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Survival of Patients with Acute Myeloid Leukemia after Allogeneic Stem Cell Transplantation: An Experience in Developing Country. 发展中国家异基因干细胞移植后急性髓系白血病患者的生存率
Q3 Medicine Pub Date : 2022-01-01 DOI: 10.18502/ijhoscr.v16i1.8443
Mahshid Mehdizadeh, Vahid Bolourian, Gholamreza Zamani, Maria Tavakoli-Ardakanii, Shayan Zamani, Mahdi Tabarraee, Abbas Hajifathali

Background: Allogeneic stem cell transplantation (allo-SCT) is the highest potential treatment for long-term survival as post-remission therapy for acute myeloid leukemia (AML). The aim of this study was to estimate the overall survival (OS) of patients with AML after allo-SCT and to identify the factors affecting them as a prognostic factor for the survival of patients. Material and Methods: In this retrospective cohort study, data of patients with AML who underwent allo-SCT at Taleghani bone marrow transplantation and cell therapy center in Tehran, Iran, from May 2009 to September 2016 were used. A total of 101 patients were enrolled and death time was considered as a failure event for them. Kaplan-Meier method, log-Rank tests, and Cox proportional hazard model were used to evaluate OS and to identify the risk factors of patient's survival. The SPSS software version 21 was used for the analysis of data and P<0.05 was considered as a significant level. Results: Of 101 patients with AML, 49 (48.5%) were males. The median age at allo-SCT was 32.76 years and 42 patients (41.6%) died. The 5-year OS and disease-free survival (DFS) was 56% (95%CI: 51-61%) and 52% (95%CI: 57-47%), respectively. Multivariate analysis by Cox regression indicated that OS has a significant relationship with primary WBC count and relapse (P=0.001). Conclusion: Our results showed that allo-SCT has nearly the same outcome in developing countries and the WBC count and relapse are effective factors on the chance of survival in AML patients after allo-SCT.

背景:同种异体干细胞移植(allo-SCT)是急性髓系白血病(AML)缓解后长期生存的最有潜力的治疗方法。本研究的目的是评估同种异体细胞移植后AML患者的总生存期(OS),并确定作为患者生存预后因素的影响因素。材料和方法:在这项回顾性队列研究中,使用了2009年5月至2016年9月在伊朗德黑兰Taleghani骨髓移植和细胞治疗中心接受同种异体sct治疗的AML患者的数据。共纳入101例患者,死亡时间被视为失败事件。采用Kaplan-Meier法、log-Rank检验和Cox比例风险模型评价OS,确定影响患者生存的危险因素。采用SPSS软件21版对数据和结果进行分析:101例AML患者中,男性49例(48.5%)。同种异体细胞移植的中位年龄为32.76岁,42例(41.6%)患者死亡。5年OS和无病生存率(DFS)分别为56% (95%CI: 51-61%)和52% (95%CI: 57-47%)。Cox回归多因素分析显示,OS与原发性WBC计数和复发有显著关系(P=0.001)。结论:我们的研究结果表明,在发展中国家,同种异体细胞移植具有几乎相同的结果,白细胞计数和复发是影响AML患者同种异体细胞移植后生存机会的有效因素。
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引用次数: 0
The Effect of Xmn -1 Polymorphism and Coinheritance of Alpha Mutations on Age at First Blood Transfusion in Iranian Patients with Homozygote IVSI-5 Mutation. Xmn -1多态性和α突变共遗传对伊朗IVSI-5纯合子突变患者首次输血年龄的影响
Q3 Medicine Pub Date : 2022-01-01 DOI: 10.18502/ijhoscr.v16i1.8441
Mozhgan Hashemieh, Zahra Al Sadat Saadatmandi, Azita Azarkeivan, Hossein Najmabadi

Background : Thalassemia syndromes are the most prevalent hereditary hemoglobinopathies in the world. Iran is located on the thalassemia belt.  In this study, the effect of Xmn -1 polymorphism and coinheritance of alpha mutations on age at first transfusion and also transfusion interval in Iranian thalassemic patients with homozygous IVSI-5 mutation were assessed. Materials and Methods : In this retrospective cross-sectional study 154 transfusion dependent thalassemia (TDT) patients (140 patients with β-thalassemia major and 14 cases with β-thalassemia intermedia) who were homozygote of IVSI-5 mutation have been participated. Blood samples were collected from participants using EDTA containers for genomic DNA analysis. DNA extraction and amplification-refractory mutation to determine the Xmn -1 polymorphism were performed. Multiplex PCR was performed to identify alpha globin deletions.  Results: The mean age of participants was 29±7, 58 of them were male and 96 were female. A significant relation between presence of Xmn -1 polymorphism and age at receiving first transfusion was detected. Coinheritance of alpha thalassemia mutation does not have significant effect on age at first transfusion or transfusion interval. Conclusion : Presence of Xmn -1 polymorphism can delay the onset of transfusion in patients with homozygote IVSI-5 mutation.

