Pub Date : 2019-12-25DOI: 10.3760/CMA.J.ISSN.1009-9921.2019.12.011
Ting Xu, Weimin Wang, Wenjuan Fan, Jing-Miao Wang, G. Fu
Objective To explore the clinical and laboratory characteristics and therapeutic effect of acute promyelocytic leukemia (APL) with t(2;17;15). Methods The G-banding technique was used for karyotypic analysis in a female patient with APL who was admitted to the First Affiliated Hospital of Zhengzhou University in December 2018. PML-RARα fusion gene was quickly detected by fluorescence in situ hybridization (FISH). The real-time quantitative polymerase chain reaction (RT-PCR) was used to detection 43 kinds of fusion gene, and the gene mutations were detected by next generation sequencing (NGS). The induction therapy was given with oral retinoic acid+ intravenous infusion of arsenic trioxide, followed by 3 courses of retinoic acid+ arsenic trioxide consolidation therapy. Results The G-banding karyotypic analysis demonstrated 46, XX, t(2;17;15) (q31;q21;q22)[8]/46, XX[2]. FISH results indicated that 62.0% of analyzed cells were positive for the PML-RARα fusion gene. RT-PCR further revealed the positive PML-RARα fusion gene transcript. NGS detection of gene mutations showed no obvious abnormalities. After 39 days of induction therapy with retinoic acid and arsenic trioxide, the patient achieved complete remission (CR). The karyotype was 46XX[20], and PML-RARα/ABL was 0/100. Then, the patient was treated with 3 courses of consolidation therapy, and the results remained in CR. Conclusions APL with complex t(2;17;15) (q31;q21;q22) is rare, and the morphological characteristics are not typical, but it is still associated with the formation of PML-RARα fusion gene. Retinoic acid+ arsenic trioxide has a good therapeutic effect, and the long-term efficacy still needs follow-up. Key words: Leukemia, promyelocytic, acute; Translocation, genetic; Gene fusion; Tretinoin; Arsenites
{"title":"Acute promyelocytic leukemia with t(2;17;15): report of one case and review of literature","authors":"Ting Xu, Weimin Wang, Wenjuan Fan, Jing-Miao Wang, G. Fu","doi":"10.3760/CMA.J.ISSN.1009-9921.2019.12.011","DOIUrl":"https://doi.org/10.3760/CMA.J.ISSN.1009-9921.2019.12.011","url":null,"abstract":"Objective \u0000To explore the clinical and laboratory characteristics and therapeutic effect of acute promyelocytic leukemia (APL) with t(2;17;15). \u0000 \u0000 \u0000Methods \u0000The G-banding technique was used for karyotypic analysis in a female patient with APL who was admitted to the First Affiliated Hospital of Zhengzhou University in December 2018. PML-RARα fusion gene was quickly detected by fluorescence in situ hybridization (FISH). The real-time quantitative polymerase chain reaction (RT-PCR) was used to detection 43 kinds of fusion gene, and the gene mutations were detected by next generation sequencing (NGS). The induction therapy was given with oral retinoic acid+ intravenous infusion of arsenic trioxide, followed by 3 courses of retinoic acid+ arsenic trioxide consolidation therapy. \u0000 \u0000 \u0000Results \u0000The G-banding karyotypic analysis demonstrated 46, XX, t(2;17;15) (q31;q21;q22)[8]/46, XX[2]. FISH results indicated that 62.0% of analyzed cells were positive for the PML-RARα fusion gene. RT-PCR further revealed the positive PML-RARα fusion gene transcript. NGS detection of gene mutations showed no obvious abnormalities. After 39 days of induction therapy with retinoic acid and arsenic trioxide, the patient achieved complete remission (CR). The karyotype was 46XX[20], and PML-RARα/ABL was 0/100. Then, the patient was treated with 3 courses of consolidation therapy, and the results remained in CR. \u0000 \u0000 \u0000Conclusions \u0000APL with complex t(2;17;15) (q31;q21;q22) is rare, and the morphological characteristics are not typical, but it is still associated with the formation of PML-RARα fusion gene. Retinoic acid+ arsenic trioxide has a good therapeutic effect, and the long-term efficacy still needs follow-up. \u0000 \u0000 \u0000Key words: \u0000Leukemia, promyelocytic, acute; Translocation, genetic; Gene fusion; Tretinoin; Arsenites","PeriodicalId":16246,"journal":{"name":"Journal of Leukemia and Lymphoma","volume":"28 1","pages":"749-752"},"PeriodicalIF":0.0,"publicationDate":"2019-12-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"48483919","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2019-12-25DOI: 10.3760/CMA.J.ISSN.1009-9921.2019.12.004
