[CIS PIDD] [cis-pidd] teenage girl with T-cell lymphopenia, low IgG, and hematuria

[CIS-PIDD]

Hi Saul,

You don’t mention serological tests for SLE (ANF/DS-DNA antibodies, ENA) or complement C3 & C4 levels.
What is her renal function and renal size on ultrasound?
If she has nephritis with an active urinary sediment she needs a renal biopsy. I assume her streptococcal serology (ASOT & DNAse AB’s) results are negative?

Regards,

Stan

Stanley Ress
Emeritus Associate Professor of Medicine, UCT
Specialist physician & Clinical Immunologist
UCT Private Academic hospital,
Anzio Road, Observatory,
Cape Town, 7925
South Africa
TEL:INTERN<tel:INTERN>. + 2721-4421966 or 4421816
FAX:   "    + 2721-(0)865173095
Cell: 0833115482
email: stan.ress at uct.ac.za<mailto:stan.ress at uct.ac.za>




From: CIS-PIDD [mailto:cis-pidd at lists.clinimmsoc.org]
Sent: 09 September 2015 05:14 PM
To: CIS-PIDD
Subject: [cis-pidd] teenage girl with T-cell lymphopenia, low IgG, and hematuria


  Dear professors and colleagues,

   I would appreciate any thoughts on a puzzling case we're following in Mexico:

   She's a 15-year-old girl from rural central Mexico with no known consanguinity.
   She has tolerated live vaccines well, and has no microcephaly, mental retardation or dysmorphic features.

    Started at age 10 with pneumonia.  She was found to have mitral stenosis.
    She then developed gross hematuria and nephrotic range proteinuria, and was diagnosed with glomerulonephritis.
    Workup showed T-cell (CD3+) lymphopenia and low serum IgG (normal IgA and IgM), with normal B-cell numbers (CD19+ 42%).  She had a normal lymphoproliferation assay and antibody response to polysaccharide antigens.

     My differential diagnosis list is very short and doesn't fit.  I was thinking maybe lupus?  But perhaps you've seen a similar patient or can think of a late-onset PID that would fit...?

      Thank you in advance for your time and any advice.

    Best regards,

Saul Lugo.





--
Saúl Oswaldo Lugo Reyes

Investigador en Ciencias Médicas
Unidad de Investigacion en Inmunodeficiencias
9° piso, Torre de Investigacion,
Instituto Nacional de Pediatria
Coyoacan 04530 DF, Mexico
+52(55)10840900 ext. 1866
--------------------------------------
"La penicilline guérit les humains; le vin les rend heureux"
"If penicillin cures sicknesses, Jerez resuscitates dead people". -Sir Alexander Fleming

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