[CIS PIDD] [cis-pidd] Markedly reduced T cells with normal immunoglobulins

[CIS-PIDD]

Absolutely seems like leaky scid.  My only caveat is that sepsis and severe malnutrition can have profound effects on T cell numbers.

Kate Sullivan
Sent from my iPhone

On Sep 11, 2017, at 8:12 AM, CIS-PIDD <cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>> wrote:

Dear all

I need your inputs in the following case......


17 months old boy, unwell for the past 4 months. Born to consanguineously married couple. Other siblings healthy.
Repeated episodes of oral thrush for the past 4 months. Some improvement with application of oral clotrimazole. Poor intake of food and loss of weight (10 kg to 9 kg). Admitted with pneumonia for the past 15 days.

On examination
Oral thrush; features of severe malnutrition (Kwashiorkor)., pedal edema; Chest - bilateral crepitations

Investigations
Anemia
No neutropenia
Lymphocyte counts 3700

Low albumin
Other liver functions, renal functions - normal

HIV - Non reactive

IgG 1640 (High)
IgA   523 (High)
IgM  110
IgE  < 1.5

NBT - normal
DHR - normal

T - 7%
B - 24%
NK - 5 %

Absolute counts
T - 332
B - 1137
NK - 227



Repeat testing after 2 days
T - 11%
B - 31%
NK - 7.7 %

Absolute counts
T - 430
B - 1172
NK - 292


T cells are markedly reduced (repeated twice, 2 days apart). What are the conditions in which T cells are markedly reduced in presence of normal/high IgG?

Is it a variant of SCID?  Leaky SCID?
(The first year of life was uneventful and this, to my knowledge is very odd for SCID)

Does severe malnutrition cause a selective reduction in T cells?

How should I evaluate this child further? Any role of IVIG in this setting?

The sum of % of T, B and NK is around 36% on first occasion and 49 % on the repeat sample ...... What could be the reasons for this? The lab expert feels it could be due to debris (but in this case there was no debris and she thus had no clue as to why the sum was not 100%)

Kindly provide your valuable inputs


Regards
Sagar



Dr.Sagar Bhattad
MD Pediatrics, DM Pediatric Clinical Immunology and Rheumatology (PGI, CHD)
Giannina Gaslini Institute, Italy (Observership in Rheumatology)
Consultant, Pediatric Immunologist and Rheumatologist, ASTER CMI Hospitals, Bengaluru
http://www.pediatricimmunologist.in/
Aster CMI Hospital, No 43/2, International Airport Rd, Sahakara Nagar, Bengaluru -560 092, Karnataka
 (Book appointment - 08043420100)



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