Jiaqi Qin
Hodgkin lymphoma (HL) is a highly curable disease, and even in advanced-stage patients, more than 90% of them can achieve long-term survival. Due to its few adverse reactions and good curative effect, the ABVD regimen is currently used as the standard first-line chemotherapy for HL. In order to further improve the efficacy, the new first-line combination therapies and salvage therapeutic regimens for relapsed/refractory HL are continuously updated, such as anti-CD30 monoclonal antibody brentuximab vedotin combined with immune checkpoint inhibitors or chemotherapy, programmed death receptor 1 with chemotherapy or chimeric antigen receptor T-cells are gradually showing their advantages. This article summarizes the treatment progress of HL at the 61st American Society of Hematology Annual Meeting 2019. Key words: Lymphoma, Hodgkin; Drug therapy, combination; Molecular targeted therapy
{"title":"Treatment progress of Hodgkin lymphoma","authors":"Jiaqi Qin","doi":"10.3760/CMA.J.ISSN.1009-9921.2019.12.004","DOIUrl":"https://doi.org/10.3760/CMA.J.ISSN.1009-9921.2019.12.004","url":null,"abstract":"Hodgkin lymphoma (HL) is a highly curable disease, and even in advanced-stage patients, more than 90% of them can achieve long-term survival. Due to its few adverse reactions and good curative effect, the ABVD regimen is currently used as the standard first-line chemotherapy for HL. In order to further improve the efficacy, the new first-line combination therapies and salvage therapeutic regimens for relapsed/refractory HL are continuously updated, such as anti-CD30 monoclonal antibody brentuximab vedotin combined with immune checkpoint inhibitors or chemotherapy, programmed death receptor 1 with chemotherapy or chimeric antigen receptor T-cells are gradually showing their advantages. This article summarizes the treatment progress of HL at the 61st American Society of Hematology Annual Meeting 2019. \u0000 \u0000 \u0000Key words: \u0000Lymphoma, Hodgkin; Drug therapy, combination; Molecular targeted therapy","PeriodicalId":16246,"journal":{"name":"Journal of Leukemia and Lymphoma","volume":"28 1","pages":"716-718"},"PeriodicalIF":0.0,"publicationDate":"2019-12-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49624216","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Objective: To explore the potential mechanism of acute promyelocytic leukemia (APL) transforming into other acute myeloid leukemia (AML) after treatment. Method: A retrospective analysis was conducted on the diagnosis, continuous remission, and recurrence treatment process of one APL patient admitted to the Affiliated Tumor Hospital of Zhengzhou University, and relevant literature was reviewed. The initial diagnosis of the patient was in accordance with APL, and after receiving standard induction and consolidation therapy, maintenance therapy with retinoic acid and arsenious acid was used. Complete relief lasts for 3 years and 4 months. Subsequently, there was an increase in bone marrow primordial granulocytes and promyelocytes, with PML-RAR α Negative, WT1 gene positive, and chromosome test shows 47, XX,+? 8 [1]/46, XX [1]; Relieve after re induction with dexcitabine+CAG regimen. After 6 months, the second recurrence occurred, with 0.874% of primordial granulocytes. It is recommended that the patient undergo sibling bone marrow matching and give up treatment. Conclusion: The conversion of APL to other AML after treatment is a special form of recurrence, and the treatment effect is poor. The reason may be related to the expansion of secondary clones in leukemia or drug-induced clone changes.
