伴幼淋巴细胞增多的慢性淋巴细胞白血病三例并文献复习

 【摘要】　目的　探讨伴幼淋巴细胞增多的慢性淋巴细胞白血病（CLL／PL）的临床特征与预后,为临床诊治提供依据。方法　回顾性分析3例CLL／PL患者的临床资料并复习相关文献,讨论其临床特征及预后。结果　CLL／PL在临床表现、免疫表型、细胞遗传学、分子生物学等方面具有特殊性。结论　CLL伴幼淋巴细胞增多与其临床特征密切相关,提示预后不良。     

RFC方案治疗B细胞幼淋巴细胞白血病一例并文献复习

 【摘要】　目的　探讨B细胞幼淋巴细胞白血病（B-PLL）临床特征。方法　报道1例33岁男性B-PLL患者的临床表现和治疗经过。结果　患者血小板减少、淋巴细胞增高,有全身B症状、伴随脾大和淋巴结肿大。患者拒绝异基因造血干细胞移植,予氟达拉滨、环磷酰胺联合利妥昔单抗组成的RFC方案化疗后,患者达到血液、骨髓、免疫表型完全缓解。结论　RFC方案可能是B-PLL合适且有效的初始治疗选择之一。     

B细胞幼淋巴细胞白血病七例免疫表型分析

目的 探讨B细胞幼淋巴细胞白血病(B-PLL)免疫表型特征.方法 通过多参数流式细胞术检测并回顾性分析7例B-PLL患者免疫表型,包括CD5、CD10、CD19、CD20、CD22、CD23、CD25、CD27、CD38、CD45、CD148、CD200、ZAP-70、FMC-7、κ、λ.结果 所有患者泛B细胞标志CD19、CD20、CD22均为阳性,其中仅有1例CD20呈中等强度表达,其余6例CD20均呈强阳性表达,CD22仅1例表达较弱,其余6例均呈中等强度表达.所有患者CD10均为阴性,4例CD5阳性,6例CD23阳性,2例FMC-7阳性,1例CD38阳性,4例CD200阳性,CD148均为阳性,轻链限制性表达检测中3例κ型,4例λ型.根据Matutes慢性淋巴细胞白血病免疫表型积分系统评分,1分1例,2、3分各3例.结论 B-PLL的诊断需要整合临床表现、外周血细胞形态学、免疫表型、细胞遗传学、分子生物学等结果综合判断,CD148、CD200或许可以成为B-PLL辅助诊断标志,B-PLL的免疫表型分布特征仍需要增加样本量进一步探讨.     

慢性淋巴细胞白血病/小B细胞淋巴瘤继发急性髓系白血病一例并文献复习

目的 提高对慢性淋巴细胞白血病/小B细胞淋巴瘤(CLL/SLL)继发髓系肿瘤的认识.方法 报道1例CLL/SLL继发急性髓系白血病患者,并对该病的发生率、危险因素及可能的机制进行文献复习.结果 CLL/SLL继发髓系肿瘤患者大多数伴有染色体异常,对接受以氟达拉滨为基础联合化疗的患者出现造血不良特征或血细胞减少应加强评估.结论 CLL/SLL继发髓系肿瘤少见,预后差,临床医师对具有危险因素的患者应提高警惕,争取早诊断、早干预.     

大颗粒淋巴细胞白血病二例并文献复习

 目的　提高对大颗粒淋巴细胞白血病的认识。方法　报道2例大颗粒淋巴细胞白血病诊断及治疗经过,并进行文献复习。结果　例1表达CD3／TCR复合体和TCR基因重排,表现为CD+3 CD+8 CD+16 CD+57 CD-56,起病缓慢,病情较稳定;例2患者不表达CD3／TCR复合体或TCR基因重排,表现为CD-3 CD+16 CD+56,病情进展迅速,病程数月即死亡。结论　细胞形态学、免疫表型、TCR基因重排等检查有助于大颗粒淋巴细胞白血病的诊断。     

B细胞淋巴瘤合并乳糜胸1例并文献复习

非霍奇金淋巴瘤(non-Hodgkin lymphoma,NHL)和霍奇金淋巴瘤(Hodgkin lymphoma,HL)均是来源于淋巴结和淋巴组织的恶性肿瘤,原发于淋巴结或淋巴结外的器官和组织,如扁桃体、鼻咽部、胃肠道、脾、骨骼或皮肤等.根据细胞起源的不同可以将NHL分为B细胞淋巴瘤、T细胞淋巴瘤、自然杀伤(natu...     

