[CIS PIDD] [cis-pidd] Lymphoproliferation and massive splenomegaly in CVID

[CIS-PIDD]

*CVID panel for the above patient:*

CD3- 93.1% (52- 78%)
*CD20- 1.15% (13- 27%)*
*CD19- 2.7% (5- 19%)*
CD56/16- 5.5% (5- 30%)
CD4- 91.6% (50- 80%)
CD8- 38.3% (28- 50%)
Class switched B cells- 11.4% (8- 31%)
Naive B cells- 52% (42- 82%)
Marginal zone like B cells- 13.4% (7- 32%)
Class switched memory B cells- 14.3% (8- 31%)
Transitional B cells- 6.3% (0.6- 8%)
Plasmoblasts- 1.7% (0.4- 3%)
Btk expression in monocytes- percentage expression in case: 71.02%;
Control: 84.3%
CD81 expression by flow cytometry in the case- 88.1%
BAFR expression by flow cytometry in the case- 95.6%
*ICOS expression by flow cytometry- percentage expression in case: 28.2%;
control: 70.3%*

1. The patient has low B cells and normal class-switched memory B cells. Is
this seen with the autoimmunity/ lymphoproliferation predominant CVID we
have in this patient? any other monogenic forms of immuno- dysregulation?

2. Would anyone use steroids or other kinds of immunosuppression to reduce
the lymphoproliferation at this point of time?

Thank you very much.

Regards,
Vignesh P

Vignesh P
MD Pediatrics,
DM resident in Pediatric Clinical Immunology and Rheumatology (Jan 2015-
Dec 2017),
Allergy Immunology Unit, Advanced Pediatrics Center,
Postgraduate Institute of Medical Education and Research,
Chandigarh, India. 160012.
E mail: vigimmc at gmail.com
Phone no: +91-9592047009, +91-9944547009

On Sun, Sep 25, 2016 at 8:49 PM, CIS-PIDD <cis-pidd at lists.clinimmsoc.org>
wrote:

> Dear all,
>
> We have an 11-year-old male child admitted under our care with the
> diagnosis of CVID. Need opinions and suggestions for some queries we have
> in the management of this child.
>
> He was incidentally detected to have massive splenomegaly (palpable till
> umbilicus) and generalized lymphadenopathy last year. No significant
> infections.
>
> *Nov' 15-*
> 1. Contrast-enhanced CT chest showed enlarged multiple mediastinal nodes
> with nodular opacities in the right upper lobe and left upper lobe.
> 2. Fine needle aspiration of mediastinal node- intermediate sized atypical
> lymphoid cells that had rim of cytoplasm and round, large nuclei, many
> showing indentations. (? Non-Hodgkin Lymphoma)
> 3. Axillary node biopsy- Reactive lymphoid hyperplasia with paracortical
> T-lymphoid expansion
> 4. IgA- *32 mg/dl (70-400)*, IgG- *216 mg/dl (700-1600)*, IgM- *<25
> (50-180)*
> 5. CD3- 84.5%; CD 19- *2.55%*
> 6. ESR- 09 mm; CRP- 7.2 mg/L; platelets- 1.3 lakhs/ cu.mm
>
> Diagnosed as CVID with atypical lymphoproliferation. On regular monthly
> replacement IVIg therapy.
>
> There was no decrease in spleen size till date. Over time, there was a
> progressive right middle lobe collapse/ consolidation. Splenic aspirate
> yielded predominant lymphocytes and lymphoplasmacytic infiltrates.
>
> *Specific questions:*
>
> There is no progression in spleen size (from Nov'15- Sep'16) and the
> peripheral nodes have actually regressed. Can this be some indolent
> malignancy like hepatosplenic T cell lymphoma? This is thought of as
> there were no features of systemic inflammation (normal ESR and CRP).
>
> Thank you.
> Eagerly awaiting your kind response.
>
> Regards
> Vignesh P
> MD Pediatrics,
> DM resident in Pediatric Clinical Immunology and Rheumatology,
> Allergy Immunology Unit, Advanced Pediatrics Center,
> Postgraduate Institute of Medical Education and Research,
> Chandigarh, India. 160012.
> E mail: vigimmc at gmail.com
> Phone no: +91-9592047009, +91-9944547009
>
> ---
>
> You are currently subscribed to cis-pidd as: vigimmc at gmail.com.
>
> To unsubscribe click here: http://cts.dundee.net/u?id=104900047.
> fc9f4e54cae45cd032b86c49d3dccf32&n=T&l=cis-pidd&o=3859566
>
> (It may be necessary to cut and paste the above URL if the line is broken)
>
> or send a blank email to leave-3859566-104900047.
> fc9f4e54cae45cd032b86c49d3dccf32 at lyris.dundee.net
>

---
You are currently subscribed to cis-pidd as: pagid at list.clinimmsoc.org.
To unsubscribe click here: http://cts.dundee.net/u?id=96396833.5a9591ccd1e327fe6bc4d1543298c482&n=T&l=cis-pidd&o=3859574
or send a blank email to leave-3859574-96396833.5a9591ccd1e327fe6bc4d1543298c482 at lyris.dundee.net
-------------- next part --------------
An HTML attachment was scrubbed...
URL: <https://pairlist7.pair.net/pipermail/pagid/attachments/20160925/f078c962/attachment-0001.html>