scholarly journals Comparison of Clinical Characteristics and Prognosis of a Series of Patients from the Spanish Registry of MDS Diagnosed with Myelodysplastic/Myeloproliferative Neoplasms According to the 2016 Reviewed Classification of the World Health Organization

Blood ◽  
2018 ◽  
Vol 132 (Supplement 1) ◽  
pp. 3048-3048
Author(s):  
Blanca Xicoy ◽  
María-José Jimenez ◽  
Olga García ◽  
Marisa Calabuig ◽  
Rosa Coll ◽  
...  
Keyword(s):  
Lactate Dehydrogenase ◽  
Clinical Characteristics ◽  
Who Classification ◽  
Conflicts Of Interest ◽  
Myeloproliferative Neoplasms ◽  
World Health ◽  
Platelet Counts ◽  
Number Of Patients ◽  
The World

Abstract Background: The 2016 reviewed classification of the World Health Organisation (WHO) defines a group of myelodysplastic/myeloproliferative neoplasms (MDS/MPN) including the chronic myelomonocytic leukemia (CMML), the myelodyplastic syndrome with ring sideroblasts and thrombocytosis (MDS-RS-T) and the MDS/MPN unclassifiable (MDS/MPN-U). The presence of typical clinical characteristics of MDS and MPN hinders the diagnosis and the prognosis of MDS/MPN-U. Aim: The objective of this study was to compare the clinical characteristics and the prognosis of a series of patients with MDS/MPN such as CMML, MDS-RS-T and MDS/NPM-U from the Spanish registry of MDS. Method: We analized 107 patients diagnosed with MDS/MPN (MDS-RS-T, MDS/NPM-U and CMML) according to the 2016 WHO classification. A comparison of the clinical characteristics, overall survival (OS) and cumulative incidence of progression (CIP) was performed. Results: Median (range) age was 74 (23-93) years and 68/107 (64%) were males. The number of patients in each group was: MDS-RS-T (n=45), MDS/MPN-U (n=29) and CMML (n=33). The main clinical characteristics of the three groups are described in Table 1. There were significant statistical differences in hemoglobin and lactate dehydrogenase levels and leukocyte, monocyte and platelet counts between the three groups. With a median (range) of follow-up of 3.1 (0.3-19.3), 3.7(0.7-10.4) and 4 (1.8-8.5) years for MDS-RS-T, MDS/MPN-U, and CMML, respectively, the OS (95%CI) at 5 years was significantly better in patients with MDS-RS-T (61% [42%; 80%]) compared to MDS/MPN-U and CMML patients (21% [1%; 41%] and 19% [3%; 35%], p=0.002) (Figure 1). The CIP (95%CI) at 5 years between the three groups was significantly different: MDS/MPN-U and CMML (40% [18%; 61%] and 32% (14%-52%, respectively) vs. MDS/RS-T 8% [0.4%; 30%]) (p=0.005) (Figure 2). Conclusions: 1) In this series of patients with MDS/MPN (MDS-RS-T, MDS/MPN-U and CMML) according to the 2016 WHO classification clinical characteristics were similar except for hemoglobin and lactate dehydrogenase levels and leukocyte, monocyte and platelet counts. 2) Patients with MDS-RS-T had longer OS and less CIP than those with MDS/MPN-U and CMML; 3) The prognosis of MDS/MPN-U and CMML were similar. Supported by grants from: AGAUR 9015-470120/2015, 2017-SGR288 (GRC), CERCA Program from Generalitat de Catalunya, and "La Caixa" Foundation. Disclosures No relevant conflicts of interest to declare.

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.

Expert Second Opinion Pathology Review of Lymphoma in the Era of the World Health Organization Classification.

Blood ◽  
2007 ◽  
Vol 110 (11) ◽  
pp. 3317-3317
Author(s):  
Matthew J. Matasar ◽  
Weiji Shi ◽  
Jonathan Silberstien ◽  
Julie T. Feldstein ◽  
Daniel Filippa ◽  
...  
Keyword(s):  
World Health Organization ◽  
Who Classification ◽  
Second Opinion ◽  
World Health ◽  
Cancer Center ◽  
Major Revision ◽  
The World ◽  
Health Organization ◽  
Pathology Review