背景:地中海贫血综合征是世界上最常见的遗传性血红蛋白病。伊朗位于地中海贫血带。本研究评估了Xmn -1多态性和α突变共遗传对伊朗地中海贫血IVSI-5纯合子突变患者首次输血年龄和输血间隔的影响。材料与方法:回顾性横断面研究154例IVSI-5突变纯合子输血依赖型地中海贫血(TDT)患者(重度β-地中海贫血140例,中度β-地中海贫血14例)。使用EDTA容器收集参与者的血液样本进行基因组DNA分析。DNA提取和扩增难解突变检测Xmn -1多态性。多重PCR检测α -珠蛋白缺失。结果:参与者平均年龄29±7岁,其中男性58人,女性96人。检测到Xmn -1多态性的存在与首次输血年龄有显著关系。α -地中海贫血突变的共遗传对首次输血年龄和输血间隔无显著影响。结论:Xmn -1多态性的存在可延缓IVSI-5纯合子突变患者输血的发生。
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引用次数: 0
Serum Albumin Levels Strongly Predict Survival Outcome of Elderly Patients with Diffuse Large B-Cell Lymphoma Treated with Rituximab-Combined Chemotherapy. 血清白蛋白水平可预测老年弥漫性大b细胞淋巴瘤患者利妥昔单抗联合化疗的生存结果。
Q3 Medicine Pub Date : 2022-01-01 DOI: 10.18502/ijhoscr.v16i1.8433
Hiroto Kaneko, Kazuho Shimura, Mihoko Yoshida, Yosuke Matsumoto, Tsutomu Kobayashi, Hitoji Uchiyama, Junya Kuroda, Masafumi Taniwaki

Background: In the current Japanese aging society, a high number of very elderly patients (age ranged from 80 to 93) with diffuse large B-cell lymphoma (DLBCL, most frequent hematological malignancy), who require chemotherapy are encountered. However, standard chemotherapy can result in severe adverse effects in elderly patients. Although various scoring systems are available to assess frailty, they are too complicated to immediately make a therapeutic decision, and studies on indications for chemotherapy in elderly patients are few. Materials and Methods: In the present study, we retrospectively analyzed the clinical records of 56 patients with DLBCL aged 80 or older who received R-CHOP or similar chemotherapy. Association of various clinical parameters, including performance status, stage, B symptom(s), laboratory data and relative dose intensity and survival outcomes was examined. Results: Pretreatment serum albumin level was identified as the only factor that predicts overall and progression-free survivals. Conclusion: We have concluded that very elderly DLBCL patients aged 80 or older with hypoalbuminemia may be unfit for standard chemotherapy, regardless of other factors. Alternative or palliative therapy should be considered for those patients.

背景:在当前的日本老龄化社会中,弥漫性大b细胞淋巴瘤(DLBCL,最常见的血液恶性肿瘤)的高龄患者(80 - 93岁)需要化疗。然而,标准化疗对老年患者会产生严重的不良反应。虽然有各种评分系统可用于评估虚弱,但它们过于复杂,无法立即做出治疗决定,而且关于老年患者化疗指征的研究很少。材料与方法:回顾性分析56例80岁及以上DLBCL患者接受R-CHOP或类似化疗的临床资料。检查各种临床参数(包括运动状态、分期、B症状、实验室数据和相对剂量强度)与生存结局的关系。结果:预处理血清白蛋白水平被确定为预测总生存率和无进展生存率的唯一因素。结论:我们已经得出结论,无论其他因素如何,80岁或以上的高龄DLBCL患者伴低白蛋白血症可能不适合标准化疗。应考虑对这些患者进行替代或姑息治疗。
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引用次数: 1
Secondary Immunodeficiency Frequency in Patients with Chronic Lymphocytic Leukemia: The Relationship with Stage and Treatment. 慢性淋巴细胞白血病患者继发免疫缺陷频率:与分期和治疗的关系。
Q3 Medicine Pub Date : 2022-01-01 DOI: 10.18502/ijhoscr.v16i1.8437
Osman Yokus, Konul Jafarli, Fettah Sametoglu, Hasan Goze, Istemi Serin

Background: Chronic lymphocytic leukemia (CLL) is one of the most common hematological malignancies. In patients with CLL, serum immunoglobulin levels decrease over time due to both the disease itself and the chemo-immunotherapeutic agents used. It was aimed to reveal the relationship between hypogammaglobulinemia and disease stage, and chemo-immunotherapies. Materials and Methods: Data were obtained by retrospectively examining 74 patients who were followed-up between 2008-2019. The relationship between all parameters (demographic characteristics, RAI stages or therapy subtypes) and serum IgG levels was analyzed. Results: Thirty-two of 74 patients received a therapy. Twenty-two patients were on combined therapy with rituximab or only rituximab and 10 were treated with chemotherapeutic agents only. The frequency of hypogammaglobulinemia was 5.4% at the diagnosis, this rate was 55% in patients receiving a therapy. Hypogammaglobulinemia was higher in advanced stages. In patients with rituximab, higher levels of IgG decrease were observed. Conclusion: Serum IgG level was significantly lower in patients with advanced-stage, received chemotherapy, especially rituximab. In addition to basal IgG, immunoglobulin levels should be checked during treatment, and follow-up period. Early replacement intravenous immunoglobulins will be important to reduce severe infection attacks due to secondary immunodeficiency.

