Abstract Double hit lymphoma (DHL) and double-expressor lymphoma (DEL) are now considered as aggressive types of diffuse large B cell lymphoma. DHL is characterized by a dual rearrangement of MYC and B cell lymphoma 2 (BCL-2) and/or B cell lymphoma 6 (BCL-6) and DEL by overexpression of MYC and BCL-2. Both DHL and DEL have aggressive presentation and are more common in elderly population. We present a case of 1 ½ years old boy who presented with bilateral proptosis, and diagnosed as non-Hodgkin lymphoma with central nervous system involvement. Immunohistochemistry revealed high expression of MYC and BCL-2. Fluorescence in situ hybridization studies done to rule out DHL showed no translocation of C-MYC, Bcl-2, and Bcl-6 and hence were confirmed as double-expressor high-grade B cell lymphoma. Dual expression of C- MYC, Bcl-2, or Bcl-6 always needs further evaluation to rule out the more aggressive DHL subtypes.
双重打击淋巴瘤(Double hit lymphoma, DHL)和双表达淋巴瘤(Double -expressor lymphoma, DEL)目前被认为是弥漫性大B细胞淋巴瘤的侵袭型。DHL的特征是MYC和B细胞淋巴瘤2 (BCL-2)和/或B细胞淋巴瘤6 (BCL-6)和DEL的双重重排，MYC和BCL-2过表达。DHL和DEL均有侵袭性表现，多见于老年人群。我们提出一个1岁半的男孩谁提出了双侧突出，并诊断为非霍奇金淋巴瘤与中枢神经系统受累。免疫组化显示MYC和BCL-2高表达。为排除DHL而进行的荧光原位杂交研究显示，C-MYC、Bcl-2和Bcl-6没有易位，因此证实为双表达高级别B细胞淋巴瘤。C- MYC、Bcl-2或Bcl-6的双重表达始终需要进一步评估以排除更具侵袭性的DHL亚型。
{"title":"Double-Expressor Lymphoma in a Young Child—A Case Report and Review of Literature","authors":"Anjali Shaju, Latha M. Sneha, Nidarshana Pandian, Suresh Chandra, Sonam Poonam Nisar, Krishnakumar Subramanian, Priyathersini Nagarajan","doi":"10.1055/s-0043-1772233","DOIUrl":"https://doi.org/10.1055/s-0043-1772233","url":null,"abstract":"Abstract Double hit lymphoma (DHL) and double-expressor lymphoma (DEL) are now considered as aggressive types of diffuse large B cell lymphoma. DHL is characterized by a dual rearrangement of MYC and B cell lymphoma 2 (BCL-2) and/or B cell lymphoma 6 (BCL-6) and DEL by overexpression of MYC and BCL-2. Both DHL and DEL have aggressive presentation and are more common in elderly population. We present a case of 1 ½ years old boy who presented with bilateral proptosis, and diagnosed as non-Hodgkin lymphoma with central nervous system involvement. Immunohistochemistry revealed high expression of MYC and BCL-2. Fluorescence in situ hybridization studies done to rule out DHL showed no translocation of C-MYC, Bcl-2, and Bcl-6 and hence were confirmed as double-expressor high-grade B cell lymphoma. Dual expression of C- MYC, Bcl-2, or Bcl-6 always needs further evaluation to rule out the more aggressive DHL subtypes.","PeriodicalId":13513,"journal":{"name":"Indian Journal of Medical and Paediatric Oncology","volume":"27 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-09-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"136015172","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}
Abstract A 51-year-old lady diagnosed as a case of metastatic carcinoma colon stage IV involving lung and left adrenal metastasis was initiated with palliative chemotherapy with FOLFOX-6 chemotherapy infusion regimen that consisted of 5-flurouracil, calcium leucovorin, and oxaliplatin and injection bevacizumab was added to cycle seven of FOLFOX-6 chemotherapy. After 2 months of initiating bevacizumab, she was presented with one episode of generalized tonic clonic seizure, headache, and visual blurring of 2 days duration and magnetic resonance imaging brain T2 fluid-attenuated inversion recovery image revealed subcortical white matter edema in bilateral frontoparietal, occipital, left temporal lobes (Fig. 1A and B), and hyperintense edema with effacement of folia in both cerebellum with nodular areas of contrast enhancement suggestive of posterior reversible encephalopathy syndrome.
{"title":"Clinical Syndrome of Seizures, Headache, and Visual Blurring in Metastatic Carcinoma Colon","authors":"Lakshmi Raj, T.M. Anoop, Rakesh CA","doi":"10.1055/s-0043-1772710","DOIUrl":"https://doi.org/10.1055/s-0043-1772710","url":null,"abstract":"Abstract A 51-year-old lady diagnosed as a case of metastatic carcinoma colon stage IV involving lung and left adrenal metastasis was initiated with palliative chemotherapy with FOLFOX-6 chemotherapy infusion regimen that consisted of 5-flurouracil, calcium leucovorin, and oxaliplatin and injection bevacizumab was added to cycle seven of FOLFOX-6 chemotherapy. After 2 months of initiating bevacizumab, she was presented with one episode of generalized tonic clonic seizure, headache, and visual blurring of 2 days duration and magnetic resonance imaging brain T2 fluid-attenuated inversion recovery image revealed subcortical white matter edema in bilateral frontoparietal, occipital, left temporal lobes (Fig. 1A and B), and hyperintense edema with effacement of folia in both cerebellum with nodular areas of contrast enhancement suggestive of posterior reversible encephalopathy syndrome.","PeriodicalId":13513,"journal":{"name":"Indian Journal of Medical and Paediatric Oncology","volume":"47 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-09-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"136015467","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}
Abstract Skin metastases from internal malignancies can be the first clinical finding of cancer recurrence. If presentation is delayed, they may ulcerate and develop secondary infection. Through this case, we will highlight a case of breast cancer recurrence with extensive cutaneous metastases that clinically completely responded to oral systemic therapy, thereby obviating the need for chemotherapy and leading to stark improvement in performance status and quality of life.
