Harahap Agnes Stephanie, Santoso Ivana, Charles Stefanny, Mulansari Nadia Ayu, Ham Maria Francisca
Anatomical Pathology Department, Faculty of Medicine, Universitas Indonesia/Dr. Cipto Mangunkusumo National Central General Hospital, Jakarta, 10430, Indonesia.
Human Cancer Research Center-Indonesian Medical Education and Research Institute, Faculty of Medicine, Universitas Indonesia, Jakarta, 10430, Indonesia.
BMC Res Notes. 2025 Jul 9;18(1):282. doi: 10.1186/s13104-025-07354-5.
Anaplastic large cell lymphoma (ALCL) is a rare and aggressive CD30-positive non-Hodgkin lymphoma with histopathological features overlapping Hodgkin and T-cell lymphomas. ALK-positive ALCL shows a better prognosis than ALK-negative cases, which are often associated with advanced disease. This study evaluates the epidemiological profile of ALCL in Indonesian population and its distinct histopathological characteristics and immunohistochemical expression patterns.
Among 93 ALCL cases (2013-2023) enrolled at Dr. Cipto Mangunkusumo Hospital, 13.9% were primary cutaneous ALCL, while 86.1% were systemic ALCL (consisted of 53.7% ALK-positive and 46.3% ALK-negative). ALK-positive patients were older (p = 0.009), with earlier-stage disease (p = 0.032) and nodal predilection (p = 0.032). ALK-negative cases demonstrated a shorter median survival of 23 months compared to ALK-positive cases of 28 months. Poor outcomes were associated with B symptoms and high Eastern Cooperative Oncology Group Performance Status (ECOG-PS) scores. Given that this study was conducted at a single, government-operated tertiary care teaching hospital in Indonesia's capital, validation through multicenter prospective studies is warranted to further refine diagnostic and therapeutic strategies.
间变性大细胞淋巴瘤(ALCL)是一种罕见且侵袭性强的CD30阳性非霍奇金淋巴瘤,其组织病理学特征与霍奇金淋巴瘤和T细胞淋巴瘤重叠。ALK阳性的ALCL预后优于ALK阴性病例,后者常与晚期疾病相关。本研究评估了印度尼西亚人群中ALCL的流行病学特征及其独特的组织病理学特征和免疫组化表达模式。
在Cipto Mangunkusumo医院登记的93例ALCL病例(2013 - 2023年)中,13.9%为原发性皮肤ALCL,而86.1%为系统性ALCL(其中53.7%为ALK阳性,46.3%为ALK阴性)。ALK阳性患者年龄较大(p = 0.009),疾病分期较早(p = 0.032)且倾向于累及淋巴结(p = 0.032)。与ALK阳性病例的中位生存期28个月相比,ALK阴性病例的中位生存期较短,为23个月。不良预后与B症状和东部肿瘤协作组体能状态(ECOG - PS)评分高有关。鉴于本研究是在印度尼西亚首都一家政府运营的三级护理教学医院进行的,有必要通过多中心前瞻性研究进行验证,以进一步完善诊断和治疗策略。