目的 探讨混合谱系白血病(MLL)基因重排阳性的急性淋巴细胞白血病(ALL)儿童的骨髓细胞形态特征及其与临床表现和预后的相关性.方法 回顾性收集首都医科大学附属北京儿童医院(我院)血液肿瘤中心采用中国儿童白血病治疗协作组(CCLG)ALL-2008方案治疗的MLL基因重排阳性的ALL初诊患儿(MLL+组),以我院CCLGALL-2008方案治疗且时问相近、同性别的MLL阴性的初诊ALL患儿为配对条件(MLL-组).MLL+组依据骨髓细胞中是否有嗜天青颗粒、核仁、空泡和伪足分为阳性亚组和阴性亚组.考察MLL+和MLL-组形态特征差异;比较MLL+组形态学阳性亚组和阴性亚组临床表现和无事件生存率(EFS),通过受试者工作曲线(ROC)探讨骨髓细胞不典型形态特征对患儿预后的预测能力.结果 2011年1月1日至2018年5月31日共收治MLL+组26例,其中男12例,女14例;年龄0.4～13岁,18例处于完全长期缓解状态,5例骨髓复发,3例确诊后放弃治疗.MLL-组30例,男16例,女14例;年龄0.8～14岁.MLL+组骨髓细胞形态与MLL-组比较差异无统计学意义,糖原染色阳性率和赋分值均低于MLL-组.骨髓原始、幼稚淋巴细胞核仁、嗜天青颗粒、空泡、伪足的形态特征与初诊时外周血WBC、性别、年龄无相关;6例MLL-AF4融合基因阳性者全部发现具有核仁,其他MLL重排者具有核仁的比例显著低于MLL-AF4+者,差异有统计学意义(P=0.028).无伪足患儿第33天MRD≥10-4的比例高于有伪足的患儿,差异有统计学意义(P=0.025),有、无核仁的患儿3年EFS[(55.0±15.0)％vs.100％],差异有统计学意义(P=0.049);有、无嗜天青颗粒的患儿3年EFS[(41.7±22.2)％vs.(83.9±10.4)％]差异无统计学意义(P=0.052);联合核仁和颗粒对MLL+组预后的ROC曲线分析结果显示,联合预测能力优于分别预测(AUC分别为0.833、0.786和0.705),P值分别为0.023、0.051和0.162.结论 MLL基因重排阳性ALL患儿骨髓白血病细胞具有独特的组化染色特征,其形态表现与MLL重排亚型相关,并可预测患者预后.

1 出血机制 皮肤黏膜出血指机体的止血与凝血功能发生障碍,导致皮肤黏膜局限或广泛出血,或受到损伤后出血不止. 临床表现为红色、暗红色出血点或有紫色斑点、斑块,一般不高出皮面,压之不褪色. 出血面直径≤2 mm称出血点,>2~5 mm称紫癜,>5 mm称淤斑,如为片状出血伴皮肤隆起则称为血肿. 生理性止血的过程如图1.

目的 探讨儿童急性B淋巴细胞白血病(B-ALL)伴有髓系抗原(CD33和/或CD13、CD117)表达阳性与表达阴性患儿的骨髓涂片中原始、幼稚细胞形态的异同,以提高ALL形态学诊断的准确率.方法 收集经细胞形态学、免疫学、细胞遗传学和分子生物学(MICM)诊断为B-ALL的患儿124例.采用流式细胞术检测B-ALL患儿髓系抗原表达,与髓系抗原表达阳性和阴性患儿的骨髓细胞形态进行回顾性分析.结果 124例B-ALL患儿中,有43例(34.7%)CD33抗原为阳性,2例(1.6%)CD33、CD13抗原阳性,2例(1.6%)CD33、CD117抗原阳性,1例(0.8%)CD117抗原阳性.在48例髓系抗原表达为阳性的患儿中,有29例患儿骨髓涂片中的原始、幼稚细胞形态不易观察、判别,占全部患儿的23.4%,占抗原阳性患儿的60.4%.结论 儿童B-ALL伴髓系抗原表达的骨髓细胞涂片中,原始及幼稚细胞形态存在多样性及异质性,多不同于髓系抗原阴性的细胞形态,不易判别.

