SHM Converge Scientific Abstract ePoster Gallery-Critical Crossroads: Navigating Thrombotic Microangiopathy and Disseminated Intravascular Coagulation

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This case describes a 39-year-old man with a history of hereditary spherocytosis status post-splenectomy who presented with syncope, fatigue, fever, and dark urine. He rapidly deteriorated with rising lactate, worsening kidney and liver function, severe coagulopathy, thrombocytopenia, and markedly elevated D-dimer. Blood cultures grew Streptococcus pneumoniae, and he developed purpura fulminans with mottled extremities, painful ischemic changes, and necrosis of multiple digits. He also developed anuria requiring hemodialysis.<br /><br />The clinical picture initially raised concern for thrombotic microangiopathy, especially TTP, because of schistocytes, hemolysis, thrombocytopenia, and organ dysfunction. However, low fibrinogen, prolonged PT/PTT/INR, very high D-dimer, and the infectious source supported sepsis-related disseminated intravascular coagulation (DIC). ADAMTS13 activity later ruled out TTP. Hematology recommended emergent therapeutic plasma exchange (TPE) because of the severity and rapid progression.<br /><br />The patient improved markedly after the first TPE and ultimately received five sessions before full recovery. The report emphasizes that distinguishing TMA from DIC can be difficult in critically ill patients, and that early recognition is essential. In selected severe cases, TPE may help by removing inflammatory mediators and restoring endothelial function, even when the final diagnosis is sepsis-associated DIC rather than TTP.<br /><br />Overall, the case highlights the importance of maintaining a broad differential, using tools such as the PLASMIC score, and initiating targeted therapy quickly to prevent irreversible ischemic injury and death.

Asset Subtitle

Jesse E. Doyle

Meta Tag

Author List Anthony El Khoury, Jesse E. Doyle, Roger Johnson

Category Clinical Vignettes

Concept Thrombotic Microangiopathy

Concept Thrombotic Thrombocytopenic Purpura

Concept Disseminated Intravascular Coagulation

Concept Thrombocytopenia

Concept Hemolysis

Distinguished Non-Finalist

Presenter Organization Indiana University

Presenting Author Jesse E. Doyle

Track Adult

Keywords

hereditary spherocytosis

splenectomy

Streptococcus pneumoniae

purpura fulminans

disseminated intravascular coagulation

thrombotic microangiopathy

thrombotic thrombocytopenic purpura

therapeutic plasma exchange

ADAMTS13

sepsis

Thrombotic Microangiopathy

Thrombotic Thrombocytopenic Purpura

Disseminated Intravascular Coagulation

Thrombocytopenia

Hemolysis