{"title":"Acute myeloid leukemia M2 transformation in acute promyelocytic leukemia after treatment: report of one case and review of literature","authors":"B. Ding, Liu Liu, H. Zhao, X. Shu, Xiaoran Wang, Hu Zhou, Xinjian Liu, Yongping Song, Zhen-hua Guo","doi":"10.3760/CMA.J.ISSN.1009-9921.2019.12.016","DOIUrl":"https://doi.org/10.3760/CMA.J.ISSN.1009-9921.2019.12.016","url":null,"abstract":"目的 \u0000探讨急性早幼粒细胞白血病（APL）治疗后转化为其他急性髓系白血病（AML）的潜在机制。 \u0000 \u0000 \u0000方法 \u0000回顾性分析郑州大学附属肿瘤医院收治的1例APL患者的诊断、连续缓解和复发的治疗过程，并复习相关文献。 \u0000 \u0000 \u0000结果 \u0000患者初始诊断符合APL，给予标准方案诱导缓解、巩固治疗后，以维甲酸+亚砷酸维持治疗。完全缓解持续3年4个月。后出现骨髓原始粒细胞和早幼粒细胞增多，PML-RARα阴性，WT1基因阳性，且染色体检查显示47，XX，+？8[1]/46，XX[1]；给予地西他滨+CAG方案再诱导后缓解。6个月后第二次复发，原始粒细胞占0.874，建议患者行同胞骨髓配型，患者放弃治疗。 \u0000 \u0000 \u0000结论 \u0000APL治疗后转化为其他AML为特殊复发形式，治疗效果差。其原因可能与白血病次级克隆扩增或药物诱导的克隆改变有关。","PeriodicalId":16246,"journal":{"name":"Journal of Leukemia and Lymphoma","volume":"28 1","pages":"766-768"},"PeriodicalIF":0.0,"publicationDate":"2019-12-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"45735189","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Objective To explore the diagnosis and treatment of T-lymphoblastic lymphoma (T-LBL) with Philadelphia chromosome positive acute myeloid leukemia (Ph+AML). Method: A retrospective analysis was conducted on the clinical manifestations, genetic examination, histopathological results, treatment process, and literature review of a patient with T-LBL and Ph+AML admitted to Jingjiang People's Hospital. The patient was currently a rare reported adult T-LBL with Ph+AML, and early pathological section fluorescence in situ hybridization (FISH) detection showed positive BCR-ABL fusion gene. After the diagnosis of T-LBL, the patient received chemotherapy with lymphoma regimen, which was effective. However, in the third course of bone marrow recovery, Ph+AML was secondary, and hydroxyurea was taken orally and white blood cells were collected separately. However, the disease progressed rapidly and the patient died in the short term. Conclusion: T-LBL with Ph+AML is very rare, and the mechanism of lineage transition is currently unclear. It can manifest as consecutive onset from the same source.
{"title":"T-lymphoblastic lymphoma complicated with Philadelphia chromosome-positive acute myeloid leukemia: report of one case and review of literature","authors":"Hao-yue Chen, Miao Sun, Chunhua Liu, Qiaoyan Han, Lu Chen","doi":"10.3760/CMA.J.ISSN.1009-9921.2019.12.013","DOIUrl":"https://doi.org/10.3760/CMA.J.ISSN.1009-9921.2019.12.013","url":null,"abstract":"目的 \u0000探讨T淋巴母细胞淋巴瘤（T-LBL）伴费城染色体阳性急性髓系白血病（Ph+ AML）的诊治方法。 \u0000 \u0000 \u0000方法 \u0000回顾性分析靖江市人民医院收治的1例T-LBL伴Ph+ AML患者的临床表现、遗传学检查、组织病理结果、治疗经过，并进行文献复习。 \u0000 \u0000 \u0000结果 \u0000该患者为目前鲜见报道的成年人T-LBL伴Ph+ AML，初期病理切片荧光原位杂交（FISH）检测提示BCR-ABL融合基因阳性。患者T-LBL确诊后予以淋巴瘤方案化疗有效，但在第3个疗程骨髓恢复期继发Ph+ AML，予以羟基脲口服及白细胞单独采集，但疾病进展迅速，患者短期内死亡。 \u0000 \u0000 \u0000结论 \u0000T-LBL伴Ph+ AML非常罕见，谱系转换的机制目前尚不清楚。可表现为相继发病，来源相同。","PeriodicalId":16246,"journal":{"name":"Journal of Leukemia and Lymphoma","volume":"28 1","pages":"758-760"},"PeriodicalIF":0.0,"publicationDate":"2019-12-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"42594053","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2019-12-25DOI: 10.3760/CMA.J.ISSN.1009-9921.2019.12.002
Qingpei Guan, Tingting Zhang, Xianhuo Wang
The immune checkpoint inhibitors (ICI) represented by programmed death 1/programmed death ligand 1 (PD-1/PD-L1) antibody opened a new era of immunotherapy. However, PD-1/PD-L1 inhibitors have not been approved for indications in the field of malignant lymphoma except for classic Hodgkin lymphoma (cHL) and primary mediastinal large B-cell lymphoma. Researchers have actively explored different lymphoma subtypes with single drugs or combined therapy, and achieved certain effect initially. The latest advances of ICI in cHL, B-cell non-Hodgkin lymphoma and T-cell lymphoma and the management of immune-related adverse events are briefly introduced in this paper. Key words: Lymphoma; Immune checkpoints; Immunotherapy; Programned death 1; Programmed death-ligand 1
