Genetic Findings of Potential Donor Origin following Hematopoietic Cell Transplantation: Recommendations on Donor Disclosure and Genetic Testing from the World Marrow Donor Association.

Following hematopoietic cell transplantation (HCT), recipients are subjected to extensive genetic testing to monitor the efficacy of the transplantation and identify relapsing malignant disease. This testing is increasingly including the use of large gene panels, which may lead to incidental identification of genetic and molecular information of potential donor origin. Deciphering whether variants are of donor origin, and if so, whether there are clinical implications for the donor can prove challenging. In response to queries from donor registries and transplant centers regarding best practices in managing donors when genetic mutations of potential donor origin are identified, the Medical Working Group of the World Marrow Donor Association established an expert group to review available evidence and develop a framework to aid decision making. These guidelines aim to provide recommendations on predonation consenting, postdonation testing of recipients, and informing and managing donors when findings of potential donor origin are identified in recipients post-transplantation. It is recognized that registries will have different access to resources and financing structures, and thus whenever possible, we have made suggestions on how recommendations can be adapted.

COVID-19 in hematological malignancies: Case series and literature review.

Earlier reports suggest that cancer patients were twice more likely to contract severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection. In this report, we describe two patients with hematological malignancies seen at the peak of the first wave of the coronavirus disease 2019 pandemic. A 61-year-old man was referred to our urology unit he was diagnosed with nodular hyperplasia and multiple myeloma and commenced on bortezomib, thalidomide, and dexamethasone combination chemotherapy. He developed a cough and fever, with SPO2 86%, He was positive for SARS-CoV-2 and died a few days later. A 42-year-old man with Hodgkin lymphoma on treatment with Adriamycin, bleomycin, vincristine, and dacarbazine with positive SARS-CoV-2 exposure was diagnosed with pleural effusion at A/E. Three days postadmission, his condition worsened with low SPO2 despite intranasal oxygen. He died after testing positive for SARS-CoV-2. Patients with hematological malignancies tend to have a greater risk of SARS-COV-2 infection and severe disease due to immunosuppression from cancer and its treatment.

Résumé Des rapports antérieurs suggèrent que les patients atteints de cancer étaient deux fois plus susceptibles de contracter le coronavirus 2 du syndrome respiratoire aigu sévère (SARS-CoV-2) infection. Dans ce rapport, nous décrivons deux patients atteints d'hémopathies malignes vus au plus fort de la première vague de la maladie à coronavirus pandémie de 2019. Un homme de 61 ans a été référé à notre unité d'urologie. On lui a diagnostiqué une hyperplasie nodulaire et un myélome multiple. commencé une chimiothérapie combinée bortézomib, thalidomide et dexaméthasone. Il a développé une toux et de la fièvre, avec SPO2 86%, Il était positif pour le SRAS-CoV-2 et est décédé quelques jours plus tard. Un homme de 42 ans atteint d'un lymphome hodgkinien sous traitement par adriamycine, bléomycine, la vincristine et la dacarbazine avec une exposition positive au SRAS-CoV-2 ont reçu un diagnostic d'épanchement pleural à l'A/E. Trois jours après l'admission, son l'état s'est aggravé avec une faible SPO2 malgré l'oxygène intranasal. Il est décédé après avoir été testé positif au SRAS-CoV-2. Les patients atteints d'hématologie les tumeurs malignes ont tendance à avoir un risque plus élevé d'infection par le SRAS-COV-2 et de maladie grave en raison de l'immunosuppression du cancer et de son traitement. Mots-clés: Traitement du cancer, tumeurs malignes hématologiques, immunosuppression, syndrome respiratoire aigu sévère coronavirus 2.

Plasma Concentration of 12-Hydroxyeicosatetraenoic Acid, Single Nucleotide Polymorphisms of 12-Lipooxygenase Gene and Vaso-Occlusion in Sickle Cell Disease.

