ACQUIRED HEMOPHILIA IN SLE. J. Pedersen 1 , J. Paty 1 , M. Panda 1 ; 1 University of Tennessee College of Medicine -Chattanooga Unit, Chattanooga, TN LEARNING OBJECTIVES: 1. Recognize the entity of acquired hemophilia secondary to antifactor VIII antibodies and its association with autoimmune diseases and occult malignancy. 2. Understand the indications for treatment of acquired hemophilia. CASE INFORMATION: A 76 year old white female with a past history of SLE and polymyalgia rheumatica, in remission on daily prednisone and Plaquanil, presented to the office with a one week history of spontaneous, extensive bruising of the right hip and thigh after arising from a chair. She denied trauma, a personal or family history of easy bruising or prolonged bleeding. Physical examination was unremarkable except for an extensive area of ecehymosis along the right inner and outer thigh. MRI revealed iliospoas hemorrhage. Initial lab revealed a normochromic, normocytic anemia with a hemoglobin of 6 (one week prior to admission her hemoglobin was 10). She had a normal platelet count, prothrombin, thrombin and bleeding times, but an elevated partial thromboplastin time (57.1 seconds). The PTT partially corrected with the addition of normal plasma, so Factor VIII assay was done which revealed decreased activity at 8% with positive titers for factor VIII inhibitor (72 Bethesda, n1 0.1 ± 0.8). Lupus anticoagulant and antiphospholipid antibodies were negative. Subsequently the patient had a spontaneous right retroperitoneal and left upper extremity hemorrhage and required factor VIII transfusions, high dose corticosteroids and oral cytoxan. Six months later she remains symptom free on 5mg of prednisone and Plaquenil 400mg daily. Her hemoglobin is 11.8; PTT-28.6 and there is no detectable Factor VllI inhibitor. DISCUSSION: Hemophilia A, as a result of Factor VIII deficiency, can be inherited or acquired. Acquired inhibitors to Factor VIII are endogenous IgG/IgM immunoglobulins which interfere with normal blood coagulation. Acquired Factor VIII inhibitors can occur in patients with inherited hemophilia A, postpartum women, patients with collagen vascular diseases such as SLE, or idiopathicafly, especially in older patients. They are also associated with occult malignancy such as lymphoma and solid organ tumors. The syndrome usually presents with significant bleeding and soft tissue ecehymoses with minimal or no trauma. Treatment of active episodes of bleeding may involve administration of exogenous porcine or human Factor VIII or treatment with activated prothrombin complex concentrates as in our patient. Prophylactic treatment with steroids, with or without immunosuppressive agents, is indicated in patients with significant bleeding or with high titers of inhibitor. year-old African-American woman with no significant past medical history presented to clinic with complaints of bilateral lower extremity swelling, easy fatiguability and generalized weakness of 2 weeks duration. She also reported increased sensitivity to cold, dryness of skin and menorrhagia. On exam, she was moderately obese, did not appear acutely ill. Vitals signs were normal. Physical exam remarkable for mild conjunctival pallor, soft heart sounds, slow relaxation phase of deep tendon reflexes and bilateral lower extremity non pitting edema. EKG showed low voltage complexes in all leads. Echocardiogram demonstrated large pericardial effusion with no tamponade. Labs revealed normocytic, normochromic anemia. Thyroid profile with TSH 2.07 (0.4 ± 6) and free thyroxine 0.30 (0.6 ± 1.7) pointed towards the diagnosis of central hypothyroidism. Other hormonal assays were done which also revealed coexisting adrenal insufficiency. MRI of brain showed flattened pituitary along the floor of sella and majority of the sella was filled with CSF.These findings were consistent with empty sella . No focal lesion in the pituitary gland were identified. Pt was diagnosed to have hypopituitarism due to primary empty sella syndrome . She was treated with thyroxine and steroids to which she responded appropriately. The pericardial effusion was presumed to be secondary to the hypothyroidism and pericardiocentesis was not performed as she was hemodynamically stable. DISCUSSION: Primary empty sella syndrome has been classically described in obese hypertensive women. It is characterized by the presence of an arachnoid herniation filled with fluid that compresses the pituitary against the sellar wall.It is often asymptomatic but may be associated with endocrine disorders. Less than 30 percent of these patients presents with symptoms suggestive of