[PAGID] ID diagnosis

[Horacio Del Olmo]

Hi I wanna comment this case, a 10 years old girl with systemic  lupus and C2 deficicncy with otitis media, meningitis , neumonias ,and  antiphosfolipid   syndrome background. Nowadays we received her with new neumonia,  tromboflebitis in left leg, fever, malar erythema, atonic seizures.
we used before she come , hydroxycloroquine and GGIV treatment because C2 deficiency, but we consider now ;we have autoimmune activation and infection, what do you think about Rituximab , GGIV and plasma transfusion in this case??? Is it effective? is it useful? any comment? any question? I appreciate your help, thank you

----- Mensaje original ----
De: Bodo Grimbacher <bodo.grimbacher at uniklinik-freiburg.de>
Para: pagid at list.clinimmsoc.org
Enviado: miércoles, 21 de noviembre, 2007 20:42:56
Asunto: Re: [PAGID] ID diagnosis

Javier,
although somewhat invasive, the bone marrow B cell subset as done (and 
published) by e.g. van Dongen in Rotterdam might be reveiling...
Yours, Bodo

> I have seen a 14 month-old girl with history of recurrent upper

 respiratoy infections, with one kidney (the other dysplastic detected in
 utero and not found posnatal), borderline low IgG, normal IgA and IgM, B
 cells 1% of lymphocytes (repeated and confirmed), CD4 65%, CD8 15%, NK
 15%. Total lymphs 3,600/uL. Height 10 percentile, weight 3 percentile.
 Not dysmorphic. She has an aunt with history of having one kidney and
 receiving "immunoglobulin shots", and also she had a splenectomy because
 "it was not working". 

> The father of this child is Caucasian and the mother is from Mexico.

> I am measuring reponse to vaccines and will follow up B cells

> Any suggestion regarding diagnosis is appreciated. Thank you.

> Javier

>

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