背景:慢性淋巴细胞白血病(CLL)是最常见的血液系统恶性肿瘤之一。在CLL患者中,由于疾病本身和使用的化学免疫治疗剂,血清免疫球蛋白水平随着时间的推移而下降。目的是揭示低γ球蛋白血症与疾病分期和化学免疫治疗之间的关系。材料与方法:对2008-2019年随访的74例患者进行回顾性分析。分析所有参数(人口统计学特征、RAI分期或治疗亚型)与血清IgG水平的关系。结果:74例患者中32例接受了治疗。22例患者联合利妥昔单抗或仅利妥昔单抗治疗,10例患者仅接受化疗药物治疗。诊断时低γ -球蛋白血症的发生率为5.4%,而在接受治疗的患者中这一发生率为55%。晚期低丙种球蛋白血症较高。在使用利妥昔单抗的患者中,观察到更高水平的IgG下降。结论:接受化疗的晚期患者血清IgG水平明显降低,尤其是利妥昔单抗。除基础IgG外,在治疗期间和随访期间应检查免疫球蛋白水平。早期静脉注射免疫球蛋白对于减少继发性免疫缺陷引起的严重感染至关重要。
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引用次数: 0
Hepatosplenic T Cell Lymphoma: Diagnostic Conundrum. 肝脾T细胞淋巴瘤:诊断难题。
Q3 Medicine Pub Date : 2022-01-01 DOI: 10.18502/ijhoscr.v16i1.8444
Zachariah Chowdhury, Yookarin Khonglah, Vandana Raphael, Pranjal Kalita, Umesh Das

Hepatosplenic T cell lymphoma (HSTCL) is a very rare and aggressive peripheral T cell lymphoma that comprises less than 1% of Non-Hodgkin lymphomas (NHL). It is derived from cytotoxic T-cells, usually of γδ T cell receptor type, and is characterized by primary extranodal disease with typical sinusoidal infiltration of the liver, spleen and bone marrow by medium-sized lymphoid cells. HSTCL occurs more frequently in immunocompromised patients, especially in those receiving long-term immunosuppressive therapy. The differential diagnosis is varied, and the clinical course is dismal with a poor response to currently available therapies. Herein we report a case of HSTCL in a 20-year-old immunocompetent male who presented with fever, pallor, weight loss, bicytopenia, hepatomegaly, and massive splenomegaly, highlighting the diagnostic conundrum and pointers towards an accurate diagnosis. The key role for diagnosis was the combination of morphologic finding of atypical lymphoid cells in the bone marrow, typical immunophenotypic profile on flow cytometry and the pattern of involvement of the liver and the spleen, even in the absence of full-fledged diagnostic panels and tools. The report of this case is an endeavor to emphasize the high index of suspicion for timely detection of such a rare entity.

