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

[CIS-PIDD]

  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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