An Unusual Case of Zero Percent Coagulopathic Factor in a Patient With Polymyalgia Rheumatica.

We present a case of a 74-year-old male with a past medical history of polymyalgia rheumatica that presented as a transfer for evaluation of hematomas of the scrotum, left groin, back, and bilateral thighs. Further questioning revealed hematuria and bleeding gums for the past month. The patient complained of left thigh pain without recent fever, chills, chest pain, or shortness of breath. A physical exam showed hematomas of the left groin, scrotum, bilateral thighs, and back with an ecchymotic appearance.  Initial pertinent laboratory workup showed decreased hemoglobin, leukocytosis, and elevated partial thromboplastin time (PTT). Therefore, a decision was made to obtain a CT angiogram of the abdomen and pelvis, which revealed retroperitoneal hematoma. Further diagnostic workup showed a coagulation factor VIII level of zero percent and mixing studies supporting the presence of an acquired factor VIII inhibitor. Therefore, the patient was treated with rituximab and recombinant factor VIIa, with an improvement of factor VIII levels to normal limits within a week.

HELLP! The Novel Use of Continuous Renal Replacement Therapy and Nitric Oxide in the Treatment of Acute Respiratory Failure in HELLP Syndrome.

HELLP (hemolysis, elevated liver enzymes, low platelets) syndrome is a rare but serious complication of pregnancy characterized by hemolytic anemia, elevated liver enzymes, and thrombocytopenia. It occurs in <1% of all pregnancies with 70% of cases developing before delivery, the majority occurring between the 27th and 37th weeks of gestation. Respiratory failure seen in HELLP syndrome clinically and radiographically appears similar to acute respiratory distress syndrome (ARDS), with presence of bilateral pulmonary opacities on imaging as well as persistent hypoxemia requiring elevated ventilator requirements. It is seen to complicate 3-10% of cases of HELLP syndrome. Pulmonary complications are theorized to occur as sequelae of the proinflammatory state induced by HELLP syndrome with endothelial dysfunction and subsequent microangiopathic hemolysis and thrombocytopenia. A robust cytokine inflammatory response similar to ARDS is seen, resulting in noncardiogenic pulmonary edema due to vasoplegia and capillary leak syndrome. We present a case of a 27-year-old uniparous female with a term pregnancy complicated by HELLP syndrome who developed respiratory failure requiring mechanical ventilation. Early CRRT and nitric oxide therapy were initiated, with the patient experiencing clinical and radiological improvement of respiratory function within 48 hours. We document the novel treatment of our patient's acute respiratory failure with CRRT and nitric oxide and delve into the literature regarding its use in acute respiratory failure and ARDS in association with HELLP syndrome.