Matynia Anna Paulina, Perkins Sherrie L, Li David
a Department of Pathology, University of Utah , Salt Lake City , Utah , United States.
Fetal Pediatr Pathol. 2018 Feb;37(1):7-14. doi: 10.1080/15513815.2017.1379042. Epub 2018 Jan 16.
Lymphomatoid granulomatosis is a EBV-driven lymphoproliferative disorder that has been reported in association with immunodeficiency, but only exceptionally in patients with hematopoietic malignancy.
A 14-year-old boy with trisomy-21 and a history of B-lymphoblastic leukemia/lymphoma (B-ALL) diagnosed 1.5 years prior, on maintenance chemotherapy, presented with fever and respiratory symptoms. Chest X-ray revealed right-lower-lobe consolidation. He was treated for pneumonia but continued to be febrile with worsening respiratory status, with development of additional pulmonary and liver nodules. No infectious etiology was identified. Following nondiagnostic lung and liver biopsies, the largest pulmonary mass was resected. The histopathologic findings were diagnostic of lymphomatoid granulomatosis. There was no residual B-ALL. The patient's status continued to deteriorate and he died shortly thereafter.
Relative immunosuppression due to maintenance therapy for B-ALL can lead to lymphomatoid granulomatosis.
淋巴瘤样肉芽肿病是一种由EB病毒驱动的淋巴增殖性疾病，已报道其与免疫缺陷有关，但仅在造血系统恶性肿瘤患者中罕见。
一名14岁男孩，患有21三体综合征，1.5年前诊断为B淋巴细胞母细胞白血病/淋巴瘤（B-ALL），正在接受维持化疗，出现发热和呼吸道症状。胸部X线显示右下叶实变。他接受了肺炎治疗，但仍持续发热，呼吸状况恶化，出现了更多肺部和肝脏结节。未发现感染病因。在肺和肝脏活检未明确诊断后，切除了最大的肺部肿块。组织病理学检查结果诊断为淋巴瘤样肉芽肿病。没有残留的B-ALL。患者的病情继续恶化，此后不久死亡。
B-ALL维持治疗导致的相对免疫抑制可引发淋巴瘤样肉芽肿病。
