Pediatric patients with common variable immunodeficiency: long-term follow-up.

BACKGROUND: Common variable immunodeficiency (CVID) is the most common form of symptomatic primary immunodeficiency disease. It is characterized by hypogammaglobulinemia, increased predisposition to infections, autoimmunity, and cancer. OBJECTIVES: This study was performed to evaluate the clinical and immunological features of a group of pediatric patients with CVID. METHODS: The study population comprised 69 individuals with CVID diagnosed during childhood. RESULTS: The patients were followed up for a mean (SD) period of 5.2 (4.3) years. The mean diagnostic delay was 4.4 (3.6) years, which was significantly lower in patients who were diagnosed recently. Children were classified according to 5 clinical phenotypes: infections only (n=39), polyclonal lymphocytic infiltration (n=17), autoimmunity (n=12), malignancy (n=7), and enteropathy (n=3). Postdiagnosis survival (10-year) was 71%. CONCLUSIONS: The high percentages of pediatric patients with CVID in Iran may be due to the considerable prevalence of parental consanguinity in the region and an underlying genetic background.

Adverse reactions of prophylactic intravenous immunoglobulin; a 13-year experience with 3004 infusions in Iranian patients with primary immunodeficiency diseases.

Intravenous immunoglobulin (IVIG) replacement therapy improves health-related quality of life in patients with a primary immunodeficiency disease, although there have been reports of adverse reactions associated with its regular administration. The study population was composed of 99 patients with primary antibody deficiencies. All the patients were diagnosed with a primary immunodeficiency disease and received at least 4 infusions of IVIG at the Children's Medical Center Hospital, Tehran, Iran over a 13-year period (1995-2007). A total of 3004 infusions were recorded, and 216 (7.2%) of these were associated with adverse reactions in 66 patients. Adverse reactions were classified as mild (172 reactions), moderate (41 reactions), and severe (3 reactions). The rate of adverse reaction varied by diagnosis from 3.35% in patients with X-linked agammaglobulinemia to 17.4% in IgG subclass deficiency. There were no age-related differences in the rates of adverse reactions. Adverse reactions to IVIG infusions are occasionally encountered; therefore, physicians and nurses should be aware of these reactions in order to manage and prevent them.

Prediction of response to splenectomy in patients with idiopathic thrombocytopenic purpura.

BACKGROUND: Chronic immune thrombocytopenic purpura (ITP) is an autoimmune disorder characterized by destruction of opsonized platelets in the reticuloendothelial system, particularly the spleen, and by resulting low platelet counts. Splenectomy is the standard second-line treatment for patients with ITP who do not respond to corticosteroids. In the present study we determined predictive factors for the response of patients with ITP to splenectomy. MATERIALS AND METHODS: For thirty-one patients with ITP referred from the Hematology Department of Shariati Hospital, open surgical splenectomy was performed between February 2002 and December 2004. All potentially important variables of response were collected and analyzed to determine predictive parameters. RESULTS: Older patients (>52) responded less positively than younger patients (<52) to splenectomy (p<0.01). Likewise, patients with a sustained remission after splenectomy had a significantly shorter interval of diagnosis of ITP to splenectomy (p<0.05) and shorter duration of corticosteroid therapy before splenectomy (p<0.05). CONCLUSIONS: This study confirms that splenectomy is an effective treatment for patients with ITP. Our findings highlight age as the major predictive factor of long-term response to splenectomy in patients with ITP.

ENT manifestations in Iranian patients with primary antibody deficiencies.

OBJECTIVE: One hundred and nine patients with primary antibody deficiencies were selected in order to determine the frequency of ENT complications. METHOD: Demographic information and ENT medical histories were collected for each patient. Duration of study for each patient was divided into two periods of before diagnosis and after diagnosis and the initiation of treatment. RESULTS: Eighty-two of 109 patients (75.2 per cent) experienced ENT infections during the course of the disease (63: otitis media, 75: sinusitis and nine: mastoiditis). At the time of diagnosis, 52 (47.7 per cent) out of 109 patients presented with an ENT symptom. The frequencies of episodes were 27 for sinusitis and 25 for otitis media (one complicated with mastoiditis). After immunoglobulin replacement therapy the incidence of otitis media was reduced from 1.75 before treatment to 0.39 after treatment per patient per year (p = 0.008). The incidence of sinusitis also significantly decreased from 2.38 to 0.78 (p value = 0.011). CONCLUSION: ENT infections are common medical problems in primary antibody deficiency patients. Persistent and recurrent ENT infections should be suspected as originating from a possible underlying immunodeficiency.

Analysis of class-switched memory B cells in patients with common variable immunodeficiency and its clinical implications.

BACKGROUND: Common variable immunodeficiency (CVID) comprises a heterogeneous group of primary immunodeficiency disorders characterized by hypogammaglobulinemia leading to recurrent infections. Some patients with CVID are more susceptible to earlier onset of respiratory disease and bronchiectasis. It has been suggested that memory B cells, characterized by CD27 expression, can be used as a means to classify subsets of CVID patients. OBJECTIVE: The aim of this study was to classify a sample of Iranian patients with CVID by quantification of peripheral blood memory B cells and immature B cells and to assess the relationship between this classification and the clinical characteristics of the patients. METHODS: The study included 29 patients with CVID and 20 healthy controls. Patients were grouped as follows, according to the quantification of peripheral memory B cells: group I had less than 0.4% switched memory B cells (CD27+, immunoglobulin [Ig] M-, IgD-) in peripheral blood lymphocytes (PBL), while in group II switched memory B cells represented more than 0.4% of PBL. Group I patients were further subdivided into groups Ia and Ib according to the proportion of CD21- peripheral B cells. The clinical and laboratory findings for the patients were then compared among the 3 groups. RESULTS: The percentage of switched memory B cells (CD27+IgM-IgD- cells in peripheral B lymphocytes) was markedly reduced in CVID patients compared with controls (P < .001). This percentage was less than 0.4% (group I) in 20 patients (69%) (P < .05). In the remaining 9 patients (group II) and all healthy controls, the percentage was greater than 0.4%. Bronchiectasis was more frequent in group I than group II (P < .05). Following subdivision of group I patients into groups Ia and Ib based on CD21 peripheral B cells, the rate of autoimmunity was found to be much higher in group Ia than group Ib. CONCLUSIONS: CVID patients with reduced numbers of switched memory B cells are more prone to recurrent respiratory infections and development of bronchiectasis, and as such, need more special care than other CVID patients. Thus, classification of CVID patients by assessment of switched memory B cells could help physicians to predict clinical prognosis of these patients.