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This report aims to illustrate the complexities of diagnosing concomitant iTTP and Evans syndrome.

April 1, 2026Clinical Case Reports0 citationsOpen Access

Concomitant Immune‐Mediated Thrombotic Thrombocytopenic Purpura and Evans Syndrome With Multiple Cerebral Infarctions in an Elderly Patient

SOShinichi OgawaKyoto University YKYangsu KimJA Toride Medical Center TITakayoshi ItoJA Toride Medical Center

Key Points

This report aims to illustrate the complexities of diagnosing concomitant iTTP and Evans syndrome.
Case report of an elderly patient with severe thrombocytopenia and hemolytic anemia.
Initial diagnosis of Evans syndrome based on clinical presentation.
ADAMTS13 activity assessed during hospitalization to confirm iTTP diagnosis.
Patient exhibited overlapping symptoms of iTTP and Evans syndrome.
Positive direct Coombs test complicated the initial diagnosis.
Early recognition of iTTP facilitated timely therapeutic intervention, addressing thrombotic complications.

Abstract

ABSTRACT We report a rare case of concomitant immune thrombotic thrombocytopenic purpura (iTTP) and Evans syndrome, complicated by multiple cerebral infarctions. The patient presented with severe thrombocytopenia and hemolytic anemia, as well as a positive direct Coombs test. The initial diagnosis was Evans syndrome. Although a negative direct Coombs test is typically characteristic of iTTP, the ADAMTS13 activity finding obtained on the fifth day of hospitalization confirmed the coexistence of iTTP. This case highlights the diagnostic challenge posed by overlapping autoimmune hematologic disorders and underscores the need to consider iTTP even in patients with a positive direct Coombs test, particularly when there are thrombotic complications such as cerebral infarctions. Awareness of such atypical presentations is essential because early recognition and prompt initiation of appropriate therapy can be lifesaving.

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