肝脾T细胞淋巴瘤(HSTCL)是一种非常罕见的侵袭性外周T细胞淋巴瘤,占非霍奇金淋巴瘤(NHL)的不到1%。它起源于细胞毒性T细胞,通常为γδ T细胞受体型,其特征是原发性结外疾病,典型的肝、脾和骨髓由中等大小的淋巴样细胞窦状浸润。HSTCL更常见于免疫功能低下的患者,特别是那些接受长期免疫抑制治疗的患者。鉴别诊断是多种多样的,临床过程令人沮丧,对目前可用的治疗反应不佳。在此,我们报告一个20岁的免疫功能正常的男性HSTCL病例,他表现为发烧,苍白,体重减轻,双细胞减少,肝肿大和脾肿大,突出了诊断难题并指出了准确诊断的方向。诊断的关键作用是结合骨髓中非典型淋巴细胞的形态学发现,流式细胞术的典型免疫表型特征以及肝脏和脾脏受累的模式,即使在没有成熟的诊断小组和工具的情况下。报告这一案件是为了强调及时发现这种罕见的实体的高度怀疑指数。
{"title":"Hepatosplenic T Cell Lymphoma: Diagnostic Conundrum.","authors":"Zachariah Chowdhury,&nbsp;Yookarin Khonglah,&nbsp;Vandana Raphael,&nbsp;Pranjal Kalita,&nbsp;Umesh Das","doi":"10.18502/ijhoscr.v16i1.8444","DOIUrl":"https://doi.org/10.18502/ijhoscr.v16i1.8444","url":null,"abstract":"<p><p>Hepatosplenic T cell lymphoma (HSTCL) is a very rare and aggressive peripheral T cell lymphoma that comprises less than 1% of Non-Hodgkin lymphomas (NHL). It is derived from cytotoxic T-cells, usually of γδ T cell receptor type, and is characterized by primary extranodal disease with typical sinusoidal infiltration of the liver, spleen and bone marrow by medium-sized lymphoid cells. HSTCL occurs more frequently in immunocompromised patients, especially in those receiving long-term immunosuppressive therapy. The differential diagnosis is varied, and the clinical course is dismal with a poor response to currently available therapies. Herein we report a case of HSTCL in a 20-year-old immunocompetent male who presented with fever, pallor, weight loss, bicytopenia, hepatomegaly, and massive splenomegaly, highlighting the diagnostic conundrum and pointers towards an accurate diagnosis. The key role for diagnosis was the combination of morphologic finding of atypical lymphoid cells in the bone marrow, typical immunophenotypic profile on flow cytometry and the pattern of involvement of the liver and the spleen, even in the absence of full-fledged diagnostic panels and tools. The report of this case is an endeavor to emphasize the high index of suspicion for timely detection of such a rare entity.</p>","PeriodicalId":38991,"journal":{"name":"International Journal of Hematology-Oncology and Stem Cell Research","volume":"16 1","pages":"66-73"},"PeriodicalIF":0.0,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/ef/4a/IJHOSCR-16-66.PMC9339126.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"40703566","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 2
Flow Cytometric DNA Ploidy Analysis in Haemato-Lymphoid Neoplasms: An Analysis of 132 Cases. 132例血淋巴肿瘤DNA倍体分析。
Q3 Medicine Pub Date : 2022-01-01 DOI: 10.18502/ijhoscr.v16i1.8440
Nishit Gupta, Aditi Mittal, Tina Dadu, Dharma Choudhary, Anil Handoo

Background: FxCycleTM Violet (FCV) based flow cytometric (FCM) DNA ploidy analysis is a rapid and simple tool that can substantiate in characterizing the biological behaviour across the spectrum of haematological malignancies and correlates with cytogenetic studies. Materials and Methods: In this prospective study, we performed simultaneous immunophenotyping with FCV based on ploidy analysis in n=132 consecutive new samples, comprising n=110 samples of haemato-lymphoid neoplasms, including acute leukemias (n=67, 60.9%), CML with myeloid blast crisis (n=1, 0.9%), MDS with excess blasts (n=2, 1.8%), mature B cell/ T cell neoplasms (n=37, 33.7%), multiple myeloma (n=3, 2.7%) along with n=22 normal samples. The FCM DNA data was compared with corresponding conventional karyotyping results, wherever available. Results: In FCM ploidy analysis (n=110), the overall DNA index (DI) ranged from 0.81 to 2.17 and S-Phase fraction (SPF) from 0.1-31.6%. Diploidy was seen in n = 90 (81.8%), low-hyperdiploidy in n = 10 (9.1%), high-hyperdiploidy in n = 7 (6.4%) with one case each (0.9% each) having near-tetraploidy, high-hypodiploidy and low-hypodiploidy. The DI of all viable cell populations in normal samples ranged from 0.96-1.05. Conventional karyotyping was performed in n=76/110 cases (70%) with n= 11/76 (15%) culture failures. The modal chromosome number ranged from 45 to 63. A concordance of 95.4% (n=62/65) was noted with corresponding FCM DI. Conclusion: FCV-based ploidy is a sensitive technique that provides complementary information and ascertains a strong correlation with conventional cytogenetics across all haemato-lymphoid neoplasms. It can detect aneuploidy in all B-ALL and myeloma cases, even in hemodiluted samples with cytogenetic culture failure; supplement the diagnoses of erythroleukemia, and provide a useful screen for a higher grade lymph node disease in lymphoma cases with SPF > 3%.

