骨髓纤维化转化急性巨核细胞白血病1例

患者,女,45岁.因反复头昏、乏力2年,低热10余天,于2003年5月16日入院.患者于2001年6月无明显诱因出现头昏、乏力,在当地医院就诊时发现脾大,当时血常规示:WBC 13.4×109 /L,Hb 78g/L,PLT338×109 /L,脾左肋下5 cm,骨髓穿刺术呈干抽,骨髓涂片显示粒、红系大致正常,骨髓活检发现骨髓组织增生极度活跃,胶原纤维及网状纤维增生,纤维组织穿插、分割造血组织而呈斑片状分布,巨核细胞散在或成簇分布,扩张的窦内充有幼稚粒、红系细胞及巨核细胞.确诊为骨髓纤维化,不规则口服羟基脲治疗.5月4日因受凉后出现低热,T 37～38 ℃,在当地经抗生素治疗低热不退.查体:贫血貌,浅表淋巴结未触及,胸骨叩痛(-),心、肺(-),肝右肋下4cm脾明显增大,达盆腔,质硬.辅助检查:血常规WBC 124×109 /L,Hb43 g/L,PLT 138×109 /L.     

不典型单核细胞型类白血病反应1例

1 病例介绍 患者男,15岁,因"咽痛,反复发热伴牙龈出血1个月余"于2009-07-06入院.患者因1个月前误吞鱼刺后出现咽痛高热症状,遂于当地医院诊治,期间查血象WBC 0.8×109/L,Hb 91 g/L,PLT32×109/L,血培养出嗜麦芽单胞菌,咽拭子培养出绿脓杆菌.2009-06-18在当地医院做骨髓细胞学检查为骨髓增生活跃,粒、红、巨三系造血减低,单核增生异常,占71.5%,其中原始15.5%,幼稚45.5%,成熟10.5%,粒红两系分类中均未见(图1).     

真性红细胞增多症并肾功能衰竭1例

患者男,73岁。因确诊真性红细胞增多症7年,乏力、关节疼痛1个月,于2004-03-12入院。患者7年前因泡沫尿伴双下肢水肿1年余入我院。当时Hb224g/L,WBC18·6×109/L,BPC504×109/L,红细胞压积(HCT)0·697。外周血中性粒细胞碱性磷酸酶(NAP)积分39分。SaO297%,尿蛋白( ),24h尿蛋白定量3·2g。血肌酐130μmol/L,尿素氮10·0mmol/L,尿酸490μmol/L。肾穿刺病理:局灶结段性肾炎;免疫荧光:IgM( ),IgG(-),IgA(-),C(-)。骨髓涂片:增生明显活跃,粒细胞系统除原粒细胞外均见,占0·50;红细胞系统增生明显活跃,占0·45,以中、晚幼红细胞增生…     

Ⅰ型戈谢病1例报告

1 病例资料 患者女性,26岁,因“反复乏力8个月”入院,2014年12月因妊娠查体发现WBC 3×109/L、PLT 50×109/L、Hb 90 g/L,转铁蛋白4.066 g/L,肝功能正常,腹部超声提示肝脾增大,自觉轻度乏力.2015年3月于当地医院查肝功能:Alb 32.6 g/L,ALP167 U/L,TG 5.36 mmol/L.4月底骨髓穿刺涂片提示:骨髓增生良好,粒系成熟受阻,巨核细胞增生尚可,产板不良.     

急性白血病化疗后合并肝肾侵袭性真菌感染一例

患者女,17岁.因"双下肢、背部疼痛2月余,发热1 d"于2009年4月28日入院.既往体健.体格检查:神清,皮肤少许淤点、淤斑,双肺呼吸音粗,心腹查体无异常,双下肢、背部压痛.血常规:白血病计数(WBC)11.7×109/L,Hb 80g/L,血小板(PLT)39 × 109/L.分类:幼稚细胞32%.骨髓涂片:增生明显活跃,可见大量的原始及幼稚淋巴细胞.     

