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   Table of Contents - Current issue
July-December 2019
Volume 8 | Issue 2
Page Nos. 53-90

Online since Thursday, October 17, 2019

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Acute chest syndrome in sickle cell disease patients: Experience from a resource constrained setting p. 53
Adeseye M Akinsete, Omotola Majiyagbe, Adetokunbo Joacquim, Edamisan O Temiye, Adebola O Akinsulie
BACKGROUND: Sickle cell anemia is the most common inheritable hemoglobin disorder in the world with very high prevalence, morbidity, and mortality in Sub-Saharan Africa. Acute chest syndrome (ACS) is one of the most common causes of mortality among individuals with the condition. The management of this condition involves watchful waiting which may be deleterious in resource-constrained settings. The use of exchange blood transfusion (EBT) has been reported to be beneficial. The use of this great intervention is even further hampered in most of the developing world. The aim of this study was to review the outcome data of children managed with an algorithm adopted in 2015 at a University Teaching Hospital in a resource-constrained setting. MATERIALS AND METHODS: This was a retrospective study from January 2015 to December 2017 at a University Teaching Hospital. Ethical approval was obtained from the Hospital's Health, Research, and Ethics committee. RESULTS: A total of 324 children with sickle cell disease were admitted in the period under review with a male-to-female ratio of 1.5:1. Thirty-three patients were suspected of having ACS, but only 12 were confirmed using the algorithm. The mean age at presentation was 8 years with over 85% of the patients having a triad of fever, cough, and chest pain. All the patients had EBT within 48 h of admission and mortality rate recorded was 16.7%. CONCLUSION: The use of the algorithm as well as the preemptive EBT improved the outcome of the children accessing care at our facility.
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Value of the Matos and Carvalho index for thalassemia trait detection, experience of single hematological center in Iraq p. 58
Safa A Faraj, Ahmed I Ansaf, Laith S Mahdi
BACKGROUND: Thalassemia trait and other low red cell index (LRCI) diseases commonly have same presentation with microcytic hypochromic anemia. Most of beta thalassemia minor (TM) people are subclinical and without specific investigation may be undiagnosed or treated as iron-deficiency anemia. Thalassemia carriers may be undiagnosed, which in turn leads to severe forms of thalassemia syndromes with poor premarital counseling in high-prevalence areas. Many trials tried to find simple diagnostic tools to differentiate between thalassemia traits and other microcytic anemia depending on blood discriminative indices that can be found in limited resource places and routine clinics using blood cell count parameters. The aim was to assess the value of Matos and Carvalho index (MCI) in detecting TM from patients presented with microcytic anemia. PATIENTS AND METHODS: The study was carried out on 171 patients who were diagnosed as cases of hypochromic microcytic anemia in Kut Hemato-oncology Center. By Measuring hematological parameters using five automated red cell discriminative indices (red blood cell (RBC) count, RBC distribution width, Shine and Lal index, MCI index, and Mentzer index [MI]) with measuring hemoglobin (Hb) A2 levels using Hb variant B thalassemia short arm program. RESULTS: Of 171 patients screened for TM, 108 patients were diagnosed as TM by Hb electrophoresis. Patients with TM presented with the mean age of 25.3 years, while the mean of age in patients with other LRCI anemia was 6.2 years. RBC count was the best index of correctly identifi ed patients as 84%, followed by MI and MCI with 74% and 72%, respectively. Furthermore, the RBC count was the best indicator Youden's indices (58.2), with high sensitivity for BT (96.3%) followed by MI with Youden's index (38). Wide thalassemia mutation play important role in this issue. CONCLUSION: RBC count are simply accessible and dependable ways for identifying beta thalassemia trait, but there are no red cells indices and methods have 100% specificity, efficacy, and sensitivity for the differentiation beta TM from other hypochromic microcytic anemia which may be due to wide thalassemia mutations.
