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

[CIS-PIDD]

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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