used for infants in the United States).

  • Persistent enteroviral infection and chronic sinusitis remain the major complications of patients with XLA.

  • Viral encephalitis caused by, in decreasing order, enterovirus, coxsackievirus, measles, and papovavirus are potentially rare and devastating complications of hypogammaglobulinemia.

  • Hearing loss due to chronic otitis media or meningoencephalitis may affect as many as one third of patients with XLA and may also affect patients with CVID and specific antibody deficiency syndromes.

  • Bronchiectasis and cor pulmonale may complicate chronic or recurrent lower respiratory infections.

  • Autoimmune diseases [4]

  • The risk of cancer in patients with CVID is 5 times higher than in matched controls. A 47-fold increase in gastric cancer and a 30-fold increase in lymphoma have been reported. The role of chronic infection with Helicobacter and other enteric pathogens in these cancers is suspected. Benign lymphoproliferative disorders are much more common, affecting up to 30% of patients, and manifest as splenomegaly, with or without diffuse lymphadenopathy. They are distinguished from lymphomas by the presence of a mixture of B and T lymphocytes and by the absence of clonal B-cell and T-cell receptor rearrangement.

  • A noncaseating granulomatous disease involving the lungs, lymph nodes, skin, bone marrow, and liver has been described in patients with CVID. [14] This entity should be differentiated from mycobacterial and fungal infections. In the small subset of patients with aggressive disease, corticosteroids and tumor necrosis factor (TNF) inhibitors are the treatments of choice. Granulomatous disease in the lungs is often associated with hilar, retroperitoneal, or abdominal lymphadenopathy.

  • Anaphylactic reactions can occur in rare instances when patients with IgA deficiency receive blood products containing IgA.

  • The risk of graft versus host disease (GVHD) is high in patients with SCID because of their inability to reject foreign antigens. Infants with SCID may present with GVHD before transplantation, due to engraftment with maternal lymphocytes before birth.

  • A dermatomyositis-like syndrome, a rare complication of Bruton disease, is a constellation of edema of subcutaneous tissue, rash, and muscle weakness. Chronic enteroviral meningoencephalitis also can be observed with this disorder.

  • Complications related to immunoglobulin therapy [20]

    • Nonanaphylactic reactions: The most common adverse reactions to IVIG are back and abdominal pain, nausea, vomiting, chills, fever, and myalgias. The infusion should be discontinued until the symptoms subside; then, it should be restarted at a slower rate after administration of premedication (eg, oral or intravenous hydration, antipyretics, antiemetics)

    • Local reactions to SCIG are common but are rarely persistent or serious.

    • Anaphylactic reactions: These are rare. They are IgE-mediated in patients with IgA deficiency and occur from seconds to hours after the infusion is started. IgG anti-IgA antibodies may be responsible for anaphylactoid reactions due to complement activation. Typical symptoms consist of flushing, facial swelling, dyspnea, and hypotension. The infusion should be stopped, and the patient should receive epinephrine, glucocorticoids, and antihistamines. Pure cutaneous reactions such as flushing and urticaria can be treated as nonanaphylactic reactions, with supportive and symptomatic therapy as needed.

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