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   Table of Contents - Current issue
Coverpage
January-June 2018
Volume 7 | Issue 1
Page Nos. 1-47

Online since Tuesday, February 13, 2018

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ORIGINAL ARTICLES  

Evaluation of chronic myeloid leukemia patients and their molecular responses to tyrosine kinase inhibitors in Erbil city, Iraq p. 1
Kawa Muhamedamin Hasan
DOI:10.4103/ijh.ijh_28_17  
Background: Chronic myeloid leukemia (CML) is one of the indolent myeloproliferative neoplasms. It is characterized by the presence of the Philadelphia chromosome, a translocation between chromosomes 9 and 22 or BCR-ABL1 gene. Objectives: The aims of this study were to evaluate characteristics of CML patients and their molecular response to tyrosine kinase inhibitors (TKI) in Erbil city in Iraq. Patients and Methods: Seventy-six patients with CML were recruited in this retrospective and prospective study from February 2014 to March 2016, at Nanakaly Hospital for Blood Diseases in Erbil city – Kurdistan region of Iraq. They were evaluated from clinical point of view and their laboratory data, and molecular responses to TKI based on polymerase chain reaction were analyzed. Results: The median age of participants was 45 years; the male: female ratio was 1:0.9. The main presenting features were abdominal fullness in 66% and splenomegaly in 95% of patients. Nearly 66% of them had low European Treatment and Outcome Study (EUTOS) score; 70% of patient had major or complete molecular responses (MMR/CMR). There was a significant difference between patients who did versus who did not achieve MMR/CMR in hemoglobin level, promyelocyte, and myelocyte percent, EUTOS, and Sokal scores (P = 0.02, 0.006, 0.03, 0.001, and 0.02, respectively). Conclusion: In the current study, CML patients were at a younger age of onset, and more high EUTOS score. The majority of patients achieved MMR with frontline Imatinib or Nilotinib and those who switched from Imatinib to Nilotinib as well.
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Role of endothelial dysfunction in relation to prothrombogenesis in polycythemia vera p. 8
Ibrahim A Mahmood, Farqad B Hamdan, Waseem F Al-Tameemi
DOI:10.4103/ijh.ijh_32_17  
Background: The morbidity and mortality in polycythemia vera (PV) are closely associated with cardiovascular diseases burden and clonal evolution. These complications were primarily attributed to abnormal rheology consequent to the raised hematocrit, leukocytosis, and thrombocytosis, and in vivo activation of leukocytes, thrombocytes, and endothelial cells. It has been established that damage of endothelium determines the release in circulation of specific markers including thrombomodulin (TM), selectins, and von Willbrand factor (vWF) which are released and favor the formation of cellular aggregates. Objectives: The objective of this study is to evaluate the pathophysiological role of endothelial dysfunction (ED) in relation to increased risk of thrombosis in PV patients. Patients and Methods: In a case–control study, 53 patients enrolled in this study from Al-Imamain Al-Khadimiyan Medical City, and the National Center for Hematology Diseases and Researches. They comprised of thirty patients with PV with mean age of − 54.87 ± 13.44 years-and another twenty-three patients with secondary polycythemia, whose mean age was 40.13 ± 12.21 years. Another thirty aged- and sex-matched, non-smokers healthy volunteers comprised 16 males and 14 females were also studied, their mean age was = 52.1 ± 11.16 years. JAK2 mutation was assessed for PV group while Serum erythropoietin (Epo), vWF and TM were determined for all patient and control group. Results: TM was significantly different among the three studied groups (P < 0.001) as well as vWF was significantly higher (P < 0.001) in patients with PV as compared to the patients with secondary polycythemia and controls. Epo level was significantly lower (P = 0.004) in the newly diagnosed patients with PV when compared to those with a history of thrombosis or longstanding disease. There is positive correlation between JAK2 and TM (r = 0.431, P = 0.017), while negative correlation with vWF (r = −0.565, P = 0.001) in PV patients. Conclusion: ED is one of mechanisms that contribute in prothrombogenesis in PV patient.
