Pure B-Cell Disorders Treatment & Management

  • Author: Issam Makhoul, MD; Chief Editor: Emmanuel C Besa, MD   more...
 
Updated: Jan 10, 2012
 

Medical Care

  • IVIG replacement therapy is the treatment of choice for most primary B-cell disorders with hypogammaglobulinemia, including XLA, 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.[9]
    • Quartier et al reported on 31 XLA patients treated early in life with IVIG.[10] 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.
    • IVIG is not indicated in persons with selective 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 S pneumoniae and H 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.
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Contributor Information and Disclosures
Author

Issam Makhoul, MD  Associate Professor, Department of Medicine, Division of Hematology/Oncology, University of Arkansas for Medical Sciences

Issam Makhoul, MD is a member of the following medical societies: American Society of Clinical Oncology and American Society of Hematology

Disclosure: Nothing to disclose.

Coauthor(s)

David Claxton, MD  Professor of Medicine, Department of Internal Medicine, Section of Hematology-Oncology, Hershey Medical Center, Pennsylvania State University College of Medicine

Disclosure: Nothing to disclose.

Witold Rybka, MD  Professor of Medicine and Pathology, Penn State Hershey College of Medicine, Director, Bone Marrow Transplant Program, Penn State Hershey Medical Center

Witold Rybka, MD is a member of the following medical societies: American Society of Hematology and Royal College of Physicians and Surgeons of Canada

Disclosure: Nothing to disclose.

Hanan Makhoul, MD  Staff Physician, Department of Internal Medicine, University of Arkansas School of Medicine

Disclosure: Nothing to disclose.

Specialty Editor Board

Charles S Greenberg, MD  Director of Thrombosis and Transglutaminase Research Laboratory, Professor, Departments of Pathology and Medicine, Division of Hematology/Oncology, Duke University Medical Center

Charles S Greenberg, MD is a member of the following medical societies: American Society of Hematology and International Society on Thrombosis and Haemostasis

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Marcel E Conrad, MD  Distinguished Professor of Medicine (Retired), University of South Alabama College of Medicine

Marcel E Conrad, MD is a member of the following medical societies: Alpha Omega Alpha, American Association for the Advancement of Science, American Association of Blood Banks, American Chemical Society, American College of Physicians, American Physiological Society, American Society for Clinical Investigation, American Society of Hematology, Association of American Physicians, Association of Military Surgeons of the US, International Society of Hematology, Society for Experimental Biology and Medicine, and Southwest Oncology Group

Disclosure: No financial interests None None

Rajalaxmi McKenna, MD, FACP  Southwest Medical Consultants, SC, Department of Medicine, Good Samaritan Hospital, Advocate Health Systems

Rajalaxmi McKenna, MD, FACP is a member of the following medical societies: American Society of Clinical Oncology, American Society of Hematology, and International Society on Thrombosis and Haemostasis

Disclosure: Nothing to disclose.

Chief Editor

Emmanuel C Besa, MD  Professor, Department of Medicine, Division of Hematologic Malignancies, Kimmel Cancer Center, Jefferson Medical College of Thomas Jefferson University

Emmanuel C Besa, MD is a member of the following medical societies: American Association for Cancer Education, American College of Clinical Pharmacology, American Federation for Medical Research, American Society of Clinical Oncology, American Society of Hematology, and New York Academy of Sciences

Disclosure: Nothing to disclose.

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