{"title":"Complete Remission of Cutaneous Metastasis from Invasive Breast Cancer with Abemaciclib plus Letrozole","authors":"Shaunak N. Valame, Dhruti D. Manek","doi":"10.1055/s-0043-1771046","DOIUrl":"https://doi.org/10.1055/s-0043-1771046","url":null,"abstract":"Abstract Skin metastases from internal malignancies can be the first clinical finding of cancer recurrence. If presentation is delayed, they may ulcerate and develop secondary infection. Through this case, we will highlight a case of breast cancer recurrence with extensive cutaneous metastases that clinically completely responded to oral systemic therapy, thereby obviating the need for chemotherapy and leading to stark improvement in performance status and quality of life.","PeriodicalId":13513,"journal":{"name":"Indian Journal of Medical and Paediatric Oncology","volume":"11 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-09-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135149174","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}
Archana Melavarige Venkatagiri, Vasudeva K Bhat, Arjun Asok, Krishnananda Prabhu
Abstract Introduction L-asparaginase is considered to be the most important component in the treatment of acute lymphoblastic leukemia (ALL). Intensifying the use of L-asparaginase during treatment for ALL has resulted in a significant rise in the percentage of children and adolescents who are cured of the disease. Asparaginase trough activity more than or equal to 100 IU/L on day 7 has been found to be the desired activity level in all childhood leukemia patients. Objectives Due to the paucity of data on biosimilar pegaspargase in the upfront setting, we planned this prospective pilot study to evaluate the levels of serum asparaginase activity (SAA) after biosimilar pegaspargase infusion. Materials and Methods It is a prospective, single-center, pilot study of 10 pediatric ALL patients for the duration of 6 months. All children less than 18 years with ALL on treatment with curative intent and receiving pegaspargase and who provided informed consent were included in this study. The enzymatic spectrophotometric method was used to determine SAA, and it was measured on the 7th and 14th days after the first dosage of pegaspargase-asparaginase, as well as on the 14th day after the second dose of pegaspargase-asparaginase, while toxicity was charted according to Common Terminology Criteria for Adverse Events (CTCAE) version 4.0. Results From 10 patients with a median age of 5.5 years, a grand total of 29 samples were taken for analysis. Children who received pegaspargase had either B-ALL or T-ALL. After the first dose, mean ± SD (standard deviation), SAA levels at day 7 was 131.3 ± 38 IU/L and at Day 14 was 94.8 ± 8 IU/L. After the second dose, mean ± SD SAA level at day 14 was 86.1 ± 15 IU/L. No patient had clinical hypersensitivity reaction and no patient reported any asparaginase-related toxicity. One patient died due to sepsis, infection with multidrug-resistant gram-negative bacteria. Conclusions Biosimilar pegaspargase maintained good SAA levels 7 and 14 days after infusion. Drug Trial Registration: Clinical Trial Registry of India vide reference CTRI/2021/08/036033 and available at https://ctri.nic.in/Clinicaltrials/pmaindet2.php?trialid=59285&EncHid=&userName=
l -天冬酰胺酶被认为是急性淋巴细胞白血病(ALL)治疗中最重要的成分。在ALL治疗期间加强l -天冬酰胺酶的使用已导致该疾病治愈的儿童和青少年百分比显著上升。所有儿童白血病患者在第7天的天冬酰胺酶谷活性大于或等于100 IU/L是理想的活性水平。由于前期缺乏生物类似药pegaspargase的数据，我们计划进行这项前瞻性先导研究，以评估生物类似药pegaspargase输注后血清天冬酰胺酶活性(SAA)水平。材料与方法这是一项前瞻性、单中心、试点研究，纳入10例儿科ALL患者，为期6个月。所有18岁以下的All患儿均接受pegaspargase治疗，并提供知情同意。采用酶促分光光度法测定SAA，分别于第一次给药后第7天、第14天和第二次给药后第14天测定SAA，并根据不良事件通用术语标准(CTCAE) 4.0版绘制毒性图。结果10例患者中位年龄5.5岁，共采集29份标本进行分析。接受pegaspargase治疗的儿童要么患有B-ALL，要么患有T-ALL。第一次给药后，第7天SAA水平为131.3±38 IU/L，第14天SAA水平为94.8±8 IU/L。第二次给药后，第14天SAA的平均±SD水平为86.1±15 IU/L。没有患者出现临床过敏反应，也没有患者报告任何与天冬酰胺酶相关的毒性。一名患者死于败血症，感染多重耐药革兰氏阴性菌。结论生物仿制药pegaspargase在输注后7、14 d仍保持良好的SAA水平。药物试验注册:印度临床试验注册中心，参考文献CTRI/2021/08/036033，可访问https://ctri.nic.in/Clinicaltrials/pmaindet2.php?trialid=59285&EncHid=&userName=
