骨髓活检在预测MDS预后中的意义

报告２４例骨髓增生异常综合征骨髓活检结果与预后的关系，结果：骨髓活检与骨髓涂片所见大致相同，但前者对粒系、巨核细胞系病态造血诊断率更高，对ＡＬＩＰ及合并纤维化是唯一的诊断手段；活检时造血细胞中红系占优者预后好，粒系占优伴ＡＬＩＰ阳性者预后差，巨核细胞增生常伴骨髓纤维增生，预后亦差。     

骨髓活检在预测MDS预后中的意义

报告２４例骨髓增生异常综合征骨髓活检结果与预后的关系，结果：骨髓活检与骨髓涂片所见大致相同，但前者对粒系、巨核细胞系病态造血诊断率更高，对ＡＬＩＰ及合并纤维化是唯一的诊断手段；活检时造血细胞中红系占优者预后好，粒系占优伴ＡＬＩＰ阳性者预后差，巨核细胞增生常伴骨髓纤维增生，预后亦差。     

慢性粒细胞白血病合并淋巴瘤后急粒变1例

慢性粒细胞白血病合并淋巴瘤后急粒变１例蔡晓燕，张明水，吴树农，舒兆龙，申雪英慢性粒细胞白血病（ＣＭＬ）的终未期表现多为骨髓纤维化和急变，但合并淋巴瘤在近年也有散在报道，我院曾收治１例慢粒患者于病程中合并淋巴瘤，在联合化疗后又急粒变，较为少见，现报道如...     

24例小儿骨髓增生异常综合征临床分析

２４例小儿骨髓增生异常综合征临床分析陈日玲，陈铭珍我院儿科自１９８０年１月至１９９４年６月共收治骨髓增生异常综合征（ＭＤＳ）２４例，其中２例演变为急性白血病（ＡＬ）。现将２４例小儿ＭＤＳ的临床资料分析如下。临床资料１．一般资料男１４例，女１０例。男：...     

STI571联合非清髓性外周血干细胞移植治疗高危慢粒

慢性粒细胞性白血病(慢粒)是一种起源于多能干细胞的肿瘤增生性疾病，异基因干细胞移植是最有希望的疗法。因移植后复发率较高，加速期(AP)、急变期或第二次慢性期(CP2)的患被认为是高危患。2002年4月～2002年7月，2例高危慢粒患在我院接受了非清髓性异基因外周血干细     

53例何杰金氏病临床分析

１９８４年３月至１９８９年１０月本院收治何杰金氏病５３例，均为病理证实。其中单纯化疗１１例，以Ⅲ、Ⅳ期为主；单纯放疗６例，均为Ⅰ、Ⅱ期病例；化疗合并放疗３６例。Ⅰ期５年生存率为９０％，Ⅱ期为８０．９５％，Ⅲ期为５５．５６％，Ⅳ期为６０％。首次治疗完全缓解者５年生存率为８２．５％，部分缓解者２０％，说明首次治疗近期疗效与远期疗效密切相关。     

垂体腺瘤的TS分期

目的：在临床实践中对垂体腺瘤进行分期，并以指导临床治疗。方法：依据肿瘤的大小及患者内分泌情况进行分期，以Ｔ表示肿瘤的大小，Ｓ表示内分泌紊乱的程度，临床分期：Ⅰ期Ｔ１Ｓ１；Ⅱ期Ｔ２Ｓ１；Ⅲ期Ｔ３Ｓ１；Ⅳ期Ｔ４Ｓ１、Ｔ１～４Ｓ２。结果：分析了自１９７５～１９９０年收治的６３１例垂体瘤患者，控制率为：Ⅰ期９４．７％，Ⅱ期９２．９％，Ⅲ期８４．６％，Ⅳ期６３．３％。经统计学处理：Ⅰ期、Ⅱ期与Ⅲ期间，Ⅰ期、     

从甲状腺癌临床与病理的联系探讨治疗问题

本文对１９７３～１９９３年收治的１４４例甲状腺癌的临床表现与病理组织学特征进行回顾性分析，探讨其治疗问题。本组病例按病理分型：乳头状癌８４例，滤泡癌３２例，透明细胞癌６例，髓样癌６例，未分化癌１６例；按肿瘤局部病变、淋巴结侵犯和远处转移临床分期：Ⅰ期６７例，Ⅱ期７３例，Ⅲ期４例，全组病例随访结果，已死亡１０例，其中８例系因甲状腺癌致死；其余均健在，因而，提出以临床征象、分期结合病理组织学特征，决定治疗方针是较为正确的。     

