Prognostic impact of blast cell counts in dysplastic bone marrow disorders (MDS and CMML I) with concomitant fibrosis

被引：0
作者：
Sigrid Machherndl-Spandl
W. Sega
H. Bösmüller
U. Germing
Ch. Gruber
K. Nachtkamp
P. Reinecke
W. R. Sperr
F. Wimazal
L. Müllauer
K. Sotlar
H. P. Horny
H. Tüchler
P. Valent
O. Krieger
机构：
[1] KH Elisabethinen,Department of Hematology, Stem Cell Transplantation, Haemostaseology and Internal Oncology
[2] KH der Barmherzigen Schwestern,Pathologic Department
[3] University of Düsseldorf,Hematologic Department
[4] University of Düsseldorf,Institute for Pathology
[5] Medical University of Vienna,Division of Hematology and Hemostaseology, Department of Internal Medicine 1
[6] Medical University of Vienna,Institute of Pathology
[7] University of Munich,Institute of Pathology
[8] Hanusch Hospital,Ludwig Boltzmann Institute for Leukemia Research and Haematology
[9] Medical University of Vienna,Ludwig Boltzmann Cluster Oncology
[10] Department of Internal Medicine I,undefined
来源：
Annals of Hematology | 2014年 / 93卷
关键词：
Myelodysplastic syndromes; Chronic myelomonocytic leukemia; Bone marrow fibrosis;
D O I：
暂无
中图分类号：
学科分类号：
摘要：
In a retrospective study, 43 patients with dysplastic neoplasms of the bone marrow (myelodysplastic syndromes and myelodysplastic/myeloproliferative-overlap neoplasms) associated with marked (grades 2–3) fibrosis were examined. Histopathologic and morphologic findings as well as cytogenetic and molecular results were correlated with clinical endpoints. Multilineage dysplasia (34 of 43 patients, 79 %) and hypercellular bone marrow (64 %) were found in most patients. In ten of 35 patients, poor risk karyotypes according to the International Prognostic Scoring System (IPSS) were recorded. The JAK2 V617F mutation was detected in four of 30 patients (13 %), and the KIT D816V mutation was found in two of 30 patients (6 %). Patients were mainly treated with palliative drugs and best supportive care. After an observation time of 1–41 (median 21) months, ten of 43 patients (23 %) had developed a secondary acute leukemia. The median survival of all 43 patients was 21.4 months (range 1.8–88.2 months). Of all prognostic parameters examined, the blast cell count at diagnosis was found to be a most reliable and most predictive marker concerning survival and leukemia progression. This confirms previous studies in dysplastic bone marrow neoplasms without fibrosis.
引用
收藏
页码：57 / 64
页数：7
相关论文
共 138 条
  • [1] Bennett JM(1982)Proposals for the classification of the myelodysplastic syndromes Br J Haematol 51 189-199
  • [2] Catovsky D(1989)Myelofibrosis in primary myelodysplastic syndromes: a clinicomorphological study of 10 cases Br J Haematol 71 499-504
  • [3] Daniel MT(1991)Clinical and cytogenetic characteristics of myelodysplastic syndromes developing myelofibrosis Cancer 68 178-183
  • [4] Pagliuca A(1991)Myelodysplastic syndromes with bone marrow fibrosis: a myelodysplastic disorder with proliferative features Ann Hematol 63 235-241
  • [5] Ohyashiki K(1992)Myelofibrosis in primary myelodysplastic syndromes: a retrospective study of 352 patients Eur J Haematol 48 208-214
  • [6] Sasao I(1993)Prognostic relevance of histological findings on bone marrow biopsy in myelodysplastic syndromes Ann Hematol 66 85-91
  • [7] Ohyashiki JH(2004)Myelofibrosis in primary myelodysplastic syndromes Clin Biol Signif Med Oncol 21 325-331
  • [8] Verhoef GE(2008)Marrow fibrosis predicts early fatal marrow failure in patients with myelodysplastic syndromes Leukemia 22 313-322
  • [9] De Wolf-Peeters C(2009)Clinical relevance of bone marrow fibrosis and CD34-positive cell clusters in primary myelodysplastic syndromes J Clin Oncol 27 754-762
  • [10] Ferrant A(2009)Myelodysplastic syndrome with fibrosis: experience of a single-institution with 139 patients Blood 114 2775-1616
12345678910