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  • ISSN: 2333-6684
    Early Online
    Volume 6, Issue 1
    Editorial
    Georges El Hachem*
    Chronic myeloid leukemia (CML) is a myeloproliferative disorder characterized by the proliferation of the granulocytic cell line while maintaining its capacity to differentiate. It is caused by a cytogenetic aberration in more than 95 % of the cases: the Philadelphia chromosome that results from the reciprocal translocation between the long arms of the chromosomes 9 and 22 [t(9;22)]. CML presents in 3 clinical phases: the chronic phase, the accelerated phase and the blast crisis.
    Case Report
    Aisha Mahesar*, Ayisha Imran, and Noman A. Malik
    P blood group antigen of the GLOB system is a glycolipid structure, also known as globoside on the Red Blood Cells (RBCs) of almost all individuals worldwide. P1PK blood group system antigens include P1, P and PK antigens. Among these, P1K phenotype is very rare and the RBCS of these individuals express P1, PK antigens. The high incidence antigen, P, is missing and anti-P antibody is present in the serum. Naturally occurring anti-P is present in the serum of individuals with the rare globoside-deficient phenotypes p, P1k, and P2k and has been implicated in hemolytic transfusion reactions, Donath-Landsteiner antibody as well as unfavorable outcomes of pregnancy. When an individual with P1K phenotype needs blood transfusion, they can receive only autologous blood or blood from another P1K phenotype or p phenotype (if P1K blood is not available). We report a case of a young boy with P1K phenotype, anti-P and px2 antibodies who developed a severe hemolytic transfusion reaction and were successfully treated conservatively.
    Tanya Milachich*, Tanya Timeva, and Atanas Shterev
    Seven years follow-up after the birth of a healthy infant, who became a bone marrow donor two years after delivery to his sick sibling, after successful preimplantation genetic diagnosis (PGD) testing by both cleavage stage and blastocyst biopsy for the purpose of diagnosis of β-thalassaemia and HLA compatibility has been described in the present case report.
    Since allogenic hematopoietic stem cell transplantation (HSCT) is the only cure available for Beta-Thalassemia. However there is a limited possibility of finding human leukocyte antigen (HLA) matched donor, among relatives. HSCT from a HLA identical sibling provides the best treatment option which reduces the incidence of graft rejection and other serious complications associated with transplantation and the major part of these cases can be cured successfully.
    Here, we present a successful pregnancy achieved after PGD for β-Thalassaemia combined with HLA typing in a family with β-thalassemia major carrier ship. A healthy girl with HLA compatibility with his affected brother was born in term and umbilical cord blood of the baby was frozen after delivery for possible future treatment. Two years after delivery the transplantation of bone marrow has been accomplished after complete immune suppression of the affected brother (5 years old). Another five years post allogenic transplantation two siblings are healthy and without complications in their development. Treatment and transfusions of the affected child were no longer needed. Being the first case in Bulgaria, this success demonstrates the feasibility of international collaboration for application of sophisticated techniques in different specialized hospitals and clinics.
    Opinion
    Georges El Hachem*
    The spleen, also known as a blood filter, is similar in structure to a large lymph node. It plays a major role in regard to red blood cells (RBCs), by removing senescent cells and serving as a pool of blood. It is also essential for the immune system: phagocytosis, antibody synthesis, removal of opsonized bacteria and antibody-coated RBCs.
    Research Article
    Maham Maryam, Maha Zahid, Maarij Khurshid, Malik Qistas Ahmad*, Khadija Yasmin, Komal Sarfraz, Chaudhry Saad Sohail, Maaz Ahmad, Ghulam Abbas, Abida Pervaiz, Muhammad Waqas, and Muhammad Yasir
    Background: Maternal anemia is a common problem in pregnancy, particularly in developing countries. The study was aimed at determining the factors associated with anemia among pregnant mothers who attended Jinnah hospital Out Patient Department (OPD) Lahore, Pakistan.
    Objective: The objective of this study is to find the factors associated with iron deficiency anemia among pregnant women visiting Jinnah hospital Lahore.
    Methods: A cross sectional study was conducted in Gynecology OPD, Jinnah hospital Lahore for one month by non-probability purposive sampling technique. Jinnah hospital is a tertiary care hospital with three units of Gynecology Department. The data was entered and analyzed by using the SPSS version 17.0 statistical software. p value ≤ 0.05 was considered as statistically significant.
    Results: Out of 150 sampled women, 128 (85.3%) were anemic (Hgb<11g/dl). Out of 128, women 42 (32.8%) had mild anemia (10.0-10.9g/dl), 82 (64.1%) had moderate anemia (7.0-9.9g/dl) and 4 (3.1%) had severe anemia (<7g/dl). There was significant association (p ≤ 0.05) of iron deficiency anemia in pregnant women with monthly income and meat consumption.
    Conclusion: In the present study, iron deficiency anemia is significantly associated with consumption of meat and monthly income.
    Research Article
    Reva Tripathi, Vertika Verma, Tejinder Singh, Smita Kaushik, Mala YM, and Nilanchali Singh
    Objective: To evaluate the biochemical profile of patients with severe anemia in pregnancy and to study relation between maternal and umbilical cord serum levels of iron, ferritin, cobalamin and folate.
    Methods: It is a prospective case control study with 200 pregnant women with severe anemia (Hb< 7 gm/dl) and pregnant women with no anemia (Hb>11gm/dl were recruited. Serum ferritin, folate and cobalamin were measured by electro-chemiluminescence method. Cord blood samples were collected for estimating the levels of iron, ferritin, folate and cobalamin.
    Results: Cases were divided into three groups, microcytic anemia (47%), macrocytic anemia (37.5%) and dimorphic anemia (15.5%). Mothers with iron deficiency were 37.5%, whereas cobalamin and folate deficiency were seen in 47% and 11% respectively. In women with microcytic anemia, iron and ferritin levels in umbilical cord blood were 5.54 and 19.41 times higher, respectively, than maternal serum values. However, in macrocytic anemia, cobalamin levels were only 1.22 times more in cord blood as compared to maternal serum values.
    Conclusion: Cord blood values show that fetus is able to compensate for low levels of maternal iron and folate but not for cobalamin, indicating the absence of preferential transfer of cobalamin to the fetus.
    Short Communication
    Vaughan K Williams1*, Georgia Antoniou2, Andrew Atkins3, and Anne Jackson4
    Intuitively it would be assumed that the quality of life of current paediatric haemophilia patients has improved due to treatment modalities and integrated haemophilia centre support programs. This study was performed to determine if this is indeed the case. A small number of 8-12 year old haemophilia patients were asked to fill out the Haem-o qol questionnaire. Adult haemophilia patients were also presented with the 8-12 year questionnaire and asked to fill it out as they remembered their childhood experiences. Examining the median overall ratings, the adults reported higher values. However, of the eleven subscales only four showed a statistically significant difference (p<0.05) between the children and adults (view of yourself, friends, dealing with haemophilia and global health). Quality of life has improved for paediatric haemophilia patients, with adults having a higher median value for all of the eleven subscales. Apart from sport and school where improved treatment has contributed to a better physical outcome a major area of improvement is the psychosocial interaction of the paediatric population. There are still areas where improvement can be made to increase the QoL of persons with haemophilia.
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