Thrombophilia in hemodialysis patients: Transfer to peritoneal dialysis is life saving.

The majority of vascular access thrombosis episodes in hemodialysis patients are due to anatomic abnormalities. Thrombophilias are inherited, acquired or mixed disorders which also predispose to venous thromboembolism. They include protein C, protein S and antithrombin deficiencies, as well as gene mutations for prothrombin and factor V Leiden. The most important of the mixed cases is hyperhomocysteinemia, which includes both a genetic and an acquired substrate. We report two patients undergoing hemodialysis who suffered from multiple thrombotic events, the first due to factor V Leiden heterozygosity and the second because of hyperhomocysteinemia due to homozygosity for MTHFR C677T mutation. As no site for vascular access was left, transfer to peritoneal dialysis for both patients improved solute clearance and quality of life with no additional thrombotic events noted.

Micro-CT-Based Quantification of Extracted Thrombus Burden Characteristics and Association With Angiographic Outcomes in Patients With ST-Elevation Myocardial Infarction: The QUEST-STEMI Study.

Background: Angiographic detection of thrombus in STEMI is associated with adverse outcomes. However, routine thrombus aspiration failed to demonstrate the anticipated benefit. Hence, management of high coronary thrombus burden remains challenging. We sought to assess for the first time extracted thrombotic material characteristics utilizing micro-computed tomography (micro-CT). Methods: One hundred thirteen STEMI patients undergoing thrombus aspiration were enrolled. Micro-CT was undertaken to quantify retrieved thrombus volume, surface, and density. Correlation of these indices with angiographic and electrocardiographic outcomes was performed. Results: Mean aspirated thrombus volume, surface, and density (±standard deviation) were 15.71 ± 20.10 mm3, 302.89 ± 692.54 mm2, and 3139.04 ± 901.88 Hounsfield units, respectively. Aspirated volume and surface were significantly higher (p < 0.001) in patients with higher angiographic thrombus burden. After multivariable analysis, independent predictors for thrombus volume were reference vessel diameter (RVD) (p = 0.011), right coronary artery (RCA) (p = 0.039), and smoking (p = 0.027), whereas RVD (p = 0.018) and RCA (p = 0.019) were predictive for thrombus surface. Thrombus volume and surface were independently associated with distal embolization (p = 0.007 and p = 0.028, respectively), no-reflow phenomenon (p = 0.002 and p = 0.006, respectively), and angiographically evident residual thrombus (p = 0.007 and p = 0.002, respectively). Higher thrombus density was correlated with worse pre-procedural TIMI flow (p < 0.001). Patients with higher aspirated volume and surface developed less ST resolution (p = 0.042 and p = 0.023, respectively). Conclusions: Angiographic outcomes linked with worse prognosis were more frequent among patients with larger extracted thrombus. Despite retrieving larger thrombus load in these patients, current thrombectomy devices fail to deal with thrombotic material adequately. Further studies of novel thrombus aspiration technologies are warranted to improve patient outcomes. Clinical Trial Registration: QUEST-STEMI trial ClinicalTrials.gov number: NCT03429608 Date of registration: February 12, 2018. The study was prospectively registered.

Platelets transfusion in Greece: Where, when, why? A national survey.

BACKGROUND: Platelet transfusion is among the most useful therapeutic tools in modern clinical settings which mean that ensuring an adequate supply is of paramount importance. AIM: The aim of our study was to record the use and wastage of platelet concentrates (PCs) in Greece, so as to come up with evidence-based interventions. METHODS: The study was conducted during May and June 2015. We evaluated the use of random-donor platelets (RDPs) and single-donor apheresis platelets (SDPs). We analyzed such parameters as hospital department and diagnosis, indication for transfusion, PCs' age at the time of transfusion, and wastage rate. RESULTS: We used data from 21 hospitals across the country. A total of 12,061 RDPs and 1189 SDPs were transfused, with an average of 4.84 (±2.72) and 1.12 (±2.73) units per episode, respectively. Most patients had been admitted to the internal medicine and hematology departments. The transfusions were mostly given prophylactically, usually in cases of acute leukemia, and mostly on the day before expiration. Wastage rate was 16.75% for RPDs and 2.70% for SDPs, primarily because of the expiration of the use-by date. CONCLUSIONS: This is the first national survey regarding platelet transfusion in Greece. Since most patients were admitted in internal medicine and hematology departments, we recommend that the staff of the abovementioned departments should undergo training on contemporary transfusion guidelines. Platelet discard rate could further be lowered through the centralization of inventory management along with the extension of the lifetime of PCs by means of emerging technologies.

Increased values of mean platelet volume and platelet size deviation width may provide a safe positive diagnosis of idiopathic thrombocytopenic purpura.

