Myeloid/lymphoid neoplasms with eosinophilia and FGFR1 rearrangement: 5 cases report and literatures review / 中华血液学杂志

Objective@#To investigate the clinic-pathological features, diagnosis and treatment of 8p11 myeloproliferative syndrome (EMS) .@*Methods@#Five patients diagnosed as EMS from Jan 2014 to May 2018 at Blood Disease Hospital, Chinese Academy of Medical Sciences were enrolled. The clinical manifestations, laboratory characteristics, treatment and outcome of these patients were summarized.@*Results@#The peripheral blood leukocyte count of 5 patients with EMS increased significantly, accompanied with an elevated absolute eosinophils value (the average as 18.89×109/L) . The hypercellularity of myeloid cells was common in bone marrow, always with the elevated proportion of eosinophils (the average as 17.24%) , but less than 5% of blast cells. The chromosome karyotype of the 5 cases differed from each other, but presenting with the same rearrangement of FGFR1 gene by fluorescence in situ hybridization technology. The average interval between onset and diagnosis was 4.8 months with a median survival of only 14 months.@*Conclusion@#EMS was a rare hematologic malignancy with poor prognosis and short survival. It was commonly to be misdiagnosed. Analysis of cytogenetics and molecular biology were helpful for early diagnosis.

Myeloid/lymphoid neoplasms with eosinophilia and FGFR1 rearrangement: 5 cases report and literatures review / 中华血液学杂志

Objective: To investigate the clinic-pathological features, diagnosis and treatment of 8p11 myeloproliferative syndrome (EMS) . Methods: Five patients diagnosed as EMS from Jan 2014 to May 2018 at Blood Disease Hospital, Chinese Academy of Medical Sciences were enrolled. The clinical manifestations, laboratory characteristics, treatment and outcome of these patients were summarized. Results: The peripheral blood leukocyte count of 5 patients with EMS increased significantly, accompanied with an elevated absolute eosinophils value (the average as 18.89×10(9)/L) . The hypercellularity of myeloid cells was common in bone marrow, always with the elevated proportion of eosinophils (the average as 17.24%) , but less than 5% of blast cells. The chromosome karyotype of the 5 cases differed from each other, but presenting with the same rearrangement of FGFR1 gene by fluorescence in situ hybridization technology. The average interval between onset and diagnosis was 4.8 months with a median survival of only 14 months. Conclusion: EMS was a rare hematologic malignancy with poor prognosis and short survival. It was commonly to be misdiagnosed. Analysis of cytogenetics and molecular biology were helpful for early diagnosis.

Rethink and Understanding of the Concept and Diagnostic Criteria of Lymphoma——Presentation of the Concept of "Low-Grade Malignant Potential Lymphoid Neoplasms" / 现代检验医学杂志

Some monoclonal lymphoid tissue lesions showing benign biological behavior have been found recently,they usually were diagnosed with lymphoma,their malignant potential is extremely limited and almost does not develop.However,it presents a challenge to the concept of lymphoma and its diagnostic standard.Based on this,the auther propose the concept of "benign lymphoma" at the theoretical level,and discusses the possibility of its existence from two aspects of clonality and lymphocyte fluidity.At the same time,based on clinical practice and diagnostic strategies,propose the " Low-grade malignant potential lymphoid neoplasms "diagnostic terms for the first time,on the one hand,it show that this type of lesion is different from lymphoma and avoids overt reatment,on the other hand,clinicians and patients were reminded to follow up observation to prevent recurrence of a small number of cases,so as to trigger the discussion on the biological essence of lymphoma.

Classificação de 86 casos de linfoma em bovinos de acordo com a Working Formulation (WF) of Non-Hodgkin's Lymphomas for Clinical Usage e a Revised European-American Classification of Lymphoid Neoplasms (REAL) / Classification of 86 bovine lymphomas cases according to the Working Formulation (WF) of Non-Hodgkin's Lymphomas for Clinical Usage and The Revised European-American Classification of Lymphoid Neoplasms (REAL)