双份脐带血干细胞移植后多次血型转变一例

<正>患者，男，17岁，因“发现皮肤瘀点、瘀斑5个半月”于2019年8月30日入院。患者自诉2019年3月15日发现皮肤瘀点、瘀斑，外院血常规：WBC计数正常，血红蛋白(Hb)83 g/L,PLT计数31×109/L;行骨髓涂片、骨髓活检等(具体结果不详)检查后诊断为骨髓增生异常综合征(MDS)伴多系血细胞发育异常，口服达那唑(每次0.2 g、每日3次)治疗5个月余效果不佳，间断输注PLT,为求进一步治疗转入我院。既往体健，家族史无特殊。     

慢性粒细胞白血病急变行异基因外周血干细胞移植后发生粒细胞肉瘤1例

患者,男,36岁。于2001年12月因“消瘦、腹胀”9个月入院。体检:脾大,左肋下6 cm;WBC 66×109／L,Hb 112 g／L,PLT 330×109／L,外周血可见中、晚幼粒细胞;骨髓检查:增生Ⅱ ,中、晚幼细胞增多为主,染色体查Ph 细胞100％,bcr／ab1融合基因( )。诊断为“慢性粒细胞白血病(慢性     

CAG联合地西他滨、左旋门冬酰胺酶和地塞米松治疗难治性急性混合细胞白血病1例

<正>1病例资料患者,女,24岁,因诊断急性白血病近7个月,发热、腹泻5~6d,于2013年4月23日入院。2012年9月底因反复出血点,瘀斑2个月余就诊于我科,查血常规示:WBC 269.45×109/L,Hb 56g/L,PLT 22×109/L,完善骨髓细胞学、白血病免疫分型检查诊断为急性髓细胞白血病(AML)伴T细胞表达,给予羟基脲降血细胞治疗后于2012年10     

异体输血感染恶性原虫报告1例

<正>患者,男,28岁,软件工程师,河南巩义市人,在南京工作。2013年5月27日,因持续高热至江苏省中医院就诊。入院查体:急性病容,脸色苍白,体温39.8℃。血常规检查:血红蛋白86 g/L,红细胞计数3.2×1012/L,白细胞计数7.2×109/L,血小板计数18×109/L,外周血中可见原始单核细胞和幼稚单核细胞为34%。骨髓细胞学检查:骨髓增生活跃,可见幼稚单核细胞和早幼粒细胞为57%。临床诊断为急性单核细胞性白血病,于当日入院开始化疗。患者住院化疗期间出现严重贫血,血红蛋白最低降至60g/L,     

异基因造血干细胞移植术后呼吸道蠊缨滴虫感染一例及文献复习

患者男,16岁.因右侧关节酸痛伴发热半个月,于2007年9月8日入院.入院体检:脾脏甲乙线4cm.血象:WBC159.3×109/L;血涂片原始细胞0.02.骨髓象:有核细胞增生极度活跃,粒系占0.89,原始+早幼粒0.06;染色体46,XY,t(9;22);bcr/abl融合基因(+),确诊为慢性粒细胞白血病-慢性期(CML-CP).胸部CT示双肺未见明显异常.给予羟基脲、伊马替尼达缓解,患者10月23日行同胞全相合异基因造血干细胞移植(allo-HSCT).     

Central nervous system lesions due to juvenile myelomonocytic leukemia progressed in a boy undergoing first line chemotherapy

Juvenile myelomonocytic leukemia is a rare malignancy that occurs in pediatric patients. Previous reports, have described leukemic cells may infiltrate many organs, such as the lungs, skin, liver, spleen, and intestines, but not the central nervous system, although central nervous system infiltration remains a point of concern in every patient with acute leukemia. Here, we present one case of a boy with juvenile myelomonocytic leukemia who developed multiple lesions in the brain while undergoing chemotherapy with 6-mercaptopurine and cytarabine. We diagnosed the central nervous system involvement by magnetic resonance imaging, cerebrospinal fluid cytology, and the patient's clinical course. He was treated with a high dose of cytarabine and intrathecal chemotherapy, then with unrelated cord blood stem cell transplantation. He has been in a first complete remission for more than 18 months after cord blood stem cell transplantation without any neurological sequelae. In conclusion, we encountered a boy with juvenile myelomonocytic leukemia who developed central nervous system lesions under standard chemotherapy. We subsequently switched treatment to central nervous system-oriented chemotherapy, which resulted in a good clinical condition and successful cord blood stem cell transplantation.     