Abstract Background: The effective management of lymphoma depends upon an accurate and precise pathologic diagnosis. However, the classification of lymphoma continues to evolve. Reports addressing the role of second opinion expert pathology review have found varying impact, and little is known regarding the predictors of a change in diagnosis. Furthermore, the impact of the World Health Organization (WHO) classification of lymphomas over the 5 years following their formal publication has not been formally assessed. Methods: All outside pathology is reviewed at Memorial Sloan-Kettering Cancer Center (MSKCC) before a clinical opinion is finalized. We performed a chart review of all externally referred lymphoma cases from 1/1/01 to 6/30/01 and from 1/1/06 to 6/30/06 with second opinions from MSKCC hematopathology. Statistical analysis was performed using Chi-square or Fisher’s exact test for univariate analysis and logistic regression for multivariate analysis. Results: 719 patients (365 in 2001, 354 in 2006) met inclusion criteria. Diagnostic revisions were classified as major or minor; major changes were those that would lead to management changes as per National Comprehensive Cancer Network guidelines. 122 patients (18% in 2001, 16% in 2006) had a major diagnostic revision and an additional 22 (4% in 2001, 2% in 2006) had confirmation of major revisions rendered previously at second opinion from another National Cancer Institute Comprehensive Cancer Center (CCC). This did not change significantly by era, with 79 major revisions (22%) in 2001 and 65 (18%) in 2006 (P=NS). An additional 55 patients [24 (7%) in 2001, 31 (9%) in 2006] received minor revisions. Common categories of major revision included changing from nondiagnostic/ambiguous to definitive [6 in 2001, 8 in 2006], definitive to nondiagnostic [9 in 2001, 9 in 2006], malignant to benign [1 in 2001, 6 in 2006], indolent B-cell lymphoma (BCL) to aggressive BCL [15 in 2001, 8 in 2006], and aggressive BCL to indolent BCL [4 in 2001, 1 in 2006]. Major diagnostic revision was significantly associated with additional immunohistochemistry (IHC) testing in 2001 (OR=2.3; 95%CI 1.3, 4). In 2006, additional IHC (OR=1.8; 95%CI 1, 3.4), repeat biopsy (OR=3.1; 95%CI 1.2, 8.0), and skin biopsy (versus lymph node biopsy; OR 3.3; 95%CI 1.6, 7.0) were significantly associated with major revision. Two of the 7 patients reclassified as benign received revisions based on additional IHC, whereas 7 of the 14 patients reclassified as malignant were revised due to either additional IHC (4) or repeat biopsy (3). No effect was seen by biopsy type, nor were patient gender, age, race or ethnicity associated with odds of major revision. Of cases seen first at another CCC, 12% in 2001 and 16% in 2006 received major revisions, compared to 19% (2001) and 16% (2006) of other cases; these differences were not statistically significant. Conclusion: The rate of clinically meaningful diagnostic revisions at second opinion expert pathology review was high for patients seen at MSKCC, and remained so despite five years of increased familiarity with the WHO classification schema. These data confirm the fact that an appropriate evaluation, including detailed IHC and an adequate biopsy specimen, plays a central role in the accurate diagnosis of lymphoma. The high rates of diagnostic revision reported here lend support to the routine application of expert second opinion hematopathology review.

scholarly journals Molecular Classification of Soft Tissue and Bone Tumors

Diagnostics ◽  
2021 ◽  
Vol 11 (12) ◽  
pp. 2326
Author(s):  
David Creytens
Keyword(s):  
Soft Tissue ◽  
World Health Organization ◽  
Bone Tumors ◽  
Who Classification ◽  
Molecular Classification ◽  
World Health ◽  
The World ◽  
Classification Of Tumors ◽  
Health Organization

Soft tissue and bone tumors constitute a large and heterogeneous group of tumors comprising >100 distinct histological types and subtypes, which are diagnosed and classified using criteria from the World Health Organization (WHO) Classification of Tumors [...]

The World Health Organization's Revised Classification of Tumours of the Larynx, Hypopharynx, and Tracheax

1993 ◽  
Vol 102 (9) ◽  
pp. 666-669 ◽  
Author(s):  
Alfio Ferlito
Keyword(s):  
Respiratory Tract ◽  
Who Classification ◽  
Current Knowledge ◽  
World Health ◽  
Histological Classification ◽  
Upper Respiratory Tract ◽  
Histological Typing ◽  
The World ◽  
Upper Respiratory

A second edition of the Histological Typing of Upper Respiratory Tract Tumours in the WHO series International Histological Classification of Tumours was published in 1991. The new edition has been entitled Histological Typing of Tumours of the Upper Respiratory Tract and Ear. The task of revising the first edition, which was published in 1978, was undertaken at the WHO Center for Upper Respiratory Tract Tumours by K. Shanmugaratnam in collaboration with L. H. Sobin and pathologists in 8 countries. Several tumour types have been added to the classification, and some have been redefined in light of current knowledge. This presentation outlines the changes in the revised WHO classification as regards tumours of the larynx, hypopharynx, and trachea and discusses the grounds for said revisions.

