BACKGROUND: Acute myeloid leukemia (AML) is a malignant disease of the bone marrow in which hematopoietic precursors are arrested in an early stage of development leading to production of abnormal cells. AML is the most common type of leukemia in adults. The most important advances can be achieved through immune checkpoint (IC) inhibitors, including cytotoxic T lymphocyte antigen 4 (CTLA-4), Programmed Death protein -1 (PD-1), and anti-programmed ligand 1 in cancer treatment over the past decade. OBJECTIVES: The aims of the current study were to evaluate the expression of CD3, CD28, CD152, CD223, and CD279 markers in T cells by flow cytometry and the expression of PD 1, CTLA 4, and lymphocyte activation gene 3 (LAG 3) expression by real time PCR in AML patients. MATERIALS AND METHODS: This is case control study carried out on 50 AML Iraqi patients, in addition to 50 apparently health person. This study was conducted at the National Center for Hematology, Department of Biology, Mustansiriyah University, and in Baghdad teaching hospital in Medical City, from January 2019 to June 2020. Moreover, the study aims to evaluate the expression of CD3, CD28, CD152, CD223, and CD279 markers in T cells by flow cytometry of AML patients and the expression of PD 1, CTLA 4, and lymphocyte activation gene 3 (LAG 3) expression by real time PCR in AML patients. RESULTS: The cellular expression of almost CD markers did not show a relationship between gender and age. Most AML patients had high CD3 and CD28 expression in cellular expression of T cells. Although there were increasing gene expression of PD-1 and LAG-3 in T cells. The cellular expression of CD279 PD-1 was high, gene expression of CTLA-4 had slightly increased, and cellular expression of CD152 CTLA-4 not significant among healthy controls. In the present manuscript, there was an increase in the expression of PD-1 CD279 in the relapse and refectory patients than the complete and partial remission, while CD3, CD28, and CD223 LAG-3 did not show differences in the expression on T cells among AML stages. Finally, the immunophenotyping of 48 from 50 patients (96%) of the present study was CD3+CD28+CD152−CD279+CD223−. CONCLUSION: Elevate the expression of PD 1, LAG 3 in almost all AML patients associated with the progression of the disease. 96% of AML patients' immunophenotyping was CD3+CD28+CD152−CD279+CD223−.

BACKGROUND: Iron deficiency is not common in thalassemia minor and nontransfusion dependent hemoglobinophaties. The majority of these patients have normal-to-high serum ferritin. OBJECTIVES: The aims of the study were to evaluate serum ferritin levels in alpha and beta thalassemia minor and intermedia and in, hemoglobin H disease, and sickle cell anemia, and to investigate the effect of iron consumption on increasing serum ferritin levels and the role of Mentzer and Srivastava indices in patients with thalassemia minor and low serum ferritin levels. MATERIALS AND METHODS: In this study, 204 patients with alpha-thalassemia minor, beta-thalassemia minor, nontransfusion-dependent thalassemia intermediate, and sickle cell disease were studied. Serum ferritin levels, Mentzer, Srivastava, and Bordbar's formula were measured using erythrocyte indices. RESULTS: Irrespective of iron deficiency status, which was 5.9% and was more common in women, total iron intake was 39%. Iron deficiency status was 3.3% in alpha thalassemia, 9.8% in beta-thalassemia, and 4.5% in sickle cell disease. High and very high serum ferritin levels are more common in beta intermediate thalassemia and sickle cell anemia. Mentzer and Srivastava indices were not significant for differentiating thalassemia minor and iron deficiency, but the Bordbar's formula in thalassemia minor with iron deficiency was statistically significant (119.75). CONCLUSION: Patients with minor thalassemia and nontransfusion dependent hemoglobinopathy had a lower prevalence of iron deficiency according due to due to serum ferritin levels compared to the general population. High and very high ferritin is more common in intermediate thalassemia, hemoglobin H, and sickle cell patients.

