O.5 Myeloproliferative disorders in the elderly: clinical presentation and role of bone marrow examination

2007 ◽  
Vol 64 ◽  
pp. S30-S31
Author(s):  
N.D. Goswami ◽  
H.J. Cohen ◽  
R.L. Yost ◽  
A.V. Rao
Blood ◽  
2004 ◽  
Vol 104 (11) ◽  
pp. 3702-3702
Author(s):  
Ken Ishiyama ◽  
Chiharu Sugimori ◽  
Hirohito Yamazaki ◽  
Akiyoshi Takami ◽  
Shinji Nakao

Abstract Some patients with aplastic anemia (AA) and approximately 40% of patients with refractory anemia (RA) of myelodysplastic syndrome exhibit karyotypic abnormalities in bone marrow dividing cells. Although some of the patients undergo evolution to acute myeloid leukemia (AML), others follow a clinical course similar to AA patients without chromosomal abnormalities. Except for several abnormalities such as −7 and 5q-, the clinical significance of such chromosomal abnormalities in bone marrow failure patients remains unclear. We recently developed a reliable HUMARA assay capable of detecting a clonal population in granulocytes which constitutes 30% or more of total granulocytes (Blood. 2003;102:1211–1216). Studying correlation between chromosomal abnormalities and the presence of clonality may help in understanding the pathogenetic role of chromosomal abnormalities in AA and RA. We thus analyzed 50 acquired AA and 28 RA female patients who were heterozygous for the HUMARA gene. Chromosomal abnormalities such as add(5)(q13), 9q–9q+ and del(7)(q14q22) were found in 8% of AA and 21% of RA patients. Clonality was detected in 38% of AA patients and 39% of RA patients. Incidence of chromosomal abnormalities in patients with clonality (27%) was higher than that in patients without clonality (4%, p<0.01). In two AA patients who respectively exhibited add(5)(q13) in 10% and +8 in 38% dividing cells, clonality was not detected and these abnormal clones became undetectable at the time of subsequent bone marrow examination. Clonality was detected in the other 2 AA patients respectively exhibiting 9q–9q+ in 40% and del(7)(q14q22) in 25% dividing cells, and in all 5 RA patients respectively exhibiting +8 in 10%, del(5)(q13q31), dup(1)(q32q12) in 90%, del(5)(q13), add(11)(q23), inv(9) in 65% and X,-X in 100% of dividing cells. None of the 50 AA patients including 2 patients with clonality and chromosomal abnormalities underwent evolution to AML during 2-year follow up while one of 28 RA patients who exhibited del(5)(q13q31) progressed to AML. The proportion of clonal granulocytes in total granulocytes estimated by the HUMARA assay remained unchanged in most patients with clonality except for the transformed one. These data indicate that the chromosomal abnormality in bone marrow dividing cells is not necessarily associated with presence of clonal granulocyte population in peripheral blood and that detection of clonality in granulcytes in bone marrow failure patients with chromosomal abnormalities of indefinite significance is useful in predicting prognosis of these patients.


Author(s):  
Donald H. Mahoney ◽  
L.C. Schreuders ◽  
Mary V. Gresik ◽  
Kenneth L. McClain

Blood ◽  
2008 ◽  
Vol 111 (10) ◽  
pp. 5109-5117 ◽  
Author(s):  
Shu Xing ◽  
Tina Ho Wanting ◽  
Wanming Zhao ◽  
Junfeng Ma ◽  
Shaofeng Wang ◽  
...  

Abstract The JAK2V617F mutation was found in most patients with myeloproliferative disorders (MPDs), including polycythemia vera, essential thrombocythemia, and primary myelofibrosis. We have generated transgenic mice expressing the mutated enzyme in the hematopoietic system driven by a vav gene promoter. The mice are viable and fertile. One line of the transgenic mice, which expressed a lower level of JAK2V617F, showed moderate elevations of blood cell counts, whereas another line with a higher level of JAK2V617F expression displayed marked increases in blood counts and developed phenotypes that closely resembled human essential thrombocythemia and polycythemia vera. The latter line of mice also developed primary myelofibrosis-like symptoms as they aged. The transgenic mice showed erythroid, megakaryocytic, and granulocytic hyperplasia in the bone marrow and spleen, displayed splenomegaly, and had reduced levels of plasma erythropoietin and thrombopoietin. They possessed an increased number of hematopoietic progenitor cells in peripheral blood, spleen, and bone marrow, and these cells formed autonomous colonies in the absence of growth factors and cytokines. The data show that JAK2V617F can cause MPDs in mice. Our study thus provides a mouse model to study the pathologic role of JAK2V617F and to develop treatment for MPDs.


