Hodgkin’s Lymphoma (HL) reveals variable epidemiological and clinico-pathological features in different geographical locations. In this retrospective study, we aimed to assess the epidemiological and clinic-pathological features, and outcome of HL patients treated at one hemato-oncology centre in Erbil, northern Iraq. Medical records of 103 HL patients treated over more than six years were reviewed. Treatment outcome was evaluated by measuring the 5-year overall and progression-free survival rates. The median age of patients was 23 years, children up to 17 years constituted 31.1%, and male to female ratio was 1:1.05. The majority (96.1%) of patients presented with lymphadenopathy. Nodular sclerosis subtype was the mostly encountered histologic type (48.5%); about half of the patients (49.5%) had stage II disease. Relapse occurred in 20 patients; the 5-year overall survival for children was better (89%) compared to adult patients (79%). The associated risk features found to have adverse effects on the survival, however, only high LDH level and presence of B-symptoms at presentation showed significant correlation. The epidemiological and clinical characteristics of HL in our locality followed the pattern in the western world. The 5-year overall and progression-free survivals were far below the international rates, a matter which may necessitate a revision to HL treatment strategy at our centre.
β-Thalassemia intermedia (β-TI) is a clinical term describing a range of clinical phenotypes that are intermediate in severity between the carrier state and β-thalassemia major (β-TM). To characterize the molecular basis of β-TI in Erbil Province, Northern Iraq, 83 unrelated patients were investigated. Detection of β-globin gene mutations was carried out by reverse hybridization assay and direct gene sequencing. All patients were screened for the XmnI polymorphism by direct sequencing of HBG2 ((G)γ promoter gene). Detection of α-globin gene deletions and triplication was carried out using the reverse hybridization assay. Four main molecular patterns were identified in association with the β-TI phenotype, namely: β(+)/β(+) (38.5%), β(+)/β(0) (21.6%), β(0)/β(0) (31.3%), and β(0)/wild type (8.4%). IVS-I-6 (T > C) was the most frequently encountered mutation (55 alleles, 34.6%), followed by IVS-II-1 (G > A) and codon 8 (-AA); furthermore, we report for the first time from Iraq two β(+) mutations, -87 (C > G) and 5' untranslated region (5'UTR) +22 (G > A). The XmnI polymorphism was detected in 47.0% of patients, mainly in association with the β(0)/β(0) genotype. The α-globin gene deletions were encountered in four cases, including one case with (- -(FIL)) double gene deletion, a report that is the first from our country. The α-globin gene triplication was detected in five of the seven heterozygous β-thalassemia (β-thal) patients. Similar to other Mediterranean countries, inheritance of mild β-globin mutations was the main molecular pattern underlying β-TI in our patients followed by the ameliorating effect of the XmnI polymorphism.
Background HbH disease results from dysfunction of three, less commonly two, α-globin genes through various combinations of deletion and non-deletion mutations. Characterization of the mutations and the underlying genotypes is fundamental for proper screening and prevention of thalassaemia in any region. The aim of this study was to explore the genetic arrangements of HbH disease and to correlate the genotypes with the clinical phenotypes. Methods A total of 44 HbH disease patients were enrolled in this study. They were clinically and haematologically assessed. The patients were tested for 21 common α-globin gene mutations using multiplex PCR and reverse hybridization. According to the genotype, the patients were categorized into two separate sub-groups, deletion and non-deletion types HbH disease. Results Within the studied HbH disease patients, eight different α-globin gene mutations were detected in nine different genetic arrangements. The --MED and -α3.7 deletions were the two most frequently encountered mutations (37.5 and 35.2% respectively). Patients with deletion genotypes constituted 70.4%. The most common detected genotype was --MED/−α3.7 (59.1%), followed by αpoly-A1α/αpoly-A1α (13.6%). For the first time, coinheritance of two relatively mild mutations (−α3.7/ααAdana) was unpredictably detected in a 1.5 year-old child with Hb of 7.1 g/dL. Conclusion The HbH disease patients’ clinical characteristics were variable with no ample difference between the deletion and non-deletion types. These results can be of benefit for the screening and management of thalassaemia in this region.
