大剂量化疗联合自体移植治疗复发髓母细胞瘤1例并文献复习

目的:探讨大剂量化疗联合自体移植治疗复发髓母细胞瘤的疗效。方法:报告1例大剂量化疗联合自体移植成功治疗复发髓母细胞瘤的经验,并结合文献复习进行分析。结果:患者使用大剂量化疗联合自体移植,术后35个月,疾病无进展、无复发。结论:大剂量化疗联合自体造血干细胞移植治疗复发髓母细胞瘤疗效优于常规化疗,值得进一步临床应用和推广。     

支气管髓细胞肉瘤一例并文献复习

 目的　提高对髓细胞肉瘤（MS）的起病方式、临床病理特点、诊断及治疗的认识。方法　结合文献对1例支气管MS患者的临床资料、病理特点、免疫表型及治疗进行分析。结果　肺MS是一种罕见肿瘤,本例为非白血病性MS,病变同时累及皮肤及支气管,临床表现为皮下结节、咳嗽及呼吸困难,CT示右肺门高密度阴影,肺不张,伴胸膜尾征。通过纤维支气管镜及皮肤活组织检查明确诊断MS,免疫组织化学MPO、CD43、LCA及CD99为阳性,而CD3、CD20及CD15为阴性。予以DA等方案化疗。结论　MS发病率低,起病多种多样,临床表现不典型,病理常规形态学观察误诊率高,结合免疫组织化学是确诊的主要依据。治疗主要按照急性髓细胞白血病的方案予以系统化疗,为达到长期无病生存,在化疗取得完全缓解后可尽早行干细胞移植。     

扁桃体髓细胞肉瘤1例并文献复习

髓细胞肉瘤(MS)是一种少见的髓细胞系恶性肿瘤的特殊表现形式,是由髓系起源的幼稚细胞组成的髓外局限性肿瘤[1]。可与急性或慢性髓细胞白血病、真性红细胞增多症和原发性骨髓纤维化等骨髓增殖性肿瘤(MPN)先后或同时发生,先于骨髓异常发生者称为非白血病性MS[2]。现报道我科收治的1例扁桃体MS,并结合文献探讨其起病方式、临床     

孤立性小肠髓系肉瘤1 例

患者男性,20岁,因“腹痛半个月、加重2 d”于2014年12月在宜昌市中心人民医院普外科行紧急剖腹探查术,术中见肿瘤位于回肠远端,大小约3 cm×4 cm,质硬,侵出浆膜,盆底大小约1.5 cm种植病灶。术后病理活检示：镜下见肠壁组织结构被破坏,代之以大量未成熟细胞弥漫性浸润,细胞胞质少,核圆形或卵圆形,染色质细致、分散,核仁小而清楚,核分裂像可见,部分细胞胞浆红染,核偏位;周围脂肪组织中见异型细胞浸润（图1A）;免疫组织化学示：CD20（-）,CD3（-）,Ki-67（LI约45%）,CD10（-）,Bcl-6（-）,Bcl-2（+）,Mum-1（散在+）, DOG-1（-）,CD117（+）,CD34（部分细胞+）,MPO（+）（图1B）;分子病理学示：EBER（-）,考虑髓系肉瘤。后转入宜昌市中心人民医院血液科继续行相关检查,骨髓细胞学示骨髓增生活跃,粒、红、巨三系增生无明显异常;骨髓免疫分型示粒细胞约占67.5%,表型较成熟,检测范围内未见免疫表型异常细胞;染色体核型（46,XY,+20）。临床诊断为孤立性小肠髓系肉瘤。于2015年1月14日给予标准DA方案化疗1个疗程。患者腹痛症状缓解,后继续给予DA、MA方案给予巩固治疗4次,随访1年,多次复查患者外周血及骨髓细胞学检查均正常。     

髓系肉瘤五例并文献复习

 目的　研究髓系肉瘤（MS）的临床病理特征、诊断及鉴别诊断方法。方法　分析5例MS的临床表现、组织学、免疫表型及随访资料,并复习相关文献。结果　患者发病年龄31 ～55岁,平均年龄41.8岁,男3例,女2例。组织学表现为相对一致的未成熟的髓系细胞弥漫分布,细胞圆形或类圆形,胞质少,弱嗜碱性,核圆形,可见小核仁。肿瘤细胞MPO、lysozyme、CD43、CD34及CD38阳性。结论　MS临床罕见,极易误诊为其他小圆细胞肿瘤,免疫组织化学、骨髓及外周血检查对确诊MS 有重要价值。     