{"title":"A Pilot Study Conducted at a Tertiary Cancer Care Center, Evaluating the Serum Asparaginase Activity in Children Suffering from Acute Lymphoblastic Leukemia after the Administration of Biosimilar Pegaspargase","authors":"Archana Melavarige Venkatagiri, Vasudeva K Bhat, Arjun Asok, Krishnananda Prabhu","doi":"10.1055/s-0043-1774806","DOIUrl":"https://doi.org/10.1055/s-0043-1774806","url":null,"abstract":"Abstract Introduction L-asparaginase is considered to be the most important component in the treatment of acute lymphoblastic leukemia (ALL). Intensifying the use of L-asparaginase during treatment for ALL has resulted in a significant rise in the percentage of children and adolescents who are cured of the disease. Asparaginase trough activity more than or equal to 100 IU/L on day 7 has been found to be the desired activity level in all childhood leukemia patients. Objectives Due to the paucity of data on biosimilar pegaspargase in the upfront setting, we planned this prospective pilot study to evaluate the levels of serum asparaginase activity (SAA) after biosimilar pegaspargase infusion. Materials and Methods It is a prospective, single-center, pilot study of 10 pediatric ALL patients for the duration of 6 months. All children less than 18 years with ALL on treatment with curative intent and receiving pegaspargase and who provided informed consent were included in this study. The enzymatic spectrophotometric method was used to determine SAA, and it was measured on the 7th and 14th days after the first dosage of pegaspargase-asparaginase, as well as on the 14th day after the second dose of pegaspargase-asparaginase, while toxicity was charted according to Common Terminology Criteria for Adverse Events (CTCAE) version 4.0. Results From 10 patients with a median age of 5.5 years, a grand total of 29 samples were taken for analysis. Children who received pegaspargase had either B-ALL or T-ALL. After the first dose, mean ± SD (standard deviation), SAA levels at day 7 was 131.3 ± 38 IU/L and at Day 14 was 94.8 ± 8 IU/L. After the second dose, mean ± SD SAA level at day 14 was 86.1 ± 15 IU/L. No patient had clinical hypersensitivity reaction and no patient reported any asparaginase-related toxicity. One patient died due to sepsis, infection with multidrug-resistant gram-negative bacteria. Conclusions Biosimilar pegaspargase maintained good SAA levels 7 and 14 days after infusion. Drug Trial Registration: Clinical Trial Registry of India vide reference CTRI/2021/08/036033 and available at https://ctri.nic.in/Clinicaltrials/pmaindet2.php?trialid=59285&EncHid=&userName=","PeriodicalId":13513,"journal":{"name":"Indian Journal of Medical and Paediatric Oncology","volume":"30 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-09-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135150383","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}
Abstract Multiple myeloma (MM) is a clonal plasma cell disorder that commonly presents with anemia, renal failure, hypercalcemia, and lytic bone lesions. MM is also frequently associated with thrombotic complications; however, it may rarely present with bleeding diathesis. We report a case of a 42-year-old gentleman with relapsed immunoglobulin G lambda MM who presented with epistaxis, gingival bleeding, and oozing at the venepuncture site. Routine tests of coagulation revealed a prolonged prothrombin time (PT), activated partial thromboplastin time (aPTT), and thrombin time. The PT and aPTT failed to correct with pooled normal plasma and the patient was thus diagnosed to have an acquired heparin-like anticoagulant (HLAC). The source of this HLAC has long been debated, but recent data have demonstrated that this HLAC may be the paraproteins produced by the malignant plasma cells. The patient was treated with intravenous protamine sulfate, repeated cycles of plasma exchange, and a daratumumab-based quadruplet regimen but eventually succumbed to an intracranial hemorrhage. HLAC is a rare but potentially fatal complication of MM that must be considered when patients with MM present with bleeding diathesis.
