Hematology Reviews

At present, there are no compounds in clin- ical development in the field of chronic myeloid leukemia (CML) or Philadelphia-posi- tive (Ph+) acute lymphoblastic leukemia (ALL) that have been documented to harbor signifi- cant activity against the imatinib-resistant T315I mutation. [...]

The β-globin gene mutation in sickle cell anemia results in anemia and repeated bouts of vascular occlusion. The cumulative effect of these vasocclusive events is progressive damage to many organs including the kidneys, lungs, and brain. The transfusion of red blood cells (RBC) can ameliorate many of these complications, but can be associated with both acute and chronic complications, including iron overload. The objective of the Best Practices in Transfusion Medicine for Patients with Sickle Cell Disease (SCD) Conference was to review the available published evidence and clinical experience surrounding the use of RBC transfusions for sickle cell disease by a panel of experts. The expert panel developed explicit clinical guidelines for the use of RBC in SCD patients. The panel also made recommendations for further research. A set of guidelines were produced for dissemination to pertinent stakeholders. If implemented, these clinical pathways have the potential to optimize the use of red blood cell transfusions in SCD.

Warfarin is the most widely used medicine for oral anticoagulant therapy (OAT). It inhibits the synthesis of coagulation factors II, VII, IX, and X in the liver and results in the production of inactive or partially active versions of these factors. Inactive coagulation factors interfere with prothrombin time measurement (Quick and Owren PT) measuring the sum of coagulation activity and inhibition. The narrow therapeutic range here involves a danger of serious complications and the risk of bleeding or thrombosis. The new-generation PT method can measure coagulation activity and inhibition separately. This new technique promotes patient care and anticoagulant medication (warfarin, dicoumarol) based on coagulation activity in vivo. Both therapy and laboratory controls should be unquestionably accurate and based solely on in vivo coagulation activity. Inactive coagulation factors (inhibition) render measurement, calibration, and harmonization. The use of the new-generation PT method based on measurement of coagulation activity in vivo could develop vitamin K antagonist (VKA) therapy for the marked benefit of patients.

Thirty-four blood samples of neonates in Dubai, UAE, with an MCV below 90 fL were checked by high performance liquid chromatography (HPLC) for hemoglobin variants to confirm a previous study carried out in Western Province of Saudi Arabia which showed a very high predictive index of such MCV for alpha (α-) thalassemia minor (ATM). MCH below 30 pg was an additional factor which supported such a prediction. The Dubai study confirmed the original finding with 100% of such neonates showing Hb Barts band. A control group of 26 neonates with an MCV between 90 and 95 fl showed Hb Barts in only 11 cases (42.3%). Of these, 6 (23.1%) were preterm babies, expected to have higher MCV. Five cases (19.2%) had an MCH below 30 pg, though MCV was 90 or higher. Three of the preterm babies also had MCH below 30. The study confirmed the Saudi results in neonates. It seems very highly probable that a term neonate with MCV below 90 and MCH below 30 has ATM.