Objective To explore the clinical effect and toxicity of daunorubicin combined with cytarabine (DA regimen) and idarubicin combined with cytarabine (IA regimen) for the treatment of patients with acute myeloid leukemia (AML) as induction chemotherapy. Methods The clinical data of 84 newly diagnosed AML patients (except M3) treated with DA or IA regimen were analyzed retrospectively. DA regimen group included 32 patients (17 males and 15 females with median age of 46 years), while IA regimen group included 52 patients (29 males and 23 females with media age of 49 years). Efficacy index was complete remission (CR), total efficiency and adverse reactions after one course of chemotherapy rate. Results In DA regimen group,the CR rate was 65.6 %(21/32), and the total efficiency rate was 75.0 %(24/32), while in IA regimen group, the CR rate was 71.2 %(31/52), and the total efficiency rate was 80.8 %(42/52), respectively, but, the differences of media survival and 5-year survival rate were not statistically significant (16.8 months vs. 24.9 months, 26 % vs. 44 %, both P>0.05). The main side effect in the two groups included hematologic (bone marrow suppression) and non-hematologic adverse reactions, with no significant difference between the two groups (all P>0.05). Conclusion For newly diagnosed AML patients, remission rate and total efficiency of DA regimen are same as IA regimen after one course treatment, and adverse events between the two regimens do not differ significantly.

Objective To explore the clinical efficacy and safety of low-dose decitabine combined with cytarabine for myelodysplastic syndromes (MDS). Methods Clinical data of 15 patients with MDS who took the therapeutic regimen with decitabine combined with cytarabine were collected from January 2012 to January 2015. The clinical efficacy and adverse effects were assessed. Results Among the 15 patients, 4 cases were complete remission (CR), 5 cases were partial remission (PR) and 6 cases were stable disease (SD) and progressive disease (PD). The total effective rate was 60.0 % (9/15). Grade Ⅲ-Ⅳ bone marrow depression occurred in 11 cases with incidence rate of 73.3 % (11/15), and the total incidence rate of infection was 40.0 % (6/15), including lung infection of 26.7 % (4/15). All the infections were controlled after active supportive treatment and anti-infection therapy. No patient died of chemotherapy. Conclusions Low-dose decitabine combined with cytarabine can effectively treat MDS and delay the progress of disease. The patients can tolerate the adverse effects in chemotherapy with a low mortality rate.

OBJECTIVE:To investigate the clinical characteristics, therapeutic outcomes and prognostic factors of primary central nervous system lymphoma (PCNSL).METHODS:Clinical records of 31 cases of PCNSL were collected, the clinical charactenstics were analyzed retrospectively. Survival curves were estimated using Kaplan-Meier survival methodology and statistical significance of continuous variables was assessed via the Cox proportional hazard model.RESULTS:The median age was 52 years, with a ratio of male to female 1:1. Headache/dizzy/limb numbness were the most common presentation and the lesions of PCNSL were primarily located at the frontal, parietal, temporal lobes and corpus callosum. All the cases were pathologically diffuse large B cell lymphoma (DLBCL), 6 cases were the type of germinal center (GC) and 25 cases of non-GC type, after craniotomy operation and biopsy. Among 31 cases, 17 patients accepted the combined treatment, 3 patients underwent simple chemotherapy, 8 patients received simple radiotherapy, the other patients accepted support therapy. The median follow-up for surviving patients was 24 months. The 1-, 3-, and 5-year survival rates were 80.6%, 55.1%, and 36.4%, respectively. The median overall survival time in the combined treatment group was significantly longer than that in simply radiotherapy. There was no significant difference in OS between the groups with and without rituximab. ECOG PS≥2 and elevated serum LDH predicted inferior survival.CONCLUSION:The clinical manifectation of PCNSL is various and complicated, and for the time being there is no optimal treatment scheme. The overall survival time of the combined treatment is longer than that in simply radiotherapy. ECOG PS≥2 and elevated serum LDH often are poor prognostic factors.

Objective To elucidate the curative effects and toxicity of bortezomib in combination with CHOP for patients with angioimmunoblastic T cell lymphoma (AITL).Methods The charts of 14 patients with AITL who received bortezomib 2 mg/m2 d1 plus CHOP regimen were reviewed.Results Among 14 patients including 12 initial and 2 refractory patients, 12 cases got remission (complete remission in 6 cases and partial remission in 6 cases).The anticipated 3-year overall survival rate was 55 ％.The median progression-free survival was 9.4 months.The anticipated 3-year progression-free survival rate was 38 ％.Grade Ⅲ-Ⅳ leucopenia was the most frequent hematological toxicity (6 cases).All of non-hematological toxicities were Ⅰ-Ⅱ grade including peripheral neurotoxicity (8 cases), nauseating and vomiting (6 cases), diarrhea (4 cases) and infection (4 cases).Conclusion The combination of bortezomib and CHOP is an effective and feasible regimen for AITL with acceptable toxicity.