背景:FxCycleTM紫罗兰(FCV)为基础的流式细胞术(FCM) DNA倍体分析是一种快速和简单的工具,可以证实在整个血液系统恶性肿瘤的生物学行为特征,并与细胞遗传学研究相关。材料和方法:在这项前瞻性研究中,我们对n=132个连续新样本进行了基于倍性分析的FCV同时免疫表型分析,包括n=110个血液淋巴样肿瘤样本,包括急性白血病(n=67, 60.9%), CML伴髓细胞危像(n=1, 0.9%), MDS伴髓细胞过多(n=2, 1.8%),成熟B细胞/ T细胞肿瘤(n=37, 33.7%),多发性骨髓瘤(n=3, 2.7%)以及n=22个正常样本。将FCM DNA数据与相应的常规核型结果进行比较。结果:FCM倍性分析(n=110),总DNA指数(DI)为0.81 ~ 2.17,s相分数(SPF)为0.1 ~ 31.6%。二倍体n = 90例(81.8%),低高二倍体n = 10例(9.1%),高高二倍体n = 7例(6.4%),近四倍体、高次二倍体和低次二倍体各1例(0.9%)。正常样本各活细胞群DI范围为0.96 ~ 1.05。常规核型分析n=76/110例(70%),培养失败n= 11/76例(15%)。模态染色体数为45 ~ 63。与相应FCM DI的一致性为95.4% (n=62/65)。结论:基于fcv的倍性是一种敏感的技术,它提供了补充信息,并确定了与所有血淋巴样肿瘤的常规细胞遗传学的强相关性。它可以检测所有B-ALL和骨髓瘤病例的非整倍体,甚至在细胞遗传学培养失败的血液稀释样本中;补充红细胞白血病的诊断,并为SPF > 3%的淋巴瘤患者提供更高级别淋巴结疾病的有用筛查。
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引用次数: 1
Heparin-Induced Thrombocytopenia in Iranian Cardiac Surgery Patients Using the 4Ts Clinical Scoring System and Laboratory Methods 使用4Ts临床评分系统和实验室方法的伊朗心脏手术患者肝素诱导的血小板减少
Q3 Medicine Pub Date : 2021-10-01 DOI: 10.18502/ijhoscr.v15i4.7478
M. Ahmadinejad, Massoumeh Shahbazi, A. Chegini
Background: Heparin-induced thrombocytopenia (HIT) is a serious adverse drug reaction. HIT diagnosis needs an algorithmic approach including clinical evaluation and laboratory tests (screening and confirmatory). Few studies have been conducted on HIT in Iran, and most existing research has been general and based on clinical evaluations alone. The present study was conducted to determine the prevalence of HIT among cardiac surgery patients using an algorithmic approach. Materials and Methods: A cross-sectional study was carried out over a period of 10 months, at Modares Hospital (Tehran, Iran) on 92 patients who were candidates for cardiac surgery. For the clinical evaluation, the 4Ts scoring system was used; in cases with 4Ts scores ≥4, a laboratory evaluation of anti-PF4/heparin antibody (Ab) was performed by enzyme-linked immunosorbent assay (ELISA) and a HIPA test too as a functional confirmatory method. The patients with 4Ts scores ≥4 who were ELISA positive (OD ≥0.2) and HIPA positive were taken as a definite case of HIT. Results: Of the 92 patients who had undergone cardiac surgery, 14 (15%) had 4Ts scores ≥4. Anti- PF4/heparin Ab was detected in eight patients using the ELISA and in six patients using the HIPA. Ultimately, definite HIT was confirmed in five of the patients. Conclusion: The prevalence of HIT was 5.4% among the cardiac surgery patients assessed in the present study. To the researchers’ knowledge, this is the first time that HIT has been evaluated in Iran using a comprehensive algorithmic approach including clinical history-taking and both immunological and functional laboratory tests, and the findings showed a slightly higher HIT frequency in this single-center study in comparison with the other studies carried out in other countries.