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Expression of CD45, CD34, CD10, and human leukocyte antigen-DR in acute lymphoblastic leukemia p. 14
Faris H Jaafar, Areej Emad Kadhom
DOI:10.4103/ijh.ijh_31_17  
Background: Immunophenotypic analysis of leukemic cells by multicolor flow cytometry using different monoclonal antibodies labeled to various flourochromes, is an important, precise and rapid investigation for diagnosis, classification, prognosis prediction, and minimal residual disease detection in acute lymphoblastic leukemia (ALL). Objectives: The aims of study were to study the expression of CD45, CD34, CD10, human leukocyte antigen-DR (HLA-DR) in B and T-ALL among 114 Iraqi patients and compare findings with other reports worldwide. Patients, Materials and Methods: A retrospective cross-sectional study was conducted on 114 ALL patients of various age groups from different hematology centers in Baghdad who were referred to Flowcytometry Department at the Bone Marrow Transplantation Center/Private Nursing Home Hospital/Medical City for immunophenotypic classification using multi-color flow cytometry from the January 1, 2016, to the August 31, 2016. Results: Out of 114 patients, 71 patients were pediatric patients and 43 were adults. Eighty-three patients were classified as B-cell ALL and 31 patients as T-cell ALL. There was significant association between male gender, high white blood cells count and T-cell ALL subtype. Negative-dim-moderate CD45, positive CD34, and positive HLA-DR expressions were significantly associated with B-cell ALL. Common ALL antigen was seen in 86.7% of the B-cell ALL patients and was accompanied cTdT expression. Aberrant myeloid antigens were observed in 22.9% of B-cell ALL patients and in 35.5% of T-cell ALL. About 4.8% of the patients with B-cell ALL had aberrant T-linage antigens while 6.5% of the patients with T-cell ALL expressed aberrant B-cell lineage antigens. Conclusion: Immunophenotypic expression of ALL cells among Iraqi patients is to somewhat in accordance with various researches worldwide making immunophenotyping flow cytometry a crucial appliance in diagnosis, classification, risk stratification, and minimal residual disease detection in ALL.
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Evaluation of the expression of CD200 and CD56 in CD34-positive adult acute myeloid leukemia and its effect on the response to induction of chemotherapy p. 20
Zainab Najah Muhsin, Subh Salem Al-Mudallal
DOI:10.4103/ijh.ijh_37_17  
Background: Acute myeloid leukemia (AML) is characterized by an excess number of myeloid cells in the marrow with maturation arrest and infiltration of bone marrow (BM) and other tissues by myeloblasts, resulting in BM failure. Objectives: The main goal of the present study is to investigate CD200 and CD56 aberrant expression in CD34-positive blasts, in newly diagnosed adult AML patients and their relation with the clinical and hematological parameters, as well as to identify their prognostic significance after induction therapy. Materials and Methods: This was a prospective cross-sectional study on thirty patients with newly diagnosed AML, who were tested for the expression of CD200, CD56 using multicolor flow cytometry and re-evaluated after induction therapy regimen. Results: CD200 and CD56 were aberrantly expressed in 53.3% and 20.0%, respectively, while coexpression of both markers was observed in 13.3%. Interestingly, both markers were expressed more in monocytic subtypes. Significantly, the induction failure in CD200 + patients was 75%, while it was 66.7% in CD56+ patients. Conclusion: The findings of this research provide insights that CD200 and CD56 were closely related to bad prognostic parameters, including high total white blood cell count, low platelet's counts, and low response to induction therapy.
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Utility of simultaneous assessment of bone marrow aspirates and trephine biopsy sections in various haematological disorders p. 26
Vandana Puri, Pooja Sharma, Mrinalini Kotru, Meera Sikka, Satendra Sharma
DOI:10.4103/ijh.ijh_33_17  
Background: Bone marrow examination is crucial diagnostic modality for evaluation of various hematological and nonhematological disorders. However, marrow aspirate smears and biopsy sections, even though performed simultaneously, are often assessed at different points of time due to different processing methods. This sometimes results in discordance in diagnosis which adds to the diagnostic dilemma and delays the treatment. aim: This study aims to compare the diagnostic accuracy and the rate of concordance between the two modalities of bone marrow examination. Materials and Methods: Three hundred simultaneously performed bone marrow aspirates and BM trephine biopsies were retrospectively analyzed over a period of 1 year. The presence or absence of concordance was recorded. The reasons for inconclusive reports were also recorded. The concordance rates for different hematological disorders were calculated and recorded as high for >80%, moderate for 50%–80%, and low for <50%. The findings of discordant cases and reasons for discordance were also tabulated. Results: A high concordance was found in cases of megaloblastic anemia, leukemias, non-Hodgkin's lymphoma, and multiple myeloma; moderate concordance was found in hypoplastic marrow and concordance was low in Hodgkin's lymphoma, chronic myeloid leukemia (CML) in blast phase, metastatic, and granulomatous involvement of bone marrow. Conclusion: Bone marrow aspiration alone is sufficient for the diagnosis of megaloblastic anemia and most of the hematological malignancies. Bone marrow biopsy is more appropriate for detection of disorders with focal marrow involvement such as lymphoproliferative disorders, metastatic cancer, focal blast crisis in CML, granulomatous lesions, and hypoplastic marrow. However, it is strongly recommended that both should be reviewed simultaneously to ensure maximum diagnostic accuracy.
