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1.
Ann Hematol ; 98(10): 2319-2328, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-31396671

RESUMO

Triple-negative primary myelofibrosis (TN-PMF) and other myeloid neoplasms with associated bone marrow fibrosis such as the myelodysplastic syndromes (MDS-F) or the myelodysplastic/myeloproliferative neoplasms (MDS/MPN-F) are rare entities, often difficult to distinguish from each other. Thirty-four patients previously diagnosed with TN-PMF (n = 14), MDS-F (n = 18), or MDS/MPN-F (n = 2) were included in the present study. After central revision of the bone marrow histology, diagnoses according to the 2016-WHO classification were TN-PMF (n = 6), MDS-F (n = 19), and MDS/MPN-F (n = 9), with TN-PMF genotype representing only 4% of a cohort of 141 molecularly annotated PMF. Genomic classification according to next-generation sequencing and cytogenetic study was performed in 28 cases. Median number of mutations was 4 (range 1-7) in cases with TP53 disruption/aneuploidy or with chromatin-spliceosome mutations versus 1 mutation (range 0-2) in other molecular subgroups (p < 0.0001). The number of mutations and the molecular classification were better than PMF and MDS conventional scoring systems to predict survival and progression to acute leukemia. In conclusion, TN-PMF is an uncommon entity when the 2016 WHO criteria are strictly applied. Genomic classification may help in the prognostic assessment of patients with myeloid neoplasms with bone marrow fibrosis.


Assuntos
Neoplasias Hematológicas , Sequenciamento de Nucleotídeos em Larga Escala , Leucemia Mieloide Aguda , Mutação , Síndromes Mielodisplásicas , Mielofibrose Primária , Adulto , Idoso , Idoso de 80 Anos ou mais , Intervalo Livre de Doença , Feminino , Seguimentos , Neoplasias Hematológicas/classificação , Neoplasias Hematológicas/genética , Neoplasias Hematológicas/mortalidade , Humanos , Leucemia Mieloide Aguda/classificação , Leucemia Mieloide Aguda/genética , Leucemia Mieloide Aguda/mortalidade , Masculino , Pessoa de Meia-Idade , Síndromes Mielodisplásicas/classificação , Síndromes Mielodisplásicas/genética , Síndromes Mielodisplásicas/mortalidade , Mielofibrose Primária/classificação , Mielofibrose Primária/genética , Mielofibrose Primária/mortalidade , Taxa de Sobrevida
2.
PLoS Comput Biol ; 15(8): e1007332, 2019 08.
Artigo em Inglês | MEDLINE | ID: mdl-31469830

RESUMO

The confluence of deep sequencing and powerful machine learning is providing an unprecedented peek at the darkest of the dark genomic matter, the non-coding genomic regions lacking any functional annotation. While deep sequencing uncovers rare tumor variants, the heterogeneity of the disease confounds the best of machine learning (ML) algorithms. Here we set out to answer if the dark-matter of the genome encompass signals that can distinguish the fine subtypes of disease that are otherwise genomically indistinguishable. We introduce a novel stochastic regularization, ReVeaL, that empowers ML to discriminate subtle cancer subtypes even from the same 'cell of origin'. Analogous to heritability, implicitly defined on whole genome, we use predictability (F1 score) definable on portions of the genome. In an effort to distinguish cancer subtypes using dark-matter DNA, we applied ReVeaL to a new WGS dataset from 727 patient samples with seven forms of hematological cancers and assessed the predictivity over several genomic regions including genic, non-dark, non-coding, non-genic, and dark. ReVeaL enabled improved discrimination of cancer subtypes for all segments of the genome. The non-genic, non-coding and dark-matter had the highest F1 scores, with dark-matter having the highest level of predictability. Based on ReVeaL's predictability of different genomic regions, dark-matter contains enough signal to significantly discriminate fine subtypes of disease. Hence, the agglomeration of rare variants, even in the hitherto unannotated and ill-understood regions of the genome, may play a substantial role in the disease etiology and deserve much more attention.