Neoplastic meningitis in patients with acute myeloid leukemia scheduled for allogeneic hematopoietic stem cell transplantation

We analyzed the frequency of neoplastic meningitis in patients with acute myeloid leukemia prior to allogeneic hematopoietic stem cell transplantation at our institution. Between 1996 and 2009, cerebrospinal fluid samples of 204 adult patients were examined during pre-transplant work-up for cell counts and, if abnormal, morphologically. We found blasts in cerebrospinal fluid samples of 17 patients with either persistent (n=9) or newly diagnosed (n=8) neoplastic meningitis. All patients proceeded to transplant. The proportion of patients with central nervous system involvement was significantly higher in patients with refractory disease at the time of transplantation compared with patients responding to prior systemic therapy (19% vs. 4.6%; P=0.003). Since most of the patients with central nervous system involvement were asymptomatic, cerebrospinal fluid evaluation should be considered at least in patients with refractory acute myeloid leukemia.     

Detection of rare malignant cells and their apoptotic fragments in cerebrospinal fluid

Routine cerebrospinal fluid cytology often fails to detect small numbers of malignant cells exfoliated from primary lymphomas of the central nervous system and metastatic carcinomas. Immunocytochemistry on poly-L-lysine-coated slides was optimised to permit unequivocal identification of a single carcinoma cell in 1 mL of cerebrospinal fluid, as well as carcinoma cell-derived apoptotic bodies that themselves contribute to enhanced diagnostic sensitivity.     

THE INTRAVENTRICULAR RESERVOIR IN THE TREATMENT OF NEUROLOGICAL DISEASE SECONDARY TO HEMATOLOGICAL MALIGNANCY: AN EIGHT YEAR EXPERIENCE

Abstract Between 1974 and 1982, 17 patients with central nervous system disease secondary to hematological malignancy had an intraventricular reservoir inserted to monitor their disease and to instil cytotoxic chemotherapy. Two other patients with acute lympho-blastic leukemia and difficult access to the cerebrospinal fluid had a reservoir inserted to facilitate intrathecal chemotherapy. All 17 patients with established disease showed clinical improvement. Thirteen patients had complete elimination of malignant cells from the cerebrospinal fluid with cytotoxic chemotherapy, and the other four had a reduction in the concentration of malignant cells. Infection and neurotoxicity were found in a significant number of cases. and therefore the good clinical resDonse associated with th; procedure must be weighed carefully against the associated toxicity.     

Immunological function of the blood-cerebrospinal fluid barrier.

Because the brain lacks a true lymphatic system, it is unclear how peripheral lymphocytes recognize foreign antigens present in the central nervous system. This report demonstrates that the choroid plexus, which constitutes the blood-cerebrospinal fluid barrier, is able to present foreign antigen to, and stimulate the proliferation of, peripheral helper T lymphocytes through an Ia-dependent, major histocompatibility complex-restricted mechanism. Furthermore, in vivo, choroid plexus epithelial cells have access to, and are capable of taking up, virus-sized particles injected elsewhere into the cerebrospinal fluid. Thus these data suggest that the blood-cerebrospinal fluid barrier may play a role in immunological communication between the central nervous system and periphery, a function relevant to the initiation of immunological responses to central nervous system infections and autoimmune processes and for the surveillance of tumor cells in the cerebrospinal fluid.     

Eosinophils in the cerebrospinal fluid.

Eosinophils in the cerebrospinal fluid are an uncommon finding that is most often the result of a helminthic infection of the central nervous system. Information from the recorded literature suggests the differential diagnosis of this clinical observation is relatively limited. Therefore, in the appropriate clinical circumstances, cryptic cases of central nervous system disease might be resolved or the diagnostic possibilities at least narrowed by finding these cells in the cerebrospinal fluid.     

