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Case ReportOpen Access
A Case of IgG Subclass Deficiency with the Initial Presentation of Transient Hypogammaimmuno-Giobulinemia of Infancy and a Review of IgG Subclass Deficiencies
Nettagul R 
,
Visitsunthorn N ,
Vichyanond P
,
Visitsunthorn N ,
Vichyanond P RA WEE NETT AGUL, MD*,
NUALANONG VISITSUNTHORN, MD*,
PAKIT VICHYANOND, MD*
Primary 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/cm
3
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
4
of2,433 cells/cm3 (N I,460-5,I60); CD
8
4,682 cells/cm3 (N 650-
2,450); CD
19
l,588 cell/cm3 (N 500-I ,500); CD
16
230 cell/cm3 (N 573
เธ‘
264). The initial diagnosis was
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
1
I ,030 mg/dl
(N 280-830), lgG
2
30 mg/dl (N 40-2,400), lgG
3
22 mg/dl (N 6-I30), lgG
4
3 mg/dl (N 3-I20). A diag-
nosis of IgG
2
subclass deficiency presenting early as transient hypogammaglobulinemia of infancy was
then made. Treatment with monthly IVIG was reinitiated and the patient is currently doing well.
Vol. 86 No.7
IMMUNODEFICIENCY DISEASE
687
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.
Key word : Immunoglobulin Subclass, Hypogammaglobulinemia
NUALANONG VISITSUNTHORN, MD*,
PAKIT VICHYANOND, MD*
Primary 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/cm
3
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
4
of2,433 cells/cm3 (N I,460-5,I60); CD
8
4,682 cells/cm3 (N 650-
2,450); CD
19
l,588 cell/cm3 (N 500-I ,500); CD
16
230 cell/cm3 (N 573
เธ‘
264). The initial diagnosis was
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
1
I ,030 mg/dl
(N 280-830), lgG
2
30 mg/dl (N 40-2,400), lgG
3
22 mg/dl (N 6-I30), lgG
4
3 mg/dl (N 3-I20). A diag-
nosis of IgG
2
subclass deficiency presenting early as transient hypogammaglobulinemia of infancy was
then made. Treatment with monthly IVIG was reinitiated and the patient is currently doing well.
Vol. 86 No.7
IMMUNODEFICIENCY DISEASE
687
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.
Key word : Immunoglobulin Subclass, Hypogammaglobulinemia
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