CONTEXT: Congenital fibrinogen deficiency is a rare inherited coagulation disorder with an estimated prevalence of 1:1,000,000 which is characterized by bleeding that varies from mild to severe and by an extremely low level or complete absence of plasma and platelet fibrinogen. AIMS: This study aims to assess prevalence, demographic parameters, clinical presentation, and hemostatic values in patients diagnosed with congenital fibrinogen deficiency. SETTINGS AND DESIGN: A retrospective descriptive study of patients diagnosed with congenital fibrinogen deficiency in Hemophilia Center/Medical City/Baghdad over a period of 7 years (from August 2008 to August 2015). SUBJECTS AND METHODS: The study included patients diagnosed with congenital fibrinogen deficiency. The confirmation of diagnosis was done based on a low fibrinogen assay. All the fibrinogen deficient patients were diagnosed on basis of having fibrinogen level (<150 mg/dl). RESULTS: There were 111 cases of other coagulation factors deficiency (rare bleeding disorders) registered in the center, congenital fibrinogen deficiency represented 25 (22.5%) of them. Out of 25 patients with congenital fibrinogen deficiency, 8 patients (32%) had fibrinogen level <20 mg/dl (afibrinogenemia) and 17 patients (68%) had fibrinogen level between (20 and 150 mg/dl) (hypofibrinogenemia). The males were 17 patients (68%) and females were 8 patients (32%). The consanguinity was present in 16 (64%) and 11 patients (44%) who had a positive family history of the diseases. The patients with afibrinogenemia mostly presented with hemarthrosis (22.7%) while patients with hypofibrinogenemia mostly presented with menorrhagia (50.0%). The patients who were their symptoms manifested before the 1^st year of their age most frequently developed life-threatening bleeding (central nervous system and gastrointestinal bleeding) which were observed in 4 cases. Most of the patients included in this study were without complications (80%) while just one patient (4%) died due to gastrointestinal bleeding others were complicated with viral hepatitis, hepatitis B in one patient (4%) and 3 patients (12%) developed hepatitis C. CONCLUSIONS: Congenital fibrinogen deficiency founded to be one of the most frequent rare bleeding disorders among Iraqi patients, with a high rate of consanguineous marriage and positive family history. There was a high frequency of life-threatening bleeding among patients who develop symptoms before the 1^st year of their age, without notable major complications among the majority of patients.

BACKGROUND: Immune thrombocytopenia (ITP) is an autoimmune hemorrhagic disorder, where autoreactive T-cells and/or autoantibodies destroy platelets and megakaryocytes in the spleen and bone marrow, respectively. To the best of our knowledge, this is the largest cohort of cases wherein patients were treated with novel combination therapy of a corticosteroid with an adjunct immunosuppressive agent for the treatment of ITP in adults. In cohort of 23 patients, 11 patients have no response and 12 patients have shown partial to complete response (CR) to the treatment. The primary aim of the present study was to explore the safety and efficacy of combination therapy in chronic ITP along by evaluating the toxicity associated with prolonged steroid exposure. The secondary aim was to compare the cost benefit with other available modalities for chronic ITP treatment. MATERIALS AND METHODS: A retrospective observational study was carried out by collecting data from electronic medical records of all the ITP patients treated at HCG Manavata Cancer Centre, India, between May 1, 2019, and April 30, 2020. Inclusion and exclusion criteria were strictly followed for data collection and analysis. RESULTS: Twelve (52%) of the 23 patients have shown response to the combinational therapy; 5 (22%) patients achieved a partial response (PR) and 7 (30%) achieved a CR. In the PR group, 3 patients developed thrombocytopenia and 1 switched to thrombopoietin receptor agonists, whereas in 7 CR patients, 6 have maintained it until end and 1 patient was switched to maintenance therapy. CONCLUSION: A combination of immunosuppressant and corticosteroid on ITP patients appeared to be effective, tolerable, with minimal adverse side effects, and an economical alternative. Therefore, this novel combination therapy may be an excellent alternative for the treatment of patients with ITP in clinical settings.

