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496 Partial immunoglobulin deficiency in association with IgG subclass deficiency
VOLUME NUMBER
493
Abstracts
87 1. PART 2
DO PATIENTS WITH INTESTINAL LYMPHANGIECTASIA NEED IMMUNOGLOBULIN REPLACEMENTTHERAPY? IMMUNE EVALUATION OF TWO CHILDREN. S.E. Pacheco. MD. B Reid. MD. H.M. Rosenblatt. MD. C.G Hanson. MD: and W.T. Shearer. MD. PhD., Houston', Texas. Because of the profound degree of hypogammaglobulinemia in primary intestinal lymphangiectasia (IL) concern has arisen for IgG replacement therapy. Limited information exists specific responses ' humoral ' iidiatric patients with I'L?& patients (#l’t y/o and X2 10 y/o) had no history of frequent infections yet were hypogamnaglobulinemic. Both were anergic and had decreased lymphocyte responses to PHA, ConA and PWM (PtO.OO1). Lymphocyte subpopulation studies in patient #2 revealed decreased numbers of CD3 cells with low percentages of CD4 and CD8 cells and increased percentages of 8 cells (CD19,CD20) (Pt0.05). Patient # 2 also had decreased lymphocyte and tetanus responses to Candida, streptokinase antiaens (P
495
These results suaaest a relatiie oreservation of B cell itmounity- despite intestinal loss of impaired immunoglobulin and cell mediated immunity. We conclude that there may be no need for IgG replacement therapy in hypogammaglobulinemic children with IL who are free of recurrent infections.
494
ANl'I-IGAANFIBoDIEs AND'lHEACCEPTAl'KXOF IGA Janne Bj&-kander, CONIAINIXPLAsMAPHCIXXS. Lennart Eamwx.tr&n, C I &lward Smith, Iars Helin, Gijteborg and Huddinqe, Sweden 'The presence of anti-IgA antibodies in serum has been associated with adverse reactions of the anaphylactoid type against plasma and plasm products in patients with total absence of IgA (IgA < 0.000001 g/l). The frequency of reactions have, however, been very low and unpredictable. Because of this we wanted to correlate the number of cxPo.sures to IgAand the findings of anti-IgA antjtxxlies. We investigated 54 patients with IgA deficiency and 7 patients with IgA + IgG subclassdeficiency with special regard to their haemaglutinin titres to anti-IgA-antibodies and their previous exposure to IgA containing blcxd or plasma. prcducts. Seventeen of the patients had not received blood or plasm products, 10 of these had nc anti-Igi+antibodies, 3 sh0+& law, 6 moderate and 1 high titres. Forty-two patients had received bleed or plasm proauCts and 17 of these had nc anti-IgA-antibodies, 10 showed law, 12 moderate and 3 high titres. There was nc statistical significance between the groups, nor was there any statistical correlation betwzen the n&r of pla5na infusions/injections and the titres of anti-IgA-antibodies. Thus - further studies are needed to understand the occurrence of anti-IgA antibodies.
263
IMMUNOGLOBULINVALUES IN CHILDREN ON CANCER CHEMOTHERAPY. M.H. Anderson, M.D., and M.F. Guill, M.D. Augusta, Ga. We orosoectivelv evaluated immunoalobulin (Ia)-. _ . values in 11 children with cancer (4 mos-13 yrs at diagnosis; 7 males): 7 with acute lymphoblastic leukemia (ALL) and 4 with solid tumors. Values were obtained at diagnosis, after induction phase of chemotherapy, and 6 months later. For the group as a whole, Ig values were decreased at each sampling point, except for IgA, The ALL patients had a which remained stable. greater decrease in all values at all points, and the mean for all Ig was below the normal range 6 months after completion of induction. The solid tumor group had a less marked decrease, and all mean values were within the normal range at 6 months. There was no correlation between the mean Ig values and number of episodes of infection in either group. Of the 3 patients with? 50% decrease in IgG, 2 (ALL) had few infection episodes; in the one patient (solid tumor) with 8 febrile episodes over a one year period, the relationship of illness to either Ig values or absolute granulocyte count is unclear. In some patients both with ALL and solid tumor there is a marked decrease in Ig values during chemotherapy. The clinical significance of these changes is not entirely clear. IgG/A/M
Leukemia
at dx p induc. 6 mos.
1141/96/150 817/70/105 6531731
54
Solid
tumor
1228/109/163 969/119/ 53 1028/118/ 90
Total 1172/100/144 8721 851 90 8031 91/ 69
496H S DEFICColumbus, OH A 15 year old male patient is described with a unique inmunoglobulin deficiency Clinical features include low birth profile. weight and failure to thrive followed by recurrent otitis media, pneumonia, recurrent varicclla and herpes zoster. Scheduled DPT, KKR and oral polio vaccination was uneventful. The patient is not sexually active and he has not received blood transfusions. One male sibling, 14 months younger, is well. Hematologic, liver and renal function is normal. Quantitative immunoglobulin levels are: IIZM IeG Itotal) IJ@ Patient <6 mg/dl 35 mg/dl 695 mg/dl Normal 150-350 50-150 600-1200 Patient 7.2 Normal 4.2-12.9 1.2-7.5 0.4-1.3 0.01-2.9 Anti-IgA antibody was absent. DPT vaccination was repeated with no antibody response after 6 weeks to tetanus antigen but an adequate antibody response to diphtheria antigen (0.48 au/ml). Intravenous immunoglobulin therapy has begun in an attempt to correct this immunodeficiency.