{"title":"Progress of immune checkpoint inhibitors in treatment of malignant lymphoma","authors":"Qingpei Guan, Tingting Zhang, Xianhuo Wang","doi":"10.3760/CMA.J.ISSN.1009-9921.2019.12.002","DOIUrl":"https://doi.org/10.3760/CMA.J.ISSN.1009-9921.2019.12.002","url":null,"abstract":"The immune checkpoint inhibitors (ICI) represented by programmed death 1/programmed death ligand 1 (PD-1/PD-L1) antibody opened a new era of immunotherapy. However, PD-1/PD-L1 inhibitors have not been approved for indications in the field of malignant lymphoma except for classic Hodgkin lymphoma (cHL) and primary mediastinal large B-cell lymphoma. Researchers have actively explored different lymphoma subtypes with single drugs or combined therapy, and achieved certain effect initially. The latest advances of ICI in cHL, B-cell non-Hodgkin lymphoma and T-cell lymphoma and the management of immune-related adverse events are briefly introduced in this paper. \u0000 \u0000 \u0000Key words: \u0000Lymphoma; Immune checkpoints; Immunotherapy; Programned death 1; Programmed death-ligand 1","PeriodicalId":16246,"journal":{"name":"Journal of Leukemia and Lymphoma","volume":"28 1","pages":"709-712"},"PeriodicalIF":0.0,"publicationDate":"2019-12-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"47866727","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2019-12-25DOI: 10.3760/CMA.J.ISSN.1009-9921.2019.12.003
Bing Xu
Follicular lymphoma (FL) is the most common indolent lymphoma. With the improvement of disease awareness and the introduction of various novel drugs, the 5-year overall survival rate has been more than 90% for the majority of FL patients. However, certain subgroups of FL patients, especially patients who progressed within 24 months after starting front-line therapy, still have worse outcome. This article reviews the progress in genetics, prognostic factors and treatment options of FL that reported at the 61st American Society of Hematology Annual Meeting 2019. Key words: Lymphoma, follicular; Genetics; Prognosis; Molecular targeted therapy
{"title":"Diagnosis and treatment progress of follicular lymphoma","authors":"Bing Xu","doi":"10.3760/CMA.J.ISSN.1009-9921.2019.12.003","DOIUrl":"https://doi.org/10.3760/CMA.J.ISSN.1009-9921.2019.12.003","url":null,"abstract":"Follicular lymphoma (FL) is the most common indolent lymphoma. With the improvement of disease awareness and the introduction of various novel drugs, the 5-year overall survival rate has been more than 90% for the majority of FL patients. However, certain subgroups of FL patients, especially patients who progressed within 24 months after starting front-line therapy, still have worse outcome. This article reviews the progress in genetics, prognostic factors and treatment options of FL that reported at the 61st American Society of Hematology Annual Meeting 2019. \u0000 \u0000 \u0000Key words: \u0000Lymphoma, follicular; Genetics; Prognosis; Molecular targeted therapy","PeriodicalId":16246,"journal":{"name":"Journal of Leukemia and Lymphoma","volume":"28 1","pages":"713-715"},"PeriodicalIF":0.0,"publicationDate":"2019-12-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"44977124","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2019-12-25DOI: 10.3760/CMA.J.ISSN.1009-9921.2019.12.005
Weiting Wang