Central nervous system involvement in acute nonlymphocytic leukemia. A prospective study of adults in remission

To identify adults with acute nonlymphocytic leukemia at risk for the development of central nervous system involvement, we performed periodic cerebrospinal fluid examinations on patients in remission. Among 58 consecutive patients monitored during first remission, central nervous system leukemia developed in nine (16 percent). Four patients, including one who was symptomatic, had central nervous system leukemia detected simultaneously with marrow relapse. Five additional patients were asymptomatic and continue to have bone marrow remission. Following central nervous system and systemic treatment, two of these five patients have never had relapse, and three had relapse in the bone marrow five, 10, and 21 months later. Factors at diagnosis associated with the subsequent development of central nervous system leukemia were elevated leukocyte count, serum lysozyme and lactate dehydrogenase, extramedullary infiltration including splenomegaly, and monocytic (FAB M4 or M5a) morphology. In six of 17 patients (35 percent) with monocytic morphology, central nervous system leukemia developed compared with only three of 41 patients (7 percent) with other subtypes (p = 0.02). Discriminant analysis identified leukocyte count, splenomegaly, and M4 or M5a morphology as the most important risk factors and led to a mathematical formula that correctly identified 90 percent of the patients. Although the risk of central nervous system leukemia in adults with acute nonlymphocytic leukemia is too low to justify routine prophylaxis, those patients recognized to be at a greater risk should receive prophylaxis or be monitored closely with periodic lumbar punctures.     

Isolated central nervous system relapse of chronic myeloid leukemia after allogeneic hematopoietic stem cell transplantation

ABSTRACT: BACKGROUND: This case report highlights the relevance of quantifying the BCR-ABL gene in cerebrospinal fluid of patients with suspected relapse of chronic myeloid leukemia in the central nervous system. Case Presentation: We report on a female patient with isolated central nervous system relapse of chronic myeloid leukemia (CML) during peripheral remission after allogeneic hematopoietic stem cell transplantation. The patient showed a progressive cognitive decline as the main symptom. MRI revealed a hydrocephalus and an increase in cell count in the cerebrospinal fluid (CSF) with around 50% immature blasts in the differential count. A highly elevated BCR-ABL/ ABL ratio was detected in the CSF, whilst the ratio for peripheral blood and bone marrow was not altered. On treatment of the malresorptive hydrocephalus with shunt surgery, the patient showed an initial cognitive improvement, followed by a secondary deterioration. At this time, the cranial MRI showed leukemic infiltration of lateral ventricles walls. Hence, intrathecal administration of cytarabine, methotrexate, and dexamethasone was initiated, which caused a significant decrease of cells in the CSF. Soon after, the patient demonstrated significant cognitive improvement with a good participation in daily activities. At a later time point, after the patient had lost the major molecular response of CML, therapy with dasatinib was initiated. In a further follow-up, the patient was neurologically and hematologically stable. CONCLUSIONS: In patients with treated CML, the rare case of an isolated CNS blast crisis has to be taken into account if neurological symptoms evolve. The analysis of BCR-ABL in the CSF is a further option for the reliable detection of primary isolated relapse of CML in these patients.     

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急性白血病(AL)中枢神经系统受累,称中枢神经系统白血病(CNSL),发生在AL任何时期,以急性淋巴细胞白血病(ALL)居高。初发时高白细胞计数、有明显髓外侵润、某些类型如M4、M5a、T细胞型、成熟Burkitt型、复发M3及有t(4;11)、Ph+遗传学特征都是CNSL高危发病因素。脑脊液(CSF)涂片中找到白血病细胞最具诊断意义。CSF白细胞>5个/μL,伴有CNS异常表现即可诊断CNSL。对CSF进一步分类有助于ALL分层治疗。CNSL治疗重在预防,常用的防治方法有鞘内化疗、全身系统治疗以及放射治疗。现代治疗模式不再强调放疗的作用,中大剂量化疗药物的早期使用可有效防治CNSL。     

急性布鲁氏菌病并发中枢神经损害患者临床特征分析

目的 分析布鲁氏菌病中枢神经损害脑脊液特征,探讨其临床诊断价值。方法 选择2007年至2014年黑龙江省农垦总局总医院住院治疗的急性布鲁氏菌病并发中枢神经损害患者20例作为观察对象。临床诊断:并发脑膜炎8例、脑膜脑炎3例、脊髓炎7例和脑脓肿2例。调查其住院病例资料,观察脑脊液常规检查各项指标,分析和评估脑脊液改变特点。结果 脑脊液异常改变,颅内压增高13例,白细胞计数增多18例(90.00%),蛋白增高19例(95.00%),葡萄糖和氯化物降低16例(80.00 %)。结论 布鲁氏菌病并发中枢神经损害患者脑脊液多发生异常改变,颅内压升高,白细胞计数增多,葡萄糖和氯化物降低为特征,诊断需要与结核性脑膜炎相鉴别。