Hepatitis B, Hepatitis C, Human immunodeficiency virus and syphilis frequency among blood donors: A single center study.

We aimed to provide updated results for seroprevalence of hepatitis B, hepatitis C viruses while presenting first data for human immunodeficiency virus and syphilis seropositivity amongst blood donors in Adana, Turkey. Screening and confirmatory test results of 62,461 donors were evaluated. HBsAg, anti-HCV, anti-HIV1/2 and syphilis seropositivity was 1.92%, 0.48%, 0.20%, 0.18% respectively, based on screening tests, and 1.66%, 0.05%, 0.003%, 0.10% respectively, according to confirmatory tests. Transfusion-transmitted infections (TTI) was more prevalent in low-educated donors. HBsAg and syphilis seropositivity rates were higher in married subjects. We found that the prevalence of HBV and HCV was significantly decreased in the last two decades in Adana. Importantly, this study provides first data in HIV and syphilis seropositivity rates among blood donors in our region and both HIV and syphilis seroprevalences were found to be low compared to many regions of Turkey. However, considering the fact that increasing number of immigrants may change prevalences and trends of TTI both in Adana and in Turkey, strict monitorization and yearly reporting of TTI rates seem necessary to be able to take proactive measures.

ß-Globin chain abnormalities with coexisting α-thalassemia mutations.

INTRODUCTION: The frequency of hemoglobinopathies is still high in Adana, the biggest city of the Cukurova Region that is located in the southern part of Turkey. Our aim was to identify the concomitant mutations in α- and ß-globin genes which lead to complex hemoglobinopathies and to establish an appropriate plan of action for each subject, particularly when prenatal diagnosis is necessary. MATERIAL AND METHODS: We studied the association between the ß-globin gene and α-thalassemia genotypes. The reverse hybridization technique was employed to perform molecular analysis, and the results were confirmed by amplification refractory mutation system (ARMS) or restriction fragment length polymorphism (RFLP) technique. RESULTS: We evaluated 36 adult subjects (28 female and 8 male; age range: 18-52 years) with concomitant mutations in their α- and ß-globin genes. The -α(3.7)/αα deletion was the commonest defect in the α-chain as expected, followed by α(3.7)/-α(3.7) deletion. Twenty-five of 36 cases were sickle cell trait with coexisting α-thalassemia, while seven Hb S/S patients had concurrent mutations in their α-genes. The coexistence of α(PolyA-2)α/αα with Hb A/D and with Hb S/D, which is very uncommon, was also detected. There was a subject with compound heterozygosity for ß-globin chain (-α(3.7)/αα with IVSI.110/S), and also a case who had -α(3.7)/αα deletion with IVSI.110/A. CONCLUSIONS: Although limited, our data suggest that it would be valuable to study coexisting α-globin mutations in subjects with sickle cell disease or ß-thalassemia trait during the screening programs for premarital couples, especially in populations with a high frequency of hemoglobinopathies.

ß-Thalassemia mutations and hemoglobinopathies in Adana, Turkey: results from a single center study.

INTRODUCTION: ß-Thalassemia and hemoglobinopathies are common genetic disorders in Turkey and in this retrospective study our aim was to determine the frequency of ß-thalassemia and hemoglobinopathies in Adana, which is one of the biggest cities located in the southern part of Turkey. MATERIAL AND METHODS: Data from 3000 individuals admitted to Seyhan Hereditary Blood Disorders Center in Adana were evaluated. The blood samples were collected into EDTA-containing tubes and hematological parameters were analyzed using an automatic cell counter. High performance liquid chromatography technique was used to determine the type of hemoglobin. Molecular screening of the ß-globin gene was performed with ß-Globin StripAssay. RESULTS: Of 3000 cases, 609 were diagnosed as ß-thalassemia or hemoglobinopathy. We have found that the rates of occurrence of ß-thalassemia and hemoglobinopathies are 13.46% and 6.83% respectively in this area. We have identified 18 different ß-thalassemia mutations and three separate abnormal hemoglobins: HbS, HbD Los Angeles, and HbE. In molecular analyses, ß-thalassemia gene mutations of IVSI.110 (G > A), codon 8 (-AA), IVSI.1 (G > A), IVSI.6 (T > C), -30 (T > A), IVSII.1 (G > A), codon 39 (C > T), codon 44 (-C), IVSI.5 (G > C), codon 5 (-CT), codon 8/9 (+G), IVSII.745 (C > G), codon 22 (7bp del), -101(C > T), codon 36/37 (-T), IVSI.15 (T > G), codon 6 (-A), -88 (G > A) were detected. CONCLUSIONS: Considering the high incidence of mutations that we have found, ß-thalassemia and hemoglobinopathies still seem to be a public health problem in Adana.

