ί-Thalassemia is a genetic disorder affecting approximately 1.5% of men and women worldwide. Many affected individuals die by the age of 30 years as a result of cardiac complications caused by iron overload. Iron overload can lead to increased oxidative stress and iron deposition in vital organs such as the heart, liver, and endocrine glands. In contrast, excessive removal of iron can lead to infection and other complications. Hence, iron hemostasis is essential for the balance of the redox system. An extensive literature search was conducted using PubMed for studies on iron chelation therapy and antioxidant use in ίthalassemia. The search parameters included the period from 1997 to the present along with specific key words. The key words searched included "antioxidants", "iron chelation", and "complications in beta thalassemia". Iron overload in patients with ί-thalassemia should be managed by a combination therapy that includes iron chelation and antioxidants, to better manage thalassemia complications and optimize the benefit/risk ratio of iron chelation therapy. Adjunct therapy with antioxidants such as zinc, vitamin D, CoQ10, and vitamin E should be considered. ί-Thalassemia patients are at risk of increased oxidative stress. Antioxidants in conjunction with chelation therapy would play a key role in restoring the antioxidant / oxidant balance while maintaining iron hemostasis.

Thrombin is a focal player in the coagulation system. Thrombin formation and platelet reactions occur concurrently immediately after a wound happens. Thrombin generation (TG) in platelet-rich plasma, therefore, is a close approximation of an integral function test of the hemostatic-thrombotic mechanism. In clotting blood, a transient wave of thrombin appears after a lag time. Clotting occurs at the start of the wave. The duration of the clotting time reflects the function of the initiation mechanism. The function of the production mechanism that is responsible for the burst is reflected in the area under the TG curve (endogenous thrombin potential, ETP). Clotting time and ETP therefore reflect different mechanisms and may or may not be correlated. Calibrated automated thrombinography (CAT) allows quantitative assessment of the TG curve in platelet-poor as well as platelet-rich plasma. All conditions (congenital, acquired, drug-induced) that increase TG cause a thrombotic tendency. All conditions (congenital, acquired, drug-induced) that decrease TG prevent thrombosis, but in excess, cause bleeding. Diminution of TG is a common denominator of all antithrombotic treatments, including anti-platelet drugs. Hence, the first law of hemostasis and thrombosis is "The more thrombin the less bleeding but the more thrombosis; the less thrombin the more bleeding but the less thrombosis". Procedures to measure TG in whole blood and at the point-of-care are under development.

CD202b (Tie-2) plays an important role in the regulation of angiogenesis, which is critical in the growth of solid tumors and hematologic malignancies. We investigated Tie-2 surface expression in blasts from 70 Egyptian patients with de novo acute myeloid leukemia (AML). We used flow cytometry to correlate Tie-2 expression with clinical and laboratory parameters and evaluated its prognostic value and impact on treatment response and clinical outcome. The study included 70 patients who were classified according to percent Tie-2 expression: group I patients (32, 45.7%), with ≤20% of cells expressing Tie-2; and group II patients (38, 54.3%), with >20% of blasts expressing Tie-2. No statistically significant difference was found between the patient groups with respect to demographic data; organomegaly; therapy response; and all laboratory data, except for CD13 expression, which was higher in group II patients. High Tie-2 surface expression was associated with a higher mortality rate. Tie-2 expression can be used to predict the survival of AML patients.