含白消安注射液的预处理方案治疗急性髓细胞白血病二例并文献复习

 目的 评价白消安注射液（ivBu）作为急性髓细胞白血病（AML）造血干细胞移植（HSCT）预处理的疗效和安全性。方法 2例AML患者采用ivBu联合环磷酰胺（Cy）进行预处理,进行HLA相合的同胞外周血干细胞移植,观察其疗效和安全性。结果 2例患者均成功植入,没有严重毒副反应、肝窦状隙阻塞综合征（SOS）、严重急性移植物抗宿主病（GVHD）发生。结论 ivBuCy2方案作为AML预处理方案安全有效。     

造血干细胞移植治疗原发性粒细胞肉瘤患者持续缓解一例并文献复习

目的 探讨原发性粒细胞肉瘤(GS)的诊断、治疗、疗效.方法 回顾性分析1例原发性GS患者的临床资料.患者经诱导化疗+非血缘异基因造血干细胞移植(allo-HSCT)治疗.诱导化疗方案:DA方案2个周期,EMA方案1个周期.allo-HSCT供者为HLA全相合的非血缘供者,预处理方案为美法仑+环磷酰胺+抗人T淋巴细胞免疫球蛋白(BuCy+ATG)方案.结果 allo-HSCT后持续缓解,至今已达80个月,无移植物抗宿主病(GVHD)发生,无GS体征,血象、骨髓象均正常,生命质量好.结论 早期化疗联合allo-HSCT治疗可使原发性GS患者得到长期持续缓解.     

急性髓系白血病合并髓系肉瘤三例并文献复习

目的 分析急性髓系白血病(AML)合并髓系肉瘤(MS)的诊断和治疗方法.方法 收集3例在上海市北站医院按照常规方案诱导和巩固治疗并在骨髓缓解期发生MS的AML患者临床资料,分析其临床特点、诊断及治疗.结果 3例患者均为女性,其中2例CBFβ-MYH11阳性,1例AML1-ETO阳性;2例发生在第2次完全缓解(CR2),1例发生在CR1;3例分别发生于颅内、阴道、乳房.化疗后3例患者均短期有效,其中1例至截稿时骨髓仍处于缓解期,1例骨髓复发后死亡,1例失访.结论 AML缓解期合并髓外病灶时,应及时通过病理检查明确诊断.系统性化疗是MS的主要治疗方法,对于中枢神经系统受累者,可参照中枢神经系统白血病的治疗方式.异基因造血干细胞移植可能是患者的最佳选择.     

透明细胞肉瘤1例并文献复习

男,32岁。1982年6月发现左手腕黄豆大无痛肿物,3年后外伤不愈,它院活检为恶性黑色素瘤。体检:左手腕尺侧肿物3×2. 5cm,质硬有渗液,肘淋巴结不大,腋下可及直径0.5cm淋巴结2枚。1986年1月行肿块切除。病理检查:肿块3×3cm,有包膜,切面灰白色。镜下,纤维组织将瘤细胞分隔包绕,可见腺样结构和裂隙。细胞呈梭形,胞浆丰富透明,少数含徐色颗粒。核大,卵圆形,核膜清晰,核仁明显。PAS ,黑色素染色阳性。病理诊断:透明细胞肉瘤。2年后,病灶3.5×3cm,腋下2枚淋巴结增至直径0.8cm,1988年5月行肿块切除加腋淋巴结清扫,病理诊断同前。8月胸片发现左上肺直径4cm块影,     

Clinical implications of acute myeloid leukemia presenting as myeloid sarcoma

In this retrospective study, we aim to analyze the characteristics, treatments, and overall survival of all patients presenting with isolated myeloid sarcoma (MS) or MS with concomitant acute myeloid leukemia (AML) compared with all patients with AML, treated during the same period. We identified patients with AML with or without MS at diagnosis, presenting to our medical center between the years 1990 and 2005. There was no statistically significant difference between the groups regarding gender, age, cytogenetic risk groups, rate of complete remission, number of cycles of chemotherapy needed to achieve complete remission, and rate of first relapse. The time to death in the MS group was not significantly different (p = 0.60) from the AML group, and radiotherapy did not affect the median time to death. Transplantation prolonged survival in both groups (p = 0.018 and p < 0.0001, respectively). Patients with MS at diagnosis might benefit from upfront aggressive treatment with hematopoietic stem cell transplantation. Copyright © 2011 John Wiley & Sons, Ltd.     

