out malignancy. She was subsequently diagnosed with mixed AIHA in setting of SLE, for which she was transfused with packed red blood cells and started on high dose steroids for treatment. The patient reported clinical improvement, leading to discharge from the hospital on oral prednisone. Discussion: SLE is a chronic autoimmune, multisystem connective tissue disease of unknown etiology with heterogenous clinical picture. AIHA
is seen in 10% of patients with SLE and very rarely precedes the development of SLE. Mixed AIHA is often more severe with a presenting hemoglobin of less than 6 mg/dL and often requires multiple lines of treatment. The presence of hemolysis markers in the setting of known SLE should be followed by a Coombs test. Likewise, any patient presenting with AIHA must be screened for SLE as presence of AIHA in general is correlated with severe SLE.
BAD BLOOD: A RARE CASE OF PARANEOPLASTIC ACQUIRED HEMOPHILIA A (AHA) IN AN ELDERLY INDIVIDUAL Kashish Shah, Het Patel, Anil Veluvolu; Willis-Knighton Medical Center, Shreveport, LA.
Introduction: Acquired Hemophilia A (AHA) is a rare bleeding disorder with an incidence of about 1.5 per million per year. While 50% of the cases are idiopathic, around 10% of the cases are associated with an underlying malignancy. Case: A 65-year-old male with a history of head and neck squamous cell carcinoma, non-small cell lung carcinoma (NSCLC) and renal cell carcinoma (diagnosed six and four months before presentation, respectively) was being treated with chemoradiation, presented to an outside facility with fatigue, weakness, and hypotension. Examination revealed swelling and tenderness over his bilateral arms, left buttock, and right thigh. The hemoglobin was 3 gm/dl and fecal occult blood testing was positive. He had no history of coagulopathy and was not on any anticoagulation. There was no evidence of active bleeding. Ultrasound of the extremities showed intramuscular hematomas in the right triceps, right quadriceps, and left gluteal musculature. Three units of packed red blood cells were transfused and the patient was transferred to our facility for further evaluation and management. Coagulation studies showed isolated elevation of activated partial thrombplastin time (aPTT), and
platelets were within normal range. Administration of aminocaproic acid and four units of fresh frozen plasma didn’t lead to any improvement in the aPTT. Suspecting AHA, empirical methylprednisolone was started, and recombinant factor VIIa and Humate-P were administered. Factor VIII activity levels were <1% and not corrected by 1:1 mixing study. Dilute Russell’s Viper Venom Time (DRVVT) test for lupus anticoagulant was negative. Factor VIII inhibitor levels were elevated, confirming the diagnosis. Rituximab was added to increase immunosuppression. Following a serial improvement in aPTT with minimal improvement in Factor VIII inhibitor levels and Factor VIII activity, the patient was discharged after three weeks of treatment with further plans to continue the remaining treatment as well as NSCLC chemoradiation on an outpatient basis. Discussion: Early diagnosis and prompt treatment plays a fundamental role in determining patient outcomes in AHA. Optimum management includes replacement of factors in the acute setting and immunosuppression with high dose steroids. Rituximab can be added in severe or refractory cases. Treatment of the underlying condition, malignancy in our case, remains imperative in achieving remission.
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