[PAGID] R: severe lymphopenia and gastroenteritis in an infant: Does it always have to be a SCID ?

[Notarangelo, Luigi]

Interesting case, Jan!! 

A significant barrier problem might lead to lymphocyte loss. Also, there is a tendency towards Th2 skewing in lymphopenic hosts, and indeed eosinophilia is commonly observed in these circumstances. What is more remarkable is that the lymphopenia appeared to be mostly a T'cell lymphopenia, whereas the number of B cells was conserved. Did the infant have a predominant T cell infiltrate in the gut, so that homing of T cells there might account at least in part for the T-cell lymphopenia?
This case also suggests that CFSE staining for proliferation may be more solid in cases like this, as one would be able to get on selected cell populations to look for dye dilution, and this could be done also if the proportion of those cells is low (such as CD4+ T cells in this case). 

Luigi D. Notarangelo
Division of Immunology, Children's Hospital
Harvard Medical School
Boston
tel: (617)-919-2276
FAX: (617)-730-0709
email: luigi.notarangelo at childrens.harvard.edu

-----Messaggio originale-----
Da: pagid-bounces at list.clinimmsoc.org per conto di Dr. med. Jan Rohr
Inviato: dom 09/12/2007 7.15
A: pagid at list.clinimmsoc.org
Oggetto: [PAGID] severe lymphopenia and gastroenteritis in an infant: Does it always have to be a SCID ?
 
Dear colleagues,

A now 15 months old little boy was referred to our Immunology Clinic for 
a second opinion after having been followed in another hospital for 7 
months.

Short summary of the history:
Parents not consanguinous, but from the same village in Serbia. The 
child had no medical problems until the age of 5 months when he 
developed severe gastroenteritis and edema lasting for more than 30 
days. Rota- and Adenovirus were both found in his stool. Blood studies 
showed severe hypoproteinemia (including all Immunglobuline types) as 
well as a profound lymphocytopenia (nadir: total lymphs - 470/µl, CD3+ 
101/µl, 19+ 244, CD4+3+ 36 /µl, CD8+3+ 46, CD16+56+ 50 /µl) - results 
were confirmed on several occasions. Lymphocyte proliferation studies 
showed a severe impaiment (PHA with and without IL-2, ConA, OKT3). A 
diagnosis of SCID was presumed and the patient received prophylactic 
antimicrobials and IgG replacement. Intestinal biopsy showed increased 
numbers of eosinophils in the duodenum, ileum and colon.
He recovered from his enteropathy after about 5 weeks and the lymphocyte 
counts slowly increased again (ALC: 1300/µl). Two weeks after that first 
episode he again suffered from a severe gastroenteritis - findings were 
identical to the above mentioned episode, except that no organisms were 
found. This episod only lasted for two weeks. Again the lymphocyte 
counts slowly rose again (ALC about 1000/µl). Again two weeks later the 
whole family suffered from norovirus-infection. However, our little 
patient had loose stools for only two days, was otherwise well and then 
recovered rapidly.
Now, 3,5 months after that last episode of gastroenteritis, he is 
thriving well, has normal lymphocyte counts, normal counts of the 
different subpopulations of lymphocytes including naive T cells and 
almost normal proliferative response to PHA and Anti-CD3/CD28-stimulation.

My questions is: Can a severe gastroenteritis explain not only the 
hypogammaglobulinemia, but also such a severe loss of lymphocytes ? Is 
there an association of what is called a "eosinophil gastroenteritis" 
with lymphopenia (I was unable to find such an associtation in the 
literature) ?

Best wishes from Germany,

Jan
 

-- 





---------------------------------------------
Dr. med. Jan Rohr
Pediatric Immunology
---------------------------------------------
Centre of Pediatrics and Adolescent Medicine 
Freiburg University Hospital
Mathildenstr. 1
79106 Freiburg
Germany
---------------------------------------------
Tel.:    +49 761-270-4300
E-mail:  Jan.Rohr at uniklinik-freiburg.de
Web:     http://www.uniklinik-freiburg.de



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