Eltrombopag for the treatment of immune thrombocytopenia: The aegean region of Turkey experience

Objective: Immune thrombocytopenia (ITP) is an immune-mediated disease characterized by transient or persistent decrease of the platelet count to less than 100x109/L. Although it is included in a benign disease group, bleeding complications may be mortal. With a better understanding of the pathophysiology of the disease, thrombopoietin receptor agonists, which came into use in recent years, seem to be an effective option in the treatment of resistant cases. This study aimed to retrospectively assess the efficacy, long-term safety, and tolerability of eltrombopag in Turkish patients with chronic ITP in the Aegean region of Turkey. Materials and Methods: Retrospective data of 40 patients with refractory ITP who were treated with eltrombopag in the Aegean region were examined and evaluated. Results: The total rate of response was 87%, and the median duration of response defined as the number of the platelets being over 50x109/L was 19.5 (interquartile range: 5-60) days. In one patient, venous sinus thrombosis was observed with no other additional risk factors due to or related to thrombosis. Another patient with complete response and irregular follow-up for 12 months was lost due to sudden death as the result of probable acute myocardial infarction. Conclusion: Although the responses to eltrombopag were satisfactory, patients need to be monitored closely for overshooting platelet counts as well as thromboembolic events. © 2015 Turkish Society of Hematology. All rights reserved

BLOOD COAGULATION & FIBRINOLYSIS

In a majority of patients with nonalcoholic fatty liver disease (NAFLD), cardiovascular risk factors including diabetes mellitus type 2, abdominal obesity, and metabolic syndrome are present. The purpose of this study is to investigate the presence of endothelial injury and the changes in coagulation parameters in NAFLD patients. For this purpose, plasma levels of von Willebrand factor (vWF), thrombomodulin, urokinase plasminogen activator (uPA), plasminogen activator inhibitor-1 (PAI-1), and tissue factor pathway inhibitor (TFPI) in patients with NAFLD were measured. There was a significant difference in vWF and TFPI levels between patient and control groups (P 0.05). According to our results, an increase in vWF and TFPI levels indicates an endothelial injury in NAFLD cases

TRANSFUSION AND APHERESIS SCIENCE

Acute lymphoblastic leukemia (ALL) is a malignant disorder resulting from the clonal proliferation of lymphoid precursors with arrested maturation. L-asparaginase is commonly used in combination chemotherapy of both pediatric and adult acute lymphoblastic leukemias. The most commonly encountered side effects of L-asparaginase are hypersensitivity reactions like pyrexia, urticaria, skin rash, and respiratory distress. There are also other side effects like anaphylaxis, coagulopathy, pancreatitis, thrombosis, and hepatic toxicity. Plasmapheresis can sometimes be appropriate to manage an overdose of drugs that circulate in the plasma compartment. We have reported plasmapheresis treatment of fulminant hepatitis in a patient with ALL after L-asparaginase treatment. (C) 2013 Elsevier Ltd. All rights reserved

ENDOCRINE

This study was designed to show the effect of propylthiouracil treatment on sCD40L, high-sensitivity C-reactive protein, and fetuin-A levels on subjects with subclinical hyperthyroidism. After checking sCD40L, high-sensitivity C-reactive protein, and fetuin-A levels of 35 patients with subclinical hyperthyroidism, each was given 50 mg tablets of propylthiouracil three times daily. After 3 months, sCD40L, high-sensitivity C-reactive protein, and fetuin-A levels were then compared to the levels before treatment. Although high-sensitivity C-reactive protein and sCD40L levels were normal in the subclinical hyperthyroidism patients compared to the healthy controls, fetuin-A levels were statistically significantly higher (*p = 0.022). After treatment, fetuin-A levels of subclinical hyperthyroidism patients decreased statistically significantly compared to the levels before treatment (**p = 0.026). sCD40L and high-sensitivity C-reactive protein levels did not have a statistically significant difference compared to the control group and post-propylthiouracil treatment. In subclinical hyperthyroidism patients, high fetuin-A levels before propylthiouracil treatment and decreases in these levels after treatment in cases with subclinical hyperthyroidism indicated the possibility of preventing long-term cardiac complications with propylthiouracil treatment

The levels of adhesion molecules in essential thrombocythemia

Aim. It is known that thrombocytosis is closely related to vascular complications and particularly thrombosis in essential thrombocytemia (ET) cases. The aim of this study is to investigate a possible correlation between adhesion molecules and vascular attacks as well as the relation of these molecules to the platelet count

Is there any relationship between imatinib mesylate medication and hypothalamic-pituitary-adrenal axis dysfunction?