{"title":"Bleeding Diathesis Secondary to a Heparin-Like Anticoagulant in a Patient with Multiple Myeloma—A Case Report and Review of Literature","authors":"Nihar Desai, Seema Biswas, Dinesh Chandra, Ruchi Gupta, Anshul Gupta, Rajesh Kashyap","doi":"10.1055/s-0043-1769789","DOIUrl":"https://doi.org/10.1055/s-0043-1769789","url":null,"abstract":"Abstract Multiple myeloma (MM) is a clonal plasma cell disorder that commonly presents with anemia, renal failure, hypercalcemia, and lytic bone lesions. MM is also frequently associated with thrombotic complications; however, it may rarely present with bleeding diathesis. We report a case of a 42-year-old gentleman with relapsed immunoglobulin G lambda MM who presented with epistaxis, gingival bleeding, and oozing at the venepuncture site. Routine tests of coagulation revealed a prolonged prothrombin time (PT), activated partial thromboplastin time (aPTT), and thrombin time. The PT and aPTT failed to correct with pooled normal plasma and the patient was thus diagnosed to have an acquired heparin-like anticoagulant (HLAC). The source of this HLAC has long been debated, but recent data have demonstrated that this HLAC may be the paraproteins produced by the malignant plasma cells. The patient was treated with intravenous protamine sulfate, repeated cycles of plasma exchange, and a daratumumab-based quadruplet regimen but eventually succumbed to an intracranial hemorrhage. HLAC is a rare but potentially fatal complication of MM that must be considered when patients with MM present with bleeding diathesis.","PeriodicalId":13513,"journal":{"name":"Indian Journal of Medical and Paediatric Oncology","volume":"1 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-09-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135149171","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}
Nachman et al in the year 1998 published the results of the Children's Cancer Group (CCG)-1882 study in which they explored the role of augmented intensive postinduction therapy among children with high-risk acute lymphoblastic leukemia (ALL).[1] Following this study, the augmented Berlin-Frankfurt-Munster (BFM) consolidation which consists of additional vincristine and asparaginase during periods of myelosuppression of the standard IB phase became the preferred postinduction therapy for high-risk ALL across several cooperative groups. Nearly 25 years later, the intercontinental BFM group have published results of the BFM-2009 study, which aimed to address a similar question.[2] In the BFM-2009 study, patients belonging to the intermediate-/high-risk group were randomized following induction therapy to either the standard IB phase or the augmented IB phase. The results of this study showed no difference in relapse incidence (19.1% vs. 20.5%; p = 0.55) or overall survival (OS) (81.9% vs. 80.3%; p = 0.46) between the standard IB and augmented IB phases, respectively. Further, a subgroup analysis failed to demonstrate an impact of the augmented regimen on either risk group (intermediate-risk or high-risk) or immunophenotype (B-ALL or T-ALL). In addition, the incidence of allergic reactions to asparaginase, infections, and pancreatitis were higher in the augmented IB arm. In contrast to the results of the BFM-2009 study, the CCG-1882 randomized study showed a significant improvement in survival following postinduction augmentation of therapy.[1] So, what accounted for the disparity between the two studies? First, the CCG-1882 study included National Cancer Institute (NCI) high-risk (age ≥ 10 years or total leukocyte count [TLC] ≥ 50 × 109 /L) patients with poor early response defined as > 25% marrow blasts on day 7, whereas the BFM-2009 study employed a different age (6 years) and TLC cutoff (≥ 20 × 109/L) in addition to day 15 measurable residual disease (MRD) and adverse cytogenetics to define risk groups eligible for randomization. Moreover, the CCG-1882 study extended intensification not only to the IB phase but also to the interim maintenance and delayed intensification phases, while the BFM-2009 study limited augmentation to the IB phase. A significant difference was also observed in the central nervous system (CNS) prophylaxis between the two studies. The BFM-2009 study restricted cranial radiotherapy (CRT) exclusively for CNS-3 disease and offered high-dose methotrexate (HDMTX) to all patients, whereas the CCG-1882 offered Capizzi I escalating intravenous MTX without leucovorin rescue plus asparaginase and prophylactic CRT for all patients. The promising results of the CCG-1882 study led to a similar randomized study (CCG-1961) but among NCI high-risk patients with rapid early response (< 25% marrow blasts on day 7), which again concluded in favor of the augmented regimen.[3] More recently, the Medical Research Council group in their UKALL-20
