SHM Converge Scientific Abstract ePoster Gallery-From the Micu to the Floors, Refractory Thrombotic Thrombocytopenic Purpura as a Complication of Adult-Onset Stills Disease

From the Micu to the Floors, Refractory Thrombotic Thrombocytopenic Purpura as a Complication of Adult-Onset Stills Disease

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This case describes a 43-year-old man with recently diagnosed adult-onset Still’s disease (AOSD) who presented with 3 days of lethargy, confusion, jaundice, abdominal pain, and vomiting. On exam, he had petechiae and abdominal distention. Labs showed severe thrombocytopenia (platelets 5K/µL from a baseline of 600K/µL), anemia (hemoglobin 9 g/dL from 12 g/dL), elevated bilirubin, and schistocytes, consistent with microangiopathic hemolytic anemia. These findings raised concern for thrombotic thrombocytopenic purpura (TTP) complicating AOSD.<br /><br />The report emphasizes that AOSD is a rare autoinflammatory disorder classically associated with fever, rash, and arthralgias, but it can also be complicated by serious hematologic syndromes such as TTP, hemolytic uremic syndrome, and macrophage activation syndrome/HLH. In this patient, TTP presented with neurologic symptoms, GI symptoms, petechiae, and thrombocytopenia.<br /><br />He was treated with daily plasmapheresis for 17 days, pulse-dose steroids followed by a taper, and four rounds of rituximab. He completed plasma exchange and rituximab successfully, and his platelets recovered to 280K/µL with normalized ADAMTS-13 testing.<br /><br />The key teaching points are that hospitalists should maintain a high suspicion for TTP in patients with AOSD who develop encephalopathy, GI symptoms, petechiae, thrombocytopenia, and hemolytic anemia. Refractory TTP should be considered when platelets remain low, symptoms fail to improve, or end-organ injury persists despite first-line therapy. Prompt recognition and early initiation of plasmapheresis and steroids are critical to reduce morbidity and mortality.

Asset Subtitle

Grace Lee

Meta Tag

Author List Grace Lee, Reema Shah, Saumya Sharma, Spencer F. Weintraub

Category Clinical Vignettes

Concept Adult-onset Stills disease

Concept Thrombotic Thrombocytopenic Purpura

Concept Total Plasma Exchange

Concept Thrombocytopenia

Concept Hemolytic Anemia

Distinguished Non-Finalist

Presenter Organization Northwell Northshore-Lij

Presenting Author Grace Lee

Track Adult

Keywords

adult-onset Still’s disease

thrombotic thrombocytopenic purpura

microangiopathic hemolytic anemia

thrombocytopenia

plasmapheresis

rituximab

ADAMTS-13

schistocytes

encephalopathy

hematologic complications

Adult-onset Stills disease

Thrombotic Thrombocytopenic Purpura

Total Plasma Exchange

Thrombocytopenia

Hemolytic Anemia