Por meio de um estudo retrospectivo, realizou-se avaliação fenotípica (histologia) e imunofenotípica (imuno-histoquímica [IHQ]) de 86 casos de linfoma bovino. Quanto ao padrão de distribuição, todos os linfomas foram incluídos como difusos. Em relação à dimensão dos linfócitos neoplásicos, 83,8% dos linfomas foram considerados como de grandes células e 11,6% como de pequenas células. Linfomas mistos, ou seja, constituídos por grandes e pequenas células, representaram apenas 4,6% dos casos. Quanto ao número de mitoses, 84,9% dos linfomas foram incluídos como de grau intermediário, 10,5% como de baixo grau e 4,6% como de alto grau. No que se refere à morfologia do núcleo, linfomas em que predominavam linfócitos não clivados (58,2%) ou linfócitos clivados (37,2%) foram mais frequentes do que aqueles em que havia uma mistura igualmente proporcional de linfócitos clivados e não clivados (4,6%). Com base nestes resultados, os 86 linfomas foram assim distribuídos utilizando-se a classificação proposta pela Working Formulation (WF) of Non-Hodgkin's Lymphomas for Clinical Usage: difuso de grandes células não clivadas (46,5%), difuso de grandes células clivadas (33,7%), difuso de pequenas e grandes células (4,6%), difuso de pequenas células - tipo plasmocitoide (7%), imunoblástico (3,5%), difuso de pequenas células - tipo intermediário (2,3%), difuso de pequenas células não clivadas (1,2%) e difuso de pequenas células não clivadas - tipo Burkitt (1,2%). Na imuno-histoquímica, 27 dos 86 (31,4%) linfomas foram positivos para o anticorpo monoclonal CD79αcy, utilizado para detecção de linfócitos B, e nenhum caso foi positivo para o anticorpo policlonal CD3, utilizado para detecção de linfócitos T. Com base nestes resultados, os 27 linfomas B foram assim distribuídos utilizando-se a Revised European-American Classification of Lymphoid Neoplasms (REAL): linfoma difuso de grandes células B (81,5%), linfomas imunoblásticos de grandes células (11,1%) e linfomas...

A retrospective study of 86 cases of bovine lymphoma classified as with diffuse pattern of distribution and verified by phenotypic (histology) and immunophenotypic (immunohistochemistry [IHC]) is presented. Regarding the size of the neoplastic lymphocytes, 83.8% was classified as large cells lymphoma and 11.6% as small cells lymphoma. Mixed lymphomas, i.e., formed by large and small cells simultaneously represented only 4.6% of all cases. Regarding their mitotic index, 84.9% of lymphomas was included in the intermediate-grade, 10.5% as low-grade and 4.6% as high-grade. Regarding the nucleus morphology, lymphomas with mostly non-cleaved cells (58.2%) or cleaved cells (37.2%) were the more frequent than those with a balanced mixed proportion of cleaved and non-cleaved cells (4.6%). Based on these results, the 86 lymphoma cases were classified by the Working Formulation (WF) of Non-Hodgkin's Lymphomas for Clinical Usage as: diffuse large non-cleaved cell (46.5%), diffuse large cleaved cell (33.7%), diffuse mixed small and large cell (4.6%), diffuse small cell - plasmacytoid (7%), immunoblastic (3.5%), diffuse small cell - intermediate (2.3%), diffuse small non-cleaved cell (1.2%), and diffuse small non-cleaved cell Burkitt's (1.2%). According to the IHC, 27 out of 86 (31.4%) lymphomas were positive to monoclonal antibody CD79αcy, used to detect B cells, and none were positive for polyclonal antibody CD3, used to detect T cells. Based on this, the 27 B-cell type lymphomas were distributed as follows: diffuse large B-cell lymphoma (81.5%), large cell immunoblastic lymphoma (11.1%), and lymphoplasmacytoid lymphoma...

Review of the 2016 WHO classification of lymphoid neoplasms / 白血病·淋巴瘤

There are some major changes win the revised 2016 WHO Classification of Lymphoid Neoplasms.Based on the clinicopathological changes and genetic/molecular findings in the past years,the new classification clarified the diagnosis and clinical management of some very early stages of lymphoproliferative disorders,refined the diagnostic criteria for some lymphoid neoplasms,and further emphasized the significance of genetic/molecular studies in the diagnosis and clinical treatment of lymphomas.A small number of new provisional entities were added to the 2016 edition.