Diffuse large B-cell lymphoma (DLBCL) is the most common subtype of non-Hodgkin lymphoma (NHL). After the treatment of standard chemotherapy R-CHOP regimen, approximately 40% of DLBCL patients fail to respond to treatment or show progress of the disease. At the 61st American Society of Hematology Annual Meeting, multiple studies reported the latest treatment progress of DLBCL, including improving the prognosis of newly diagnosed patients and providing more treatment options for relapsed/refractory DLBCL patients. Key words: Lymphoma, large B-cell, diffuse; Recurrence; Refractory; Drug therapy, combination; Molecular targeted therapy; Immunotherapy
{"title":"Treatment progress of diffuse large B-cell lymphoma","authors":"Weiting Wang","doi":"10.3760/CMA.J.ISSN.1009-9921.2019.12.005","DOIUrl":"https://doi.org/10.3760/CMA.J.ISSN.1009-9921.2019.12.005","url":null,"abstract":"Diffuse large B-cell lymphoma (DLBCL) is the most common subtype of non-Hodgkin lymphoma (NHL). After the treatment of standard chemotherapy R-CHOP regimen, approximately 40% of DLBCL patients fail to respond to treatment or show progress of the disease. At the 61st American Society of Hematology Annual Meeting, multiple studies reported the latest treatment progress of DLBCL, including improving the prognosis of newly diagnosed patients and providing more treatment options for relapsed/refractory DLBCL patients. \u0000 \u0000 \u0000Key words: \u0000Lymphoma, large B-cell, diffuse; Recurrence; Refractory; Drug therapy, combination; Molecular targeted therapy; Immunotherapy","PeriodicalId":16246,"journal":{"name":"Journal of Leukemia and Lymphoma","volume":"28 1","pages":"719-723"},"PeriodicalIF":0.0,"publicationDate":"2019-12-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41339067","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Objective To investigate the current status of family hardiness and its influencing factors in children with leukemia. Methods The children with leukemia and their caregivers in the Children's Hospital of Shanxi from August to November 2017 were enrolled. A questionnaire survey was conducted using a convenient sampling method, and 100 questionnaires were distributed. The questionnaire included the General Status Questionnaire, the Family Hardiness Index (FHI), the Coping Health Inventory for Parents (CHIP) and the Positive and Negative Affect Scale (PANAS). Results A total of 92 valid questionnaires were collected. Among the scores of family hardiness in children with leukemia [(3.29±0.43) points], the responsibility score [(3.32±0.45) points] was higher than the control score [(3.31±0.46) points] and the challenge score [(3.23±0.53) points], and the challenge score was the lowest. The score of frequency of coping styles used by the caregiver of the child with leukemia was (3.64±0.70) points, the most frequent coping style used by the caregiver was "family unity, optimism, cooperative attitude" [(3.73±0.89) points], and the positive [(3.28±0.84) points] and negative [(2.51±0.80) points] emotions were in a moderate state. The child's sex, age, stage of chemotherapy and medical insurance status, the caregiver of the child, the age of the caregiver, the family's place of residence, and the education level were the related factors affecting the family hardiness score (all P < 0.01). The age of child, CHIP-1, CHIP-2, positive emotion and negative emotion were independent factors affecting the family hardiness (all P < 0.05). The CHIP, CHIP-1, CHIP-2 and positive emotion were positively correlated with the family hardiness (r values were 0.827, 0.883, 0.707 and 0.846, all P < 0.01); the negative emotion was negatively correlated with the family hardiness (r=-0.832, P < 0.01). Conclusion The family hardiness of children with leukemia is in the middle and upper level, the children's age, caregiver's coping style, positive emotion and negative emotion are factors affecting the family hardiness. Key words: Leukemia; Child; Family hardiness; Influencing factors; Psychotherapy