Nachman等人于1998年发表了儿童癌症组(CCG)-1882研究的结果，在该研究中，他们探讨了增强强化诱导后治疗在高风险急性淋巴细胞白血病(ALL)儿童中的作用在这项研究之后，在标准IB期骨髓抑制期间，由额外的长春新碱和天冬酰胺酶组成的增强柏林-法兰克福-明斯特(BFM)巩固在几个合作组中成为高危ALL诱导后治疗的首选方法。近25年后，洲际BFM小组发表了BFM-2009研究的结果，旨在解决类似的问题在BFM-2009研究中，属于中/高危组的患者在诱导治疗后随机分为标准IB期或增强IB期。本研究结果显示复发率无差异(19.1% vs 20.5%;p = 0.55)或总生存期(OS) (81.9% vs. 80.3%;p = 0.46)，分别为标准IB期和增强IB期。此外，亚组分析未能证明增强方案对风险组(中度风险或高风险)或免疫表型(B-ALL或T-ALL)的影响。此外，对天冬酰胺酶的过敏反应、感染和胰腺炎的发生率在增强IB组更高。与BFM-2009研究的结果相反，CCG-1882随机研究显示，诱导后强化治疗后生存率显著提高那么，是什么导致了两项研究之间的差异呢?首先，CCG-1882研究纳入了美国国家癌症研究所(NCI)高风险(年龄≥10岁或总白细胞计数[TLC]≥50 × 109/L)早期反应差的患者，第7天定义为骨髓原细胞为> 25%，而BFM-2009研究采用了不同的年龄(6岁)和TLC截止值(≥20 × 109/L)，以及第15天可测量的残留疾病(MRD)和不良细胞遗传学来定义符合随机化条件的风险组。此外，CCG-1882研究不仅将强化扩展到IB期，还将强化扩展到中期维持期和延迟强化期，而BFM-2009研究将强化限制到IB期。两项研究在中枢神经系统(CNS)预防方面也观察到显著差异。BFM-2009研究仅限制CNS-3疾病的颅放射治疗(CRT)，并为所有患者提供高剂量甲氨蝶呤(HDMTX)，而CCG-1882研究为所有患者提供Capizzi I级静脉注射MTX，不含亚叶酸钙挽救加天冬酰胺酶和预防性CRT。CCG-1882研究的令人鼓舞的结果导致了一项类似的随机研究(CCG-1961)，但在NCI高风险患者中进行了快速的早期反应(第7天骨髓细胞< 25%)，该研究再次得出有利于增强方案的结论最近，医学研究委员会小组在他们的UKALL-2003试验中也以随机方式研究了诱导后增强在高风险ALL患者中的作用在这项研究中，根据NCI风险和第8天反应定义的标准中危患者，如果在诱导第29天MRD超过0.01%，则随机接受强化诱导后治疗或风险特异性标准治疗。在这种情况下，增强治疗包括在巩固期和延迟强化期额外8剂量的聚乙二醇化天冬酰胺酶和额外18剂量的长春新碱，以及Capizzi I升级MTX。研究结果显示，在接受强化治疗的患者中，无事件生存率(89.6% vs. 82.8%， p = 0.04)和OS (92.9% vs. 88%， p = 0.16)更高。虽然这项研究强调了在诱导结束时对顽固性疾病患者进行强化治疗的重要性，但它并没有明确确定增强BFM巩固的益处先前的几项研究表明，在B细胞和t细胞ALL中，在第78天(巩固期结束)实现MRD清除的重要性。在COG AALL0232研究中，所有高危B-ALL患者均接受四种药物诱导，随后进行强化IB巩固在本研究评估的2473例MRD患者中，685例患者在诱导结束时MRD呈阳性(≥0.01%)，而只有一小部分患者(n = 45)在巩固结束时MRD仍呈阳性这表明，对于基于第29天MRD定义的高风险ALL患者，采用增强IB巩固可能是合理的，因为它具有更有效清除MRD的潜力。BFM-2009研究的一个局限性是缺乏第29天的MRD细节，这可能揭示了MRD阳性疾病患者接受BFM骨干化疗增强IB巩固的益处。 同样值得注意的是，COG AALL0232研究中的患者在中期维持期间随机接受Capizzi I MTX或HDMTX, HDTMX的效果(与Capizzi I MTX相比)在MRD阳性患者中更为明显。根据COG ALL0232研究的数据和UKALL-2011研究的最新发现，HDMTX可能在缓解骨髓疾病方面发挥重要作用，其益处可能不仅限于避难所
{"title":"To Augment or Not to Augment Consolidation Therapy for High-Risk Childhood Acute Lymphoblastic Leukemia","authors":"Shyam Srinivasan","doi":"10.1055/s-0043-1774778","DOIUrl":"https://doi.org/10.1055/s-0043-1774778","url":null,"abstract":"Nachman et al in the year 1998 published the results of the Children's Cancer Group (CCG)-1882 study in which they explored the role of augmented intensive postinduction therapy among children with high-risk acute lymphoblastic leukemia (ALL).[1] Following this study, the augmented Berlin-Frankfurt-Munster (BFM) consolidation which consists of additional vincristine and asparaginase during periods of myelosuppression of the standard IB phase became the preferred postinduction therapy for high-risk ALL across several cooperative groups. Nearly 25 years later, the intercontinental BFM group have published results of the BFM-2009 study, which aimed to address a similar question.[2] In the BFM-2009 study, patients belonging to the intermediate-/high-risk group were randomized following induction therapy to either the standard IB phase or the augmented IB phase. The results of this study showed no difference in relapse incidence (19.1% vs. 20.5%; p = 0.55) or overall survival (OS) (81.9% vs. 80.3%; p = 0.46) between the standard IB and augmented IB phases, respectively. Further, a subgroup analysis failed to demonstrate an impact of the augmented regimen on either risk group (intermediate-risk or high-risk) or immunophenotype (B-ALL or T-ALL). In addition, the incidence of allergic reactions to asparaginase, infections, and pancreatitis were higher in the augmented IB arm. In contrast to the results of the BFM-2009 study, the CCG-1882 randomized study showed a significant improvement in survival following postinduction augmentation of therapy.[1] So, what accounted for the disparity between the two studies? First, the CCG-1882 study included National Cancer Institute (NCI) high-risk (age ≥ 10 years or total leukocyte count [TLC] ≥ 50 × 109 /L) patients with poor early response defined as > 25% marrow blasts on day 7, whereas the BFM-2009 study employed a different age (6 years) and TLC cutoff (≥ 20 × 109/L) in addition to day 15 measurable residual disease (MRD) and adverse cytogenetics to define risk groups eligible for randomization. Moreover, the CCG-1882 study extended intensification not only to the IB phase but also to the interim maintenance and delayed intensification phases, while the BFM-2009 study limited augmentation to the IB phase. A significant difference was also observed in the central nervous system (CNS) prophylaxis between the two studies. The BFM-2009 study restricted cranial radiotherapy (CRT) exclusively for CNS-3 disease and offered high-dose methotrexate (HDMTX) to all patients, whereas the CCG-1882 offered Capizzi I escalating intravenous MTX without leucovorin rescue plus asparaginase and prophylactic CRT for all patients. The promising results of the CCG-1882 study led to a similar randomized study (CCG-1961) but among NCI high-risk patients with rapid early response (< 25% marrow blasts on day 7), which again concluded in favor of the augmented regimen.[3] More recently, the Medical Research Council group in their UKALL-20","PeriodicalId":13513,"journal":{"name":"Indian Journal of Medical and Paediatric Oncology","volume":"5 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-09-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135150386","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}
Debanjan Sikdar, Sweety Gupta, Ravi Roshan, Shruti Agarwal, Deepa M. Joseph, Manoj Gupta
Abstract Intracranial extraskeletal mesenchymal chondrosarcoma, which is characterized by undifferentiated mesenchymal cells in the presence of occasional pockets of mature hyaline cartilage, is rare in our clinical practice and commonly seen in young adults. In the pediatric population, only a few cases have been reported. In this article, we describe a case of primary recurrent intracranial mesenchymal chondrosarcoma in an 11-year-old boy well treated by surgery, radiation, and chemotherapy. We also reviewed all previously published reports on pediatric patients on the basis of their manifestations and management.
