Bettini Roberto, Redaelli Sandro, Marzetta Katia, Maino Carlo, Sala Daniela, Saccà Vittoria, Gorini Maurizio
Patologia Medica, Università degli Studi dell'Insubria, Ospedale di Circolo, Varese.
Recenti Prog Med. 2004 Jul-Aug;95(7-8):358-64.
A series of 24 consecutive patients affected by myelofibrosis with myeloid metaplasia was reviewed. The clinical-pathological features at onset were similar to those reported in literature; in particular in all the patients we observed splenomegaly and the typical leuko-erythroblastic picture in peripheral blood. The median survival of our series was of 57 months; the deaths were caused by severe anaemia and/or infection and/or haemorrhage; the blastic terminal transformations were rare. According to other authors, unfavourable prognostic factors in our patients were: male sex, advanced age, hepatomegaly, the presence of systemic signs, anaemia, leukocytosis, leukopenia, high number of circulating erythroblasts, thrombocytopenia, osteomyelosclerosis. We have confirmed the clinical value of three staging prognostic systems: the system proposed by Njoku based on haemoglobin level and reticulocytes number, the system proposed by Visani based on haemoglobin and circulating myeloid precursors number, the system proposed by Dupriez based on haemoglobin and white blood cells number.
回顾了一系列连续的24例患有骨髓纤维化伴髓外化生的患者。发病时的临床病理特征与文献报道相似；特别是在所有患者中，我们都观察到脾肿大以及外周血中典型的幼稚粒-幼红细胞象。我们这组患者的中位生存期为57个月；死亡原因是严重贫血和/或感染和/或出血；原始细胞终末转化很少见。根据其他作者的观点，我们患者中不良的预后因素为：男性、高龄、肝肿大、全身症状的存在、贫血、白细胞增多、白细胞减少、循环幼红细胞数量多、血小板减少、骨髓硬化。我们证实了三种分期预后系统的临床价值：Njoku基于血红蛋白水平和网织红细胞数量提出的系统、Visani基于血红蛋白和循环髓系前体细胞数量提出的系统、Dupriez基于血红蛋白和白细胞数量提出的系统。
