Süheyl Asma

Prophylactic red blood cell exchange may be beneficial in the management of sickle cell disease in pregnancy

Sickle cell disease (SCD) is associated with chronic hemolysis and painful episodes. Pregnancy accelerates sickle cell complications, including prepartum and postpartum vasoocclusive crisis, pulmonary complications, and preeclampsia or eclampsia. Fetal complications include preterm birth and its associated risks, intrauterine growth restriction, and a high rate of perinatal mortality. The purpose of this study was to evaluate pregnancy outcomes in patients with SCD who underwent planned preventive red blood cell exchange (RBCX).

Beta-thalassemia intermedia associated with moyamoya syndrome.

Moyamoya syndrome (MMS) is a progressive disorder. We report a 19-year-old boy with beta-thalassemia who presented with a left hemiparesis. Brain MRI showed old middle cerebral artery and left frontal subcortical white matter infarcts. Brain magnetic resonance angiography and digital subtraction angiography revealed occlusion of the bilateral internal carotid arteries with a rich network of basal collateral vessels. To our knowledge this is the third report of beta-thalassemia intermedia and MMS, and the first report of a patient in Turkey. It emphasizes the potential for cerebral infarct due to anemia, protein S and thrombocytosis.

Safety, therapeutic effectiveness, and cost of parenteral iron therapy

Patients have to discontinue the use of oral iron therapy due to the development of side effects and lack of long-term adherence to medication for iron deficiency anemia. This study aimed to evaluate the therapeutic effectiveness, safety, and cost of intravenous iron sucrose therapy. The computerized database and medical records of 453 patients diagnosed with iron deficiency anemia who received intravenous iron sucrose therapy for iron deficiency anemia between 2004 and 2008 were reviewed. The improvement of hematologic parameters and cost of therapy were evaluated 4 weeks after therapy. 453 patients (443 females, 10 males; age: 44.2 ± 12.3 years) received iron sucrose therapy. Mean hemoglobin, hematocrit, and mean corpuscular volume values were 8.2 ± 1.4 g/dL, 26.9 ± 3.8%, and 66.1 ± 7.8 fL, respectively, before therapy and 11.5 ± 1.0 g/dL, 35.8 ± 2.5%, 76.5 ± 6.1 fL, respectively, after therapy (P < 0.001). A mean ferritin level of 3.4 ± 2.4 ng/mL before therapy increased to 65.9 ± 40.6 ng/mL after therapy (P < 0.001). All patients responded to intravenous iron therapy (transferrin saturation values of the patients were >50%). The mean cost of therapy was 143.07 ± 29.13 US dollars. The therapy was well tolerated. Although the cost of intravenous iron sucrose therapy may seem high, a lack of adherence to therapy and side effects including gastrointestinal irritation during oral iron therapy were not experienced during intravenous therapy.

The association of pagophagia with Helicobacter pylori infection in patients with iron-deficiency anemia

This study aimed to determine the relationship between pagophagia (compulsive ice eating) and H.pylori infection in patients with iron-deficiency anemia. We identified H.pylori infection using the 13C-urea breath test in 45 patients with iron-deficiency anemia (group 1) and 55 patients with iron-deficiency anemia and pagophagia (group 2). Subgroups for testing oral intestinal iron absorption were randomly assigned from both groups. These subgroups consisted of (a) 10 patients with iron-deficiency anemia, (b) 10 patients with iron-deficiency anemia and pagophagia, (c) 10 patients with iron-deficiency anemia, pagophagia, and H.pylori infection before the eradication of H.pylori and (d) subgroup c after eradication therapy. There was no difference in the rate of H.pylori infection in the iron-deficiency anemia groups, with or without pagophagia. Furthermore, oral intestinal iron absorption was not influenced by pagophagia and/or H.pylori infection. Pagophagia did not increase the risk of H.pylori infection in patients with iron-deficiency anemia. Pagophagia and H.pylori infection do not synergistically affect the development of intestinal iron absorption abnormalities.

Granulocyte-colony stimulating factor administration among hemoglobin S trait donors: A single center experience from the Eastern Mediterranean region

Assessment of Hemoglobin S trait donors has gained importance together with the increased allogeneic peripheral stem cell transplant activity for sickle cell disease in the regions where the disease is prevalent. Outcomes of Granulocyte‐Colony Stimulating Factor (G‐CSF) administration are obscure for hemoglobin S trait donors. This study aims at investigating the incidence of hemoglobin S carrier status and outcomes of G‐CSF administration among donors who live in Eastern Mediterranean region.

Plasma‐exchange treatment for severe carbamazepine intoxication: A case study

Acute poisoning is an important cause of morbidity and mortality during childhood. This manuscript reports the positive outcome of a pediatric case with a history of accidental carbamazepine intake treated using plasma exchange. A 3‐year‐old male presented with severe carbamazepine intoxication. He was comatose and had generalized tonic clonic seizure, ventricular tachycardia, and hypotension. Although he did not respond to classical therapies, we performed two sessions of plasma exchange. The patient recovered rapidly and was discharged from the hospital six days from the time of carbamazepine ingestion with no complication or neurologic impairment. Plasma exchange can be performed safely in very small children, and it might be the first line treatment, particularly for intoxication with drugs that have high plasma‐protein‐binding properties. J. Clin. Apheresis 29:178–180, 2014.

QTc prolongation during peripheral stem cell apheresis in healthy volunteers.