{"title":"Primary Intracranial Extraskeletal Mesenchymal Chondrosarcoma of the Brain in a Pediatric Patient: A Case Report and Review of Literature","authors":"Debanjan Sikdar, Sweety Gupta, Ravi Roshan, Shruti Agarwal, Deepa M. Joseph, Manoj Gupta","doi":"10.1055/s-0043-1770925","DOIUrl":"https://doi.org/10.1055/s-0043-1770925","url":null,"abstract":"Abstract Intracranial extraskeletal mesenchymal chondrosarcoma, which is characterized by undifferentiated mesenchymal cells in the presence of occasional pockets of mature hyaline cartilage, is rare in our clinical practice and commonly seen in young adults. In the pediatric population, only a few cases have been reported. In this article, we describe a case of primary recurrent intracranial mesenchymal chondrosarcoma in an 11-year-old boy well treated by surgery, radiation, and chemotherapy. We also reviewed all previously published reports on pediatric patients on the basis of their manifestations and management.","PeriodicalId":13513,"journal":{"name":"Indian Journal of Medical and Paediatric Oncology","volume":"32 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-09-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135553041","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}
Abstract T-cell lymphomas are rare neoplasms that have complex pathology. The multiparameter approach has been recommended by World Health Organization (WHO) for the classification of T-cell lymphomas taking into account morphology, immunophenotype, genetics, and clinical features. This also includes division established on the possible cell-of-origin (COO) from T regulatory or T-follicular helper (TFH) cells. The recent WHO-HAEM5 has classified entities as precursor T-lymphoblastic neoplasms, mature T-cell neoplasms, Epstein-Barr virus (EBV)-related T- and NK/T-cell lymphomas, and tumor-like lesions with T-cells predominance. Distinct entities have been recognized within the anaplastic large cell lymphoma (ALCL) family founded on the status of anaplastic lymphoma kinase (ALK) gene rearrangement: ALK-positive and molecularly heterogeneous ALK-negative. The family of lymphomas arising from TFH cells consists of three distinct nodal TFH cell lymphoma entities: angioimmunoblastic-type, follicular-type, and not otherwise specified. These three entities show significant clinical and immunophenotypic overlap. The cases that do not qualify for ALCL or nodal TFH cell lymphomas are labelled as peripheral T-cell lymphomas-not otherwise specified after ruling out nodal EBV-positive T- and NK- cell lymphoma. The new category termed tumor-like lesions with T cell predominance has a high chance to be misdiagnosed as lymphoma. This category includes entities such as Kikuchi-Fujimoto disease, indolent T-lymphoblastic proliferation, and autoimmune lymphoproliferative syndrome. For pathologists, diagnosing nodal T-cell lymphomas may be thought-provoking due to their broad histopathologic spectrum that mimics reactive as well as other neoplastic processes. This review provides a comprehensive diagnostic criterion of the most commonly encountered nodal T-cell and NK cell lymphomas in day-to-day training and an algorithmic approach.
