髓系/自然杀伤细胞前体细胞急性白血病一例并文献复习

目的 提高对髓系/自然杀伤细胞前体细胞急性白血病的认识.方法 报道一例髓系/自然杀伤细胞前体细胞急性白血病的临床特点、诊断和治疗经过,并进行文献复习.结果 以DA方案治疗2个疗程后达完全缓解,此后以大剂量阿糖胞苷巩固化疗,目前患者处于持续完全缓解状态.结论 髓系/自然杀伤细胞前体细胞急性白血病对急性髓系白血病化疗方案的反应优于对急性淋巴细胞白血病化疗方案的反应,参照急性髓系白血病的化疗方案大部分可以达到完全缓解,但总体来说缓解期短,缓解后易复发,患者的中位生存期仅为19个月.     

杂合性急性白血病7例报告

目的 :观察急性杂合性白血病的免疫标志抗原及患者的临床特点、疗效、预后 ,以探讨合适的化疗方案。方法 :1骨髓的细胞形态学 ;2白血病细胞免疫表型标记 ( APAAP)技术。结果 :急性杂合性白血病主要以淋系表达为主 ,其中 4例表达 T淋巴细胞抗原 ;急性杂合性白血病患者临床髓外浸润明显 ,且化疗效果差。结论 :急性杂合性白血病患者临床治疗效果差 ,生存期短 ,预后差 ,合适的化疗方案还有待进一步探索     

髓细胞肉瘤临床病理特征并文献复习

目的:探讨髓细胞肉瘤（myeloid sarcoma,MS）的临床表型、病理特征及治疗反应。方法:对西京医院2015年9月至2018年12月间收治的经组织病理学证实的7例MS的临床资料进行分析,并复习相关文献。结果:7例MS患者中位发病年龄40岁（22~62岁）。1例诊断MS时合并骨髓增生异常综合征（MDS）,其余6例骨髓形态学检查正常。7例MS病变分别累及皮肤、淋巴结、胰腺、胆管、乳腺、胸壁及眼眶。6例患者接受联合化疗、去甲基化治疗或自体造血干细胞移植（ASCT）。中位生存期7个月。其中4例死亡患者中,1例患者诊断后8个月进展至急性髓系白血病（AML）,经多疗程联合化疗后,生存期达31个月,3例未进展至AML的孤立性MS生存期仅5个月左右。至随访结束时,2例患者存活,疾病仍处于完全缓解状态。1例患者发病时伴发MDS,多疗程化疗方案含去甲基化药物;另1例患者接受大剂量化疗联合ASCT治疗。结论:进展至AML的MS,其临床预后可能不劣于孤立性MS;由去甲基化药物或ASCT组成的强化治疗方案似乎有益于改善MS的临床预后。     

小剂量化疗治疗难治性急性髓系白血病疗效观察

 目的：探讨难治性急性髓细胞白血病按需小剂量化疗的临床疗效。方法：34例难治性急性髓细胞白血病患者随机分为两组,治疗组18例予按需小剂量化疗,对照组16例仅给予支持治疗。结果：治疗组共予91个疗程化疗,CR3例次,PR34例次,NR54例次。CR﹢PR﹦40.7%,生存期为11~20个月;对照组采取支持治疗,未见缓解者,生存期为2~7个月,仅1例超过了6月。在缓解率及生存期上有显著性差异。结论：按需小剂量化疗在难治性急性髓细胞白血病的治疗中是一有效治疗措施。     

影响老年急性髓系白血病患者预后的危险因素分析

目的 探讨影响老年急性髓系白血病患者预后的危险因素.方法 回顾性分析121例老年急性髓系白血病患者的临床资料.对比不同临床资料患者的完全缓解率和中位生存期.通过多因素Cox模型分析统计影响老年急性髓系白血病患者预后的危险因素.结果 本研究患者的中位生存期为131 d(95%可信区间109~154 d),诱导化疗后的完全缓解率为29.75%.年龄≤70岁、PS评分﹤2分、原发急性髓系白血病、骨髓原始细胞比例≤50%、接受标准化疗以及白细胞CD34表达阴性患者的完全缓解率升高(P﹤0.05);年龄≤70岁、PS评分﹤2分、原发急性髓系白血病、初治时的白细胞计数≤50×109/L、骨髓原始细胞比例≤50%、接受标准化疗以及白细胞CD34表达阴性患者的中位生存期延长(P﹤0.05);多因素Cox模型分析结果显示,年龄、PS评分、初治时白细胞计数以及治疗方案是影响老年急性髓系白血病患者预后的危险因素(P﹤0.05).结论 年龄、PS评分、初治时白细胞计数以及治疗方案是影响老年急性髓系白血病患者预后的危险因素.临床应通过整体评估,制定个体化的化疗方案,以改善患者的预后.     