Background and Novel Aspect of this Work: In the light of previous findings that inflammation predisposes to intercellular adhesion and microvascular occlusion in sickle cell disease (SCD), this study investigated the relationship between the number of vaso-occlusive events in SCD, plasma levels of the pro-inflammatory molecules 12-Hydroxyeicosatetraenoic acid (12-HETE), TNF-α and IL-1ß; and single nucleotide polymorphisms (SNPs) in the gene 12-Lipooxygenase (ALOX-12), which encodes the enzyme 12-Lipoxygenase that catalyzes the biosynthesis of 12-HETE. Objective: To evaluate the relationship between vaso-occlusion in SCD and plasma concentrations of 12-HETE, TNF-α, and IL-1ß; and single nucleotide polymorphisms (SNPs) in ALOX-12 gene. Participants and Methods: In 50 HbSS patients, the numbers of vaso-occlusive crisis requiring hospital treatment in the previous 1 year and the vaso-occlusive complications of SCD developed to date (e.g stroke) were added to obtain the vaso-occlusive events (VOE) score. In the HbSS patients and 30 healthy sibling control persons, plasma concentrations of 12-HETE, TNF-α and IL-1ß were measured by ELISA, the ALOX12 SNPs rs2073438 and rs1126667 detected by DNA sequencing, and the accrued data statistically analyzed. Results: Compared to SCD patients with VOE score 0-1, those with scores ≥3 had higher plasma levels of 12-HETE (p < 0.0001) and TNF-α (p = 0.19), but not IL-1ß (p = 0.27). VOE score showed strong direct correlation with plasma level of 12-HETE (r = 0.65, p < 0.0001), but not with TNF-α nor IL-1ß. Neither VOE score nor plasma concentration of 12-HETE showed any relationship with the ALOX12 SNPs rs2073438 and rs1126667. Conclusion: The strong direct correlation of VOE score with plasma concentration of 12-HETE suggests that the clinical relevance of this pro-inflammatory molecule in SCD-associated vaso-occlusion needs to be evaluated in further studies.

Socio-economic determinants of prenatal anaemia in rural communities of South-West Nigeria: a preliminary report.

BACKGROUND: Anaemia is common worldwide and pregnant women are one of the most vulnerable group. Although, anaemia in the general population including pregnant women is multi-factorial in aetiology, the most frequent cause in pregnancy worldwide is iron deficiency. In Nigeria, an estimated prevalence of anaemia among pregnant women ranges from 35-75%. Anaemia in pregnancy (AIP) is associated with significant perinatal and maternal morbidity and mortality including premature birth and low birth weight. AIM: The aim of this study was to determine the prevalence, demographic and socio-economic determinants of anaemia in pregnancy in a rural community of South-West Nigeria. MATERIALS AND METHODS: One-hundred and fifty consenting pregnant women aged 18-42 years in the three trimesters were recruited from four primary health centres of Ikene Local Government of Ogun State of Nigeria after ethical approval was obtained from the Ethics Unit of the Medical officer of Health of the Local Government. Pre-tested interviewer-administered questionnaire was used to collect data on socio-demographic information and 24-hour dietary recall. Using a finger prick, the haemoglobin concentration of each respondent was determined with a haemoglobinometer (DG-300HB manufactured by DouBle, China). Data was analyzed using the Statistical Package for Social Sciences (SPSS) version 20. RESULTS: All the respondents belonged to low socio-economic class. The mean haemoglobin (Hb) concentration obtained in this study was 10.22±1.60 g/dL with a range of 6-14.8 g/dL. Using WHO cut-off Hb concentration of 11 g/dL, the prevalence of anaemia in this study was 67.3%. The frequency of anaemia increased with increase in age group. P=0.010. About 21.4% of those with adequate dietary iron intake were anaemic when compared with 72.1% (98 of 136) of those with inadequate dietary iron intake who were anaemic. AOR-0.090; 95% CI- 0.018-0.457; P=0.004. CONCLUSION: Increasing age, low socio-economic status, poor health education and low dietary iron intake were the predominant socio-economic determinants of prenatal anaemia in the population studied. Efforts must be intensified to alleviate poverty in rural areas and give health education on iron-rich foods to girls and women of children-bearing age in the rural communities.

