Eosinophilia-associated myeloproliferative neoplasms

2020 ◽  
pp. 204-221
Author(s):  
Andreas Reiter ◽  
Nicholas C.P. Cross
Keyword(s):  
Myeloproliferative Neoplasms ◽  
Jak2 V617f ◽  
Accurate Diagnosis ◽  
World Health ◽  
Disease Stage ◽  
Not Otherwise Specified ◽  
The World ◽  
Tk Inhibitors ◽  
Health Organization ◽  
Kit D816v

Accurate diagnosis of eosinophilia-associated disorders remains problematic. The World Health Organization (WHO) 2008 classification defines a rare subgroup: myeloid and lymphoid neoplasms with eosinophilia and abnormalities of PDGFRA, PDGFRB, or FGFR1 (MLN-eo), of which by far the most common is FIP1L1-PDGFRA. It is likely that other tyrosine kinase (TK) fusions will be incorporated into this category in due course. For other cases, the finding of increased numbers of blasts and/or proof of clonality is the basis for chronic eosinophilic leukaemia, not otherwise specified (CEL-NOS); however, in practice this diagnosis is only possible for a small minority of cases. It is also important to recognize cases that do not fulfil the diagnostic criteria for MLN-eo or CEL-NOS but who harbour KIT D816V or JAK2 V617F. As for treatment, disease stage (chronic/blast phase), potential clinical course (indolent/aggressive), potential sensitivity to imatinib, or alternative TK inhibitors and allogeneic stem cell transplantation need to be considered on an individual basis.

Haematopathology of classic myeloproliferative neoplasms

2020 ◽  
pp. 45-62
Author(s):  
Hans Michael Kvasnicka ◽  
Jürgen Thiele
Keyword(s):  
Continuous Improvement ◽  
Who Classification ◽  
Myeloproliferative Neoplasms ◽  
Primary Myelofibrosis ◽  
World Health ◽  
Polycythaemia Vera ◽  
The World ◽  
Health Organization ◽  
Diagnostic Importance

The classification of the World Health Organization (WHO) continues to advocate the diagnostic importance of bone marrow (BM) morphology in the diagnostic workup of myeloproliferative neoplasms (MPN). In this regard, distinctive histological BM patterns characterize specific subtypes of MPN and are the key to a meaningful clinical and molecular-defined risk stratification of patients. In this regard, the morphological denominator includes a characteristic megakaryocytic proliferation along with variable changes in the granulopoiesis and erythropoiesis. Importantly, diagnosis of MPN requires absence of relevant dysgranulopoiesis or dyserythropoiesis. In terms of clinical practice, the concept of precursor stages provides the possibility of an early intervention by appropriate therapeutic regimens that might prevent fatal complications like thrombosis and haemorrhage, especially in early stages of polycythaemia vera or in primary myelofibrosis. However, the WHO classification is not aimed to capture all biological true cases of MPN or guarantee a complete diagnostic specificity and thus might be in need of continuous improvement following clinical experience.

5-azacytidine for the treatment of massive hepatosplenomegaly in a case of myelodysplastic/myeloproliferative neoplasm-unclassifiable

2021 ◽  
Vol 2 (5) ◽  
Author(s):  
Mariarita Sciumè ◽  
◽  
Alessandro Loglio ◽  
Enrico Barozzi ◽  
Giusy Ceparano ◽  
...  
Keyword(s):  
Standard Treatment ◽  
Myeloproliferative Neoplasms ◽  
Myeloproliferative Neoplasm ◽  
World Health ◽  
World Health Organization Classification ◽  
Treatment Algorithms ◽  
Distinct Category ◽  
The World ◽  
Health Organization ◽  
Rare Diagnosis

Myelodysplastic/Myeloproliferative Neoplasms (MDS/MPN) represent a distinct category of myeloid diseases in the World Health Organization classification, defined at diagnosis by clinical, morphologic and laboratory features which overlap both those of MDS and MPN. Within the “Overlap” MDS/MPN syndromes, MDS/MPN-Unclassifiable (MDS/MPN-U) is the least well characterized. MDS/MPN-U is a rare diagnosis, making up less than 5% of all myeloid disorders with no standard prognostic or treatment algorithms. 5-azacytidine is a standard treatment for MDS, but controversial results are available about its role for MDS/MPN-U and its effectiveness on extramedullary disease and hepatosplenomegaly. We reported the clinical management of a MDS/MPN-U patient characterized by massive hepatosplenomegaly with optimal response to 5-azacytidine.