结节性淋巴细胞为主型霍奇金淋巴瘤向富含T细胞/组织细胞的大B细胞淋巴瘤样转化一例并文献复习

目的:探讨结节性淋巴细胞为主型霍奇金淋巴瘤（NLPHL）经治疗后复发并向富含T细胞/组织细胞的大B细胞淋巴瘤（THRLBCL）样转化患者的临床特点,提高对NLPHL和THRLBC鉴别诊断与治疗的认识。方法:回顾性分析复旦大学附属华东医院2019年10月收治的1例NLPHL治疗后复发并向THRLBCL样转化患者的治疗经过,并进行相关文献复习。结果:该患者经ABVD方案治疗后复发,再次经病理活组织及免疫组织化学检查诊断为THRLBCL样转化,经R-CHOP方案治疗后完全缓解。结论:NLPHL化疗效果不佳时需考虑是否存在向THRLBCL样转化的可能,需及时行活组织病理检查,调整治疗方案。     

慢性淋巴细胞白血病幼淋变一例

患者男,59岁。确诊慢性淋巴细胞白血病(CLL)4年,本次因“反复发热、干咳2个月”于2004年8月10日收住院。患者于2000年3月发现血象异常WBC37.3×109/L,Hb92g/L,Plt121×109/L,超声检查提示脾大,盆腹腔多发肿大淋巴结;骨髓象示:CLL。予皮下注射α干扰素300万U,1/2d～1/3d,治疗持续4年,其中2001年至2002年辅以中药治疗。病情控制平稳,未再复查骨髓象。2004年5月以来无明显诱因出现发热,体温38℃左右,伴干咳,抗感染治疗可有好转,但上述症状反复发作。既往史:2002年患肺结核,抗结核治疗半年后痊愈。个人史及家族史无特殊。入院查体:体温37…     

伴复杂核型的慢性髓系白血病急髓变合并T淋巴细胞淋巴瘤/白血病1例并文献复习北大核心CSCD

慢性髓细胞白血病（chronic myelogenous leukemia,CML）是一种常见的发生于多能造血干细胞的骨髓增生性疾病,95％以上的患者存在t（9：22）染色体移位并形成一个小的22号染色体,称为Ph染色体,与之对应的基因为bcr-abl融合基因,是CML的细胞遗传学标志。     

Acute liver failure because of chronic lymphocytic leukemia: case report and review of the literature

Infiltration of the liver by hematologic malignancies is an uncommon cause of liver failure. B-Cell chronic lymphocytic leukemia (cll) is a usually indolent disease that may infiltrate the liver, but based on a review of the literature, has never been reported to induce acute liver failure. Here, we describe the case of a 78-year-old woman with acute liver failure secondary to infiltration with cll being unresponsive to chemotherapy and causing death. This case is notable because of its atypical presentation and ultimate poor prognosis.     

急性髓系白血病合并慢性淋巴细胞白血病一例报道及文献复习

 目的 探讨急性髓系白血病（AML）合并慢性淋巴细胞白血病（CLL）的临床特点、病因、诊断、治疗及预后。方法 临床诊断1例AML合并CLL,并就相关文献进行复习。结果 患者经MA方案（米托蒽醌10 mg／d第1 ~ 3天,阿糖胞苷150 mg／d第1,3,5,7天,200 mg第2,4,6天）化疗后取得完全缓解,但CD19阳性的淋巴细胞（表达CD20,CD23,SIgM,部分表达CD5,CD22,CD25）仍然存在,于9个月后AML复发未能再次缓解而死亡。结论 AML合并CLL为一种具有特殊生物学特征的罕见疾病,免疫分型和细胞遗传学技术在疾病的诊断和认识中发挥重要作用,治疗应以AML为主。     