Multiple drug combinations of bortezomib, lenalidomide, and thalidomide for first-line treatment in adults with transplant-ineligible multiple myeloma: a network meta-analysis.

BACKGROUND: Multiple myeloma is a bone marrow-based hematological malignancy accounting for approximately two per cent of cancers. First-line treatment for transplant-ineligible individuals consists of multiple drug combinations of bortezomib (V), lenalidomide (R), or thalidomide (T). However, access to these medicines is restricted in many countries worldwide. OBJECTIVES: To assess and compare the effectiveness and safety of multiple drug combinations of V, R, and T for adults with newly diagnosed transplant-ineligible multiple myeloma and to inform an application for the inclusion of these medicines into the World Health Organization's (WHO) list of essential medicines. SEARCH METHODS: We searched CENTRAL and MEDLINE, conference proceedings and study registries on 14 February 2019 for randomised controlled trials (RCTs) comparing multiple drug combinations of V, R and T for adults with newly diagnosed transplant-ineligible multiple myeloma. SELECTION CRITERIA: We included RCTs comparing combination therapies of V, R, and T, plus melphalan and prednisone (MP) or dexamethasone (D) for first-line treatment of adults with transplant-ineligible multiple myeloma. We excluded trials including adults with relapsed or refractory disease, trials comparing drug therapies to other types of therapy and trials including second-generation novel agents. DATA COLLECTION AND ANALYSIS: Two review authors independently extracted data and assessed risk of bias of included trials. As effect measures we used hazard ratios (HRs) for overall survival (OS) and progression-free survival (PFS) and risk ratios (RRs) for adverse events. An HR or RR < 1 indicates an advantage for the intervention compared to the main comparator MP. Where available, we extracted quality of life (QoL) data (scores of standardised questionnaires). Results quoted are from network meta-analysis (NMA) unless stated. MAIN RESULTS: We included 25 studies (148 references) comprising 11,403 participants and 21 treatment regimens. Treatments were differentiated between restricted treatment duration (treatment with a pre-specified amount of cycles) and continuous therapy (treatment administered until disease progression, the person becomes intolerant to the drug, or treatment given for a prolonged period). Continuous therapies are indicated with a "c". Risk of bias was generally high across studies due to the open-label study design. Overall survival (OS) Evidence suggests that treatment with RD (HR 0.63 (95% confidence interval (CI) 0.40 to 0.99), median OS 55.2 months (35.2 to 87.0)); TMP (HR 0.75 (95% CI 0.58 to 0.97), median OS: 46.4 months (35.9 to 60.0)); and VRDc (HR 0.49 (95% CI 0.26 to 0.92), median OS 71.0 months (37.8 to 133.8)) probably increases survival compared to median reported OS of 34.8 months with MP (moderate certainty). Treatment with VMP may result in a large increase in OS, compared to MP (HR 0.70 (95% CI 0.45 to 1.07), median OS 49.7 months (32.5 to 77.3)), low certainty). Progression-free survival (PFS) Treatment withRD (HR 0.65 (95% CI0.44 to 0.96), median PFS: 24.9 months (16.9 to 36.8)); TMP (HR 0.63 (95% CI 0.50 to 0.78), median PFS:25.7 months (20.8 to 32.4)); VMP (HR 0.56 (95% CI 0.35 to 0.90), median PFS: 28.9 months (18.0 to 46.3)); and VRDc (HR 0.34 (95% CI 0.20 to 0.58), median PFS: 47.6 months (27.9 to 81.0)) may result in a large increase in PFS (low certainty) compared to MP (median reported PFS: 16.2 months). Adverse events The risk of polyneuropathies may be lower with RD compared to treatment with MP (RR 0.57 (95% CI 0.16 to 1.99), risk for RD: 0.5% (0.1 to 1.8), mean reported risk for MP: 0.9% (10 of 1074 patients affected), low certainty). However, the CIs are also compatible with no difference or an increase in neuropathies. Treatment with TMP (RR 4.44 (95% CI1.77 to 11.11), risk: 4.0% (1.6 to 10.0)) and VMP (RR 88.22 (95% CI 5.36 to 1451.11), risk: 79.4% (4.8 to 1306.0)) probably results in a large increase in polyneuropathies compared to MP (moderate certainty). No study reported the amount of participants with grade ≥ 3 polyneuropathies for treatment with VRDc. VMP probably increases the proportion of participants with serious adverse events (SAEs) compared to MP (RR 1.28 (95% CI 1.06 to 1.54), risk for VMP: 46.2% (38.3 to 55.6), mean risk for MP: 36.1% (177 of 490 patients affected), moderate certainty). RD, TMP, and VRDc were not connected to MP in the network and the risk of SAEs could not be compared. Treatment with RD (RR 4.18 (95% CI 2.13 to 8.20), NMA-risk: 38.5% (19.6 to 75.4)); and TMP (RR 4.10 (95% CI 2.40 to 7.01), risk: 37.7% (22.1 to 64.5)) results in a large increase of withdrawals from the trial due to adverse events (high certainty) compared to MP (mean reported risk: 9.2% (77 of 837 patients withdrew)). The risk is probably slightly increased with VMP (RR 1.06 (95% CI 0.63 to 1.81), risk: 9.75% (5.8 to 16.7), moderate certainty), while it is much increased with VRDc (RR 8.92 (95% CI 3.82 to 20.84), risk: 82.1% (35.1 to 191.7), high certainty) compared to MP. Quality of life QoL was reported in four studies for seven different treatment regimens (MP, MPc, RD, RMP, RMPc, TMP, TMPc) and was measured with four different tools. Assessment and reporting differed between studies and could not be meta-analysed. However, all studies reported an improvement of QoL after initiation of anti-myeloma treatment for all assessed treatment regimens. AUTHORS' CONCLUSIONS: Based on our four pre-selected comparisons of interest, continuous treatment with VRD had the largest survival benefit compared with MP, while RD and TMP also probably considerably increase survival. However, treatment combinations of V, R, and T also substantially increase the incidence of AEs, and lead to a higher risk of treatment discontinuation. Their effectiveness and safety profiles may best be analysed in further randomised head-to-head trials. Further trials should focus on consistent reporting of safety outcomes and should use a standardised instrument to evaluate QoL to ensure comparability of treatment-combinations.