神经胶质细胞瘤并发MDS─—急粒变1例

神经胶质细胞瘤并发ＭＤＳ─—急粒变１例杨云芳，竺永昌，黄一苹，董幼亭神经胶质细胞瘤化疗后并发骨髓增生异常综合征（ＭＤＳ），再演变为急性粒细胞白血病（Ｍ＿１）的复合癌，国内未见报道，本院收治１例报告如下：患者女性，２４岁。因阵发性头疼伴恶心呕吐１０天于...     

复方青黛片联合化疗治疗慢粒急髓变7例分析

慢性粒细胞白血病(简称慢粒)急性变为慢粒终末期，属难治性白血病的一种类型，临床上慢粒慢性期约经过1年～3年可发展为急性期。慢粒急变目前多采用与急性白血病相同的联合化疗，但缓解率较低。1999年5月—2001年4月，我们采用复方青黛联合化疗对7例慢粒急髓变患者进行治疗与观察，现总结报告如下。……     

Myelofibrosis in primary myelodysplastic syndromes

In a retrospective study of 236 patients with primary myelodysplastic syndromes (MDS), 130 cases (55.1%) revealed myelofibrosis in bone marrow biopsies. It was observed that fibrosis mostly occurs focally or patchy, and collagen deposits were found very rarely (only four patients). The histopathology of bone marrow biopsies revealed several differences between fibrotic and non-fibrotic MDS: cellularity is significantly higher, dysmegakaryopoiesis is more pronounced, plasmocytes and mast cells are more often increased, and disturbance of marrow topography (particularly of the MK- and G-line) can be found more frequently in MDS with myelofibrosis. Reticulin fibrosis occurred in all subtypes of MDS; however, there was a higher incidence in chronic myelomonocytic leukemia. The frequency of abnormal growth of GM-progenitors was significantly higher in the MDS cases with myelofibrosis, compared to the cases without fibrosis. Clinical data showed significantly higher WBC, more frequent presence of immature granulocytes, and higher percentage of myeloblasts in peripheral blood and bone marrow in MDS with myelofibrosis compared to cases without myelofibrosis. Life expectancy was reduced to 13 mo, compared with 35 mo in MDS without fibrosis (p=0.00055). Time to leukemic transformation was 32 mo in MDS with fibrosis, compared with >56 mo in MDS without fibrosis (p=0.015). Myelofibrosis therefore seems to herald a poor prognosis.     

Discrepancy in diagnosis of primary myelofibrosis between referral and tertiary care centers

Primary myelofibrosis (PMF) is myeloproliferative neoplasm whose diagnosis is based on a combination of clinical and pathology criteria. We evaluated 560 consecutive patients who were diagnosed with PMF upon a referral to our center and evaluated the frequency of and reasons for diagnostic discordance. Discordance in the diagnosis was found in 70 (12.5%) patients. Discordant cases had a significantly lower grade of bone marrow fibrosis (grade 0–1), more likely to be JAK2V617F-mutation negative, and have no peripheral blood blasts, possibly explaining the difficulty in making a proper diagnosis and underscoring the need for a complete evaluation at a tertiary center.     

Platelet Derived Growth Factor Expression, Myelofibrosis and Chronic Myelogenous Leukemia

CML is often associated with myelofibrosis, and fibrosis in the accelerated phase is one of the diagnostic criteria for this accelerated phase. In this review, the mechanism of myelofibrosis associated with CML is discussed with emphasis on the cell origin of the production and release of platelet derived growth factor (PDCF) and its interaction with marrow fibroblasts. In the initial stage of myelofibrosis in chronic phase CML, atypical small megakaryocytes might leak PDGF, possibly PDGF-AB, together with other growth factors. As the clinical phase of the disease progreses to accelerated or blastic phase, a larger quantity of PDGF-AB or PDGF-BB might be secreted from blastic cells with myeloid phenotype. In addition some fibroblasts may be attracted by the PDGF and proliferate, and deposit collagen as well as fibronectin in the bone marrow stroma.     

