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   Table of Contents - Current issue
Coverpage
January-March 2020
Volume 11 | Issue 1
Page Nos. 1-37

Online since Friday, March 13, 2020

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REVIEW ARTICLE  

Pure red cell aplasia, a disease of a great diversity p. 1
Mona M Alfaraj, Hussain H Al Saeed
DOI:10.4103/joah.joah_63_19  
Pure red cell aplasia (PRCA) is a type of normocytic or sometimes macrocytic anemia characterized by reticulocytopenia. The cause of hypo erythropoiesis is varied. It could be of a congenital or an acquired type. A congenital PRCA is different than anemia caused by congenital dyserythropoiesis. The classification of acquired PRCA can be primary when no cause is identified or secondary due to underling or associated pathology. The primary PRCA is a disease of exclusion. There are many disorders such as autoimmune disorders, hematological and nonhematological malignancies, some infections, and medications that cause secondary PRCA. Immune-mediated mechanism plays an important role in both primary and secondary types. Therefore, immunomodulating agents are important in the treatment of primary type and many secondary types. Other therapy modalities include surgery, plasmapheresis, and hemopoietic stem cell transplantation. The assessment of hemoglobin level and absolute reticulocyte count with the frequency of blood transfusion are important to monitor therapy response achievement. Achievement of complete response is important to eliminate demands for blood transfusion and to avoid blood-transfusion risks. Herein, we review this disorder which has broad diversity, its causes, approach to its cause, therapy modalities.
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ORIGINAL ARTICLES Top

Suggested modification of Janus associated kinase 2-tree algorithm for the detection of Janus associated kinase 2 V617F-positive polycythemia rubra vera patients in Pakistani population p. 7
Mohammad Abdul Naeem, Mohammad Iqbal
DOI:10.4103/joah.joah_60_19  
OBJECTIVE: The Janus associated kinase-2 (JAK2 mutation V617F and exon 12) is detected in most polycythemia vera (PV) patients. It can easily be picked up by real-time polymerase chain reaction (PCR). This technique is easy to perform and is very sensitive requiring minimally invasive techniques. The decision when to order JAK2 mutation test by PCR is important to curb its wastage. To save precious laboratory resources, a peripheral blood finding-based algorithm adjusted to hemoglobin levels of patients (PV) in Pakistan has been purposed, which is a modification of algorithm recently developed by Mahe et al. for the rationalization of JAK2 analysis. METHODS: To assist with the screening of patients being considered for JAK mutation for PV patients in the Pakistani population, we modified clinical decision rule “JAK2-tree” as modified JAK2-tree based on patients' full blood count. RESULTS: We tested both classical and modified JAK2-tree algorithms on two independent data sets, one an unselected population-based sample comprising 51 individuals and other on historical clinical laboratory referral set comprising 51 JAK2-positive cases of PV. Sensitivity for both the algorithms was calculated and compared. CONCLUSION: Our work supports a “modified” decision-tree-based screening approach for Pakistani population to optimize the selection of patients most appropriate for JAK2 V617F testing.
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Epidemiology and characteristics of sickle cell patients admitted to hospitals in Jazan region, Saudi Arabia Highly accessed article p. 10
Ahmad Ali Hazzazi, Mohssen Hassen Ageeli, Abdulaziz Mohammed Alfaqih, Abdullah Ali Jaafari, Hafiz Mosa Malhan, Mohammed Mosa Bakkar
DOI:10.4103/joah.joah_67_19  
INTRODUCTION: Sickle cell anemia (SCA) is an autosomal recessive illness caused by the formation of abnormal hemoglobin S. Sickle cell disease has many complications such as vaso-occlusive crisis (VOC), gallstones, stroke, acute chest syndrome (ACS), and others. OBJECTIVES: This study aimed to describe the epidemiology and characteristics of sickle cell patients admitted to hospitals in Jazan region. MATERIALS AND METHODS: A cross-sectional study conducted in four hospitals in Jazan region including King Fahad Central Hospital, Prince Mohammed Bin Nasser Hospital, Sabya, and Abu-Arish General Hospital to estimate the prevalence of admitted sickle cell patients and their characteristics. All patients admitted to medicine and pediatric wards from July 15, 2018, to August 15, 2018, were included in the study. Data were collected by interview. RESULTS: Four hundred and two of 446 patients have agreed to participate with a response rate of 90%. Ninety-one (22.6%) were SCA patients, among them, 45 (49.5%) were male and 46 (50.1%) were female, with a mean age of 18.81 ± 11.05 years. Most of them were admitted due to VOC 56% (n = 51) and ACS 12.1% (n = 11). Almost all sickle patients had VOC 91.2% (n = 83), and more than half of them had ACS 58.2% (n = 53) as a complication. CONCLUSIONS: SCA was the number one cause of admissions in this study. Approximately, one out of each four patients admitted to Jazan hospitals is a sickle cell patient. VOC represents the most frequent complication of sickle disease, followed by ACS. Further studies and interventions to reduce this burden and improve sickle cell patients' health status are recommended.
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Methylation status of p15 and E-cadherin gene in a cohort of Egyptian acute monocytic leukemia patients p. 15
Neveen Lewis Mikhael, Mona Wagdy Ayad, Omar Ghallab, Irene Lewis Mikhael
DOI:10.4103/joah.joah_78_19  
BACKGROUND: Acute monocytic leukemia (AML) is a common hematological malignancy with different subtypes. AML with monocytic involvement (AML M5) is the most frequent subtype among adults. Many genetic and epigenetic abnormalities were described in adult AML. Methylation status in AML is studied for classification, as prognostic marker and even for therapeutic implications. p15 and E-cadherin promotor region methylation are frequently implicated in different kinds of hematological and nonhematological malignancies. AIM OF THE WORK: The aim is to detect methylation status of p15 and E-cadherin in a group of adult Egyptian monocytic leukemia patients and relate it to further events; relapse and central nervous system (CNS) infiltration. Patients and methods: The study was conducted on 76 acute monocytic leukemia patients and 50 healthy controls. PCR specific methylation for p15 and E cadherin was done for both groups. RESULTS: p15 and E-cadherin aberrant methylation are very frequent in adult acute monocytic leukemia. p15 methylation is related to CNS infiltration, while p15 and E-cadherin methylation status is related to relapse. CONCLUSIONS: Methylation of p15 and E-cadherin can be added to prognostic markers of acute monocytic leukemias.
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CASE REPORTS Top

