BACKGROUND: Thalassemia syndromes and structural hemoglobin variants generate blood crisis of variable clinical symptoms, ranging from mild-to-moderate hematological disorder to severe, lifelong, transfusion-dependent anemia. The aim of current study was to uncover the prevalence of thalassemia and other hemoglobinopathies in the Erbil governorate, Kurdistan region of Iraq. MATERIALS AND METHODS: The available data of thalassemia major, thalassemia intermedia, sickle cell disease, sickle cell trait, and HbH and HbE until the end of 2020 were collected retrospectively from Erbil Thalassemia Center in Erbil governorate, Kurdistan region of Iraq and analyzed by using Microsoft Excel (Version 2016). RESULTS: An increase in the prevalence of thalassemia syndromes from 30.8/100,000 in 2015 to 37.3/100,000 individuals in the population in 2020 was revealed. The prevalence of all hemoglobinopathies combined increased from 31.9/100,000 to 42.7/100,000 individuals of the population. Thalassemia major was the predominant condition among the hemoglobinopathies with 758 (78.71%) cases out of 963 cases at the end of 2020. CONCLUSION: This rise might be attributed to a large number of consanguineous marriages, the lack of effective prevention programs, and poor legislation. There is an emergent requirement for a preventive program, entailing identification of carriers, genetic counseling, guidelines to differentiate between other microcytic anemias with thalassemia traits, antenatal diagnosis, public education, and sustained legislation.

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BACKGROUND: Complete blood count and cell counter generated red blood cell (RBC) parameters help in morphological typing of anemia. Still the importance of microscopic examination of peripheral blood smears (PBSs) cannot be excluded to interpret the cause of anemia. AIMS AND OBJECTIVES: The present study was done to diagnose the type of anemia by examination of PBS and automated cell counter generated parameters and to compare the findings between these two methods. MATERIALS AND METHODS: During the 6-months study, 9981 anemic blood samples were evaluated. PBS findings and cell counter generated RBC parameters and histograms were evaluated and compared. RESULTS: Among 9941 samples, microcytic hypochromic anemia consists of the majority of cases (5048, 50.47%), followed by normocytic normochromic (2187, 21.97%), dimorphic (1297, 12.99%), and lastly, hemolytic anemia (722, 7.24%). Compared with RBC parameters and peripheral smear findings, dimorphic and hemolytic anemia showed significant difference (P < 0.0001). When compared with RBC histogram, 4747 (47.56%) cases showed left shift, 820 (8.21%) cases showed right shift, and 2278 (22.82%) cases showed normal bell-shaped curve suggesting microcytic, macrocytic, and normocytic normochromic anemia, respectively. 635 (6.90%) and 1447 (14.50%) number of cases showed bimodal and broad-base histogram suggesting dimorphic and hemolytic anemia, respectively. CONCLUSION: PBS examination along with RBC histogram study can be able to categorize the type of anemia in the majority of cases. Each method should be used as complementary to each other that increases diagnostic accuracy.

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BACKGROUND: Childhood acute lymphoblastic leukemia (ALL) is the most prevalent malignant disease (25%–30%) and the most common type of leukemia (75%–80%) among children. It is not a single disease with significant phenotypic and genotypic variability that has diagnostic and prognostic implications. This study aims to provide the immunophenotypic profile of childhood ALL in Iraqi patients and to explore the frequency of aberrant myeloid antigen expression and their association with hematological parameters. PATIENTS, MATERIALS AND METHODS: The records of 67 pediatric patients diagnosed as ALL were reviewed for their flow cytometric immunophenotyping results at presentation. RESULTS: B-ALL constituted 76.1% of the cases and 23.9% were T-ALL. There was a highly significant statistical relation between higher age interval and T-ALL phenotypes (P = 0.001). Higher hemoglobin (Hb) level and white blood cell count were significantly related with T-ALL subtype (P = 0.039 and < 0.001, respectively). CD34, HLA-DR, CD10, and CD79a were significantly correlated with B-ALL compared to T-ALL (P = 0.007, <0.001, <0.001, and <0.001, respectively). With no significant differences, aberrant myeloid antigen expression was found in 51% of B-ALL and in 25% of T-ALL cases; however, CD34 expression was substantially related with aberrant myeloid antigen expression (P = 0.001). CONCLUSION: Aberrant myeloid antigens were expressed in 44.9% of ALL patients with insignificant differences between B- and T-ALL phenotypes. CD34 was significantly associated with B-lineage ALL and with aberrant myeloid antigen expression. T-ALL children are older and have significantly higher Hb concentration and white blood cell count. No correlation was found between aberrant myeloid expression and hematological parameters in B-ALL.