Pulmonary Fibrosis with Megakaryocytoid Cell Infiltration and Chronic Myelogenous Leukemia

The relationship between fibrosis and megakaryocytic infiltration in the lungs of patients with Philadelphia-chromosome (Ph¹)-positive chronic myelogenous leukemia (CML) is the focus of this review. Ph¹-positive megakaryocytes are thought to reach and accumulate in the pulmonary vasculature through the marrow-blood barrier. In 21 autopsied patients with accelerated phase or blastic crisis of CML, megakaryocytic infiltration to the lungs was demonstrated in 10 patients, myelofibrosis in 12 patients and both in 5 patients. In 2 of the 10 patients with increased megakaryocytes in the lungs, relatively new fibrosis and alveolar damage in the acute phase were demonstrated with the simultaneous occurrence of myelofibrosis-“myelopulmonary fibrosis.” Leakage of mitogenic factors resulting from an abnormality in the packaging mechanism of alpha-granules in these cells termed acquired grey platelet syndrome could cause Fibrosis in the lungs as well as the bone marrow.     

The relevance of reticulin stain-measured fibrosis at diagnosis in chronic myelogenous leukemia

Although collagen myelofibrosis indicates poor prognosis in late stages of chronic myelogenous leukemia, the significance of reticulin stain-measured fibrosis in newly diagnosed patients is unknown. One hundred and thirty-eight patients with untreated or minimally treated chronic phase Philadelphia chromosome-positive chronic myelogenous leukemia had reticulin stain studies made on their bone marrows at diagnosis. Reticulin fibrosis was graded on a scale of 1 to 4. Significant (Grade 3 or 4) fibrosis was noted in 65 patients (47%). Compared with patients with mild (Grade 1 to 2) reticulin fibrosis, those with significant fibrosis had a higher incidence of splenomegaly greater than or equal to 10 cm (29% versus 49%; P = 0.02), hemoglobin less than 10 g/dl (19% versus 49%; P less than 0.01), weight loss greater than or equal to 6.75 kg (10% versus 30%; P = 0.11), marrow blasts greater than or equal to 5% (7% versus 28%; P less than 0.01), peripheral blasts greater than or equal to 3% (30% versus 46%; P = 0.09), and additional karyotypic abnormalities (1% versus 17%; P less than 0.01). The incidence of thrombocytosis was similar in the two groups. Prognostically, median survival was significantly shorter for the 26 patients with Grade 4, compared with the 39 patients with Grade 3, and the 73 patients with Grade 1 or 2 reticulin fibrosis (32 versus 49 versus 57 months; P = 0.03). Reticulin fibrosis is a useful biologic and prognostic index in newly diagnosed patients with chronic phase chronic myelogenous leukemia.     

STI571治疗慢性髓系白血病的临床疗效与毒副作用观察

背景与目的:bcr-abl融合基因翻译的蛋白产物P210bcr-abl的酪氨酸激酶(proteintyrosinekinase,PTK)活性异常增高被认为是导致慢性髓系白血病(chronicmyeloidleukmeia,CML)发病的根本原因。STI571能高效特异性抑制P210bcr-abl的PTK活性,在临床应用中获得了显著的疗效,但对急变期患者的治疗效果维持时间短。本研究观察和比较了STI571治疗慢性期与加速/急变期CML患者的临床疗效和所发生的不良反应,并从细胞遗传学的角度对急变期患者STI571耐药机制进行初步的分析。方法:选择接受STI571治疗的CML患者22例,其中慢性期6例,加速/急变期16例。按照血液学缓解和细胞遗传学缓解的标准,结合骨髓细胞形态学分析、骨髓细胞G显带技术分析和间期荧光原位杂交检测结果,对患者STI571治疗前和治疗3个月后的血液学和细胞遗传学缓解情况进行分析,并对3个月内出现耐药复发的患者进行核型演化分析。同时密切观察各系统发生的不良反应及严重程度。结果:6例(100%)慢性期CML患者获血液学完全缓解和细胞遗传学缓解,4例(25%)加速/急变期CML患者获血液学完全缓解,8例(50%)获不同程度的细胞遗传学反应。获血液学完全缓解和细胞遗传学反应的百分率两组比较均有统计学差异(P<0.05)。3例急变期CML患者出现耐药复发,其中2例可见2Ph和其它新     

Idiopathic acquired sideroblastic anemia terminating in acute myelofibrosis: case report and review of leterature.