Assuntos
Algoritmos , DNA de Neoplasias/genética , Neoplasias Hematológicas/classificação , Neoplasias Hematológicas/genética , Modelos Genéticos , Biologia Computacional , Bases de Dados de Ácidos Nucleicos , Frequência do Gene , Genoma Humano , Sequenciamento de Nucleotídeos em Larga Escala , Humanos , Aprendizado de Máquina , Polimorfismo de Nucleotídeo Único , RNA não Traduzido/genética , Processos Estocásticos , Sequenciamento Completo do Genoma
3.
Surg Pathol Clin ; 12(3): 651-669, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31352979

RESUMO

In this article we provide a practical and comprehensive review of myeloid neoplasms with overlapping myelodysplastic (MDS) and myeloproliferative (MPN) features, with emphasis on recent updates in classification, particularly the utility of morphologic, cytogenetic, and molecular findings in better defining and classifying these disease entities. We provide the reader with a summary of the most recent developments and updates that have helped further our understanding of the genomic landscape, clinicopathologic features, and prognostic elements of myeloid neoplasms with MDS/MPN features.


Assuntos
Neoplasias Hematológicas/patologia , Síndromes Mielodisplásicas/patologia , Transtornos Mieloproliferativos/patologia , Feminino , Genes Neoplásicos/genética , Neoplasias Hematológicas/classificação , Neoplasias Hematológicas/genética , Humanos , Cariótipo , Masculino , Mutação/genética , Síndromes Mielodisplásicas/classificação , Síndromes Mielodisplásicas/genética , Transtornos Mieloproliferativos/classificação , Transtornos Mieloproliferativos/genética , Prognóstico
5.
BMC Bioinformatics ; 20(Suppl 5): 181, 2019 Apr 25.
Artigo em Inglês | MEDLINE | ID: mdl-31272372

RESUMO

BACKGROUND: Within the cancer domain, ontologies play an important role in the integration and annotation of data in order to support numerous biomedical tools and applications. This work seeks to leverage existing standards in immunophenotyping cell types found in hematologic malignancies to provide an ontological representation of them to aid in data annotation and analysis for patient data. RESULTS: We have developed the Cancer Cell Ontology according to OBO Foundry principles as an extension of the Cell Ontology. We define classes in Cancer Cell Ontology by using a genus-differentia approach using logical axioms capturing the expression of cellular surface markers in order to represent types of hematologic malignancies. By adopting conventions used in the Cell Ontology, we have created human and computer-readable definitions for 300 classes of blood cancers, based on the EGIL classification system for leukemias, and relying upon additional classification approaches for multiple myelomas and other hematologic malignancies. CONCLUSION: We have demonstrated a proof of concept for leveraging the built-in logical axioms of the ontology in order to classify patient surface marker data into appropriate diagnostic categories. We plan to integrate our ontology into existing tools for flow cytometry data analysis to facilitate the automated diagnosis of hematologic malignancies.


Assuntos
Ontologias Biológicas , Neoplasias Hematológicas/patologia , Linhagem Celular Tumoral , Neoplasias Hematológicas/classificação , Neoplasias Hematológicas/metabolismo , Humanos , Imunofenotipagem , Aprendizado de Máquina , Células-Tronco Neoplásicas/citologia , Células-Tronco Neoplásicas/metabolismo
8.
Int J Cancer ; 145(2): 347-359, 2019 07 15.
Artigo em Inglês | MEDLINE | ID: mdl-30614529

RESUMO

Overweight/obesity, adult attained height and physical activity are possible risk factors for hematological malignancies. This meta-analysis aims to evaluate the associations between these factors and hematological cancer risk in adults. Eligible cohort studies were sought in PubMed up to May 31, 2016; overall, 44 studies were included in the present analyses. Pooled relative risk estimates were calculated using random-effects models; separate analyses were conducted for non-Hodgkin lymphoma (NHL) and subtypes (diffuse large B-cell lymphoma, DLBCL; follicular cell lymphoma; small lymphocytic lymphoma/chronic lymphocytic leukemia, SLL/CLL), Hodgkin lymphoma (HL), multiple myeloma (MM), leukemia and subtypes (acute lymphoblastic leukemia, acute myeloid leukemia, AML). Obesity was associated with increased risk of NHL, HL, MM, leukemia overall and AML in both sexes, as well as with higher DLBCL risk in women; the dose-response meta-regression analysis confirmed these associations. Less pronounced effects were observed regarding overweight, as it was associated with increased MM risk in both sexes, NHL risk in males, DLBCL and overall leukemia risk in females. Taller men presented with significantly higher risk of NHL and taller women were affected by higher risk of NHL, DLBCL, FL, CLL/SLL, MM, leukemia and AML. On the other hand, physical activity and abdominal fatness were not associated with the risk of hematological malignancies. In conclusion, this meta-analysis highlights the pivotal role of anthropometric measures in shaping the risk of hematological malignancies in adults. Additional, well-designed studies stemming from all the continents are needed for the further substantiation and generalization of the results.


