Current Issue : July - September Volume : 2014 Issue Number : 3 Articles : 8 Articles
Background: Nijmegen Breakage Syndrome (NBS) is a rare autosomal recessive DNA repair disorder characterized\nby immune deficiency, microcephaly, mental retardation and a disposition for the development of hematological\nmalignancies. So far, mostly pediatric patients have been described, since the underlying condition is often fatal\nbefore adulthood. Many patients diagnosed with Hodgkin lymphoma (HL) due to this DNA repair defect receive\nreduced treatment followed by early progression and fatal outcome.\nCase presentation: We describe here a 26-year old male caucasian patient with NBS who presented with multi\norgan failure due to HL. Immediate intensive chemotherapy lead to complete remission and reversed organ failure.\nConclusion: We show that application of standard chemotherapy can lead to long-term disease free survival in\npatients with a DNA repair disorder. Furthermore, we describe here, to the best of our knowledge, the first adult\npatient with NBS and HL....
Background: Peripheral pancytopenia is not a disease by itself; rather it describes simultaneous presence of\nanemia, leucopenia and thrombocytopenia resulting from a number of disease processes. Only a few systematic\nstudies have been published on the topic of pancytopenia, although extensive studies have been done for its\ndifferent etiological factors like aplastic anemia, megaloblastic anemia, leukemia, etc. Thus, this study was carried\nout to investigate for and to identify the causes of pancytopenia, to find out the frequency of different causes, to\ndetermine the incidence of pancytopenia in relation to sex and age and to compare our findings with those of\nother similar studies from this part of the world.\nMethods: This was a prospective study conducted in the Department of Pathology of a teaching institute and a\ntertiary care hospital in southern Maharashtra, India, over a period of two years. All the patients referred to the\ncentral clinical laboratory for routine complete blood count (CBC) and peripheral smear (PS) examination, from\nboth - the outpatient and the inpatient departments, were screened for pancytopenia. Of these, a total number of\n250 cases that fulfilled the diagnostic criteria were selected.\nDetailed hematological investigations followed by bone marrow aspiration wherever indicated and possible were\nperformed according to standard methods to ascertain the causes of pancytopenia.\nResults: A definite male preponderance was observed, the male to female ratio being 2.6 : 1. The majority of cases\nwere encountered in 3rd and 4th decades. Hypersplenism (29.2%), Infections (25.6%), Myelosuppressants (16.8%)\nand Megaloblastosis (13.2%) were the four most common causes in this large series on pancytopenia from a single\ncentre in India.\nConclusion: Detailed clinical history and meticulous physical examination along with baseline hematological\ninvestigations, provides invaluable information in the complete workup of pancytopenic patients, helping in\nsystematic planning of further investigations to diagnose and ascertain the cause, avoiding a battery of unnecessary\ntests....
Background: Transfusion-transmissible infections (TTIs) pose a major health risk in Cameroon given the high\nprevalence of such pathogens and increased demands for blood donations in the local communities. This study\naims at establishing the prevalence of commonly encountered TTIs among blood donors and transfusion-related\ncomplications among recipients in an urban center of Cameroon.\nMethods: A total of 477 blood donors and 83 blood recipients were recruited by consecutive sampling at the\nLaquintinie Hospital in Douala (LHD), Cameroon. Serum samples from blood donors were tested by quantitative\nenzyme-linked immunosorbent assays (ELISA) and/or using various Rapid diagnostic test (RDT) for presence of\nHepatits B (HBV) viral antigens, and antibodies to human immunodeficiency (HIV-1/2), Hepatits B (HCV) and\nTreponema pallidum. Recipient�s medical records were also analyzed for possible transfusion-associated\ncomplications.\nResults: The male/female sex ratio of the blood donors was 4/1 with a mean age of 30.2 (Sd = 8.3) years. Of all\nblood donors, 64/467 (13.7%) were infected by at least one of the four TTIs. Infected volunteer donors represented\n8.3% while infected family donors comprised 14.3% of the donor population. The prevalence of HCV, HIV, HBV and\nT. pallidum were 1.3%, 1.8%, 3.5%, and 8.1%, respectively. More than half of the blood recipients were female\n(78.3%) and the mean age was 20.6 (SD = 16.1) years. The causes of severe anemia indicative of transfusion in\nrecipients varied with wards (postpartum hemorrhage, caesarean section, uterine or cervical lacerations, abortions,\nurinary tract infections, severe malaria, vaso-occlusive attacks, wounds and gastrointestinal bleeding). The most\nfrequent complications were chills and hematuria, which represented 46.1% of all observed complications. Other\ncomplications such as nausea, vomiting, jaundice, sudden diarrhea, anxiety, tachycardia, or hyperthermia were also\nfound in recipients. Three cases of deaths occurred during the study, including a girl of less than one year.\nConclusion: This study confirms the presence of blood-borne infectious diseases in blood donors at the LHD,\nidentifying T. pallidum as the greatest threat to blood safety in the region, and hematuria as the most common\nimmunological complications in blood recipients....