Molecular characterization of alpha-Thalassemia in Adana, Turkey: A single center study.

BACKGROUND/AIM: alpha-Thalassemia (alpha-thal) is a widespread genetic disorder throughout the world caused primarily by reduced synthesis of the alpha-globin chains, and it has been found at a high incidence in Turkey. Our aim in this study was to determine the frequency and molecular properties of alpha-thal in Adana, Turkey. MATERIAL AND METHODS: A total of 3,000 individuals comprising premarital couples or patients with anemia were screened between 2007 and 2008. Hematological parameters were analyzed using an automatic cell counter, and to detect the carriers of hemoglobin variants, high-performance liquid chromatography was used. Molecular screening of the alpha-globin gene was carried out by an Alpha-Globin StripAssay which is based on multiplex PCR for specific amplification. RESULTS: We have identified 225 cases with alpha-thal and found that the prevalence of alpha-thal is 7.5% in this area. In molecular analyses, the alpha-thal gene mutations alpha(3.7), alpha(4.2), --(MED), --(20.5), alpha(PA-2)alpha, alphaalphaalpha(anti-3.7), and alpha(PA-1)alpha were detected. CONCLUSION: Our results showed that the alpha-thal mutations represent a great heterogeneity and that the -alpha(3.7) deletion has the highest frequency in Adana.

Plasmapheresis in the treatment of hyperthyroidism associated with agranulocytosis: A case report.

Plasmapheresis, also known as therapeutic plasma exchange, is used in the treatment of several disorders. Temporary improvement after plasmapheresis in cases with thyrotoxicosis has been reported. A 55-year-old woman presented with agranulocytosis induced by propylthiouracil and clinical signs of heart failure. Three sessions of plasmapheresis were performed. We observed an improvement of thyroid hormone levels and clinical findings as well. Plasmapheresis can be an option when drug treatment of thyrotoxicosis fails.

Silicone gel sheet dressing for sclerodermatous type chronic graftversus- host-disease (cGVHD).

Systemic sclerosis is an autoimmune disease characterized by endothelial cell injury, fibroblast activation and immunological aberrations. Generalized form of the disease involves skin and other organs. Progressive sclerodermatous type cGVHD is the difficult type to treat. Immunosuppressors are the most commonly used treatment regimens. Topical silicone gel sheet (SGS) were first used in the treatment of burn wound and following their initial successes have begun to be used in the treatment of hypertrophic scars and keloids. To best of our knowledge, this is the first patient with extensive sclerodermatous type cGVHD in whom SGS was applied on to the skin of the antecubital region. After a six months application of SGS, the skin of this region was remarkably soft and thick compared to other regions of the arm. The result indicate that SGS may be an useful tool for the treatment of extensive sclerodermatous type cGVHD.

Monitoring of peripheral blood CD34+ cell counts on the first day of apheresis is highly predictive for efficient CD34+ cell yield.

