ADVERSE PREGNANCY OUTCOMES IN SICKLE CELL TRAIT: A PROSPECTIVE COHORT STUDY EVALUATING CLINICAL AND HAEMATOLOGICAL PARAMETERS IN POSTPARTUM MOTHERS AND NEW-BORNS.

Abstract – Sickle cell trait (SCT) is a condition caused by the inheritance of a single allele of the abnormal haemoglobin beta gene, HbS. Carriers of SCT are generally asymptomatic, and they do not manifest the haematological and clinical abnormalities of sickle cell anaemia (SCA). However, there is evidence that they display some of the symptoms in stressful situations. Pregnancy is a stressful physiological event, and it is not clear if SCT adversely affects pregnancy outcomes, particularly those from the developing countries, who regularly suffer from nutritional insufficiency. Objective – This study aims to investigate pregnancy outcomes in Sudanese women with SCT. Subjects and methods – Pregnant women with (HbAS, n=34) and without (HbAA, n=60) SCT were recruited during their first trimester by El Obeid Hospital, Kordofan, Western Sudan. Ethical approval from the Faculty of Medicine, University of Khartoum, and informed consent from the participants were obtained. Detailed anthropometric, haematological, clinical, obstetric and birth outcome data were documented. Blood samples were collected at enrolment and delivery. Results – At enrolment, the first trimester, the SCT group did not manifest SCA symptoms, and there was no difference in any of the haematological parameters between the SCT and control groups. At delivery, the women with SCT compared with the control group had lower levels of haemoglobin (Hb, p=0.000), packed cell volume (PCV, p=0.000), mean corpuscular haemoglobin (MCH, p=0.002) and neutrophil counts (p=0.045) and higher mean corpuscular volume (MCV, p=0.000) and platelet counts (p=0.000). Similarly, at delivery, the babies of SCT women had lower birth weight (p=0.000), lower Hb (p=0.045), PCV (p=0.000), MCH (p=0.000) and higher neutrophil (p=0.004) and platelet counts (p=0.000) than the babies of the healthy control group. Additionally, there were more miscarriages, stillbirths and admissions to the Special Care Baby Unit (SCBU) in the SCT group. Conclusions – The study revealed that SCT is associated with adverse pregnancy outcomes, including maternal and neonatal anaemia, low birth weight and increased risk of still birth, miscarriage and admission to SCBU. Therefore, pregnant women with SCT should be regarded as a high-risk group, and given pre-conceptual advice and multidisciplinary antenatal and postnatal care

Accuracy of Platelet Counting by Optical and Impedance Methods in Patients with Thrombocytopaenia and Microcytosis

Objectives: Obtaining accurate platelet counts in microcytic blood samples is challenging, even with the most reliable automated haematology analysers. The CELL-DYN™ Sapphire (Abbott Laboratories, Chicago, Illinois, USA) analyser uses both optical density and electronic impedance methods for platelet counting. This study aimed to evaluate the accuracy of optical density and electrical impedance methods in determining true platelet counts in thrombocytopaenic samples with microcytosis as defined by low mean corpuscular volume (MCV) of red blood cells. Additionally, the impact of microcytosis on platelet count accuracy was evaluated. Methods: This study was carried out between February and December 2014 at the Haematology Laboratory of the Sultan Qaboos University Hospital in Muscat, Oman. Blood samples were collected and analysed from 189 patients with thrombocytopaenia and MCV values of <76 femtolitres. Platelet counts were tested using both optical and impedance methods. Stained peripheral blood films for each sample were then reviewed as a reference method to confirm platelet counts. Results: The platelet counts estimated by the impedance method were on average 30% higher than those estimated by the optical method (P <0.001). The estimated intraclass correlation coefficient was 0.52 (95% confidence interval: 0.41–0.62), indicating moderate reliability between the methods. The degree of agreement between methods ranged from -85.5 to 24.3 with an estimated bias of -30, suggesting that these methods generate different platelet results. Conclusion: The impedance method significantly overestimated platelet counts in microcytic and thrombocytopaenic blood samples. Further attention is therefore needed to improve the accuracy of platelet counts, particularly for patients with conditions associated with microcytosis

Duplication 1q as primary and 3q in t(3;13) as secondary aberration in Fanconi anemia: Implications and literature review

