Iron deficiency anemia is most common nutritional deficiency disorder in India and remains a formidable health challenge. Girls in the period of later school age and early adolescence are prone to develop iron deficiency. Iron deficiency leads to many non-hematological disturbances which include growth and development, depressed immune function in infants; reduces physical work capacity; decreases the cognitive function in both infants and adolescents. Present study was done to know the prevalence of iron deficiency in both the anemic and non anemic school going adolescent girls, to assess the effect of iron deficiency on cognitive functions in anemic iron deficient and non-anemic iron deficient school girls in a village school situated in central India. Methods. A secondary school having girl students in the age group of 12–15 years studying in sixth to ninth standard was selected. Serum ferritin concentration was estimated by ELISA. For assessing the cognitive function mathematics score, one multi-component test for memory, attention and verbal learning and Intelligent Quotient scores of the students were used. Results. Scholastic Performance, IQ and Scores of Mental balance, Attention & Concentration, Verbal Memory and Recognition were decreased in iron deficient girls, both anemic and non anemic as compared to the non iron deficient girls.

The aim of this study was to evaluate a laboratory-guided therapeutic algorithm of preoperative anemia. 335 patients with elective hip or knee arthroplasty were included in this retrospective before-after study. Group I (n = 101) underwent conventional preoperative procedures before algorithm implementation. Group II (n = 234) underwent algorithm-guided preoperative anemia management. A hemoglobin-level of 13 g/dL was the therapeutic cut-off for men and women. Reticulocyte hemoglobin content (CHr) and soluble transferrin receptor (sTfR)/log ferritin ratio were used in the form of the Thomas plot. Iron deficiency (ID) was substituted with 1000 mg iron intravenous (i.v.) and 10000 international units (I.U.) of erythropoiesis-stimulating agent (ESA) subcutaneous (s.c.) or i.v., anemia of chronic disease (ACD) (without functional ID) with 40000 I.U. ESA s.c. or i.v and additionally 200 mg iron i.v. Substituted anemic patients in Group II (n = 32) showed a distinctly higher preoperative (Hb-median 13 versus 11.95 g/dL) (P < 0.01) and postoperative (Hb-median 9.75 versus 9.0 g/dL) (P < 0.05) Hb level compared with untreated anemic patients in Group I (n = 24). In Group II red blood cell (RBC) units (35 units/234 patients) were reduced by 44% compared with Group I (27 units/101 patients). Algorithm-guided preoperative anemia management raises perioperative Hb-level and reduces blood use.

Endocrine complications due to haemosiderosis are present in a significant number of patients with beta-thalassemia major (BTM) worldwide and often become barriers in their desire for parenthood. Thus, although spontaneous fertility can occur, the majority of females with BTM is infertile due to hypogonadotropic hypogonadism (HH) and need assisted reproductive techniques. Infertility in these women seems to be attributed to iron deposition and iron-induced oxidative stress (OS) in various endocrine organs, such as hypothalamus, pituitary, and female reproductive system, but also through the iron effect on other organs, such as liver and pancreas, contributing to the impaired metabolism of hormones and serum antioxidants. Nevertheless, the gonadal function of these patients is usually intact and fertility is usually retrievable. Meanwhile, a significant prooxidants/antioxidants imbalance with subsequent increased (OS) exists in patients with BTM, which is mainly caused by tissue injury due to overproduction of free radicals by secondary iron overload, but also due to alteration in serum trace elements and antioxidant enzymes. Not only using the appropriate antioxidants, essential trace elements, and minerals, but also regulating the advanced glycation end products, could probably reduce the extent of oxidative damage and related complications and retrieve BTM women's infertility.

