[CIS PIDD] [cis-pidd] Repeated infections in a 55 year old

[Seppänen Mikko]

Most of those infections would be at least as combination atypical for primary antibody defs as well as for adult onset T defs, most findings however make me think of long term use/abuse of steroids (inhaled+p.o.; and yes Munchhausen's springs to mind if the nurse is clever, sorry).

Have You checked for M components, are T cells oligoclonal, is there anything to suggest CLL?

mikko seppänen, FINLAND

________________________________________
Lähettäjä: jag aggarwal [jag.aggarwal at gmail.com]
Lähetetty: 18. tammikuuta 2013 20:47
Vastaanottaja: CIS-PIDD
Aihe: [cis-pidd] Repeated infections in a 55 year old

I have a 55 year old nurse who has had recurrent skin infections,  abscess left groin, recurrent eye infections, UTIs,  Often requires IV antibiotics for teh control of infections.

Significant abnormal findings from her immune evaluation are as follows:

1.    Low IgG  at 599 ( n 700 - 1600 )
2.    Low IgG1 at 321 ( n 422 - 1292 )
3.    Low C1q at 8.2 ( n 11.8 - 24.4 )
4.    Positive to HLA class I and II
5.    Hepatitis screen Non reactive to all the antigens
6.    Lymphocyte response to Tetenus   LOW
7.    Lymphocyte resposne to Candida LOW
8.    Abs count CD8+HLA-DR+Lymphocytes  elevated at 242 ( N upto 117 )
9.    CD4 + lyphocytes elevated at 63%  ( n 30 - 58 % )
10.  Low CD38+ IgM- at 1.2 %  ( N 4.1 - 42 % )
11.  Low absolute count of CD+38 IgM- cells at 2.7 ( N 7 - 153 )
12.  Elevated CD27+ IgM+ IgD- % of CD19+ B cells  ( N 0 - 5.3 % )
13.  elevated CD3, CD4 cells

Normal labs include:  CBC, ANA, RA, TSH, T4, ESR, C3, C4, C1INH, CRYOGLOBULINS, CRP,  AH50, IgA, IgM, Neutrophil Oxidative burst,  Tetenus antibody titres, Lymphocyte response to PWM, PHA,  Staph enterotoxin B, Normal switched memory cells,  Normal CD 8, Cd16. Cd19  B  cells

Patient has had a satisfactory response to Pneumovax 23 vaccination with post vaccination titres being in the satisfactory range for 65% of the serotypes.

Any thoughts on this lab clinical and lab profile ?

Thanks

Jag Aggarwal MD
Kansas City MO

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