Today, voluntary donation of peripheral blood stem cells by healthy donors for allogeneic hemopoietic cell transplantation is common worldwide. Such donations are associated with small but measurable risks of morbidity and mortality. Most complications are associated with citrate infusion during cell collection. We studied the effects of citrate infusion on the QTc and other vital parameters during and after peripheral stem cell apheresis in volunteers.

Effectiveness of Fludarabine- and Busulfan-Based Conditioning Regimens in Patients With Acute Myeloblastic Leukemia: 8-Year Experience in a Single Center

OBJECTIVE Allogeneic hematopoietic stem cell transplantation (allo-HSCT) is a curative treatment for acute myeloblastic leukemia (AML). Because the conditioning regimen of busulfan plus cyclophosphamide carries significant risks of toxicity, we evaluated the factors affecting survival after fludarabine replacement instead of cyclophosphamide. METHODS The study included 55 patients who underwent allo-HSCT for AML and received busulfan, fludarabine, and antithymocyte globulin (ATG). RESULTS Forty-eight patients received a myeloablative regimen; 7 patients received a reduced-intensity conditioning regimen. The neutrophil and platelet engraftment times were 12 days (range 9 to 20) and 12 days (range 7 to 19), respectively. Graft-vs-host disease (GvHD) developed in 10% and 50% of the patients, respectively. Seven patients received donor lymphocyte infusion. Of them, 5 patients developed grade I or II GvHD, one grade IV GvHD. The median follow-up period was 20.6 months. The predicted progression-free survival (PFS) at 1 and 3 years after transplantation was 78% and 74%, respectively. The overall survival (OS) at 1, 3, and 5 years was 76%, 74%, and 62%, respectively. Treatment-related mortality (infection in 1 patient, GvHD in 2 patients) occurred in 3 patients (5.5%). Multivariate analysis revealed that OS and PFS were not influenced by age, dose of busulfan or ATG, or presence of cytomegalovirus antigenemia. Acute GvHD and pretransplantation minimal residual disease positivity negatively affected the transplant outcome. The presence of active disease at the time of transplantation was found as an independent risk factor for AML. CONCLUSIONS Busulfan- and fludarabine-based conditioning regimens are effective for AML, and have acceptable toxicity, morbidity, and mortality.

Secondary erythrocytosis due to waterpipe smoking: A case report

In recent years, waterpipe smoking has gradually increased in our country, as in the world. Waterpipe smoking is a severe public health problem as cigarette smoking. Although the toxic substances are considered to be softened through passing from water, there is exposure to toxic substances and risk for contamination with some type of severe diseases. In addition, erythropoiesis is known to be induced with increased carboxy hemoglobin amount in blood and related secondary erythrocytosis may develop. Smoking-related tissue hypoxia is among the most common causes of secondary erythrocytosis. Herein, we presented a case that was admitted to family medicine clinic with various symptoms and diagnosed with narghile-related secondary erythocytosis.

Effects of two doses of anti-T lymphocyte globulin-Fresenius given after full-match sibling stem cell transplantation in acute myeloblastic leukemia patients who underwent myeloablative fludarabine/busulfan conditioning

OBJECTIVE/BACKGROUND Anti-T lymphocyte globulin Fresenius (rATG-F; ATG-Fresenius) and antithymocyte globulin (thymoglobulin), which are included in transplant protocols, are used to reduce the risk of chronic graft-versus-host disease (cGVHD) or suppress allograft rejection. Available clinical studies have been conducted in heterogenous patient populations and with different administration protocols including stem cell sources. Additionally, the pharmacokinetics of ATG is variable, and the clinically effective dose of rATG-F, in particular, is not exactly known. The aim of the study was to investigate the clinical outcomes of acute myeloid leukemia (AML) patients who underwent hemopoietic peripheral stem cell transplantation from full-matched sibling donors and given two different doses of r-ATG-F. METHODS This was a single-center, retrospective chart review conducted between July 2005 and July 2016. Sixty-nine consecutive AML patients who underwent transplant with fludarabine- and busulfan-based conditioning were included in the study. Patients in Group 1 received 15 mg/kg body weight rATG-F to 2013 (n = 46), and Group 2 received 30 mg/kg of rATG-F dose begining in 2013 to reduce to cGVHD (n = 23). Cyclosporine and methotrexate were used to treat acute GVHD (aGVHD) prophylaxis. Outcome parameters were compared between the groups. RESULTS Although the recommended dose r-ATG-F had led to a decrease in the cumulative incidence of cGVHD (27 [58.7%] vs. 8 [34.8%]; p = .03), it also increased the infection rate at 1 year (3 [6.5%] vs. 4 [17.4%]; p = .02). The two groups were similar in terms of engraftment time, aGVHD, relapse, nonrelapse mortality, and rATG-F-related toxicity. A Cox regression model revealed that aGVHD III-IV was associated with increased nonrelapse mortality at 1 year (hazard ratio = 18.2; 95% confidence interval, 1.667-199.255; p = <.02). No patients developed rATG-F-related severe adverse events (Common Terminology Criteria grade 4 or 5). CONCLUSION Dose difference of rATG-F did not influence survival parameters; however, increasing the dose to 30 mg/kg seems to be effective for reducing cGVHD with an increase in infection rate requiring close monitoring of infections in AML patients who received myeloablative fludarabine/busulfan conditioning.