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BACKGROUND: Febrile neutropenia (FN) is a serious problem, especially in hematologic malignancies, and can cause high mortality rates and it occurs in 10%–20% of patients with lymphoma. The aim of this research is to assess the risk factors for FN, and the impact of FN on overall survival (OS) in patients with diffuse large B-cell lymphoma (DLBCL). MATERIALS AND METHODS: The study included 263 patients who were diagnosed with DLBCL and treated with mostly R-CHOP-based chemotherapy. Data including gender, age, Ann Arbor stage, International Prognostic Index (IPI) score, immunohistologic subtype, treatment regimens, response to treatment, and any FN episode were recorded. The factors predicting FN were analyzed. RESULTS: Significant predictors of FN were the number of chemotherapy lines received and IPI score. The median OS was significantly different between DLBCL patients who had at least one FN episode during the first-line chemotherapy and those who did not (P < 0,001). Significant predictors of OS in the multivariate analysis were the number of chemotherapy lines received, stage, Eastern Cooperative Oncology Group, and disease status. CONCLUSION: Our study reveals that OS is significantly shorter in patients who had an FN episode than those who did not. Therefore, it is crucial to demonstrate all factors related to FN to prevent FN episodes. In our study, the number of chemotherapy lines received and IPI score was found to be significant predictors of FN. Close follow-up should be done in these patients as the risk of FN is higher.

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BACKGROUND: Some of the platelet count disorders have no single clinical or laboratory diagnostic finding and bone marrow examination may be required which is invasive, time consuming. Platelet indices are readily available parameters by recent hematology autoanalyzers and could provide some important information and can differentiate between several mechanisms of platelet disorders. AIM OF STUDY: To evaluate and interpret different platelet indices (plateletcrit, mean platelet volume [MPV], platelet distribution width [PDW], platelet-large cell ratio [P-LCR]) in patients with quantitative platelet disorders. PATIENTS AND METHODS: A prospective cross-sectional study was carried out from November 2019 to September 2020 in different Iraqi hematology centers and conducted on 160 adult patients from 3 hematology centers, from November 2019 to September 2020, 80 patients have thrombocytopenia, and 80 patients with thrombocytosis. Platelet count and indices (MPV, P-LCR, PDW) were determined using automated analyzers. For each hematological parameter, two measurements were taken at different time interval and the mean value of these two records was relied on. RESULTS: A significant increase in all platelet indices (MPV, PDW, P-LCR) with cutoff values of 7.9 femtoliters (fl), 15.3%, and 12.6%, respectively, P = 0.000 was observed in primary thrombocytosis, with 90% sensitivity for MPV and 50% specificity for PDW. In immune thrombocytopenia (ITP), all platelet indices (MPV, PDW, and P-LCR) were significantly higher than in hypoproductive thrombocytopenia with cutoff values of 7.9fl, 15.3%, and 12.9%, respectively. MPV has a sensitivity of 97% and specificity of 50%, P-LCR had a sensitivity of 100%, and PDW had a sensitivity and specificity of 77% and 70%, respectively. CONCLUSIONS: These abnormalities in platelet indices are of value for differentiation of platelet quantitative disorders, higher value in ITP in comparison with other causes of thrombocytopenia, and for primary thrombocytosis, the value is higher than reactive thrombocytosis.

