Thrombotic thrombocytopenic purpura (TTP) is a potentially fatal disorder that requires urgent identification and treatment. The association of TTP with systemic lupus erythematosus (SLE) and vasculitis has been reported, however, never simultaneously. A 33-year-old woman with a history of SLE presented with acute abdominal pain, fever, arthralgias, and skin rash. She had acute severe hypertension, diffuse abdominal tenderness, and petechial rash. Diagnostic work-up revealed active urine sediment with proteinuria and hematuria and elevated creatinine, anemia, and thrombocytopenia. She was diagnosed with acute lupus nephritis and early microangiopathic hemolytic anemia in the setting of hypertensive urgency and started on intravenous methylprednisolone 500 mg once a day. Within 48 hours, she developed shock with multiorgan dysfunction and succumbed to her illness. Laboratory tests later showed ADAMTS13 activity less than 10% consistent with TTP and p-antineutrophil cytoplasmic antibody (ANCA) positivity. Autopsy revealed small-vessel vasculitis of the visceral organs. Kidney biopsy demonstrated diffuse proliferative glomerulonephritis. This case illustrates the occurrence of SLE nephritis, p-ANCA vasculitis, and severe TTP with rapidly fatal course, and the importance of having a low threshold for initiating plasma exchange therapy. Here, we discuss the case and provide a literature review on cases of TTP with SLE and vasculitis.
Acute myeloid leukemia (AML) is a complex disease with a variety of presentations. A large pericardial effusion is rare, occurring in less than 0.5% of all patients with AML prior to treatment. A 34-year-old male presented with dyspnea, malaise, and weight loss. On physical exam, he was noted to be hypoxic, tachypneic, tachycardic, and hypotensive. He had cervical lymphadenopathy and jugular venous distention. His WBC count was 110 bil/L with 33% blasts. Bone marrow biopsy confirmed AML with 60% blasts. Leukemic cells were also seen in the cerebrospinal fluid on lumbar puncture. An echocardiogram revealed a large pericardial effusion causing tamponade. He underwent emergent pericardiocentesis, and malignant cells were present in the pericardial fluid. Induction therapy with standard dose cytarabine and daunorubicin was initiated, and bone marrow biopsy 14 days later showed no residual AML. This case demonstrates the importance of a thorough evaluation of each organ system when caring for a patient with AML.
Aortic dissection is a life-threatening condition in which the inner layer of the aorta tears. Blood surges through the tear, causing the inner and middle layers of the aorta to separate (dissect). It is considered a medical emergency. We report a case of a healthy 56-year-old male who presented to the emergency room with sudden onset of epigastric pain radiating to his back. His blood pressure was 167/91 mmHg, equal in both arms. His lipase was elevated at 1258 U/L, and he was clinically diagnosed with acute pancreatitis (AP). He denied any alcohol consumption, had no evidence for gallstones, and had normal triglyceride level. Two days later, he endorsed new suprapubic tenderness radiating to his scrotum, along with worsening epigastric pain. A MRCP demonstrated evidence of an aortic dissection (AD). CT angiography demonstrated a Stanford type B AD extending into the proximal common iliac arteries. His aortic dissection was managed medically with rapid blood pressure control. The patient had excellent recovery and was discharged home without any surgical intervention.
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