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Assessment of anthracycline-induced long-term cardiotoxicity in patients with hematological malignancies p. 63
Aws Sabah Al-Rubaye, Ahmed S Noori, Tareq Abdullah Saleh, Ibrahim Khalil Ibrahim
BACKGROUND: Anthracyclines are antibiotic antineoplastic drugs used in the treatment of various hematological and solid malignancies. They are well-known for their cardiotoxic side effects which are related to their cumulative dosages. Many investigations are used to detect such cardiotoxicity, the gold standard of which is the endomyocardial biopsy that has a limited use because it is an invasive procedure. The aims of this study were to explore the cardiotoxicity induced by anthracyclines in patients with hematological malignancies by using electrocardiography (ECG) and echocardiography and to determine relationship between the detected changes in ECG and echocardiography, with some demographic and clinical variables. PATIENTS AND METHODS: A cross-sectional study of fifty patients was recruited at Al-Imamein Al-Kadhemein Medical City from March 2014 to January 2015. ECG and echocardiography were done to the patients to study several parameters such as ejection fraction (EF), diastolic dysfunction, QRS voltage, QRS duration, and corrected QT (QTc) both at baseline and reassessment. Reassessment was done after completing induction for leukemia patients and at mid-term reassessment for lymphoma patients. RESULTS: ECG showed reduction in QRS voltage, increase in QRS duration, and increase in QTc, all of which showed statistical significance and may reflect the effect on the depolarization and repolarization on the myocardium. The echo study showed the development of systolic left ventricular (LV) dysfunction (EF <55%) in 12% of patients and a statistically significant reduction in the mean of EF of the study group. It also showed statistically significant development of new diastolic dysfunction. Statistically significant association between reduction of QRS voltage (>30% from baseline) with the development of LV dysfunction was also found. CONCLUSIONS: Significant changes in QRS voltage, QRS duration, QTc, as well as the occurrence of LV systolic dysfunction and diastolic dysfunction were noted. The mean cumulative dose at which LV systolic dysfunction occurred was about 250 mg/m2.
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Acute myeloblastic leukemia: Important clinical and epidemiological facts from Hiwa Hospital in Sulaimaniyah, Iraq p. 69
Shwan Ali Tawfiq, Ahmed Khudair Yassin, Hisham A AlGetta, Kawa Muhamedamin Hasan
BACKGROUND: Acute myeloblastic leukemia is the most common acute leukemia in adults, with both diagnostic and therapeutic challenges. It has a poor treatment outcome both locally and internationally. The aim of this study was to investigate some epidemiological aspects and treatment outcome of AML patients treated in single center. PATIENTS AND METHODS: A retrospective observational study extended from February 2013 to February 2018 and included 98 patients who referred to Hiwa Hematology Oncology Hospital in Sulaymaniyah city. All patients had met the diagnostic criteria by flow cytometry for de novo acute myeloid leukemia (AML). Electronic records of all patients were reviewed carefully. RESULTS: Ninety-eight patients (46 females and 52 males) with a mean age of 42 years were included the study, with new diagnosis of AML, most of the patients were below the age of 60 years (81%), while only 19% were 60 years of age or older. The most frequent subtype is AML M3 (n = 25; 25.5%) of patients, followed by M1, M2 (n = 16; 16.3%) each, and M5 (n = 15; 15.3%) with M4, M0, M6, M7, and acute leukemia of ambiguous lineage comprising the remainder, respectively. The clinical features at the diagnosis of the 98 patients included pallor in 91 patients (92.9%), easy fatigability in 82 patients (83.6%), while bleeding tendency was present in 46 patients (46.9%) in the form of ecchymosis, petechial hemorrhage, epistaxis, or abnormal vaginal bleeding. Fever was present in 85 patients (86.7%), while pain in the form of headache, generalized body ache, or bone pain was initially manifested in 29 patients (29.6%). The survival of our patients at 1 year is (88%) M3 patients, while it was (58%) for non-M3 patient. CONCLUSIONS: Clinical and epidemiological characteristics were different in some aspect, while comparable in other when compared to published studies. treatment outcome and survival data were comparable to international data.
