[CIS PIDD] [cis-pidd] Opinion on GLILD

[CIS-PIDD]

Dear all,

I would appreciate your input on a case of young man who is 35 years old,
Caucasian, father of two healthy daughters.

He has - recurrent sinopulmonary tract infections for years
             - AIHA off and on 10 years ago but seems to stable now
             - ITP in 2013
             - Pulmonary nodules waxing and waning since 2007
             - Hypothyroidism
             - Cerebellar mass 2015 (pathology - small vessel
angiitis/vasculitis with an associated parenchymal mixed inflammatory
infiltrate, no neoplasm, no virus, fungus, AFB, bacteria or spirochetes but
a single cell positive for EBV) Patient had diplopia with decreased motor
strength of hand initially but gradually improving on his own. Mass still
persists though.
              - Chronic diarrhea, colon biopsy showed acute cryptitis and
crypt abscesses with almost complete absence of plasma cells in colonic
mucosa in 2016. Negative parasites.
              - Splenomegaly
              - IgG 1.2 g/L, IgA < 0.1 g/L, IgM 0.3 g/L -  on SCIG since
2015 and infection free
              - Eczema - intermittent

Genetic testing for CTLA4 (sanger) and LRBA negative. (A panel of gene
testing was done at Dr. Rosenzweig's lab).

 He is quite well in general (only mild DLCO on pulmonary function test)
but the most recent CT showed possible GLILD. Open lung Bx was pursued and
confirmed GLILD.


*Questions *

*1. Should we treat his GLILD? If so, with what?*
*2. Even though we do not know the molecular cause of his phenotypes,
should we do BMT since he is quite well now before he has more
complications? *

Juthaporn Cowan MD, PhD, FRCPC
Assistant Professor
Division of Infectious Diseases, Department of Medicine
Department of Biochemistry, Microbiology, and Immunology
University of Ottawa & The Ottawa Hospital
Associate Scientist
The Ottawa Hospital Research Institute
Tel 613-737-8899 ext 79617
Fax 613-737-8352

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