Objective To study the efficacy and safety of CHOP chemotherapy and FCD chemotherapy for previously untreated indolent lymphoma. Methods Between January 2008 and January 2015,59 patients with indolent non - Hodgkin lymphoma were included into the study. 32 of them received CHOP regimen,27 of them received FCD regimen. The clinical therapeutic effect and bone marrow suppression after chemo-therapy of two groups were observed. Results There was no significant difference in overall response between CHOP group and FCD group(75%vs. 66. 7% ,P ﹥ 0. 05). There was significant difference in myelo - suppression between CHOP group and FCD group(43. 8% vs. 81. 4% ,P﹤ 0. 05). Conclusion CHOP regimen may exert similar therapeutic effects with FCD regimen on patients with previously untreated indolent lym-phoma,but FCD regimen has more significant myelo - suppression than CHOP regimen.

>套细胞淋巴瘤(mantle cell lymphoma,MCL)在非霍奇金B细胞性淋巴瘤中仅占2.5% 6 .7%。根据淋巴瘤细胞的形态将其分为经典型MCL(common form of MCL,C-MCL)和变异型MCL。母细胞型MCL(blastic variant form of MCL,BVMCL)约占套细胞淋巴瘤的6.3% [1] ,在形态上不易与淋巴母细胞淋巴瘤或其他大细胞淋巴瘤相鉴别,需免疫组化确诊。MCL患者可在发病时即表现为母细胞型,也可在疾病进展过

Objective To investigate the effects of ubiquitin isopeptidase inhibitor Ⅰ on proliferation and apoptosis of human leukemic K562 cells.Methods Human leukemic K562 cells were treated with different concentration of ubiquitin isopeptidase inhibitor Ⅰ.Cell proliferation was monitored by MTT assay.Cell apoptosis was analyzed by flow cytometry after Annexin V/PI staining,and mRNA expression levels of bcl-2,bax and Caspase-3 were quantified by reverse transcription-polymerase chain reaction (RT-PCR).Results MTT assay showed that the proliferation of K562 cells was markedly inhibited by ubiquitin isopeptidase inhibitor Ⅰ in a time-and-dose dependent manner.The Annexin V positive rate of K562 cells after treatment with ubiquitin isopeptidase inhibitor Ⅰ was significantly increased by FCM analysis (P ＜ 0.05).The early apoptosis rate of K562 cells treated with 100 nmol/L ubiquitin isopeptidas inhibitor Ⅰ by 72 h,was obviously increased compared to control cells [(15.4±1.3) ％ vs (4.1±0.9) ％,P ＜ 0.01].The mRNA levels of Caspase-3 and bax were up-regulated and bcl-2 was down-regulated after treated with ubiquitin isopeptidase inhibitor Ⅰ,and the differences were statistically significant from control cells (P ＜ 0.05).Conclusion Ubiquitin isopeptidase inhibitor Ⅰ has inhibitory effect on proliferation and inductive effect on apoptosis of K562 leukemia cells,implying its possible application in treating leukemia.

Objective To evaluate the efficacy and safety of the modified Hyper-CVAD/MA regimen on patients with invasive non-Hodgkin lymphoma (NHL).Methods Clinical data of 42 invasive NHL patients treated with modified Hyper-CVAD/MA regimen from June 2006 to July 2012 were retrospectively analyzed.Results 19 patients (45.3 ％) got complete response (CR) and 13 patients (31.0 ％) got partial response (PR),with response rate as 76.2 ％ (32/42).The 1-year disease free survival (DFS) rate and overall survival (OS) rate were 79.2 ％ and 83.7 ％ respectively,and those for 3-year were 65.3 ％ and 64.2 ％ respectively.There were poor prognosis and lower CR rate for patients with B system,advanced stage,central nervous system (CNS) and extra-node involved and relapsed.The main adverse reactions were bone marrow suppression,infection,mucositis,and toxicity of liver,kidney and CNS.Conclusion Hyper-CVAD/MA regimen can achieve a satisfactory result in the treatment of invasive NHL patients,and the correlated complications can be controlled.

