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

Sun Sep 25 19:11:00 EDT 2016

Dear Vignesh,

If he had a biopsy (excisional or core) of an enlarged axillary node that
was negative for malignancy as read by a hematopathologist, I don't think
that mediastinoscopy is necessary.  As I'm sure you know, hepatosplenic T
cell lymphoma is rare in adults and presumably even more rare in children
and it usually doesn't involve lymph nodes.  While lymph nodes in indolent
lymphomas can wax and wane, the persistently enlarged spleen again sways me
away from a malignant diagnosis.  How big were the enlarged nodes?  Did he
have lymphadenopathy above and below the diaphragm?  What is the patient's
LDH?  Is there any clonality in the T cell population in the peripheral
blood?  I assume flow cytometry on the lymph node was negative for
clonality.  His platelets are low, but is his hemoglobin normal?  What
about neutrophils and monocytes?  In other words, is there anything to
suggest bone marrow involvement?

Also, I apologize if I missed this in your summary, but why is his right
middle lobe collapsed?

I agree with pursuing a genetic diagnosis.

Warm regards,
Jennifer

Jennifer Grossman MD, FRCPC
Hematologist
Division of Hematology and Hematologic Malignancies
Alberta Health Services
Calgary, Alberta
Ph: 403-944-1564  Fax: 403-944-2102

On Sun, Sep 25, 2016 at 11:06 PM, CIS-PIDD <cis-pidd at lists.clinimmsoc.org>
wrote:

> Thanks, Dr.Sokolic, Dr.Yu, and Dr.Grimbacher for the comments. Would
> proceed with the mediastinoscopy for a core biopsy to rule out a neoplasm.
> Also, he needs a genetic diagnosis for his condition.
> ICOS expression by flow cytometry in this patient is much lower when
> compared to control. Any significance for this?
>
> 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 Mon, Sep 26, 2016 at 1:52 AM, CIS-PIDD <cis-pidd at lists.clinimmsoc.org>
> wrote:
>
>> Dear Vignesh,
>> Establishing the genetic diagnosis would be key for me:
>> APDS? CTLA4? ALPS? LRBA?
>> This might affect your treatment options and prognosis.
>> Best, Bodo
>>
>> ****************************************
>>
>> Univ.-Prof. Dr. med. B. Grimbacher
>>
>>
>>
>> Scientific-Director
>>
>> CCI-Center for Chronic Immunodeficiency
>>
>> UNIVERSITÄTSKLINIKUM FREIBURG
>>
>> Tel.: 0761 270-77731  Fax: -77744
>>
>> Breisacherstraße 115, 79106 Freiburg
>>
>> bodo.grimbacher at uniklinik-freiburg.de
>>
>> www.uniklinik-freiburg.de/cci
>>
>> Von: CIS-PIDD <cis-pidd at lists.clinimmsoc.org>
>> Antworten an: CIS-PIDD <cis-pidd at lyris.dundee.net>
>> Datum: Sunday 25 September 2016 17:19
>> An: CIS-PIDD <cis-pidd at lyris.dundee.net>
>> Betreff: [cis-pidd] Lymphoproliferation and massive splenomegaly in CVID
>>
>> 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
>>
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