目的了解白血病患儿家庭适应力现状及其影响因素。方法选取2017年8月至11月山西省儿童医院白血病患儿及其护理人员为研究对象。采用方便抽样法进行问卷调查，共发放问卷100份。调查问卷包括一般状况问卷、家庭适应力指数(FHI)、父母应对健康量表(CHIP)和积极消极情绪量表(PANAS)。结果共回收有效问卷92份。在白血病患儿家庭适应力得分[(3.29±0.43)分]中，责任得分[(3.32±0.45)分]高于对照组得分[(3.31±0.46)分]和挑战得分[(3.23±0.53)分]，挑战得分最低。白血病患儿的照料者应对方式频次得分为(3.64±0.70)分，照料者最常使用的应对方式为“家庭团结、乐观、合作态度”[(3.73±0.89)分]，积极情绪[(3.28±0.84)分]和消极情绪[(2.51±0.80)分]处于中等状态。儿童的性别、年龄、化疗阶段及医疗保险状况、儿童的照顾者、照顾者的年龄、家庭居住地点、文化程度是影响家庭抗寒性评分的相关因素(均P < 0.01)。儿童年龄、CHIP-1、CHIP-2、积极情绪和消极情绪是影响家庭适应力的独立因素(均P < 0.05)。CHIP、CHIP-1、CHIP-2、积极情绪与家庭适应力呈正相关(r值分别为0.827、0.883、0.707、0.846,P均< 0.01);负性情绪与家庭适应力呈负相关(r=-0.832, P < 0.01)。结论白血病患儿家庭适应力处于中高水平，儿童年龄、照顾者应对方式、积极情绪和消极情绪是影响家庭适应力的因素。关键词:白血病;孩子;家庭耐寒性;影响因素;心理治疗
{"title":"Study on the family hardiness and its influencing factors in children with leukemia","authors":"Xiaohuan Wang, Lin Wang, Yanli Cheng, Guoping Hao, Haiyan Lu, Jing Wang, Taoli Suo, Yunzhen Xue","doi":"10.3760/CMA.J.ISSN.1009-9921.2019.12.012","DOIUrl":"https://doi.org/10.3760/CMA.J.ISSN.1009-9921.2019.12.012","url":null,"abstract":"Objective \u0000To investigate the current status of family hardiness and its influencing factors in children with leukemia. \u0000 \u0000 \u0000Methods \u0000The children with leukemia and their caregivers in the Children's Hospital of Shanxi from August to November 2017 were enrolled. A questionnaire survey was conducted using a convenient sampling method, and 100 questionnaires were distributed. The questionnaire included the General Status Questionnaire, the Family Hardiness Index (FHI), the Coping Health Inventory for Parents (CHIP) and the Positive and Negative Affect Scale (PANAS). \u0000 \u0000 \u0000Results \u0000A total of 92 valid questionnaires were collected. Among the scores of family hardiness in children with leukemia [(3.29±0.43) points], the responsibility score [(3.32±0.45) points] was higher than the control score [(3.31±0.46) points] and the challenge score [(3.23±0.53) points], and the challenge score was the lowest. The score of frequency of coping styles used by the caregiver of the child with leukemia was (3.64±0.70) points, the most frequent coping style used by the caregiver was \"family unity, optimism, cooperative attitude\" [(3.73±0.89) points], and the positive [(3.28±0.84) points] and negative [(2.51±0.80) points] emotions were in a moderate state. The child's sex, age, stage of chemotherapy and medical insurance status, the caregiver of the child, the age of the caregiver, the family's place of residence, and the education level were the related factors affecting the family hardiness score (all P < 0.01). The age of child, CHIP-1, CHIP-2, positive emotion and negative emotion were independent factors affecting the family hardiness (all P < 0.05). The CHIP, CHIP-1, CHIP-2 and positive emotion were positively correlated with the family hardiness (r values were 0.827, 0.883, 0.707 and 0.846, all P < 0.01); the negative emotion was negatively correlated with the family hardiness (r=-0.832, P < 0.01). \u0000 \u0000 \u0000Conclusion \u0000The family hardiness of children with leukemia is in the middle and upper level, the children's age, caregiver's coping style, positive emotion and negative emotion are factors affecting the family hardiness. \u0000 \u0000 \u0000Key words: \u0000Leukemia; Child; Family hardiness; Influencing factors; Psychotherapy","PeriodicalId":16246,"journal":{"name":"Journal of Leukemia and Lymphoma","volume":"28 1","pages":"753-757"},"PeriodicalIF":0.0,"publicationDate":"2019-12-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"48830383","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2019-11-25DOI: 10.3760/CMA.J.ISSN.1009-9921.2019.11.001
Yiyi Lu, Lin Lin
