scholarly journals Pure Erythroid Leukaemia- Report of An Extremely Rare Case with Dismal Prognosis

2021 ◽  
Vol 39 (4) ◽  
pp. 266-268
Author(s):  
SM Khodeza Nahar Begum ◽  
MA Khan
Keyword(s):  
De Novo ◽  
World Health ◽  
Abnormal Karyotype ◽  
Clinicopathologic Characteristics ◽  
Male Predominance ◽  
Aggressive Form ◽  
Myeloid Neoplasms ◽  
Dismal Prognosis ◽  
The World ◽  
Health Organization

Pure erythroid leukaemia (PEL) is a rare and aggressive form of acute leukaemia whose biology remains poorly characterized. The category of acute erythroid leukaemia was signiûcantly revised in the 2016 revision to the World Health Organization (WHO) classiûcation of myeloid neoplasms. In the previous 2008 WHO classiûcation, acute erythroid leukaemia was categorized into two subtypes: erythroleukaemia and pure erythroid leukaemia (PEL), whereas in the 2016 WHO update, erythroleukaemia was merged into myelodysplastic syndrome and PEL becomes the only type of acute erythroid leukaemia. De novo pure erythroid leukaemia is a disease of adults (median age 68 years), exhibits a striking male predominance, is universally associated with an abnormal karyotype and has an exceedingly poor overall median survival of 1.4 months. Given the limited number of reports of this rare and diagnostically challenging entity, we report clinicopathologic characteristics of a case of PEL, diagnosis was made by the bone marrow morphology and immunophenotyping. J Bangladesh Coll Phys Surg 2021; 39: 266-268

Clinico-pathologic characteristics and outcomes of the World Health Organization (WHO) provisional entity de novo acute myeloid leukemia with mutated RUNX1

2020 ◽  
Vol 33 (9) ◽  
pp. 1678-1689
Author(s):  
Andrés E. Quesada ◽  
Guillermo Montalban-Bravo ◽  
Rajyalakshmi Luthra ◽  
Keyur P. Patel ◽  
Koji Sasaki ◽  
...  
Keyword(s):  
Acute Myeloid Leukemia ◽  
World Health Organization ◽  
Myeloid Leukemia ◽  
De Novo ◽  
World Health ◽  
The World ◽  
Health Organization ◽  
Acute Myeloid

The World Health Organization (WHO) classification of the myeloid neoplasms

Blood ◽  
2002 ◽  
Vol 100 (7) ◽  
pp. 2292-2302 ◽  
Author(s):  
James W. Vardiman ◽  
Nancy Lee Harris ◽  
Richard D. Brunning
Keyword(s):  
World Health Organization ◽  
Who Classification ◽  
World Health ◽  
Classification Schemes ◽  
Myeloid Neoplasms ◽  
Lymphoid Neoplasms ◽  
The World ◽  
Health Organization ◽  
Collaborative Efforts

A World Health Organization (WHO) classification of hematopoietic and lymphoid neoplasms has recently been published. This classification was developed through the collaborative efforts of the Society for Hematopathology, the European Association of Hematopathologists, and more than 100 clinical hematologists and scientists who are internationally recognized for their expertise in hematopoietic neoplasms. For the lymphoid neoplasms, this classification provides a refinement of the entities described in the Revised European-American Lymphoma (REAL) Classification—a system that is now used worldwide. To date, however, there has been no published explanation or rationale given for the WHO classification of the myeloid neoplasms. The purpose of this communication is to outline briefly the WHO classification of malignant myeloid diseases, to draw attention to major differences between it and antecedent classification schemes, and to provide the rationale for those differences.

scholarly journals Myeloid neoplasms with eosinophilia

Blood ◽  
2017 ◽  
Vol 129 (6) ◽  
pp. 704-714 ◽  
Author(s):  
Andreas Reiter ◽  
Jason Gotlib
Keyword(s):  
De Novo ◽  
Myeloproliferative Neoplasms ◽  
Disease Free Survival ◽  
World Health ◽  
Hematopoietic Stem ◽  
Chronic Eosinophilic Leukemia ◽  
Molecular Abnormalities ◽  
Myeloid Neoplasms ◽  
Variable Sensitivity ◽  
Health Organization

Abstract Molecular diagnostics has generated substantial dividends in dissecting the genetic basis of myeloid neoplasms with eosinophilia. The family of diseases generated by dysregulated fusion tyrosine kinase (TK) genes is recognized by the World Health Organization (WHO) category, “Myeloid/lymphoid neoplasms with eosinophilia and rearrangement of PDGFRA, PDGFRB, or FGFR1, or with PCM1-JAK2.” In addition to myeloproliferative neoplasms (MPN), these patients can present with myelodysplastic syndrome/MPN, as well as de novo or secondary mixed-phenotype leukemias or lymphomas. Eosinophilia is a common, but not invariable, feature of these diseases. The natural history of PDGFRA- and PDGFRB-rearranged neoplasms has been dramatically altered by imatinib. In contrast, patients with FGFR1 and JAK2 fusion TK genes exhibit a more aggressive course and variable sensitivity to current TK inhibitors, and in most cases, long-term disease-free survival may only be achievable with allogeneic hematopoietic stem cell transplantation. Similar poor prognosis outcomes may be observed with rearrangements of FLT3 or ABL1 (eg, both of which commonly partner with ETV6), and further investigation is needed to validate their inclusion in the current WHO-defined group of eosinophilia-associated TK fusion-driven neoplasms. The diagnosis chronic eosinophilic leukemia, not otherwise specified (CEL, NOS) is assigned to patients with MPN with eosinophilia and nonspecific cytogenetic/molecular abnormalities and/or increased myeloblasts. Myeloid mutation panels have identified somatic variants in patients with a provisional diagnosis of hypereosinophilia of undetermined significance, reclassifying some of these cases as eosinophilia-associated neoplasms. Looking forward, one of the many challenges will be how to use the results of molecular profiling to guide prognosis and selection of actionable therapeutic targets.