Background: Complete blood count (CBC) and reticulocyte (Retics) are routine hematology tests useful for the\ndifferential diagnosis of anemia and other medical conditions. However, it has been presumed that they are not\nused as regular as they should be in medical practice in Addis Ababa hospitals.\nMethods: A hospital-based cross-sectional questionnaire survey was conducted during November-December 2010,\nin which 408 clinicians participated and their response on the use of CBC and Retics was assessed. The always/\nfrequently (A/F) response was considered to reflect routine use of the CBC/Retics parameters by the clinicians. The\nChi square test was used to study statistical associations among different variables.\nResult: Only four of 13 parameters in CBC were frequently or always used by more than 85% of the clinicians.\nHealth Officers were observed to use 12 of the 13 CBC parameters less than the other professional group; interns\nand residents demonstrated highest use of CBC results. More than a third of cliniciansââ?¬â?¢ preferred white blood cell\n(WBC) differential report in percentages than the more useful absolute number report. Reticulocyte parameters\nwere not being used by majority of clinicians in patient management. Clinicians rated ââ?¬Ë?averageââ?¬â?¢ regarding the\nadequacy of clinical laboratory methods course they took during medical education. As service users, clinicians\nindicated mm3 as unit of preference in cell count on the laboratory report form.\nConclusion: Overall, most clinicians do not use much of the data provided on routine CBC report. Additional\nresearch is needed to understand the issue further. Responsible bodies should promote the appropriate use of\nCBC/Retics reports by clinicians....
Background: Interleukin-6 (IL-6) is a multifunctional cytokine that is produced by many different cell types, and\nplays an important role in the regulation of inflammation, immune responses, the acute-phase response, and\nhematopoiesis. Previous laboratory and clinical studies have shown that IL-6 causes a significant decrease in serum\niron levels. Therefore, we conducted an epidemiological study to examine the association between serum IL-6 and\niron levels.\nMethods: In total, 280 Japanese individuals aged 20ââ?¬â??78 years were enrolled when they visited a clinic located in an\nurban area for Helicobacter pylori (H. pylori) infection tests and subsequent eradication; 65.3% were infected with\nH. pylori. Subjects with gastric cancer, idiopathic thrombocytopenia, or IL-6 > 10 pg/mL were excluded from the\nstudy. Serum iron and IL-6 levels were measured using the 2-nitroso-5-(N-propyl-3-sulfopropylamino) phenol\nmethod and chemiluminescence enzyme immunoassay, respectively.\nResults: Geometric mean iron and IL-6 levels were 111.5 Ã?µg/dL and 1.77 pg/mL, respectively, for men, and 89.4 Ã?µg/dL\nand 1.55 pg/mL, respectively, for women. The logarithm of serum iron levels was negatively correlated with the\nlogarithm of IL-6 levels in men (r = -0.19, p = 0.047), but not in women (r = -0.035, p = 0.65). Regression analysis,\nadjusted for sex, age, and H. pylori infection status, showed that the logarithm of serum iron levels was significantly\nassociated with a decreased logarithm of IL-6 levels (Ã?Ÿ = -0.053, p = 0.041). The odds ratio for low serum iron levels\nadjusted for sex, age, and H. pylori infection status was 7.88 (95% CI 1.29ââ?¬â??48.06) in those with an IL-6 level > 4 pg/mL.\nConclusion: Lower serum iron levels are significantly associated with higher serum IL-6 levels among Japanese adults....
Background: Anaemia is a common complication of infection with the human immunodeficiency virus (HIV) and\nmay have various causes. The aim of this study was to determine the prevalence and related factors of anemia in\nHAART-naive HIV positive Patients.\nMethods: A retrospective study was conducted on HAART naive HIV positive patients at the Gondar University\nHospital between September 2011 and August 2012. Socio-demographic and immunohematological (hemoglobin\nand CD4+ T cells) data were collected carefully from the existing ART logbook and patient follow up cards.\nAnaemia was defined according to the WHO criteria.\nResults: The overall prevalence of anaemia was 138 (35%). Female HAART naive HIV positive patients had\nsignificantly (P < 0.05) higher prevalence of anaemia than males (62% Vs 38%). The prevalence of anaemia at\ndifferent CD4 level was; 6 (4%) with CD4 count greater than 500 cells/Ã?µL, 18 (13%) with a CD4 count of 350ââ?¬â??500\ncells/Ã?µL, 37 (27%) with a CD4 count of 200ââ?¬â??349 cells/Ã?µL, 44 (32%) with a CD4 count of 100ââ?¬â??199 cells/Ã?µL, 14 (10%)\nwith a CD4 count of 50ââ?¬â??99 and 19 (14%) with CD4 count of less than 50 cells/Ã?µL.\nConclusions: Our findings showed that one-third of HAART naÃ?¯ve HIV positive patients were anaemic and the\nincrease in prevalence of anaemia with decreased CD4 cell count was statistically significant. Therefore, early\ndiagnosis and treatment of anaemia in these patients are essential....
