Further Outpatient Care
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Scrupulous follow-up of infections is necessary for patients with leukocyte adhesion deficiency.
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As noted above, outpatient management of infections has become customary despite the complexity of care and the stress to families.
Further Inpatient Care
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Leukocyte adhesion deficiency type I (LAD I) is often associated with life-threatening infections requiring intensive care. Coordinating medical management between immunologists, infectious disease specialists, pulmonologists, and surgical specialists is challenging.
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Excellent laboratory and radiology support mandates hospitalization in tertiary children's medical centers.
Inpatient & Outpatient Medications
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Patients with leukocyte adhesion deficiency I are at risk for fungal infections with Candida species because of their frequent need for broad-spectrum antibiotics.
Transfer
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Most clinical immunologists strongly believe that the great complexity of medical problems for any primary immunodeficiency disease requires the patient to be managed by an immunologist.
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The subtle signs of infection, the need to offer stem cell transplantation, and the early deaths in patients with leukocyte adhesion deficiency I that is not properly treated suggest that frequent monitoring by a clinical immunologist is essential.
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The major services for transfer are the intensive care and surgical teams.
Deterrence/Prevention
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Prenatal diagnosis for leukocyte adhesion deficiency I is possible by chorionic villus sampling or amniocentesis using DNA methodology in families where the exact mutations have been established. Fetal blood sampling and fluorocytometric testing for the presence of CD18 on lymphocytes, monocytes, and neutrophils can establish the diagnosis when DNA analysis is not available.
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In leukocyte adhesion deficiency II, ultrasonography for growth retardation, skeletal abnormalities, and distinctive facial features can establish the diagnosis prenatally in some families.
Complications
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Patients with leukocyte adhesion deficiency I are at risk for graft versus host disease post–stem cell reconstitution, although graft versus host disease has been less common than in other transplantation settings.
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Patients with leukocyte adhesion deficiency II experience growth failure and mental retardation, although they are less likely to die of infection. Reports of these patients are so rare that other complications may not be observed adequately yet.
Prognosis
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Without hematopoietic stem cell transplantation (HSCT), patients with leukocyte adhesion deficiency I who have an absence of CD18 expression usually die from infection within 2 years of life. The published experience with hematopoietic stem cell transplantation has been excellent with complete immunologic reconstitution. Earlier reviews of leukocyte adhesion deficiency I indicated that unreconstituted patients most often succumbed to bacterial infections. In the author's experience, early recognition and aggressive therapy of bacterial and fungal infections is usually successful. In contrast, no specific therapy is available for the crouplike infections of suspected viral etiology or for aseptic meningitis.
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Patients with leukocyte adhesion deficiency II show severe developmental delay, which has not been significantly prevented even when fucose replacement seemed to decrease infections and improve phagocytic functions.
Patient Education
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The inability to easily detect infection is extremely stressful to families. Aggressive and complicated antibiotic and surgical care may be required for prolonged periods in the home setting, adding further stress.
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Adequate informed consent for stem cell reconstitution must review the high risk for life-threatening infection during the preparative myeloablative regimen in addition to the risk for failure to engraft and graft versus host disease. Although successful complete immune reconstitution from bone marrow transplantation is reported using fully matched related and unrelated donors or haploidentical donors (ie, parents), patients with leukocyte adhesion deficiency I cannot be guaranteed to have benign courses.
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The Immune Deficiency Foundation (some states have local chapters) is an important resource for education and for support for patients and families with any primary immunodeficiency disease. The current address is as follows: 25 W. Chesapeake Ave, Suite 206
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Towson, MD 21204
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Phone number: 877-666-0866
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Web site: www.primaryimmune.org
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The Jeffrey Modell Foundation also provides educational support and raises funds for research. The current address is as follows: 747 3rd Ave
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New York, NY 10017
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Phone: 800-JEFF-844
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Web site: www.jmfworld.org
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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.
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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.
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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.