{"title":"An Approach to Nodal T- and NK-Cell Lymphomas—A Systemic Review","authors":"Mayur Parkhi, A. Bal","doi":"10.1055/s-0043-1772192","DOIUrl":"https://doi.org/10.1055/s-0043-1772192","url":null,"abstract":"Abstract T-cell lymphomas are rare neoplasms that have complex pathology. The multiparameter approach has been recommended by World Health Organization (WHO) for the classification of T-cell lymphomas taking into account morphology, immunophenotype, genetics, and clinical features. This also includes division established on the possible cell-of-origin (COO) from T regulatory or T-follicular helper (TFH) cells. The recent WHO-HAEM5 has classified entities as precursor T-lymphoblastic neoplasms, mature T-cell neoplasms, Epstein-Barr virus (EBV)-related T- and NK/T-cell lymphomas, and tumor-like lesions with T-cells predominance. Distinct entities have been recognized within the anaplastic large cell lymphoma (ALCL) family founded on the status of anaplastic lymphoma kinase (ALK) gene rearrangement: ALK-positive and molecularly heterogeneous ALK-negative. The family of lymphomas arising from TFH cells consists of three distinct nodal TFH cell lymphoma entities: angioimmunoblastic-type, follicular-type, and not otherwise specified. These three entities show significant clinical and immunophenotypic overlap. The cases that do not qualify for ALCL or nodal TFH cell lymphomas are labelled as peripheral T-cell lymphomas-not otherwise specified after ruling out nodal EBV-positive T- and NK- cell lymphoma. The new category termed tumor-like lesions with T cell predominance has a high chance to be misdiagnosed as lymphoma. This category includes entities such as Kikuchi-Fujimoto disease, indolent T-lymphoblastic proliferation, and autoimmune lymphoproliferative syndrome. For pathologists, diagnosing nodal T-cell lymphomas may be thought-provoking due to their broad histopathologic spectrum that mimics reactive as well as other neoplastic processes. This review provides a comprehensive diagnostic criterion of the most commonly encountered nodal T-cell and NK cell lymphomas in day-to-day training and an algorithmic approach.","PeriodicalId":13513,"journal":{"name":"Indian Journal of Medical and Paediatric Oncology","volume":" ","pages":""},"PeriodicalIF":0.2,"publicationDate":"2023-08-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"42126965","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}
A. Bapna, S. Patni, N. Patni, A. Gupta, A. Samar, Naresh Ledwani, T. Gupta, P. Agarwal
Abstract Introduction Risk assessment by various methods for HR +/HER2– early-stage breast cancer (EBC) patients help clinicians stratify risk and tailor individual treatment. Multiple prognostic tests are available, both free and expensive. Free prognostic tools, the Nottingham Prognostic Index (NPI), and modified Adjuvant Online (mAOL) rely on clinical parameters. CanAssist Breast (CAB) considers both clinical parameters and tumor biology for assessing the risk of recurrence. Objectives The objective is to assess risk by CAB, NPI, and mAOL and discern the differences in the risk stratification in the EBC cohort of Bhagwan Mahaveer Cancer Hospital and Research Centre, Jaipur, Rajasthan, India. Methods Study cohort comprises 100 patients. Risk concordance was assessed by the kappa correlation coefficient and restratification analysis between risk groups of CAB, NPI, and mAOL was assessed using a two-sided p -value. Results Cohort was predominated by patients aged above 50, with T2/N0/G2 tumors. Low-risk (LR) and high-risk (HR) proportions by CAB, NPI, and mAOL were 67:33, 19:81, and 14:86, respectively. Across both age groups, CAB stratified more patients as LR compared with NPI and mAOL. In subgroups of patients with N0, G2, and T2 tumors, CAB identified significantly ( p < 0.0001) higher (3–8 times) patients as LR than NPI and mAOL. In patients with T1/G1 tumors, risk proportions were similar by all three tools. Interestingly, CAB LR (57%) was four times that of NPI (14%) in the N1 subgroup. In G3 tumors CAB LR was 13%. mAOL failed to identify LR in the N1 and G3 subgroups and NPI in the G3 subgroup. There was poor agreement between CAB and NPI/mAOL (k 0.14 [95% confidence interval: 0.03–0.24]/0.11 [0.02–0.20]). Up to 11% of mAOL/NPI LR were detected as HR by CAB and up to 63% of mAOL and NPI HR as LR by CAB. Conclusion Prognostication by tools that use clinical parameters alone might be inadequate. Prognostication using CAB that integrates critical biomarkers indicative of tumor biology along with clinical parameters could be significant. The earlier published data on CAB across various ethnic cohorts and its comparable performance with Oncotype DX makes CAB a relevant prognostic test in HR +/HER2– EBC to make decisions on chemotherapy use.
