A RARE PRESENTATION OF ADULT ONSET STILLS DISEASE WITH THROMBOTIC THROMBOCYTOPENIC PURPURA
Michelle Benjamin, Cameron Windham, Shidestini Martinez, Jasmeen Uppal, Warda Maqsood, Jamie Fernan, Dinesh Keerty, Saleha Dar, Samina Hayat; Department of Medicine, Louisiana State University, Shreveport, LA.
Introduction: Thrombotic Thrombocytopenic Purpura is a rare auto-inflammatory disorder characterized by decreased activity of ADAMTS13 and microthrombi throughout the small vessels. History of autoimmune disease is a significant risk factor. Adult-Onset Still’s Disease is a rare autoinflammatory condition which has been anecdotally associated with TTP. Case: A 25-year-old female with Adult-Onset Still’s Disease (AOSD) presented with complaints of fatigue. One week prior to presentation, she experienced fever, vomiting and diarrhea. She then presented to Rheumatology clinic for follow-up. Lab work revealed platelet count 7, hemoglobin 6.9, MCV 65, haptoglobin <8, INR 0.87, Creatinine 3.8. Due to patient’s presentation of anemia, acute kidney injury, thrombocytopenia and recent febrile illness, there was high suspicion for Thrombotic Thrombocytopenic Purpura. The patient’s PLASMIC score was six and appropriate labs were ordered. Peripheral smear revealed abundant schistocytes, thus Hematology and Pathology were consulted. Plasmapheresis and methylprednisolone were immediately started. ADAMTS 13 Activity and ADAMTS 13 were <5% and 1.5 respectively; confirming a diagnosis of Secondary/Acquired TTP. Further lab work made a diagnosis of DIC, ITP and HUS unlikely. She also received Rituximab and Caplacizumab due to her high-risk status. Despite plasmapheresis, medical
management, and improvement in ADAMTS 13 to ninety-three, our patient experienced a slow rise in platelets, plateauing at 60. Bone marrow biopsy revealed a mildly hypocellular bone marrow with granuloerythopoeitic hypoplasia. After twenty-one sessions of plasmapheresis, decision was made to hold further plasmapheresis and monitor patient’s response to medical management. However, on day twenty-three of hospitalization the patient experienced an acute change in mental status. MRI brain was unremarkable. From a hematology standpoint, confusion could be a rare form of CNS TTP which occurs without microangiopathic hemolytic anemia. We decided to reinitiate plasmapheresis, which resulted in significant neurological recovery following two rounds of plasmapheresis. Discussion: Reported cases of TTP with Still’s Disease are exceedingly rare. Our patient had several risk factors including her autoimmune disease, African ancestry, female gender, and recent viral gastroenteritis. While it is more commonly associated with antiphospholipid antibody syndrome and SLE, clinicians must keep a high suspicion for TTP in the presence of AOSD. Delay in identification and treatment carries a high morality. Further, approximately thirty percent of patients with history of TTP relapse within ten years. Management of AOSD patients will require a multidisciplinary team including Internal Medicine, Rheumatology, Hematology and Pathology.
HYPERCALCEMIC CRISIS FROM A MASSIVE BENIGN PARATHYROID ADENOMA Griffin Farrish, William Benton, Guido DeJesus, Joel Silverberg; Department of Medicine, LSU Health Sciences Center, Baton Rouge, LA.
Introduction: A hypercalcemic crisis is characterized by an albumin-corrected serum calcium of >14 mg/dL with associated organ dysfunction. End- organ damage can manifest with neurologic, renal, cardiac and gastrointestinal symptoms. Primary hyperparathyroidism is the most common cause of hypercalcemia, but rarely can increase calcium levels sufficiently to induce a crisis.
presented with nausea, vomiting, abdominal pain, polydipsia, polyuria, fatigue and bilateral UE/LE “bone pain” which had been worsening for the past month-and-a-half. Recent outpatient labs showed hypercalcemia and an AKI. At admission, labs revealed a calcium of 20.82, a creatinine of 2.0 and a PTH of 1792.4. She was placed on telemetry and treated with aggressive fluids, calcitonin and zoledronic acid. Additional labs including SPEP, UPEP, free light chains, PTHrP, urine Ca/Cr clearance ratio 16
Case: A 36-year-old woman with HTN and HLD
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