Fanconi anemia (FA) is a genetic disorder of chromosomal instability. One of the routine methods of confirmation for FA includes chromosome breakage analysis, using crosslink-inducing agents. Bone marrow karyotyping at diagnosis and further follow-up will aid in detecting aberrations. If present, they might be indicative of a possible karyotype evolution, leading to poor prognosis. We report a FA patient with two distinct karyotypes: 46,XY,dup(1)(q21q31) in the first diagnostic sample and 46,XY,dup(1)(q21q31),der(13)t(3;13)(?q26;p12) in the second, after a span of two years. We discuss the possible karyotypic evolution, the implications of chromosome 13 involvement and regions/genes on chromosomes 1q and 3q in FA. The importance of periodic examination of bone marrow in these patients, for detection of cryptic aberrations which might lead them to MDS/AML, is also discussed

Can Gestational Anemia be Alleviated with Increased Awareness of its Causes and Management Strategies? Implications for Health Care Services

Objectives: We conducted this study to assess the risk factors of gestational anemia and evaluate the effectiveness of a culturally-tailored nutrition educational intervention on hemoglobin (Hb) status among pregnant Omani women. Newborn birthweight was used as a birth outcome. Methods: The study was conducted in two phases. The first phase investigated the risk factors associated with gestational anemia in 206 Omani women who were ≥ 20 years old and had at least completed 12 weeks of gestation. A suitable sample was recruited at a tertiary teaching hospital in Muscat, Oman. Hb status was recorded, and backward linear regression was used to analyze the demographic and obstetric variables associated with Hb levels. In the second phase, a specially designed culturally-tailored nutrition educational intervention was delivered to women in the study group by trained research assistants whereas women in the control group received routine care only. The Hb levels of the pregnant women and birth weight of newborns after the intervention were evaluated in the second phase of the study. Results: The prevalence of gestational anemia among 206 pregnant Omani women was 41.7%. A significant negative relationship was found between Hb and parity whereas a positive relationship was found between Hb and gestational age. The Hb level increased as the gestational age advanced (β = 0.31, p < 0.050) and decreased as the parity increased (β = -0.22, p < 0.050). The pre-post mean difference of Hb levels in the study group was 11.0 g/dL and in the control group was 10.7 g/dL. The difference between the pre- and post-test Hb levels for the study group was significant (t = 3.58, p = 0.001), indicating that the culturally-tailored nutrition education intervention was effective in improving the Hb level in pregnant Omani women. No significant difference was found between the study and control group with respect to birth outcomes. Conclusion: The prevalence of gestational anemia is high in pregnant Omani women. The use of a specially designed culturally-tailored nutrition education intervention for pregnant women supplemented with follow-up reminders can reduce the occurrence of gestational anemia. Such programs are ultimately necessary in light of the high prevalence of gestational anemia in developing countries

First Implementation of Transfusion Consent Policy in Oman : Audit of compliance from a tertiary care university hospital

Objectives: Transfusions are a common medical intervention. Discussion of the benefits, risks and alternatives with the patient is mandated by many legislations prior to planned transfusions. At the Sultan Qaboos University Hospital (SQUH), Muscat, Oman, a written transfusion consent policy was introduced in March 2014. This was the first time such a policy was implemented in Oman. This study therefore aimed to assess adherence to this policy among different specialties within SQUH. Methods: The medical records of patients who underwent elective transfusions between June and August 2014 were reviewed to assess the presence of transfusion consent forms. If present, the consent forms were examined for completeness of patient, physician and witness information. Results: In total, the records of 446 transfused patients (299 adult and 147 paediatric patients) were assessed. Haematology patients accounted for 50% of adult patients and 71% of paediatric patients. Consent was obtained for 75% of adult and 91% of paediatric patients. The highest adherence rate was observed among adult and paediatric haematology specialists (95% and 97%, respectively). Consent forms were correctly filled out with all details provided for 51% and 52% of adult and paediatric patients, respectively. Among inadequately completed forms, the most common error was a lack of witness details (20–25%). Conclusion: In most cases, the pre-transfusion consent policy was successfully adhered to at SQUH. However, further work is required to ensure full compliance with the consent procedure within different specialties. Implementation of transfusion consent in other hospitals in the country is recommended

Comparative analysis of four methods for enumeration of platelet counts in thrombocytopenic patients