2021 ◽  
pp. 1-6
Author(s):  
Eveline Vancraeynest ◽  
Marleen Renard ◽  
Thomas Tousseyn ◽  
Christophe M. Deroose ◽  
Anne Uyttebroeck ◽  
...  

1990 ◽  
Vol 116 (6) ◽  
pp. 919-920 ◽  
Author(s):  
Ammar Hayani ◽  
Donald H. Mahoney ◽  
Donald J. Fernbach

2002 ◽  
Vol 24 (4) ◽  
pp. 221-223 ◽  
Author(s):  
D. Gómez-Almaguer ◽  
G.J. Ruiz-Argüelles ◽  
B. López-MartÍnez ◽  
E. Estrada ◽  
E. Lobato-Mendiza´bal ◽  
...  

Blood ◽  
2015 ◽  
Vol 126 (23) ◽  
pp. 5010-5010 ◽  
Author(s):  
Sayako Yuda ◽  
Dai Maruyama ◽  
Hiroaki Kurihara ◽  
Akiko Miyagi Maeshima ◽  
Kosuke Toyoda ◽  
...  

Abstract Introduction The Lugano Classification incorporating recommendations of 18-fluoro-2-deoxy-D-glucose (FDG)-positron emission tomography/computed tomography (PET/CT) in the staging and response assessment of FDG-avid lymphomas was published. This classification is based on plenty of reports that suggested that evaluation with FDG-PET/CT improved the accuracy of the staging and response assessment of FDG-avid lymphomas, especially of diffuse large B-cell lymphoma and Hodgkin lymphoma. However, we are not sure of the role of FDG-PET/CT in indolent B-cell lymphomas, such as follicular lymphoma (FL). Patients and Methods Patients who were initially diagnosed as having FL of grade 1 to 3a at our institution between 2010 and 2012 were included in this study. We analyzed the number of nodal areas and the location of extranodal diseases identified by FDG-PET/CT added to the conventional evaluation consisting of CT, bone marrow examination and upper gastrointestinal endoscopy. The clinical stage by the conventional evaluation was compared to that by the Lugano Classification using FDG-PET/CT. It was also investigated whether adding PET/CT to the conventional evaluation might have had any influence on the decision regarding the initial treatment for patients with FL. Results A total of 67 patients with a median age of 62 years (range: 39-85) were included in this analysis. In comparison with CT, FDG-PET/CT identified a higher number of nodal areas in 11 patients (16%). Most of the extranodal sites except bone marrow and gastrointestinal tract were more frequently detected by PET-CT. Bone marrow examination detected 22 patients (33%) with bone marrow involvement, while PET-CT detected only 4 patients (6%). Gastrointestinal lesions were identified in 15 patients (22%) with conventional evaluation and in 4 patients (6%) by PET-CT (Table 1). In one of these 4 patients, endoscopic biopsy revealed that the PET-CT positive lesion was adenoma. In seven patients (10%), upstaging occurred through conventional evaluation plus PET-CT: 3 patients were upstaged from stage I to II, 2 from stage II to IV, 1 from stage II to III, and 1 from stage III to IV (Table 2). International Prognostic Index (IPI) and Follicular Lymphoma International Prognostic Index (FLIPI) were revised upward in 9 patients (13%) and 12 patients (18%), respectively. However, the change of stage, IPI, or FLIPI did not affect the decision regarding the initial treatment. Conclusion Our data suggest that FDG-PET/CT cannot take the place of the conventional evaluation, especially in patients with FL, because of the low sensitivity of involvements in bone marrow and gastrointestinal tract, although it may be helpful to use FDG-PET/CT in the staging of FL. Moreover, FDG-PET/CT might not have had any impact on the decision regarding the treatment strategy in FL. That may be partly because the lesions detected only by FDG-PET/CT did not affect the judgment of tumor burden. Prospective evaluation of the influence of FDG-PET/CT on the clinical outcomes is needed to establish an appropriate evaluation in the staging of patients with FL. Disclosures Maruyama: Takeda Pharmaceutical Company Limited: Honoraria; Eisai Co., Ltd.: Honoraria. Kobayashi:Nippon Shinyaku: Honoraria; Pfizer: Research Funding. Tobinai:Gilead Sciences: Research Funding.


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