Assuntos
Exercício , Neoplasias Hematológicas/epidemiologia , Obesidade/epidemiologia , Sobrepeso/epidemiologia , Adulto , Antropometria , Estudos de Coortes , Feminino , Neoplasias Hematológicas/classificação , Neoplasias Hematológicas/etiologia , Humanos , Masculino , Obesidade/complicações , Sobrepeso/complicações , Fatores Sexuais
9.
Am J Hematol ; 94(3): 363-377, 2019 03.
Artigo em Inglês | MEDLINE | ID: mdl-30536695

RESUMO

OVERVIEW: Systemic mastocytosis (SM) results from a clonal proliferation of abnormal mast cells (MCs) in extra-cutaneous organs. DIAGNOSIS: The major criterion is presence of multifocal clusters of abnormal MC in the bone marrow. Minor diagnostic criteria include elevated serum tryptase level, abnormal MC CD25 expression, and presence of KITD816V mutation. RISK STRATIFICATION: Establishing SM subtype as per the World Health Organization classification system is an important first step. Broadly, patients either have indolent/smoldering SM (ISM/SSM) or advanced SM, the latter includes aggressive SM (ASM), SM with associated hematological neoplasm (SM-AHN), and mast cell leukemia (MCL). Identification of poor-risk mutations (ie, ASXL1, RUNX1, SRSF2, NRAS) further refines the risk stratification. Recently, clinical and hybrid clinical-molecular risk models have been developed to more accurately assign prognosis in SM patients. MANAGEMENT: ISM patients have a normal life expectancy and treatment is generally limited to anaphylaxis prevention/symptom control/osteoporosis treatment. Patients with advanced SM frequently need MC cytoreductive therapy to ameliorate disease-related organ dysfunction. High response rates have been seen with small-molecule inhibitors that target mutant-KIT, including midostaurin (Food and Drug Administration approved) or avapritinib (investigational). Other options for MC cytoreduction include cladribine or interferon-α, although head-to-head comparisons are lacking. Treatment of SM-AHN primarily targets the AHN component, if an aggressive disease such as acute myeloid leukemia is present. Allogeneic stem cell transplant can be considered in such patients, or in those with relapsed/refractory advanced SM. Imatinib has a limited therapeutic role in SM; effective cytoreduction is limited to those with imatinib-sensitive KIT mutations.


Assuntos
Antineoplásicos/uso terapêutico , Biomarcadores Tumorais/genética , Gerenciamento Clínico , Neoplasias Hematológicas/terapia , Transplante de Células-Tronco Hematopoéticas , Mastocitose Sistêmica/terapia , Biomarcadores Tumorais/metabolismo , Medula Óssea/efeitos dos fármacos , Medula Óssea/metabolismo , Medula Óssea/patologia , Cladribina/uso terapêutico , Neoplasias Hematológicas/classificação , Neoplasias Hematológicas/diagnóstico , Neoplasias Hematológicas/mortalidade , Humanos , Interferon-alfa/uso terapêutico , Subunidade alfa de Receptor de Interleucina-2/genética , Subunidade alfa de Receptor de Interleucina-2/metabolismo , Mastócitos/efeitos dos fármacos , Mastócitos/metabolismo , Mastócitos/patologia , Mastocitose Sistêmica/classificação , Mastocitose Sistêmica/diagnóstico , Mastocitose Sistêmica/mortalidade , Inibidores de Proteínas Quinases/uso terapêutico , Proteínas Proto-Oncogênicas c-kit/antagonistas & inibidores , Proteínas Proto-Oncogênicas c-kit/genética , Proteínas Proto-Oncogênicas c-kit/metabolismo , Medição de Risco , Estaurosporina/análogos & derivados , Estaurosporina/uso terapêutico , Análise de Sobrevida , Transplante Homólogo , Triptases/sangue , Triptases/genética
10.
Arch Pathol Lab Med ; 143(1): 13-22, 2019 01.
Artigo em Inglês | MEDLINE | ID: mdl-29372845

