Pure B-Cell Disorders Treatment & Management

Updated: Dec 27, 2017
  • Author: Issam Makhoul, MD; Chief Editor: Emmanuel C Besa, MD  more...
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Treatment

Medical Care

Intravenous immunoglobulin (IVIG) replacement therapy is the treatment of choice for most primary B-cell disorders with hypogammaglobulinemia, including X-linked agammaglobulinemia (XLA), common variable immunodeficiency (CVID), immunodeficiency with thymoma, and most of the combined immunodeficiencies. Do not administer IVIG to patients with IgG subclass deficiency unless they do not produce antibodies and do not respond to prophylactic antibiotics. [14]

Quartier et al reported on 31 XLA patients treated early in life with IVIG. [15] The higher the trough level of immunoglobulin, the lower the incidence of acute bacterial infections requiring hospitalization, with the best results obtained with levels of more than 800 mg/dL and the worst results with levels of less than 500 mg/dL. However, chronic bronchitis and sinusitis were not prevented by this treatment.

Other aspects of IVIG therapy are as follows:

  • IVIG is not indicated in persons with selective transient hypogammaglobulinemia of infancy (THI)
  • IVIG is not indicated for IgG subclass deficiency unless a broader deficiency of antibody production is present
  • For selective IgA deficiency, IVIG therapy is not indicated; oral administration of immunoglobulin may improve chronic diarrhea; immunoglobulin preparations containing low levels of IgA and washed blood products should be used in cases of concomitant IgG deficiency
  • High doses of IVIG or intrathecal immunoglobulin may be beneficial in enteroviral meningoencephalitis

Do not immunize these patients with live attenuated vaccines. Focus efforts on the treatment of infections, allergic reactions, and autoimmune and GI diseases. Aggressive and prolonged antibiotic therapy covering Streptococcus pneumoniae and Haemophilus influenzae is indicated. Prophylactic antibiotic therapy has been recommended for patients with frequent infections. A course of metronidazole may result in dramatic improvement of the diarrhea and, to a certain extent, of malabsorption syndrome. Prophylactic antibiotic therapy may significantly decrease the incidence of infections.

Interleukin-2 may improve in vitro lymphocyte function in persons with CVID. However, because of limited use in humans, no final conclusions can be made regarding its efficacy.

Parenteral vitamin B-12 is used in persons with pernicious anemia.

Hematopoietic stem cell transplantation (HCT) has proved successful for curing a number of primary immunodeficiencies. For example, the Primary Immune Deficiency Treatment Consortium (PIDTC) reported on 100 children with severe combined immunodeficiency (SCID)—including 68 patients with typical SCID and 32 with leaky SCID, Omenn syndrome, or reticular dysgenesis—who underwent allogeneic HCT from 2010 to 2014. The 2-year overall survival was 90%, but was 95% for those who were infection-free at HCT vs 81% for those with active infection (P = 0.009). [16]

Gene therapy is being used in patients with immune deficiencies who lack a suitable donor.  Disorders in which progress has been made include adenosine deaminase deficiency, X-linked SCID, chronic granulomatous disease, and Wiskott-Aldrich syndrome. [17]

 

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