急性T／B细胞双表型白血病四例并文献复习

 目的　总结急性T／B细胞双表型白血病的临床及实验室特征,以提高认识。方法　报道该院诊治的4 例,复习文献报道的10例,共14例急性T／B细胞双表型白血病患者资料,总结其临床表现、实验室特征及预后。结果　急性T／B细胞双表型白血病以中青年男性相对多见,14例患者中男10例（年龄在17～46岁）,临床表现符合急性淋巴细胞白血病的一般特点,该类型白血病对急性淋巴细胞白血病的常规化疗方案反应差,疾病进行性恶化,中位生存期仅10个月,6例患者诊断后12个月内死亡。结论　急性T／B细胞双表型白血病极为少见,预后恶劣。 提高对该病的认识,制订合适的治疗策略很有必要。     

急性双系列白血病诊断与预后观察

目的明确急性双系列白血病（aBLL）的诊断,判断患者预后。方法回顾性分析aBLL病例的临床和实验室资料,明确其白血病细胞特征以及治疗疗效和生存情况。结果共计筛选了352例患者,符合aBLL诊断的11例,其中男4例,女7例,中位年龄38岁,发病时白细胞中位计数56．3×10^9／L。髓／B淋巴细胞混合（My-B）aBLL患者数多于髓／T淋巴细胞混合（My-T）aBLL患者（分别为8例和3例）。7例患者进行了细胞遗传学分析,仅1例有复杂核型改变,其余均为标准核型。诱导治疗缓解率为45．5％（5／11）,反应率（CR＋PR）为54．5％（6／11）,1个疗程缓解2例,缓解持续时间3-14个月,中位时间11个月。生存时间1～23个月,中位生存时间10个月。诱导治疗期死亡1例。结论aBLL是一种少见的急性白血病亚型,诊断需要结合细胞形态学和细胞免疫表型的检测结果。该采用急性白血病常用化疗方案,疗效差,预后不良。     

急性混合细胞性白血病八例的临床及生物学特征

 目的 研究急性混合细胞性白血病（MAL）的临床特征、实验室指标、治疗及预后。方法 对8例MAL骨髓标本均进行细胞形态学、免疫学、遗传学分析,运用急性淋巴细胞白血病化疗方案或兼顾髓、淋二系方案化疗。结果 FAB分型显示急性淋巴细胞白血病（ALL-L2）3例,ALL-L1 4例,急性髓系白血病（AML-M1）1例,免疫分型显示MAL患者均有髓、淋二系表达,其中3例为T、B、髓3系共表达。染色体核型分析可见异常核型,但无特征性改变。该类患者大多数对治疗反应差,预后不良。结论 MAL具有独特的临床和生物学特征,通常预后较差。     

多发性骨髓瘤继发急性髓系白血病自发缓解一例并文献复习

目的观察多发性骨髓瘤继发急性髓系白血病出现自发缓解的影响因素、病理变化、自然病程,以探究多发性骨髓瘤病情变化过程中的诊查治疗思路。方法回顾性分析上海中医药大学附属龙华医院收治的1例多发性骨髓瘤继发急性髓系白血病患者的临床资料,并复习相关文献。结果该例患者确诊多发性骨髓瘤后行BCD方案诱导化疗,病程中出现继发急性髓系白血病,暂缓治疗1个月后,复查血象、骨髓象提示急性髓系白血病自发缓解,约4个月后患者急性白血病复发,最终死亡。结论多发性骨髓瘤治疗过程中出现继发急性髓系白血病的案例并不少见,且多提示病情变化,预后不良。本例患者虽罕见地出现了继发白血病的自发缓解,但无进展生存时间短,相关个体化诊治方案需进一步探索。     

成熟自然杀伤细胞白血病1例报告并文献复习

 目的 提高对自然杀伤（NK）细胞恶性病的认识。方法 报道1例 成熟NK细胞白血病诊断及治疗经过,并进行文献复习。结果 患者外周血淋巴细胞进行性增高,确诊后给予COP方案化疗,化疗后7 d内死亡,生存期54 d。结论 成熟NK细胞白血病病程侵袭,且治疗反应差,预后不良。     