The intriguing occurrence of Acute Myeloid Leukemia in a case of Acute Lymphoblastic leukemia:Report of two cases p. 21
Manasi Chetan Mundada, Faiq Ahmed, Mohan Krishna Pasam, Sudha Murthy, A Santa, Veerendra Patil
DOI:10.4103/joah.joah_64_19  
The aim of this study is to study the two cases of acute lymphoblastic leukemia (ALL) who relapsed as acute myeloid leukemia (AML). Presented here are reports of two cases of pediatric ALL who developed change of lineage to AML at relapse. This change in phenotype, which involves the conversion of one phenotype to other phenotype during the course of disease or at relapse is a rare phenomenon rarely described in literature. The immunophenotypic and molecular findings are described. The present study emphasizes the need of immunophenotyping and molecular workup at relapse. Also adds to the repertoire of the published literature on this rare entity.
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The double jeopardy of leukemia and dengue: A report of three cases p. 25
Reshma Gopal Kini, Christol Blanch Moras
DOI:10.4103/joah.joah_66_19  
Dengue is predominantly a self-limiting illness. The association of dengue with new onset of leukemia has been rarely reported. We describe herein a series of three patients diagnosed with acute lymphoblastic leukemia, chronic myeloid leukemia, and acute promyelocytic leukemia who presented with concurrent dengue infection at a tertiary care institute in the southwestern coastal region of India. In spite of the different types of leukemias, we observed similar trends in their blood parameters, which were comparable with those of nonleukemic dengue patients. The transfusion profile of each of these patients is described. We could conclude that even in the presence of leukemia, dengue tends to be self-limited. No such comparative case reports have been published so far, and with an increasing incidence of dengue in the world, the occurrence of the two might not remain a remote possibility.
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Isolated myeloid sarcoma: A unique porta hepatis location in a 2-year-old child p. 29
Adil Abdelhamed Abbas, Hatim Qasim Almaghraby
DOI:10.4103/joah.joah_73_19  
Myeloid sarcoma (MS) or chloroma represents extramedullary accumulation of immature myeloid cells. In the World Health Organization classifications, these tumors are called granulocytic sarcomas. MS may occur concomitantly or precede the development of myeloid tumors such as acute myeloblastic leukemia (AML) by several weeks to months. Occasionally, MS represents extramedullary blast transformation of chronic myeloid leukemia and other chronic myeloproliferative neoplasms and myelodysplastic syndromes or indicates relapse in previously treated patients. Immunohistochemistry represents the gold standard of histopathological diagnosis, given the similarity of the MS medium to large-size tumor cells to many other malignant cell infiltrates making them hard to identify. Diagnosis is often difficult to reach and is missed in up to 50% of the isolated MS lesions, especially when immunohistochemistry is not properly used. MS can affect variety of body tissue organs such as the skin, bone, gastrointestinal tract, mucosal tissue, and the central nervous system. Isolated MS of the biliary system is rare. We report a 2-year-old boy with isolated MS affecting the porta hepatis (PH) who responded well to AML-type chemotherapy. Few cases of MS were reported in pediatric patients affecting the liver and biliary system. The development of isolated MS in the PH is extremely rare and has not been previously reported in the literature. To our knowledge, this is the first child to be reported with sole involvement of such location.
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BRIEF REPORT Top

Inherited and acquired thrombophilia as a modifier of clinical course of chronic immune thrombocytopenia p. 33
M Kumar, A Panigrahi, P Mondal, R De, S Datta, T Dolai, P Chakraborty, M Bhattacharyya, M Ghosh
DOI:10.4103/joah.joah_75_19  
Patients with immune thrombocytopenia (ITP) exhibit striking heterogeneity in bleeding manifestations even at similar platelet counts. We report the prevalence and impact of thrombophilia marker expression in chronic ITP patients. For the present study, patients with chronic ITP were clinically assessed at regular intervals using the bleeding assessment tool for ITP, and bleeding was compared among patients with and without thrombophilia marker expression (thrombophilia markers analyzed included clot-based assays for protein C, protein S, Pro C Global®, FVIII levels, and lupus anticoagulant assay). Thirty-six patients (25.5%) tested positive for at least one thrombophilia marker, and the remaining 105 patients (74.5%) were negative for all markers. Patients expressing at least one thrombophilia marker had significantly less bleeding than those without. We conclude that a part of heterogeneity in the clinical presentation of chronic ITP can be explained by the presence of thrombophilia.
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IMAGE IN HEMATOLOGY Top

Inferior vena cava filter eroding the aorta p. 37
Mayyadah H Alabdely, Hazza A Alzahrani
DOI:10.4103/joah.joah_71_19  
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