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

[CIS-PIDD]

She could also be losing IgG and T > B lymphocytes in her urine.



On Sep 9, 2015, at 10:27 AM, CIS-PIDD <cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>> wrote:

Lymphopenia is common with SLE, Behcets Sjogrens.

If she has had a lot of steroids, the low IgG could just be from that.

Kate Sullivan, MD PhD
Wallace Chair
Chief of Allergy Immunology
ARC 1216 CHOP
3615 Civic Center Blvd.
Philadelphia, PA 19104
(p) 215-590-1697
(f) 267-426-0363



On Sep 9, 2015, at 11:14 AM, CIS-PIDD <cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>> wrote:


  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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