Abdul Rahman Seif-Aldin, Loutfi Karam, Turk Tareq, Rahman Ali Abdul, Kherbek Haidara, Ghanem Abdulmoniem, Alshehabi Zuheir
Faculty of Medicine, Cancer Research Center, Tishreen University, Latakia, Syria.
Department of Pathology, Tishreen University, Latakia, Syria.
Ann Med Surg (Lond). 2022 Jun 25;79:104085. doi: 10.1016/j.amsu.2022.104085. eCollection 2022 Jul.
Anaplastic Large-cell Lymphoma (ALCL) is a rare but aggressive type of NHL that develop from mature post-thymic T-cells. ALCL constitutes approximately 2% of all lymphoid neoplasm. It is typically found among children and young adults, accounting for 10-15% of pediatric NHL, compared to 2% of adult NHL.
A 12-year-old Syrian boy was admitted to our hospital due to epistaxis, anorexia, weight loss and night sweats. The physical examination revealed preauricular, postauricular and submandibular lymphadenopathy. Pathological examination of the biopsy suggested Classical Hodgkin Lymphoma. Later on, Immunohistochemistry staining confirmed the diagnosis of ALK-negative Anaplastic Large Cell Lymphoma.
Systemic ALCL can be categorized into two major groups based on the expression of Anaplastic Lymphoma Kinase (ALK) protein: Systemic ALK + positive and Systemic ALK-negative. The majority of pediatric cases show an overexpression of (ALK), however, pediatric ALK-negative ALCL can occur in rare cases.
The aim of this article is to report a rare case of pediatric ALK-negative anaplastic large cell lymphoma that developed a rapid & aggressive growth within a few months despite the chemotherapy treatment and unfortunately led to the patient's death.
间变性大细胞淋巴瘤（ALCL）是一种罕见但侵袭性强的非霍奇金淋巴瘤，由成熟的胸腺后T细胞发展而来。ALCL约占所有淋巴肿瘤的2%。它通常在儿童和年轻人中出现，占儿童非霍奇金淋巴瘤的10 - 15%，而成人非霍奇金淋巴瘤中仅占2%。
一名12岁的叙利亚男孩因鼻出血、厌食、体重减轻和盗汗入院。体格检查发现耳前、耳后和颌下淋巴结肿大。活检的病理检查提示经典型霍奇金淋巴瘤。后来，免疫组化染色确诊为ALK阴性间变性大细胞淋巴瘤。
系统性ALCL可根据间变性淋巴瘤激酶（ALK）蛋白的表达分为两大类：系统性ALK阳性和系统性ALK阴性。大多数儿科病例显示（ALK）过表达，然而，儿科ALK阴性ALCL在罕见情况下也会发生。
本文旨在报告一例罕见的儿科ALK阴性间变性大细胞淋巴瘤病例，该病例尽管接受了化疗，但在几个月内迅速侵袭性生长，不幸导致患者死亡。
