Seval Akpinar

Dorsal sural nerve conduction study in vitamin B12 deficiency with megaloblastic anemia

Abstract  Peripheral neuropathy is frequently observed in B12 deficiency. In spite of this, there is little knowledge about peripheral neuropathy in B12 deficiency because the severity of clinical involvement of the central nervous system clearly outweighs signs and symptoms due to peripheral nervous system involvement. We primarily investigated peripheral neuropathy with dorsal sural conduction study, which is a new method for detection of early peripheral neuropathy, in B12 deficiency with megaloblastic anemia. Conventional nerve conduction studies and tibial sensory‐evoked potential (SEP) recording were also performed. Twenty‐eight B12‐deficient patients (15 male, 13 female, mean age 65.8 years) with megaloblastic anemia and 18 age‐ and sex‐matched controls were included in the study. Although dorsal sural sensory nerve action potentials (SNAPs) were not recorded in 15 (54%) of 28 patients, only 9 (32%) of them were found to have polyneuropathy by conventional conduction studies. Furthermore, patients with dorsal sural SNAP had mean lower amplitude, mean longer latency, and slower velocity response when compared with controls. Twenty patients (71%) were diagnosed as having myelopathy by the combination of tibial SEP and neurological findings. Two patients whose dorsal sural SNAPs were not recorded had normal tibial SEP responses; therefore, these patients were considered to have isolated peripheral neuropathy. As a result, we conclude that dorsal sural nerve conduction study is a reliable method for detection of early peripheral neuropathy in B12 deficiency.

Turkish Chronic Myeloid Leukemia Study: Retrospective Sectional Analysis of CML Patients

Objective: here have been tremendous changes in treatment and follow-up of patients with chronic myeloid leukemia (CML) in the last decade. Especially, regular publication and updating of NCCN and ELN guidelines have provided enermous rationale and base for close monitorization of patients with CML. But, it is stil needed to have registry results retrospectively to evaluate daily CML practices. Materials and Methods: In this article, we have evaluated 1133 patients’ results with CML in terms of demographical features, disease status, response, resistance and use of second-generation TKIs. Results: The response rate has been found relatively high in comparison with previously published articles, and we detected that there was a lack of appropriate and adequate molecular response assessment. Conclusion: We concluded that we need to improve registry systems and increase the availability of molecular response assessment to provide high-quality patient care. Conflict of interest:None declared.

Sticky Platelet Syndrome in Patients with Uninduced Venous Thrombosis

Objective: Sticky platelet syndrome (SPS) is a common autosomal dominant inherited platelet disorder. SPS is characterized by platelet hyperreactivity and is associated with arterial and venous thrombosis. The aim of this study was to determine the role of SPS in patients with uninduced venous thrombosis. Material and Methods: The study included 28 patients (15 male and 13 female) with uninduced venous thrombosis. SPS was defined according to Mammen’s aggregation method, which is described in detail elsewhere. Results: According to the defined ranges for platelet hyperreactivity, 3 (50%) patients, 2 (33%), and 1 (17%) (n =6 [21%]) with a confirmed diagnosis were classified as type II, I, and III SPS, respectively. In 1 patient SPS was the only hereditary abnormality noted. The other 5 patients carried other inherited coagulation defects, in addition to SPS. Conclusion: The present findings indicate that the prevalence of SPS was 21% in the patients with uninduced venous thrombosis. We therefore suggest that SPS should be considered in the differential diagnosis of such cases. Conflict of interest:None declared.

Does defibrotide prophylaxis decrease the risk of acute graft versus host disease following allogeneic hematopoietic cell transplantation

There is some preliminary evidence, that veno-occlusive disease prophylaxis with defibrotide (DF) may also have a role in decreasing risk of acute graft-versus-host disease (aGvHD) by preventing tissue damage. In this study, we aimed to investigate the role of DF prophylaxis on the development of aGvHD at D+180. One hundred ninety-five consecutive adult patients receiving allogeneic HCT were retrospectively evaluated in 3 groups: no DF, DF/post-HCT (DF D+1 to D+14) and DF/pre-HCT (DF for 14 days concurrently with conditioning). The total (p: 0.057) and grades III/IV (p: 0.051) aGvHD rates at D+180 were 46.5%, 40%, 25.5% and 15.5%, 11.2%, 0% in patients on no DF, DF/post-HCT and DF/pre-HCT. DF may have a role in decreasing incidence and severity of aGvHD, especially if used concurrently with conditioning regimen.

A multicenter experience of thrombotic microangiopathies in Turkey: The Turkish Hematology Research and Education Group (ThREG)-TMA01 study

Thrombotic microangiopathies (TMAs) are rare, but life-threatening disorders characterized by microangiopathic hemolytic anemia and thrombocytopenia (MAHAT) associated with multiorgan dysfunction as a result of microvascular thrombosis and tissue ischemia. The differentiation of the etiology is of utmost importance as the pathophysiological basis will dictate the choice of appropriate treatment. We retrospectively evaluated 154 (99 females and 55 males) patients who received therapeutic plasma exchange (TPE) due to a presumptive diagnosis of TMA, who had serum ADAMTS13 activity/anti-ADAMTS13 antibody analysis at the time of hospital admission. The median age of the study cohort was 36 (14-84). 67 (43.5%), 32 (20.8%), 27 (17.5%) and 28 (18.2%) patients were diagnosed as thrombotic thrombocytopenic purpura (TTP), infection/complement-associated hemolytic uremic syndrome (IA/CA-HUS), secondary TMA and TMA-not otherwise specified (TMA-NOS), respectively. Patients received a median of 18 (1-75) plasma volume exchanges for 14 (153) days. 81 (52.6%) patients received concomitant steroid therapy with TPE. Treatment responses could be evaluated in 137 patients. 90 patients (65.7%) achieved clinical remission following TPE, while 47 (34.3%) patients had non-responsive disease. 25 (18.2%) non-responsive patients died during follow-up. Our study present real-life data on the distribution and follow-up of patients with TMAs who were referred to therapeutic apheresis centers for the application of TPE.