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Thyroid dysfunction in chronic myeloid leukemia patients on nilotinib p. 33
Khaleed J Khaleel, Ahmed H Matloob, Abdalsalam Hatim
DOI:10.4103/ijh.ijh_39_17  
Background: The use of tyrosine kinase inhibitors has dramatically improved the prognosis of chronic myeloid leukemia (CML). Nilotinib has been reported to be associated with hypothyroidism and hyperthyroidism. Objectives: The current study aims to evaluate the prevalence of thyroid dysfunction in a sample of Iraqi patients with CML (chronic phase) treated with nilotinib and its possible association with grade of other hematological parameters. Patients and Methods: Thirty-one patients with CML and the same number of healthy controls were enrolled in this cross-sectional study. All the patients were on nilotinib hydrochloride for at least 6 months. Results: Approximately 10% of the patients were having hypothyroidism and 3% were hyperthyroid while the rest (87%) were normal regarding thyroid function. There was a significant difference between the study and control group in thyroid stimulating hormone levels (P < 0.05) with the level being higher in the study group. Conclusion: Thyroid dysfunction, particularly hypothyroidism is a clinically important adverse effect of nilotinib. Monitoring of thyroid function is required for patients taking this drug.
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Prevalence of hepatitis C virus seropositivity among multitransfused patients with hereditary anemias in Basra, Iraq p. 39
Oula Abdullah Najim, Meaad Kadhum Hassan
DOI:10.4103/ijh.ijh_41_17  
Background: Hepatitis C virus (HCV) infection is a global public health problem and is a leading cause of morbidity and death, with regional variations in genotype prevalence. Objectives: This study aimed to explore the prevalence of anti-HCV seropositivity among patients with hereditary anemias, the association of anti-HCV seropositivity with selected risk factors and the common HCV genotypes. Patients and Methods: This prospective descriptive study was carried out on patients with hereditary anemias from January 2010 through December 2014 registered at the Center for Hereditary Blood Diseases. A total of 2778 patients (1282 males and 1496 females) were recruited, their mean age was 13.19 ± 9.40 years. Patients were screened for HCV antibodies using an enzyme-linked immunosorbent assay test. Quantitation of the HCV viral load and genotype were assessed using polymerase chain reaction (PCR). Results: The frequency of anti-HCV seropositivity was higher in 2010 (33.7%) and declined progressively over the following 4 years (31.7%, 20.2%, 10.8%, and 9.2%), respectively. Out of 424 patients with two positive anti-HCV tests at least 6 months apart, 215 (50.7%) had positive results with PCR. The most frequent genotype was 4 in 43 (56.5%), followed by 1 in 31 (40.7%) patients; 1a: 21.1% and 1b: 19.6%. A significant association was found between anti-HCV seropositivity and type of disease, and deferoxamine pump use, P < 0.05. Conclusions: Anti-HCV seropositivity is declining among multitransfused patients with hereditary anemias in Basra, Iraq, and genotype 4 is the most common in these patients.
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CASE REPORT Top

Management of a patient with Rosai–Dorfman syndrome and large adrenal pheochromocytoma p. 45
Taha O Mahwi, Najmaddin Khoshnaw
DOI:10.4103/ijh.ijh_45_17  
Sinus histiocytosis with massive lymphadenopathy that known as Rosai Dorfman syndrome (RDS)is a rare histiocytic disease which includes benign lymph nodes (LN) enlargement with extra-lymphatic soft tissue involvement. The concomitant presentation of RDS with an adrenal tumor is rarely encountered among our patients. Here in we describe a middle-aged man, presented with a chronic history of repeated LN enlargement. The nodes had mild local discomfort and tenderness with associated fever. LN biopsy showed sinus histiocytosis with lymphophagocytosis characterized as emperipolesis and diagnosis of RDS was decided. After one year, the patient presented with high blood pressure, raised blood sugar, obesity, and abdominal pain. Computerized tomography showed a large adrenal mass, and the patient was treated urgently for his malignant blood pressure followed by surgical excision of the left adrenal mass. The biopsy subsequently revealed non-malignant adrenal tumor (pheochromocytoma). We are reporting successful treatment of rare case with RDS concomitantly presented with large adrenal pheochromocytoma. He was treated medically and surgically, the patient got recovery. RDS is a rare disease but we should consider it in patients with repeated benign lymphadenopathy. we are emphasizing that knowledge about RDS and its disease associations may be informative to the general medical community.
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