INTRODUCTION: It has been shown recently that platelet indices like mean platelet volume (MPV), platelet size deviation width (PDW) and platelet-to-large-cell ratio (P-LCR) are helpful in the discrimination between hyperdestructive thrombocytopenia like idiopathic thrombocytopenic purpura (ITP) and hypoproductive thrombocytopenia (HT). The aim of the study is to assess the reliability of these indices in the differentiation of ITP from other thrombocytopenias. METHODS: We recruited 134 thrombocytopenic patients (69 men, 65 women) who were divided into two groups according to the underlying disease: group I (n = 63) included ITP patients, whereas group II (n = 71) included patients with HT due to myelosuppression secondary to chemotherapy for hematological malignancies. Platelet indices were derived from a Sysmex automated cell counter. Sensitivity, specificity, positive prognostic value, negative prognostic value, efficiency and Youden index were calculated. RESULTS: Concerning MPV and PDW indices, sensitivity, specificity, positive prognostic value, negative prognostic value, efficiency and Youden index were 100% for the diagnosis of ITP. On the contrary, the values for P-LCR were significantly lower. CONCLUSIONS: MPV and PDW can be safely relied on for a positive diagnosis of ITP. MPV and PDW were superior to P-LCR.

Defective platelet aggregation in myelodysplastic syndromes.

INTRODUCTION: Hemorrhagic tendency in patients with myelodysplastic syndrome (MDS) is mainly attributed to thrombocytopenia. However, platelet function in these patients has not been thoroughly investigated. AIM: The aim of our study is to evaluate platelet function in patients with primary MDS. METHODS: Platelet function was studied with aggregometry in response to ristocetin, collagen, ADP and adrenaline in 26 MDS patients and 15 healthy individuals. RESULTS: Aggregation was defective in 21 patients (80.7%). Adrenaline was the agonist with the most profound defect (45.9%), followed by ADP (58.7%), whereas aggregation with ristocetin and collagen was at the borderline. Abnormal aggregation to all four agonists was detected in 6 patients (23%). On the contrary, aggregation results were normal in only 5 patients (19.2%). RAEB-t (refractory anemia with excess blasts in transformation) patients were most seriously affected. CONCLUSIONS: MDS patients have impaired platelet aggregation in response to one or more stimulants. Platelet aggregation was not statistically different between MDS patients at early stages of the disease (<12 months) and those at later stages (>12 months). Defective platelet aggregation is strongly related to MDS of worse prognosis. None of our patients was detected to have hyperfunctional platelets, defined as platelets aggregating spontaneously. Functional defects in MDS do not elicit hemorrhagic tendency.

Elevated plasma levels of miR-29a are associated with hemolysis in patients with hypertrophic cardiomyopathy.

BACKGROUND: miR-29a is a small non-coding RNA that is known to repress collagen synthesis. Interestingly, elevated plasma miR-29a was reported to correlate with pronounced myocardial fibrosis in patients with hypertrophic cardiomyopathy. The objective of this study was to elucidate the origin of plasma miR-29a, and evaluate its significance as a biomarker. METHODS: miR-29a expression was evaluated in plasma (n=50) and myocardial samples (n=4) from patients with hypertrophic cardiomyopathy using RT-qPCR. RESULTS: Although miR-29a was highly expressed in the myocardium, miR-29a plasma levels did not show any correlation with serum troponin I levels (rs=-0.12, p=0.43), and the heart does not release significant amounts of miR-29a into the circulation via exosome secretion. Conversely, miR-29a was present in red blood cells, and plasma levels correlated significantly with markers of hemolysis: lactic dehydrogenase (rs=0.36, p=0.01) and the absorbance of oxyhemoglobin at 414nm (rs=0.39, p=0.006). Furthermore, the association between serum haptoglobin and the maximal blood flow velocity in the left ventricle outflow tract (rs=-0.42, p=0.008) indicated that intravascular hemolysis is a manifestation of the disease. CONCLUSIONS: miR-29a is highly expressed in myocardial tissue from patients with hypertrophic cardiomyopathy. In contrast, plasma miR-29a is primarily of nonmyocardial origin and is correlated significantly with the extent of hemolysis observed in these patients.

Differential diagnosis and treatment of primary, cutaneous, anaplastic large cell lymphoma: not always an easy task.

Primary cutaneous anaplastic large cell lymphoma (PC-ALCL) is a rare and distinct neoplasm appearing de novo on the skin. We present a case of a 75-year-old man diagnosed with PC-ALCL in his left femoral region. We describe the morphology of lesions along with the differential diagnosis, treatment, clinical course and prognosis. We further discuss parameters concerning treatment that should be considered when a PC-ALCL is diagnosed. Our case report demonstrates the complexity in classification, staging, differential diagnosis and therapy selection of PC-ALCLs. It is crucial to emphasize the importance of clinical criteria in diagnosing a PC-ALCL in combination with immunohistochemistry.