Molecular risk stratification of myeloproliferative neoplasms

2020 ◽  
pp. 79-92
Author(s):  
Paola Guglielmelli ◽  
Alessandro M. Vannucchi
Keyword(s):  
Mutational Analysis ◽  
Myeloproliferative Neoplasms ◽  
Jak2 V617f ◽  
World Health ◽  
Driver Mutations ◽  
Chronic Myeloproliferative Neoplasms ◽  
Molecular Landscape ◽  
Health Organization ◽  
The Impact ◽  
Risk Categories

Recent advances in understanding the molecular landscape of chronic myeloproliferative neoplasms (MPN) have remarkably improved the diagnostic approach to these disorders. The three phenotypic driver mutations, involving JAK2 (V617F, exon 12 mutations), MPL, and CALR, are major diagnostic criteria in the World Health Organization (WHO) classification, and point to different risk categories. Subclonal mutations in genes of the epigenetic regulation and the spliceosome deserve major prognostication significance and contribute to identify categories of patients with different survival and risk of leukaemia. This chapter will address these aspects and elucidate how mutational analysis may contribute to advanced assessment of MPN patients, as well as its the impact on prognosis for those with MPN.

scholarly journals Acquired Myelodysplasia or Myelodysplastic Syndrome: Clearing the Fog

2013 ◽  
Vol 2013 ◽  
pp. 1-11 ◽  
Author(s):  
Ethan A. Natelson ◽  
David Pyatt
Keyword(s):  
Myeloid Leukemia ◽  
Myeloproliferative Neoplasms ◽  
Bone Marrow Failure ◽  
Myeloproliferative Disorders ◽  
World Health ◽  
Clinical Settings ◽  
Hematologic Disorders ◽  
Immune Mediated ◽  
The World ◽  
Health Organization

Myelodysplastic syndromes (MDS) are clonal myeloid disorders characterized by progressive peripheral blood cytopenias associated with ineffective myelopoiesis. They are typically considered neoplasms because of frequent genetic aberrations and patient-limited survival with progression to acute myeloid leukemia (AML) or death related to the consequences of bone marrow failure including infection, hemorrhage, and iron overload. A progression to AML has always been recognized among the myeloproliferative disorders (MPD) but occurs only rarely among those with essential thrombocythemia (ET). Yet, the World Health Organization (WHO) has chosen to apply the designation myeloproliferative neoplasms (MPN), for all MPD but has not similarly recommended that all MDS become the myelodysplastic neoplasms (MDN). This apparent dichotomy may reflect the extremely diverse nature of MDS. Moreover, the term MDS is occasionally inappropriately applied to hematologic disorders associated with acquired morphologic myelodysplastic features which may rather represent potentially reversible hematological responses to immune-mediated factors, nutritional deficiency states, and disordered myelopoietic responses to various pharmaceutical, herbal, or other potentially myelotoxic compounds. We emphasize the clinical settings, and the histopathologic features, of such AMD that should trigger a search for a reversible underlying condition that may be nonneoplastic and not MDS.

