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858 Transient hypogammaglobulinemia of infancy: Long term follow-up
J ALLERGY CLIN IMMUNOL VOLUME 97, NUMBER 1, PART3
857
858
Abstracts
Prevalence of Humoral Immunodeficiency in the African. American and Hispanic Populations of East Harlem M.Lewis. M. Muiashiro. 3". Hunmn. L. Miller. K. St~rber. NY, NY To date few studies have attempted to establish the prevalence ot humoral immunodeficiency in African-American and Hispanic populations. To determine the prevalence of humorai immunodeficieacy in this population, we measured serum IgG, IgM, and IgA levels along with IgO subclasses in 202 African-American and Hispanic patients who attended a general medical clinic in East Harlem in NYC. The patient population consisted of 43 males and 159 females with a mean age of 57.3 years. There were 147 Hispanic patients, 51 AfricanAmerican patients and 2 Native-American patients. The mean IgG level was 1319.3 ± 371.9 mg/di with 4 patients below the lower limil of the normal values (800-1800 mg/di). The mean IgA level was 335 ± 83.8 rag/all with 2 patients below the lower limit of normal values (100-400 rag/all) while the mean IgM level was 172.5 + 83.3 mg/dl with 1 patient below the lower limit of normal values (40-200 mg/dl). The mean IgG1 level was 811 + 231.0 mg/dl with 1 patient below the lower limit of normal values (420-1290 mg/dl). The mean IgG2 level was 441.6 + 128.9 rag/all ( normal range 115-745 mg/dl) while the mean IgG3 level was 91.9 + 42.4 rag/all with 9 patients below the lower limit of normal values (40-200 mg/dl) and the mean IgG4 subclass level was 62.7 ± 31.5 rag/all (normal range 1-290 mg/dl). Ot the 7 patients with low IgG, IgA and IgM levels, 2 had asthma, 2 had allergic rhinitis, and 3 had rheumatoid arthritis. The IgGl deficient patient had recurrent paeumocoecal pneumonia and 2 of the 7 lgG3 deficient patients had allergic rhinitis with asthma. Taken together, om data indicate that humoral immunodeficieacy may be more prevalent in the low socioeconomic African-American and Hispanic populations than previously recognized. Future studies using larger sample sizes may be needed to determine the true prevalence of humoral immunodeficiency in this community.
859
Transient Hypogammaglobulinemia of Infancy: Long T e r m F o l l o w - U p . SJ McGeady. MD. JL Beaasoleil. MD, Phila., PA. We extend a previous report of infants with decreased levels of one or more immunoglobulin isotypes and intact antibody production to include 35 subjects with follow-up to age 5 years in seven subjects and age 8 years in one subject. Prospective immunoglobulin determinations revealed the following abnormalities:
860
Decreased Decreased IgM IgA 34 (99%) 0-11 mos. (n=35) 6 07%) 14 (64%) 0 12-23 mo (n=22) 0 10 (83%) 24-35 mo (n=12) I (12%) 6 (75%) 36-47 mo (n=8) 0 7 (77%) 48-59 mo (n--9) 0 5(71%) 5 years (n=7) 0 I (100%) 1 (100%) 6 years (n=l) 1 (100%) 0 1 (100%) 7 years (n=l) 1 (100%) 0 8years (n=l) 0 We conclude that hypogammaglobulinemia of infanc usually resolves with age, though this may take as long as seven years. A minority of cases actually represent the prodrome of selective lgA deficiency or other immunoglobulin abnormality. AGE
Decreased IgG 22 (63%) 7 (32%) 3 (25%) 2 (25%) 2 (22%) I (14%)
Severe Asthma, Difficult Inactions, Hypogammaglobulinemia. SR Lane
397
and HD,
Mild SJ
Bi~elsen MD, Hoorestown NJ. There appears to be a subgroup of severe asthmatics with particular difficulty handling infection. We report a group of 4 steroid dependent asthmatics who have had prolonged bouts of sinusitis and bronchitis accompanied by worsening of their asthma and often resulting in hospitalization. The most severe required mechanical ventilation on four occasions. The second had chronic sinusitis, nasal polyps (requiring endoscopic surgery) and multiple acute infections. The final two had bronchial infections with unusual organisms (Pseudomonas, Acinetobacter, and M. azium), and often required IV antibiotics. All 4 were found to have a mild antibody deficiency (either total igG <600, low subclass levels, or low specific antibody titers). Therapy with IVIG proved beneficial in all patients as demonstrated by decreased prednisone use, less hospital days, fewer infections and improved asthma symptoms. Severe asthmatics, particularly those with difficult infections, may respond to IVIG and should, therefore, be evaluated for immune deficiency.
