Question

Case Study - Diseases of the Immune System.

Peter was born after an uneventful pregnancy and weighed 3.1kg. At 3 months, he developed otitis media and an upper respiratory tract infection. At the ages of 5 months and 11 months, he was admitted to hospital with Hemophilus influenza pneumonia. The infections responded promptly to the appropriate antibiotics on each occasion. When 16 months old, he had a relapse of otitis media. He is the fourth child of unrelated parents: his three sisters show no predisposition to infection.

Examination at the age of 18 months showed a pale, thin child whose height and weight were below the third centile. There were no other abnormal features. He had been fully immunized as an infant (at 2, 3 and 4 months) with tetanus and diphtheria toxoids, whole-cell pertussis, Haemophilus vaccine and oral polio. In addition, he had received measles, mumps and rubella vaccine at 12 months. All immunizations were uneventful.

Immunological investigations into the cause of his recurrent infections are shown below. Both parents deny family history of immune system disorders. After carefully studying the laboratory results, the medical staff decided upon a diagnosis and began treatment.  

Serological Laboratory Test Results for Peter

*Normal range for age 18 months shown in brackets.

Quantitative serum immunoglobulins (g/l)
IgG	0.17	[5.5-10.0]
IgA	Not detected	[0.3-0.8]
IgM	0.07	[0.4-1.8]
Antibody activity
Immunization responses
Tetanus toxoid - no detectable IgG antibodies
Diphtheria toxoid - no detectable IgG antibodies
Polio - no IgG antibodies detected
Measles - no IgG antibodies detected
Rubella - no IgG antibodies detected
Isohemagglutinins (IgM) not detected (blood group A Rh+)
Blood lymphocyte sub populations (x109/l)
Total lymphocyte count	3.5	[2.5-5.0]
T lymphocytes (CD3)	3.02	[1.5-3.0]
B lymphocytes (CD23)	<0.03	[0.1-0.4]
(CD19)	<0.1	[0.3-1.0]
(CD20)	<0.1	[0.3-1.0]

What do you think Peter's therapy should include to help him?

What do you think the diagnosis for Peter was?

Would you venture a name for Peter's condition?

Answer 1

1, There is a low IgG and IgM indicates a higher chance of getting an infection. antibody deficiency is associated with higher susceptibility to infection by bacteria. this systemic defect CD20 was the first B-cell specific difference antigens. CD20 Immunotherapy using chimeric mAbs used for treating B-cell neoplasia for autoimmune disease. Intravenous immunoglobulin therapy is helpful instead of antibiotic prophylaxis.
2, Hypogammaglobulinemia
It is an immune disorder result in the reduction of type of gamma globulins(CD20, CD19) including antibodies that help to fight against infection. patient will have infection and symptoms like coughing, throat pain, fever, ear pain, congestion, and nausea, etc.
3, It is a congenital intrinsic immune system defect. peter developed with pneumonia, it makes complication like cancer, because immature B cell it does not make adequate antibodies which can venture peter condition with lymphoma or Granulomatorus-lymphocytic interstitial lung disease(GLILD)