Acute kidney injury in children: A study of etiology, clinical profile, and short-term outcomes at the University of Abuja Teaching Hospital, Gwagwalada, Abuja, Nigeria.

A major hindrance in programs designed to reduce deaths from acute kidney injury (AKI) is that the extent and nature of AKI are often unknown. This article reports the etiology, clinical profile, and short-term outcomes of children managed for AKI at the University of Abuja Teaching Hospital, Gwagwalada, Abuja, Nigeria. Children aged one month to 15 years managed for AKI (identified by pediatric RIFLE criteria) from January 2017 to December 2017 were followed up for a short period of four weeks following the AKI. Multivariate Cox regression model was used to analyze the predictors of mortality. An annual prevalence of 26 AKI cases per 1000 children was recorded with 43 AKI cases from 1634 children seen during the 12-month period. The median age was 48 months. Twenty-two were males (51.2%). Sepsis (20, 46.6%), acute glomerulonephritis (5, 11.6%), diarrheal dehydration (5, 11.6%), severe falciparum malaria (4, 9.3%), and hemolyticuremic syndrome (4, 9.3%) were the major causes of the AKI. Fourteen children were managed conservatively, while 29 children that required dialysis had access to it. Thirteen children died (percentage mortality of 30.2%). The hazard of dying was eight times more in male gender [95% confidence interval (CI); 1.03-72.9, P = 0.017] and was lower in children without pulmonary edema by 0.14 (95% CI; 0.03-0.63, P = 0.01). In our setting, mortality from AKI is still high, and male children and those with pulmonary edema should be closely managed for AKI to reduce this high mortality.

