eMedicine Specialties > Dermatology > Allergy & Immunology
Severe Combined Immunodeficiency: Follow-up
Updated: Dec 8, 2008
Follow-up
Further Outpatient Care
- Ensure regular follow-up visits to monitor the immune system, with specialist physicians monitoring the severe combined immunodeficiency (SCID) patient.
Inpatient & Outpatient Medications
- Patients require prophylactic antibiotics and IVIG prior to bone marrow replacement.
- Patients who have ADA deficiency need enzyme replacement therapy. Patients need to have their immune function monitored and prophylaxis provided depending on their immune status.
- For those patients who underwent bone marrow transplantation, medication therapy to prevent GVHD must be maintained.4
Complications
- Patients who receive bone marrow transplants develop GVHD and the adverse effects of the medications necessary to control GVHD.
- Patients are at risk for infections from inadequate immune reconstitution from bone marrow transplantation or enzyme replacement. Gene therapy has been associated with virus-induced malignancies.
Prognosis
- Untreated SCID is fatal.
- Treatment with bone marrow transplantation or enzyme replacement to reconstitute the immune system can lead to long-term survival.
Patient Education
- For information and links to other useful sites and for information regarding a support group, see the Severe Combined Immunodeficiency Web site.
Miscellaneous
Medicolegal Pitfalls
- Failure to diagnose the condition
More on Severe Combined Immunodeficiency |
| Overview: Severe Combined Immunodeficiency |
| Differential Diagnoses & Workup: Severe Combined Immunodeficiency |
| Treatment & Medication: Severe Combined Immunodeficiency |
Follow-up: Severe Combined Immunodeficiency |
| References |
| « Previous Page |
References
Puck JM,. Population-based newborn screening for severe combined immunodeficiency: steps toward implementation. J Allergy Clin Immunol. Oct 2007;120(4):760-8. [Medline].
Ariga T. Gene therapy for primary immunodeficiency diseases: recent progress and misgivings. Curr Pharm Des. 2006;12(5):549-56. [Medline].
Fischer A, Hacein-Bey S, Le Deist F, de Saint Basile G, Cavazzana-Calvo M. Gene therapy for human severe combined immunodeficiencies. Immunity. Jul 2001;15(1):1-4. [Medline].
Friedrich W, Hönig M, Müller SM. Long-term follow-up in patients with severe combined immunodeficiency treated by bone marrow transplantation. Immunol Res. 2007;38(1-3):165-73. [Medline].
Bonilla FA, Geha RS. 2. Update on primary immunodeficiency diseases. J Allergy Clin Immunol. Feb 2006;117(2 Suppl Mini-Primer):S435-41. [Medline].
Buckley RH, Schiff RI, Schiff SE, Markert ML, Williams LW, Harville TO, et al. Human severe combined immunodeficiency: genetic, phenotypic, and functional diversity in one hundred eight infants. J Pediatr. Mar 1997;130(3):378-87. [Medline].
Buckley RH, Schiff SE, Schiff RI, Markert L, Williams LW, Roberts JL, et al. Hematopoietic stem-cell transplantation for the treatment of severe combined immunodeficiency. N Engl J Med. Feb 18 1999;340(7):508-16. [Medline].
De Raeve L, Song M, Levy J, Mascart-Lemone F. Cutaneous lesions as a clue to severe combined immunodeficiency. Pediatr Dermatol. Mar 1992;9(1):49-51. [Medline].
Fischer A. Primary immunodeficiency diseases: an experimental model for molecular medicine. Lancet. Jun 9 2001;357(9271):1863-9. [Medline].
Gaspar HB, Gilmour KC, Jones AM. Severe combined immunodeficiency--molecular pathogenesis and diagnosis. Arch Dis Child. Feb 2001;84(2):169-73. [Medline].
Gennery AR, Cant AJ. Diagnosis of severe combined immunodeficiency. J Clin Pathol. Mar 2001;54(3):191-5. [Medline].
Grunebaum E, Mazzolari E, Porta F, Dallera D, Atkinson A, Reid B, et al. Bone marrow transplantation for severe combined immune deficiency. JAMA. Feb 1 2006;295(5):508-18. [Medline].
Kovanen PE, Leonard WJ. Cytokines and immunodeficiency diseases: critical roles of the gamma(c)-dependent cytokines interleukins 2, 4, 7, 9, 15, and 21, and their signaling pathways. Immunol Rev. Dec 2004;202:67-83. [Medline].
Postigo Llorente C, Ivars Amorós J, Ortiz de Frutos FJ, Regueiro JR, Llamas Martín R, Guerra Tapia A, et al. Cutaneous lesions in severe combined immunodeficiency: two case reports and a review of the literature. Pediatr Dermatol. Dec 1991;8(4):314-21. [Medline].
Roifman CM, Zhang J, Chitayat D, Sharfe N. A partial deficiency of interleukin-7R alpha is sufficient to abrogate T-cell development and cause severe combined immunodeficiency. Blood. Oct 15 2000;96(8):2803-7. [Medline].
Rosen FS. Severe combined immunodeficiency: a pediatric emergency. J Pediatr. Mar 1997;130(3):345-6. [Medline].
Tsuji Y, Imai K, Kajiwara M, Aoki Y, Isoda T, Tomizawa D, et al. Hematopoietic stem cell transplantation for 30 patients with primary immunodeficiency diseases: 20 years experience of a single team. Bone Marrow Transplant. Mar 2006;37(5):469-77. [Medline].
Further Reading
Keywords
combined immunodeficiency, SCID, primary immunodeficiency, SCID with B cells, SCID without B cells
Follow-up: Severe Combined Immunodeficiency