A 49-year-old man with malignant hypertension, acute kidney injury and mental deterioration was referred to our hospital. We initially observed microangiopathic hemolytic anemia, thrombocytopenia and kidney damage, indicating he had thrombotic microangiopathy (TMA). We considered TMA was caused by malignant hypertension and therefore did not start plasma therapy. The French TMA reference center reported that platelet counts and serum creatine levels have high values for predicting severe ADAMTS13 deficiency. The patient fully recovered from his illness after treatment with antihypertensive drugs and intermittent hemodialysis. This case might thus be useful to understand the proper differential diagnosis and treatment of TMA.
SUMMARYA 29-year-old Japanese man presented with fever, dyspnoea and non-productive cough after massive inhalation of evaporant from a polytetrafluoroethylenecoated cooking pan. Chest CT scan showed diffuse interstitial infiltration in both lungs. Based on the patient history, images and the pan he brought to the hospital, polymer fume fever was strongly suspected. His symptoms dramatically improved over the following 2 days after admission. BACKGROUND
Rationale:Acquired inhibitors of coagulation are antibodies that either inhibit the activity or increase the clearance of a clotting factor. Acquired factor V deficiency is a rare coagulation disorder, and it can sometimes be life threatening.Patient concerns:We describe a case of a 90-year-old Japanese male with acquired factor V deficiency. He was previously misdiagnosed with congenital factor V deficiency when he presented with hemoptysis and a negative factor V inhibitor test result at a different hospital 5 years earlier. Coagulopathy recurred with ecchymosis when he sustained a bruise after falling on a bush.Diagnosis:Although the factor V inhibitor test result was negative and a mixing study suggested a deficiency pattern, we diagnosed the patient with acquired factor V deficiency on the basis of no history of bleeding diathesis, a lack of response to multiple fresh frozen plasma transfusion, and clinical response to corticosteroid therapy.Interventions:Intravenous methylprednisolone was administered at 500 mg/day for 3 days, followed by oral prednisolone at 1 mg/kg/day.Outcomes:Coagulation test results improved and symptoms resolved 2 weeks after corticosteroid administration.Lessons:This case report suggests that clearance-facilitating antibodies exist without the presence of neutralizing inhibitors. When patients present with coagulation factor V deficiency in the absence of coagulation inhibitors, acquired factor V deficiency should also be considered.
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