~anhypogammaglob~Unemla after Anficonvulsant T h e r a p y . C K C h u n g M D M P H . L G Bfll/ps P h D . H W S c h r o e d e r Jr M D P h D . B i r m i n g h a m , A L Depre~ion of serum IgA has been as~ciated with phenytoin exposure, but panhypogammaglobulinemia is rarely reported. We describe a previously healthy 60 yo white male with normal serum protein concentrations who developed Common Variable Immonodeficiency within 3 months of starting phenytoin for a seizare. The drug was terminated when a rash developed. The rash resolved on predaiaone. Carbamar~ine and divalpruex sodium were subsequently terminated for elevations in serum hepatic enzyme concentrations and rashes. He remains asymptematic for seizures on phenobarbital. Ten weeks after exposure to phenytoin, he developed sinusitis and bronchitis. His serum immunoglobulins were: IgG of 329 with IgGi,3 deficioncy, IgA < 10, and IgM < 16 mg/dL He failed to respond to challenge with tetanus and pnemnococeal vaccines. The O- patient displayed no isohemagglutinin anti-A or anfi-B rites. HLA typing revealed DQ5;DRI;B44,g;A1,25. Delayed hypersensitivity tests were anergic. There were less than 35 B colls/mm3 in the blood (hi> 100 B cells/ram3), but T cell numbers and CD4/CD8 ratio were normal. The patient suffered through Giardia diarrhea and a perforated tympanic membrane. Six mtmths after panhypogammaglobulinemia was initially discovered, serum IgA and IgM remained depressed and IgG had declined to 52 mg/dl. A bone marrow aspirate demonmmted the presence of all B cell compartments, but the absolute numbers were proportionately diminished. The parleut remains oll ]WIG 300 mg/kg q 4 weeks with clinical improvement. Recently, peripheral B cell numbers have been rising. Panhypogamnmglobufinemia must he considered in patients with infections following anticonvulsaut therapy.
857
858
Abstracts
Prevalence of Humoral Immunodeficiency in the African. American and Hispanic Populations of East Harlem M.Lewis. M. Muiashiro. 3". Hunmn. L. Miller. K. St~rber. NY, NY To date few studies have attempted to establish the prevalence ot humoral immunodeficiency in African-American and Hispanic populations. To determine the prevalence of humorai immunodeficieacy in this population, we measured serum IgG, IgM, and IgA levels along with IgO subclasses in 202 African-American and Hispanic patients who attended a general medical clinic in East Harlem in NYC. The patient population consisted of 43 males and 159 females with a mean age of 57.3 years. There were 147 Hispanic patients, 51 AfricanAmerican patients and 2 Native-American patients. The mean IgG level was 1319.3 ± 371.9 mg/di with 4 patients below the lower limil of the normal values (800-1800 mg/di). The mean IgA level was 335 ± 83.8 rag/all with 2 patients below the lower limit of normal values (100-400 rag/all) while the mean IgM level was 172.5 + 83.3 mg/dl with 1 patient below the lower limit of normal values (40-200 mg/dl). The mean IgG1 level was 811 + 231.0 mg/dl with 1 patient below the lower limit of normal values (420-1290 mg/dl). The mean IgG2 level was 441.6 + 128.9 rag/all ( normal range 115-745 mg/dl) while the mean IgG3 level was 91.9 + 42.4 rag/all with 9 patients below the lower limit of normal values (40-200 mg/dl) and the mean IgG4 subclass level was 62.7 ± 31.5 rag/all (normal range 1-290 mg/dl). Ot the 7 patients with low IgG, IgA and IgM levels, 2 had asthma, 2 had allergic rhinitis, and 3 had rheumatoid arthritis. The IgGl deficient patient had recurrent paeumocoecal pneumonia and 2 of the 7 lgG3 deficient patients had allergic rhinitis with asthma. Taken together, om data indicate that humoral immunodeficieacy may be more prevalent in the low socioeconomic African-American and Hispanic populations than previously recognized. Future studies using larger sample sizes may be needed to determine the true prevalence of humoral immunodeficiency in this community.
