A Case of lgG Subclass Deficiency with the Initial Presen tation of Transient Hypogammaimmuno-Globulinemia of Infancy and a Review of IgG Subclass Deficiencies

RA WEE NETTA GUL, MD*, NUALANONG VISITSUNTHORN, MD*, PAKIT VICHYANOND, MD*

Affiliation : * Division of Allergy and Immunology. Department of Pediatrics. Faculty of Medicine Siriraj Hospital, Mahidol Univer sity, Bangkok 10700, Thailand.

AbstractPrimary immunodeficiency diseases are not common in children. The possibility of an immunological defect should be considered in any individual with repeated infections. A definite diagnosis for immodeficiency is sometimes difficult to achieve because of overlapping clinical mani festations. Immunoglobulin subclass deficiency is an immunological deficiency disease with which, one or more IgG subclasses are deficient. T cell immunity is normal. Patients may develop recurrent bacterial and respiratory infections or could remain asymptomatic11).
Objective : The authors report a case of immunoglobulin G subclass deficiency presenting initially as transient hypogammaglobulinemia of infancy
Case Report : A 2 month-old boy presented to Siriraj Hospital with a history of chronic pro tracted diarrhea, disseminated scabies and sepsis. On presentation, he had generalized scaly and maculo papular rash with no palpable lymph nodes. CBC revealed WBC 22,100 cells/cm3 with PMN 42 per cent, lymphocytes 38 per cent, Eosinophils 4 per cent, Basophil 2 per cent and platelets 254,000/cm3• The immunoglobulin levels were as follows: IgG I8I mg/dl, IgA < 6.6 mg/dl, lgM 26.3 mg/dl. Lym phocyte enumerations revealed CD of2,433 cells/cm3 (N I,460-5,I60); CD 4,682 cells/cm3 (N 650- 4 8 2,450); CD l,588 cell/cm3 (N 500-I ,500); CD 230 cell/cm3 (N 573 ± 264). The initial diagnosis was 19 16 X-linked agammaglobulinemia vs common variable immunodeficiency disease. His diarrhea and five courses of sepsis responded well to antibiotics administration and courses of intravenous immunoglobulin (IVIG) replacement. His through lgG became normal at 2 years of age (after I2 months of IVIG). IVIG was stopped and the diagnosis was changed to transient hypogammaglobulinemia of infancy (THI). Nevertheless, during his 4 month follow-up he developed recurrent sinopulmonary infections (i.e, otitis media and pneumonia). Repeated immunoglobulin profile showed IgG I ,200 mg/dl, lgA I35 mg/dl, IgM 26 mg/dl, IgG subclass were IgG I ,030 mg/dl 1 (N 280-830), lgG 30 mg/dl (N 40-2,400), lgG 22 mg/dl (N 6-I30), lgG 3 mg/dl (N 3-I20). A diag 2 3 4 nosis of IgG subclass deficiency presenting early as transient hypogammaglobulinemia of infancy was 2 then made. Treatment with monthly IVIG was reinitiated and the patient is currently doing well.
Conclusion : The authors present a case of IgG subclass deficiency presenting as transient hypogammaglbulinemia of infancy. Follow-up of the immune profile and clinical manifestation is necessary for a definite diagnosis.

Keywords : Immunoglobulin Subclass, Hypogammaglobulinemia

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