Chang J C, Aly E S
Department of Medicine, Wright State University School of Medicine, Good Samaritan Hospital, Dayton, Ohio 45406, USA.
Am J Med Sci. 2001 Feb;321(2):124-8. doi: 10.1097/00000441-200102000-00003.
Although the classical triad of thrombocytopenia, microangiopathic hemolytic anemia, and neurological changes has been well recognized, thrombotic thrombocytopenic purpura (TTP) sometimes presents with atypical clinical features. Some cases of acute respiratory distress syndrome (ARDS) were suspected to be a manifestation of TTP.
We examined retrospectively the medical records of patients with known TTP from 1981 to 1998. The patients who developed ARDS simultaneously with TTP were identified. Clinical, laboratory, and other pertinent data were reviewed; the clinical outcome was documented.
Seven out of 56 patients with an established diagnosis of TTP were identified as having ARDS. All 7 patients had progressive dyspnea, persistent hypoxemia with diffuse pulmonary infiltrates, and no evidence of localized pneumonia, pulmonary emboli, congestive heart failure, or other identifiable lung diseases. In addition to ARDS, 4 patients developed progressive neurological (mental) changes, 3 developed fever, and 2 developed renal failure. Six patients were treated with ventilatory support in the ICU. Upon establishing the diagnosis of TTP, 6 patients were treated with exchange plasmapheresis (EP) and 1 patient with an infusion of fresh frozen plasma. Within 2 days of initiation of EP, 4 patients showed marked respiratory improvement; shortly thereafter, mechanical ventilation was ended. Complete and long-lasting remission was achieved in all 4 patients. Three patients died because of delayed diagnosis and untimely employment of EP.
Life-threatening ARDS can be associated with TTP. It is recommended that all patients with ARDS and unexplained thrombocytopenia be evaluated for unrecognized TTP. Early recognition of ARDS associated with TTP and timely EP is critical to a favorable outcome.
尽管血小板减少、微血管病性溶血性贫血和神经学改变的经典三联征已广为人知，但血栓性血小板减少性紫癜（TTP）有时会表现出非典型临床特征。一些急性呼吸窘迫综合征（ARDS）病例被怀疑是TTP的一种表现形式。
我们回顾性研究了1981年至1998年已知患有TTP患者的病历。确定了同时发生ARDS的TTP患者。回顾了临床、实验室及其他相关数据；记录了临床结局。
在确诊为TTP的56例患者中，有7例被确定患有ARDS。所有7例患者均有进行性呼吸困难、持续性低氧血症伴弥漫性肺部浸润，且无局限性肺炎、肺栓塞、充血性心力衰竭或其他可识别肺部疾病的证据。除ARDS外，4例患者出现进行性神经（精神）改变，3例发热，2例出现肾衰竭。6例患者在重症监护病房接受通气支持治疗。确诊TTP后，6例患者接受了血浆置换（EP）治疗，1例患者输注了新鲜冷冻血浆。在开始EP治疗的2天内，4例患者呼吸明显改善；此后不久，机械通气结束。所有4例患者均实现了完全且持久的缓解。3例患者因诊断延迟和未及时采用EP治疗而死亡。
危及生命的ARDS可能与TTP相关。建议对所有患有ARDS且血小板减少原因不明的患者进行评估，以排查未被识别的TTP。早期识别与TTP相关的ARDS并及时进行EP治疗对取得良好结局至关重要。