Background and objectives: In adults, acute myeloid leukaemias constitute 80% of all acute leukaemia cases; its incidence progressively increases with age To define the clinical and haematological parameters of adult acute myeloid leukaemia in Erbil City. Methods: A painstaking analysis of hospital records of 94 adult patients with acute myeloid leukaemia was undertaken. The cases were diagnosed and managed at Nanakaly hospital during the years 2006-2009. Diagnosis was based on peripheral blood and marrow findings. The myeloid origin was confirmed by cytochemistry, and morphological subtyping was done according to the French-American-British (FAB) criteria. Microsoft excel version 2007 was employed for carrying out statistical analysis. Results: The studied group included 58 males and 36 females (M: F = 1.6:1). Their ages ranged between 16 and 75 years with a mean age of 33.8 years. Pallor was the commonest presenting feature (70.2%) followed by bleeding (22.3%) then fever (7.5%). The mean value of Hb was (7.6) g/dl, WBCs (34.5 x10-6 /L), platelets (39.6 x10-9 /L); the percentage of blast cells in peripheral blood was 42.5% and in the bone marrow was 65.9%. AML-M2 was the most frequent FAB subtype 24.4%. Conclusion: AML was commoner in males than in females (1.6:1). AML-M2 was the commonest FAB subtype. Pallor was the commonest presenting feature. Sudan black-B stain was a useful tool in the diagnosis of myeloid leukaemia .
ObjectivesWe aimed to detect the incidence of disseminated intravascular coagulation (DIC) in patients with acute leukemia (AL) and find out its association with types of AL and patients’ clinical and pathological parameters.MethodsIn this prospective study, 59 newly diagnosed adults with AL were clinically examined and screened for DIC presentation time. Coagulation tests, including prothrombin time, activated partial thromboplastin time, fibrinogen level, D-dimer, antithrombin, and protein C and protein S levels were all assessed. The International Society for Thrombosis and Hemostasis scoring system was adopted to diagnose overt DIC.ResultsThe age of the studied patients ranged from 15 to 81 years with a median of 41 years; male to female ratio was 1.1:1. acute myeloid leukemia (AML) constituted 64.4% of the total cases (38 patients). DIC was detected in 28 patients (47.5%); its incidence was higher in AML than in acute lymphoblastic leukemia (ALL) (52.6% vs. 38.1%). Overt DIC was significantly associated with bleeding manifestations, duration of symptoms, and leukocytosis (P-values = 0.050, 0.044, and 0.003, respectively). Bleeding events were encountered in 50.8% of patients (25 AML and 5 ALL patients). Bleeding was associated significantly with leukocytosis, thrombocytopenia, and low fibrinogen level. Thrombosis was found in two patients (3.4%) at presentation.ConclusionsOvert DIC was common in patients with AL at presentation, mostly in AML. Routine testing for coagulopathy in newly diagnosed AL patients will possibly aid in improving the overall patients’ survival.
Background: HbH disease results from dysfunction of three, less commonly two, α-globin genes through various combinations of deletion and non-deletion mutations. Characterization of the mutations and the underlying genotypes is fundamental for proper screening and prevention of thalassaemia in any region. The aim of this study was to explore the genetic arrangements of HbH disease and to correlate the genotypes with the clinical phenotypes.Methods: A total of 44 HbH disease patients were enrolled in this study. They were clinically and haematologically assessed. The patients were tested for 21 common α-globin gene mutations using multiplex PCR and reverse hybridization. According to the genotype, the patients were categorized into two separate sub-groups, deletion and non-deletion types HbH disease. Results: Within the studied HbH disease patients, eight different α-globin gene mutations were detected in nine different genetic arrangements. The --MED and -α3.7 deletions were the two most frequently encountered mutations (37.5% and 35.2% respectively). Patients with deletion genotypes constituted 70.4%. The most common detected genotype was --MED/-α3.7 (59.1%), followed by αpoly-A1α/αpoly-A1α (13.6%). For the first time, coinheritance of two relatively mild mutations (-α3.7/ααAdana) was unpredictably detected in a 1.5 year-old child with Hb of 7.1 g/dL. Conclusion: The HbH disease patients’ clinical characteristics were variable with no ample difference between the deletion and non-deletion types. These results can be of benefit for the screening and management of thalassaemia in this region.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.
hi@scite.ai
10624 S. Eastern Ave., Ste. A-614
Henderson, NV 89052, USA
Copyright © 2024 scite LLC. All rights reserved.
Made with 💙 for researchers
Part of the Research Solutions Family.