Saad Eldeen Bakheet Omayma, Yusof Nurasyikin, Raja Zahratul Azma, Ithnin Azlin, Abdul Aziz Suria, Alias Hamidah
Department of Pathology, Faculty of Medicine, Universiti Kebangsaan Malaysia Medical Center, Jalan Yaakob Latiff, Bandar Tun Razak, 56000 Cheras, Kuala Lumpur, Malaysia.
Department of Pediatric, Faculty of Medicine, Universiti Kebangsaan Malaysia Medical Center, Jalan Yaakob Latiff, Bandar Tun Razak, 56000 Cheras, Kuala Lumpur, Malaysia.
Indian J Hematol Blood Transfus. 2016 Jun;32(Suppl 1):262-6. doi: 10.1007/s12288-015-0582-6. Epub 2015 Aug 29.
Secondary sea-blue histiocytosis occurs more frequently than the primary form and occurs consequent to a wide range of metabolic and haematologic disorders including thalassaemia. We report an 18-year-old Chinese boy with transfusion-dependent HbE-beta thalassaemia who complained of pain and swelling at the left iliac crest region for 2 months duration. Physical examination revealed pallor with hepatosplenomegaly. Local examination revealed a huge swelling 12 cm × 12 cm in diameter, firm in consistency and tender. Histopathological examination of the mass revealed an osteosarcoma. His bone marrow aspirate showed numerous sea-blue histiocytes, the cytoplasm of which was closely packed with fine granules that stained blue with May-Grunwald-Giemsa. The nuclei were centrally located in some cells and displaced towards the periphery in other cells. There was no malignant cell infiltration in the marrow. The case is reported due to the co-incidental dual pathology in our patient (HbE-beta thalassaemia and osteosarcoma) and the unusual bone marrow finding of numerous sea-blue histiocytes.
继发性海蓝色组织细胞增多症比原发性更为常见,继发于多种代谢和血液系统疾病,包括地中海贫血。我们报告一名18岁的中国男孩,患有依赖输血的血红蛋白E-β地中海贫血,他主诉左髂嵴区域疼痛和肿胀2个月。体格检查发现面色苍白伴肝脾肿大。局部检查发现一个直径12厘米×12厘米的巨大肿块,质地坚硬且有压痛。肿块的组织病理学检查显示为骨肉瘤。他的骨髓穿刺显示有大量海蓝色组织细胞,其细胞质紧密充满细颗粒,用May-Grunwald-Giemsa染色呈蓝色。细胞核在一些细胞中位于中央,在其他细胞中则移向周边。骨髓中无恶性细胞浸润。报告该病例是因为我们的患者存在巧合的双重病理情况(血红蛋白E-β地中海贫血和骨肉瘤)以及骨髓中发现大量海蓝色组织细胞这一不寻常的情况。