Case Report: Microangiopathic Hemolytic Anemia With Normal ADAMTS13 Activity

Area of Science:

• Hematology
• Oncology
• Internal Medicine

Background:

• Thrombotic microangiopathies (TMAs) are a diverse group of disorders characterized by microvascular thrombosis, microangiopathic hemolytic anemia (MAHA), and thrombocytopenia.
• Differential diagnosis of TMAs is complex, often requiring evaluation of coagulation parameters and specific biomarkers like ADAMTS13 activity.

Observation:

• An 82-year-old woman presented with symptoms suggestive of thrombotic microangiopathy, initially mimicking thrombotic thrombocytopenic purpura (TTP).
• Elevated ADAMTS13 activity and altered coagulation parameters (elevated D-dimer, fibrinogen consumption) prompted further investigation.
• The patient's condition evolved, showing signs suggestive of secondary hemophagocytic syndrome and a cytokine storm, with hyperferritinemia.

Findings:

• Diagnostic workup revealed cancer-related microangiopathic hemolytic anemia (CR-MAHA) secondary to relapsing metastatic breast cancer.
• Treatment with prednisone and fresh frozen plasma effectively controlled hemolysis.
• Due to the patient's age and critical condition, oncological therapies were not feasible, and palliative care was initiated.

Implications:

• This case underscores the importance of a comprehensive diagnostic approach for TMAs, especially in elderly patients with comorbidities.
• CR-MAHA is a critical diagnosis to consider, as it requires specific management strategies and impacts treatment decisions.
• The management of CR-MAHA in elderly or critically ill patients may involve supportive care and palliative measures when advanced therapies are contraindicated.