Hematology & immune disorders

Primary immunodeficiency disorders

CR3 deficiency

Last author update: 1 October 2015
Last staff update: 25 February 2021

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PubMed Search: CR3 deficiency

Shamayel Mohammed, M.D.
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Cite this page: Mohammed S. CR3 deficiency. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/lymphnodesCR3deficiency.html. Accessed April 14th, 2024.
Definition / general
  • CR3 deficiency is an autosomal recessive inherited deficiency of the leukocyte beta2 integrin receptor CD11/18 (CR3) causing leukocyte adhesion deficiency syndrome I (LAD I) (Clin Exp Immunol 2000;121:133)
  • Migration and adhesion of leukocytes from the bloodstream to site of infection involves multiple steps of the adhesion cascade
  • Adhesion molecules are expressed on endothelial cells and leukocytes.
  • CD11/CD18 (CR3), also known as the leukocyte beta2 integrin receptor, is one of the main adhesion molecules essential for leukocyte adhesion to endothelial cells and chemotaxis
  • Defects in these adhesion molecules as well other adhesion molecules result in recognized clinical syndromes
  • The deficiency / defect leads to impaired phagocytic function
  • Four distinct complement receptors, CR1, CR2, CR3 and CR4, have been described for the surface bound complement fraction C3 and its cleavage fragments such as iC3b
  • Complement and complement receptors are an integral part of the immune defense
  • CR3 (CD11b/18) and CR4 (CD11c/18) both bind to iC3b and facilitate adequate adhesion of leucocytes with the vascular endothelium
  • Receptors such as CR3 act as ligands for adhesion molecules, and are present on phagocytic cells
  • CR3 and CR4 have an important role in host resistance to infection
Clinical features
  • Recurrent cutaneous infections and gingivitis
  • Delayed separation of the umbilical cord and secondary omphalitis
  • Severe recurrent Staphylococcus aureus and gram negative bacterial infections
  • Periodontitis and impaired wound healing
  • Leukocytosis with lack of pus formation
  • Absence of CD18 and the associated alpha subunit molecules CD11a, CD11b and CD11c on the surface of leukocytes is demonstrated by flow cytometry using CD11 and CD18 monoclonal antibodies
  • Sequence analysis can define the exact molecular defect in the beta 2 subunit
  • Neutrophilia
Case reports
  • Symptomatic
  • Bone marrow and hematopoietic stem cell transplant
Clinical images

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Skin lesions

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Gingivitis, periodontitis

Microscopic (histologic) description
  • Severe lymph node hypoplasia with small, poorly delineated germinal centers
  • Lymphoid tissue, including the thymus, is depleted of lymphocytes (Pediatr Pathol 1992;12:119)
  • Biopsies of infected tissues demonstrate inflammatory infiltrates completely devoid of neutrophils
Microscopic (histologic) images

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Clinical and histological profile

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IL-17 signature

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Skin biopsy

Molecular / cytogenetics description
Differential diagnosis
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