Changing Concepts of Diagnostic Criteria of Myeloproliferative Disorders and the Molecular Etiology and Classification of Myeloproliferative Neoplasms: From Dameshek 1950 to Vainchenker 2005 and Beyond

2014 ◽  
Vol 133 (1) ◽  
pp. 36-51 ◽  
Author(s):  
Jan Jacques Michiels ◽  
Zwi Berneman ◽  
Wilfried Schroyens ◽  
Hendrik De Raeve
Keyword(s):  
Bone Marrow ◽  
Polycythemia Vera ◽  
Myeloproliferative Neoplasms ◽  
Primary Myelofibrosis ◽  
Myeloproliferative Disorders ◽  
Jak2v617f Mutation ◽  
Wild Type ◽  
Mutation Load ◽  
Molecular Etiology ◽  
Calr Mutations

The Polycythemia Vera Study Group (PVSG) and WHO classifications distinguished the Philadelphia (Ph1) chromosome-positive chronic myeloid leukemia from the Ph1-negative myeloproliferative neoplasms (MPN) essential thrombocythemia (ET), polycythemia vera (PV) and primary myelofibrosis (MF) or primary megakaryocytic granulocytic myeloproliferation (PMGM). Half of PVSG/WHO-defined ET patients show low serum erythropoietin levels and carry the JAK2V617F mutation, indicating prodromal PV. The positive predictive value of a JAK2V617F PCR test is 95% for the diagnosis of PV, and about 50% for ET and MF. The WHO-defined JAK2V617F-positive ET comprises three ET phenotypes at clinical and bone marrow level when the integrated WHO and European Clinical, Molecular and Pathological (ECMP) criteria are applied: normocellular ET (WHO-ET), hypercellular ET due to increased erythropoiesis (prodromal PV) and hypercellular ET associated with megakaryocytic granulocytic myeloproliferation (EMGM). Four main molecular types of clonal MPN can be distinguished: JAK2V617F-positive ET and PV; JAK2 wild-type ET carrying the MPL515; mutations in the calreticulin (CALR) gene in JAK2/MPL wild-type ET and MF, and a small proportion of JAK2/MPL/CALR wild-type ET and MF patients. The JAK2V617F mutation load is low in heterozygous normocellular WHO-ET. The JAK2V617F mutation load in hetero-/homozygous PV and EMGM is clearly related to MPN disease burden in terms of splenomegaly, constitutional symptoms and fibrosis. The JAK2 wild-type ET carrying the MPL515 mutation is featured by clustered small and giant megakaryocytes with hyperlobulated stag-horn-like nuclei, in a normocellular bone marrow (WHO-ET), and lacks features of PV. JAK2/MPL wild-type, CALR mutated hypercellular ET associated with PMGM is featured by dense clustered large immature dysmorphic megakaryocytes and bulky (cloud-like) hyperchromatic nuclei, which are never seen in WHO-ECMP-defined JAK2V617F mutated ET, EMGM and PV, and neither in JAK2 wild-type ET carrying the MPL515 mutation. Two thirds of JAK2/MPL wild-type ET and MF patients carry one of the CALR mutations as the cause of the third distinct MPN entity. WHO-ECMP criteria are recommended to diagnose, classify and stage the broad spectrum of MPN of various molecular etiologies.

scholarly journals Preclinical characterization of the selective JAK1/2 inhibitor INCB018424: therapeutic implications for the treatment of myeloproliferative neoplasms

Blood ◽  
2010 ◽  
Vol 115 (15) ◽  
pp. 3109-3117 ◽  
Author(s):  
Alfonso Quintás-Cardama ◽  
Kris Vaddi ◽  
Phillip Liu ◽  
Taghi Manshouri ◽  
Jun Li ◽  
...  
Keyword(s):  
Polycythemia Vera ◽  
Interleukin 6 ◽  
Myeloproliferative Neoplasms ◽  
Primary Cultures ◽  
Myeloproliferative Neoplasm ◽  
Primary Myelofibrosis ◽  
Experimental Models ◽  
Jak2v617f Mutation ◽  
Erythroid Progenitor ◽  
Therapeutic Implications

