Fassio warsaw

UniversityofFlorence
ImmunoallergologyUnit, Dept. of Biomedicine
June 9th, 2009
Lack of efficacy of Cyclosporine A in a patient with episodic angioedema and eosinophilia(Gleich’s syndrome)
Fassio F, Matucci A, Vultaggio A, Rossi O, Almerigogna F, Agostini C, LiottaF, Annunziato F, Romagnani S, Maggi E

Clinicalpresentation: Eosinophilia
A 29yo man referstoourUnitforpersistenteosinophilia, up to>18k/mm3
1000-1500/mm3
No target organinvolvement
(skin, airways, cardiovascular, G-I, neurologic)
No geneticabnormalities
(FIP1L1, Bcr/Abl, JAK)

Clinicalpresentation: Angioedema
RecurrentAngioedema, in the last 6 years
No evident trigger: foodintake, drugs, insectstings…
No C1-INH deficiency, either quantitative or functional
Diagnosticworkup: negative

Clinicalevolution: EAE
CCS
AE
After a fewdays in hospital…
5000/mm3
EpisodicAngioedemawithEosinophilia: Gleich’s Syndrome

Diagnosis: Gleich’s Syndrome
RecurrentAngioedema + Eosinophilia
DescribedbyGleich in 1984
Unclearetiology
Verygoodprognosis (no target organinvolvement)
Angioedemaattacks can betreatedwithcorticosteroids…
…but no prophylactictherapy!

TherapeuticApproach: Gleich’s Syndrome
Demonstrationofanaberrant T Lymphocytepopulation
(CD3-CD4+CD5+)
CourseofCyAas a steroid-sparingagent…
Failure!

TherapeuticApproach: Gleich’s Syndrome
Increased production of Th2 cytokines, ↑↑↑ IL-5
Anti IL-5 MoAb
Procedure for compassionate useofMepolizumabcurrentlyongoing…

fil.fassio@tin.it
UniversityofFlorence
ImmunoallergologyUnit, Dept. of Biomedicine
June 9th, 2009
Thank you!

Fassio warsaw

by filfassio on Nov 30, 2010

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