Leukocyte Adhesion Deficiency Treatment & Management

Updated: Oct 09, 2019
  • Author: Stephen J Nervi, MD; Chief Editor: Harumi Jyonouchi, MD  more...
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Treatment

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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