The purpose of this study was to evaluate the correlation of preleukapheresis circulating CD 34+ cells/micro L, white blood cells (WBC), and platelet counts on the first day of apheresis with the yield of collected CD 34+ cell counts in 40 patients with hematological malignancies (n = 29) and solid tumors (n = 11). The median numbers of apheresis cycles, numbers of CD 34+ cells, peripheral blood (PB) mononuclear cells, and total nucleated cells collected were 2 (range, 1-4), 5.5 x 106/kg (range, 0.05-33.78), 2.59 x 108/kg (range, 0.04-20.68), and 7.36 x 108/kg (range, 0.15-28.08), respectively. There was a strong correlation between the number of preleukapheresis circulating CD 34+ cells/micro L and the yield of collected CD 34+ cells per kilogram (r = 0.962, p < 0.001). The threshold levels of PB C 34+ cell/micro L to obtain > or =1 x 106/kg and > or =2.5 x 106/kg CD 34+ cell in one collection were 12/micro L and 34/ micro L, respectively. Fifteen of 17 (88%) patients who had > or =34 CD 34+ cells/ micro L in the PB before collection reached the level of > or =2.5 x 106/kg in a single apheresis. Despite a low r value, WBC and platelet counts on the first day of apheresis also correlated with the yield of collected daily CD 34+ cells per kilogram (r = 0.482, p < 0.01 and r = 0.496 p < 0.01, respectively). These data suggest that preleukapheresis circulating CD 34+ cells/ micro L correlated significantly better with the yield of collected CD 34+ cells than WBC and platelet counts on the first day of apheresis. Using a value of 34/micro L preleukapheresis circulating CD 34+ cells as a guide for the timing of peripheral blood stem cells collections can be time saving and cost-effective.

Influence of post-transplant recombinant human granulocyte colony-stimulating factor administration on peritransplant morbidity in patients undergoing autologous stem cell transplantation.

This study evaluated of the effect of post-transplant recombinant human granulocyte colony-stimulating factor (rhG-CSF) administration on the parameters of peritransplant morbidity. Three sequential and consecutive cohorts of 20 patients each received either post-transplant rhG-CSF at a dose of 5 micro g/kg/d i.v. in the morning, starting on d 0, d 5, or no rhG-CSF. Patients who received rhG-CSF starting on d 0 and 5 recovered granulocytes more rapidly than those not receiving rhG-CSF (P < 0.001 for ANC >or= 0.5 and 1 x 10(9)/l). RhG-CSF administration was not significantly associated with more rapid platelet engraftment. RhG-CSF administration starting on d 0 and 5 was significantly associated with a decreased duration of fever (P = 0.002 and 0.001 respectively), antibiotic administration (P < 0.001 and 0.006 respectively) and shorter hospitalization (P < 0.001 and 0.001 respectively) compared with the reference group. There was no difference between the d 0 and d 5 arms regarding the parameters of peritransplant morbidity. In conclusion, rhG-CSF administration was associated with a faster granulocyte recovery, shorter hospitalization, and shorter period of fever and non-prophylactic antibiotic administration. This study also showed that starting rhG-CSF administration on d 5 may be as effective as d 0 on the clinical outcome and may be an economical approach in routine clinical practice in this cost-conscious era.

An unusual presentation of plasma cell dyscrasias: cardiac tamponade due to myelomatous infiltration.

Pericardial involvement, a rare complication of multiple myeloma (MM), is caused by amyloidosis, infections, bleeding abnormalities or plasma cell infiltration, usually at a late or terminal stage of the disease. Three cases of MM with pericardial involvement are reported here and discussed in the light of current literature. In a retrospective review of all patients with MM at two institutions, three cases of pericardial involvement were identified. In one case, we were able to obtain cytospin preparations of the pericardiocentesis fluid. In the remaining two patients, the pericardial biopsy specimen was obtained via a pericardial window. All patients had progressive dyspnea and signs of pericardial tamponade. The pericardiocentesis fluid showed infiltration with plasma cells in one of the three patients, who had a progressive and fatal course. In the second patient pericardial invasion was proven by biopsy and the third was diagnosed with a plasma cell leukemia but developed a pericardial effusion demonstrated by pericardial biopsy. All these three patients died of progressive disease without any response to chemotherapy and supportive measures. In conclusion, optimal treatment for malignant involvement of the pericardium by myeloma cells has not yet been established and is often fatal.