BACKGROUND: Peripheral blood film morphology in neonates is significantly different from the adults as neonatal erythrocytes show a marked heterogeneity. Moreover, there seem to be a more significant numbers of irregular shaped red blood cells such as stomatocytes, keratocytes, schistocytes, and acantocytes in newborns, particularly in preterm infants. This review study focused on the red blood cell morphology in term and preterm neonates to detect clues to distinguish between the peripheral blood film of newborns under physiological and pathological conditions.
METHODS: The peripheral blood findings and blood cell counts in preterm and term neonates were studied and compared to each other using available scientific databases and indexing systems such as PubMed and Google Scholar.
RESULTS: This approach is a simple, cost-effective, quick, and informative method to distinguish between physiological and pathological conditions in neonates and detect hematological disorders.
CONCLUSIONS: Peripheral blood film plays a crucial role in diagnosing anemia and blood-related diseases, determining the type of anemia, and identifying specific morphological abnormalities of red cell membrane disorders.

Objective To delineate the etiology, symptomatology, and treatment of sickle cell intrahepatic cholestasis (SCIC). Sickle cell disease (SCD) is the most frequently inherited hematologic disease, and SCIC is one rare and often fatal complication and comorbid disease. The literature contains only a small number of case reports involving SCIC and hence limited guidance can be obtained. Methods We reviewed the scientific literature to evaluate the science of SCIC to determine if there were consistencies in presentation, evaluation, treatment, and clinical outcomes. Results We reviewed 6 case reports and a limited number of clinical papers on SCIC. We reported consistencies in clinical presentation and treatment outcomes among cases as well as serological and hematological finding. Conclusions While there is some consistency in the symptom presentation of individuals with SCIC, reliable evaluation and clinical procedures were not demonstrated in what we reviewed. Further research is needed to delineate the attributes of this complicated disease that occurs within SCD.

Black individuals are underrepresented in randomized clinical trials (RCTs). Willingness to participate is a frequently cited explanation. However, the few studies that have investigated willingness to participate demonstrated no difference between Black individuals and other groups. We sought to measure willingness to participate by focusing on sickle cell disease (SCD), in which approximately 90% of affected individuals are Black. We conducted an analysis of 17 RCTs. A level of clarity was defined and correlated with each article\'s transparency in reporting patient enrollment data. Calculated measures of acceptance ranged from 32% to 93.5%. Calculated completion rates ranged from 58.8% to 100%. Weighted measures of acceptance and completion were 59.1% and 83.8%, respectively. Our study is limited by focusing solely on studies pertinent to SCD and only a minority of publications reviewed provided sufficient patient enrollment data. Yet, our results suggest that decreased willingness to participate does not account for underrepresentation of Black individuals.

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Fetal hemoglobin (HbF) is a physiologic protein tetramer that is crucial for a developing fetus to survive in utero. Maternal hemoglobin has a relatively lower affinity for oxygen, and thus allows for an efficient transfer of oxygen from maternal to fetal blood. In addition to fulfilling a critical physiologic role, HbF is also known to alleviate symptoms of sickle-cell disease (SCD). The concentration of HbF depends on several factors. HbF is elevated in inherited conditions, such as hereditary persistence of HbF, hereditary spherocytosis, and thalassemia. The level of HbF is also increased in acquired states, such as pregnancy, aplastic anemia, thyrotoxicosis, hepatoma, myeloproliferative disorders, or hypoplastic myelodysplastic syndrome. It has been identified that some genetic loci have significant influence on HbF levels. The XmnI polymorphism, the HMIP locus, and the BCL11A gene are responsible for 45% of variations in HbF levels. Although SCD has been well described in the subpopulations of Africa, it is less common in the subpopulations of India. We describe a case of SCD, in which a patient with high HbF level presented at a very late age (27 years old). We presume the patient\'s inherently elevated HbF levels were able to compensate for the hypoxic episodes associated with SCD. The onset of symptoms was delayed as a result of elevated HbF levels.

Sickle cell disease (SCD) is an inherited chronic haemolytic anaemia whose clinical manifestations arise from the tendency of the haemoglobin to polymerize and deform red blood cells into the characteristic sickle shape due to a single nucleotide change in the β-globin. Vascular occlusion of small and large vessels can lead to chronic damage of multiple organs including brain, lung, bone, kidney, liver, spleen, and retina. However, the extent to which SCD impacts myocardial function is not very clear. Cardiovascular manifestations include both right and left ventricular systolic and diastolic dysfunction, elevated cardiac output, cardiomegaly and myocardial ischaemia. Progressive heart damage from iron overload occurs in patients requiring routine transfusion therapy. Pulmonary hypertension resulting from intravascular haemolysis has also been recognized as a major complication that independently correlates with survival. This review summarizes all available data for the heart complications in SCD to update the physicians for their appearance, diagnostic procedures and possible management.

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The substitution of one amino acid in the hemoglobin molecule results in sickle hemoglobin. As a result, RBCs sickle in low oxygen states causing occlusion of blood vessels, increased viscosity, and inflammation. These RBCs are prematurely removed from the circulation, resulting in a chronic hemolytic anemia. With newborn screening and early treatment, the death rate among children with SCD has declined. In addition, a variety of treatments are being introduced to help manage the various manifestations of disease. Transfusion, simple or exchange, is a mainstay of therapy, since it reduces the amount of Hgb S in circulation and suppresses erythropoiesis. Transfusion is indicated for symptomatic anemia and specifically to prevent stroke (first or recurrent), during acute stroke, and for acute chest syndrome. Unfortunately, transfusion carries risks for infectious disease transmission, as well as immunologic and inflammatory sequelae. For patients with SCD who may be chronically transfused, iron overload occurs frequently. In addition, due to differences in RBC antigens between donors and recipients, these patients are at increased risk for development of RBC alloantibodies, which can complicate further transfusion. It is, therefore, important to prevent alloimmunization by transfusing leukoreduced RBCs that match the patient for the C, E, and K1 antigens. Human progenitor cell (from bone marrow, peripheral blood stem cells, or umbilical blood) transplant can cure the disease, and is used for patients with severe disease for whom conventional therapy may not be effective.

Haematology analysers provide quick and accurate results in most situations. However, spurious results, related either to platelets (part I of this report) or to other parameters from the cell blood count (CBC) may be observed in several instances. Spuriously low white blood cell (WBC) counts may be observed because of agglutination in the presence of ethylenediamine tetra-acetic acid (EDTA). Cryoglobulins, lipids, insufficiently lysed red blood cells (RBC), erythroblasts and platelet aggregates are common situations increasing WBC counts. In most of these instances flagging and/or an abnormal WBC differential scattergram will alert the operator. Several situations lead to abnormal haemoglobin measurement or to abnormal RBC count, including lipids, agglutinins, cryoglobulins and elevated WBC counts. Mean (red) cell volume (MCV) may be also subject to spurious determination, because of agglutinins, excess of glucose or salts and technological considerations. In turn, abnormality related to one measured parameter will lead to abnormal calculated RBC indices: mean cell haemoglobin content (MCHC) is certainly the most important RBC indices to consider, as it is as important as flags generated by the haematology analysers (HA) in alerting the user to a spurious result. In many circumstances, several of the measured parameters from CBC may be altered, and the discovery of a spurious change on one parameter frequently means that the validity of other parameters should be considered. Sensitive flags now allow the identification of several spurious counts, but only the most sophisticated HA have optimal flagging and more simple HA, especially those without a WBC differential scattergram, do not possess the same sensitivity for detecting anomalous results. Reticulocytes are integrated now into the CBC in many HA, and several situations may lead to abnormal counts.

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