Tuesday, November 20, 2012

Immunocompromising conditions

Immunocompromising Conditions (List from eMedicine)
a) Congenital
b) Acquired
c) Iatrogenic / self inflicted

a) Congenital Conditions

These most commonly affect the fetus and newborn. Hemoglobinopathy may be noted.
Syndromes
  • Partial albinism with immunodeficiency (Griscelli) syndrome[2, 3]
  • Hemorrhagic hereditary telangiectasia (Rendu-Osler disease)[4]
  • Immunodeficiency-centromeric instability-facial anomalies (ICF) syndrome
  • Kabuki syndrome
  • Partial albinism, immunodeficiency, and progressive white matter disease (PAID) syndrome
  • Autoimmune polyendocrinopathy syndrome type 1
  • Rubinstein-Taybi syndrome[5]
  • Hermansky-Pudlak-2 syndrome
  • CHARGE syndrome[6]
  • Other dysmorphology or immunodeficiency syndromes
  • Stromal interaction molecule 1 mutation[7]
B-cell defects[8]
  • Antibody deficiency with transcobalamin II deficiency
  • Antibody deficiency with normal or high immunoglobulin (Ig) levels
  • Common variable immunodeficiency
  • IgG heavy-chain deletion
  • IgG subclass deficiency
  • Kappa-chain deficiency
  • Organic cation transporter 2 deficiency
  • Selective IgA deficiency
  • Selective IgM deficiency
  • Selective antipolysaccharide antibody deficiency
  • Transient hypogammaglobulinemia of infancy or early childhood
  • Thymoma with agammaglobulinemia
  • X-linked (Bruton) agammaglobulinemia
  • X-linked hyper-IgM syndrome
  • X-linked hypogammaglobulinemia with growth hormone deficiency
Combined B-cell and T-cell defects
  • Adenosine deaminase deficiency
  • Artemis deficiency
  • Ataxia-telangiectasia syndrome
  • Bare lymphocyte syndrome (major histocompatability complex class I/II deficiency)
  • DOCK8 mutations[9]
  • Interleukin (IL)-2R alpha or gamma deficiency
  • Intestinal lymphangiectasia
  • Janus kinase 3 (JAK3) deficiency
  • Nuclear factor-kappaB essential modifier (NEMO) deficiency (Dupuis-Girod, 2002; Courtois, 2006; Smahi, 2002; Zonana, 2000)
  • Nijmegen breakage syndrome
  • Purine nucleoside phosphorylase deficiency
  • Recombination activation gene (RAG) 1 or 2 deficiency
  • Reticular dysgenesis
  • Swiss-type severe combined immunodeficiency
  • T-cell receptor deficiency
  • X-linked lymphoproliferative syndrome
  • X-linked severe combined immunodeficiency
  • Zeta-associated protein of 70 kDa (ZAP-70) tyrosine kinase deficiency
T-cell defects
  • Biotin-dependent multiple carboxylase deficiency
  • Chronic mucocutaneous candidiasis
  • DiGeorge (velocardiofacial) syndrome
  • Fas defect
  • Nezelof syndrome
  • Short-limbed dwarfism or cartilage-hair hypoplasia
Macrophage, cytokine, and miscellaneous defects
  • Mendelian susceptibility to mycobacterial diseases (MSMD)
    • Interferon-gamma deficiency
    • Interferon-gamma receptor I or II deficiency
    • IL-12 deficiency
    • IL-12 receptor deficiency
    • STAT1 mutations
    • NEMO
    • CYBB
  • IL-1 receptor–associated kinase 4 (IRAK4) deficiency
  • MYD88 deficiency
  • Toll-like receptor 5 mutations
  • Apolipoprotein L-I deficiency
  • UNC-93B deficiency
  • Toll-like receptor 3 mutations
  • TRIF and TRAF3 mutations
  • Plasminogen activator inhibitor-1 4G/4G promoter genotype
  • Anti-interferon-gamma antibodies
  • IL-18 polymorphisms
  • RANTES promoter gene polymorphisms[11]
  • Deficiency of chemokine receptor CCR5[12, 13]
  • Toll-like receptor 4 mutations
  • IL-8 RA (chemokine CXC motif receptor 1 [CXCR1]) mutations
  • CXCR4 mutations (Whim syndrome)
  • STAT 5 mutations
  • NOD2 gene polymorphisms
  • IL-6 polymorphisms
  • Activating killer immunoglobulinlike receptor gene polymorphisms
  • Dectin-1 deficiency
  • CARD9 mutations
  • Polymorphisms in cytokine-inducible SRC homology 2 domain protein (CISH)
  • Polymorphisms in Mal/TIRAP and Interleukin-10
  • Autoantibodies against IL-6[14]
  • Polymorphisms in the IL-8 promoter gene[15]
  • IL-12 receptor deficiency (Vinh, 2011)
Phagocyte deficiency or dysfunction
  • Chediak-Higashi syndrome
  • Chronic granulomatous disease
  • Chronic idiopathic neutropenia
  • Cyclic neutropenia
  • Glycogen storage disease 1b
  • Hyper-IgE/recurrent infection (Job) syndrome (Janus kinase protein tyrosine kinase 2 [Tyk2], signal transducer and activator of transcription [STAT] 3, and STAT 1 mutations)[16]
  • Kostmann syndrome
  • Leukocyte adhesion deficiency (including CD11 or CD18 deficiency)
  • Myeloperoxidase deficiency
  • Neutrophil actin dysfunction
  • Papillon-Lefèvre syndrome
  • Specific granule deficiency
  • Shwachman-Diamond syndrome
Complement deficiencies (Ram, 2010)
  • Mannose-binding lectin (Mannan-binding protein) deficiency[17, 18]
  • Deficiencies of C1q, C1r, C1rs, C4, C2, C3, or C5-9
  • Deficiencies of factor D, factor P, factor I, factor H, or properdin
  • Ficolin-3 (H-ficolin) deficiency[19]
Other conditions
  • Asplenia[20]
  • Ciliary dyskinesia, Kartagener syndrome, and other disorders
  • Galactosemia and other metabolic conditions
  • Lymphedema (congenital)
  • Trisomy 21 and other genetic disorders
  • Other anatomic defects (eg, midline dermal sinus, Mondini defect of the inner ear, fistulae, cysts, duplications, meningeal defects, iron overload, decreased sensation)