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BACKGROUND: Anemia of β thalassemia results from a combination of ineffective erythropoiesis and hemolysis. This stimulates erythropoietin (EPO) production, which causes expansion of the bone marrow and may lead to serious deformities of the skull and long bones. Ineffective erythropoiesis also induces the release of growth differentiation factor 15 (GDF-15) and transforming growth factor-beta (TGF-β) which have been identified as regulators of hepcidin expression. OBJECTIVE: The objective is to evaluate the level of TGF-β, GDF-15, and EPO in patients with thalassemia syndrome. PATIENTS, MATERIALS AND METHODS: Patient samples were collected from Thalassemia Center of Ibn Al-Baladi Hospital. This study included 35 patients with thalassemia, 18 patients with beta-thalassemia major and 17 patients with beta-thalassemia intermedia. The age of studied group was 3–17 years. Twenty control healthy subjects were included for comparison who were age- and sex-matched with the patients group. Gel tube was used for collection of serum for enzyme-linked immunosorbent assay test for GDF-15, TGF-β, and EPO). RESULTS: There was a highly significant difference in GDF-15 and EPO levels among studied groups (P < 0.001). In addition, there was no significant difference in TGF-β level among studied groups (P > 0.05). TGF-β, GDF-15, and EPO were not significantly correlated to splenomegaly, hepatosplenomegaly, and frequency of blood transfusion duration in patients with beta-thalassemia major (P > 0.05), while TGF-β and EPO were significantly correlated to splenomegaly, hepatosplenomegaly in patients with beta-thalassemia intermedia but GDF-15 was not significantly correlated. In patients with beta-thalassemia major, EPO was negatively correlated to hemoglobin, packed cell volume, mean corpuscular volume, and red blood cells (RBC) count whereas GDF-15 significantly correlated to lymphocyte and neutrophil counts. TGF-β was significantly correlated to platelet count. In patients with beta-thalassemia intermedia, EPO and GDF-15 were not correlated to any hematological parameters whereas TGF-β was significantly correlated to RBC counts. CONCLUSION: Marker of erythropoiesis GDF-15, EPO was highly expressed in patient with beta-thalassemia major and beta-thalassemia intermedia as compared to the control group and this can be used as a future therapeutic goal for the suppression of ineffective erythropoiesis.

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BACKGROUND: Global pandemic COVID-19 is an acute respiratory illness with a high rate of hospitalization and death rate. Red cell distribution width coefficient of variation (RDW-CV), a routine component of complete blood count (CBC) automatically generated by most hematology analyzers is a useful predictor of clinical outcomes in critically ill patients and in those with infection and sepsis. RDW will provide information for early risk stratification of COVID-19 patients, thereby enabling timely intervention for reducing morbidity and mortality. In such a massive pandemic, early stratification of cases based on routinely available biomarkers can be of great help inefficient utilization of critical care and laboratory assets. MATERIALS AND METHODS: We retrospectively studied the significance and correlation of RDW (CV) (admission) with the severity of clinical illness in 800 confirmed cases of COVID-19 between August 2020 and October 2020 at our hospital. Demographic and clinical details were obtained from medical records; data pertaining to CBC were retrieved through electronic records of our fully automated hematology analyzer (NihonKoden 5 part auto analyzer Model-MEK– 7300K). Statistical workup was done and results were analyzed. RESULTS: Of 800 patients, 60% were male. RDW (CV) >14.5 (elevated) seen in 52% males and 47% females. Elevated RDW was noted in 43.6% (300/688) nonsevere illnesses (mild and moderate), 82% (92/112) in the severe illness group. The mean RDW (CV) for mild, moderate, and severe cases was found to be 14.21 ± 0.61, 15.32 ± 0.67, and 16.34 ± 1.64, respectively. The number of survivors was 704 (88%). The number of people who died was 96 (12%). Elevated RDW was seen in 74% (71/96) who died and 45% (321/704) of people who survived. To determine the efficacy of RDW (CV) in identifying the severity of disease, a ROC curve was used in which a cutoff value of 13.65 is obtained with a sensitivity of 97.3% and specificity of 85%. CONCLUSION: Higher RDW (CV) was found to have a significant association with clinical severity and mortality prediction. Hence, it can be considered as one of the important hematological parameters in the workup to efficiently stratify the patients at the earliest in COVID-19.