背景:肝素诱导的血小板减少症(HIT)是一种严重的药物不良反应。HIT诊断需要一种算法方法,包括临床评估和实验室检查(筛查和确认)。伊朗对HIT进行的研究很少,大多数现有研究都是一般性的,仅基于临床评估。本研究采用算法方法确定心脏手术患者中HIT的患病率。材料和方法:在Modares医院(德黑兰,伊朗)对92例心脏手术患者进行了为期10个月的横断面研究。临床评价采用4Ts评分系统;在4Ts评分≥4的病例中,采用酶联免疫吸附试验(ELISA)和HIPA试验进行抗pf4 /肝素抗体(Ab)的实验室评估,作为功能确认方法。4Ts评分≥4且ELISA阳性(OD≥0.2)、HIPA阳性的患者作为HIT的确诊病例。结果:92例心脏手术患者中,14例(15%)4Ts评分≥4分。8例患者采用ELISA法检测抗PF4/肝素Ab, 6例患者采用HIPA法检测抗PF4/肝素Ab。最终,5例患者确诊为HIT。结论:本组心脏手术患者HIT发生率为5.4%。据研究人员所知,这是伊朗首次使用综合算法方法对HIT进行评估,包括临床病史采集和免疫学和功能实验室测试,结果显示,与其他国家进行的其他研究相比,该单中心研究的HIT频率略高。
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引用次数: 0
Concomitant Essential Thrombocythemia and Mature B -Lymphoproliferative Disorder in a Patient 合并原发性血小板增多症和成熟B淋巴细胞增生性疾病1例
Q3 Medicine Pub Date : 2021-10-01 DOI: 10.18502/ijhoscr.v15i4.7481
A. Butt, Ruhul Quddus, Natasha Ali
A-64-year old male presented with cough, weight loss, and maculopapular rash for 15-20 days. On examination, he was found to have cervical lymphadenopathy and splenomegaly. His leukocyte count was 62.1x109/L, platelets were 1169x109/L and LDH was 816 IU/L. Peripheral blood film showed a leukoerythroblastic picture with thrombocytosis. He was started on hydroxyurea and allopurinol. Subsequently, bone marrow evaluation was done which depicted increased lymphoid cells with an M:E ratio of 4:1. Cellular areas exhibited an increase in myeloid precursors along with prominent lymphoid cells and abundant megakaryocytes. Immunohistochemistry showed an increase in B-lymphocytes. Grade MF-2 reticulin fibrosis was noted. Overall findings suggested essential thrombocythemia (ET). On flow cytometry, CD45-positive lymphoid cells population was 31% and showed reactivity to Pan-B-markers with lambda light chain restriction. Janus Kinase 2 (JAK 2) mutation was detected while BCR-ABL1 translocation was negative. A diagnosis of ET progressing to myelofibrosis and mature B-lymphoproliferative disorder was made. Hydroxyurea and allopurinol were stopped while ruxolitinib was introduced and 2.5 years later he remains stable on this treatment.
男性,64岁,咳嗽,体重减轻,黄斑丘疹15-20天。经检查,发现他有颈淋巴肿大及脾肿大。白细胞62.1 × 109/L,血小板1169x109/L, LDH 816 IU/L。外周血膜显示成白细胞图像,伴血小板增多。他开始服用羟基脲和别嘌呤醇。随后,骨髓评估显示淋巴样细胞增加,M:E比为4:1。细胞区髓系前体增多,淋巴样细胞突出,巨核细胞丰富。免疫组化显示b淋巴细胞增多。观察到MF-2级网状蛋白纤维化。总体结果提示原发性血小板增多症(ET)。流式细胞术显示,cd45阳性淋巴细胞占31%,对泛b标记具有轻链限制性反应。检测到Janus Kinase 2 (JAK 2)突变,BCR-ABL1易位阴性。诊断为ET进展为骨髓纤维化和成熟b淋巴细胞增生性疾病。在引入鲁索利替尼的同时停用羟基脲和别嘌呤醇,2.5年后患者在这种治疗下保持稳定。
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引用次数: 0
Evaluation of the Preventive Effects of Carvedilol on Trastuzumab-Induced Cardiotoxicity in Early-Stage and Locally Advanced HER2-Positive Breast Cancer Patients 卡维地洛对早期和局部晚期her2阳性乳腺癌患者曲妥珠单抗诱导的心脏毒性的预防作用评价
Q3 Medicine Pub Date : 2021-10-01 DOI: 10.18502/ijhoscr.v15i4.7475
M. Esfandbod, Mina Sadat Naderi, Azadeh Sadatnaseri, A. Ahmadi, M. Noroozi, Saeid Sadeghi Joni
Background: Trastuzumab is an efficient monoclonal antibody used in the treatment of Her2-positive breast cancer. Despite its prominent effect on Her2-positive patients’ disease-free Survival. Trastuzumab-induced cardiotoxicity is still one of the main challenges. Angiotensin-converting enzyme inhibitors (ACE inhibitors) are one of the most potent agents used in heart failure, which also showed confirmed cardioprotective effects against anthracycline and doxorubicin. We aimed to assess the cardioprotective effects of Carvedilol in a randomized clinical trial study. Materials and Methods: sixty non-metastatic Her-2 positive patients (30 cases; 30 controls) were entered into the study via a simple randomization method.Carvedilol was administered for the patients with the starting dose of 3.125 mg twice a day and started 7 days before trastuzumab administration. The