AbstractConstitutive JAK2 activation in hematopoietic cells by the JAK2V617F mutation recapitulates myeloproliferative neoplasm (MPN) phenotypes in mice, establishing JAK2 inhibition as a potential therapeutic strategy. Although most polycythemia vera patients carry the JAK2V617F mutation, half of those with essential thrombocythemia or primary myelofibrosis do not, suggesting alternative mechanisms for constitutive JAK-STAT signaling in MPNs. Most patients with primary myelofibrosis have elevated levels of JAK-dependent proinflammatory cytokines (eg, interleukin-6) consistent with our observation of JAK1 hyperactivation. Accordingly, we evaluated the effectiveness of selective JAK1/2 inhibition in experimental models relevant to MPNs and report on the effects of INCB018424, the first potent, selective, oral JAK1/JAK2 inhibitor to enter the clinic. INCB018424 inhibited interleukin-6 signaling (50% inhibitory concentration [IC50] = 281nM), and proliferation of JAK2V617F+ Ba/F3 cells (IC50 = 127nM). In primary cultures, INCB018424 preferentially suppressed erythroid progenitor colony formation from JAK2V617F+ polycythemia vera patients (IC50 = 67nM) versus healthy donors (IC50 > 400nM). In a mouse model of JAK2V617F+ MPN, oral INCB018424 markedly reduced splenomegaly and circulating levels of inflammatory cytokines, and preferentially eliminated neoplastic cells, resulting in significantly prolonged survival without myelosuppressive or immunosuppressive effects. Preliminary clinical results support these preclinical data and establish INCB018424 as a promising oral agent for the treatment of MPNs.

Loss of Wild-Type Jak2 Allele Enhances Myeloid Cell Expansion and Accelerates Myelofibrosis in Jak2V617F Knock-in Mice

Blood ◽  
2012 ◽  
Vol 120 (21) ◽  
pp. 809-809
Author(s):  
Hajime Akada ◽  
Saeko Akada ◽  
Dongqing Yan ◽  
Robert Hutchison ◽  
Golam Mohi
Keyword(s):  
Polycythemia Vera ◽  
Cell Expansion ◽  
Conflicts Of Interest ◽  
Myeloproliferative Neoplasms ◽  
Philadelphia Chromosome ◽  
Myeloid Cell ◽  
Negative Regulator ◽  
Jak2v617f Mutation ◽  
Common Mutation ◽  
Wild Type

Abstract Abstract 809 The activating JAK2V617F mutation is the most common mutation found in Philadelphia chromosome (Ph)-negative myeloproliferative neoplasms (MPNs), which include polycythemia vera (PV), essential thrombocythemia (ET) and primary myelofibrosis (PMF). Although a majority of MPN patients carry heterozygous JAK2V617F mutation, loss of heterozygosity (LOH) on chromosome 9p involving JAK2 has been observed in ∼30% of patients with MPNs particularly in PV and PMF. JAK2V617F homozygosity through 9pLOH has been linked to more severe MPN phenotype. However, the contribution of 9pLOH in the pathogenesis of MPNs remains unclear. To investigate the role of wild-type JAK2 in MPNs induced by JAK2V617F, we have utilized conditional Jak2 knock-out and Jak2V617F knock-in alleles and generated heterozygous, hemizygous and homozygous Jak2V617F mice. Whereas heterozygous Jak2V617F expression results in a polycythemia vera-like disease in mice, loss of wild-type Jak2 allele in hemizygous or homozygous Jak2V617F mice results in a significantly greater increase in reticulocytes, white blood cells, neutrophils and platelets in the peripheral blood and larger spleen size. We also have found that hemizygous or homozygous Jak2V617F expression significantly increased megakaryocyte-erythroid progenitors in the bone marrow and spleens and marked infiltration of neutrophils in the liver compared with heterozygous Jak2V617F. More importantly, hemizygous or homozygous Jak2V617F mice show accelerated myelofibrosis compared with heterozygous Jak2V617F-expressing mice. Thus, loss of wild type Jak2 allele increases myeloid cell expansion and enhances the severity of the MPN. Together, these results suggest that wild-type Jak2 serves as a negative regulator of MPN induced by Jak2V617F. Disclosures: No relevant conflicts of interest to declare.