Direct sequencing of the PIG-A gene has demonstrated distinct mutations in all PNH patients These mutations can be found in all blood lineages, consistent with a genetic lesion occurring in hematopoietic stem cell(s) All types of mutations have been observed No particular clustering of mutations has emerged, even if most of the mutations occur in exon 2, probably because it is the largest. If we compare the type of mutations found in PNH with those found in G6PD deficiency --another (this time inherited) X-linked disorder of a housekeeping gene --a clear discrepancy is evident. In PNH, a vast majority of mutations have extremely severe functional consequences, (i.e., production of truncated proteins, leading to a complete GPI deficiency -the PNH type III phenotype), whereas missense mutations are rare (usually resulting in the PNH type II phenotype); in G6PD deficiency almost all mutations are missense, leading to single amino acid substitutions with conserved (although possibly altered) function. Two considerations may be relevant to this discrepancy: i. in inherited disorders, mutations in a housekeeping gene may be lethal, and thus have been selected to allow residual functional activity; by contrast, null mutations can be seen if they are somatically acquired, because they do not affect organ development and pertains only to a small fraction of somatic cells, which may survive albeit with some functional abnormality; ii. if missense mutations in PNH are rare, it means that the gene must be seriously damaged for leading to a clonal expansion. Indeed, the rare missense mutations found in PNH patients are associated with a marked reduction of GPI-linked proteins, indicating that they usually have affected either mRNA stability or protein function, or both. Remarkably, different types of mutations may account for different cell phenotypes in PNH patients, especially when the erythrocytes are considered. In fact, RBCs harboring a mutation leading to the complete inactivation of the PIG-A gene product will unequivocally show a complete deficiency of all GPI-APs from their surface (the so-called PNH type III phenotype). In contrast, mutations leading to partial inactivation of the PIG-A gene (for instance missence mutations) may lead to a partial deficiency of GPI-APs, known as the PNH type II phenotype. Of note, this biochemical finding has direct functional consequences; in fact, as discussed later on, type II and type III erythrocytes have a quite distinct susceptibility to complement-mediated lysis Quite surprisingly, some PNH patients may harbor at the same time type II and type III cells, suggesting that distinct PIG-A mutated HSCs may expand concomitantly (Endo et al 1995; Nafa et al 1998). Indeed, even phenotypically identical PNH cells may carry distinct PIG-A genotypes (Endo et al 1995

Erythrocytes are produced by a complex and finely regulated process of erythropoiesis. It starts with a pluripotential stem cell that, in addition of its self replication capacity, can give rise to separate cell lineages. Erythropoiesis passes from the stem cell through the multipotent progenitor CFU-GEMM (colony-forming unit granulocyte erythroid monocyte and megakaryocyte), and then BFU-E (burst-forming unit erythroid) and CFU-E (colonyforming unit eryhtroid), to the first recognizable erythrocyte precursor in the bone marrow, the pronormoblast. This cell gives rise to a series of progressively smaller normoblasts with increasing content of hemoglobin. The nucleus is finally extruded from the late normoblast leading to mature red blood cell through the reticulocyte stage. Erythropoiesis ends with the mature circulating red cell, which is a non-nucleated biconcave disc, performing its function of oxygen delivery. In this process, the glycoprotein hormone erythropoietin has been known as the major humoral regulator of red cell production. It is now well established that erythropoietin stimulates erythropoiesis, at least in part, by protecting erythroblasts from apoptosis. Human mature erythrocytes are terminally differentiated cells that are devoid of mitochondria, as well as of nucleus and other organelles. In circulation, the red cell is constantly tested for its capacity to undergo marked cellular deformation. This ability to change its shape is essential for optimal cell function, since the resting diameter of the human red cell far exceeds that of the capillaries and splenic endothelial slits through which it must pass (Mohandas & Groner, 1989). A two dimensional network of proteins interacting between transmembrane location and cytoplasmic surface of the plasma membrane gives the red blood cell its properties of elasticity and flexibility that allows the success of this journey. The mature erythrocyte is unable to self-repair and has no capacity to synthesize proteins. Therefore, its lifespan is finite and is shortened further when the cell’s environment becomes hostile or when the erythrocyte’s ability to cope with damaging extracellular influences becomes impaired. The erythrocyte limited lifespan implies that, as in other cells, life and death are well regulated for erythrocytes, in spite of their lack of capacity for protein synthesis (Bosman et al., 2005). In the present review, we aim to show updated information concerning erythrocyte death in order to contribute to the understanding of the physiopathological relationship of this process with the development of anemia.

