common variable immunodeficiency (cvid) is one of the primary immunodeficiencies, which usually presents with recurrent bacterial infections, particularly in respiratory and gastrointestinal systems and hypogammaglobulinemia. we present here a case of cvid who was suffering from chronic watery diarrhea since 3 months before admission. past medical history was uneventful about recurrent infectio...

primary immunodeficiencies constitute a group of highly complex congenital disorders most of which are characterized by a very poor prognosis. allogeneic hematopoietic stem cell transplantation (hsct) has become an established curative treatment approach in many of these disorders, which may be permanently corrected. in this presentation basic and practical aspects of hsct are presented, with a...

background: primary cutaneous lymphomas (pcls) represent a heterogeneous group of t- and b-cell lymphomas that present in the skin with no evidence of extracutaneous disease at the time of diagnosis. the aim of this study was to assess and report the epidemiological characteristics of pcls in isfahan, isfahan province, iran — as a main province of iran. materials and methods: a total of 99 pati...

Levels of Kappa-deleting recombination excision circles (KRECs), T-cell receptor excision circles (TRECs), and T-cell repertoire diversity were evaluated in 1038 samples of 124 children with primary immunodeficiency, of whom 102 (54 with severe combined immunodeficiency and 48 with other types of immunodeficiency) underwent hematopoietic stem cell transplantation. Twenty-two not transplanted pa...

epidemiological studies have shown wide geographical and racial variation in the prevalence and patterns of immunodeficiency disorders. to determine the frequency of primary immunodeficiencies (pid) in iran, the iranian primary immunodeficiencies registry (ipidr) was organized in 1999. the diagnosis of immunodeficiency in our patients was based on standard criteria. the patient’s data were extr...

background: frequent infections is among the most frequent clinical dilemmas for primary care physicians. immunodeficiency disorders are a heterogeneous group of illnesses that predispose patients to the recurrent infections, autoimmunity and malignancies. the aim of this study was to evaluate the clinical and laboratory data collected for the final diagnosis of patients referred with recurrent...

Background: Primary antibody deficiency, the most common primary immunodeficiency disorder, represents a heterogeneous spectrum of conditions caused by a defect in any critical stage of B cell development and is characterized by impaired production of normal amounts of antigen-specific antibodies. Objective: This retrospective study aimed at description and analysis of demographic, clinical, im...

background: primary antibody deficiency, the most common primary immunodeficiency disorder, represents a heterogeneous spectrum of conditions caused by a defect in any critical stage of b cell development and is characterized by impaired production of normal amounts of antigen-specific antibodies. objective: this retrospective study aimed at description and analysis of demographic, clinical, im...

severe combined immunodeficiency (scid) represents a rare group of primary immunodeficiency disorders (pids), with known or unknown genetic alterations. here, we report a new interleukin 2 receptor, gamma chain (il-2rg) mutation in an iranian scid newborn.the patient was a 6-day old boy with a family history of pid. the child was screened using a molecular-based analysis for the assessment of t...