Respiratory dysfunction in thrombotic thrombocytopenic purpura.

Thrombotic thrombocytopenic purpura is a rare disease characterized by microangiopathic hemolytic anemia, thrombocytopenia, neurologic abnormalities, fever and renal dysfunction. in six of seven consecutive patients with thrombotic thrombocytopenic purpura seen in an eight month period, respiratory impairment was present. Respiratory dysfunction was characterized by tachypnea, hypoxemia nad infiltrates on chest roentgenogram. Five patients required mechanical ventilation. Two patients had cardiogenic pulmonary edema, but they remained hypoxemic despite treatment for pulmonary edema and maintenance of normal pulmonary capillary wedge pressure for more than 36 hours. Four patients died and autopsies revealed pulmonary edema, hemorrhage and hyaline thrombi. Pathologic examination of the heart also showed hyaline thrombi. Information from out patients with thrombotic thrombocytopenic purpura implicates respiratory dysfunction as a component of this disease as well as the classically described pentad. Cardiogenic and noncardiogenic pulmonary edema and possibly bleeding into the lung contributed to pulmonary impairment.