493
Abstracts
87 1. PART 2
DO PATIENTS WITH INTESTINAL LYMPHANGIECTASIA NEED IMMUNOGLOBULIN REPLACEMENTTHERAPY? IMMUNE EVALUATION OF TWO CHILDREN. S.E. Pacheco. MD. B Reid. MD. H.M. Rosenblatt. MD. C.G Hanson. MD: and W.T. Shearer. MD. PhD., Houston', Texas. Because of the profound degree of hypogammaglobulinemia in primary intestinal lymphangiectasia (IL) concern has arisen for IgG replacement therapy. Limited information exists specific responses ' humoral ' iidiatric patients with I'L?& patients (#l’t y/o and X2 10 y/o) had no history of frequent infections yet were hypogamnaglobulinemic. Both were anergic and had decreased lymphocyte responses to PHA, ConA and PWM (PtO.OO1). Lymphocyte subpopulation studies in patient #2 revealed decreased numbers of CD3 cells with low percentages of CD4 and CD8 cells and increased percentages of 8 cells (CD19,CD20) (Pt0.05). Patient # 2 also had decreased lymphocyte and tetanus responses to Candida, streptokinase antiaens (P
495
These results suaaest a relatiie oreservation of B cell itmounity- despite intestinal loss of impaired immunoglobulin and cell mediated immunity. We conclude that there may be no need for IgG replacement therapy in hypogammaglobulinemic children with IL who are free of recurrent infections.
494
ANl'I-IGAANFIBoDIEs AND'lHEACCEPTAl'KXOF IGA Janne Bj&-kander, CONIAINIXPLAsMAPHCIXXS. Lennart Eamwx.tr&n, C I &lward Smith, Iars Helin, Gijteborg and Huddinqe, Sweden 'The presence of anti-IgA antibodies in serum has been associated with adverse reactions of the anaphylactoid type against plasma and plasm products in patients with total absence of IgA (IgA < 0.000001 g/l). The frequency of reactions have, however, been very low and unpredictable. Because of this we wanted to correlate the number of cxPo.sures to IgAand the findings of anti-IgA antjtxxlies. We investigated 54 patients with IgA deficiency and 7 patients with IgA + IgG subclassdeficiency with special regard to their haemaglutinin titres to anti-IgA-antibodies and their previous exposure to IgA containing blcxd or plasma. prcducts. Seventeen of the patients had not received blood or plasm products, 10 of these had nc anti-Igi+antibodies, 3 sh0+& law, 6 moderate and 1 high titres. Forty-two patients had received bleed or plasm proauCts and 17 of these had nc anti-IgA-antibodies, 10 showed law, 12 moderate and 3 high titres. There was nc statistical significance between the groups, nor was there any statistical correlation betwzen the n&r of pla5na infusions/injections and the titres of anti-IgA-antibodies. Thus - further studies are needed to understand the occurrence of anti-IgA antibodies.
263
IMMUNOGLOBULINVALUES IN CHILDREN ON CANCER CHEMOTHERAPY. M.H. Anderson, M.D., and M.F. Guill, M.D. Augusta, Ga. We orosoectivelv evaluated immunoalobulin (Ia)-. _ . values in 11 children with cancer (4 mos-13 yrs at diagnosis; 7 males): 7 with acute lymphoblastic leukemia (ALL) and 4 with solid tumors. Values were obtained at diagnosis, after induction phase of chemotherapy, and 6 months later. For the group as a whole, Ig values were decreased at each sampling point, except for IgA, The ALL patients had a which remained stable. greater decrease in all values at all points, and the mean for all Ig was below the normal range 6 months after completion of induction. The solid tumor group had a less marked decrease, and all mean values were within the normal range at 6 months. There was no correlation between the mean Ig values and number of episodes of infection in either group. Of the 3 patients with? 50% decrease in IgG, 2 (ALL) had few infection episodes; in the one patient (solid tumor) with 8 febrile episodes over a one year period, the relationship of illness to either Ig values or absolute granulocyte count is unclear. In some patients both with ALL and solid tumor there is a marked decrease in Ig values during chemotherapy. The clinical significance of these changes is not entirely clear. IgG/A/M
Leukemia
at dx p induc. 6 mos.
1141/96/150 817/70/105 6531731
54
Solid
tumor
1228/109/163 969/119/ 53 1028/118/ 90
Total 1172/100/144 8721 851 90 8031 91/ 69
496H S DEFICColumbus, OH A 15 year old male patient is described with a unique inmunoglobulin deficiency Clinical features include low birth profile. weight and failure to thrive followed by recurrent otitis media, pneumonia, recurrent varicclla and herpes zoster. Scheduled DPT, KKR and oral polio vaccination was uneventful. The patient is not sexually active and he has not received blood transfusions. One male sibling, 14 months younger, is well. Hematologic, liver and renal function is normal. Quantitative immunoglobulin levels are: IIZM IeG Itotal) IJ@ Patient <6 mg/dl 35 mg/dl 695 mg/dl Normal 150-350 50-150 600-1200 Patient 7.2 Normal 4.2-12.9 1.2-7.5 0.4-1.3 0.01-2.9 Anti-IgA antibody was absent. DPT vaccination was repeated with no antibody response after 6 weeks to tetanus antigen but an adequate antibody response to diphtheria antigen (0.48 au/ml). Intravenous immunoglobulin therapy has begun in an attempt to correct this immunodeficiency.