scholarly journals Transgenic expression of JAK2V617F causes myeloproliferative disorders in mice

Blood ◽  
2008 ◽  
Vol 111 (10) ◽  
pp. 5109-5117 ◽  
Author(s):  
Shu Xing ◽  
Tina Ho Wanting ◽  
Wanming Zhao ◽  
Junfeng Ma ◽  
Shaofeng Wang ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Transgenic Mice ◽  
Polycythemia Vera ◽  
Essential Thrombocythemia ◽  
Gene Promoter ◽  
Primary Myelofibrosis ◽  
Myeloproliferative Disorders ◽  
Transgenic Expression ◽  
Cell Counts

Abstract The JAK2V617F mutation was found in most patients with myeloproliferative disorders (MPDs), including polycythemia vera, essential thrombocythemia, and primary myelofibrosis. We have generated transgenic mice expressing the mutated enzyme in the hematopoietic system driven by a vav gene promoter. The mice are viable and fertile. One line of the transgenic mice, which expressed a lower level of JAK2V617F, showed moderate elevations of blood cell counts, whereas another line with a higher level of JAK2V617F expression displayed marked increases in blood counts and developed phenotypes that closely resembled human essential thrombocythemia and polycythemia vera. The latter line of mice also developed primary myelofibrosis-like symptoms as they aged. The transgenic mice showed erythroid, megakaryocytic, and granulocytic hyperplasia in the bone marrow and spleen, displayed splenomegaly, and had reduced levels of plasma erythropoietin and thrombopoietin. They possessed an increased number of hematopoietic progenitor cells in peripheral blood, spleen, and bone marrow, and these cells formed autonomous colonies in the absence of growth factors and cytokines. The data show that JAK2V617F can cause MPDs in mice. Our study thus provides a mouse model to study the pathologic role of JAK2V617F and to develop treatment for MPDs.

scholarly journals MPL W515 L/K mutations in myeloproliferative neoplasms

2019 ◽  
Vol 20 (1) ◽  
Author(s):  
Sohaila Eldeweny ◽  
Hosny Ibrahim ◽  
Ghada Elsayed ◽  
Mohamed Samra
Keyword(s):  
Bone Marrow ◽  
Polycythemia Vera ◽  
Ethnic Groups ◽  
Essential Thrombocythemia ◽  
Myeloproliferative Neoplasms ◽  
Primary Myelofibrosis ◽  
Myelogenous Leukemia ◽  
Egyptian Population ◽  
Ph Chromosome ◽  
Pathological Variant

Abstract Background Myeloproliferative neoplasms (MPNs) describe a group of diseases involving the bone marrow (BM). Classical MPNs are classified into chronic myelogenous leukemia (CML), polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (PMF). This classification is based on the presence of Philadelphia (Ph) chromosome (BCR/ABL1). CML is BCR/ABL1-positive while PV, ET, and PMF are negative. JAK2 p. Val617Phe pathological variant is the most associated mutation in BCR/ABL1-negative MPNs. The frequency of JAK2 p. Val617Phe is 90–95% in PV patients, 50–60% in ET, and 40–50% in patients with PMF. Studies on MPL gene led to the revelation of a gain of function pathological variants in JAK2 p. Val617Phe-negative myeloproliferative neoplasms (MPNs). MPL p. W515 L/K pathological variants are the most common across all mutations in MPL gene. The prevalence of these pathological variants over the Egyptian population is not clear enough. In the present study, we aimed to investigate the prevalence of MPL p. W515 L/K pathological variants in the Philadelphia (Ph)-negative MPNs over the Egyptian population. Results We have tested 60 patients with Ph-negative MPNs for MPL p. W515 L/K pathological variants. Median age was 51 (22–73) years. No MPL p. W515 L/K pathological variants were detected among our patients. JAK2 p. Val617Phe in PV and PMF patients showed significantly lower frequency than other studies. Splenomegaly was significantly higher in ET patients compared to other studies. Conclusion MPL p. W515 L/K pathological variants are rare across the Egyptian Ph-negative MPNs, and further studies on a large number are recommended. MPN patients in Egypt are younger compared to different ethnic groups.

scholarly journals Bone marrow megakaryocytic activation predicts fibrotic evolution of Philadelphia-negative myeloproliferative neoplasms.