RESUMO

CONTEXT.­: Myeloid neoplasms with familial occurrence have been rarely reported in the past. With the advance of molecular technology and better understanding of the molecular pathogenesis of myeloid neoplasms, investigating the genetic causes of familial acute myeloid leukemia or myelodysplastic syndrome has become feasible in the clinical setting. Recent studies have identified a rapidly expanding list of germline mutations associated with increased risks of developing myeloid neoplasm in the affected families. It is important to recognize these entities, as such a diagnosis may dictate a unique approach in clinical management and surveillance for the patients and carriers. OBJECTIVE.­: To raise the awareness of myeloid neoplasms arising in the setting of familial inheritance among practicing pathologists. DATA SOURCES.­: Based on recent literature and the 2016 revision of the World Health Organization classification of hematopoietic neoplasms, we provide an up-to-date review of myeloid neoplasm with germline predisposition. CONCLUSIONS.­: This short review focuses on the clinical, pathologic, and molecular characterization of myeloid neoplasm with germline predisposition. We emphasize the important features that will help practicing pathologists to recognize these newly described entities.


Assuntos
Neoplasias Hematológicas/genética , Leucemia Mieloide Aguda/genética , Síndromes Mielodisplásicas/genética , Predisposição Genética para Doença , Genótipo , Mutação em Linhagem Germinativa , Neoplasias Hematológicas/classificação , Neoplasias Hematológicas/patologia , Humanos , Leucemia Mieloide Aguda/classificação , Leucemia Mieloide Aguda/patologia , Síndromes Mielodisplásicas/classificação , Síndromes Mielodisplásicas/patologia , Patologistas , Organização Mundial da Saúde
11.
Hematology Am Soc Hematol Educ Program ; 2018(1): 286-300, 2018 11 30.
Artigo em Inglês | MEDLINE | ID: mdl-30504323

RESUMO

Over the past decade, there has been exponential growth in the number of genome sequencing studies performed across a spectrum of human diseases as sequencing technologies and analytic pipelines improve and costs decline. Pediatric hematologic malignancies have been no exception, with a multitude of next generation sequencing studies conducted on large cohorts of patients in recent years. These efforts have defined the mutational landscape of a number of leukemia subtypes and also identified germ-line genetic variants biologically and clinically relevant to pediatric leukemias. The findings have deepened our understanding of the biology of many childhood leukemias. Additionally, a number of recent discoveries may positively impact the care of pediatric leukemia patients through refinement of risk stratification, identification of targetable genetic lesions, and determination of risk for therapy-related toxicity. Although incredibly promising, many questions remain, including the biologic significance of identified genetic lesions and their clinical implications in the context of contemporary therapy. Importantly, the identification of germ-line mutations and variants with possible implications for members of the patient's family raises challenging ethical questions. Here, we review emerging genomic data germane to pediatric hematologic malignancies.


Assuntos
Genômica , Neoplasias Hematológicas/classificação , Neoplasias Hematológicas/genética , Neoplasias Hematológicas/metabolismo , Sequenciamento de Nucleotídeos em Larga Escala , Adolescente , Criança , Pré-Escolar , Feminino , Neoplasias Hematológicas/patologia , Humanos , Lactente , Recém-Nascido , Masculino
13.
Blood Cancer J ; 8(2): 15, 2018 02 09.
Artigo em Inglês | MEDLINE | ID: mdl-29426921