Clinical and Laboratory Predictors of Frequency of Painful Crises among Sickle Cell Anaemia Patients in Nigeria.

INTRODUCTION: The severity of Sickle Cell Anaemia (SCA) in terms of frequency of painful Vaso-Occlusive Crises (VOC) may be affected by clinical and haematological parameters amongst others. Elucidation of these factors in a given disease prevalent environment is necessary for prompt and effective management of patients with frequent painful VOC. AIM: This study aimed at determining the clinical and laboratory predictors of frequency of painful VOC among SCA patients in Enugu, Southeastern Nigeria. MATERIALS AND METHODS: It was a cross-sectional study of 100 consecutive SCA patients receiving care at the University of Nigeria Teaching Hospital, Enugu, Nigeria between May 2012 and February 2014. The eligible patients were categorized into two groups namely; Group A and Group B. Group A/study group (severe disease) comprised SCA patients who had experienced three or more painful crises (≥3 crises) in the last one year preceding the study but, currently in steady state, while Group B/control group (mild-moderate disease), comprised SCA patients matched for age, sex, highest educational status, and occupation but who have had no painful crisis or had only one or two painful crises (0-2 crises) in the last one year preceding the study and currently in steady state. RESULTS: The overall mean age of the patients was 18.4±12.2 (range=2-52) years. The mean values of the haematological parameters including haemoglobin concentration, white cell count, platelet count, and neutrophil count were significantly higher in those with severe crises than mild-moderate crises (p<0.05). Sickle cell related complications including Avascular Necrosis (AVN) and leg ulcers were significantly higher in the study group than the control group (p<0.05). CONCLUSION: There was significant association between the frequency of crises and haemogblobin level, platelet and neutrophil counts and some clinical parameters: AVN, nephropathy and stroke. Future preventive interventions for reduction in frequency of crisis amongst patients with SCA could be targeted at controlling the blood levels of the identified haematological parameters.

Awareness, Knowledge, and Acceptance of Haematopoietic Stem Cell Transplantation for Sickle Cell Anaemia in Nigeria.

Background. Sickle cell anaemia (SCA) is an inherited condition whose clinical manifestations arise from the tendency of haemoglobin to polymerize and deform red blood cells into characteristic sickle shape. Allogeneic bone marrow transplantation offers a cure. The aim of this study was to determine the level of awareness, knowledge, and acceptance of this beneficial procedure in Nigeria. Materials and Methods. This multicentre cross-sectional study was conducted in 7 tertiary hospitals in Nigeria in 2015. Approval was obtained from each institution's research and ethics committee. A pretested structured questionnaire was administered to respondents aged 18 years and above and to the parents or guardians of those below 18 years of age. Results. There were 265 respondents comprising 120 males and 145 females. One hundred and seventy-one (64.5%) respondents were aware of BMT for the treatment of SCA. About 67.8% (116 of 171) of those who were aware believed SCA can be cured with BMT (p = 0.001) and 49.7% (85 of 171) of the respondents accepted BMT (p = 0.001). Conclusion. Awareness of BMT in Nigeria is low when compared with reports from developed countries. The knowledge is poor and acceptance is low. With adequate information, improved education, and psychological support, more Nigerians will embrace BMT.

Pattern and prevelence of alloimmunization in multiply transfused patients with sickle cell disease in Nigeria.

