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Leukocyte Adhesion Deficiency Treatment & Management

  • Author: Stephen J Nervi, MD; Chief Editor: Harumi Jyonouchi, MD  more...
 
Updated: Nov 18, 2014
 

Medical Care

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  • Bone marrow and other stem cell transplantation are the therapies of choice in leukocyte adhesion deficiency (LAD) and have a very high success rate.[10, 11, 12] Thus, bone marrow or other stem cell reconstitution is a first-line treatment for severe leukocyte adhesion deficiency type I, in which less than 1% CD18 expression is detected. Donors may provide human leukocyte antigen (HLA)-matched, related, haploidentical, or unrelated HLA–matched hematopoietic stem cells. The high rate of successful engraftment in patients with leukocyte adhesion deficiency I is thought to be due to absence of CD11a/CD18 expression on lymphocytes; antibodies directed against this integrin also seem to improve engraftment of bone marrow stem cells and prevent graft versus host disease in patients who underwent hematopoietic stem cell transplantation (HSCT) for other disorders. However, not all patients are candidates for early bone marrow transplants.
  • Other intervention measures for leukocyte adhesion deficiency I have included prophylactic antibiosis, interferon-gamma, and leukocyte transfusions; none of these has shown significant benefit.
  • Gene therapy with insertion of the CD18 subunit is currently under investigation. Because patients with decreased expression of CD18 (1-30%) have a milder disease, partial reconstitution is anticipated to provide clinical benefit.
  • Leukocyte adhesion deficiency II does not require prophylactic antibiosis. Fucose replacement administered orally or intravenously has variable effectiveness in improving phagocytic functions.
  • The use of granulocyte transfusions has been advocated. Donors must be carefully screened to prevent transmission of infection. In the author's experience, the efficacy of granulocyte transfusions was difficult to prove, and pulmonary sequestration compromise lung severely with marked febrile reactions.
  • Interferon-gamma showed no efficacy in one patient (single case report).
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Surgical Care

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  • Surgical procedures for leukocyte adhesion deficiency I are of high risk and require flawless postoperative care because of the delayed wound healing and risk for further infection.
  • Complications of surgical procedures in leukocyte adhesion deficiency II have not been reported.
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Consultations

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  • Consultations with surgeons, pulmonologists, and intensivists are often mandatory. The clinical immunologist must work closely with these consultants because the lack of inflammation leads to the underestimation of infection by inexperienced medical personnel.
  • Bone marrow transplantation teams are mandatory for therapy of severe leukocyte adhesion deficiency I.
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Diet

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  • A normal nutritious diet for age group is appropriate.
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Activity

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  • No restrictions are advised.
  • Obviously, care of skin and mucous membranes as portals of entry for infection requires excellent hygiene.
  • Injuries are slow to heal and are at high risk for secondary infection.
  • Prophylactic antibiotics for injuries are generally used conventionally; the major application is for animal or human bites.
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Contributor Information and Disclosures
Author

Stephen J Nervi, MD Staff Physician, Department of Dermatology, University of Medicine and Dentistry of New Jersey-New Jersey Medical School

Stephen J Nervi, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, Sigma Xi

Disclosure: Nothing to disclose.

Coauthor(s)

Robert A Schwartz, MD, MPH Professor and Head of Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, Rutgers New Jersey Medical School; Visiting Professor, Rutgers University School of Public Affairs and Administration

Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, New York Academy of Medicine, American Academy of Dermatology, American College of Physicians, Sigma Xi

Disclosure: Nothing to disclose.

Monika I Sidor, MD Resident Physician, Department of Surgery, University of Michigan at Ann Arbor Medical School

Monika I Sidor, MD is a member of the following medical societies: Sigma Xi

Disclosure: Nothing to disclose.

Specialty Editor Board

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

David J Valacer, MD 

David J Valacer, MD is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American Academy of Pediatrics, American Association for the Advancement of Science, American Thoracic Society, New York Academy of Sciences

Disclosure: Nothing to disclose.

Chief Editor

Harumi Jyonouchi, MD Faculty, Division of Allergy/Immunology and Infectious Diseases, Department of Pediatrics, Saint Peter's University Hospital

Harumi Jyonouchi, MD is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American Academy of Pediatrics, American Association of Immunologists, American Medical Association, Clinical Immunology Society, New York Academy of Sciences, Society for Experimental Biology and Medicine, Society for Pediatric Research, Society for Mucosal Immunology

Disclosure: Nothing to disclose.

Additional Contributors

Terry W Chin, MD, PhD Associate Clinical Professor, Department of Pediatrics, University of California, Irvine, School of Medicine; Associate Director, Cystic Fibrosis Center, Attending Staff Physician, Department of Pediatric Pulmonology, Allergy, and Immunology, Memorial Miller Children's Hospital

Terry W Chin, MD, PhD is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American Association of Immunologists, American College of Allergy, Asthma and Immunology, American College of Chest Physicians, American Federation for Clinical Research, American Thoracic Society, California Society of Allergy, Asthma and Immunology, California Thoracic Society, Clinical Immunology Society, Los Angeles Pediatric Society, Western Society for Pediatric Research

Disclosure: Nothing to disclose.

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Labial ulceration from which Escherichia coli was cultured in an 8-month-old girl with leukocyte adhesion deficiency type 1 (LAD I). Note the thin bluish scar at the superior aspect of the labia from an earlier cellulitis.
This 3-year-old girl had leukocyte adhesion deficiency type I (LAD I) with complete absence of CD18 expression. Note the typical gingivostomatitis, which was culture-negative for any pathogen.
This 10-month-old patient with severe leukocyte adhesion deficiency type I (LAD I) developed a cervical adenitis caused by Klebsiella pneumoniae. Following incision and drainage, wound healing took 4 months.
 
 
 
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