Literature review - Polycythaemia Vera
Nicola Richards, Nichola H Lawrence, Indu Singh, Michelle Butina
Int. J. Bio. Lab. Sci 2021(10)2:112-123【PDF】

Polycythemia vera is a rare, acquired clonal disorder of the hematopoietic stem cells resulting in unregulated proliferation of erythropoiesis leading to an accumulation of red cells. The main complications of the disorder arise from hyperviscosity and an increased risk of thrombosis. In recent years, advances in next generation sequencing (NGS) techniques have identified promising targets to enable more accurate risk stratification of patients as well as targets for new classes of therapeutic drugs. This article aims to review the current research in this field to provide an overview of the key features of this disease.

Key words: Polycythemia vera, myeloproliferative neoplasms, erythrocytosis.

Literature review - Primary Myelofibrosis
Michelle Butina, Nicola Richards, Indu Singh, Nichola H Lawrence
Int. J. Bio. Lab. Sci 2021(10)2:102-111【PDF】

Primary myelofibrosis (PMF) is a Philadelphia negative myeloproliferative neoplasm characterized by bone marrow fibrosis, splenomegaly, anemia, constitutional symptoms, and extramedullary hematopoiesis. As a clonal hematopoietic stem cell disorder, it is often accompanied by a disease-initiating driver mutation and shortened survival. Diagnosis is often based on bone marrow findings. Diagnosis is supported by the presence of janus kinase 2 (JAK2), calreticulin (CALR), or thrombopoietin receptor protein (MPL) mutation, found in approximately 90% of patients. In 2016, the World Health Organization divided PMF into pre-fibrotic and overt categories to aid in distinguishing PMF from essential thrombocythemia. Several prognostic systems, using a variety of clinical and genetic features, have been developed to aid in therapeutic decision-making. Treatment focuses on alleviation of symptoms and an increase in overall survival. Treatment options have historically been limited. However, the therapeutic landscape is changing with the development of new JAK inhibitors.

Key words: Myelofibrosis, myeloproliferative neoplasm, prefibrotic myelofibrosis, primary myelofibrosis, overt myelofibrosis.

Literature review: An Introduction to Myeloproliferative Neoplasms
Michelle Butina, Nicola Richards, Nichola H Lawrence, Indu Singh
Int. J. Bio. Lab. Sci 2021(10)2:86-90【PDF】

The Philadelphia chromosome negative myeloproliferative neoplasms (MPNs) are a rare group of chronic hematological diseases that are closely related. They arise from one of three disease-initiating driver mutations that cause over-activation of the JAK-STAT pathway resulting in disease of the hematopoietic system. These three MPNs have overlapping clinical and diagnostic features making diagnosis challenging. The World Health Organization (WHO) diagnostic criteria continues to evolve as more advances are made in understanding these complex diseases. As such, prognostic models for risk stratification are also evolving and newer models (e.g., Mutation-Enhanced International Prognostic Score System) incorporate genetic and molecular features. The major and most common complications include thrombohemorrhagic manifestations and progression to acute leukemia. The development of Janus kinase inhibitors has changed the treatment landscape of MPNs, yet treatment options are at this time still limited due to the complexities of these diseases.

Key words: Myeloproliferative neoplasm, polycythemia vera, essential thrombocythemia, primary myelofibrosis.

Literature Review: Essential Thrombocythemia
Nichola H Lawrence, Indu Singh, Michelle Butina, Nicola Richards
Int. J. Bio. Lab. Sci 2021(10)2:91-101【PDF】

Essential Thrombocythemia (ET), a clonal hematopoietic stem cell disorder, is one of the classic Philadelphia negative myeloproliferative neoplasms and is characterized by thrombocytosis with bone marrow megakaryocytic hyperplasia. Mutations in Janus kinase 2 (JAK2), calreticulin (CALR), or myeloproliferative leukemia (MPL) are found in approximately 80-90% of patients. Over the past decade, new molecular and clinical knowledge in ET has led to a significant improvement in the diagnostic, prognostic, and therapeutic processes. Despite these advancements, many uncertainties remain regarding clinical decision-making. In the 2016 revised World Health Organization (WHO) classification true ET requires meeting all 4 major criteria described below or 3 major criteria and one minor criterion. Because of the revised WHO classification, study data has been re-evaluated and the revised International Prognostic Score of Thrombosis for Essential Thrombocythemia risk stratification was devised allowing clinicians to assign patients to the appropriate risk group in a 4-tiered system. In ET patient’s transformation to acute myeloid leukemia and/or post-ET myelofibrosis are rare events. Current treatment in ET is primarily indicated for the purposes of preventing vascular complications which have been reported to be the leading cause of death. Thrombotic complications can occur in up to 24% of patients with 13% developing a vascular event before diagnosis. Mutational status has an impact on thrombotic risk with a lower rate of thrombosis seen in CALR-mutants as compared to JAK2 V617F/MPL mutants and triple-negative cases. Mutations other than JAK2, CALR, or MPL have been found in approximately 53% of patients with ET with the most frequent being TET2, ASXL1, DNMT3A, and SF3B1.

Key words: Essential Thrombocythemia, JAK2 mutation, CALR mutation, MPL mutation, Platelets.

Literature review: Position of Laboratory Scientist, Analyst, and Technologist in Standard Occupation Classification
Bon-Kyeong Koo
Int. J. Bio. Lab. Sci 2021(10)2:75-85【PDF】

Terms for medical laboratory personnel were researched using code names in the standard occupational classification and the job titles for a total of 46 countries including the International Federation of Biomedical Laboratory Science (IFBLS) and European Association for Professions in Biomedical Science (EPBS) through Google search. In the case of the technologist or technician type, the identities used by medical laboratory personnel include biomedical laboratory health technician, clinical diagnostic laboratory technician, clinical laboratory technologist, medical laboratory technologist, medical laboratory technician, medical technologist, biomedical analysis technician, clinical analysis technician, and medical analytics technician. For the analyst type, professional titles include bio analyst, biomedical analyst, and medical analyst, whereas for the scientist type, professional designations include biomedical scientist and medical laboratory scientist. Additionally, other professional titles may include bioengineer and medical technical laboratory assistant. In most countries, medical laboratory technologists and technicians belong to the Major Group 3 Technicians and Associate Professionals in the International Standard Classification of Occupations 2008 (ISCO-08). Biomedical scientists or medical laboratory scientists in the United Kingdom (UK), Ireland, Australia, and New Zealand are categorized as Major Group 2 Professionals according to their standard occupational classification. Medical laboratory personnel must be distinguished in the International Standard Classification of Occupations because they have different education levels, experience levels, and responsibilities. Medical laboratory personnel with a bachelor's degree qualification should be moved to the Major Group 2 Professionals. Medical laboratory personnel with an associate degree or diploma qualification should be designated as Major Group 3 Technicians and Associate Professionals. A medical technologist and a medical technician or a medical laboratory technologist and a laboratory medical technician are not properly recognized by individuals with similar terms. This review proposes a new professional designation of "Medical Laboratory Analyst and Biomedical Analyst" as unified terms for medical laboratory personnel with a bachelor's degree qualification (excluding the title of medical laboratory scientist and biomedical scientist).

Key words: Associate professionals, Biomedical analyst and Medical laboratory analyst, Biomedical scientist and Medical laboratory scientist, Medical laboratory technologist and technician, Professionals

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