Haematologica ◽  
2020 ◽  
pp. 0-0
Author(s):  
Mattia Schino ◽  
Vincenzo Fiorentino ◽  
Elena Rossi ◽  
Silvia Betti ◽  
Monica Di Cecca ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Myeloproliferative Neoplasms ◽  
Rapid Evolution ◽  
Progression Free Survival ◽  
Jak2 V617f ◽  
Primary Myelofibrosis ◽  
Serum Levels ◽  
Chronic Myeloproliferative Neoplasms ◽  
Calr Mutations ◽  
Wbc Count

Philadelphia-negative chronic myeloproliferative neoplasms (MPNs) have been traditionally considered as indistinctly slowly progressing conditions; recent evidence proves that a subset of cases have a rapid evolution, so that MPNs’ prognosis needs to be personalized. We identified a new morphological parameter, defined as Megakaryocytic Activation (M-ACT) based on the coexistence of megakaryocytic emperipolesis, megakaryocytes (MK) clusters formation and evidence of arrangement of collagen fibers around the perimeter of MK. We retrospectively analyzed the bone marrow biopsy of two MPNs cohorts of patients with polycythemia (PV) (n=64) and non-PV patients [including essential thrombocythemia (ET), and early/prefibrotic primary myelofibrosis (PMF)] (n=222). M-ACT showed a significant correlation with splenomegaly, white blood cell (WBC) count, and LDH serum levels in both groups, with JAK2 V617F allele burden in PV patients, and with CALR mutations, and platelet count in non-PV patients. Progression-free survival, defined as PV-to-secondary MF progression and non-PV-to-overt PMF, was worse in both PV and early/prefibrotic PMF patients with M-ACT in comparison to those without M-ACT (P<.0001). Interestingly, M-ACT was not found in the subgroup of ET patients. In conclusion, M-ACT can be helpful in the differential diagnosis of MPNs and can represent a new morphologic parameter with a predictive value for progression of MPNs.

The Behavior of Cytogenetic/Molecular Markers Associated with JAK2 Mutation Status Before and After Primary Myelofibrosis Progression Supports the Hypothesis of the Leukemic Clone's Independence From the JAK2 Mutation

Blood ◽  
2012 ◽  
Vol 120 (21) ◽  
pp. 5058-5058
Author(s):  
Francesco Albano ◽  
Antonella Zagaria ◽  
Luisa Anelli ◽  
Nicoletta Coccaro ◽  
Giuseppina Tota ◽  
...  
Keyword(s):  
Conflicts Of Interest ◽  
Myeloproliferative Neoplasms ◽  
Chronic Phase ◽  
Primary Myelofibrosis ◽  
Jak2v617f Mutation ◽  
Wild Type ◽  
Jak2 Mutation ◽  
Cytogenetic Abnormalities ◽  
Qrt Pcr ◽  
Leukemic Clone