Granulocytic sarcoma: 32 cases and review of the literature

Thirty-two cases of granulocytic sarcoma (GS) are reported in this paper. Age range was from 16 - 70 years. GS was accompanied by AML in 13 cases, ALL (My+) in one case, CML in 11 cases and MDS in two cases. GS was diagnosed simultaneously with leukemia in five cases and preceded the leukemia in eight. Lymph node and soft tissue were the most commonly detected localizations. Seven cases had first been diagnosed as NHL. Histopathologically blastic, immature and mature variants were found in 11, nine and 11 cases respectively and overall survival was shortest in the blastic type. Myeloperoxidase and lysozyme were found to be positive in 30 and 24 cases respectively. Therapy was radiation in five cases and surgery in three. Systemic chemotherapy was given to the cases. The clinical outcome of the patients after the diagnosis of GS was poor. GS is a unique entity; prognosis is poor but it is important to detect the signaling pathways associated with migration of myeloid cells to the extra-medullary tissues. The critical factors for detecting this interesting tumor are to be aware of this disease, cooperation between clinician and pathologist and the application of special stains to detect the myeloid origin.     

Primary liver cancer in a referral hospital in Hawaii

This is a retrospective review of 43 patients who had primary liver cancer diagnosed during 1974-1983. Patients' ages ranged from 27 to 84 years (median 52.5). Nine of 39 patients with hepatoma were females, while two of the four patients with cholangiocarcinoma were women. Hepatitis surface antigen was positive in 90% tested, and 62% had cirrhosis. Also, 60-65% were heavy users of alcohol and cigarettes. Alpha-fetoprotein was elevated in one of four white patients, and in six of eight patients of other races (75%). Tissue diagnosis was obtained by peritoneoscopy in 16, by percutaneous biopsy in 7, by laparotomy in 9, and at autopsy in 11. Only one of 11 patients who were explored has his lesion resected. About half of the cases diagnosed antemortem died 1 month or less after diagnosis. The median survival of hepatoma patients who had no specific treatment or systemic chemotherapy was 2 months. Two patients who received chemotherapy in conjunction with occlusion of the hepatic artery lived 16 to 19 months.     

卵巢原发性肺型小细胞癌8例临床病理分析并文献复习

目的:探讨卵巢原发性肺型小细胞癌（small cell ovarian carcinoma of the pulmonary type，SCCOPT）的临床病理特点、诊断、治疗及预后。方法:对2010年01月至2021年12月在我院手术并病理确诊的8例SCCOPT患者资料进行回顾性分析。结果:8例患者平均发病年龄54.25岁（35～68岁）。临床表现主要为腹痛或伴阴道流血。8例患者中，7例患者为Ⅱ-Ⅳb期。除1例Ⅳb期外，其余7例患者均接受了满意的肿瘤细胞减灭术并辅助4~8疗程紫杉醇+顺铂/卡铂方案化疗。其中7例已死亡，中位生存时间16.5月（6~49月）；即使经过积极手术并规律足量化疗的7例患者中6例死亡，但经过积极治疗患者最短生存时间为10个月，4例患者生存时间近2年或以上，1例患者在33个月后出现全身多器官转移，经再次化疗后病情缓解仍生存。结论:SCCOPT发病罕见，恶性程度高，病死率高，预后欠佳。满意的肿瘤细胞减灭术治疗并辅助足量规律化疗是主要的治疗手段。     

Comparison in treatments of nonleukemic granulocytic sarcoma: report of two cases and a review of 72 cases in the literature

BACKGROUND: The purpose of this study was to reveal the clinical characteristics of nonleukemic granulocytic sarcoma (GS) and an association between the therapeutic regimens and the nonleukemic period. METHOD: Clinical records of 2 patients reported here and 72 patients gathered using a literature search on Medline from other institutions were analyzed. The patients consisted of 57 patients who preceded acute nonlymphoblastic leukemia (ANLL) and 17 patients who did not develop ANLL. These patients were divided into 3 groups by therapeutic regimens; Group I included 12 patients who received only biopsy or surgical resection of the tumor, Group II was 20 patients who received local irradiation for the tumor, and Group III consisted of 42 patients who received systemic chemotherapy. The nonleukemic periods between these groups were compared. In Group III, the period in the patients who were treated with chemotherapy given to ANLL was compared with that in the patients who received chemotherapy used for malignant lymphoproliferative disorders (MLPDs). RESULTS: Thirty-five patients (47%) initially were misdiagnosed, and the disease was most often malignant lymphoma. Preferential sites of GS were the small intestine, mediastinum, epidural site, uterus, and ovary, which often are difficult for the detection and diagnosis in addition to the skin and lymph nodes known commonly. The nonleukemic period after the diagnosis of GS was significantly longer in Group III than in the other groups (median, 12 months in Group III vs. 3 and 6 months in Groups I and II, respectively). The aggressive chemotherapy given to ANLL led to a longer nonleukemic period than the chemotherapy used for MLPDs. CONCLUSIONS: To reduce the risk of subsequent ANLL in patients with nonleukemic GS, it is important that accurate histologic diagnosis is established initially for GS and that all isolated cases of GS, even those that appear to be cured by resection or irradiation of the tumor, are treated with intensive chemotherapy similar to that used to treat ANLL during the nonleukemic period as soon as possible.     

