[ relationship between T CD4[+] cells count and IL-17, IL-11 serum level in idiopathic thrombocytopenic purpura]

Immune Thrombocytopenic Purpura [ITP] is an acquired autoimmune disorder characterized by a low platelet count; because of anti platelet auto-antibodies. ITP patients have auto antibodies against platelet antigens. T CD4[+] lymphocytes are effective cells in immune system that has an important role in auto reactive antibody production and class switching. The pathophisiology and mechanism of ITP is complex and unknown. Numerous studies have difference results about role of T cells in ITP patients. T lymphocytes have been characterized to different subsets. To further investigate about the pathogenesis of ITP, we studied the role of T CD4[+] cells and cytokines attributed with platelet count. Therefore, in this research, we evaluated T CD4[+] lymphocytes count and interleukin 17 [IL-17], interleukin 11 [IL-11] levels in ITP in comparison with control. In a case-control study, we have studied 60 patients with ITP and 50 normal individuals as the control group. Peripheral blood mononuclear cells were isolated by ficoll histopaque 1.077. T CD4[+] cells count in ITP patients and control subjects were studied by flow cytometry method and serum interleukin 17 [IL-17], interleukin 11 [IL-11] concentration were measured by enzyme-linked immunosorbent assay [ELISA] test. All data were expressed as mean +/- SD. Differences between means were considered significant at the P<0.05. Tests were performed using SPSS software version 16. This study showed, T CD4+ cells and plasma IL-17 concentration were not significantly different between patients with ITP and the control group. But plasma IL-11 levels were significantly increased in immune thrombocytopenic purpura patients in comparison with controls [P=0.031]. In summary, our study indicated a role of IL-11 in ITP patients, also showed that ITP may not be associated with changes of plasma IL-17 levels and T CD4[+] cells count relative to control population. Therefore, measurement of plasma IL-11 levels may be important criteria in development of ITP. In addition, it is concluded that determination of IL-11 can be a diagnostic marker to recognize thrombocytopenic purpura patients

Imerslund-Gräsbeck Syndrome: report of two cases in a family

Imerslund-Gräsbeck Syndrome is an inherited disorder which present as a megaloblastic anemia followed by selective vitamine B12 deficiency and is associated with mild proteineuria. This rare disease is easily treatable if detected. In this paper two members of a family with Imerslund-Gräsbeck Syndrome are reported

Primary kaposi sarcoma of penis in HIV negative patient

Primary Kaposi sarcoma of penis is very rare. We will introduce a 47 years old male patient referred to our clinic from dermatology service, in this report. The patient suffered from itchy penile papules around coronal region. The lab tests had revealed a negative serology of HIV but tissue PCR was positive for Human Herpesvirus-8 [HHV8]. Histological findings were compatible with Kaposi sarcoma. Primary Kaposi sarcoma of penis is rare but could occur in HIV negative patients.

[Primary Immuno-deficiencies [PID]]

Primary immune-deficiencies [PID] are associated with a wide range of clinical disorders along with variable symptoms. The aim of this study was to evaluate and improve our knowledge regarding PID from patients that were referred to Booali Sina Hospital. We evaluated all of PID's that were referred to Booali Sina Hospital from their data records. Demography, clinical and laboratory data were recorded and then analyzed. In the duration of 3 years, we had 10 patients with PID [7 males and 3 females]. Of these cases, 5 had hum oral [50%], 1case had phagocytic [10%], 3 cases had cellular [30%] and 1 case had hyper IgE syndrome [10%]. Many of them had respiratory and otitis media infections, while a few patients had adenitis, gastroenteritis, liver abscesses, bleedings and malignancy. PID is a diverse disorder that involves different immune systems. Knowledge from patient's clinical symptoms and consideration in their differential diagnosis can be helpful in early diagnosis and an effective treatment

[Wiscott-Aldrich syndrome [WAS]]

Wiscott-Aldrich syndrome [WAS] is an x-linked immune-deficiency disorder associated with eczema, recurrent infections, and increased frequency of autoimmune diseases, malignancy and thrombocytopenia with small size platelet. Our case is an 11 year old from a non- related parent, who was admitted with abdominal pain [hematoma], lower extremities bleeding [ecchymosed] and vomiting. Also, he had many admition cause bleeding in different areas. Lastly, he had retinal bleeding associated with blindness from 1 year ago

Nitroblue tetrazolium test in patients with beta-thalassemia major

To assess the neutrophil function in thalassemia major [TM] patients and compare it with the control group, and to recognize its relevant factors. This was a retrospective cohort study, which was carried out from October 2007 to February 2008 in the Thalassemia Research Center in Boo Ali Sina Hospital in Sari, Mazandaran, north of Iran. The study population consisted of TM patients in Boo Ali Sina Teaching Hospital. The method of sampling in the case group was systematic, and it was target based in the control group. The sample size determined was based on previous studies. Thalassemia major was diagnosed based on hemoglobin electrophoresis [case group]. The control group was their brothers and sisters, who had +/- 5 years of age difference, and were of the same gender as the patients. Data collection was based on interview, investigating demographic characteristics, and also obtaining medical information from the medical records of the patients. The neutrophil function was assessed by performing nitroblue tetrazolium [NBT] reduction test. The test was carried out on both groups, and the data were analyzed by software using SPSS version13.0. In this study, 39 patients and 39 healthy controls were compared. The average age of the patients was 21.6 +/- 5.3 years, and it was 22.4 +/- 5.1 years in healthy controls [p=0.7]. There was a significant correlation between the tests' results, and the patients' age [p=0.008]. The rate of impaired NBT results in the patients was 36%, while it was 10% in controls, which were significantly different. The neutrophil activity based on NBT test was 89.9 +/- 11.6% in the case group, and 93.7 +/- 2.51% in the control group, [p=0.025]. This study indicates that neutrophil activity in thalassemic patients was significantly lower, compared to the normal control group, especially in young patients. Based on the results, evaluation of neutrophil function, and pyogenic infections in TM patients seems necessary

Fournier Gangrene: a series of 12 patients

Fournier gangrene is a rare, rapidly progressive, necrotizing fasciitis of the external genitalia, the perineum, or the abdominal wall that is associated with high morbidity and mortality. In this series, we describe 12 patients with Fournier gangrene who had presented to our medical center. Twelve men had been diagnosed with Fournier gangrene in Shohada-e- Tajrish hospital between March 2002 and September 2005. Their medical records were reviewed and the Fournier Gangrene Severity Index scores before and after the treatment were determined. Fifty percent of the patients were diabetic and their mean age was 58.2 ' 17.8 years. The mean delay between the onset of the disease and the admission was 4.9 days and the mortality rate was 16.6%. The median Fournier Gangrene Severity Index scores before the admission and at the time of discharge were 4.5 [range, 0 to 11] and 0 [range, 0 to 9], respectively [P = .005]. One of the patients who died had the scores of 11 and 9, respectively. Split-thickness skin graft was performed for 5 patients [41.7%]. In Fournier gangrene, a rapid diagnosis and emergent surgical intervention is crucial. The Fournier Gangrene Severity Index seems to be an excellent tool for outcome prediction