Background: Imatinib mesylate [tyrosine kinase (TK) inhibitor] is a novel medication in the treatment of chronic myelogenous leukaemia (CML). TK is also essential in hypothalamo-pituitary-adrenal (HPA) axis. Purpose: The aim of this study was to evaluate HPA axis in patients treated with imatinib. Twenty-five patients were included in this study. Methods: Glucagon stimulation test (GST) and low-dose (1 mu g) adrenocorticotropin test (LDSST) were used to assess the HPA gland axis. Results: Seventeen (68%) subjects had impaired peak response when a cortisol cut-off value is accepted as 500 nmol/L. Twelve (48%) out of 17 subjects also failed to show a response to LDSST. Therefore, 12 patients (48%) were defined as HPA deficient. Only two of these 25 patients had morning serum cortisol < 200 nmol/l (7.22 mu g/dl), and failed the GST and/or LDSST, indicating that the majority had partial glucocorticoid deficiency. If the cut-off presume for LDSST is from 500 to 600 nmol/l, 16 patients (64%) would have failed both the GST and LDSST. Conclusion: Our results indicate an increased prevalence of subclinical glucocorticoid deficiency in patients receiving imatinib mesylate for CML. Therefore under stressed conditions, such as intercurrent illness state, overt and untreated partial glucocorticoid deficiency in CML patients become life threatening

Does imatinib mesylate therapy cause growth hormone deficiency?

Objective: The purpose of this study was to determine whether or not imatinib mesylate therapy induces growth hormone deficiency (GHD). Subjects and Methods: Seventeen patients with chronic myloid leukemia (CML) were enrolled in the study. The glucagon stimulation test (GST), and standard deviation scores (SDSs) of insulin-like growth factor 1 (IGF-I) and insulin-like growth factor binding protein (IGFBP-3) were used to determine GHD. The L-dopa test was performed on those with IGF-I SDSs above the -1.8 cut-off level. Results: Of the 17 patients in the study, 12 (70%) had severe GHD (serum GH level -1.8 showed insufficient GH response to L-dopa stimulation. Nine subjects (52%) had both severe GHD based on GST response and IGF-I SDS below -1.8. If an IGF-I SDS cut-off value 1<-3 were used, 5 out of 17 subjects (30%) would be classified as GH deficient. These same patients also showed severe GHD based on GST response. Conclusions: The data showed that a large number of patients on imatinib mesylate therapy had GH deficiency. A study involving a larger number of patients with a matched control group is needed to confirm the present observations. Copyright (C) 2009 S. Karger AG, Base

hemoglobinuria

Paroxysmal nocturnal hemoglobinuria (PNH) is a rare, acquired disease caused by clonal expansion of one or more hematopoietic stem cell (HSC) lines due to a somatic mutation of the phosphatidylinositol glycan anchor (PIG-A) gene located on Xp22.1. PNH incidence is 1.5-2 cases per million of the population per year. PNH can affect multiple systems in the body and requires multidisciplinary clinical management. Patients can manifest with severe pancytopenia, life-threatening thrombosis affecting the hepatic, abdominal, cerebral, and subdermal veins, and high requirements for blood transfusion due to haemolytic anemia. PNH can also be associated with bone marrow failure. Advances in diagnostic techniques and a targeted therapeutic approach for PNH have emerged in the last two decades. Eculizumab, a promising humanized monoclonal antibody against C5, is the first approved therapy for PNH