eMedicine Specialties > Pediatrics: General Medicine > Allergy & Immunology

Leukocyte Adhesion Deficiency: Treatment & Medication

Author: Stephen J Nervi, MD, Staff Physician, Department of Dermatology, University of Medicine and Dentistry of New Jersey, New Jersey School of Medicine
Coauthor(s): Robert A Schwartz, MD, MPH, Professor and Head, Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, UMDNJ-New Jersey Medical School; Monika I Sidor, MD, Staff Physician, Department of Surgery, University of Michigan at Ann Arbor
Contributor Information and Disclosures

Updated: Sep 9, 2009

Treatment

Medical Care

  • Bone marrow and other stem cell transplantation are the therapies of choice in leukocyte adhesion deficiency (LAD) and have a very high success rate.7 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).

Surgical Care

  • 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.

Consultations

  • 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.

Diet

  • A normal nutritious diet for age group is appropriate.

Activity

  • 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.

Medication

  • Bacterial infections require aggressive first-line antibiotic therapy, frequently with intravenous agents.
  • Patients with leukocyte adhesion deficiency II can generally be treated as outpatients.
  • After initial diagnosis and stabilization, patients with leukocyte adhesion deficiency I can usually complete parenteral antibiotics in the home setting.

More on Leukocyte Adhesion Deficiency

Overview: Leukocyte Adhesion Deficiency
Differential Diagnoses & Workup: Leukocyte Adhesion Deficiency
Treatment & Medication: Leukocyte Adhesion Deficiency
Follow-up: Leukocyte Adhesion Deficiency
Multimedia: Leukocyte Adhesion Deficiency
References
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References

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Further Reading

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Keywords

leukocyte adhesion deficiency, leukocyte adhesion deficiency type 1, LAD 1, LAD 2, LAD I, LAD II, leukocytosis, localized bacterial infections, CDG-IIc, neutropenia, leukocytosis, congenital disorders of glycosylation, dysfunctional lipid-linked oligosaccharide precursor synthesis, dysfunctional trimming/processing of the protein-bound oligosaccharide, aseptic meningitis, crouplike syndromes, severe mental retardation and developmental delay, neurologic impairment, short stature, periodontitis, colitis, oral ulcerations, hematopoietic stem cell transplantation, delayed umbilical cord separation, omphalitis, perirectal cellulitis, labial cellulitis, otitis media, Staphylococcus species, Candida albicans, bacterial typhlitis, treatment, diagnosis

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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 School of Medicine
Stephen J Nervi, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, and Sigma Xi
Disclosure: Nothing to disclose.

Coauthor(s)

Robert A Schwartz, MD, MPH, Professor and Head, Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, UMDNJ-New Jersey Medical School
Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, and Sigma Xi
Disclosure: Nothing to disclose.

Monika I Sidor, MD, Staff Physician, Department of Surgery, University of Michigan at Ann Arbor
Monika I Sidor, MD is a member of the following medical societies: Sigma Xi
Disclosure: Nothing to disclose.

Medical Editor

Terry Chin, MD, PhD, Associate Professor of Pediatrics, Pediatric Allergy/Immunology/Pulmonology, Department of Pediatrics, University of California Irvine School of Medicine; Associate Director, Miller Children's Hospital at Long Beach Memorial Medical Center
Terry 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 Thoracic Society, California Thoracic Society, Clinical Immunology Society, and Western Society for Pediatric Research
Disclosure: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from financial planner; Avanir Pharma Stock Investment from financial planner ; WebMD Salary and stock Employment and investment from financial planner

Managing Editor

David J Valacer, MD, Consulting Staff, Hoffman La Roche Pharmaceuticals
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, and New York Academy of Sciences
Disclosure: Nothing to disclose.

CME Editor

David Pallares, MD, Clinical Assistant Professor, Department of Pediatrics, Division of Allergy and Immunology, University of Louisville
David Pallares, MD is a member of the following medical societies: American Academy of Allergy Asthma and Immunology
Disclosure: Nothing to disclose.

Chief Editor

Harumi Jyonouchi, MD, Associate Professor, Division of Pulmonary Allergy/Immunology and Infectious Diseases, Department of Pediatrics, UMDNJ-New Jersey Medical School
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 Mucosal Immunology, and Society for Pediatric Research
Disclosure: Nothing to disclose.

 
 
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