Department of Emergency Medicine, Vanderbilt University Medical Center, Nashville, Tennessee.
Department of Internal Medicine, Vanderbilt University Medical Center, Nashville, Tennessee.
J Emerg Med. 2021 Aug;61(2):147-150. doi: 10.1016/j.jemermed.2021.03.002. Epub 2021 May 24.
Sulfhemoglobinemia is a rare dyshemoglobinemia that presents similarly to methemoglobinemia.
An 83-year-old woman with stage IV ovarian cancer presented to the Emergency Department after a near syncopal spell and was found to be cyanotic with a pulse oximetry reading of 71%. Pulse oximetry improved to only the mid-80s range with administration of high-flow oxygen. Her arterial blood gas on supplemental high-flow oxygen demonstrated a PaO of 413 mm Hg and methemoglobin of 1.2%, but also noted the interference of the co-oximetry with sulfhemoglobinemia. Further history revealed that the patient had recently been started on phenazopyridine. The phenazopyridine was stopped, an exchange transfusion was offered but declined, and the patient was discharged to home hospice. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: The diagnosis of sulfhemoglobinemia can be challenging given that routine co-oximetry does not identify it. The clue to the diagnosis is that the cyanotic-appearing patient has a normal or elevated PaO and seems to be less ill than expected, given the degree of cyanosis. Sulfhemoglobinemia does not reverse with the administration of methylene blue.
高铁血红蛋白血症是一种罕见的血红蛋白血症,其表现与高铁血红蛋白血症相似。
一名 83 岁的 IV 期卵巢癌女性在近乎晕厥后到急诊科就诊,发现其发绀,脉搏血氧饱和度读数为 71%。给予高流量吸氧后,脉搏血氧饱和度仅改善至 80 年代中期范围。她在补充高流量氧气时的动脉血气显示 PaO 为 413mmHg,高铁血红蛋白为 1.2%,但也注意到 co-oximetry 受到高铁血红蛋白血症的干扰。进一步的病史显示,患者最近开始使用 phenazopyridine。停用 phenazopyridine,建议进行换血治疗但被拒绝,患者出院至家庭临终关怀。
为什么急诊医生应该了解这一点?:由于常规 co-oximetry 无法识别高铁血红蛋白血症,因此高铁血红蛋白血症的诊断具有挑战性。诊断的线索是,发绀外观的患者 PaO 正常或升高,并且考虑到发绀程度,看起来比预期的病状要轻。高铁血红蛋白血症不会因亚甲蓝的给药而逆转。