Indolent Mastocytosis in 159 Adults: Smoldering but Not Bone Marrow Only Variant Is Prognostically Significant.

Blood ◽  
2009 ◽  
Vol 114 (22) ◽  
pp. 2906-2906
Author(s):  
Animesh D. Pardanani ◽  
Ken-Hong Lim ◽  
Terra L Lasho ◽  
Christy Finke ◽  
Rebecca McClure ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Acute Leukemia ◽  
Clinical Characteristics ◽  
Who Classification ◽  
Conflicts Of Interest ◽  
World Health ◽  
Serum Tryptase ◽  
Cytogenetic Abnormalities ◽  
Tryptase Level ◽  
Health Organization

Abstract Abstract 2906 Poster Board II-882 Background: The World Health Organization (WHO) classification system recognizes indolent SM (ISM) as an entity characterized by low systemic mast cell (MC) burden but frequent skin involvement. The WHO system also recognizes 2 provisional ISM sub-variants: smoldering SM (SSM) and bone marrow (BM) mastocytosis (BMM). The two are respectively characterized by increased systemic MC burden (presence of ≥ 2 ‘B-findings') and BM MC infiltration in the absence of skin or multiorgan visceral lesions. The prognostic relevance of this sub-classification remains unclear. Methods: The study population was drawn from a larger cohort of 342 adult SM patients (Blood. 2009 Jun 4;113(23):5727). Clinical data and BM histology were reviewed, and the diagnosis of ISM and its subclassification confirmed per the 2008 WHO proposal. The primary objectives of the study were to: (i) describe the clinical characteristics of a large cohort of ISM patients, within the context of the WHO classification; (ii) evaluate the prevalence of molecular and cytogenetic abnormalities; (iii) evaluate whether ISM subclassification is prognostically relevant on the basis of survival and risk of transformation to acute leukemia or aggressive SM (ASM). Results: (i) Clinical characteristics: 159 ISM patients were evaluated (69 males; median age 49 years, range 19-84); 22 (14%) had SSM, 36 (23%) BMM, and 101 (63%) ‘ISM-other' (ISMo). By definition, ‘B-findings' were absent in BMM patients. ‘B-findings' in SSM (and ISMo) patients was as follows: hepatosplenomegaly and/or lymphadenopathy 91% (21%), BM MC >30% or serum tryptase level >200ng/mL 68% (8%), and hypercellular BM or dysmyelopoiesis without cytopenias 50% (2%). SSM patients were older (p<0.01) and more frequently displayed constitutional symptoms (p<0.01), and anemia (p<0.01). MC mediator-release symptoms (p=0.01), including anaphylaxis (p<0.01), were more frequent in BMM. The following MC-mediators were studied: serum tryptase (57%), beta prostaglandin F2alpha (45%), urine methylhistamine (32%), and urine histamine (21%) – significant differences were noted amongst the ISM subgroups with the following relationship: SSM>ISMo>BMM (p<0.01). (ii) Molecular and cytogenetic abnormalities: Complete karyotype information was available for 55 patients; only 1 patient (with ISMo) had an abnormal karyotype. Fifty nine patients were screened for KITD816V and JAK2V617F; JAK2V617F was universally absent, and distribution of KITD816V was not significantly different amongst the ISM subgroups: SSM (100%), BMM (92%), and ISMo (69%). (iii) Survival and risk of disease transformation: At a median follow of 27 months (range <1-417), 26 deaths were recorded (ISMo 14, SSM 10, and BMM 2), and the combined median survival was 198 months: ISMo 301 months, SSM 120 months, and BMM (not reached) (p<0.01; Figure). Transformation to acute leukemia and ASM was seen in 1 patient (SSM) and 3 patients (2 SSM and 1 ISMo), respectively. Conclusions: Among WHO-defined ISM patients, SSM is associated with significantly shorter survival whereas BMM and ISMo are prognostically similar. If these observations are confirmed by others, consideration may be given to classifying SSM as a distinct subcategory of SM, instead of a sub-variant of ISM as it currently stands. Disclosures: No relevant conflicts of interest to declare.

