# Progression From Early Multiterritorial Punctate Infarcts to Massive Stroke in Fulminant Thrombotic Thrombocytopenic Purpura Despite Aggressive Therapy: A Therapeutic Dilemma in Initiating Antithrombotic Therapy

## TL;DR

A rare case of severe thrombotic thrombocytopenic purpura (TTP) led to progressive neurological complications despite aggressive treatment, highlighting challenges in managing antithrombotic therapy.

## Contribution

This case report presents a rare progression of TTP to massive stroke and explores the dilemma of initiating antithrombotic therapy in such scenarios.

## Key findings

- Despite aggressive treatment, the patient developed a large cerebral artery stroke and multifocal infarctions.
- The case highlights the difficulty in managing antithrombotic therapy in TTP with severe thrombocytopenia.
- Neurological complications can occur despite maximal medical therapy in refractory TTP.

## Abstract

Thrombotic thrombocytopenic purpura (TTP) is a rare, life-threatening thrombotic microangiopathy characterized by a profound deficiency in ADAMTS13 activity. It can present with a wide range of clinical features, including thrombocytopenia, hemolytic anemia, renal impairment, fever, and neurologic manifestations. Prompt recognition and treatment with plasma exchange and immunosuppressive therapy significantly improve survival. However, in rare cases, TTP may become refractory to aggressive treatment and progress rapidly. We report the case of a 62-year-old woman who presented with abdominal pain and acute confusion and was diagnosed with TTP complicated by multiterritorial punctate cerebral infarcts, following laboratory findings that revealed severe thrombocytopenia, hemolytic anemia, and markedly reduced ADAMTS13 activity. Despite aggressive treatment with plasma exchange, corticosteroids, rituximab, cyclophosphamide, and caplacizumab, the patient’s condition worsened, progressing to a large left middle cerebral artery ischemic stroke in addition to multifocal infarctions, seizures, encephalopathy, and ultimately, respiratory failure. This case highlights the diagnostic and therapeutic challenges of refractory TTP and underscores the devastating neurologic complications that can occur despite maximal medical therapy. It also raises important questions about the dilemma of whether or when to initiate antithrombotic therapy in the setting of severe thrombocytopenia and ongoing microvascular thrombosis.