BACKGROUND: Acute promyelocytic leukemia (APL) is a well-defined subtype of acute myeloic leukemia (AML) and known by t (15;17)(PML-RARA) mutation. About 20%–40% of AML patients indicate FMS-like tyrosine kinase 3 (FLT3) mutations. FLT3 mutations contain two famous mutations: FLT3-internal tandem duplication (ITD) and FLT3-tyrosine kinase II domain. OBJECTIVES: Many studies have been done on FLT3-ITD. In these studies have been acknowledged that the FLT3-ITD mutation had a poor prognosis of on AML patients. This study was performed on two APL and APL + FLT3-ITD groups. This study aimed to compare the differences in blood laboratory assays between APL and APL + FLT3-ITD patients. METHODS: This study contained 73 patients which divided into two groups: APL and FLT3-ITD + APL. The study methods included: cell counting and peripheral blood smear, extraction of mRNA and DNA, and cDNA synthesis electrophoresis. RESULTS: This study was ruled out on patients involved with APL in GHAEM hospital Mashhad, Iran. All patients were diagnosed with t (15;17) (PML-RARA). The age range of patients was 7–63 years (mean: 30.86). Fifty-eight (79.5%) of patients (male: 22 and female: 36) were involved solely with APL and 15 (20.5%) of them (male: 10 and female: 5) were APL + FLT3-ITD mutation. Blood parameters that were analyzed included white blood cell, red blood cell, hemoglobin, hematocrit, mean cell volume, and platelet count. Each group of patients' population was categorized into high risk factors and low risk factors. CONCLUSIONS: the consequence of the current study demonstrated that FLT3-ITD mutation had a bad effect on laboratory assays in patients involved with APL.

BACKGROUND: Familial Mediterranean fever (FMF) is characterized by a rise in acute phase reactants (APRs) during the attacks and occasionally in between the attacks. Although mean platelet volume (MPV) is generally not changed, low MPV could be used as an inflammatory marker in FMF and as an indicator of other diseases. This prospective case–control study was carried out in Sulaymaniyah, Iraq, to assess MPV in Kurdish children with different FMF mutations during and in-between the attacks versus healthy controls. PATIENTS AND METHODS: From 2011 to 2020, 56 gene-positive FMF patients (Group I) and 60 healthy controls (Group II) were enrolled. Besides the routine APRs, MPV was measured in both groups and categorized into low (<7 fl), normal (7–11 fl) and high (>11 fl). Informed consents from all participants and ethical institutional approval were obtained. RESULTS: Group I age range was 23 months to 16 years with a male to female ratio of 1.8:1 while Group II age ranged from 21 months to 15 years with a male to female ratio of 1.4:1. MPV was normal in (n = 44, 78.6%) of Group I versus (n = 44, 73.3%) of Group II (P = 0.05); low in (n = 8, 14.3%) of Group I versus (n = 4, 6.7%) of Group II (P = 0.05) and high in (n = 4, 7.1%) of Group I versus (n = 4, 6.7%) of Group II (P = 0.05). Low MPV values were relatively more frequent among the homozygotes (n = 4, 50%) and complex heterozygotes (n = 2, 25%) (P = 0.003). CONCLUSION: The current study showed that MPV was not statistically different between FMF patients and controls, while abnormal MPVs were associated with specific genotypes that may indicate coexistent problems.

BACKGROUND: Hematological changes involving all blood cells are some of the most common complications in both tuberculosis (TB) and malaria infection. The changes induced by malaria infection are diverse, and the first line anti-TB treatment regimen which involves two phases may alter these changes in TB participants co-infected with malaria (TB/MP). OBJECTIVE: In this study, we aimed to ascertain the impact of malaria co-infection on leukocyte indices of TB-infected participants at pre-treatment, intensive and continuation phase therapy. MATERIALS AND METHODS: In this cross-sectional study, 180 participants were recruited comprising; 60 (35 TB and 25 TB malaria) participants before treatment, sixty (36 TB and 24 TB-Malaria) participants after intensive phase treatment and sixty (27 TB and 33 TB-Malaria) participants after continuation phase therapy. Whole blood was used for the measurement of total (total white blood cell [TWBC]) and differential white cell count, Platelet count, and packed cell volume (PCV). RESULTS: Before initiation of treatment, TWBC, neutrophil, lymphocyte, platelet count, and neutrophil-lymphocyte ratio were significantly reduced (P = 0.041, 0.022, 0.046, and 0.026, respectively), whereas eosinophil count was significantly increased in TB/Malaria participants compared to TB participants (P = 0.043). There was no significant change in these parameters after intensive phase treatment (P > 0.05). However, after continuation phase treatment, PCV was significantly reduced, while eosinophil was significantly increased in TB/Malaria participants compared with TB participants (P = 0.046 and 0.045, respectively). CONCLUSION: Malaria co-infection induces the significant reduction in leukocyte indices of TB patients at pretreatment but not at the intensive and continuation phase anti-TB therapy except eosinophils count which was increased before treatment and continuation phase treatment.