859
Transient Hypogammaglobulinemia of Infancy: Long T e r m F o l l o w - U p . SJ McGeady. MD. JL Beaasoleil. MD, Phila., PA. We extend a previous report of infants with decreased levels of one or more immunoglobulin isotypes and intact antibody production to include 35 subjects with follow-up to age 5 years in seven subjects and age 8 years in one subject. Prospective immunoglobulin determinations revealed the following abnormalities:
860
Decreased Decreased IgM IgA 34 (99%) 0-11 mos. (n=35) 6 07%) 14 (64%) 0 12-23 mo (n=22) 0 10 (83%) 24-35 mo (n=12) I (12%) 6 (75%) 36-47 mo (n=8) 0 7 (77%) 48-59 mo (n--9) 0 5(71%) 5 years (n=7) 0 I (100%) 1 (100%) 6 years (n=l) 1 (100%) 0 1 (100%) 7 years (n=l) 1 (100%) 0 8years (n=l) 0 We conclude that hypogammaglobulinemia of infanc usually resolves with age, though this may take as long as seven years. A minority of cases actually represent the prodrome of selective lgA deficiency or other immunoglobulin abnormality. AGE
Decreased IgG 22 (63%) 7 (32%) 3 (25%) 2 (25%) 2 (22%) I (14%)
Severe Asthma, Difficult Inactions, Hypogammaglobulinemia. SR Lane
397
and HD,
Mild SJ
Bi~elsen MD, Hoorestown NJ. There appears to be a subgroup of severe asthmatics with particular difficulty handling infection. We report a group of 4 steroid dependent asthmatics who have had prolonged bouts of sinusitis and bronchitis accompanied by worsening of their asthma and often resulting in hospitalization. The most severe required mechanical ventilation on four occasions. The second had chronic sinusitis, nasal polyps (requiring endoscopic surgery) and multiple acute infections. The final two had bronchial infections with unusual organisms (Pseudomonas, Acinetobacter, and M. azium), and often required IV antibiotics. All 4 were found to have a mild antibody deficiency (either total igG <600, low subclass levels, or low specific antibody titers). Therapy with IVIG proved beneficial in all patients as demonstrated by decreased prednisone use, less hospital days, fewer infections and improved asthma symptoms. Severe asthmatics, particularly those with difficult infections, may respond to IVIG and should, therefore, be evaluated for immune deficiency.
~anhypogammaglob~Unemla after Anficonvulsant T h e r a p y . C K C h u n g M D M P H . L G Bfll/ps P h D . H W S c h r o e d e r Jr M D P h D . B i r m i n g h a m , A L Depre~ion of serum IgA has been as~ciated with phenytoin exposure, but panhypogammaglobulinemia is rarely reported. We describe a previously healthy 60 yo white male with normal serum protein concentrations who developed Common Variable Immonodeficiency within 3 months of starting phenytoin for a seizare. The drug was terminated when a rash developed. The rash resolved on predaiaone. Carbamar~ine and divalpruex sodium were subsequently terminated for elevations in serum hepatic enzyme concentrations and rashes. He remains asymptematic for seizures on phenobarbital. Ten weeks after exposure to phenytoin, he developed sinusitis and bronchitis. His serum immunoglobulins were: IgG of 329 with IgGi,3 deficioncy, IgA < 10, and IgM < 16 mg/dL He failed to respond to challenge with tetanus and pnemnococeal vaccines. The O- patient displayed no isohemagglutinin anti-A or anfi-B rites. HLA typing revealed DQ5;DRI;B44,g;A1,25. Delayed hypersensitivity tests were anergic. There were less than 35 B colls/mm3 in the blood (hi> 100 B cells/ram3), but T cell numbers and CD4/CD8 ratio were normal. The patient suffered through Giardia diarrhea and a perforated tympanic membrane. Six mtmths after panhypogammaglobulinemia was initially discovered, serum IgA and IgM remained depressed and IgG had declined to 52 mg/dl. A bone marrow aspirate demonmmted the presence of all B cell compartments, but the absolute numbers were proportionately diminished. The parleut remains oll ]WIG 300 mg/kg q 4 weeks with clinical improvement. Recently, peripheral B cell numbers have been rising. Panhypogamnmglobufinemia must he considered in patients with infections following anticonvulsaut therapy.