Na Yong Sub, Park Sang-Gon
Department of Pulmonology, Chosun University Hospital, Gwangju, South Korea.
Department of Hemato-Oncology, Chosun University Hospital, Gwangju, South Korea.
Am J Case Rep. 2019 Feb 3;20:146-150. doi: 10.12659/AJCR.912682.
BACKGROUND Tumor lysis syndrome (TLS) is an oncologic emergency resulting from the massive destruction of tumor cells after cytotoxic chemotherapy for chemosensitive malignancies with a high tumor burden. Its clinical manifestations include severe electrolyte disturbances, metabolic acidosis, acute renal failure secondary to urate deposition in the kidney, heart, and skeletal muscle, and nervous system dysfunction. We report an extremely rare case of spontaneous TLS (STLS) in idiopathic primary myelofibrosis (PMF). CASE REPORT A 51-year-old Korean man was admitted to our hospital with general weakness and left-side abdominal pain. The patient was diagnosed with acute urate nephropathy with hyperphosphatemia, hyperkalemia, hypocalcemia, and metabolic acidosis. Splenomegaly was accompanied by leukocytosis and a peripheral blood smear revealed immature granulocytes without blast cells. Bone marrow biopsy showed PMF. Initially, we presumed it was a spontaneous tumor lysis syndrome of PMF. We immediately performed emergency hemodialysis. We concluded that the patient, who had chronic hyperuricemia due to undiagnosed PMF, was recently admitted to the emergency room with STLS due to overwork and dehydration. CONCLUSIONS We present an extremely rare case of STLS in idiopathic PMF. The mechanism of chronic hyperuricemia in our case might be rapid cell turnover due to ineffective erythropoiesis of PMF.
背景 肿瘤溶解综合征（TLS）是一种肿瘤急症，由对具有高肿瘤负荷的化疗敏感恶性肿瘤进行细胞毒性化疗后肿瘤细胞大量破坏所致。其临床表现包括严重的电解质紊乱、代谢性酸中毒、因尿酸盐在肾脏、心脏和骨骼肌沉积继发的急性肾衰竭以及神经系统功能障碍。我们报告了一例原发性骨髓纤维化（PMF）中极为罕见的自发性TLS（STLS）病例。病例报告 一名51岁的韩国男性因全身乏力和左侧腹痛入院。该患者被诊断为急性尿酸盐肾病，伴有高磷血症、高钾血症、低钙血症和代谢性酸中毒。脾肿大伴有白细胞增多，外周血涂片显示有未成熟粒细胞但无原始细胞。骨髓活检显示为PMF。最初，我们推测这是PMF的自发性肿瘤溶解综合征。我们立即进行了紧急血液透析。我们得出结论，该患者因未被诊断出的PMF患有慢性高尿酸血症，近期因过度劳累和脱水而以STLS入住急诊室。结论 我们报告了一例原发性骨髓纤维化中极为罕见的自发性肿瘤溶解综合征病例。本例慢性高尿酸血症的机制可能是PMF无效红细胞生成导致的细胞快速更新。
