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

[CIS-PIDD]

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<mailto:cis-pidd at lists.clinimmsoc.org>>
Antworten an: CIS-PIDD <cis-pidd at lyris.dundee.net<mailto:cis-pidd at lyris.dundee.net>>
Datum: Sunday 25 September 2016 17:19
An: CIS-PIDD <cis-pidd at lyris.dundee.net<mailto: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<http://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<mailto:vigimmc at gmail.com>
Phone no: +91-9592047009, +91-9944547009

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