RESUMO

The new edition of the 2016 World Health Organization (WHO) classification system for tumors of the hematopoietic and lymphoid tissues was published in September 2017. Under the category of myeloproliferative neoplasms (MPNs), the revised document includes seven subcategories: chronic myeloid leukemia, chronic neutrophilic leukemia, polycythemia vera (PV), primary myelofibrosis (PMF), essential thrombocythemia (ET), chronic eosinophilic leukemia-not otherwise specified and MPN, unclassifiable (MPN-U); of note, mastocytosis is no longer classified under the MPN category. In the current review, we focus on the diagnostic criteria for JAK2/CALR/MPL mutation-related MPNs: PV, ET, and PMF. In this regard, the 2016 changes were aimed at facilitating the distinction between masked PV and JAK2-mutated ET and between prefibrotic/early and overtly fibrotic PMF. In the current communication, we (i) provide practically useful resource tables and graphs on the new diagnostic criteria including outcome, (ii) elaborate on the rationale for the 2016 changes, (iii) discuss the complementary role of mutation screening, (iv) address ongoing controversies and propose solutions, (v) attend to the challenges of applying WHO criteria in routine clinical practice, and (vi) outline future directions from the perspectives of the clinical pathologist.


Assuntos
Neoplasias Hematológicas/classificação , Transtornos Mieloproliferativos/classificação , Neoplasias Hematológicas/metabolismo , Neoplasias Hematológicas/patologia , Neoplasias Hematológicas/terapia , Transtornos Mieloproliferativos/metabolismo , Transtornos Mieloproliferativos/patologia , Transtornos Mieloproliferativos/terapia , Organização Mundial da Saúde
14.
J Clin Lab Anal ; 32(5): e22392, 2018 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-29356090

RESUMO

BACKGROUND: Automated cell counters have become more and more sophisticated with passing years. The numerical and graphic data both provide useful clues for suspecting a diagnosis especially when the workload is very high. AIM: We present our experience of useful information provided by graphic displays of an automated cell counter in hematological malignancies in a cancer hospital where a large number of complete blood count (CBC) requests are received either before or during chemotherapy. This study was conducted to assess the usefulness of hematology cell counter, viz. WBC-Diff (WBC differential), WBC/BASO (WBC basophil) and IMI (immature myeloid information) channel scatter plots, and the flaggings generated in various hematological malignancies. MATERIAL & METHODS: The graphic displays have been compiled over a period of 1 year (October 2015-September 2016) from blood samples of various solid and hematological malignancies (approximately 400 per day) received for routine CBC in the laboratory. Approximately 50 000 scattergrams have been analyzed during the study period. The findings were confirmed by peripheral blood smear examination. RESULTS: The scattergram analysis on XE-2100 is very sensitive as well as specific for diagnosing acute leukemia, viz. acute myeloid leukemia, acute lymphoblastic leukemia; chronic myeloproliferative disorders, viz. chronic myeloid leukemia; and chronic lymphoproliferative disorder especially chronic lymphocytic leukemia. CONCLUSION: It is suggested that the laboratories using the hematology analyzers be aware of graphic display patterns in addition to flaggings generated which provide additional information and give clue toward the diagnosis even before peripheral smear examination.


Assuntos
Automação Laboratorial/métodos , Neoplasias Hematológicas/diagnóstico , Testes Hematológicos , Transtornos Mieloproliferativos/diagnóstico , Contagem de Células , Neoplasias Hematológicas/classificação , Humanos , Leucemia/classificação , Leucemia/diagnóstico , Contagem de Leucócitos
15.
Int J Lab Hematol ; 40(2): 107-114, 2018 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-29345409

RESUMO

Monocytosis is a common finding that is caused by a wide variety of neoplastic and non-neoplastic conditions. The adequate evaluation of monocytosis involves the integration of laboratory data, morphology, clinical findings, and the judicious use of ancillary studies. We review the literature on monocytosis, including the 2017 revised 4th edition of the World Health Organization classification of hematopoietic neoplasms. We present a review of monocytosis with practical guidelines on how to approach both routine and challenging cases.


Assuntos
Neoplasias Hematológicas/classificação , Leucocitose , Monócitos/patologia , Humanos , Leucocitose/diagnóstico , Leucocitose/etiologia , Guias de Prática Clínica como Assunto
16.
J Clin Invest ; 128(1): 427-445, 2018 01 02.
Artigo em Inglês | MEDLINE | ID: mdl-29227286

