[CIS PIDD] [cis-pidd] CVID with 868mg/dL of IgM,

Sat Sep 16 11:26:36 EDT 2017

Dear Bodo,
N,t we do not had excluded AID deficiency, we suspect some type of HIGM (because the constant high IgM), but since the onset of the disease was 30 yo, and there is no evidence of infectious history on her childhood, and no evidence of consanguinity we decanted for CVID diagnosis.
When you said adult female, means late onset of the disease?

Thanks

Luis

De: cis-pidd at lyris.dundee.net [mailto:cis-pidd at lyris.dundee.net] En nombre de CIS-PIDD
Enviado el: sábado, 16 de septiembre de 2017 9:15
Para: CIS-PIDD <cis-pidd at lyris.dundee.net>
Asunto: Re: [cis-pidd] CVID with 868mg/dL of IgM,

Luis,
In addition to APDS you have excluded AID, right?
We have adult female AID patients...
(Of course a genetic approach is probably most revealing.)
Yours, Bodo

****************************************
Univ.-Prof. Dr. med. B. Grimbacher

Scientific-Director
CCI-Center for Chronic Immunodeficiency
UNIVERSITÄTSKLINIKUM FREIBURG
Tel.: 0761 270-77731  Fax: -77744
Breisacherstraße 115, 79106 Freiburg
bodo.grimbacher at uniklinik-freiburg.de<mailto:bodo.grimbacher at uniklinik-freiburg.de>
www.uniklinik-freiburg.de/cci<http://www.uniklinik-freiburg.de/cci>

Von: <cis-pidd at lyris.dundee.net<mailto:cis-pidd at lyris.dundee.net>> on behalf of CIS-PIDD <cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>>
Antworten an: CIS-PIDD <cis-pidd at lyris.dundee.net<mailto:cis-pidd at lyris.dundee.net>>
Datum: Thursday, 14 September 2017 14:40
An: CIS-PIDD <cis-pidd at lyris.dundee.net<mailto:cis-pidd at lyris.dundee.net>>
Betreff: [cis-pidd] CVID with 868mg/dL of IgM,

Dear Fellows,

We have a CVID patient, female 34 years old with a 3 years history of recurrent upper respiratory tract infections, a couple of pneumonias, chronic diarrhea, and splenomegaly. Before that she was normal, and there was not any relevant infection in her childhood, and without family history of infections. The Immunoglobulins were tested several time, with absent IgA, an low IgG (around 300mg/dL to 600 mg/dL on average), nevertheless the IgM was always elevated (between 300 to 400 mg/dL). On the analysis her CD19+ cells were almost all negative for CD27 and positive for IgD.

She start IVIG 400 mg/kg, one year ago and she was doing well until a couple of months ago when she start again with the same infectious pattern, even within the first week after the IVIG administration. Her cell counts are low, but within limits (leukocytes 4200, with 1100 lymphocytes, and 2800 neutrophils and 200 monocytes (round numbers), Hemoglobin, 11,6 and platelets 152000) and this is a constant in all the measurements, even during an infectious process.

In the last Immunoglobulin measure (previous to the IVIG administration), her IgM was in 868 mg/dl, and the night after the IVIG administration  she complain about headache, elevated body temperature, dizziness, and  generalized pain.

My concern is regard the apparently lack of action of the IVIG, any suggestion for a complementary treatment? and second, the raising IgM level, should I concern regard a waldenstrom's macroglobulinemia? Or those levels are within acceptable for a CVID diagnosis? How common is this pattern in CVID?

Thanks for your help

Luis Alberto Pedroza, Ph.D.

Colegio de Ciencias de la Salud, COCSA
Universidad San Francisco de Quito
lpedroza at usfq.edu.ec<mailto:lpedroza at usfq.edu.ec>
Diego de Robles y Vía Interoceánica, Quito, Ecuador

________________________________
[http://www4.usfq.edu.ec/owa/logo_usfq.png]

Luis Alberto Pedroza, Ph.D.
Profesor de Inmunología
Colegio de Ciencias de la Salud, COCSA
Universidad San Francisco de Quito
T: (+593) 2 297-1700 ext. 1783
Correo: lpedroza at usfq.edu.ec<mailto:lpedroza at usfq.edu.ec>
Diego de Robles y Vía Interoceánica, Quito, Ecuador
http://www.usfq.edu.ec

Nota de descargo: La información contenida en éste e-mail es confidencial y sólo puede ser utilizada por el individuo o la institución a la cual está dirigido. Esta información no debe ser distribuida ni copiada total o parcialmente por ningún medio sin la autorización de la USFQ. La institución no asume responsabilidad sobre información, opiniones o criterios contenidos en este mail que no estén relacionados con asuntos oficiales de nuestra institución. Disclaimer: The information in this e-mail is confidential and intended only for the use of the person or institution to which it is addressed. This information is considered provisional and referential; it cannot be totally or partially distributed nor copied by any media without authorization from USFQ. The institution does not assume responsibility about the information, opinions or criteria in this e-mail.

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________________________________
[http://www4.usfq.edu.ec/owa/logo_usfq.png]             Luis Alberto Pedroza, Ph.D.
Profesor de Inmunología
Colegio de Ciencias de la Salud, COCSA
Universidad San Francisco de Quito
T: (+593) 2 297-1700 ext. 1783
Correo: lpedroza at usfq.edu.ec
Diego de Robles y Vía Interoceánica, Quito, Ecuador
http://www.usfq.edu.ec

Nota de descargo: La información contenida en éste e-mail es confidencial y sólo puede ser utilizada por el individuo o la institución a la cual está dirigido. Esta información no debe ser distribuida ni copiada total o parcialmente por ningún medio sin la autorización de la USFQ. La institución no asume responsabilidad sobre información, opiniones o criterios contenidos en este mail que no estén relacionados con asuntos oficiales de nuestra institución. Disclaimer: The information in this e-mail is confidential and intended only for the use of the person or institution to which it is addressed. This information is considered provisional and referential; it cannot be totally or partially distributed nor copied by any media without authorization from USFQ. The institution does not assume responsibility about the information, opinions or criteria in this e-mail.

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