Objective To investigate calreticulin (CALR) gene mutations classification in BCR-ABL1 negative myeloproliterative neoplasms (MPN), and its relationship with clinical manifestations. Methods Genomic DNA polymerase chain reaction (PCR) amplification product Sanger sequencing method was used to detect the mutation of exon 9 of CALR gene in 236 patients with BCR-ABL1 negative MPN (excluding polycythemia vera and negative CALR mutations) in Ruijin Hospital of Shanghai Jiao Tong University School of Medicine from November 2015 to November 2018. The mutations were classified into 52 bp deletion (type 1) mutation, 5 bp insertion (type 2) mutation and other mutation types according to PCR sequencing analysis. The clinical characteristics of the carriers with two kinds of mutations in 198 patients with essential thrombocythemia (ET) and 38 primary myelofibrosis (PMF) were compared. For the types of mutations that could not be determined, they were classified according to the α-helix propensity score of the mutant protein peptide chain or the degree of retention of the negatively charged amino acid residues, and the differences between the two classification methods were also compared. Results Among 236 patients, the CALR gene type 1 or type 2 mutation was detected in 206 cases (87.3%), including 173 ET patients (99 cases of type 1 mutation and 74 cases of type 2 mutation) and 33 PMF patients (28 cases of type 1 mutation and 5 cases of type 2 mutation). The CALR non-type 1 or non-type 2 mutation was detected in 30 cases, including 25 ET patients and 5 PMF patients. Among 173 ET patients with CALR gene mutation, the white blood cell count (WBC) of patients with type 1 mutation was higher than that of patients with type 2 mutation [(8.6±2.7)×109/L vs. (7.6±2.4)×109/L, t = 2.45, P = 0.015]. Among 33 PMF patients with CALR gene mutation, the age of patients with type 1 mutation was older than that of patients with type 2 mutation [(58±13) years old vs. (41±16) years old, t = 2.51, P = 0.018]. According to the α-helix propensity score of mutant protein peptide chain and the degree of retention of the negatively charged amino acid residues, 27 kinds of non-type 1 or non-type 2 mutations were classified by using sequencing method, and there were differences between the two methods. According to the α-helix propensity score of the mutant protein peptide chain, the proportion of type 1/type 1-like mutation in PMF patients was higher than that in ET patients [78.9% (30/38) vs. 56.6% (112/198), P < 0.01]. According to the degree of retention of negatively charged amino acid residues in the mutant protein peptide chain, the isoelectric point (pI) value of the mutant protein peptide chain was higher than that of the wild type sequence. The pI value of the type 1-like mutant protein peptide chain was higher than that of the type 2-like mutation (11.79±0.15 vs. 10.02±0.42, t = 11.51, P < 0.01). Conclusions Type 1 mutated ET patients may be closely rela
{"title":"Significance of calreticulin gene mutations classification in BCR-ABL1 negative myeloproliferative neoplasms","authors":"Yiyi Lu, Lin Lin","doi":"10.3760/CMA.J.ISSN.1009-9921.2019.11.001","DOIUrl":"https://doi.org/10.3760/CMA.J.ISSN.1009-9921.2019.11.001","url":null,"abstract":"Objective \u0000To investigate calreticulin (CALR) gene mutations classification in BCR-ABL1 negative myeloproliterative neoplasms (MPN), and its relationship with clinical manifestations. \u0000 \u0000 \u0000Methods \u0000Genomic DNA polymerase chain reaction (PCR) amplification product Sanger sequencing method was used to detect the mutation of exon 9 of CALR gene in 236 patients with BCR-ABL1 negative MPN (excluding polycythemia vera and negative CALR mutations) in Ruijin Hospital of Shanghai Jiao Tong University School of Medicine