BACKGROUND: Acute lymphoblastic leukemia (ALL) is the most common malignancy in children. It accounts for one-fourth of all childhood cancers and 72% of all cases of childhood leukemia. OBJECTIVE: The objective was to evaluate the significant increase in serum lactate dehydrogenase (LDH) enzyme levels in patients with ALL with respect to patients' characters, clinical presentation, and patients' induction outcome. PATIENTS AND METHODS: A prospective study had been conducted during the period from November 1, 2017 to October 31, 2018, included 86 patients newly diagnosed ALL patients under the age of 15^th years, admitted to the pediatric hemato-oncology unit in the Child's Central Teaching Hospital the data were collect from the patients, included, age, sex, clinical presentation, investigation and induction outcome of ALL patients to undergo analysis of study. RESULTS: Of a total (86) ALL patients started induction therapy, only (75/86) of them completed induction phase of therapy and those were enrolled in analysis of this study, while (11/86) did not complete induction therapy and excluded from the study (because 10 died, and one patient loss follow-up during induction). The mean age was 4.7 years. The male-to-female ratio was 1.26:1. LDH level ranged from 100 to 1995 U/L. There was a significant association between LDH level at day 0 and each of age and ALL risk group and no association with gender, hepatomegaly, splenomegaly, lymphadenopathy, central nervous system status, and induction outcome (remission/no remission). The mean of LDH levels at diagnosis was highly elevated in patients with ALL (726 ± 422 U/L); the response to induction treatment was observed by the significant decrease in mean LDH level (324 ± 201 U/L) at day 28^th of treatment P value (0.0001). CONCLUSIONS: The serum LDH level was highly elevated at diagnosis in the majority of ALL patients and decreased significantly in response to chemotherapy. The estimation of serum LDH level is easy, and available, so it may be a helpful tool in evaluating the clinical aspect of the disease, the response to induction chemotherapy.

CONTEXT: Bleeding in severe hemophilia B is minimized by factor IX (FIX) replacement either as once- or twice-weekly prophylaxis. Recent trials have focused greater interest on once-weekly prophylaxis using standard recombinant FIX (rFIX) or enhanced half-life rFIX. Limited data are available on optimal prophylaxis regimens of FIX replacements for patients with hemophilia B. AIMS: This study aimed to evaluate the efficacy of once-weekly prophylaxis compared with twice-weekly prophylaxis with BeneFIX (rFIX) in children with severe hemophilia B. SETTINGS AND DESIGN: This study is a retrospective, two-period study from January 2012 to December 2017. SUBJECTS AND METHODS: The study assessed the efficacy of 3-year twice-weekly prophylaxis with BeneFIX (rFIX) in patients with severe hemophilia B followed by 3-year once-weekly prophylaxis, then comparing once weekly versus twice weekly at a given period. The primary efficacy endpoint was the annualized bleeding rate (ABR) and the secondary endpoint included Functional Independence Score for patients with Hemophilia (FISH) scoring. RESULTS: There was no statistically significant difference in the bleeding per year and the joint bleeding per year between once- and twice-weekly prophylactic treatment regimens (P > 0.05). There was no statistically significant difference in ABR between once- and twice-weekly prophylaxis, 11.9 and 9.1 bleeds per year, respectively (P > 0.05). In addition, there was no statistically significant difference in the hospitalization and school absence between once- and twice-weekly prophylactic treatment regimens (P > 0.05). There was no statistically significant difference in FISH score between once- and twice-weekly protocol (P > 0.05), but factor consumption was significantly higher in the twice-weekly protocol compared with once-weekly protocol (P < 0.001). CONCLUSIONS: Once-weekly prophylaxis was effective and tolerated prophylaxis for most patients included in this study. Once-weekly prophylaxis is an effective alternative to twice-weekly prophylaxis, and both the regimens reduce ABR in children with severe hemophilia B.