Acute myeloid leukaemia presenting with mediastinal myeloid sarcoma: report of three cases and review of literature

Although myeloid sarcomas (MS) are frequently associated with acute myeloid leukaemia (AML), the occurrence of mediastinal MS is a much rarer event. The authors describe a distinct group of three AML patients with mediastinal MS and complex cytogenetics presenting at their centre over a 7-year period. Clinical features consistent with superior vena caval obstruction were noted at presentation in two of the three patients. Mediastinal mass was detected on routine chest radiography, and biopsies confirmed the diagnosis of MS. One patient relapsed after consolidation chemotherapy and died from progressive disease. Two patients underwent allogeneic haemopoietic stem cell transplant, but succumbed to transplant related complications. Review of mediastinal MS over the last 20 years shows that a significant proportion of patients have complex cytogenetic abnormalities and a poor long-term prognosis. Early and accurate diagnosis is essential and patients should be managed along the lines of high risk AML.     

双侧乳腺孤立性粒细胞肉瘤1例报告并文献复习

目的:讨论乳腺孤立性粒细胞肉瘤的临床及病理特征。方法:回顾性分析被确诊为双侧乳腺孤立性粒细胞肉瘤患者的临床资料、组织形态学、骨髓常规及免疫表型特征，并复习相关文献。结果:1例在发病前无白血病病史的62 岁女性患者体检发现双侧乳腺无痛性肿块。组织病理学可见肿瘤细胞呈列兵样排列或弥漫性分布，肿瘤细胞形态及大小一致，呈圆形或卵圆形，细胞质少，细胞核呈圆形或卵圆形，核分裂象及核仁易见，可见未成熟嗜酸性粒细胞散在分布。免疫组化提示MPO、CD45、CD34、CD68、CD117和CD43呈阳性，CD20、CD3、CD30、CD10和CKpan呈阴性。结论:双侧乳腺孤立性粒细胞肉瘤罕见。由于缺乏特异性临床特征，通常被误诊为淋巴瘤或其它良性肿瘤。肿瘤局部切除、系统化疗及放疗是其主要治疗手段。     

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.     

原发鼻咽部髓系肉瘤缓解后皮肤复发一例并文献复习

目的:提高对原发性髓系肉瘤的认识。方法:回顾性分析安徽医科大学第二附属医院2017年6月诊治的1例首发症状累及鼻咽部的原发性髓系肉瘤患者的临床资料,并复习相关文献。结果:该患者以鼻塞伴耳鸣就诊,接受多次活组织检查及免疫组织化学检查,历经约半年时间确诊髓系肉瘤。采用急性髓系白血病（AML）化疗方案治疗,达到缓解后2年余以新发皮肤包块形式复发,进一步完善骨髓相关检查提示骨髓未受累,仍为孤立性髓系肉瘤,再次行AML化疗方案诱导化疗后皮肤包块完全消失。患者复发后6个月,病情平稳,仍处于随访治疗中。结论:原发鼻咽部髓系肉瘤缺乏特异性,容易被误诊;孤立性髓系肉瘤预后优于AML,诱导化疗缓解后孤立性复发少见,通常较快进展至白血病期;早期和正确诊断对预后十分重要,临床医生应提高对原发性髓系肉瘤的认识。     

Dose-intensive chemotherapy with growth factor or autologous bone marrow/stem cell transplant support in first-line treatment of advanced or metastatic adult soft tissue sarcoma: a systematic review