Acute lymphocytic leukemia with eosinophilia: two case reports and a literature review

Acute lymphocytic leukemia (ALL) associated with eosinophilia is a rare occurrence, but a distinct clinicopathological entity. There have been approximately 44 cases reported in the world literature to date. We report 2 previously healthy young men, aged 20 and 32 years, who presented with marked eosinophilia, and were later diagnosed with pre-B ALL. The patients suffered from significant complications related to eosinophil toxicity, including respiratory failure, myocardial infarction, and a cerebrovascular accident during initial hospitalization. They were treated with high-dose steroids resulting in a rapid suppression of the eosinophilia. Both patients also received chemotherapy according to the standard protocol for ALL; unfortunately, case 1 expired within 2 years of diagnosis from complications related to sepsis and multi-organ failure. We also review the literature and compare the demographics, clinical features, and outcomes of several case studies reported.     

Hybrid form of hairy cell leukemia and chronic lymphocytic leukemia

We report a case with mixed features of hairy cell leukemia (HCL) and chronic lymphocytic leukemia (CLL), which may represent a hybrid form of these two entities. Hairy projections were demonstrated on leukemic cells in the peripheral blood. Surface marker studies of blood and spleen specimens by flow cytometry and immunohistochemistry showed immunophenotype characteristic of HCL, namely, monoclonal IgG-kappa, positive reactions to CD 11c, CD 19, CD 20, Cd 22, and HLA-DR, but negative reactions to CD 3, CD 5, CD 7 and CD 10. The only atypical finding was the absence of CD 25. Immunogenotyping showed rearrangement of heavy-chain and kappa light chain genes. Leukemic cells were also positive for tartrate-resistant acid phosphatase (TRAP). A pseudosinus pattern was demonstrated in the spleen. However, the leukemic cells in the spleen showed atypical cytologic features. Clinically, the patient had generalized lymphadenopathy, high leukocyte counts, Coombs' negative hemolysis, hypoimmunoglobulinemia and IgG-kappa monoclonal gammopathy, features more consistent with CLL than HCL. Although only CD 11c, CD 22, CD 25 and TRAP are characteristic for HLC and CD 5, characteristic for CLL, a panel of eight markers is recommended for the differential diagnosis of HCL, CLL and other low-grade B-cell neoplasms, which may share some common features, making a clear-cut diagnosis difficult.     

以原发性脾淋巴瘤为表现的弥漫性大B细胞淋巴瘤1例并文献复习

目的:探讨原发性脾淋巴瘤(PSL)的诊断以及原发性脾弥漫性大B细胞淋巴瘤的治疗。方法:报告1例以PSL为表现的弥漫性大B细胞淋巴瘤并结合文献进行复习。结果:患者表现为左上腹疼痛,无发热、盗汗,无浅表淋巴结肿大,CT证实脾脏内有占位性病变,脾脏病理示弥漫性大B细胞淋巴瘤,外周血和骨髓检查正常,脾切除术后PET/CT未见明显与淋巴瘤相关的18F-脱氧葡萄糖摄取异常增高表现,后给予联合化疗,随访8月余仍处于完全缓解期。结论:PSL是一少见肿瘤,采取脾切除后继以CHOP±利妥昔单抗治疗原发性脾弥漫性大B细胞淋巴瘤能够达到长期生存的目的。     

Fluorescence in situ hybridization detection of cytogenetic abnormalities in B-cell chronic lymphocytic leukemia/small lymphocytic lymphoma