BACKGROUND: Congenital FXIII deficiency is a rare genetic bleeding disorder that is inherited in an autosomal recessive pattern with a frequency of 1/2 million individuals in the human population. Deficiency of FXIII is associated with significant bleeding disorders. AIMS: This study aimed to evaluate the demographic parameters, clinical presentations, and outcome of patients who were diagnosed with congenital factor XIII deficiency. SUBJECTS AND METHODS: A retrospective descriptive study of patients who were diagnosed with congenital FXIII deficiency over a period from August 2008 to August 2016 was conducted. The study included patients who were diagnosed by having bleeding tendency and normal standard coagulation tests (normal platelet count, normal prothrombin time; normal partial thromboplastin time, and normal bleeding time) and the diagnosis was confirmed by clot solubility test in 5M urea. The diagnosis was made in the Hemophilia Ward, Children Welfare Teaching Hospital, Medical City, Baghdad. RESULTS: There were 111 cases of other coagulation factors' deficiency (rare bleeding disorders) registered in the center, congenital FXIII deficiency represented 24 (22%) of them. Males represented 14 (58.3%) and females 10 (41.7%) of patients. Most of the patients (41.7%) had their symptoms during the 1^st year of life. Positive consanguinity was found in 100% of patients and 14 (58.3%) patients had a family history of FXIII deficiency. Umbilical cord bleeding and gum bleeding were present in 37.5% and 20.8%, respectively, and they were the most common first presenting symptoms of FXIII-deficient patients, while muscle hematoma (28.5%) and joint bleeding (24.7%) were the most common presenting symptoms in follow-up visits. The majority of the patients (79.1%) did not develop complications, the complications were developed in 3 (12.4%) patients, which were intracranial bleeding in 2 (8.3%) patients and liver hematoma in 1 (4.1%) patient. One patient (4.1%) developed recurrent abortion and one patient (4.1%) developed hepatitis C. No death was reported during the study period. CONCLUSIONS: FXIII deficiency founded to be a more common rare bleeding disorder among Iraqi patients, with a high rate of consanguineous marriage. Umbilical cord bleeding and gum bleeding were the most common presenting symptoms for FXIII deficiency. There was a considerable delay in the diagnosis of FXIII deficiency in the majority of patients.

BACKGROUND: Early autologous hemopoietic stem cell transplant is the best option for treatment of relapsed or refractory Hodgkin lymphoma (HL), which is the standard of care at the time being. The aim of this study was to evaluate the outcome of patients with relapsed/refractory (R/R) HL who received autologous hemopoietic stem cell transplant. METHODS: This is a cohort study with data obtained from the patient's sheets and then with follow-up from January/2014 to May/2017. Analysis involved 48 patients with R/R HL. Those patients received high-dose chemotherapy followed by autologous hemopoietic stem cell transplantation (ASCT). Disease status before ASCT, chemo-mobilization protocols, and stem cell collection and ASCT procedure were recorded. The posttransplantation complications and 30-day mortality were also recorded. Re-evaluation of disease status was done at day 100 post transplantation and the patients were followed up for any evidence of relapse or progression till the end of the study. Comparison of various predictors affecting overall survival (OS) and progression-free survival (PFS) was also performed. RESULTS: The 3-year PFS and OS for the patients with R/R Hodgkin disease who received ASCT were 80% and 70.2%, respectively, with various predictors affecting them. Patients with disease status before ASCT as partial remission and resistant have shorter mean OS and PFS that are not statistically significant (P = 0.325 for OS and 0.45 for PFS). Patients with the number of pretransplant treatment regimens more than 2 have a statistically significant shorter mean PFS and statistically nonsignificant OS (P = 0.4 for OS and 0.06 for PFS). The first 30-day posttransplantation mortality (procedure-related death) was 6.3% due to sepsis. CONCLUSION: The mean PFS was inversely affected by the number of treatment lines received prior to ASCT.