BACKGROUND AND STUDY OBJECTIVES: Blood transfusion is central in the prevention and treatment of certain chronic complications of sickle cell disease. It is indispensible in correcting anaemias as well as in the practice of exchange blood transfusion. These gains are largely limited by formation of allo-antibodies. Several studies demonstrated varying frequencies of allo-immunization in various patient groups. The effect of the racial differences between the donor and recipient pool, which has been subsumed in this study, has continuously created a confounding effect on the results of previous studies. AIM: This study was aimed at determining the pattern and frequency of allo-immunization in multiply transfused sickle cell patients, in a racially matched donor and recipient population. PATIENTS AND METHODS: This was a cross-sectional case-controlled study involving 80 Nigerian sickle cell disease patients who had received three or more units of packed red cells in the within 4 weeks of the study and 40 controls (who were SCD that had not been transfused in their life time). Antibody screening and identification was done using the Diamed microtyping system. RESULTS: Frequency of allo-immunization was determined to be 18.7 % (15/80) among the previously transfused and 5 % (15/120) in all sickle cell disease patients. Auto-antibodies were detected in 1.25 % of the study group and 2.5 % of the control, and all reacted with the Kell and Lutheran blood group antigens. The pattern of allo-antibodies found showed; 46.7 % Rhesus, 40 % Kell, while Lutheran and Duffy 13.3 %, each. CONCLUSION: Sickle cell disease patients are particularly susceptible to development of allo-antibodies despite racial similarities between the donor and recipient population. The most common allo-antibodies are Rhesus, Kell and Lutheran and Duffy respectively in order of decreasing frequency. Development of auto-antibodies seems to be independent of blood transfusion in sickle cell disease with possibly different pathogenetic mechanism. Policy on extended red cell phenotyping for common antigens will reduce allo-immunization among multiply transfused patients.

Sonographic evaluation of the spleen among sickle cell disease patients in a teaching hospital in Nigeria.

BACKGROUND: Regional variations in size and parenchyma echo-texture of the spleen among sickle cell disease (SCD) patients have been documented in various publications. The objectives of this study were to assess the size and parenchyma echo-texture of the spleen of SCD patients and ascertain the relationship of age, height and weight with the spleen sizes. METHODS: This was a cross sectional study involving 103 each of SCD and age matched control subjects. Aloka ST- 550 -3500 ultrasound machine with 3.5 and 5 MHz convex transducers was used to scan the subjects over a 15 months period (September, 2012 to November, 2013). The age, height and weight of each subject were recorded. RESULTS: The spleen sizes of SCD patients were generally larger than those of the controls (p < 0.05). Abnormal spleen parenchyma of varied appearances was found among the SCD subjects. There were negative correlations between mean spleen sizes and height, weight and age in SCD patients but positive correlations were found between them in the controls. CONCLUSION: Routine sonographic assessment of spleen size and echo-texture is useful in the management of SCD patients.

Priapism in homozygous sickle cell patients: important clinical and laboratory associations.

OBJECTIVE: To evaluate the relationship between the occurrence of priapism and important steady-state clinical and laboratory parameters in homozygous sickle cell disease (SCD). SUBJECTS AND METHODS: Steady-state clinical and laboratory data were obtained from the medical records of 126 male patients seen in the clinic over a 7-year period. Estimated prevalence rates, correlation coefficients and independent t tests were calculated to assess the relationship between priapism and several important clinical and laboratory indices. Patient data on age, haemoglobin concentrations, the frequency of crises per annum, leucocyte counts, platelet counts, serum bilirubin and aspartate transaminase were evaluated. RESULTS: The prevalence of priapism was determined to be 21.4%, and 22.2% of those affected had erectile dysfunction. There was a significant positive correlation between priapism and older age (p = 0.049) and lower leucocyte counts (p = 0.008). There was no significant relationship with other clinical or laboratory indices. CONCLUSION: About 1 in 4 of all homozygous older SCD patients had priapism, and an approximately similar ratio developed erectile dysfunction; they also had lower steady-state leucocyte counts. Other clinical and laboratory indicators of disease severity in SCD did not positively correlate with the occurrence of priapism, and this may imply an alternative pathogenetic mechanism.

Addition of multimodal therapy to standard management of steady state sickle cell disease.