Abstract Abstract 5058 Leukemic transformation has been reported in 15–30% of primary myelofibrosis (PMF). However, very little is known about the molecular bases responsible for acute myeloid leukemia (AML) transformation. JAK2 status analysis of paired chronic myeloproliferative neoplasms (MPN) and AML samples showed that during AML transformation the JAK2 mutation could be lost. Two probable models have been proposed to explain the MPN evolution to AML: a) JAK2-mutated AML usually arose from PMF or myelofibrotic transformation of ET/PV as a consequence of genetic instability conferred by the presence of JAK2 mutations; b) JAK2 wild-type AML generally developed in patients with chronic-phase ET or PV, frequently as a consequence of a clone selection driven by the therapy (Thoennissen NH et al. Blood 2010, 115:2882; Beer PA et al., Blood 2010, 115:2891; Spivak JL, Blood 2010, 115:2727). In this report we describe two PMF cases with the JAK2V617F mutation associated with molecular/cytogenetic abnormalities who developed JAK2-wild type AML characterized by a leukemic clone bearing a new cytogenetic aberration. At the PMF diagnosis, Case #1 showed a normal karyotype 46, XY[20] and resulted JAK2V617F positive. Molecular analyses performed at the time of AML transformation revealed the presence of a JAK2V617F negative clone bearing a novel t(12;18)(p13;q12) rearrangement. As the t(12;18) breakpoints were located centromerically to SETBP1 (18q12. 3), quantitative real-time PCR (qRT-PCR) experiments were made, showing SETBP1 overexpression. To investigate the occurrence of SETBP1 dysregulation and the presence of t(12;18) at PMF onset, qRT-PCR and Fluorescence in situ hybridization were performed, revealing gene overexpression and absence of the chromosomal translocation, respectively. At PMF onset, Case #2 harbored a novel t(3;5)(q27. 1;q31. 1) in addition to the JAK2V617F mutation. At the time of AML evolution, disappearance of the t(3;5)(q27. 1;q31. 1) and leukemic clone expansion of t(3;3)(q21. 3;q26. 2) was associated with disappearance of the JAK2V617F mutation. In contrast to literature data showing that JAK2 mutation loss is commonly associated with AML transformation after PV and ET, our findings suggest that evolution to JAK2-wild type AML could also occur in JAK2V617F PMF patients. The presence of different cytogenetic abnormalities associated with PMF and AML allowed us to follow the sequence of molecular events that lead to JAK2V617F disappearance, indicating that MPN and AML are clonally unrelated and probably generated by the transformation of different stem cell levels. Moreover, the well-documented clonal heterogeneity landscape in our cases demonstrated that the genomic instability responsible for AML transformation already existed at PMF onset and was not generated either by JAK2V617F mutation expression or by the therapy. Disclosures: No relevant conflicts of interest to declare.

Molecular Characterization Of Polycythemia Vera Based On The Relationship Of JAK2V617F and 9pUPD

Blood ◽  
2013 ◽  
Vol 122 (21) ◽  
pp. 1607-1607
Author(s):  
Linghua Wang ◽  
Sabina Swierczek ◽  
Kimberly Hickman ◽  
Soo-Jin Kim ◽  
David A. Wheeler ◽  
...  
Keyword(s):  
Polycythemia Vera ◽  
Conflicts Of Interest ◽  
Molecular Subtype ◽  
Myeloproliferative Neoplasms ◽  
Uniparental Disomy ◽  
Jak2v617f Mutation ◽  
Small Subset ◽  
Wild Type ◽  
The Stability ◽  
The Relationship

