Kidney Week

Abstract: FR-PO0439

Fulminant Thrombotic Thrombocytopenic Purpura Presenting with Disseminated Intravascular Coagulation (DIC)-Related Coagulopathy: The Fatal Dilemma of Deferring Caplacizumab

Session Information

• AKI: Case Reports - TMA, Vasculitis, Immune-Mediated Injury, and Systemic Disease
  October 23, 2026 | Location: Exhibit Hall A, Convention Center
  Abstract Time: 10:00 AM - 12:00 PM

Category: Acute Kidney Injury

• 102 AKI: Clinical, Outcomes, and Trials

Authors

• Shibuya, Chika, Shonan Kamakura Sogo Byoin, Kamakura, Kanagawa Prefecture, Japan

Chika Shibuya

• Hidaka, Sumi, Shonan Kamakura Sogo Byoin, Kamakura, Kanagawa Prefecture, Japan

Sumi Hidaka, MD, PhD

• Mochida, Yasuhiro, Shonan Kamakura Sogo Byoin, Kamakura, Kanagawa Prefecture, Japan

Yasuhiro Mochida

• Ishioka, Kunihiro, Shonan Kamakura Sogo Byoin, Kamakura, Kanagawa Prefecture, Japan

Kunihiro Ishioka, MD

• Oka, Machiko, Shonan Kamakura Sogo Byoin, Kamakura, Kanagawa Prefecture, Japan

Machiko Oka, MD

• Yanai, Mitsuru, Shonan Kamakura Sogo Byoin, Kamakura, Kanagawa Prefecture, Japan

Mitsuru Yanai, MD, PhD

• Ohtake, Takayasu, Shonan Kamakura Sogo Byoin, Kamakura, Kanagawa Prefecture, Japan

Takayasu Ohtake, MD, PhD

• Kobayashi, Shuzo, Shonan Kamakura Sogo Byoin, Kamakura, Kanagawa Prefecture, Japan

Shuzo Kobayashi, MD, PhD

Group or Team Name

• Shonan Kamakura General Hospital, Kidney Disease and Transplant Center

Introduction

Thrombotic thrombocytopenic purpura (TTP) is a life-threatening thrombotic microangiopathy. Although caplacizumab has transformed the management of TTP, its initiation remains challenging in patients with severe coagulopathy and active bleeding.

Case Description

A previously healthy woman in her 50s presented with hematuria and genital bleeding, followed by rapid neurological deterioration. Laboratory testing showed hemolytic anemia and severe thrombocytopenia, with a platelet count of 14,000/µL, while coagulation parameters were initially normal. TTP was suspected, and she was transferred to our hospital. On arrival, 10 hours after the initial presentation, she had developed severe DIC-like coagulopathy, with PT-INR 1.73, APTT 99 seconds, fibrinogen 255.1 mg/dL, FDP 16.5 µg/mL, and TAT 200 ng/mL; her platelet count had decreased to 3,000/µL. Schistocytes were prominent, accounting for 30%.
Although her PLASMIC score was high at 6, caplacizumab was not administered because of active genital bleeding and severe coagulopathy. She was treated with emergent plasma exchange and high-dose corticosteroids. However, she suffered cardiac arrest on hospital day 3 and died despite ECMO support. After her death, ADAMTS13 activity revealed severe deficiency (<10% ) with a positive inhibitor. Autopsy demonstrated widespread platelet- and fibrin-rich microthrombi in multiple organs, confirming that the underlying pathophysiology was predominantly thrombotic rather than hemorrhagic coagulopathy alone.

Discussion

The autopsy finding of extensive thrombosis suggests that caplacizumab administration may have been beneficial. This case highlights a critical therapeutic dilemma in fulminant TTP: rapidly progressive DIC-related coagulopathy may obscure the diagnosis and delay initiation of caplacizumab therapy.

Take-away Lessons
Discordance: In fulminant TTP, coagulopathy and concomitant DIC may occur; however, the predominant pathophysiology remains catastrophic microvascular thrombosis.
Urgent Initiation: When TTP is suspected, the presence of coagulopathy alone should not necessarily preclude early use of caplacizumab, because the risk of fatal thrombosis may outweigh the risk of bleeding.