Most people on folic acid to boost erythropoiesis and prophylactic antimicrobials, the standard management of steady state sickle cell disease (SCD), have unacceptable numbers of crises. The objective of this study was to evaluate the effects of adding multimodal therapy with potassium thiocyanate and omega-3 fatty acids to the standard management of steady state SCD. Pre- and post-treatment numbers of crises and other disease indices were compared in 16 HbSS individuals on folic acid and paludrine after 12 months of adding eicosapentaenoic acid 15 mg/kg/day, docosahexaenoic acid 10 mg/kg/day, and potassium thiocyanate 1-2 mL/day, each milliliter of which contained 250 mg of thiocyanate and 100 micrograms of iodine to prevent hypothyroidism: a possible side-effect due to competitive inhibition of the transport of iodide into the thyroid gland by thiocyanate. Median number of crises reduced from 3/yr to 1/yr (P < 0.0001). There was no evidence of impaired thyroid function. Plasma level of tri-iodothyronine improved (P < 0.0001). Steady state full blood count and bilirubin level did not change significantly. The findings suggest that addition of potassium thiocyanate and eicosapentaenoic and docosahexaenoic acids to standard management of steady state SCD reduces the number of crises. This observation needs to be evaluated in larger studies.

Increased prevalence of activated protein C resistance during pregnancy may implicate venous thrombo embolic disorders as a common cause of maternal mortality in Nigeria

Background: Acquired resistance to protein C in pregnancy has been established as one of the factors associated with thromboembolic phenomenon, an important cause of maternal mortality and morbidity. Objectives: To establish the mean levels of PCA ratio (measure of protein C resistance) of among our pregnant women since maternal mortality rate of the country is on the increase despite efforts to reduce this trend. Materials and Methods: A prospective study was carried out in a tertiary institution in Enugu State, Southeastern Nigeria over the 7 months period from May 2010 to November 2010. Two hundred pregnant women and 50 non pregnant female controls were recruited and PCA ratio, (coagulometric assay) were determined. Results: There was a non significant difference between the mean and standard deviation PCA ratio of the female non pregnant controls and pregnant women in 2nd trimester 4.32±0.4 and 4.30±0.4 respectively. A significant difference was noted between the controls and pregnant women in 3rd trimester 4.32±0.4 and 3.87±0.5 respectively also between the pregnant women in their 2nd and 3rd trimester 4.30±0.4 and 3.87±0.5 respectively. Conclusion: There is increased protein resistance C in our pregnant women. This may implicate thromboembolic disorders as one of the leading causes of increase maternal mortality despite a downward trend in the prevalence of post partum haemorrhage

Pilot study of omega-3 fatty acid supplements in sickle cell disease.

In a previous retrospective study, it was observed that the greater the amounts of the omega-3 fatty acids eicosapentaenoic acid (EPA) and docosahexaenoic acid (DHA) in the blood, the lesser the number of complications of sickle cell disease (SCD) and the higher the steady state haemoglobin level. SCD causes ischaemia-reperfusion injury and inflammation; which can be ameliorated by a metabolite of DHA that down-regulates expression of pro-inflammatory genes. The objectives of this prospective pilot study were to evaluate the effects of DHA and EPA supplements in SCD, and test the hypothesis that these effects are mediated partly by reducing inflammation. Oral DHA and EPA supplements were given to 16 SCD patients for 6 months. We then compared pre- and post-supplementation values of number of crisis, steady state Hb, plasma unconjugated bilirubin and three indices of inflammation: plasma interleukin-6, blood neutrophil and platelet counts. There was a significant reduction in the plasma level of unconjugated bilirubin, and the number of sickle cell crisis; but not in the markers of inflammation. The pilot data suggest that DHA and EPA supplements reduce the number of crisis and steady state haemolysis in SCD; but provide no evidence that these effects are mediated by reducing inflammation.

Challenges for allogeneic hematopoietic stem cell transplantation in chronic myeloid leukemia in the era of tyrosine kinase inhibitors.