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Blood cell activation as an indicator of prothrombogenesis in polycythemia vera: Case control study p. 74
Ibrahim A Mahmood, Farqad B Hamdan, Waseem F Al-Tameemi
BACKGROUND: Polycythemia vera (PV) is a chronic myeloproliferative stem cell disease usually combined with JAK2 mutation. Active JAK2 mutation directly promote platelets and granulocytes activation and indirectly initiate endothelial activation. Such interactions can provoke endothelial injury with subsequent release of procoagulant factors. The aim of this study was to understand of this study is to understand the pathophysiology of increased thrombosis in PV in terms of the effect of JAK2V617F gene mutation in relation to the intensity of the neutrophil CD11b and platelet CD62P. SUBJECTS AND METHODS: A group of 53 patients was examined comprised 30 PV patients and 23 with secondary polycythemia. In parallel, 30 aged- and sex-matched, nonsmokers healthy volunteers served as a control group. Hemoglobin level, packed cell volume, white and red blood cells count, mean corpuscular volume, and platelet count were estimated for all the 83 participants. Flow cytometry for the detection of neutrophil CD11b and platelet CD62P were done for all the participants; whereas quantitative polymerase chain reaction technique for the assessment of JAK2 mutation rate was done for the 30 PV patients only. RESULTS: We were able to detect significantly higher neutrophil CD11b and platelet CD62P expressions in PV patients. Within PV, JAK2 mutation rate was significantly higher in those with a history of thrombosis. A positive relationship was demonstrated between the JAK2 mutation rate and each of CD11b and CD62P. CONCLUSION: There is an overexpression of neutrophil CD11b and platelets CD62P in patients with PV which can be considered as a marker of procoagulant activity in blood cells. Active JAK2 mutation directly promote platelets and granulocytes activation and indirectly initiate endothelial activation and in turn endothelium into a proadhesive and procoagulant surface.
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A female with classical paroxysmal nocturnal hemoglobinuria misdiagnosed as megaloblastic anemia p. 81
Sana D Jalal, Shahla M Saeed, Nawshirwan G Rashid, Abdulhamid S Saeed
Paroxysmal nocturnal hemoglobinuria (PNH) is a rare acquired disorder of the hematopoietic stem cells, which manifests as bone marrow failure, hemolytic anemia, and thrombosis. PNH affects both sexes equally and may present at any age, although it is most often diagnosed in young adulthood. Here, we report a case of a middle-aged woman, presented with one and a half year history of anemia, significant fatigue, backache, and persistent high erythrocyte sedimentation rate. The patient has no organomegaly and did not give a history of thrombotic events previously. She was considered as a case of megaloblastic anemia at another center and treated accordingly. Her full blood count showed mild macrocytic anemia with slight polychromasia. The hemolytic anemia workup revealed slightly raised serum bilirubin (unconjugated) and undetectable haptoglobin with hemosiderin in urine. Ham's test was positive, and the diagnosis of PNH was confirmed by flow cytometry. The patient was treated with short-course steroids and folic acid, and her general condition has become stable.
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Peutz–Jeghers syndrome: An unusual cause of iron-deficiency anemia p. 84
Pakesh Baishya, Evarisalin Marbaniang, Jaya Mishra, Donkupar Khongwar, Dathiadam Tongper, Pranjal Kalita
Peutz–Jeghers syndrome (PJS) is a rare disorder, which is inherited as autosomal dominant. It is characterized by mucocutaneous melanotic spots and multiple hamartomatous polyps with an increased risk of cancer predispositions. PJS can present with complications such as iron-deficiency anemia as a result of bleeding from the gastrointestinal polyps and intestinal obstruction and/or intussusception if the polyps are of large size. We present a case of PJS with iron-deficiency anemia in a 16-year-old boy as a result of occult gastrointestinal bleeding from the hamartomatous gastrointestinal polyps.
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Anemia with erythroid hyperplasia: An unusual presentation associated with parvovirus infection p. 87
Kriti Chauhan, Nikhil Shandilya, Varun Hatwal
Most of the patients with parvovirus B19 infection manifest with anemia and erythroid hypoplasia in bone marrow when the immunity of host is not competent enough to kill the virus like in posttransplant patients on immunosuppressive drugs and immunodeficiency syndromes. Herein, we present a case of parvovirus infection in an immunocompetent patient presenting with anemia, showing erythroid hyperplasia with hemophagocytosis in bone marrow, and also discuss the pathogenesis with review of literature in such a rare presentation.
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Dimorphic anemia and cytopenias p. 90
Prita Pradhan
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