Di Micco Lucia, Mirenghi Francesco, Morelli Emanuela, De Simone Emanuele
UOC Nefrologia e Dialisi, Ospedale Del Mare, ASL Napoli 1 Centro, Napoli, Italia.
UOC Nefrologia, Azienda Ospedaliera Dei Colli, Napoli, Italia.
G Ital Nefrol. 2019 Jul 24;36(4):2019-vol4.
Differentiation syndrome (DS), previously known as retinoic acid syndrome or ATRA (all-trans retinoic acid) or ATO (arsenic trioxide) syndrome, is a life-threatening complication of the therapy with differentiating agents in patients with acute promyelocytic leukemia (APL). The latter is a rare subtype of acute myeloid leukemia and represents a hematological emergency. The clinical manifestations of DS, after induction therapy with differentiating agents, include unexplained fever, acute respiratory distress with interstitial pulmonary infiltrates, unexplained hypotension, peripheral edema, congestive heart failure and acute renal failure. The therapy is based on early intravenous administration of high-dose dexamethasone, in order to counteract the cytokine storm responsible for the DS. Among the supportive measures for the management of DS, furosemide (in 87% of patients) and dialysis (12% of patients) are used to manage acute renal failure, peripheral and pulmonary edema. We describe a case of acute renal failure, treated with haemodialysis, in a young patient with APL and an early and severe DS after induction therapy. This is a rare condition, not well known among nephrologists, where early recognition and treatment are crucial for the prognosis.
分化综合征(DS),以前称为维甲酸综合征或全反式维甲酸(ATRA)或三氧化二砷(ATO)综合征,是急性早幼粒细胞白血病(APL)患者使用分化剂治疗时危及生命的并发症。后者是急性髓系白血病的一种罕见亚型,属于血液学急症。使用分化剂进行诱导治疗后,DS的临床表现包括不明原因发热、伴有间质性肺浸润的急性呼吸窘迫、不明原因低血压、外周水肿、充血性心力衰竭和急性肾衰竭。治疗基于早期静脉注射大剂量地塞米松,以对抗引发DS的细胞因子风暴。在DS管理的支持措施中,速尿(87%的患者使用)和透析(12%的患者使用)用于处理急性肾衰竭、外周和肺水肿。我们描述了一例年轻APL患者在诱导治疗后出现早期严重DS并接受血液透析治疗急性肾衰竭的病例。这是一种罕见情况,肾科医生对此了解不多,早期识别和治疗对预后至关重要。