The differentiation syndrome is an inflammatory reaction with increased capillary permeability that occurs in up to 25% of patients with acute promyelocytic leukemia treated with all-trans retinoic acid. A 50-year-old...The differentiation syndrome is an inflammatory reaction with increased capillary permeability that occurs in up to 25% of patients with acute promyelocytic leukemia treated with all-trans retinoic acid. A 50-year-old man with acute promyelocytic leukemia underwent chemotherapy with idarubicin and all-trans retinoic acid. On day +21 the patient developed pruritic prepatelar papules as well as several 10 mm subcutaneous nodules in both thighs accompanied by persistent fever. On the day +25 the patient presented with bilateral pulmonary crackles, infiltrates in the right lower lobe and severe hypotension which required dopamine infusion. Biopsy of one of the thighs nodules was performed. A Sweet syndrome associated to a differentiation syndrome was suspected. All-trans retinoic acid therapy was discontinued and dexamethasone was administered. In 48 h the patient showed remission of the fever and the infiltrates and the skin lesions acquired a residual aspect. It is debatable whether these two syndromes are distinct entities with common mechanisms or whether they are poles of the same spectrum. Dermatologists and hematologists must be aware of these two syndromes and its pathophysiologic association.展开更多
文摘目的:筛选预测初发高危急性早幼粒细胞白血病(acute promyelocytic leukemia,APL)发生分化综合征的因素,比较翻倍白细胞(WBC_(Double))与峰值白细胞(WBC_(Peak))的预测价值。方法:回顾性分析我院血液科收治的78例经亚砷酸诱导治疗的初诊高危APL患者的临床特征,比较WBC_(Double)和WBC_(Peak)分化综合征的分化程度。结果:分化综合征的发生率为57.69%(45/78),分化综合征最常见的临床症状为发热,重度分化综合征组发热、肺部浸润、心包积液发生率明显高于轻度分化综合征组,差异有统计学意义(P<0.05)。高危APL患者分化综合征的发生与化疗后WBC_(Peak)(45.21×10^(9)/L vs 25.24×10^(9)/L,P<0.001)和化疗后WBC_(Double)(37.59×10^(9)/L vs 17.46×10^(9)/L,P=0.007)有关。与WBC_(Peak)组比较,WBC_(Double)组早于分化事件的发生率高(68.57%vs 40.00%,P=0.016),2组间重度分化、轻度分化和未分化差异均有统计学意义(P=0.002);WBC_(Peak)组出现4个以上分化综合征症状的患者比例更多(28.57%vs 5.71%,P=0.012)。与WBCNO-_(Double)组比较,WBC_(Double)组患者诊断时白细胞计数(P=0.003)、谷丙转氨酶(P=0.040)、外周血早幼粒细胞数(P=0.047)差异有统计学意义。结论:化疗后WBC_(Double)和化疗后WBC_(Peak)是分化综合征的危险因素,化疗后WBC_(Double)较WBC_(Peak)提示分化综合征的发生更有优势。
文摘The differentiation syndrome is an inflammatory reaction with increased capillary permeability that occurs in up to 25% of patients with acute promyelocytic leukemia treated with all-trans retinoic acid. A 50-year-old man with acute promyelocytic leukemia underwent chemotherapy with idarubicin and all-trans retinoic acid. On day +21 the patient developed pruritic prepatelar papules as well as several 10 mm subcutaneous nodules in both thighs accompanied by persistent fever. On the day +25 the patient presented with bilateral pulmonary crackles, infiltrates in the right lower lobe and severe hypotension which required dopamine infusion. Biopsy of one of the thighs nodules was performed. A Sweet syndrome associated to a differentiation syndrome was suspected. All-trans retinoic acid therapy was discontinued and dexamethasone was administered. In 48 h the patient showed remission of the fever and the infiltrates and the skin lesions acquired a residual aspect. It is debatable whether these two syndromes are distinct entities with common mechanisms or whether they are poles of the same spectrum. Dermatologists and hematologists must be aware of these two syndromes and its pathophysiologic association.