Acute myelofibrosis is a rare but distinct accelerated variant of agnogenic myeloid metaplasia that is characterized by marked anemia, peripheral blood myeloblastosis and normoblastosis, a lack of teardrop poikilocytosis, and prominent myelofibrosis. There is usually no palpable hepatosplenomegaly or lymph node enlargement. The clinical course is remarkable short. We describe a 63-year-old man who presented with idiopathic acquired sideroblastic anemia and subsequently developed acute myelofibrosis. Intensive polychemotherapy with vincristine, cytosine arabinoside, and prednisone and a later trial of oxymetholone therapy were ineffective. He died 134 days after the diagnosis of acute myelofibrosis was established. The 11 previously reported cases of acute myelofibrosis are reviewed, and the relationships of acute myelofibrosis to other myeloproliferative disorders and to idiopathic acquired sideroblastic anemia are discussed.     

恶性血液病继发骨髓纤维化71例病理及临床研究

 【摘要】 目的 探讨继发性骨髓纤维化(secondary myelofibrosis,SMF)患者的临床及骨髓病理学特征。方法 对71例继发性骨髓纤维化患者的临床表现、外周血涂片、骨髓涂片及骨髓活检情况进行了回顾性研究,同时对不同疾病骨髓纤维化程度与巨核细胞数目作了相关分析。结果 分析71例SMF患者,其原发病情况为慢性粒细胞白血病(CML)20例(28.2%),急性淋巴细胞白血病（ALL）6例（8.5%）,急性髓系细胞白血病(AML)、骨髓增生异常综合症（MDS）、淋巴瘤各10例（14.1%）,多发性骨髓瘤（MM）、淋巴增殖性疾病（LPD）各4例（5.6%）,骨髓增殖性肿瘤（MPN）3例,慢性淋巴细胞白血病（CLL）2例,骨髓转移癌、传染性单核细胞增多症各1例。骨髓纤维化程度与巨核细胞数具有明显相关性（P＜0.05）。结论 继发性骨髓纤维化患者骨髓切片巨核细胞增生明显,且与纤维化程度有相关性。这可能与巨核细胞克隆性增殖释放的多种细胞因子积极参与骨纤的发生发展密切相关。     

Cytogenetic study in primary myelofibrosis at diagnosis: Clinical and histological association and impact on survival according to WHO 2017 classification in an Italian multicenter series

We analyzed cytogenetic data at diagnosis in 395 primary myelofibrosis (PMF) patients to evaluate any possible association between karyotype and WHO 2017 classification and its impact on prognosis. All the cases were diagnosed and followed at five Italian Hematological Centers between November 1983 and December 2016. An abnormal karyotype (AK) was found in 69 patients and clustered differently according to bone marrow fibrosis grade as it was found in 31 (27.0%) cases with overt fibrotic and 38 (13.6%) with pre‐fibrotic PMF (p = 0.001). Sex, anemia, thrombocytopenia, circulating blasts ≥1%, higher lactate dehydrogenase, and International Prognostic Scoring System risk classes were all significantly associated with karyotype. At a median follow‐up of >6 years, 101 deaths were recorded. Survival was different between AK and normal karyotype (NK) patients with an estimated median overall survival (OS) of 11.6 and 25.7 years, respectively (p = 0.0148). In conclusion, in our cohort around 20% of patients had an AK, more frequently in subjects with an advanced bone marrow fibrosis grade and clinical‐laboratory features indicative of a more aggressive disease. This study shows that an AK confers a more severe clinical phenotype and impacts adversely on OS, thus representing an additional parameter to be considered in the evaluation of PMF prognosis.     

Clonal Analysis of Agnogenic Myeloid Metaplasia

Agnogenic myeloid metaplasia (AMM) is a chronic myeloproliferative disorder that leads to a sustained proliferation of megakaryocytes and an increase of reticulin fibers within the bone marrow. Blood and bone marrow samples from patients with advanced AMM with fully developed myelofibrosis as well as cases in the cellular phase of the disease were investigated for clonality. Clonality was studied by X-linked restriction length polymorphism in conjunction with DNA methylation patterns. Granulocytes and total bone marrow cells proved to be monoclonal in origin whereas at least a minor portion of the peripheral lymphocytes were not clonally derived. Our findings indicate that the cellular phase of AMM as well as the fully developed disease progressed to myelofibrosis represent a monoclonal proliferation of pluripotent hematopoietic stem cells.