Pure B-Cell Disorders Medication

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

Medication Summary

The goals of pharmacotherapy are to reduce morbidity and to prevent complications.

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Immunoglobulins

Class Summary

Provide immediate passive immunity. Replacement therapy in antibody deficiency states.

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Immune globulin, intravenous (Gamimune, Gammagard, Sandoglobulin, Gammar-P)

 

Induces elevated antiviral or antibacterial antibody titers for 1 mo. May increase CSF IgG (10%).

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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.

References

  1. O'Gorman MR. Measurement of CD40 ligand (CD154) expression on resting and in vitro-activated T cells. Curr Protoc Cytom. May 2001;Chapter 6:Unit 6.7. [Medline].

  2. Van Hoeyveld E, Zhang PX, De Boeck K, Fuleihan R, Bossuyt X. Hyper-immunoglobulin M syndrome caused by a mutation in the promotor for CD40L. Immunology. Apr 2007;120(4):497-501. [Medline].

  3. Bussone G, Mouthon L. Autoimmune manifestations in primary immune deficiencies. Autoimmun Rev. Feb 2009;8(4):332-6. [Medline].

  4. Jesus AA, Duarte AJ, Oliveira JB. Autoimmunity in hyper-IgM syndrome. J Clin Immunol. May 2008;28 Suppl 1:S62-6. [Medline].

  5. Levy J, Espanol-Boren T, Thomas C, et al. Clinical spectrum of X-linked hyper-IgM syndrome. J Pediatr. Jul 1997;131(1 Pt 1):47-54. [Medline].

  6. Poliani PL, Facchetti F, Ravanini M, Gennery AR, Villa A, Roifman CM, et al. Early defects in human T-cell development severely affect distribution and maturation of thymic stromal cells: possible implications for the pathophysiology of Omenn syndrome. Blood. Jul 2 2009;114(1):105-8. [Medline].

  7. McCusker C, Hotte S, Le Deist F, Hirschfeld AF, Mitchell D, Nguyen VH, et al. Relative CD4 lymphopenia and a skewed memory phenotype are the main immunologic abnormalities in a child with Omenn syndrome due to homozygous RAG1-C2633T hypomorphic mutation. Clin Immunol. Jun 2009;131(3):447-55. [Medline].

  8. Grimbacher B, Holland SM, Gallin JI, et al. Hyper-IgE syndrome with recurrent infections--an autosomal dominant multisystem disorder. N Engl J Med. Mar 4 1999;340(9):692-702. [Medline].

  9. Hoernes M, Seger R, Reichenbach J. Modern management of primary B-cell immunodeficiencies. Pediatr Allergy Immunol. Dec 2011;22(8):758-69. [Medline].

  10. Quartier P, Debre M, De Blic J, et al. Early and prolonged intravenous immunoglobulin replacement therapy in childhood agammaglobulinemia: a retrospective survey of 31 patients. J Pediatr. May 1999;134(5):589-96. [Medline].

  11. Berlucchi M, Soresina A, Redaelli De Zinis LO, Valetti L, Valotti R, Lougaris V, et al. Sensorineural hearing loss in primary antibody deficiency disorders. J Pediatr. Aug 2008;153(2):293-6. [Medline].

  12. Kainulainen L, Varpula M, Liippo K, et al. Pulmonary abnormalities in patients with primary hypogammaglobulinemia. J Allergy Clin Immunol. Nov 1999;104(5):1031-6. [Medline].

  13. Mechanic LJ, Dikman S, Cunningham-Rundles C. Granulomatous disease in common variable immunodeficiency. Ann Intern Med. Oct 15 1997;127(8 Pt 1):613-7. [Medline].

  14. Ballow M, O'Neil KM. Approach to the Patient With Recurrent Infections. In: Middleton E Jr, Reed CM, Ellis EF, Adkinson NF Jr, Yunginger JW, Busse WW, eds. Allergy: Principles & Practice. 5th ed. St. Louis, Mo: Mosby-Year Book; 1998:. 735-59.

  15. Behrman RE, Kliegman RM, Jenson HB, eds. Primary B-Cell diseases. In: Nelson's Textbook of Pediatrics. 16th ed. Philadelphia, Pa: WB Saunders; 2000:. 596-606.

  16. Berger M. Goals of therapy in antibody deficiency syndromes. J Allergy Clin Immunol. Nov 1999;104(5):911-3. [Medline].

  17. Bezrodnik L, Samara R, Krasovec S, et al. Progressive multifocal leukoencephalopathy in a patient with hypogammaglobulinemia. Clin Infect Dis. Jul 1998;27(1):181-4. [Medline].

  18. Bjoro K, Haaland T, Skaug K, Froland SS. The spectrum of hepatobiliary disease in primary hypogammaglobulinaemia. J Intern Med. May 1999;245(5):517-24. [Medline].

  19. Bjoro K, Skaug K, Haaland T, Froland SS. Long-term outcome of chronic hepatitis C virus infection in primary hypogammaglobulinaemia. QJM. Aug 1999;92(8):433-41. [Medline].

  20. Dreskin SC, Goldsmith PK, Gallin JI. Immunoglobulins in the hyperimmunoglobulin E and recurrent infection (Job's) syndrome. Deficiency of anti-Staphylococcus aureus immunoglobulin A. J Clin Invest. Jan 1985;75(1):26-34. [Medline].

  21. Hadzic N, Pagliuca A, Rela M, et al. Correction of the hyper-IgM syndrome after liver and bone marrow transplantation. N Engl J Med. Feb 3 2000;342(5):320-4. [Medline].

  22. Jones AM, Gaspar HB. Immunogenetics: changing the face of immunodeficiency. J Clin Pathol. Jan 2000;53(1):60-5. [Medline].

  23. Kohn DB. Gene therapy for genetic haematological disorders and immunodeficiencies. J Intern Med. Apr 2001;249(4):379-90. [Medline].

  24. LeBien TW. Fates of human B-cell precursors. Blood. Jul 1 2000;96(1):9-23. [Medline].

  25. Sanna PP, Burton DR. Role of antibodies in controlling viral disease: lessons from experiments of nature and gene knockouts. J Virol. Nov 2000;74(21):9813-7. [Medline].

  26. Satterthwaite AB, Witte ON. The role of Bruton's tyrosine kinase in B-cell development and function: a genetic perspective. Immunol Rev. Jun 2000;175:120-7. [Medline].

  27. Scholl PR, O'Gorman MR, Pachman LM, et al. Correction of neutropenia and hypogammaglobulinemia in X-linked hyper- IgM syndrome by allogeneic bone marrow transplantation. Bone Marrow Transplant. Dec 1998;22(12):1215-8. [Medline].

  28. Smith JK, Krishnaswamy GH, Dykes R, et al. Clinical manifestations of IgE hypogammaglobulinemia. Ann Allergy Asthma Immunol. Mar 1997;78(3):313-8. [Medline].

  29. Sneller MC. Common variable immunodeficiency. Am J Med Sci. Jan 2001;321(1):42-8. [Medline].

  30. Tarr PE, Sneller MC, Mechanic LJ, et al. Infections in patients with immunodeficiency with thymoma (Good syndrome). Report of 5 cases and review of the literature. Medicine (Baltimore). Mar 2001;80(2):123-33. [Medline].

 
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