血管免疫母细胞性T细胞淋巴瘤继发急性髓系白血病三例并文献复习

目的 探讨血管免疫母细胞性T细胞淋巴瘤（AITL）继发急性髓系白血病（AML）的临床特征、发病机制、诊断、治疗及预后。方法 回顾性分析3例AITL继发AML患者的临床资料,包括免疫组织化学、流式细胞术结果,并进行文献复习。结果 3例均为老年男性患者,初诊为AITL,分别于治疗后的8、14及34个月继发AML。继发AML后,1例未治疗于10个月后死亡,1例积极治疗后于13个月后死亡,另1例失访。结论 AITL有继发AML的风险,其发病机制目前尚不清楚,预后较差。     

皮下脂膜炎样T细胞淋巴瘤的临床特征及预后分析

目的 探讨皮下脂膜炎样T细胞淋巴瘤（SPTCL）的临床表现、病理特点、治疗方案及预后。方法 回顾性分析经病理证实的5例SPTCL患者的临床病理特征、诊断治疗及预后。结果 5例患者病理都存在皮下脂肪组织内中等偏大的非典型性淋巴细胞浸润,肿瘤细胞表达细胞毒T细胞的免疫表型。5例患者均有无痛性皮下结节的临床表现,2例伴发热,1例侵犯骨髓。5例患者均为晚期（Ⅲ~Ⅳ期）;4例患者一般状况好（ECOG仅为1分）;2例LDH升高,3例β2-MG升高。3例初治方案为CHOP,1例为BACOP,1例为BEACOP,随访至今1例死亡,1例失访,其余3例至今完全缓解。中位生存时间44（3~100）月,中位首次疾病无进展生存期8（0~96）月。结论 SPTCL发病率极低,主要累及皮下脂肪组织,目前尚无统一治疗方案,晚期患者CHOP方案疗效欠佳,含依托泊苷的高强度化疗方案可能成为有效的治疗手段。     

Clinical features and treatment outcomes of advanced stage primary hepatic angiosarcoma

BackgroundPrimary hepatic angiosarcoma is a very rare malignancy with a poor prognosis. While surgical resection has been validated as curative choice, most cases are diagnosed too late for resection. Nonetheless, treatment protocols have not been established and also there are very few reports on the clinical features and treatment outcomes.Patients and methodsAmong 11 939 patients diagnosed with primary hepatic tumors from January 1985 to December 2007 at two centers, five patients were diagnosed with primary hepatic angiosarcoma. We analyzed patients’ demographics, tumor characteristics, treatment modality, and outcomes using imaging, serology, and pathology.ResultsAll five patients were diagnosed at advanced stage with distant metastases. The most common symptom was abdominal pain. The levels of the tumor markers were within the normal range and serological tests were negative for hepatitis B and C viruses. Two of four patients who received chemotherapy died <3 months after diagnosis, but the other two patients survived >6 months.ConclusionsA combination of chemotherapy resulted in an improved outcome for two of four patients, suggesting the potential usefulness of palliative chemotherapy to improve survival. This case study may aid in planning chemotherapy for patients with advanced hepatic angiosarcoma.     

30例细支气管肺泡癌的诊断和外科治疗

目的：探讨细支气管肺泡癌的临床诊断和外科治疗。方法：回顾性分析我科1993年5月-2007年10月收治的30例细支气管肺泡癌临床资料。结果：30例周围型肺内肿块术前均未明确诊断，切除率100％，术后中位生存76．8个月，其中术后化疗者79．6个月。结论：周围型细支气管肺泡癌临床上缺乏特异性表现，手术切除率高，术后化疗与否，生存期无显著差异。     

原始神经外胚层瘤16例临床分析

目的:分析原始神经外胚层瘤(primitive neuroectodermal tumors,PNET)的病理学特征、诊断及治疗。方法:收集16例PNET患者的临床资料,肿瘤位于颅内4例,纵隔4例,眶内2例,胸髓2例,下肢2例,颈部1例,腹腔1例。6例单纯手术切除,10例行术后放、化疗。结果:16例中14例死亡,中位生存期为22个月,6例单纯手术切除组及10例术后放、化疗组的中位生存期分别为13个月、28个月,2组相比差异有统计学意义(P=0.0194)。结论:PNET是一种高度恶性的神经上皮瘤,侵袭性强,手术加放疗加化疗是PNET治疗的最佳选择。