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

[CIS-PIDD]

I agree with the previous comments. Your most immediate concern should be
the risk of hyperviscosity and thrombosis with the next IVIG treatment.
Knowing about clonality is important. I would also look for rheumatoid
factor (some of these monoclonals are IgM anti-IgG or anti-IgA) that would
further increase the risk. I would also measure serum viscosity. Regardless
of those results, I would switch to SCIG for safety reasons.

She is old for CD40L and young for Waldenstroms but at a prime age for
lymphoma.

Richard Wasserman
Dallas

On Thu, Sep 14, 2017 at 9:24 AM, CIS-PIDD <cis-pidd at lists.clinimmsoc.org>
wrote:

> 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 <+358%209%2047180201>
> GSM +358 50 4279606 <+358%2050%204279606>
> fax +358 9 47174703 <+358%209%2047174703>
>
> CIS-PIDD <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
> 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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-- 
Richard L. Wasserman, MD, PhD
Allergy Partners of North Texas
7777 Forest Lane, Suite B-332
Dallas, Texas 75230
Office (972) 566-7788
Fax (972) 566-8837
Cell (214) 697-7211

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