Increased incidence of iron deficiency anemia secondary to inadequate iron intake in institutionalized, young patients with cerebral palsy.

We observed high incidence of anemia in patients with cerebral palsy sheltered in a specialized institution in Thessaloniki, Greece. Therefore, we decided to investigate its cause. We studied 108 patients, and assessed complete blood cell count, peripheral blood smear, serum iron, ferritin, folate, B12 and the presence of hemoglobin or parasites in the stools. In all cases, anemia was hypochromic and microcytic. Approximately 33% of patients suffered from hypochromic anemia, whereas 38% were iron deficient. There was no statistical difference in the incidence of iron deficiency between different age groups. All tests for fecal occult blood or intestinal parasites were negative. Folic acid and B12 levels were within normal range in all cases. We also found that 87 and 95.6% of patients on liquid diet were anemic and iron deficient, respectively, compared to only 18.8 and 22.3% of patients on normal diet. The high incidence of anemia was attributed to iron deficiency which was secondary to inadequate iron intake and decreased iron absorption. Thus, it would not be irrational to consider iron supplementation as the first measure in such patients and postpone endoscopic procedures for a later stage, unless there are clinical or laboratory findings (such as fecal occult blood) suggestive of gastrointestinal blood loss.

Veno-occlusive disease of the liver during induction therapy for acute lymphoblastic leukemia.

Veno-occlusive disease (VOD) of the liver, also termed as sinusoidal obstruction syndrome, constitutes a well-known complication of high-dose cytoreductive chemotherapy prior to allogeneic or autologous bone marrow transplantation and is associated with the intensity of treatment [1]. On the contrary, there is only one report of VOD during induction therapy for acute lymphoblastic leukemia in a patient with Marfan syndrome, who was successfully treated with defibrotide [2]. In this article, we present the first fatal case of VOD during induction therapy for acute lymphoblastic leukemia.

Haemarthrosis after superwarfarin poisoning.

INTRODUCTION: Superwarfarins are widely used as rodenticides. They are similar to warfarin, but they are more potent and act longer. In case of poisoning, they cause severe bleeding, usually from multiple sites. CASE REPORT: A 67-yr-old man was admitted with melaena, epistaxis and haemarthrosis in his left knee. PT, INR and aPTT were markedly increased. Initially, the patient was treated with blood and fresh frozen plasma (FFP) transfusions. However at the second day, PT, INR and aPTT were even worse. The combination of persistent coagulopathy, normal mixing studies, normal liver function tests and absence of hepatic failure or malabsorption syndromes lead to the suspicion of vitK dependent clotting factors deficiency due to superwarfarin poisoning. Indeed, the patient admitted a suicide attempt with rodenticide, although he had previously denied it. Psychiatric evaluation revealed a disturbed personality. Melaena stopped after 7 d. Then, the patient was administered 30 mg of vitK daily for a total period of 4 months. CONCLUSIONS: Superwarfarin poisoning leads to severe bleeding, usually from multiple sites. Prolonged treatment with high doses of vitK is necessary. Haemarthrosis, as a complication of superwarfarin poisoning, is presented here for the first time in literature.

Discrimination indices as screening tests for beta-thalassemic trait.

The two most frequent microcytic anemias are beta-thalassemic trait (beta-TT) and iron deficiency anemia (IDA). Several discrimination indices have been proposed to distinguish between these two conditions. These indices are derived from several simple red blood cell indices, like red blood cell (RBC) count, mean cell volume, and RBC distribution width (RDW), as these are provided by electronic cell counters. The purpose of the study is to examine the diagnostic accuracy of six discrimination indices in the differentiation between IDA and beta-TT. The six discrimination indices that were examined were as follows: Mentzer Index (MI), Green & King Index (G&K), RDW Index (RDWI), England & Fraser Index (E&F), RDW, and RBC count. We calculated these indices on 373 patients (205 men, 168 women) with beta-TT and 120 patients (50 men, 70 women) with IDA, as well as their sensitivity, specificity, positive and negative prognostic value, efficiency, and Youden's index (YI). G&K shows the highest reliability, followed by E&F, RBC count, MI, and RDWI. On the contrary, RDW completely failed to differentiate between IDA and beta-TT. G&K proved to be the most reliable index as it had the highest sensitivity (75.06%), efficiency (80.12%), and YI (70.86%) for the detection of beta-TT. These six discrimination indices cannot be relied on for a safe differential diagnosis between beta-TT and IDA. They do have high specificity, but their sensitivity for the detection of beta-TT is not satisfactory. Consequently, they cannot be used neither as a screening tool for beta-TT because they could result in a significant number of false negative results.