Case Report: Microangiopathic Hemolytic Anemia With Normal ADAMTS13 Activity

Abstract

Thrombotic microangiopathies (TMAs) include a heterogeneous group of diseases characterized by abnormalities in the vessel walls of arterioles and capillaries resulting in microvascular thrombosis that typically presents with a microangiopathic hemolytic anemia (MAHA) and severe thrombocytopenia. We describe here the case of an 82-year-old woman, who came to our attention for a clinical condition consistent with thrombotic microangiopathy. Even if initially highly suggestive for a thrombotic thrombocytopenic purpura (TTP), the elevated ADAMTS13 activity together with the alteration of the main coagulation parameters (D-dimer elevation, fibrinogen consumption, slightly prolonged prothrombin time), induced us to consider several other diseases in the differential diagnostic process. The case evolved toward a suspected overlapped secondary hemophagocytic syndrome, though the hyperferritinemia was finally interpreted within the frame of a cytokine storm. After a complex diagnostic workup, the clinical and biochemical parameters guided us toward the diagnosis of a cancer-related microangiopathic hemolytic anemia (CR-MAHA) secondary to a relapsing breast cancer with multiple metastatic localizations. Prednisone 1 mg/kg body weight was started, and several units of fresh frozen plasma were infused, obtaining a good control of the hemolysis. No specific oncological therapies were, however, possible, due to the older age and the critically compromised general condition of the patient; therefore, after clinical stabilization, the patient was discharged for treatment in a palliative care Hospital.

Two Unusual Cases of Thrombotic Microangiopathies

“…Thrombotic microangiopathies (TMA) represent a clinically heterogeneous group of hematological disorders characterized by dysfunctions of the arteriolar and capillary walls leading to microvascular thrombosis and substantial thrombocytopenia clinically [1]. The present case presentation highlights the wide clinical spectrum of presentations of TMA and the importance of maintaining a high index of suspicion for its atypical and unusual presentations and prompt institution of proper treatment, including drug-induced thrombotic microangiopathy and complement-mediated thrombotic microangiopathies.…”