dose has been increased in a three-week period to reach 12.5 mg twice a day and continued until the end of therapy. All the patients underwent an echocardiography after receiving Adriamycin and Cyclophosphamide in order to measure basal Ejection Fraction (EF) and Pulmonary Artery Pressure (PAP). Each patient underwent a follow-up echocardiography in 3,6,9 and 12 months after initiation of the treatment. Finally, all the patients went through the last episode of echocardiography 1 month after the end of treatment. All the Measured PAP and EF has been recorded and analyzed Results: EF and PAP changes for both groups had no significant changes during the course of treatment with Trastuzmab (p-value = 0.628 and p-value = 0.723, respectively). Seven patients in the intervention group and 2 patients in the control group presented with EF decrease. Also, 8 patients in the intervention and 9 patients in the control groups showed PAP increase. Conclusion: According to our results, in patients with HER2-positive breast cancer treated with trastuzumab, Carvedilol showed no significant protective effect on trastuzumab-induced cardiotoxicity.
背景:曲妥珠单抗是一种用于治疗her2阳性乳腺癌的有效单克隆抗体。尽管它对her2阳性患者的无病生存有显著影响。曲妥珠单抗引起的心脏毒性仍然是主要的挑战之一。血管紧张素转换酶抑制剂(ACE抑制剂)是治疗心力衰竭最有效的药物之一,对蒽环类药物和阿霉素也有保护心脏的作用。我们的目的是在一项随机临床试验研究中评估卡维地洛的心脏保护作用。材料与方法:60例非转移性Her-2阳性患者(30例;30名对照者)通过简单的随机化方法进入研究。卡维地洛在曲妥珠单抗给药前7天开始给药,起始剂量为3.125 mg,每天2次。在三周的时间内,剂量增加到12.5毫克,每天两次,并持续到治疗结束。所有患者在接受阿霉素和环磷酰胺治疗后都进行了超声心动图检查,以测量基础射血分数(EF)和肺动脉压(PAP)。每位患者在治疗开始后3、6、9和12个月接受了随访超声心动图检查。最后,所有患者在治疗结束1个月后进行最后一次超声心动图检查。结果:两组患者在曲妥珠单抗治疗过程中,EF和PAP的变化均无显著变化(p值分别为0.628和0.723)。干预组7例,对照组2例出现EF下降。干预组8例,对照组9例,PAP升高。结论:根据我们的研究结果,在接受曲妥珠单抗治疗的her2阳性乳腺癌患者中,卡维地洛对曲妥珠单抗诱导的心脏毒性没有明显的保护作用。
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引用次数: 5
Survival of Post-Transplant Lymphoproliferative Disorder after Kidney Transplantation in Patients under Rapamycin Treatment 接受雷帕霉素治疗的肾移植后淋巴增生性疾病患者的生存
Q3 Medicine Pub Date : 2021-10-01 DOI: 10.18502/ijhoscr.v15i4.7479
Farzaneh Ashrafi, S. Shahidi, V. Mehrzad, M. Mortazavi, Sayyideh Forough Hosseini
Background: One of the important causes of mortality and morbidity in kidney transplanted patients is Post Transplant Lymphoproliferative Disease (PTLD), which is due to immunosuppression therapy and viral activity. It seems that Rapamycin, with dual antineoplastic and immunosuppressive effects, may have a pivotal role in the treatment of PTLD patients and preserving transplanted kidneys. Methods and Materials: Twenty patients with PTLD were enrolled. Immunosuppressive therapy was reduced or ceased, and Rapamycin was initiated at the time of PTLD diagnosis. We evaluated the effects of switching immunosuppressive drugs to Rapamycin on graft status, the response of tumor, and 6, 12 months, and 5-year survival in patients. Results: PTLD remission was achieved in 14 patients, while six patients died; no relapse was detected in recovered patients. The median of PTLD free time was 25 months, and the mean overall survival in patients with PTLD treated by Rapamycin was 84.8 (95% CI=61.3-108.23).The five-year survival rate was 67%, 12 months survival was 73.8%, and six months' survival was 80%. The response rate to Rapamycin and immunosuppression reduction alone was 46.6%. Four out of 13 Diffuse Large B-Cell Lymphoma patients achieved a complete response just only after the reduction of immunosuppressive drugs and the consumption of Rapamycin. Conclusion: The present study demonstrated the effectiveness of conversion from immunosuppressive medication, particularly of Calcineurin inhibitors to Rapamycin in PTLD patients. However, more research is needed to confirm the Rapamycin effect on patients with PTLD.
