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

CIS-PIDD cis-pidd at lists.clinimmsoc.org
Thu Sep 14 13:36:12 EDT 2017


Dear Fellows thanks so much for your input respect this case,
Answer your question:

1.       Regard APDS, there are cases with late onset (adult)? We also sent this patient for WES, but the results will be ready in several months.

2.       The Immunoglobulin levels were always tested on the previous day of the IVIG, so we do not know if those values change, what we observed is that IgM increased from 400 to 800 within 2 months.

3.       Currently we do not have clinical evidence of MALT lymphoma. three years ago when she start with the clinical issues, she was screened for several malignancy and was all normal. An endoscopy showed H pylori but without evidence of malignancy. She have splenomegaly but as far as I understand is not massive.

4.       Regard other signs of combined immunodeficiency, she never had any relevant infectious process before the age of 30; but some infections, perhaps could interpreted as opportunist, (viral conjunctivitis, Chronic diarrhea, and a pneumonic process even with the IVIG treatment), unfortunately in the las episped only few times the samples were sent for culture and was always negative. We tested for CD4/CD8, and they are within range although the CD4/CD8 ratio is low (0,8), also we tested CD45 RA/RO on CD4+ and most of the cells are CD45RA. We do not have access to many antibodies so we have limited information regard the cell profile.

5.       We already asked for the protein electrophoresis, and auto antibodies, as well as Cryptosporidium on stools and an abdominal ultrasound.

Thanks so much for your comments,

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


De: cis-pidd at lyris.dundee.net [mailto:cis-pidd at lyris.dundee.net] En nombre de CIS-PIDD
Enviado el: jueves, 14 de septiembre de 2017 10:53
Para: CIS-PIDD <cis-pidd at lyris.dundee.net>
Asunto: Re: [cis-pidd] CVID with 868mg/dL of IgM,

Consider APDS?





-------- Ursprüngliche Nachricht --------
Von: CIS-PIDD <cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>>
Datum: 14.09.17 16:27 (GMT+01:00)
An: CIS-PIDD <cis-pidd at lyris.dundee.net<mailto:cis-pidd at lyris.dundee.net>>
Betreff: Re: [cis-pidd] CVID with 868mg/dL of IgM,

Just some thoughts,

 Check for M component (IEF), consider SCIg, highest IgM levels usually in those CVIDs who have lymphoproliferative phenotype.

During treatment, what are the troughs? Have these been falling?

Any protein losing enteropathy, anything to suggest MALT lymphoma? Masdive splenomegaly?

IgG predg seems a bit too high for AICDA, UNG (marginal zone B cells+, no smB) or (CD40L, but this is female)/CD40, but scatter plots often contain some unspecific scatter, thus if MZB&smB very low, are there any signs of combined immunodeficiency?

ATB,

M

Oyl Mikko Seppänen
Harvinaissairauksien yksikkö (HAKE)

Head, Rare Disease Center,
Helsinki University Hospital (HUH)
FINLAND

phone +358 947180201
GSM +358 50 4279606
fax +358 9 47174703

CIS-PIDD <cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>> kirjoitti 14.9.2017 kello 13.41:
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


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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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