The Applicability Of The WHO Classification In Pediatric AML. A NOPHO-AML Study

Blood ◽  
2013 ◽  
Vol 122 (21) ◽  
pp. 1354-1354
Author(s):  
Julie Damgaard Sandahl ◽  
Eigil Kjeldsen ◽  
Jonas Abrahamsson ◽  
Shau-Yin Ha ◽  
Jesper Heldrup ◽  
...  
Keyword(s):  
Myeloid Leukemia ◽  
Who Classification ◽  
Conflicts Of Interest ◽  
Large Population ◽  
Nordic Countries ◽  
World Health ◽  
Genetic Characteristics ◽  
Total Cohort ◽  
Pediatric Aml

Abstract Introduction The World Health Organization (WHO) classification of myeloid neoplasms and acute leukemia was revised in 2008 and is based on genetic characteristics and morphology. The classification incorporates newly recognized entities and emphasizes, more than previously, the pivotal role of cytogenetic abnormalities aiming at identifying biological and clinical entities as basis for a stratified treatment. The classification of acute myeloid leukemia (AML) is primarily based on adult studies. However, the frequency of cytogenetic changes varies among children and adults and the relevance of the 2008 revised WHO classification in pediatric AML has not yet been elucidated. The aim of this study was to evaluate the usability of the WHO classification when applied to a large population-based pediatric AML cohort. Methods We included children 0-18 years of age diagnosed with de novo AML in the Nordic countries (Sweden, Norway, Finland, Iceland and Denmark) and Hong Kong treated according to the NOPHO-AML-1993 and 2004 protocols in the period January 1993 to December 2012. Clinical, morphologic, and genetic data were retrieved from the database. Patients with myeloid leukemia of Down syndrome, acute promyelocytic leukemia, and therapy-related AML were excluded. The karyotypes from the Nordic countries were centrally reviewed with annual evaluation of the karyograms by the NOPHO cytogenetic working group. Morphological and molecular analyses and immunophenotyping were carried out at regional centers. The patients were classified according to the revised 2008 WHO classification using genetic parameters and the French-American-British (FAB) classification. Results In the NOPHO database we identified 609 children aged 0-18 years diagnosed with AML. In 13 cases, the karyotype analyses either failed or considered uninformative and were excluded. Among the remaining 596 informative cases (98%), 241 (40%) were categorized as AML with recurrent genetic abnormalities; t(8;21)(q22;q22);RUNX1-RUNX1T1 (n=69), inv(16)(p13.1q22) or t(16;16)(p13.1;q22);CBFB-MYH11 (n=47), t(9;11)(p21;q23);MLLT3-MLL (n=61), t(6;9)(p22;q34);DEK-NUP214 (n=5), inv(3)(q21q26.2) or t(3;3)(q21;q26.2);RPN1-EVI1 (n=1), t(1;22)(p13;q13)/RBM15-MKL1 (n=4), NPM1 mutated (n=13), and CEBPA mutated (n=10). Furthermore, 23 children were classified in the provisional entity AML with FLT3-ITD. Based on the karyotype, 92 children (16%) were classified as AML with myelodysplasia-related cytogenetical changes (AML-MDS). This group was dominated by unbalanced abnormalities (9%) with -7/del(7q) being the most common (4% of the total cohort) and complex karyotypes (6% of the total cohort). AML with balanced myelodysplasia-related changes was found in 0.5%. The largest group was the highly heterogeneous AML not otherwise specified (AML-NOS) (n=263) (44%), and included some of the well-defined 11q23 abnormalities. In the 2001 version of the WHO classification, “AML with abnormalities of 11q23; MLL,” was listed separately, but in the 2008 edition only AML with t(9;11)(p22;q23);MLLT3-MLL was listed as an entity. The AML-NOS cases were sub-classified according to morphology, with FAB M5 being most common, found in 22% of NOS, followed by FAB M1, FAB M2, and FAB M4, all found in 18%. In conclusion, 40% of the children were classified in clinically relevant entities, but the WHO classification allocated 16% of the children to AML-MDS, the relevance is unknown in children, and 44% to the NOS group. This large and unspecified group limits the applicability of the WHO classification in children with AML suggesting that additional considerations are warranted for relevant classification of pediatric AML. Disclosures: No relevant conflicts of interest to declare.

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.

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