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BACKGROUND: Iron overload certainly will develop in β-thalassemia major. Iron homeostasis was mostly regulated by hepcidin that synthesized in the liver and encoded by the hepcidin antimicrobial peptide (HAMP) gene. HAMP and HFE genes, respectively, encode iron-regulating proteins (Hepcidin and HFE). The iron overload's possibility will increase if there is an interaction between β-thalassemia and HAMP-HFE gene mutations. AIM OF STUDY: In β-thalassemia major patients, we need to identify mutations in iron-regulating genes (HAMP and HFE genes), their impact on the iron overload, and their association with some clinicopathological parameters. PATIENTS AND METHODS: During a period of 5 months from (November 2020 to March 2021), a case–control study was conducted. It included 80 patients and controls aged ≥14 years and divided into two groups: thalassemic patient group included 40 patients who were diagnosed by complete blood count, blood film, and hemoglobin – electrophoresis as β-thalassemia major and control group included 40 unrelated, apparently healthy controls that were age and gender matched with thalassemic patient group. Complete blood count, liver and renal function tests, serum ferritin, and DNA extraction were performed. RESULTS: There was a statistically significant difference between study groups by H63D mutations. The proportion of CG genotype was significantly higher among thalassemic patient group than that in controls. There was no statistically significant difference (P = 0.082) between study groups by G71D mutations. Serum ferritin and Alanine transaminase (ALT) levels were significantly higher in patients with CG and GG genotypes compared to that in patients with CC genotype of H63D. CONCLUSION: H63D is associated with iron overload in β-thalassemia patients with unapparent effect on biochemical and hematological data except for ALT and serum ferritin. This could allow early diagnosis and proper treatment to overcome the complications of iron overload in those patients.

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Aplastic anemia (AA) is a rare hematological disorder which is life-threatening in pregnancy. It is mainly caused by the destruction of pluripotent stem cells of bone marrow. There is high risk in pregnancy due to risk of hemorrhage and infection due to pancytopenia. Fetus is also at high risk of intrauterine growth restriction, risk of intrauterine death, and preterm labor. Hence, managing aplastic anemia in pregnancy is a great challenge for obstetricians. It requires a multidisciplinary approach involving hematologist, obstetrician, and critical care specialist for the management of AA in pregnancy. We report a case of AA with pregnancy diagnosed during first trimester.

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BACKGROUND: Blood transfusion carries the risk of transfusion-transmissible infections (TTIs) if not properly screened. As per protocol blood donors who are found reactive for TTIs are requested to come for counseling and directed for further management. Many of them are either not interested or do not follow-up their visit to blood center. This study is undertaken to determine the rate of seroprevalence of TTIs and the attitude of reactive blood donors in response to post donation notification and counseling. MATERIALS AND METHODOLOGY: This observational study considers the blood donations from January 2019 to April 2021. Blood donors with reactive test results identified by different TTIs markers were notified, and their response rates were evaluated. RESULTS: During this study, 8904 donations were recorded out of which 171 donors were found to be reactive (1.92%), only 142 donors were contacted (89.30%), and only 74 reactive donors could be counseled (52.11%). CONCLUSION: This study shows low prevalence of TTI reactivity among blood donors and recommends strengthening of donor notification and counseling practices in blood centers and raises the question of need for central notification system for the traceability of reactive blood donors to prevent the spread of TTIs in the community.