Abstract The gain-of-function mutation at codon 617 of JAK2 (JAK2V617F) is the most common somatic event observed in patients with polycythemia vera (PV), occurring in over 95% of PV patients. JAK2V617F confers cytokine hypersensitivity and cytokine-independent growth of erythroid progenitors, which are characteristic features of PV. Homozygous JAK2V617F is observed in about half of PV patients, whereas it is rarely seen in essential thrombocythemia (2-4%) and other myeloproliferative neoplasms. Homozygous JAK2V617F has been assumed to result from homozygous recombination, leading to uniparental disomy on 9p (9pUPD). It has been reported that the JAK2 46/1 (GGCC) haplotype may predispose carriers to the JAK2V617F mutation, and the JAK2V617F mutation facilities the acquisition of homozygous JAK2V617F. Challenging this view is a single study reporting 9pUPD in two PV subjects with wild-type JAK2, suggesting that in these two individuals, 9pUPD might have preceded the JAK2V617F mutation (Blood. 2011;118(24):6468-6470). However, the relationship between JAK2V617F and 9pUPD, the frequency of this new PV molecular subtype, its clinical relevance, and the stability of this genotype need to be systematically defined in a larger sample cohort. To address this, we combined whole-exome sequencing (WXS) of DNA from 31 consecutive PV patients with high-resolution SNP arrays, and further validated our findings in two additional cohorts comprising 59 PV consecutive patients collected from a single institution. In addition, we investigated the stability of each molecular subtype by using serial samples collected from 25 PV patients. We obtained an average of 125x coverage on JAK2 locus by WXS (Illumina Hiseq2000) and 2225x coverage by targeted deep sequencing using Ion PGM sequencer. Analysis of these data shows that the relationship between the JAK2 locus and 9pUPD is more complex than originally assumed. We defined 4 subgroups: 41% of patients had JAK2V617F in a heterozygous state without detectable 9pUPD (Subgroup I); 43% of patients had JAK2V617F with an allelic fraction in direct proportion to the level of 9pUPD (Subgroup II; homozygous JAK2V617F); 10% of patients harbored 9pUPD at approximately twice the level of the JAK2V617F allelic burden (Subgroup III; UPD with heterozygous JAK2V617F); and a small subset (6%) of patients exhibited trisomy of 9p, generating 3 copies of the JAK2 allele by chromosome duplication (Subgroup IV). No difference in the frequency of the JAK2 46/1 (GGCC) haplotype was found among these 4 subgroups. We found that this subtype classification was stable over time in over 60% of patients, whereas it transformed among the 9pUPD-positive subtypes in the remaining patients, indicating the outgrowth of a new PV subclone. While 2 PV patients with 9pUPD and wild-type JAK2 were previously reported (Blood. 2011;118(24):6468-6470), we now show a relative high proportion of PV patients having the novel, previously not recognized JAK2 genotype; i.e. JAK2 9pUPD with heterozygous JAK2V617F mutation. Our study will provide novel perspectives on the molecular basis of the evolution of PV and a better understanding of the roles of JAK2V617F and 9pUPD in this disease. Disclosures: No relevant conflicts of interest to declare.

scholarly journals A review of current knowledge of myeloproliferative disorders in the horse

2021 ◽  
Vol 63 (1) ◽  
Author(s):  
Katy Satué ◽  
Juan Carlos Gardon ◽  
Ana Muñoz
Keyword(s):  
Bone Marrow ◽  
Myeloid Leukemia ◽  
Current Knowledge ◽  
Myeloproliferative Neoplasms ◽  
Myeloproliferative Disorders ◽  
World Health ◽  
Chronic Granulocytic Leukemia ◽  
Current State ◽  
Multiple Cell ◽  
Health Organization

AbstractMyeloid disorders are conditions being characterized by abnormal proliferation and development of myeloid lineage including granulocytes (neutrophils, eosinophils and basophils), monocytes, erythroids, and megakaryocytes precursor cells. Myeloid leukemia, based on clinical presentation and proliferative rate of neoplastic cells, is divided into acute (AML) and myeloproliferative neoplasms (MPN). The most commonly myeloid leukemia reported in horses are AML-M4 (myelomonocytic) and AML-M5 (monocytic). Isolated cases of AML-M6B (acute erythroid leukemia), and chronic granulocytic leukemia have also been reported. Additionally, bone marrow disorders with dysplastic alterations and ineffective hematopoiesis affecting single or multiple cell lineages or myelodysplastic diseases (MDS), have also been reported in horses. MDSs have increased myeloblasts numbers in blood or bone marrow, although less than 20%, which is the minimum level required for diagnosis of AML. This review performed a detailed description of the current state of knowlegde of the myeloproliferative disorders in horses following the criteria established by the World Health Organization.

scholarly journals JAK2V617F mutation in a patient with B-cell chronic lymphocytic leukemia and prefibrotic primary myelofibrosis