Background and aim : Cerebral vascular accident (CVA) or stroke is one of the major complications of hemoglobin SS (HgbSS) disease, which has a negative impact on physical and neurocognitive function in children. Overt cerebral infarction usually affects physical and cognitive function, but silent infarction affects neurocognitive function rather than physical function. We aimed to determine whether neurocognitive function was a predictor of silent infarction. Patients and method: A total of 60 children with sickle cell disease (SCD; HgbSS), aged 6-12 years were included in this study. They were subjected to history taking, clinical examination, medical file review, severity score, brain magnetic resonance imaging, and neuropsychological evaluation. The children with a documented history of CVA and those with both clinical and MRI indications of an infarct were classified as having an overt infarct. The children with MRI scans suggestive of infarction without the presence of corresponding clinical findings were classified as having silent infarct. Children with no pathology in medical records or MRI were classified as normal. Results: Central nervous system (CNS) abnormalities were identified on MRI in 33.3% of the children. The highest frequency of damage was in the frontal lobe. In fact, 90% (n = 18) of the CVA and silent group had sustained some type of frontal lobe injury. The overt CVA groups evidenced a greater frequency of symptoms and lower hemoglobin than the silent infarct group or the group without CNS pathology. The children with documented clinical strokes performed more poorly than the studied peers on tasks requiring sustained attention and effort or tasks that were associated with frontal lobe involvement. In the area of attention, children who showed silent strokes on the MRI also showed similar impairments as their peers who had sustained overt strokes. Conclusion: Silent stroke is not an uncommon phenomenon in children with SCD and may lead to neurocognitive impairment; moreover, neuropsychological evaluation is a good predictor. Recommendations: Neuropsychological evaluation should be performed for severe sicklers and those with attention deficit, and the abnormal results should be confirmed by MRI.

Rare inherited clotting factor deficiencies are challenging, presenting significant diagnostic and therapeutic difficulties. The prevalence of these disorders is strongly influenced by a population's racial mix. The clinical presentations of these disorders display great variability in type and severity. This study was a retrospective review of a single center. We reviewed the medical charts of all patients diagnosed with rare clotting factor deficiencies presented at our institute over the span of 25 years (1985-2009). We identified 38 patients (19 males and 19 females). Of these patients, 1 had afibrinogenemia deficiency, and 2, 3, 6, 1, 9, 6, and 9 patients were diagnosed with FII FV, FVII, FX, FXI, FXII, and FXIII deficiency, respectively and 1 patient with combined FV and FVIII deficiency . All patients were diagnosed using standard clotting factor functional assays. Seventeen patients had positive family history for bleeding. Currently, 27 patients remain alive, 2 are dead, and 9 have been lost to follow-up. As expected, all patients with FXII deficiency had no bleeding symptoms; one had deep vein thrombosis and another presented with transient ischemic attack. Interestingly, we found that half the patients with FXII deficiency had skeletal deformities. The diagnosis of rare clotting factor deficiency is not common in our institute. There was delay in diagnosis, which could have been due to lack of awareness and availability of diagnostic tests in small hospitals. The limited exposure to blood products resulted in fewer viral infections. Disability was a common problem in this set of patients.

We sought to elucidate the relationship between vitamin D deficiency rickets and anemia. This hospital-based study enrolled infants with rickets who were seen in a 300-bed children's hospital as outpatients or inpatients. Hematological parameters were studied and all cases were categorized as active or healed, based on radiological evidence. For the purpose of this study, iron-deficiency anemia was defined as hemoglobin (Hb) below 11 g/L and mean corpuscular volume (MCV) below 70. In 95 infants, Hb levels ranged from 5 to 14 g/L and 48% of active rickets cases were considered anemic. Hb levels in active rickets indicated anemia and were significantly lower than in healed subjects (10.48 g/L vs. 11.52 g/L, p = 0.037). MCV was low in the active rickets group. Many recent cases of rickets and anemia were reported as myelofibrosis and responded well to vitamin D and calcium. We conclude that 3 types of anemia are associated with vitamin D deficiency rickets. The most common is iron deficiency anemia due to common nutritional and other factors; the second most common is hemolytic anemia, known as Northern syndrome, that has been reported in Native Americans in Canada. The third is a very rare anemia due to myelofibrosis involving platelets and neutrophils.

We report a 23-year-old woman with sickle cell disease and rheumatic heart disease. She presented with a pulmonary embolism, despite anticoagulation, and unresolving left lower lobe pneumonia. Chest computed tomography showed hilar and mediastinal lymphadenopathy. Transbronchial fine-needle aspiration was consistent with reactive lymphadenopathy; however, Tru-Cut lung biopsy and transesophageal lymph node biopsy led to a diagnosis of lymphoma. We report this case as a rare association between sickle cell disease and non- Hodgkin lymphoma, possibly leading to a hypercoagulable state and recurrent pulmonary emboli. This case highlights the importance of considering hematological malignancies in the differential diagnosis of lymphadenopathy in sickle cell disease and the necessity of obtaining Tru-Cut biopsies in suspected cases of malignancy.