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

Sun Sep 25 19:58:46 EDT 2016

Dear Dr. Grossman,

Thanks for the suggestion.
There was anemia with the hemoglobin of 9.1 g/dl (91 g/L). Neutrophil and
lymphocyte counts were essentially normal. A bone marrow examination was
also carried out and it was reported to be normal. No features of
hemolysis/ spherocytosis in the peripheral smear. An HPLC was also carried
out- normal. LDH values were 891 U/L (not a great rise).
We would have a look at flow plots again for double positive T cells and
request for clonality studies in peripheral blood.

Kind 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 Mon, Sep 26, 2016 at 4:41 AM, CIS-PIDD <cis-pidd at lists.clinimmsoc.org>
wrote:

> 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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