<正>患者:男,19岁,主因"反复发热伴外周血3系减低5个月"入院。患者入院5个月前出现发热,于当地医院就诊,查血常规示白细胞2.51×109/L,中性粒细胞0.749,血红蛋白70 g/L,血小板63×109/L。生化乳酸脱氢酶1 093 U/L,天冬氨酸转氨酶103.6 U/L,丙氨酸转氨酶133 U/L,三酰甘油(TG)1.41 mmol/L。凝血功能:血纤蛋白原1.547 g/L,铁蛋白1 069.9μg/L。腹部超声及腹部CT示脾肿大。骨髓

Objective To analysis the characteristics of nosocomial infection of hematological disease,so as to provide reference for clinical therapy.Methods The characteristics of clinically isolated bacterium from the patients in hematology department of Beijing Friendship Hospital from the July 2008 to July 2011 was analyzed.Results Among the total of 232 isolates of bacterium,the Gram-negative bacilli was 62.9%,the Gram-positive cocci was 37.1%.The rate of Gram-negative bacilli from blood,sputum and urine culture was 29.4 %,46.6 % and 10.3 %,respectively.The rate of Gram-positive cocci from blood,sputum and urine culture was 14%,47.7% and 10.4 %,respectively.Gram-negative bacilli were most sensitive to Carbapenems,the sensitive rate was more than 90%.The 19% of pseudomonas aeruginosa was resistant to imipenem,and the 14.3% of acinetobacter baumannii was resistant to imipenem.The amikacin sulphate and tobramycin were more sensitive to them,the sensitive rate was 100%.The Gram-positive cocci were more sensitive to linezolid and vancomycin,but resistant to clindamycin and multiple quinolones.The sensitive rate of Enterococcus faecium to vancomycin was only 71.4%,and the sensitive rate to linezolid was 100%.Conclusion Patients with hematopathy are the main population of nosocomial infections,the Gram-negative bacilli are the most common pathogens.Respiratory tract is the important site.The prompt microbiologcal examination and drug sensitivity tests are important to rationally select antibiotics,reduce infection incidence,and decrease the occurence of drug resistant strains in hematology department.

目的 分析肾移植术后弥漫大B细胞淋巴瘤的治疗方法.方法 回顾性分析5例肾移植术后弥漫大B细胞淋巴瘤患者临床资料并文献复习,5例患者1例放弃治疗,4例予以R-CHOP方案化疗,治疗的4例中3例达到完全缓解.结果 5例患者中3例完全缓解,1例放弃治疗,1例无效.结论 肾移植术后淋巴增殖性疾病(PTLD)可以应用多种方法治疗,目前没有标准的治疗方案,还需继续探索有效的治疗方法.

Objective:To describe the clinical and pathological features,treatment and prognosis of langerhans cell neoplasms.Methods:A group of patients was described,including 3 cases of langerhans cell histiocytosis(LCH),and 1 case of langerhans cell sarcoma(LCS).The related literatures were reviewed.Results:Three patients with LCH were 29-year-old woman,20-year-old man and 35-year-old man.The involved organs including skeleton(2/3),liver(2/3)、hypothalamus-pituitary gland(diabetes insipidus)(2/3),skin(1/3),lymph node(1/3),thyroid gland(1/3),lung(1/3).Pathological diagnosis of biopsy was positive for CDla and S-100.2 patients received 4 cycles of chemotherapy.One achieved partial remission,the other had no response.The patient with LCS was a 77-year-old woman,who exhibited lymphadenopathy and was diagnosed by lymph node biopsy.She was involved in several organs,aggravated rapidly and died of multiple organs failure.Conclusion: LCH is not common in adult,with no special clinical manifestations and is easy of diagnostic errors and missed diagnosis.The treatment should be individualization.LCS is rare,with intensive invasion and aggravated rapidly.The prognosis is poor.

目的研究氟达拉滨继发单纯红细胞再生障碍性贫血(PRCA)的临床特点及治疗方法。方法回顾分析3例氟达拉滨继发PRCA病例资料,并复习相关文献。结果 3例在化疗后1～6个疗程出现PRCA,需要输血治疗,并需要停止原发病的治疗。结论氟达拉滨继发PRCA,一旦出现,通常治疗困难,并会延误原发病的治疗。目前有用利妥昔单抗治疗成功的报道。氟达拉滨导致PRCA的机制目前还不清楚,需要进一步研究。

