[CIS-PAGID] low ch50 with normal complement levels?

[Nacho Gonzalez]

Dear Dr. Kumar

The most common complement deficiency is C2def. However, alternative pathway
deficiencies (except for properdin deficiency) usually go with slighly
decrease in CH50 (but not completely absent as in classical pathway
deficiencies).
I suggest checking AP50 also and Factor B,I,D (even D) in case of decreased
AP50 levels. I think it could be interesting for the long-term management.


Luis Ignacio Gonzalez Granado
Immunodeficiencies Unit.
Hospital 12 octubre. Madrid. Spain

2010/11/16 Ashish Kumar <Ashish.Kumar at cchmc.org>


>  Dear Friends,

>

> I recently saw a set of twin girls who were born at 32 weeks with twin-twin

> transfusion syndrome; the smaller of the two has needed a couple

> hospitalizations with URIs due to hypoxia. She has chronic rhinorrhea, a

> history of wheezing that responds to bronchodilator therapy. Someone checked

> her ch50 and it was <10; recheck showed the same. Her twin was then checked

> and hers too was <10. Their complement levels are all normal, except I don't

> have results on C2. They are 18 months old, have normal immune globulins,

> lymphocyte numbers and no serious infections. The smaller twin hasn't needed

> hospitalization since March, even though she has had a couple URIs since

> then - probably because of the season, growth and better asthma control. So,

> they were sent to me for consult because of the low ch50. Since the testing

> is sensitive to sample handling, I thought to repeat it and it is still low.

> I cannot reconcile the history of no serious infections with low ch50 but

> normal complement levels. Is this just a testing aberration? Any

> suggestions/ideas?

>

> Thanks!

> Ashish Kumar

>

>  Ashish Kumar, MD, PhD

> Assistant Professor

> Cincinnati Children's Hospital Medical Center

> Cincinnati, OH

>

>

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