背景:肾移植患者死亡和发病的重要原因之一是移植后淋巴细胞增生性疾病(PTLD),这是由免疫抑制治疗和病毒活性引起的。雷帕霉素具有双重抗肿瘤和免疫抑制作用,可能在PTLD患者的治疗和保存移植肾方面发挥关键作用。方法与材料:选取20例PTLD患者。减少或停止免疫抑制治疗,并在PTLD诊断时开始使用雷帕霉素。我们评估了将免疫抑制药物转换为雷帕霉素对移植物状态、肿瘤反应以及患者6个月、12个月和5年生存率的影响。结果:14例患者PTLD缓解,6例死亡;痊愈患者无复发。PTLD空闲时间中位数为25个月,接受雷帕霉素治疗的PTLD患者的平均总生存期为84.8 (95% CI=61.3-108.23)。5年生存率67%,12个月生存率73.8%,6个月生存率80%。单纯雷帕霉素联合免疫抑制治疗的有效率为46.6%。13例弥漫性大b细胞淋巴瘤患者中有4例仅在减少免疫抑制药物和使用雷帕霉素后才获得完全缓解。结论:本研究证明了PTLD患者从免疫抑制药物,特别是钙调磷酸酶抑制剂到雷帕霉素的转换是有效的。然而,需要更多的研究来证实雷帕霉素对PTLD患者的作用。
{"title":"Survival of Post-Transplant Lymphoproliferative Disorder after Kidney Transplantation in Patients under Rapamycin Treatment","authors":"Farzaneh Ashrafi, S. Shahidi, V. Mehrzad, M. Mortazavi, Sayyideh Forough Hosseini","doi":"10.18502/ijhoscr.v15i4.7479","DOIUrl":"https://doi.org/10.18502/ijhoscr.v15i4.7479","url":null,"abstract":"Background: One of the important causes of mortality and morbidity in kidney transplanted patients is Post Transplant Lymphoproliferative Disease (PTLD), which is due to immunosuppression therapy and viral activity. It seems that Rapamycin, with dual antineoplastic and immunosuppressive effects, may have a pivotal role in the treatment of PTLD patients and preserving transplanted kidneys. Methods and Materials: Twenty patients with PTLD were enrolled. Immunosuppressive therapy was reduced or ceased, and Rapamycin was initiated at the time of PTLD diagnosis. We evaluated the effects of switching immunosuppressive drugs to Rapamycin on graft status, the response of tumor, and 6, 12 months, and 5-year survival in patients. Results: PTLD remission was achieved in 14 patients, while six patients died; no relapse was detected in recovered patients. The median of PTLD free time was 25 months, and the mean overall survival in patients with PTLD treated by Rapamycin was 84.8 (95% CI=61.3-108.23).The five-year survival rate was 67%, 12 months survival was 73.8%, and six months' survival was 80%. The response rate to Rapamycin and immunosuppression reduction alone was 46.6%. Four out of 13 Diffuse Large B-Cell Lymphoma patients achieved a complete response just only after the reduction of immunosuppressive drugs and the consumption of Rapamycin. Conclusion: The present study demonstrated the effectiveness of conversion from immunosuppressive medication, particularly of Calcineurin inhibitors to Rapamycin in PTLD patients. However, more research is needed to confirm the Rapamycin effect on patients with PTLD.","PeriodicalId":38991,"journal":{"name":"International Journal of Hematology-Oncology and Stem Cell Research","volume":"7 1","pages":"239 - 248"},"PeriodicalIF":0.0,"publicationDate":"2021-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"79726203","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}
引用次数: 1
期刊
International Journal of Hematology-Oncology and Stem Cell Research
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