[CIS PIDD] [cis-pidd] AW: Conflicting complement test results

[alex malbran]

Dear Mike,
The story is consistent with a C3 nephritic factor. Check factor B and AP50 again. Do mixing studies with inactivated patient plasma a a control. Best regards,
Alejandro Malbrán


> From: Fabian.Hauck at med.uni-muenchen.de

> To: cis-pidd at lists.clinimmsoc.org

> Date: Tue, 22 Apr 2014 07:16:58 +0200

> Subject: [cis-pidd] AW: Conflicting complement test results

> 

> Dear Mike,

> 

> I agreewith you  that normal CH50 is dependent on C2 function and that normal CH50 argues against C2D. However, complement analysis is interference-pone. You should check in a second lab and if results are still conflicting go for genetics. A lot of C2-deficient patientes have reduced IgG2- and IGg4-subclasses (but this will probably not help establishing the diagnosis).

> 

> Best reards,

> 

> Fabian, Hauner Children Hospital, Munich 

> 

> -----Ursprüngliche Nachricht-----

> Von: Keller, Michael [mailto:MKeller at childrensnational.org] 

> Gesendet: Montag, 21. April 2014 20:50

> An: CIS-PIDD

> Betreff: [cis-pidd] Conflicting complement test results

> 

> Greetings everyone,

> 

> We have an odd case, and would be most grateful for everyone's thoughts.

> 

> Our patient is a 4-year-old boy with history of mastoiditis complicated by an epidural brain abscess and sinus venous thrombosis at 22 months of age.   Surgical cultures grew streptococcus pneumonia.   He had recurrent sinopulmonary infections, beginning in the second year of life, with multiple ear infections prior to the mastoiditis episode.

> 

> He was a full-term infant, born to non-consanguinous parents of Hispanic ancestry.

> There is no family history of immune disorders, autoimmune/rheumatologic disorders, early malignancies, or unexplained deaths or miscarriages.

> 

> He has had all vaccinations without issue to date.

> 

> His workup immediately following the epidural abscess was as follows:

> 

> IgG/IgA/IgM normal

> S. pneumonia titers protective to 8/14 tested Hib titer protective

> C2 level: <10 (normal 22-39 mcg/ml)

> C3 149

> C4 19

> C1q: 1789 units/ml

> CH50 initially <12 (confirmed on repeat blood draw)

> C2 function: 0

> 

> He was started on PenVK prophylaxis after these findings.

> 

> Oddly, when I met him roughly a year later, his labs were as follows:

> 

> CH50: 217 (176-382)

> AH50: 74 (normal 77-159)

> Properdin level: 34.6 (22.3-67.6)

> C2 level: 20.1

> C2 function: 0

> IgG 851 mg/dl , IgA 57, IgM 131

> The commercial TLR assay was normal.

> Lymphocyte flow cytometry was essentially normal: abs CD3 3451, CD3/4: 1906, CD3/8: 1082, CD19 1391, CD16/56(CD3-): 258, CD45RA:CD45RO ratio (within CD4+ Tcells) of 4:1.

> S. pneumonia IgG titers were protective to 6/14 tested serotypes N meningitides IgG titers were 4/4 protective VZV, Measles IgG were both protective

> 

> He's doing fairly on antibiotic prophylaxis, and his only other medical issues are moderate asthma which improved with flovent use and possible developing ADHD.  He is still on PenVK, and we are boosting him with pneumonax given these results.

> 

> I'm a bit puzzled by the conflicting data between the C2 function and the CH50/AH50.    It doesn't make much sense to have relatively intact classical complement activity with absent C2 function.

> 

> Thanks in advance!

> 

> Mike

> 

> ---------------

> Michael D. Keller MD

> Division of Allergy / Immunology

> Children's National Health System

> 111 Michigan Ave NW, Room 1W-314B

> Washington, DC 20010

> Clinic: 202.476.3016

> Office: 202.476.5843

> Fax: 202.476.2280

> www.childrensnational.org

> 

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