{"title":"Risk Stratification of Early Breast Cancer (HR +/HER 2–) by CanAssist Breast and Its Corelation with Other Online Prognostic Tools: Experience from a Single Center","authors":"A. Bapna, S. Patni, N. Patni, A. Gupta, A. Samar, Naresh Ledwani, T. Gupta, P. Agarwal","doi":"10.1055/s-0043-1771404","DOIUrl":"https://doi.org/10.1055/s-0043-1771404","url":null,"abstract":"Abstract Introduction Risk assessment by various methods for HR +/HER2– early-stage breast cancer (EBC) patients help clinicians stratify risk and tailor individual treatment. Multiple prognostic tests are available, both free and expensive. Free prognostic tools, the Nottingham Prognostic Index (NPI), and modified Adjuvant Online (mAOL) rely on clinical parameters. CanAssist Breast (CAB) considers both clinical parameters and tumor biology for assessing the risk of recurrence. Objectives The objective is to assess risk by CAB, NPI, and mAOL and discern the differences in the risk stratification in the EBC cohort of Bhagwan Mahaveer Cancer Hospital and Research Centre, Jaipur, Rajasthan, India. Methods Study cohort comprises 100 patients. Risk concordance was assessed by the kappa correlation coefficient and restratification analysis between risk groups of CAB, NPI, and mAOL was assessed using a two-sided p -value. Results Cohort was predominated by patients aged above 50, with T2/N0/G2 tumors. Low-risk (LR) and high-risk (HR) proportions by CAB, NPI, and mAOL were 67:33, 19:81, and 14:86, respectively. Across both age groups, CAB stratified more patients as LR compared with NPI and mAOL. In subgroups of patients with N0, G2, and T2 tumors, CAB identified significantly ( p < 0.0001) higher (3–8 times) patients as LR than NPI and mAOL. In patients with T1/G1 tumors, risk proportions were similar by all three tools. Interestingly, CAB LR (57%) was four times that of NPI (14%) in the N1 subgroup. In G3 tumors CAB LR was 13%. mAOL failed to identify LR in the N1 and G3 subgroups and NPI in the G3 subgroup. There was poor agreement between CAB and NPI/mAOL (k 0.14 [95% confidence interval: 0.03–0.24]/0.11 [0.02–0.20]). Up to 11% of mAOL/NPI LR were detected as HR by CAB and up to 63% of mAOL and NPI HR as LR by CAB. Conclusion Prognostication by tools that use clinical parameters alone might be inadequate. Prognostication using CAB that integrates critical biomarkers indicative of tumor biology along with clinical parameters could be significant. The earlier published data on CAB across various ethnic cohorts and its comparable performance with Oncotype DX makes CAB a relevant prognostic test in HR +/HER2– EBC to make decisions on chemotherapy use.","PeriodicalId":13513,"journal":{"name":"Indian Journal of Medical and Paediatric Oncology","volume":" ","pages":""},"PeriodicalIF":0.2,"publicationDate":"2023-08-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"42652290","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}
Suvir Singh, Rintu Sharma, Jagdeep K. Singh, P. Paul
Abstract Bone marrow fibrosis with lymphoproliferative disorders is rare with the exception of hairy cell leukemia and nodular sclerosis Hodgkin's lymphoma. We report the case of a 63-year-old gentleman with indolent B-cell lymphoma presenting with myelofibrosis and aplasia. He was evaluated for pancytopenia with no organomegaly or lymphadenopathy. Bone marrow aspiration was a dry tap and biopsy revealed a hypocellular marrow with a cellularity of 10 to 20% with absent megakaryocytes and grade 2 to 3 reticulin fibrosis. Myeloproliferative neoplasms were ruled out based on morphology and absence of myeloid mutations on next-generation sequencing. Further sections revealed interstitial infiltrates of lymphoid cells with round, clumped chromatin and inconspicuous nucleoli, which on immunohistochemistry (IHC) were positive for CD20 and BCL2, and negative for CD5, CD10, BCL6, annexin A1, cyclin D1, and TdT. The final diagnosis was thus confirmed as CD5-negative low-grade B-cell lymphoma and he was initiated on therapy with a combination of Bendamustine and Rituximab. He had resolution of symptoms and cytopenia after six cycles of the same. Presence of significant myelofibrosis on the background of a hypocellular marrow can mimic several subtypes of myeloproliferative neoplasms or myelodysplastic syndromes, providing a diagnostic challenge. IHC is essential in determining the exact subtype to decide further therapy. Based on literature search, only a handful of patients with this presentation have been described and this case will be a valuable addition to the same.
{"title":"Deceptive Presentation of Low-Grade Lymphoma with Grade 3 Marrow Fibrosis and Aplasia: Diagnostic and Clinical Considerations","authors":"Suvir Singh, Rintu Sharma, Jagdeep K. Singh, P. Paul","doi":"10.1055/s-0043-1771264","DOIUrl":"https://doi.org/10.1055/s-0043-1771264","url":null,"abstract":"Abstract Bone marrow fibrosis with lymphoproliferative disorders is rare with the exception of hairy cell leukemia and nodular sclerosis Hodgkin's lymphoma. We report the case of a 63-year-old gentleman with indolent B-cell lymphoma presenting with myelofibrosis and aplasia. He was evaluated for pancytopenia with no organomegaly or lymphadenopathy. Bone marrow aspiration was a dry tap and biopsy revealed a hypocellular marrow with a cellularity of 10 to 20% with absent megakaryocytes and grade 2 to 3 reticulin fibrosis. Myeloproliferative neoplasms were ruled out based on morphology and absence of myeloid mutations on next-generation sequencing. Further sections revealed interstitial infiltrates of lymphoid cells with round, clumped chromatin and inconspicuous nucleoli, which on immunohistochemistry (IHC) were positive for CD20 and BCL2, and negative for CD5, CD10, BCL6, annexin A1, cyclin D1, and TdT. The final diagnosis was thus confirmed as CD5-negative low-grade B-cell lymphoma and he was initiated on therapy with a combination of Bendamustine and Rituximab. He had resolution of symptoms and cytopenia after six cycles of the same. Presence of significant myelofibrosis on the background of a hypocellular marrow can mimic several subtypes of myeloproliferative neoplasms or myelodysplastic syndromes, providing a diagnostic challenge. IHC is essential in determining the exact subtype to decide further therapy. Based on literature search, only a handful of patients with this presentation have been described and this case will be a valuable addition to the same.","PeriodicalId":13513,"journal":{"name":"Indian Journal of Medical and Paediatric Oncology","volume":" ","pages":""},"PeriodicalIF":0.2,"publicationDate":"2023-08-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"48941060","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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