The 2008 revision of the World Health Organization (WHO) classification of myeloid neoplasms and acute leukemia: rationale and important changes

Blood ◽  
2009 ◽  
Vol 114 (5) ◽  
pp. 937-951 ◽  
Author(s):  
James W. Vardiman ◽  
Jüergen Thiele ◽  
Daniel A. Arber ◽  
Richard D. Brunning ◽  
Michael J. Borowitz ◽  
...  
Keyword(s):  
Acute Leukemia ◽  
World Health Organization ◽  
Who Classification ◽  
World Health ◽  
Lymphoid Tissues ◽  
Myeloid Neoplasms ◽  
The World ◽  
Genetic Features ◽  
Health Organization

Recently the World Health Organization (WHO), in collaboration with the European Association for Haematopathology and the Society for Hematopathology, published a revised and updated edition of the WHO Classification of Tumors of the Hematopoietic and Lymphoid Tissues. The 4th edition of the WHO classification incorporates new information that has emerged from scientific and clinical studies in the interval since the publication of the 3rd edition in 2001, and includes new criteria for the recognition of some previously described neoplasms as well as clarification and refinement of the defining criteria for others. It also adds entities—some defined principally by genetic features—that have only recently been characterized. In this paper, the classification of myeloid neoplasms and acute leukemia is highlighted with the aim of familiarizing hematologists, clinical scientists, and hematopathologists not only with the major changes in the classification but also with the rationale for those changes.

scholarly journals The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia

Blood ◽  
2016 ◽  
Vol 127 (20) ◽  
pp. 2391-2405 ◽  
Author(s):  
Daniel A. Arber ◽  
Attilio Orazi ◽  
Robert Hasserjian ◽  
Jürgen Thiele ◽  
Michael J. Borowitz ◽  
...  
Keyword(s):  
Acute Leukemia ◽  
World Health Organization ◽  
Who Classification ◽  
World Health ◽  
Lymphoid Tissues ◽  
Acute Leukemias ◽  
Myeloid Neoplasms ◽  
The World ◽  
Health Organization

Abstract The World Health Organization (WHO) classification of tumors of the hematopoietic and lymphoid tissues was last updated in 2008. Since then, there have been numerous advances in the identification of unique biomarkers associated with some myeloid neoplasms and acute leukemias, largely derived from gene expression analysis and next-generation sequencing that can significantly improve the diagnostic criteria as well as the prognostic relevance of entities currently included in the WHO classification and that also suggest new entities that should be added. Therefore, there is a clear need for a revision to the current classification. The revisions to the categories of myeloid neoplasms and acute leukemia will be published in a monograph in 2016 and reflect a consensus of opinion of hematopathologists, hematologists, oncologists, and geneticists. The 2016 edition represents a revision of the prior classification rather than an entirely new classification and attempts to incorporate new clinical, prognostic, morphologic, immunophenotypic, and genetic data that have emerged since the last edition. The major changes in the classification and their rationale are presented here.

scholarly journals A Nature-Inspired Design Yields a New Class of Steroids Against Trypanosomatids

Molecules ◽  
2019 ◽  
Vol 24 (20) ◽  
pp. 3800
Author(s):  
Elena Aguilera ◽  
Cintya Perdomo ◽  
Alejandra Espindola ◽  
Ileana Corvo ◽  
Paula Faral-Tello ◽  
...  
Keyword(s):  
De Novo ◽  
Low Cost ◽  
World Health ◽  
New Class ◽  
The World ◽  
Low Toxicity ◽  
Health Organization ◽  
Toxicity In Vitro

Chagas disease and Leishmaniasis are neglected endemic protozoan diseases recognized as public health problems by the World Health Organization. These diseases affect millions of people around the world however, efficient and low-cost treatments are not available. Different steroid molecules with antimicrobial and antiparasitic activity were isolated from diverse organisms (ticks, plants, fungi). These molecules have complex structures that make de novo synthesis extremely difficult. In this work, we designed new and simpler compounds with antiparasitic potential inspired in natural steroids and synthesized a series of nineteen steroidal arylideneketones and thiazolidenehydrazines. We explored their biological activity against Leishmania infantum, Leishmania amazonensis, and Trypanosoma cruzi in vitro and in vivo. We also assayed their genotoxicity and acute toxicity in vitro and in mice. The best compound, a steroidal thiosemicarbazone compound 8 (ID_1260) was active in vitro (IC50 200 nM) and in vivo (60% infection reduction at 50 mg/kg) in Leishmania and T. cruzi. It also has low toxicity in vitro and in vivo (LD50 >2000 mg/kg) and no genotoxic effects, being a promising compound for anti-trypanosomatid drug development.

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