Phenotyping and follow up of forty-seven Iranian patients with common variable immunodeficiency

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• 作者：Saba Arshi^a ; Mohammad Nabavi^a ; Mohammad Hasan Bemanian^a ; Ramin Shakeri^b ; Behrang Taghvaei^a ; Babak Ghalebaghi^a ; ^c ; Delara Babaie^d ; Ahmad Bahrami^a ; Morteza Fallahpour^a ; Hossein Esmaeilzadeh^e ; Mahsa Rekabi^a ; Javad Amadian^a ; Narjes Eslami^a ; Sima Shokri^a ; Farhad Jalali^a ; Nadieh Akbarpour^a ; Rasol Molatefi^a ; ^{molatefi.r@tak.iums.ac.ir" class="auth_mail" title="E-mail the corresponding author} ; ^{rmolatefi@yahoo.com" class="auth_mail" title="E-mail the corresponding author} ; Nima Rezaei^f ; ^g ; ^h
• 关键词：Common variable immune deficiency (CVID) ; Follow up ; Consanguinity ; Age ; Iran
• 刊名：Allergologia et Immunopathologia
• 出版年：2016
• 出版时间：May-June 2016
• 年：2016
• 卷：44
• 期：3
• 页码：226-231
• 全文大小：490 K

文摘

Common variable immune deficiency (CVID) is a heterogeneous syndrome with a wide variety of signs and symptoms. This study describes the phenotyping and survival of the CVID patients in the allergy and clinical immunology department of Rasol-E-Akram Hospital of Iran University of Medical Sciences in Tehran.

Method

We retrospectively reviewed hospital files of CVID patients in our department until January 2014. All patients were diagnosed with standard diagnostic criteria of CVID, treated and visited monthly, during the follow-up period. We divided the patients into four phenotypes; infection only, cytopenia, polyclonal lymphocytic infiltration and unexplained enteropathy. The immunologic, demographic and clinical findings in different phenotypes were analysed.

Results

The study included 47 CVID patients with mean age at onset of symptoms and diagnosis of 11.2 and 20.2 years, respectively. Phenotyping of our patients was: only infection (62%), cytopenia (26%) and PLI (19%) and 94% of cases had only one phenotype. We did not find a significant relation between the clinical phenotypes and immunologic or demographic data. Rate of parental consanguinity in our cases was 47%. Parental consanguinity was related to lower age at onset, lower age at diagnosis and higher baseline IgG levels. Patients with malignancy and autoimmunity had significantly higher age at onset. Our patients were followed-up for 6.9 years and the mortality rate during this time was 6%.

Conclusions

Parental consanguinity and age at onset of CVID symptoms may have important roles in CVID manifestations.