Pathology 36
TheOne - 11-21-06 16:21 Bookmark and Share

A 35-year-old male presents to an infectious disease specialist with recurrent infections with encapsulated bacterial organisms. The history indicates that these infections have become apparent in the last 6 months. Laboratory findings indicates that the total immunoglobulin level exceeded 900 mg/dL with a low CH50 (hemolytic complement) level. Which of the following is the correct diagnosis?

A. Acquired hypogammaglobulinemia (common variable hypogammaglobulinemia)
B. C3 deficiency
C. Hyper-IgM syndrome
D. Wiskott-Aldrich syndrome
E. X-linked infantile hypogammaglobulinemia

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#1
Re: Pathology 36
nrp - 11-22-06 06:02

A?

#2
Re: Pathology 36
Youngdoctor - 11-24-06 23:49


A 35-year-old male presents to an infectious disease specialist with recurrent infections with encapsulated bacterial organisms. The history indicates that these infections have become apparent in the last 6 months. Laboratory findings indicates that the total immunoglobulin level exceeded 900 mg/dL with a low CH50 (hemolytic complement) level. Which of the following is the correct diagnosis?

A. Acquired hypogammaglobulinemia (common variable hypogammaglobulinemia)
B. C3 deficiency
C. Hyper-IgM syndrome
D. Wiskott-Aldrich syndrome
E. X-linked infantile hypogammaglobulinemia


I think Wiskott-Aldrich syndrome.I am not sure.It looks like Selective IgA immunodeficincy.It is difficult to vary.

#3
Re: Pathology 36
TheOne - 11-26-06 16:33

Explanation: The correct answer is B. Individuals with C3 deficiency have an increased susceptibility to recurrent bacterial infections, especially with encapsulated bacteria. This susceptibility illustrates the important role of C3 as an opsonin. C3 deficiency is often not detected until later life. Acquired hypogammaglobulinemia, also known as common variable hypogammaglobulinemia, (choice A) is ruled out by the fact that the total immunoglobulin level is greater than 300 mg/dL. In hyper-IgM syndrome (choice C) there is very little, if any, isotype switching, resulting in patients who are IgG and IgA deficient, but synthesize large amounts of polyclonal IgM. These patients are susceptible to pyogenic infections, and thus the disorder is detected very early in life. In Wiskott-Aldrich syndrome (choice D), a combined immunodeficiency syndrome, immune abnormalities are apparent at birth. Patients usually have low IgM levels with elevated levels of serum IgA and IgE; recurrent pyogenic infections, eczema, and thrombocytopenia are characteristic Patients with X-linked infantile hypogammaglobulinemia (choice E) are detected in the first 4-8 months of life and have total immunoglobulin levels less than 200 mg/dL. Recurrent pyogenic infections are characteristic of this disorder.

#4
Re: Pathology 36
galaxy - 05-28-10 02:30

A 35-year-old male presents to an infectious disease specialist with recurrent infections with encapsulated bacterial organisms. The history indicates that these infections have become apparent in the last 6 months. Laboratory findings indicates that the total immunoglobulin level exceeded 900 mg/dL with a low CH50 (hemolytic complement) level. Which of the following is the correct diagnosis?

B. C3 deficiency

#5
Re: Pathology 36
babbu5508 - 06-21-10 15:35

B. C3 deficiency...recurrent pneumococcal infections, total immunoglobulin level exceeded 900 mg/dL with a low CH50 (hemolytic complement) level.

#6
Re: Pathology 36
bingousmle - 06-30-10 21:53

individuals with C3 deficiency have an increased susceptibility to recurrent bacterial infections, especially with encapsulated bacteria.

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