from November 2015 to November 2018. The mutations were classified into 52 bp deletion (type 1) mutation, 5 bp insertion (type 2) mutation and other mutation types according to PCR sequencing analysis. The clinical characteristics of the carriers with two kinds of mutations in 198 patients with essential thrombocythemia (ET) and 38 primary myelofibrosis (PMF) were compared. For the types of mutations that could not be determined, they were classified according to the α-helix propensity score of the mutant protein peptide chain or the degree of retention of the negatively charged amino acid residues, and the differences between the two classification methods were also compared. \u0000 \u0000 \u0000Results \u0000Among 236 patients, the CALR gene type 1 or type 2 mutation was detected in 206 cases (87.3%), including 173 ET patients (99 cases of type 1 mutation and 74 cases of type 2 mutation) and 33 PMF patients (28 cases of type 1 mutation and 5 cases of type 2 mutation). The CALR non-type 1 or non-type 2 mutation was detected in 30 cases, including 25 ET patients and 5 PMF patients. Among 173 ET patients with CALR gene mutation, the white blood cell count (WBC) of patients with type 1 mutation was higher than that of patients with type 2 mutation [(8.6±2.7)×109/L vs. (7.6±2.4)×109/L, t = 2.45, P = 0.015]. Among 33 PMF patients with CALR gene mutation, the age of patients with type 1 mutation was older than that of patients with type 2 mutation [(58±13) years old vs. (41±16) years old, t = 2.51, P = 0.018]. According to the α-helix propensity score of mutant protein peptide chain and the degree of retention of the negatively charged amino acid residues, 27 kinds of non-type 1 or non-type 2 mutations were classified by using sequencing method, and there were differences between the two methods. According to the α-helix propensity score of the mutant protein peptide chain, the proportion of type 1/type 1-like mutation in PMF patients was higher than that in ET patients [78.9% (30/38) vs. 56.6% (112/198), P < 0.01]. According to the degree of retention of negatively charged amino acid residues in the mutant protein peptide chain, the isoelectric point (pI) value of the mutant protein peptide chain was higher than that of the wild type sequence. The pI value of the type 1-like mutant protein peptide chain was higher than that of the type 2-like mutation (11.79±0.15 vs. 10.02±0.42, t = 11.51, P < 0.01). \u0000 \u0000 \u0000Conclusions \u0000Type 1 mutated ET patients may be closely rela","PeriodicalId":16246,"journal":{"name":"Journal of Leukemia and Lymphoma","volume":"28 1","pages":"641-646"},"PeriodicalIF":0.0,"publicationDate":"2019-11-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"42809338","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Rectal mucosa-associated lymphoid tissue lymphoma: report of one case and review of literature","authors":"Wei Zhang, S. Bao, Yan-ping Ma, Lijun Jiang, Lijun Song, Y. Wei, F. Zhi, Yue Sun, Yeqiong Li","doi":"10.3760/CMA.J.ISSN.1009-9921.2019.11.015","DOIUrl":"https://doi.org/10.3760/CMA.J.ISSN.1009-9921.2019.11.015","url":null,"abstract":"目的 \u0000提高临床医生对直肠黏膜相关淋巴样组织结外边缘区（MALT）B细胞淋巴瘤的认识。 \u0000 \u0000 \u0000方法 \u0000回顾性分析宁夏回族自治区人民医院1例直肠MALT淋巴瘤患者的临床资料，并进行文献复习。 \u0000 \u0000 \u0000结果 \u0000直肠MALT淋巴瘤伴浆细胞分化与髓外浆细胞瘤（EMP）可通过细胞形态学、免疫组织化学、血清游离轻链测定、细胞遗传学及分子生物学进行鉴别。直肠MALT淋巴瘤抗幽门螺旋杆菌（Hp）治疗有效。 \u0000 \u0000 \u0000结论 \u0000直肠MALT淋巴瘤临床罕见，部分淋巴瘤细胞可伴浆细胞样分化，抗生素治疗可以作为其一线治疗方案。","PeriodicalId":16246,"journal":{"name":"Journal of Leukemia and Lymphoma","volume":"28 1","pages":"695-697"},"PeriodicalIF":0.0,"publicationDate":"2019-11-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"43033626","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
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