Severe combined immunodeficiency

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Severe combined immunodeficiency

Immune System

Immune System

Thymus histology
Spleen histology
Lymph node histology
Introduction to the immune system
Cytokines
Innate immune system
Complement system
T-cell development
B-cell development
MHC class I and MHC class II molecules
T-cell activation
B-cell activation, differentiation, and contraction
Cell-mediated immunity of CD4 cells
Cell-mediated immunity of natural killer and CD8 cells
Antibody classes
Somatic hypermutation and affinity maturation
VDJ rearrangement
Contracting the immune response and peripheral tolerance
B- and T-cell memory
Anergy, exhaustion, and clonal deletion
Vaccinations
Type I hypersensitivity
Type II hypersensitivity
Type III hypersensitivity
Type IV hypersensitivity
Sepsis
Neonatal sepsis
Abscesses
Food allergy
Anaphylaxis
Asthma
Immune thrombocytopenia
Autoimmune hemolytic anemia
Hemolytic disease of the newborn
Rheumatic heart disease
Myasthenia gravis
Graves disease
Pemphigus vulgaris
Serum sickness
Systemic lupus erythematosus
Poststreptococcal glomerulonephritis
Graft-versus-host disease
Contact dermatitis
Transplant rejection
Cytomegalovirus infection after transplant (NORD)
Post-transplant lymphoproliferative disorders (NORD)
X-linked agammaglobulinemia
Selective immunoglobulin A deficiency
Common variable immunodeficiency
IgG subclass deficiency
Hyperimmunoglobulin E syndrome
Isolated primary immunoglobulin M deficiency
Thymic aplasia
DiGeorge syndrome
Severe combined immunodeficiency
Adenosine deaminase deficiency
Ataxia-telangiectasia
Hyper IgM syndrome
Wiskott-Aldrich syndrome
Leukocyte adhesion deficiency
Chediak-Higashi syndrome
Chronic granulomatous disease
Complement deficiency
Hereditary angioedema
Asplenia
Thymoma
Ruptured spleen
Immunodeficiencies: T-cell and B-cell disorders: Pathology review
Immunodeficiencies: Combined T-cell and B-cell disorders: Pathology review
Immunodeficiencies: Phagocyte and complement dysfunction: Pathology review

Key Takeaways

Severe combined immunodeficiency (SCID) is a severe form of primary immunodeficiency, caused by a genetic mutation that affects the development and function of white blood cells, which are responsible for fighting off infections. It may result from an X-linked recessive defect in IL-2R (interleukin-2 receptor) gamma chain or an autosomal recessive defect in adenosine deaminase deficiency. As a result, individuals with SCID are highly susceptible to infections, particularly bacterial and viral infections, and often present with failure to thrive, chronic diarrhea, thrush, and recurrent infections. Treatment involves HSCT (hematopoietic stem cell transplantation, avoiding live vaccines, antimicrobial prophylaxis, and isolation to prevent catching an infection.