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

[CIS-PIDD]

Dear Luis Alberto Pedroza

For waldenstrom you have to check for monoclonality.
I would also carefully screen for lymphoma

Chronic infections, autoimmune disease and especially liver disease can
often display polyclonal elevated IgM. (I've seen some CVID patients with
primary biliary cholangitis and elevated IgM...  CMV and EBV infection..)

Finally... CVID picture with splenomegaly, enteropathy, lymphopenia
(cd4/cd8 ratio?) and elevated IgM I would consider APDS (PIK3CD, PI3KR1,
PTEN)

Boaz Palterer, MD
Clinical immunologist
Dept of Clinical and Experimental Medicine
University of Florence, Italy
Boaz.palterer at unifi.it

On Sep 14, 2017 14:41, "CIS-PIDD" <cis-pidd at lists.clinimmsoc.org> wrote:

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
Diego de Robles y Vía Interoceánica, Quito, Ecuador


------------------------------
  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 <+593%202-297-1700>
Correo: lpedroza at usfq.edu.ec
Diego de Robles y Vía Interoceánica, Quito, Ecuador
http://www.usfq.edu.ec
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