A systematic review was performed to determine whether first-line dose-intensive chemotherapy supported by growth factor or autologous bone marrow/stem cell transplantation improves response rate, time-to-disease progression, or survival compared with standard-dose chemotherapy in patients with inoperable, locally advanced, or metastatic soft tissue sarcoma. The MEDLINE, EMBASE, and Cochrane Library databases were searched. Three randomized trials (2 phase 3, 1 phase 2), 12 phase 2, and 5 phase 1 dose-escalation trials were located. One randomized trial (N=314) did not detect significant differences in response rate (P=.65) or survival (log-rank P=.98) between high-dose doxorubicin plus ifosfamide with granulocyte macrophage colony-stimulating factor and doxorubicin plus ifosfamide at standard doses. Progression-free survival, however, was significantly longer in the high-dose arm (log-rank P=.03). Higher rates of thrombocytopenia, infection, grade 3 of 4 asthenia, and stomatitis were observed with high-dose compared with standard-dose chemotherapy. Preliminary results from a second randomized trial (N=162) indicated no benefit with respect to tumor response for an intensified mesna, doxorubicin (Adriamycin), ifosfamide, and dacarbazine regimen with granulocyte colony-stimulating factor support compared with standard doxorubicin, ifosfamide, and dacarbazine. Grade 4 thrombocytopenia was significantly higher with the high-dose regimen. Four phase 2 trials of high-dose regimens observed tumor response rates greater than 50%. Phase 1 trials reported dose-limiting toxicity for dose-intensive chemotherapy regimens. On the basis of the available evidence, high-dose chemotherapy with growth factor or autologous bone marrow/stem cell transplantation should not be used in the routine treatment of patients with inoperable, locally advanced, or metastatic soft tissue sarcoma.     

Rare myeloid sarcoma/acute myeloid leukemia with adrenal mass after allogeneic mobilization peripheral blood stem cell transplantation

Myeloid sarcoma （MS） is a rare hematological neoplasm that develops either de novo or concurrently with acute myeloid leukemia （AML）. This neoplasm can also be an initial manifestation of relapse in a previously treated AML that is in remission. A 44-year-old male patient was diagnosed with testis MS in a local hospital in August 2010. After one month, bone marrow biopsy and aspiration confirmed the diagnosis ofAML. Allogeneic mobilization peripheral blood stem cell transplantation was performed, with the sister of the patient as donor, after complete remission （CR） was achieved by chemotherapy. Five months after treatment, an adrenal mass was detected by positron emission tomography-computed tomography （PET-CT）. Radiotherapy was performed for the localized mass after a multidisciplinary team （MDT） discussion. The patient is still alive as of May 2013, with no evidence of recurrent MS or leukemia.     

Myeloid sarcoma is associated with superior event-free survival and overall survival compared with acute myeloid leukemia

BACKGROUND.: It is unknown whether patients with nonleukemic myeloid sarcoma (MS) and those with acute myeloid leukemia (AML) have similar responses to anti-AML treatment. In the current study, the authors addressed this question by matching MS patients with analogous AML patients and comparing their clinical outcomes. METHODS.: Twenty-three consecutive patients with MS and 1720 consecutive patients with AML were identified who presented at The University of Texas M. D. Anderson Cancer Center from 1990 to 2004. All AML patients and 16 MS patients received cytarabine plus idarubicin or fludarabine as induction remission therapy. Treated MS patients and AML patients were matched according to cytogenetics, age, Zubrod performance status, and time of treatment. Event-free survival (EFS) and overall survival (OS) were compared using Kaplan-Meier analyses. RESULTS.: Complete response rates were 69% in patients with MS and 57% in patients with AML (P = .45). The respective 2-year EFS and OS rates were 32% and 18% (P = .08) and 43% and 29% (P = .11). Matches were identified for 14 MS patients who were paired repeatedly with 91 AML patients to produce 94 matches (3 AML patients were matched twice). EFS was longer in 56 MS pair mates, shorter in 26 pair mates, and similar in 12 pair mates (P = .01; Fisher exact test). OS analyses yielded similar results. CONCLUSIONS.: Anti-AML therapy was highly effective in patients with nonleukemic MS. The results from this study emphasize the need to treat patients who have nonleukemic MS with AML-type therapy. Cancer 2008. (c) 2008 American Cancer Society.