[ABS]Phospholipid asymmetry is well maintained in erythrocyte (RBC) membranes with phosphatidylserine (PS) exclusively present in the inner leaflet. Eryptosis, the suicidal death of RBCs, is characterized by cell shrinkage, membrane blebbing, and cell membrane phospholipids scrambling with PS exposure at the cell surface. Erythrocytes exposing PS are recognized, bound, engulfed, and degraded by macrophages. Eryptosis thus fosters clearance of affected RBCs from circulating blood, which may aggravate anemia in pathological conditions. Thalassemia patients are more sensitive to the eryptotic depletion and osmotic shock which may affect RBC membrane phospholipid asymmetry. We aimed in this work to determine the RBC PS exposure in splenectomized and nonsplenectomized β-thalassemia major (β-TM) patients and correlate it with the clinical presentation and laboratory data. RBCs were stained for annexin V to detect phosphatidylserine (PS) exposure in 46 β-TM patients (27 splenectomized and 19 nonsplenectomized) compared to 17 healthy subjects as a control group. We observed a significant increase in RBC PS exposure in β-TM patients compared to control group (P = .0001). Erythrocyte PS exposure was significantly higher in splenectomized β-TM patients compared with nonsplenectomized β-TM patients (P = .001). No correlation was found between RBC PS exposure and clinical or hematological data of β-TM patients, but there was a positive correlation between RBC PS exposure and ferritin level in β-TM patients have higher levels of RBC PS exposure, and splenectomy was shown to aggravate RBC PS exposure without aggravation of anemia.

We describe a method to isolate small mononucleated Hodgkin (H) cells from multinucleated Reed Sternberg (RS) cells of Hodgkin lymphoma using the ScreenCell filter device. This filtration-based approach lends itself to future clinical applications in that it enables the separation of H and RS cells from lymph node biopsies, bone marrow aspirates, pleural effusions, and blood, including the isolation of monoclonal Hodgkin precursor cells from the blood.

Congenital Factor VII (FVII) deficiency can be divided into two groups: cases of "true" deficiency, or cross-reactive material (CRM) negative and variants that are cross-reactive material positive.The first form is commonly recognized as Type I condition whereas the second one is known as Type II. FVII deficiency has been occasionally associated with thrombotic events, mainly venous. The reasons underlying this peculiar manifestation are unknown even though in the majority of associated patients thrombotic risk factors are present. The purpose of the present study was to investigate if a thrombotic event was more frequent in Type I or in Type II defect.The majority of patients with FVII deficiency and thrombosis belong to Type II defects. In the following paper we discuss the possible role of the dysfunctional FVII cross-reaction material as a contributory cause for the occurrence of thrombosis.

Hematopoietic stem cells (HSCs) and blood cell progenitors, such as maturing leucocytes, steadily enter from bone marrow (BM) into the circulation under steady-state conditions, and their mobilization is dramatically amplified during stress conditions and by mediators such as granulocyte colony-stimulating factor (G-CSF). This mobilization is dependent upon bone remodeling, the proteolytic enzymes of bone marrow-derived stromal cells, and adhesion molecules such as integrin, but the main mechanisms controlling this traffic are still unclear. The nervous system, as the most important regulator of the body, can affect the mobilization network by secreting catecholamines, so that denervation of catecholaminergic fibers in the BM of mice could lead to declining mobilization in steady state and stress situations, even in the presence of other intact environmental factors in the BM. Thus, due to the importance of the nervous system, we have attempted to give a general overview of how the nervous system is involved in the mobilization of HSCs in this review. Then, we will try to describe the mobilization process induced by the nervous system, which consists of 3 mechanisms: stromal cell-derived factor 1 (SDF-1)/CXC chemokine receptor type 4 (CXCR4), proteolytic enzymes, and bone remodeling.

Multiple myeloma (MM) is a plasma cell disorder that occurs in about 10% of all hematologic cancers. The majority of patients (99%) are over 50 years of age when diagnosed. In the bone marrow (BM), stromal and hematopoietic stem cells (HSCs) are responsible for the production of blood cells. Therefore any destruction or/and changes within the BM undesirably impacts a wide range of hematopoiesis, causing diseases and influencing patient survival. In order to establish an effective therapeutic strategy, recognition of the biology and evaluation of bioinformatics models for myeloma cells are necessary to assist in determining suitable methods to cure or prevent disease complications in patients. This review presents the evaluation of molecular and cellular aspects of MM such as genetic translocation, genetic analysis, cell surface marker, transcription factors, and chemokine signaling pathways. It also briefly reviews some of the mechanisms involved in MM in order to develop a better understanding for use in future studies.

Infantile pyknocytosis (IP) is an under-recognized hematological entity of newborns that can cause a severe neonatal hemolytic anemia. A careful, prompt, and accurate peripheral blood smear examination is essential to establish the diagnosis. Here we describe the clinical features and histological parameters of 1 case of IP. Spontaneous resolution usually occurs by 4 to 6 months, but red blood cell transfusion may be needed if the anemia is severe.

Platelet hyperreactivity is an important but under-recognized cause of idiopathic arterial thrombosis. We describe a case of arterial thrombosis in a previously healthy 12-year-old girl after minor trauma to the knee, resulting in lower extremity amputation. Family history was positive for arterial and venous thrombosis, but an extensive work-up for the usual inherited thrombophilia risk factors was negative. The patient was eventually diagnosed with platelet hyperreactivity and remains on life-long antiplatelet therapy.Consideration of platelet hyperreactivity in the evaluation of unusual arterial thrombotic events allows for targeted therapy, potential avoidance of future events, and timely screening of family members.

The HEMOX Analyzer (TCS Scientific) has been used to measure the full oxygen-dissociation curve (ODC) and to calculate P(50) and the Hill coefficient. The effects of different anticoagulants on sample stability and P(50) values have not been evaluated extensively for this instrument. We characterized an artificial hemoglobin (Equil QC463) for quality control (QC) and compared P(50) values for blood samples drawn into 3 different anticoagulants (acid citrate dextrose [ACD], heparin, and EDTA). P(50) values were not stable in ACD but were stable in heparin and EDTA anticoagulants for up to 4 days. Tests with Equil QC463 showed that P(50) values were quite sensitive to small variations in buffer pH. Use of the correct anticoagulant and strict control of buffer pH are 2 parameters that need to be accounted for in best-practices use of this hemoximeter and before determining P(50).