Background: Even with the most reliable automated blood cell analyzers, obtaining an accurate platelet count in patients with thrombocytopenia is still challenging especially when making decisions for platelet transfusions. The Cell-Dyn Sapphire offers three methods for platelet counting including the optical, impedance, and immunological techniques. Aim: A comparative analysis evaluating the performance of these three methods, along with the manual technique, was performed in thrombocytopenic patients. Materials and Methods: A total of 32 blood samples from patients without a history of chemotherapy and a platelet count <50 ×- 109/l were tested in parallel by four different methods. Results: Compared to other techniques, impedance method provided an overestimation of platelet count (P = 0.0008) and failed to show a result in 15% of cases with low platelet counts (<15 ×- 109/l). Good to excellent correlations and reliability values were evidenced among study methods, but a poor reliability was noticed between the impedance and immunological methods with an intraclass correlation coefficient of 0.49 (confidence interval: 0.15–0.73; P = 0.003). In the bias analysis, the impedance method showed the highest levels of disagreement with other techniques. Summary/Conclusion: Collectively, these results provide evidence that the optical or immunological technique appear to be superior to the impedance method in estimating low platelet counts with the automated analyzer Cell-Dyn Sapphire. As such, the healthcare staff and the physicians must be aware of this limitation, especially in the presence of severe thrombocytopenia, when a decision of platelet transfusion has to be made

Genital Ulcer Development in Patients with Acute Promyelocytic Leukaemia Treated with All-Trans Retinoic Acid: A Case Series

We report here four cases of genital ulcers that developed after the administration of all-trans retinoic acid (ATRA) for the treatment of acute promyelocytic leukemia (APL). Between October 2007 and March 2010, three males and one female (age range 19-35 years) were identified to have genital ulcers after being prescribed all-trans retinoic acid (ATRA) as a part of chemotherapy for APL. This is the first series of cases describing genital ulcers, as a unique and rare complication of ATRA used for treatment of APL in these patients, with no other cause identified. Following temporary cessation of ATRA for a few days in these three cases, improvement of the ulcers was noted

Myelomatous Pleural Effusion: Case report and review of the literature

Plasma cell myeloma is an uncommon disease which, besides primarily involving the bone marrow, has a tendency to involve other organs thus presenting with different clinical manifestations. While pleural effusions are infrequent in this disease, true myelomatous pleural effusions are extremely rare. We report the case of a middle-aged Omani man with relapsed plasma cell myeloma who developed bilateral pleural effusions. The diagnosis of myelomatous pleural effusion was made by finding many abnormal plasma cells as well as a high level of a monoclonal protein (IgG κ) in the pleural fluid. In spite of a good initial response to therapy, the patient had progressive disease and died 6 months later with bacterial sepsis. We present a review of the literature that indicates the rarity of such a manifestation and its association with poor prognosis and short survival

Biclonal Gammopathy in Chronic Lymphocytic Leukemia: Case Report and Review of the Literature

Monoclonal gammopathies are frequently seen in B-cell malignancies. Monoclonal proteins are seen in a significant proportion of patients with chronic lymphocytic leukemia (CLL), which is a clonal disorder of mature B cells. The use of more sensitive laboratory methods has enabled the detection of monoclonal proteins or light chains in the serum and/or urine in the majority of these patients. The presence of some of these monoclonal proteins may explain the different autoimmune phenomena that are associated with this disease. Some reports indicate that the finding of monoclonal proteins has a negative impact on patients’ survival. The presence of two different monoclonal proteins (i.e. biclonal gammopathy) is on the other hand rare. Most of the reported cases in the literature are of patients with plasma cell disorders. In this report, we describe a rare occurrence of biclonal gammopathy in a patient with CLL. Serum protein electrophoresis and immunofixation, which were negative at the time of initial diagnosis, showed biclonal immunoglobin A (IgA) kappa and IgA lambda during the course of the disease. The patient’s disease showed steady progression, despite multiple treatments. Although this could just be the result of using more sensitive laboratory techniques, biclonal gammopathy in this patient likely reflects the evolution of another clone, which would explain the encountered resistance to therapy. Because of paucity of reports, the impact of biclonal gammopathies in such patients is not known and an effort to collectively report the presentation and outcome of these patients is needed to further understand the pathophysiology and clinical significance of such a finding