RESUMO

As new generations of targeted therapies emerge and tumor genome sequencing discovers increasingly comprehensive mutation repertoires, the functional relationships of mutations to tumor phenotypes remain largely unknown. Here, we measured ex vivo sensitivity of 246 blood cancers to 63 drugs alongside genome, transcriptome, and DNA methylome analysis to understand determinants of drug response. We assembled a primary blood cancer cell encyclopedia data set that revealed disease-specific sensitivities for each cancer. Within chronic lymphocytic leukemia (CLL), responses to 62% of drugs were associated with 2 or more mutations, and linked the B cell receptor (BCR) pathway to trisomy 12, an important driver of CLL. Based on drug responses, the disease could be organized into phenotypic subgroups characterized by exploitable dependencies on BCR, mTOR, or MEK signaling and associated with mutations, gene expression, and DNA methylation. Fourteen percent of CLLs were driven by mTOR signaling in a non-BCR-dependent manner. Multivariate modeling revealed immunoglobulin heavy chain variable gene (IGHV) mutation status and trisomy 12 as the most important modulators of response to kinase inhibitors in CLL. Ex vivo drug responses were associated with outcome. This study overcomes the perception that most mutations do not influence drug response of cancer, and points to an updated approach to understanding tumor biology, with implications for biomarker discovery and cancer care.


Assuntos
Antineoplásicos/uso terapêutico , Bases de Dados Factuais , Neoplasias Hematológicas , Leucemia Linfocítica Crônica de Células B , Modelos Biológicos , Transdução de Sinais , Cromossomos Humanos Par 12/genética , Cromossomos Humanos Par 12/metabolismo , Feminino , Neoplasias Hematológicas/classificação , Neoplasias Hematológicas/tratamento farmacológico , Neoplasias Hematológicas/genética , Neoplasias Hematológicas/patologia , Humanos , Leucemia Linfocítica Crônica de Células B/classificação , Leucemia Linfocítica Crônica de Células B/tratamento farmacológico , Leucemia Linfocítica Crônica de Células B/patologia , Masculino , Proteínas de Neoplasias/genética , Proteínas de Neoplasias/metabolismo , Trissomia/genética
17.
Curr Opin Hematol ; 25(2): 120-128, 2018 03.
Artigo em Inglês | MEDLINE | ID: mdl-29256927

RESUMO

PURPOSE OF REVIEW: This review highlights the main changes in the revised 2016 WHO classification of myeloid neoplasms (published in 2017) that impact diagnosis and ultimately impact management of patients with these diseases. RECENT FINDINGS: The revision was based on data accumulated since the 2008 WHO classification, much of which relate to new molecular genetic information about these neoplasms. This massive recent influx of data concerning the significance of pathogenic mutations has affected all myeloid neoplasm categories. The new information has been incorporated as part of the diagnostic criteria of many diseases and has led to the creation of new provisional entities defined by genetic features. Germline mutations that predispose to myeloid neoplasms are also emerging as important findings that impact disease classification. SUMMARY: The growing body of genetic data have not only altered the classification of myeloid neoplasms, but are also impacting patient management. Genetically-defined disease categories have characteristic prognoses and predicted clinical behavior. Some mutations are associated with responsiveness to certain therapies, including those that target relevant oncogenes. The disease categories in the new classification facilitate the application of risk-adapted therapy based on the most recently available data.


Assuntos
Neoplasias Hematológicas , Mutação , Transtornos Mieloproliferativos , Organização Mundial da Saúde , Neoplasias Hematológicas/classificação , Neoplasias Hematológicas/diagnóstico , Neoplasias Hematológicas/genética , Neoplasias Hematológicas/terapia , Humanos , Transtornos Mieloproliferativos/classificação , Transtornos Mieloproliferativos/diagnóstico , Transtornos Mieloproliferativos/genética , Transtornos Mieloproliferativos/terapia
18.
J Pediatr Hematol Oncol ; 39(7): 528-537, 2017 10.
Artigo em Inglês | MEDLINE | ID: mdl-28906324

