Score of Agarwal and Cunningham-Rundles for Management of a Patient with Hypogammaglobulinemia

Description

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

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Description

Specialty

ICD-10