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BACKGROUND: Acute myeloid leukemia (AML) is a heterogeneous malignant disease of hematopoietic tissue. It is characterized by accumulation of abnormal blast cells mainly in bone marrow. Hepcidin is a small bioactive peptide hormone produced in many tissues mainly by the liver, macrophage, and adipocyte and it has been proposed as a marker of inflammation. The aims of study were to assess the changes in serum levels of hepcidin, interleukin-6, and ferritin in addition to iron-binding capacity levels in patients with AML before and after chemotherapy treatment and to compare their levels to healthy controls. MATERIALS AND METHODS: This study includes 43 AML patients (24 males and 19 females). They were divided into two groups: Group 1: Patients with AML before starting chemotherapy and Group 2: after chemotherapy. The protocol used was (3 + 7) where doxorubicin was given from day 1 to day 3 and Cytarabine (Ara-C) was given from day 1 to day 7 then evaluation is done on 28^th day to evaluate response of patients. The control group (Group 3) included 43 healthy controls (24 males and 19 females) who were matched with patients group in gender and age. RESULTS: Serum samples were investigated before and after treatment and compared with its corresponding data of healthy control group and then statistically analyzed. Results revealed that: the prevalence of AML was higher in males than in females. Hepcidin levels were significantly higher in serum of (AML) patients (Group 2) compared to newly diagnosed (Group 1) and to healthy controls (P < 0.0001). Serum (interleukin-6) levels were higher but not statistically significant in (Group 1) when compared to (Group 2) while it was statistically significantly when compared to healthy controls (P < 0.214 and P < 0.0001, respectively). Regarding serum levels of ferritin and total iron capacity (TIBC) predicted highly significant increase for all patients when compared to controls. CONCLUSION: Hepcidin and interleukin-6 may be used as diagnostic criteria for treatment response of AML and also can utilized as biomarkers for the progression of the AML.

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BACKGROUND: A case − control study was carried out to evaluate the health-related quality of life of adult hemophilic patients. MATERIALS AND METHODS: All registered hemophilia cases (40 cases, 36 hemophilia A and 4 hemophilia B) at Jin Blood Center in Duhok/Iraq as well as 40 normal age-matched healthy male individuals were assessed using the medical outcome study “MOS-SF (version 1.0)” so called RAND 36-item health survey 1.0, that assess eight health status scales namely: physical functioning, role-limitation due to physical health, role-limitation due to emotional problem, vitality (energy/fatigue), emotion well-being, bodily pain, social functioning, and general health. RESULTS: The study included eight patients with mild hemophilia, 18 patients with moderate hemophilia, and 14 patients with severe hemophilia, with ages range from 17 to 57 years with the mean age of 27.85 years (±1.65). Patients with severe hemophilia were diagnosed significantly at earlier age compared with those with mild hemophilia. The study confirmed significantly reduced quality of life (QoL) in all 8 assessed areas particularly among severely affected patients with a P value consistently <0.001. The most affected domain was the role limitation due to physical health at 22.56% and emotional well-being at 32.71. All assessed areas were significantly preserved if early prophylaxis initiated. Other factors that were linked significantly with diminished QoL include the development of hemophilic arthropathy. The factors that did not show significant impact included positive viral hepatitis markers, presence of life-threatening bleedings, socioeconomic state, and positive family history. CONCLUSION: Hemophilic patient displayed significant impairment of QoL, particularly after the development of arthropathy and restriction of physical activity and can be preserved with early prophylactic therapy.

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BACKGROUND: Acute lymphoblastic leukemia (ALL) is a heterogeneous disorder characterized by the proliferation of immature lymphoid cells that accumulate in the bone marrow, peripheral blood, and extramedullary sites, causing the clinical manifestations of the disease. Lymphoid enhancer-binding factor-1 (LEF1) is a target gene and central mediator for the Wingless-type signaling pathway, and it has an important role in normal hematopoiesis. High LEF1 expression was reported as a prognostic marker in many types of hematological and nonhematological malignancies. AIM OF THE STUDY: To evaluate the serum level of LEF1 in pediatric patients with ALL and its correlation with other hematological and clinical prognostic factors (white blood cells [WBC] count, age, gender, central nervous system involvement, and response to treatment). PATIENTS, MATERIALS, AND METHODS: This cross-sectional study was conducted on 60 children; 20 patients with newly diagnosed ALL before starting induction therapy, 20 patients with ALL during remission (postinduction), and 20 healthy controls. Measurement of serum LEF1 level was done by enzyme-linked immunosorbent assay. RESULTS: Serum level of LEF1 was higher in newly diagnosed patients than in either patients at remission or controls with highly significant differences. There is a significant positive correlation with total WBC count and no significant correlation between LEF1 level and other hematological and clinical parameters or with immunophenotypic subtypes. There was no significant correlation between LEF1 serum level and response to remission induction. CONCLUSION: A high serum concentration of LEF1 is found in newly diagnosed patients with ALL and showed a significant positive correlation with total WBC count.

