Evidence that prefibrotic myelofibrosis is aligned along a clinical and biological continuum featuring primary myelofibrosis.
Evidence that prefibrotic myelofibrosis is aligned along a clinical and biological continuum featuring primary myelofibrosis.
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预纤维化的骨髓纤维化沿临床和生物连续体排列的证据，具有原发性骨髓纤维化。
DOI：
10.1371/journal.pone.0035631
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发表时间：
2012
期刊：
影响因子：
3.7
通讯作者：
Magrini U
中科院分区：
文献类型：
--
作者：
Barosi G;Rosti V;Bonetti E;Campanelli R;Carolei A;Catarsi P;Isgrò AM;Lupo L;Massa M;Poletto V;Viarengo G;Villani L;Magrini U
在世卫组织的诊断分类中，预纤维化骨髓纤维化（pre - MF）被归入原发性骨髓纤维化（PMF）类别。然而，这一归类缺乏有力证据。
我们对683例被世卫组织诊断为PMF的连续患者进行了研究，以确定pre - MF是否可能处于临床和生物学的连续统一体中。
与PMF纤维化型相比，pre - MF（132例）呈现出女性居多、年龄更小、血红蛋白更高、血小板计数更高、白细胞计数更低、脾脏指数更小以及内脏静脉血栓形成发生率更高的特点。从pre - MF到早期和进展期骨髓（BM）纤维化的PMF纤维化型，男女比例和血红蛋白稳步下降，而年龄上升。可能的情况是，循环CD34 +细胞、乳酸脱氢酶（LDH）水平和染色体异常频率增加，而CD34 +细胞上的CXCR4表达以及血清胆固醇沿着骨髓纤维化的连续统一体下降。整个PMF病例队列的中位生存期为21年。pre - MF患者、早期骨髓纤维化的PMF纤维化型患者以及进展期骨髓纤维化的PMF纤维化型患者在诊断后10年的生存率分别为98%、81%和56%。
pre - MF是PMF的一种表现形式，具有非常惰性的表型。这一观点的主要影响在于对PMF有了新的临床认识，以及需要改进对该疾病的预后预测。
In the WHO diagnostic classification, prefibrotic myelofibrosis (pre-MF) is included in the category of primary myelofibrosis (PMF). However, strong evidence for this position is lacking. We investigated whether pre-MF may be aligned along a clinical and biological continuum in 683 consecutive patients who received a WHO diagnosis of PMF. As compared with PMF-fibrotic type, pre-MF (132 cases) showed female dominance, younger age, higher hemoglobin, higher platelet count, lower white blood cell count, smaller spleen index and higher incidence of splanchnic vein thrombosis. Female to male ratio and hemoglobin steadily decreased, while age increased from pre-MF to PMF- fibrotic type with early and to advanced bone marrow (BM) fibrosis. Likely, circulating CD34+ cells, LDH levels, and frequency of chromosomal abnormalities increased, while CXCR4 expression on CD34+ cells and serum cholesterol decreased along the continuum of BM fibrosis. Median survival of the entire cohort of PMF cases was 21 years. Ninety-eight, eighty-one and fifty-six percent of patients with pre-MF, PMF-fibrotic type with early and with advanced BM fibrosis, respectively, were alive at 10 years from diagnosis. Pre-MF is a presentation mode of PMF with a very indolent phenotype. The major consequences of this contention is a new clinical vision of PMF, and the need to improve prognosis prediction of the disease.
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影响因子：
2.6
作者：
Hussein, Kais;Bock, Oliver;Kreipe, Hans
通讯作者：
Kreipe, Hans
影响因子：
3.5
作者：
Koopmans, Suzanne M.;Bot, Freek J.;Hebeda, Konnie M.
通讯作者：
Hebeda, Konnie M.
影响因子：
20.3
作者：
Barosi, G;Viarengo, G;Frassoni, F
通讯作者：
Frassoni, F
影响因子：
20.3
作者：
Cervantes, Francisco;Dupriez, Brigitte;Tefferi, Ayalew
通讯作者：
Tefferi, Ayalew
影响因子：
2.4
作者：
Allegra, Alessandro;Alonci, Andrea;Musolino, Caterina
通讯作者：
Musolino, Caterina