Disease Information for Hypogammaglobulinemia/Agammaglobulinemia

Clinical Manifestations
Signs & Symptoms
Depigmented patches, skin
Chronic Diarrhea in a Child
Diarrhea and Weight Loss
Diarrhea in Children
Diarrhea, chronic
Diarrhea, recurrent
Fatty food intolerance
Poor weight gain/child
Recurrent chest infections/bronchitis/sign
Recurrent URI/Colds/LRI infections
Short stature
Short stature Child
Weight Loss
Clinical Presentation & Variations
Presentation/Recurrent Infections
Presentation/Recurrent respiratory infections
Presentation/Agressive pneumonia Immuncompromised
Disease Progression
Course/Chronic disorder
Onset/Six months
Demographics & Risk Factors
Established Disease Population
Patient/Chronic lymphocytic leukemia
Population Group
Population/Pediatrics population
Sex & Age Groups
Population/Boy patient
Population/Older baby 6-12 months
Laboratory Tests
Microbiology & Serology Findings
Common vaccination antibodies absent/low
Abnormal Lab Findings (Non Measured)
Absent/Low B-cells on Flow Cytometry
Abnormal Lab Findings - Decreased
C4, serum (Lab)
Gamma globulin (Lab)
IGE/Immunoglobulin E (Lab)
IGG/Immunoglobulin G (Lab)
Associated Diseases & Rule outs
Rule Outs
Cystic fibrosis (mucoviscidosis)
Associated Disease & Complications
Aseptic meningitis syndrome
Autoimmune disease
Bacterial infection, general
Coxacki virus infection
Giardia infection/intestinal
Growth retardation/failure
Hemolytic anemia/autoimmune
Hemophilus influenza infection/Hemophilus B
Hypogammaglobulinemia, acquired
Immune deficiency , acquired (AIDS/HIV) disease/assn
Immune suppressed status
Malabsorption/Steatorrhea syndrome
Megaloblastic anemia/disease
Meningitis, pneumococcal
Meningococcal arthritis
Meningococcal meningitis
Otitis media, chronic
Pneumococcus infection
Pneumonia, bacterial
Pneumonia, pneumococcal
Pneumonia, recurrent
Poliomyelitis, acute
Rheumatoid arthritis
Sinusitis, chronic
Vaccine associated paralytic poliomyelitis
Asthma Children
Disease Mechanism & Classification
CLASS/Humoral Immune System Disorder (ex)
CLASS/Pediatric disorders (ex)
CLASS/Hematologic (category)
CLASS/Spleen/thymus/RES/immune system (category)
Pathophysiology/Gene locus Xq21.3-q22
Pathophysiology/Infection resistant to usual treatments
Pathophysiology/Proteins/blood disorder (ex)
Pathophysiology/Fat Malabsorption
Pathophysiology/Fat soluble Vitamin Malabsorption
Pathophysiology/Chemotaxis failure/phagocytes/effect
Pathophysiology/Immune physiology/defective
Pathophysiology/Poor immune resp/encapsulated bacteria
Pathophysiology/Poor slow recovery/ bacteria infection
PROCESS/Biochemical (category)
Drug Therapy - Indication
RX/Gamma globulin/prophylaxis/low dose

Hypogammaglobulinemia is a disorder that is caused by a lack of B-lymphocytes and a resulting low level of immunglobulins (antibodies) in the blood; Immunoglobulins play a dual role in the immune response by recognizing foreign antigens and triggering a biological response that culminates in the elimination of the antigen; Antibody deficiency is associated with recurrent infections with specific types of bacteria; In pure B-cell disorders, cellular immunity generally is intact and the frequency of viral, fungal, and mycobacterial ( tuberculosis) infections is not increased; There are 5 major types of immunoglobulins: immunoglobulin G, immunoglobulin M (IgM), immunoglobulin A (IgA), immunoglobulin D (IgD), and immunoglobulin E (IgE); The most common congenital abnormalities of B lymphocyte production include:

Hypogammaglobulinemia (Common Variable Immunodeficiency)

Ig A Deficiency X-linked Agammaglobulinemia (Bruton Disease), Transient hypogammaglobulinemia of infancy,

Hypogammaglobulinemia or Common Variable Immunodeficiency (CVID); CVID is the second most common cause of hypogammaglobulinemia and affects both sexes equally; The incidence is about 1 in 50,000 people; In most patients there is a reduced amount of the immunoglobulins IgG, IgA, and IgM in the blood; It is an immune deficiency disorder that can be acquired or inherited; In most cases, there is no family history of immunodeficiency; However, in instances where more than one family member is affected, an autosomal recessive mode of inheritance is suggested; In about 5% of the cases, one or more of the family members can be found to be IgA deficient; IgA deficiency is the most common antibody deficiency syndrome, about 1 case in 700 persons; Recurrent infections may occur in as many as 50% of IgA-deficient patients, but most of these individuals are healthy; Recurrent or chronic upper and lower respiratory tract infections is common as is Giardia lamblia infections of the gastrointestinal tract; Patients with undetectable levels of IgA antibodies may develop severe allergic reactions if they receive blood products

---------[NORD 2005]---------------


External Links Related to Hypogammaglobulinemia/Agammaglobulinemia
PubMed (National Library of Medicine)
NGC (National Guideline Clearinghouse)
Medscape (eMedicine)
Harrison's Online (accessmedicine)
NEJM (The New England Journal of Medicine)