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Excessive menstruation after attaining menarche during adolescent age group is known as puberty menorrhagia (PM). The most common presentation includes anemia due to excessive blood loss. We are reporting a rare case of PM secondary to a congenital bleeding disorder.

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BACKGROUND: Iron replacement may change platelet counts. Parenteral iron treatment has different effects on platelets count in some diseases. the aim of this study was to inspect the effect of parenteral ferric carboxymaltose treatment on platelet counts and other hemogram parameters in females who have no other diseases, but iron deficiency. MATERIALS AND METHODS: This study was conducted in a university hospital located in Ankara, Turkey, between January 2020 and October 2021. A total of 239 female participants who were diagnosed with iron deficiency and who were given parenteral ferric carboxymaltose treatment were included in the study. Participants were divided into two groups according to the existence of anemia. Hemogram parameters, ferritin, and C-reactive protein levels before and 3 months after parenteral ferric carboxymaltose treatment were compared between these two groups. RESULTS: Hemoglobin and ferritin levels were increased (P < 0.001) and platelet counts (P < 0.001) and mean platelet volumes (MPVs) (P = 0.015) were decreased after parenteral ferric carboxymaltose treatment. In both groups, hemoglobin levels were increased and platelet counts were decreased, but in the group with patients having anemia, changes in hemoglobin levels and platelet counts were more prominent after treatment (P < 0.001). When compared to initiation of treatment, there was a significant negative correlation after treatment regarding changes in hemoglobin levels and platelet counts (r = ‒0.369, P < 0.001). CONCLUSION: Parenteral ferric carboxymaltose treatment resulted in a decrease in platelet counts and MPV values compared to initiation of treatment. Parenteral ferric carboxymaltose treatment resulted in decreases in platelet counts and independent from correction of anemia.

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BACKGROUND: Sickle cell disease (SCD) is the second-most common hemoglobin disorder in Duhok governorate. Health-related quality of life (HRQoL) instrument provides physicians with the patient's perspectives of their disease and thus more patient-oriented care. MATERIALS AND METHODS: In this case–control study, a total of 70 adults (≥18 years old) registered as SCD at the center for blood diseases in Duhok as well as 70 age- and sex-matched healthy controls were enrolled. Enrolled patients had their records and treatment reviewed, were clinically assessed, and appropriately investigated. All patients and controls had their HRQoL scored using the SF36 questionnaire, which consists of eight domains, namely physical function, role limitation physical, role limitation emotional, vitality, emotional well-being, social function, bodily pain, and general health perception. RESULTS: The mean standard deviation age of the SCD patients enrolled was 26.2 (8.9) years and included 32 males and 38 females. The patients had significantly lower HRQoL scores in all eight domains when compared to their matched controls. The most affected domain was general health. Within the patients' group, it was noted that HRQoL scores were negatively correlated with age in several domains, most significantly with general health (P = 0.011). On the other hand, there was no significant difference in HRQoL in relevance to gender, marital status, education, or employment. The most significant negative correlations of HRQoL scores were documented with the annual number of pain episodes and hospital admissions observed with all eight domains. CONCLUSIONS: The current study documented that in adults with SCD, HRQoL in all domains was significantly worse than in healthy controls and that it gets worse with age. The most significant contributors to the worse HRQoL are pain episodes and hospital admissions. The study underscores the importance of HRQoL assessments to enable attending physicians provide more patient-centered management.

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