目的 观察以中剂量阿糖胞苷为主方案治疗非霍奇金淋巴瘤的治疗效果.方法 对23例非霍奇金淋巴瘤患者以中剂量阿糖胞苷为主的方案进行化疗.其中T淋巴母细胞性淋巴瘤5例,B淋巴母细胞性淋巴瘤5例,原发性中枢神经系统弥漫性大B细胞淋巴瘤3例,难治复发的弥漫性大B细胞淋巴瘤3例,伯基特淋巴瘤2例,NK/T细胞性淋巴瘤2例,血管免疫母细胞性T细胞淋巴瘤1例、ALK阴性的问变性大细胞淋巴瘤1例,复发的套细胞淋巴瘤1例.结果 完全缓解17例,部分缓解5例,无效1例.主要不良反应为骨髓抑制,Ⅲ～Ⅳ度白细胞和血小板减少的发生率分别为100%和78.2%.结论 以中剂量阿糖胞苷为主的方案治疗非霍奇金淋巴瘤疗效较好,其骨髓的毒性反应也非常明显.

Objective To explore the acquired deficiencies of vitamin K-dependent coagulation factors in etiology, clinical characteristics and treatment. Methods Retrospective analysis was performed on the data of etiology, clinical manifestations of 45 patients with acquired deficiencies of vitamin K-dependent coagulation factor. All patients were treated with Vitamin K1 10 -40 mg/d, i. v. , for three months. Some patients with severe blooding were additionally treated with fresh freezing plasma or prothromibin complex. Prothrombin time(PT) and activated partial thromboplastic time(APTT) were measured using Stago automatic blood coagulation analyzer before and after treatment. Ⅱ , Ⅶ, Ⅸ and Ⅹ were measured in some patients. Results Among the 45 cases, no certain cause was found in 19 cases (42.2%), anticoagulant rodenticides poison was a common cause ( 11 cases,42.3% ). The main presentations was hemorrhage, the most common bleeding sites were mucosa (77.8%) (35/45)and hematuria (46.7%) ( 21/45 ). After vitamin K1 treatment, PT and APTT had shortened remarkably from ( 110.35 ± 35.36 ) s,(98.91 ±48.98)s to (13.48 ±2. 17)s,(33.25 ±6.95)s,respectively(t=19.10 and 6.19,Ps ＜0.01)and the activities of factor Ⅱ、Ⅶ、Ⅸ、Ⅹ had rapidly increased from ( 17.48 ± 10.93 ) %, ( 10.23 ± 5.68 )%, ( 11.98 ±4.69)%,(12.93±7.48)% to (70. 12 ±21.31)%,(92.76 ±29. 15)%,(88.64 ±40. 21)%,(63.97 ±20.11)%(t=12.13,14.43,13.27and9. 74,respectively,Ps＜0. 01).Conclusions The histories of patients with acquired deficiencies of vitamin K-dependent coagulation factors are usually hiding, therefore it is easily misdiagnosed. It is worth of detecting PT and APTT in diagnosis and monitoring. Using vitamin K1 10 -40 mg/d is effective and safety.

A 36-year-old man with acute leukemia received a DA regimen(an IV infusion of daunomycin 100 mg once daily on days 1-3+an IV infusion of arabinoside 100 mg in the morning and 150 mg at night on days 1-7).On day 6 of admission,a chest CT scan showed pericardial thickening.On day 11,the patient developed cough,shortness of breath,palpitation.His blood pressure was 145/105 mm Hg and heart rate was 120 beats/min.His heart sound was distant.Based on the findings of ultrasound cardiogram,pericardial effusion and acute cardiac tamponade were considered.He underwent pericardiocentesis immediately;however,the treatment failed.On day 12,the patient died from respiratory and circulatory failure.

Objective:To analyse the clinical characteristics of non-leukemic myeloid sarcoma(NMS) ,including the incidence,diagnosis,prognosis and treatment tactics.Method:To summarize and analyze a newly identified gingival NMS case reported from Beijing Friendship Hospital and conducted literature review on relevant cases reported.Result:It was quite common for non-leukemic myeloid sarcoma to base in lymphonode,skin,reproductive organs and digestive organs.Therefore the reported case was quite rare considering its location.The disease was quite often misdiagnosed as malignant lymphoma.The classic morphologic features of the tumors appear all stages of myeloid differentiation and immunohistochemistry features of NMS with positive MPO,CD43,and CD68.Conclusion:To reduce the rise of subsequent ANLL in patients with NMS and prolong the survival time,it is critical to make an early and accurate histologic diagnosis and conduct timely intensive chemotherapy with ANLL regimen.