Background. Pancytopenia has multiple etiologies like megaloblastic anemia, aplastic anemia, leukemia, and various infections. We investigated the clinical, etiological and hematological profile including bone marrow morphology of patients with pancytopenia in relation to their vitamin B12 and folic acid status at a tertiary care referral hospital in north India. Methods. A total of 140 consecutive patients with pancytopenia were selected from June 2007 to December 2008. Bone marrow examination and other tests were carried out as warranted, including serum cobalamin and folate assays using liquid chromatography mass spectroscopy (LC MS/MS). Results. The study population consisted of 92 males and 48 females with a mean age of 32.8 years. Megaloblastic anemia 60.7%, aplastic anemia (7.8%), and leukemia (9.2%) were common causes. Infectious causes (16.4% of all cases) included leishmaniasis, HIV–AIDS, malaria and tuberculosis. Severe cobalamin deficiency (B12 < 100 pg/mL) was seen in 81% of all patients including 91.6% of patients with MA. In contrast, only 7.14% of all pancytopenic patients were folate deficient. Folate deficiency (<5 ng/mL) was seen in just 5% MA patients. Combined cobalamin and folate deficiency was seen in 5 patients (3.51%). Conclusion. Cobalamin deficiency was found to be more common in our setting and is largely underdiagnosed in the age of folate supplementation. Infectious diseases like tuberculosis, leishmaniasis, and increasingly HIV are important and treatable causes of pancytopenia. This is in contrast with the developed nations where the bulk of disease is due to malignancy or marrow aplasia.

Objectives. Anemia is a major public health problem throughout the world which assumes prominence in pregnant mothers. Patients with severe anemia continue to present themselves at term or in labor. This study was conducted to compare the improvements in hematological parameters of patients receiving partial exchange blood transfusion and transfusion of packed cells without exchange. Methods. One hundred and twenty-five severely anemic antenatal mothers were admitted from outpatient service. Partial exchange transfusion was given to sixty-six patients while fifty-nine received transfusion of packed cells with frusemide cover. Results. The two groups were comparable in terms of age, height, weight, religion, diet, education, occupation of self and husband, and income. Hemoglobin level in Group 1 was comparatively less than Group 2 at prelevel (5.2 ± 1.5 versus 6.6 ± 2.3, P = 0.001) and postlevel (7.2 ± 1.5 versus 8.6 ± 1.8, P = 0.001), respectively, but there was no significant difference between the two modes of transfusion (2.09 ± 1.6 versus 2.01 ± 1.5, P = 0.78). Conclusion. The study produced an equally significant improvement in hematological parameters in partial exchange and packed cell transfusion. Platelet counts were significantly less in partial exchange as compared with packed cell transfusion.

Sickle cell anemia is one of the best studied inherited diseases, and despite being caused by a single point mutation in the HBB gene, multiple pleiotropic effects of the abnormal hemoglobin S production range from vaso-occlusive crisis, stroke, and pulmonary hypertension to osteonecrosis and leg ulcers. Urogenital function is not spared, and although priapism is most frequently remembered, other related clinical manifestations have been described, such as nocturia, enuresis, increased frequence of lower urinary tract infections, urinary incontinence, hypogonadism, and testicular infarction. Studies on sickle cell vaso-occlusion and priapism using both in vitro and in vivo models have shed light on the pathogenesis of some of these events. The authors review what is known about the deleterious effects of sickling on the genitourinary tract and how the role of cyclic nucleotides signaling and protein kinases may help understand the pathophysiology underlying these manifestations and develop novel therapies in the setting of urogenital disorders in sickle cell disease.

Anemia is a clinical manifestation with high prevalence in the world, reaching about 30% of the total inhabitants of the planet. It is responsible for a great reduction in quality of life of affected people. The present paper aimed to obtain the frequency of anemia in the blood counts of a sample from the population of registered patients of a Family Health Team in Dourados, MS, Brazil. 462 patients, who had blood counts done from February 2008 to March 2009, were included in this research. The frequency of anemia was 22.7% in the studied population, including all age groups.

Background. Erythropoietin deficiency and anemia occur in Chronic Kidney Disease (CKD) and may be treated with Erythropoietin Stimulating Agents (ESAs). The optimal hemoglobin, in non-End Stage Renal Disease CKD, is controversial. Methods. We review three recent randomized trials in anemia in CKD: CHOIR, CREATE, and TREAT. Results. CHOIR (N = 1432) was terminated early with more frequent death and cardiovascular outcomes in the higher Hb group (HR 1.34: 95% C.I. 1.03–1.74, P = .03). CREATE (N = 603) showed no difference in primary cardiovascular endpoints. Stroke was more common in the higher Hb group (HR 1.92; 95% C.I. 1.38–2.68; P < .001) in TREAT (N = 4038). Conclusions. There is no benefit to an Hb outside the 10–12 g/dL range in this population. To avoid transfusions and improve Quality of Life, ESAs should be used cautiously, especially in patients with Diabetes, CKD, risk factors for stroke, and ESA resistance.