Following the introduction of the tyrosine kinase inhibitor (TKI) imatinib in the treatment of chronic myeloid leukemia (CML) patients, the allogeneic hematopoietic stem cell transplantation (HSCT) scene in CML has changed dramatically. The number of patients receiving HSCT in first chronic phase (CP) has declined rapidly, as allogeneic HSCT in CP is now performed in these patients only in case of failure or intolerance of TKIs. Second, those CML patients who undergo allogeneic HSCT represent a selection of high-risk patients due to more advanced disease with high rates of accelerated or blast phase (being associated with an increased relapse risk), advanced age and relevant co-morbidities. Efforts at meeting these special challenges are being developed: treatment with TKIs aims to improve the pre-transplant remission status before HSCT. Dose-reduced conditioning protocols were introduced to decrease transplant-related mortality in patients with co-morbidities or older age. In the post-transplant period, TKIs may be administered for prophylaxis and for treatment of post-transplant relapse. Still, the outcome of patients in advanced CML phases remains guarded, and requires an improvement in current transplant strategies.

Ophthalmic manifestations of leukemia in a tertiary hospital population of adult nigerian africans.

PURPOSE: To determine the prevalence and pattern of leukemic ophthalmopathy among adults at the University of Nigeria Teaching Hospital (UNTH), Enugu, south-eastern, Nigeria. MATERIALS AND METHODS: This prospective, observational case series surveyed adult leukemia patients presenting at UNTH's departments of Hematology/Immunology and Ophthalmology from July 2003 to August 2008. The demographic profile, clinical data from for each individual in the cohort were statistically collated and analyzed. A P <0.05 was considered as statistically significant. RESULTS: There were 72 participants (45 males and 27 females), aged 32.7 ± 9.8 years (range, 18 years to 72 years). Leukemic ophthalmopathy was present in 77.8% of subjects. The leading ophthalmic manifestations of leukemia were retinal vascular abnormalities in 50.0% of subjects, conjunctival pallor in 27.8% of subjects, sub-conjunctival hemorrhage in 19.4% of subjects, and retinal hemorrhage in 16.7% of subjects. Ocular co-morbidity was present in 47.2% of subjects. Vision loss occurred in 37.5% of subjects, of which 32.1% was leukemia related, and the remaining due to ocular co-morbidity. Leukemic ophthalmopathy was more prevalent in chronic leukemia (P <0.05), frequently affected the ocular posterior segment (P < 0.05), and often resulted from secondary hematologic complications (P <0.05). There was no gender difference in the prevalence of leukemia (P = 0.0822) or leukemic ophthalmopathy (P = 0.6624). CONCLUSION: The prevalence of leukemic ophthalmopathy in Enugu is high. It is often associated with significant ocular co-morbidity and vision loss. These have implications for clinicians involved in leukemia management. Early diagnosis and regular ophthalmic examinations are recommended to optimize treatment outcomes.

Second allogeneic stem cell transplantation in myeloid malignancies.

For patients with myeloid malignancies who relapse after allogeneic stem cell transplantation (allo-SCT), one salvage option is a second SCT. We retrospectively analyzed outcomes of the second allo-SCT in 25 patients who received at least 2 allografts from related/unrelated donors due to relapse of acute myeloid leukemia, myelodysplastic syndrome or myelofibrosis after the first SCT. A minority of the acute myeloid leukemia/myelodysplastic syndrome patients had reached complete hematological remission before the second SCT (6/25, 24%). Reduced conditioning strategies were performed in the majority (n = 23). Complete remission was achieved in all 21 cases with available data after the second SCT, but relapse was seen in 11/25 patients (44%). After a median follow-up of 18 months (range 6-47), 8/25 patients (32%) were still alive, and of those, 6 (24%) were in stable remission. In 9 cases mortality was associated to relapse and in 8 cases to transplant-related causes (treatment-related mortality; 8/25, 32%). In conclusion, a second SCT offers the chance of stable remission for some patients relapsing with a myeloid malignancy after a first allo-SCT, although high treatment-related mortality and relapse rates remain a problem. Efforts should concentrate on an optimization of conditioning strategies, immunosuppression and post-transplant surveillance for this specific situation.