Background: Anemia is a major health problem worldwide. Because of health and socioeconomic problems, the\nprevalence of anemia is higher in developing countries. Children and pregnant women are the most vulnerable\ngroups to anemia. The aim of the present study was to determine the magnitude of anemia among school children.\nMethods: A cross-sectional household survey was conducted in January 2011 on 423 children, aged 6ââ?¬â??14 years,\nselected through systematic random sampling method. Sociodemographic and anthropometric data were collected\nusing a pre-tested questionnaire. Capillary blood was taken from the fingertip of each child and hemoglobin was\nmeasured using HaemoCue digital photometer. All the necessary safety measures were taken during blood collection.\nAnthropometric indicators were measured using WHOââ?¬â?¢s guideline. Data analysis was made using SPSS Version 16.0 for\nWindows. The association between predictors and outcome variables were measured by a stepwise logistic regression\nmodel. Ethical permission was obtained; consent of the parents/guardian was taken and confidentiality was\nmaintained.\nResult: A total of 404 children were studied. The mean age was 10.21(SD Ã?± 1.89) years. The proportion of females\nwas 217(53.7%). The mean hemoglobin level for both sexes was 11.59(SD Ã?± 1.97 g/dl). The current prevalence of\nanemia was 152(37.6%), out of which, 73(18.1%) had mild while 79(19.6%) of them had moderate anemia. The\nprevalence of anemia among the age group of 6ââ?¬â??11 years was 118(40.5%) while the prevalence among the group\nof 12ââ?¬â??14 years old children was 34(30.1%). Among the selected variables in the logistic regression analysis, low\nfamily income [OR = 4.925, 95% CI(1.063,22.820)], mothersââ?¬â?¢ education [OR = 4.621, 95% CI(1.383,15.439)], intake of\nplant food [OR = 3.847, 95% CI(2.068, 7.157)] and intake of animal food [OR = 2.37, 95% CI(1.040,5.402)] were\nsignificantly and independently associated with anemia.\nConclusion: Anemia is a moderate public health problem in the study area. Family income, educational status of\nparents and inadequate plant and animal food intake are the predictors of anemia. Improving the economic status\nof the family, women education and health education about balanced animal and plant food consumption are\nrecommended strategies to reduce the burden of anemia....
Background: Prior to diagnosis, patients with haematological cancers often have multiple primary care consultations,\nresulting in diagnostic delay. They are less likely to be referred urgently to hospital and often present as emergencies.\nWe examined patient perspectives of time to help-seeking and diagnosis, as well as associated symptoms and\nexperiences.\nMethods: The UKââ?¬â?¢s Haematological Malignancy Research Network (www.hmrn.org) routinely collects data on all\npatients newly diagnosed with myeloma, lymphoma and leukaemia (>2000 annually; population 3.6 million).\nWith clinical agreement, patients are also invited to participate in an on-going survey about the circumstances\nleading to their diagnosis (presence/absence of symptoms; type of symptom(s) and date(s) of onset; date medical\nadvice first sought (help-seeking); summary of important experiences in the time before diagnosis). From 2004ââ?¬â??2011,\n8858 patients were approached and 5038 agreed they could be contacted for research purposes; 3329 requested and\nreturned a completed questionnaire. The duration of the total interval (symptom onset to diagnosis), patient interval\n(symptom onset to help-seeking) and diagnostic interval (help-seeking to diagnosis) was examined by patient\ncharacteristics and diagnosis. Type and frequency of symptoms were examined collectively, by diagnosis and\ncompared to UK Referral Guidelines.\nResults: Around one-third of patients were asymptomatic at diagnosis. In those with symptoms, the median patient\ninterval tended to be shorter than the diagnostic interval across most diseases. Intervals varied markedly by diagnosis:\nacute myeloid leukaemia being 41 days (Interquartile range (IQR) 17ââ?¬â??85), diffuse large B-cell lymphoma 98 days (IQR\n53ââ?¬â??192) and myeloma 163 days (IQR 84ââ?¬â??306). Many symptoms corresponded to those cited in UK Referral Guidelines,\nbut some were rarely reported (e.g. pain on drinking alcohol). By contrast others, absent from the guidance, were more\nfrequent (e.g. stomach and bowel problems). Symptoms such as tiredness and pain were common across all diseases,\nalthough some specificity was evident by sub-type, such as lymphadenopathy in lymphoma and bleeding and\nbruising in acute leukaemia.\nConclusions: Pathways to diagnosis are varied and can be unacceptably prolonged, particularly for myeloma and\nsome lymphomas. More evidence is needed, along with interventions to reduce time-to-diagnosis, such as public\neducation campaigns and GP decision-making aids, as well as refinement of existing Referral Guidelines...
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