Agarwal and Cunningham-Rundles reported a score that can help to direct the management of a patient with hypogammaglobulinemia. This is based on clinical and laboratory findings. The authors are from Mount Sinai School of Medicine in New York City.
Patient selection: hypogammaglobulinemia
Laboratory parameters:
(1) serum IgG in mg/dL
(2) serum IgA in mg/dL
(3) serum IgM in mg/dL
(4) protective antibodies to diphtheria or tetanus toxoid
(5) percent of pneumococcal serotypes with protective antibodies
Clinical history:
(1) number of episodes of pneumonia during lifetime
(2) number of upper respiratory infections per year
(3) number of courses of antibiotics per year
(4) autoimmune disease (ITP, AIHA, etc)
(5) serious non-lung infections (sepsis, meningitis, osteomyelitis, empyema, septic arthritis)
(6) splenomegaly or splenectomy
(7) lymphadenopathy
(8) infectious diarrhea other than Clostridium difficile
(9) gastrointestinal disease (malabsorption, chronic gastroenteritis, inflammatory-bowel like disease)
(10) weight loss or failure to thrive
(11) hospitalizations within the past 5 years
Other:
(1) pulmonary function test as percent of predicted (FEV1/FVC or total lung capacity)
(2) bronchiectasis
Parameter |
Finding |
Points |
---|---|---|
serum IgG |
>= 600 mg/dL |
0 |
|
350 to 599 mg/dL |
1 |
|
150 to 349 mg/dL |
3 |
|
< 150 mg/dL |
5 |
serum IgA |
normal |
0 |
|
reduced |
3 |
|
greatly reduced (<= 10 mg/dL) |
5 |
serum IgM |
normal |
0 |
|
reduced |
3 |
|
greatly reduced (<= 15 mg/dL) |
5 |
protective antibodies to diphtheria or tetanus |
yes |
0 |
|
no |
5 |
percent protective pneumococcal serotypes |
>= 50% |
0 |
|
20 to 49% |
3 |
|
< 20% |
5 |
episodes of pneumonia during lifetime |
0 |
0 |
|
1 |
1 |
|
2 |
2 |
|
3 |
3 |
|
4 |
4 |
|
>= 5 |
5 |
upper respiratory infections in past year |
0 |
0 |
|
1 |
1 |
|
2 |
2 |
|
3 |
3 |
|
>= 4 |
5 |
antibiotic courses per year |
0 |
0 |
|
1 |
1 |
|
2 |
2 |
|
3 |
3 |
|
4 |
4 |
|
>= 5 |
5 |
autoimmune disease |
absent |
0 |
|
present |
3 |
serious infection other than pneumonia |
absent |
0 |
|
present |
5 |
splenectomy or splenomegaly |
no |
0 |
|
yes |
3 |
lymphadenopathy |
no |
0 |
|
yes |
3 |
infectious diarrhea |
no |
0 |
|
yes |
3 |
gastrointestinal disease |
no |
0 |
|
yes |
3 |
weight loss or failure to thrive |
no |
0 |
|
yes |
3 |
hospitalizations in past 5 years |
none in past 5 years |
0 |
|
1 |
1 |
|
2 |
2 |
|
3 |
3 |
|
4 |
4 |
|
>= 5 |
5 |
pulmonary function tests |
normal |
0 |
|
70 to 79% of predicted |
1 |
|
60 to 69% of predicted |
3 |
|
< 60% of predicted |
5 |
bronciectasis |
absent |
0 |
|
present |
5 |
laboratory score =
= SUM(points for the laboratory items)
cumulative score =
= SUM(points for all of the items)
Interpretation:
• minimum laboratory score: 0
• maximum laboratory score: 25
• minimum cumulative score: 0
• maximum cumulative score: 78
Laboratory Score |
Cumulative Score |
Management |
---|---|---|
>= 10 |
NA |
treat with immunoglobulin |
< 10 |
>= 17 |
treat with immunoglobulin |
< 10 |
10 to 16 |
consider immunoglobulin |
< 10 |
0 to 9 |
monitor |
Specialty: Immunology/Rheumatology, Infectious Diseases
ICD-10: ,