Routine cytogenetic analysis of B-cell chronic lymphocytic leukemia/small lymphocytic lymphoma (B-CLL/SLL) frequently fails to identify an abnormal clone due to the low rate of spontaneous mitoses and poor response to mitogen stimulation. Recent studies utilizing interphase fluorescence in situ hybridization (FISH) suggest that prognostically significant chromosomal abnormalities occur more frequently in B-CLL/SLL than has been previously recognized. The purpose of this study was to compare the chromosomal abnormalities detected by karyotyping and FISH in cases of B-CLL/SLL, and to correlate these with clinical features and survival. Seventy-two cases were studied for chromosome 3, 12 or 18 aneuploidy, and for rearrangements involving 11q13, 11q23, 13q14, 14q32 and 17p13. The median age of the patients was 54 years (range, 30 - 87 years). Clinical staging of B-CLL patients showed that 70% of the patients were Rai stage 0, 1, or 2, and 30% stage 3 or 4. Karyotyping identified chromosomal abnormalities in 31% of the cases, whereas FISH studies were abnormal in 72% of cases including 64% of the cases with normal karyotypes. The most common abnormalities were deletion 13q14 (46%), trisomy 12 (21%), and 14q32 rearrangements (21%). At diagnosis, patients with trisomy 12 were more likely to have a high LDH (P = 0.04), but no other significant differences in the clinical or laboratory features, Rai stage, or survival were found among patients with normal cytogenetics vs. those with chromosomal abnormalities. Univariate analysis showed that B-symptoms (P = 0.044), anemia (P = 0.0006), absolute lymphocytosis ≥ 30,000/mm³ (P = 0.029), and Rai stage 3 or 4 (P = 0.0038) at initial presentation were associated with an increased risk of death, but only Rai stage 3 or 4 (P = 0.0038) was significant in multivariate analysis. Interphase FISH studies improve the cytogenetic diagnosis when performed in conjunction with karyotyping in B-CLL/SLL, but the prognostic relevance of various abnormalities could not be confirmed in this study.     

WT1基因在儿童急性B淋巴细胞白血病中的表达及临床意义

目的:探讨WT1(Wilms tumor gene 1)基因在儿童急性B淋巴细胞白血病（B-ALL）中的表达及临床意义。方法:应用实时荧光定量PCR方法检测77例初诊B-ALL患儿WT1基因的相对表达水平,比较患者年龄、性别、流式亚型、染色体核型、临床危险分级等因素的基因表达差异,并随访分析初诊WT1基因表达水平对患儿预后的影响。结果:77例初诊B-ALL患者中,WT1基因阳性率96.10%（74/77）,0~2岁患儿初诊WT1水平高于3~13岁组（P=0.02）,WT1基因表达在性别上无统计学差异（P=0.229）。在对B-ALL流式亚型间WT1的分析中显示,Pro-B-ALL患者表达量最高,Pre-B-ALL次之,Com-B-ALL最低（两两比较差异,P值分别为0.002,0.008,0.040）。临床高危（P=0.041）、t(4;11）（P=0.034）、初诊白细胞大于50×109/L（P=0.009）的患者有更高的WT1表达。首次诱导化疗后细胞形态学缓解与否,其初诊时WT1表达无明显差异（P=0.84）,但长期随访得到持续缓解的患者初诊WT1表达低于复发或不缓解的患者。结论:WT1基因在儿童B-ALL中表达的高低可能提示患者白血病细胞的分化水平,并与长期预后相关。     

High incidence of relapse after autologous stem-cell transplantation for B-cell chronic lymphocytic leukemia or small lymphocytic lymphoma

Background: High-dose therapy followed by autologous stem-cell transplantation (autoSCT) induces complete remissions in the majority of patients with advanced B-cell chronic lymphocytic leukemia or small lymphocytic lymphoma (B-CLL). However, the long-term utility of this therapy for B-CLL is unknown.Patients and methods: Sixteen previously treated patients with B-CLL were transplanted using autologous blood (n = 13) or bone marrow (n = 3). The median age of the patients was 49 years (range 44–60 years), and the median number of prior chemotherapy regimens was two. Patients were eligible for transplantation if they had chemosensitive disease and no morphologic evidence of malignant cells in the graft. Preparative regimens included cyclophosphamide and total-body-irradiation, with or without cytarabine, or BEAC.Results: All patients engrafted and achieved a complete remission posttransplant. Ten patients were alive at a median of 41 months (range 22–125 months), and five were disease-free. Eight patients have relapsed and six have died (three from progressive malignancy). The projected three-year overall survival, failure-free survival and relapse rates were 68%, 37%, and 56%, respectively.Conclusions: AutoSCT for advanced B-CLL is associated with a high relapse rate. Whether this therapy can prolong life or produce cures is uncertain.