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

[CIS-PIDD]

Dear Fellows,
first thanks all of you for your help in this case,

I just got the protein electrophoresis that  seems normal only with a minor increase on the alpha 1 peak.
We also tested the serum Beta 2 micro globulin and is in 4900 ng/ml (normal 600 to 2000 ng/ml)
is this beta 2microglobulin normal for a CVID patient? or AID deficiency? should we looking for a Lymphoma? (so far no evidence of enlarged lymph nodes, only the splenomegaly), the patient still with fatigue and diarrhea as a main features with ocasional respiratory features.

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



________________________________
De: Luis Alberto Pedroza
Enviado: jueves, 14 de septiembre de 2017 7:40
Para: CIS-PIDD
Asunto: 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
Diego de Robles y Vía Interoceánica, Quito, Ecuador
http://www.usfq.edu.ec

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