BACKGROUND: Hemoglobin (Hb) is a tetramer of two alpha and two beta globin polypeptide chains, The fractions of HbF, HbA2, and HbA vary gradually in pediatric population (age 0–12 years) and show a dynamic change in the 1st year of life. OBJECTIVE: The objective of the study is to establish the levels of normal hemoglobin (Hb) fractions by using high-performance liquid chromatography (HPLC) in Indian pediatric population. MATERIALS AND METHODS: A total of 169 children of 0–12 years of age were recruited from a pediatric outpatient clinic. Eleven cord blood samples from normal deliveries were collected, and 2 ml peripheral blood was drawn from each subject in EDTA vial for CBC and Hb HPLC. CBC was performed by hematology analyzer XT 2000i, and the proportion of HbA, HbA₂, and HbF was obtained from HPLC using the β thalassemia short program. RESULTS: The fractions of HbF, HbA₂, and HbA gradually changed with increasing age. HbF levels decreased rapidly from 80.9% ± 0.48% (mean ± standard deviation) in the cord blood to 3.6% ± 1.04% at 6 months of age. HbA₂ was 0% in cord blood, was 0.04 ± 0.12 in newborn, reached to a level of 2.52% ± 0.3% at 6–12 months of age, and thereafter marginally increased to 2.65 ± 0.89% at 2–12 years of age. HbA was 20.41% ± 5.14% in the cord blood and increased substantially to 84.7% ± 1.83% at 6–12 months of age. CONCLUSION: HbF levels show a more dynamic change in the first 6 months of life than later half. It reaches adult levels by the age of 2 years. HbA₂ levels reach a plateau at 6 months of age. HbA levels rise substantially until the 6^th month and sustain thereafter. These data can serve as a quick practical reference guide for the analysis of Hb fractions in the Indian pediatric population.

Selective serotonin reuptake inhibitors are commonly used in the treatment of many psychiatric diseases today. Their common side effects consist of gastrointestinal side effects, sexual dysfunction, headache, insomnia, and sedation, whereas hematological side effects have been reported, although rarely. In this article, we presented a case of thrombocytopenia, which is a rare side effect emerging after the escitalopram use, belonging to a 19-year-old female patient who had a generalized anxiety disorder and no hematological history was noted. We aimed to discuss the development mechanism of thrombocytopenia due to the escitalopram use.

Recurrent hemarthrosis is a common entity in children. Although recurrent hemarthrosis most often associated with hemophilia (VIII or IX deficiency), but rarely it can be associated with factor VII deficiency (FVIID). It is a strong mimicker of hemophilic hemarthrosis. Once hemophilia is excluded as a cause of recurrent hemarthrosis, congenital FVIID needs to be considered for long-term planning of treatment and avoiding unnecessary transfusion of factor concentrates. Clinical presentation of FVIID has a varied spectrum and does not correlate with factor levels. Here, we present a case of recurrent hemarthrosis secondary to FVIID.

A positive direct antiglobulin test is a criterion for the diagnosis of systemic lupus erythematosus (SLE). In general, severe hemolysis is absent in SLE. Sometimes, these patients may show hemolysis when presenting with antiphospholipid syndrome (APS). It is essential to exclude an underlying alloantibody along with autoantibody. We had reported a case of a 24-year-old female SLE along with an APS patient requiring transfusion support with underlying allo-anti-S antibody. We provided two units of S antigen-negative best-matched units to the patient who tolerated it well and showed improvement.

India has been facing the problem of malaria for centuries. Peripheral smear microscopy remains the gold standard for diagnosis; however, it is a tedious process and requires qualified staff. Flow cytometric-based hematology analyzers' scattergrams can be of vital use in identifying the abnormal scattergrams for malaria. We report a patient with fever and chills who presented to the outpatient department and was further evaluated for pyrexia of unknown origin. 6-Diff complete blood count analyzer incidentally showed abnormal scattergram which was evaluated by a pathologist and confirmed the presence of Plasmodium vivax. The accuracy in detection of malaria diagnosis can vary based on species, parasite load, immunity, and clinical context. Pathologist and technical staff should analyze any abnormal scattergram and hematological data along with peripheral smear to identify the parasites.