2015 ◽  
Vol 143 (11-12) ◽  
pp. 739-743 ◽  
Author(s):  
Slobodan Ristic ◽  
Milica Radojkovic ◽  
Tatjana Kostic ◽  
Vesna Spasovski ◽  
Sonja Pavlovic ◽  
...  
Keyword(s):  
Chronic Lymphocytic Leukemia ◽  
B Cell ◽  
Myeloproliferative Neoplasms ◽  
Primary Myelofibrosis ◽  
Lymphocytic Leukemia ◽  
Jak2v617f Mutation ◽  
Predisposing Factor ◽  
Lymphoid Leukemia ◽  
Hematopoietic Stem ◽  
Cell Clones

Introduction. Secondary malignancies, particularly solid tumors, are common in patients with chronic lymphocytic leukemia (CLL), but association of myeloproliferative neoplasms and chronic lymphocytic leukemia in the same patient is very rare. Case Outline. We report of a 67-year-old man with B-cell chronic lymphoid leukemia (B-CLL) who developed primary myelofibrosis (PMF) nine years after initial diagnosis. Patient received alkylation agents and purine analogue, which can be a predisposing factor for the development of myeloproliferative neoplasms. JAK2V617F mutation was not present initially at the time of CLL diagnosis, but was found after nine years when PMF occurred, which indicates that B-CLL and PMF represent two separate clonal origin neoplasms. Conclusion. Pathogenic mechanisms for the development of myeloproliferative and lymphoproliferative neoplasms in the same patient are unknown. Further research is needed to determine whether these malignancies originate from two different cell clones or arise from the same pluripotent hematopoietic stem cell.

scholarly journals The Prevalence of Myeloproliferative Disorders in A Group of Iraqi Patients And Its Relation To Blood Indices Parameters

2020 ◽  
Vol 8 (A) ◽  
pp. 660-665
Author(s):  
Marwa Abdulnabi ◽  
Enass Abdul Kareem Dagher Al-Saadi
Keyword(s):  
Bone Marrow ◽  
Blood Cells ◽  
Myeloproliferative Neoplasms ◽  
Myeloproliferative Disorders ◽  
Myelogenous Leukemia ◽  
High Exposure ◽  
Female Ratio ◽  
Blood Samples ◽  
Blood Indices ◽  
Male To Female

AIM: The aim of this study was to measure the prevalence of myeloproliferative disorders in a sample of Iraqi patients and to measure the changes in patients’ blood parameters. BACKGROUND: Myeloproliferative disorders are a group of neoplasms affecting the bone marrow progenitor cells characterized by excess cells with a risk of transforming to acute leukemia. There is a gap in knowledge about the prevalence of Iraqi population. Thus, we investigated the prevalence and distribution of different types of myeloproliferative disorders in a sample of Iraqi patients. MATERIALS AND METHODS: Cross-sectional study is done at the National Center of Hematology from November 2019 till March 2020 on 75 patients who were diagnosed by a specialist hematopathologist to have one subtype of myeloproliferative disorders (MPDs). Blood samples were taken from them and analyzed to get complete blood count, blood film, bone marrow aspirate, and biopsy that were analyzed for each patient. Blood samples were taken from them and analyzed in terms of blood indices, which include red blood cells, white blood cells, and platelets. RESULTS: The 75 patients were found to be comprising 35 chronic myelogenous leukemia (CML) patients (46.7%), myelofibrosis 22 patients (29.3%), essential thrombocythemia (ET) 9 patients (12%), and polycythemia vera (PV) 9 patients (12%). In terms of male/female ratios, they were as follows: Myeloproliferative neoplasms (MPNs) male-to-female ratio is 1.2, CML= 0.94, myelofibrosis= 2.14 and ET= 0.5 and PV male-to-female ratio is 2. CONCLUSIONS : MPN male-to-female ratio in Iraq, which is 1.2, CML is the most common subtype. Regarding myelofibrosis, in our study, the male-to-female ratio is 2.14, which is much higher other countries. This could be attributed to high exposure to benzene and toluene which are well known to be causative agents for myelofibrosis. Regarding ET or PV, the male-to-female ratios were compatible with other countries.

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