b) Acquired Conditions

These conditions may interfere directly with the immune system or may disrupt barrier function.
  • Malnutrition
  • HIV infection: Although human immunodeficiency virus (HIV) infection is a considerable cause of immunodeficiency worldwide, immunocompromise is most likely to result from common problems, including asthma, diabetes, malnutrition, and cancer, among others.
  • Trauma
    • Burns
    • Lacerations and abrasions
  • Medical conditions
    • Collagen vascular
    • GI tract
    • Hematologic or oncologic
    • Hepatic
    • Metabolic
    • Pregnancy
    • Pulmonary, particularly asthma and cystic fibrosis (CF)
    • Renal
    • Skin and mucous membrane
    • Viral infections (eg, cytomegalovirus [CMV] infection,[21] measles)
    • Other anatomic or physiologic problems (eg, fistulae, cysts, obstructions, iron overload, decreased sensation)
  • Acquired asplenia (Ram, 2010)
  • Acquired lymphedema
  • Other conditions that injure or bypass barrier function
    • Parasitic infections
    • Animal and insect bites or scratches

c) Iatrogenic or Self-inflicted Conditions

These conditions may directly interfere with the immune system or may disrupt barrier function.
Use of drugs and/or therapies (eg, radiation therapy), which may interfere with normal flora, decrease gastric acidity and ciliary motility, and be directly immunomodulating[22]
Trauma
  • Injections (eg, insulin injections, intravenous [IV] drug use, others)
  • Operative and other incisions
  • Vascular, osseous, tracheal, gastric, bladder, joint, peritoneal, wound, or ventricular access or drainage devices
  • Internal foreign bodies
  • Major surgery[23]
Treatment
  • For leukemia or lymphoma
  • Bone marrow or stem-cell transplantation
  • Solid organ transplantation (Yin, 2011)
  • Therapy for autoimmune or inflammatory disorders
  • TNF-alpha inhibitors[24]
  • Monoclonal antibodies and related small molecules
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