cover of episode Unraveling Lymphoid Malignancies: Leukaemia and Lymphoma Explained with Dr. Joanna Czerwinski

Unraveling Lymphoid Malignancies: Leukaemia and Lymphoma Explained with Dr. Joanna Czerwinski

2024/10/10
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Joanna Czerwinski: 我经常将血液系统恶性肿瘤分为淋巴样和髓样疾病,因为它们起源于造血干细胞,并进一步分为淋巴样和髓样干细胞。淋巴样干细胞产生B细胞、T细胞和自然杀伤细胞,而髓样干细胞产生红细胞、各种粒细胞和血小板。白血病是血液中的癌症,淋巴瘤是淋巴系统中的癌症,两者在骨髓中可能重叠。慢性淋巴细胞白血病是最常见的淋巴样疾病之一,急性淋巴细胞白血病是儿童中最常见的癌症。淋巴样恶性肿瘤的早期症状可能很少,但当病情恶化时,会出现一些症状,例如体重减轻、发烧、盗汗和严重疲劳。患者还会出现反复感染、贫血、血小板减少症和白细胞减少症等症状。简单的全血细胞计数检查通常可以发现淋巴样疾病。急性淋巴细胞白血病通常在发病几周内出现全血细胞减少症和循环中的淋巴母细胞。淋巴样恶性肿瘤的分类取决于细胞在其发育过程中的位置。淋巴瘤有超过80种类型,分为B细胞淋巴瘤和T细胞淋巴瘤,其侵袭性从惰性到侵袭性不等。淋巴样疾病的检查始于全血细胞计数、血涂片和外周血流式细胞术。急性或侵袭性疾病通常在患者就诊前仅持续很短时间,而慢性惰性疾病可能持续数月甚至数年。急性白血病中,淋巴母细胞会挤占骨髓,导致正常细胞生成减少,从而出现再生障碍样表现。急性白血病的分期基于骨髓的分子或基因检测,慢性淋巴细胞白血病的分期基于细胞减少症、淋巴结肿大以及肝脾肿大。淋巴瘤的分期采用Ann Arbor分期系统,基于CT或PET引导下的淋巴结受累情况以及其他器官的侵犯。骨髓瘤的分期采用修订的国际分期系统,基于白蛋白、LDH、基因组学和β2-微球蛋白等指标。大多数淋巴样恶性肿瘤在50岁以上确诊,可能与年龄相关的基因突变有关。化疗、放疗、某些免疫疗法以及自身免疫性疾病都可能增加患淋巴瘤和白血病的风险。吸烟与淋巴样恶性肿瘤之间存在轻微关联。急性淋巴细胞白血病的治疗通常包括数月的化疗,许多患者需要进行同种异体干细胞移植。弥漫大B细胞淋巴瘤的一线治疗是R-CHOP化疗,而滤泡性淋巴瘤的治疗取决于症状和疾病进展情况。滤泡性淋巴瘤是一种较惰性的淋巴瘤,并非所有患者都需要立即进行治疗。伯基特淋巴瘤和滤泡性淋巴瘤可在青少年中发生,但大多数淋巴瘤发生在40岁或50岁以上。霍奇金淋巴瘤和非霍奇金淋巴瘤的区别在于霍奇金细胞或里德-施特伯格细胞的存在。非霍奇金淋巴瘤有80多种类型,主要包括B细胞和T细胞淋巴瘤,自然杀伤细胞淋巴瘤和白血病相对罕见。晚期淋巴瘤可能出现肠梗阻或呼吸窘迫等症状。某些类型的侵袭性淋巴瘤,如伯基特淋巴瘤和弥漫大B细胞淋巴瘤,可能以肠道为原发部位。霍奇金淋巴瘤有五种类型,其中大多数属于经典霍奇金淋巴瘤。霍奇金淋巴瘤的预后通常良好,但取决于患者的健康状况和年龄。非霍奇金淋巴瘤比霍奇金淋巴瘤更为常见。淋巴样恶性肿瘤的治疗方案取决于全血细胞计数、生化指标、活检结果以及患者是否有全身症状。流式细胞术通过激光分析单个细胞来识别细胞克隆。建议由放射科医生进行超声引导下核心活检。在南澳大利亚,骨髓活检在镇静下进行,通常为日间手术。在某些情况下,手术可能需要参与淋巴样恶性肿瘤的治疗。淋巴样恶性肿瘤的治疗方法包括化疗、单克隆抗体、免疫疗法和靶向治疗。利妥昔单抗是一种靶向CD20的单克隆抗体,可用于表达CD20的B细胞恶性肿瘤。在过去十年中,人们越来越关注慢性淋巴细胞白血病的非化疗治疗方法。布鲁顿酪氨酸激酶抑制剂(BTK抑制剂)是一种靶向B细胞受体以抑制增殖和粘附因子的靶向药物。淋巴样恶性肿瘤患者的免疫系统受损,需要关注免疫球蛋白水平、感染预防和疫苗接种。淋巴样恶性肿瘤患者的肿瘤免疫监视功能受损,患第二种恶性肿瘤的风险增加。化疗可导致继发性白血病和骨髓增生异常综合征。单克隆抗体是输注药物,而BTK抑制剂是处方药。目前,基因检测主要用于评估患者的风险状况。淋巴样恶性肿瘤的治疗需要多学科团队(MDT)的合作。慢性淋巴细胞白血病(CLL)的许多病例是偶然发现的,并且许多患者可能终生不需要治疗。大多数淋巴瘤最终需要治疗,但一些惰性淋巴瘤可以在数年内进行观察等待。未来淋巴样恶性肿瘤的治疗可能更加个性化,并可能使用CAR-T疗法和双特异性T细胞接合剂等新技术。

Deep Dive

Chapters
This chapter introduces lymphoid malignancies, encompassing lymphocytic leukemia and lymphoma. It explains that these blood cancers originate from lymphocytes and range in severity. The key difference between leukemia (in the blood) and lymphoma (in the lymphatic system) is clarified.
  • Lymphoid malignancies originate from lymphocytes.
  • Leukemia is a cancer in the blood system.
  • Lymphoma is a cancer in the lymphatic system.

Shownotes Transcript

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Join Dr Gavin Nimon) (Orthopaedic Surgeon) for an enlightening episode with Dr. Joanna Czerwinski, a leading haematologist from Flinders Public Hospital, as she unravels the intricacies of lymphoid malignancies. Get ready to demystify the complexities of lymphocytic leukaemia and lymphoma — two formidable blood cancers that originate from lymphocytes. Dr. Czerwinski clarifies the distinctions between leukaemias, which impact the blood, and lymphomas, affecting the lymphatic system, with a deep dive into chronic lymphocytic leukaemia and acute lymphoblastic leukaemia, the latter being notably the most common cancer in children. Gain insight into how these conditions manifest in clinical practice and the critical symptoms they present, including constitutional symptoms and recurrent infections.The conversation broadens to explore the landscape of lymphoma varieties, focusing on the nuances between diffuse large B-cell lymphoma (DLBCL) and follicular lymphoma. We explore cutting-edge treatment protocols like R-CHOP chemotherapy and delve into the role of innovative therapies including monoclonal antibodies and Bruton tyrosine kinase inhibitors. Dr. Czerwinski sheds light on the vital role of patient immunity and the risks arising from compromised tumor surveillance, providing a thorough examination of the treatment journey and long-term care. Stay informed on the distinctions between Hodgkin's and non-Hodgkin's lymphomas, their prognosis, and the age-related occurrences that can influence outcomes.

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