Classification of Myeloproliferative Neoplasms and Prognostic Factors

2012 ◽  
pp. 419-424 ◽  
Author(s):  
Francesco Passamonti
Keyword(s):  
Leukocyte Count ◽  
Myeloproliferative Neoplasms ◽  
Jak2 V617f ◽  
World Health ◽  
Red Blood Cell Transfusion ◽  
International Prognostic Scoring System ◽  
Prior History ◽  
Prognostic Information ◽  
Health Organization ◽  
Two Parameters

Overview: Myeloproliferative neoplasms (MPNs) are currently diagnosed according to the World Health Organization (WHO) criteria. Molecular profiling should include the analysis of JAK2 V617F (first, exon 12 only in V617F-negative polycythemia vera [PV]) and MPL mutations (in V617F-negative essential thrombocythemia [ET] and myelofibrosis [MF]). For patients with PV and ET, the risk stratification of low- and high-risk disease requires only two parameters: older than age 60 and prior history of thrombosis. Additionally, it might be important to monitor leukocyte count and know the mutational profile. Survival of patients with MF is defined by the International Prognostic Scoring System (IPSS) model at diagnosis and the Dynamic IPSS (DIPSS) anytime during the course of the disease. The IPSS and the DIPSS are based on patient age older than age 65, presence of constitutional symptoms, hemoglobin level less than 10 g/dL, leukocyte count greater than 25 × 109/L, and circulating blast cells 1% or greater. The DIPSS-plus adds critical prognostic information and suggests also considering cytogenetic categories, platelet count, and red blood cell transfusion need.

scholarly journals Potassium Levels in COVID Subjects: Current Observations and New Possibilities for its use in COVID Diagnosis

2021 ◽  
pp. 1-8
Author(s):  
Sriram Padmanabhan
Keyword(s):  
Clinical Presentation ◽  
Accurate Diagnosis ◽  
World Health ◽  
Detection Methods ◽  
Potassium Ions ◽  
Infected Person ◽  
Pharmacological Treatments ◽  
The World ◽  
Health Organization ◽  
Global Threat

Severe Acute Respiratory Syndrome Coronavirus-2 (SARS-CoV-2) causing covid infection in humans is a major global threat to healthcare and economy. According to the recent statistics of the World Health Organization (WHO), the disease has already involved all continents, with almost 117,498,522 cases with more than 2,606,626 deaths all over the globe until March 2021. It is thus, imperative to study and develop pharmacological treatments suitable for the prevention and treatment ofCOVID-19. The COVID causing virus is mainly transmitted through cough or sneeze droplets generated by an infected person. Hence its early and accurate diagnosis appears essential for minimizing spread, prevention and eventually containment of the pandemic. Also, since the clinical presentation of the COVID infection is varied starting from asymptomatic to severe cases, it reinforces the need for detection methods that are simple, early and with good sensitivity and specificity. This article reviews impact of potassium ions in functioning of various organs in humans and its possible role in COVID disease progression.

scholarly journals Primary cutaneous DLBCL non-GCB type: challenges of a rare case

Open Medicine ◽  
2020 ◽  
Vol 15 (1) ◽  
pp. 119-125 ◽  
Author(s):  
Antonello Sica ◽  
Paola Vitiello ◽  
Stefano Caccavale ◽  
Caterina Sagnelli ◽  
Armando Calogero ◽  
...  
Keyword(s):  
B Cell ◽  
World Health ◽  
B Cell Lymphomas ◽  
Not Otherwise Specified ◽  
Cutaneous Lymphomas ◽  
The World ◽  
Molecular Landscape ◽  
Health Organization

AbstractSeveral types of B-cell lymphomas, including both primary cutaneous lymphomas and systemic lymphomas, may affect the skin, with partially overlapping clinical, morphological and immunohistochemical features. Currently, the World Health Organization (WHO) classification of primary cutaneous B-cell lymphomas does not include diffuse large B-cell lymphomas (DLBCL) and considers leg-type DLBCL the only primary cutaneous DLBCL. Here we report the case of a 72-year-old white woman with a primary cutaneous neoplasm comprised of large cells with round nuclei, irregularly clumped chromatin and one or more inconspicuous nucleoli. The immunohistochemistry demonstrated positivity for CD20 and MUM1, with no significant genetic translocations detected by fluorescence in-situ hybridization. After staging, we considered this neoplasm a primary cutaneous DLBCL with a non-germinal center phenotype, not otherwise specified, inconsistent with a leg-type DLBCL. Because of this view, we underscore the need for greater knowledge of the molecular landscape of B-cell lymphomas in order to reconsider the classification of such neoplasms in the skin.

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