hormonal deficiency. Recent literature reports only about 10 percent of these patients present with hypopituitarism as in our patient. The most common causes of central hypothyroidism are pituitary adenomas,pituitary apoplexy, infiltrative lesions. Pericardial effusion is one of the common cardiovascular manifestation in hypothyroid patients but most of these cases are described in patients with primary hypothyroidism. There are only few case reports describing pericardial effusion as presenting manifestation in patients with central hypothyroidism. These pericardial effisions are usually large but rarely progress to tamponade. They usually respond well to thyroxine supplementation. for unexplained anemia (Hb 6 g/dl). The patient had a history of chronic diarrhea with weight loss secondary to a diagnosis of CVID in 1994. Multiple colonoscopies revealed only nonspecific colitis. CT scan showed extensive mesenteric lymphadenopathy and thickening of the small bowel wall. He underwent EGD which showed healed duodenal erosions with copious bile in the esopaghus and stomach. A small bowel follow-through revealed proximal bowel dilatation and a partially obstructing process in the mid-jejunum (see Figure) . Exploratory laparotomy with partial small bowel resection revealed malignant lymphoma (large B-cell type). Bone marrow biopsy was normal. Tests for HIV antibody were repeatedly negative. The patient was discharged for outpatient oncological treatment. DISCUSSION: GI lymphomas have been reported rarely in patients with CVID, but are not usually considered among the initial differential diagnoses. We present this case to call attention to non-specific symptomatic presentation of lymphomas of the GI tract (Lai Ping So and Mayer, Semin Gastrointest Dis 1997; 8:22; Gottesman et al., Leuk Lymphoma 1999; 32: 589) . Figure  Legend . Barium failed to progress rapidly to the distal jejunum (left panel), but was present after 15 minutes (right panel), indicating a partially obstructing process with considerable proximal bowel dilatation. An 84 year old woman with end-stage renal failure, congestive heart failure, insulin-dependent diabetes, depression, and osteoarthritis begins to express a desire to discontinue hemodialysis after many years. She feels sad, exhausted, and a burden to her family. The patient has been widowed for five years. Her medications include Celexa and Vicodin. She has four attentive children who all live in the same city. She is cared for primarily by her youngest daughter, who lives with the patient, administers medications and transports the patient to dialysis. Intent on caring well for her mother, and worried that pain was interfering with the patient's appetite, the patient's daughter has been administering increasing doses of Vicodin. While the patient has made a number of comments about discontinuing dialysis to each of her children, none of the children have talked with each other, hoping to avoid such discussions, as well as differences of opinion between them. The patient's thoughts have not been assessed during the last year by either her nephrologist or primary care physician. An endof-life consultation team met with the patient and her daughter. Clarification of the appropriate use of pain medicine allowed the patient to control her pain with only occasional use of Vicodin, leading to increased energy, independence, and enthusiasm. Assessment of the patient's CESD depression score showed an increase from 14 (six months earlier) to 22. The patient's Celexa dose was adjusted. The patient's daughter was invited to attend support classes for family taking care of patients with end-stage illness and grew more comfortable talking about her fears and sense of responsibility. With renewed energy and improved mood, the patient withdrew her desire to discontinue dialysis for now. In a family meeting, all four children were able to recognize their own feelings and to better coordinate care. DISCUSSION: Even among closely-knit families and well-supported patients, miscommunication is possible, especially around the highly-charged issues of advance care planning for patients with end-stage illness. Assessing for medication over-and underprescribing, and ruling out depression as an etiology for withdrawal of support requests, are good first steps during discussions. Understanding the motivations and influences of relevant family members is necessary as well. The key to good advance care planning is to begin the discussions well in advance of the decisions as there is often much to learn and minor clinical interventions can have major effects on planning variables.