RESUMO

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare aggressive hematologic malignancy characterized by frequent skin involvement that most commonly affects older patients. BPDCN is known to have a poor prognosis. Our objective was to assess if outcome and disease prognosis were independently influenced by age when evaluated with clinical presentation, sex, and treatment regimens. We conducted a systematic review to identify BPDCN cases, to compare pediatric BPDCN cases with adult cases. A total of 125 publications were identified detailing 356 cases. Including 1 pediatric case from our institution, 74 were children, and 283 were adults aged 19 or over. Age was shown to be an independent prognostic factor predictive of more favorable outcomes across measures including initial response to therapy, likelihood of relapse, and overall survival at follow-up. The distribution of affected organs at diagnosis was similar across children and adults and type of clinical presentation did not disproportionately influence 1 age group's prognosis over the other. Acute lymphoblastic leukemia-type chemotherapy regimens were shown to be superior to other chemotherapy regimens (acute myeloid leukemia, lymphoma, acute lymphoblastic leukemia/lymphoma, other, or none) in inducing complete remission. Allogeneic stem cell transplantation was shown to increase mean survival time. Future research may be directed toward elucidating the further morphologic, cytogenetic, and cytochemical differences between younger and older BPDCN patients.


Assuntos
Células Dendríticas/patologia , Neoplasias Hematológicas/patologia , Neoplasias Hematológicas/terapia , Adulto , Criança , Neoplasias Hematológicas/classificação , Transplante de Células-Tronco Hematopoéticas , Humanos , Leucemia Mieloide Aguda/patologia , Leucemia-Linfoma Linfoblástico de Células Precursoras/patologia , Leucemia-Linfoma Linfoblástico de Células Precursoras/terapia , Prognóstico , Neoplasias Cutâneas/patologia , Adulto Jovem
19.
Sci Rep ; 7(1): 7435, 2017 08 07.
Artigo em Inglês | MEDLINE | ID: mdl-28785116

RESUMO

To assess radiological procedures and imaging characteristics in patients with intramammary hematological malignancies (IHM). Radiological imaging studies of histopathological proven IHM cases from ten German University affiliated breast imaging centers from 1997-2012 were retrospectively evaluated. Imaging modalities included ultrasound (US), mammography and magnetic resonance imaging (MRI). Two radiologists blinded to the histopathological diagnoses independently assessed all imaging studies. Imaging studies of 101 patients with 204 intramammary lesions were included. Most patients were women (95%) with a median age of 64 years. IHM were classified as Non Hodgkin lymphoma (77.2%), plasmacytoma (11.9%), leukemia (9.9%), and Hodgkin lymphoma (1%). The mean lesion size was 15.8 ± 10.1 mm. Most IHM presented in mammography as lesions with comparable density to the surrounding tissue, and a round or irregular shape with indistinct margins. On US, most lesions were of irregular shape with complex echo pattern and indistinct margins. MRI shows lesions with irregular or spiculated margins and miscellaneous enhancement patterns. Using US or MRI, IHM were more frequently classified as BI-RADS 4 or 5 than using mammography (96.2% and 89.3% versus 75.3%). IHM can present with miscellaneous radiological patterns. Sensitivity for detection of IHM lesions was higher in US and MRI than in mammography.


Assuntos
Neoplasias da Mama/diagnóstico por imagem , Neoplasias da Mama/patologia , Neoplasias Hematológicas/classificação , Neoplasias Hematológicas/diagnóstico por imagem , Adulto , Idoso , Idoso de 80 Anos ou mais , Feminino , Alemanha , Neoplasias Hematológicas/patologia , Humanos , Imagem por Ressonância Magnética , Masculino , Mamografia , Pessoa de Meia-Idade , Estudos Retrospectivos , Sensibilidade e Especificidade , Carga Tumoral , Ultrassonografia Mamária , Adulto Jovem
20.
Cleve Clin J Med ; 84(7): 528-534, 2017 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-28696193

RESUMO

Patients with hematologic cancers such as leukemia, lymphoma, and multiple myeloma are living longer than ever. The survival rate of patients with most hematologic cancers has doubled since 1974, and these once-terminal diagnoses are now chronic health conditions. This article reviews the care of patients with hematologic cancers, including those previously treated for childhood, adolescent, and young-adult cancers, discusses the role of primary care in a multidisciplinary team approach, and reviews innovative ways to deliver needed care.


Assuntos
Neoplasias Hematológicas , Administração dos Cuidados ao Paciente/métodos , Neoplasias Hematológicas/classificação , Neoplasias Hematológicas/terapia , Humanos , Equipe de Assistência ao Paciente , Atenção Primária à Saúde/métodos , Taxa de Sobrevida/tendências
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