Cocaine-induced microangiopathic hemolytic anemia mimicking idiopathic thrombotic thrombocytopenic purpura: A case report and review of the literature

Abstract

Our understanding of the pathogenesis of idiopathic thrombotic thrombocytopenic purpura (TTP) has increased, but remains incomplete, particularly with respect to cases of suspected TTP that are either unresponsive to therapeutic plasma exchange (TPE) or have normal ADAMTS13 (a disintegrin-like and metalloprotease with thrombospondin type 1 motif 13) activity. A 53-year-old woman presented with severe anemia (hemoglobin 1.8 g/dL) and clinical and laboratory findings consistent with TTP in conjunction with acute cocaine use. The patient was treated with TPE until the pre-treatment ADAMTS13 activity was reported as normal without evidence of an inhibitor. TPE was stopped and the patient continued to improve without treatment. This patient's microangiopathic hemolytic anemia (MAHA) appeared to be secondary to cocaine use. The proposed pathogenesis is likely a combination of cocaine-induced vasoconstriction, vascular damage, platelet activation, and procoagulation. This is the fifth published report of cocaine-induced MAHA and to our knowledge the first with ADAMTS13 testing.

A case off TTP temporally associated with cocaine use: Implications for treatment of suspected DITMA

“…This case alters the known risk and benefits of treatment of suspected thrombotic microangiopathy (TMA) associated with cocaine use. Several publications have varying positions on supportive care when there is high suspicion of DITMA given the related morbidity (28%) and mortality (2%) of plasma exchange . Cocaine has been associated with microangiopathic hemolytic anemia in several cases including one patient with reduced ADAMTS13 level with the presence of an inhibitor .…”

“…The resultant ischemia causes multi organ dysfunction with mortality reported to be as high as 90% in the pre‐treatment era . TTP is part of a larger group of similar disorders referred to as microangiopathic hemolytic anemias (MAHA) . These disorders are characterized by small vessel occlusion and resultant end organ dysfunction.…”

“…MAHA is known to be associated with cocaine use . The clinical syndrome of MAHA and cocaine use is often referred to as drug‐induced thrombotic microangiopathy (DITMA) when other causes have been ruled out and ADAMTS13 values are not diagnostic of TTP.…”

“…Current guidelines recommend initiation of plasma exchange until TTP has been ruled out given the success of therapy and mortality and morbidity associated with delayed treatment of TTP . When there is a high suspicion of DITMA the morbidity (28%) and mortality (2%) associated with plasma exchange must also be weighed, and current literature suggests supportive therapy when there is a high suspicion for DITMA …”

Pediatric Pulmonary Hemorrhage vs. Extrapulmonary Bleeding in the Differential Diagnosis of Hemoptysis

“…Such case is rare but similar cases were previously reported describing an acute anemia associated with an alveolar hemorrhage after inhalation of cocaine. 22,23 The pathogenesis is not clear yet but an opinion was expressed that cocaineinduced vasoconstriction, vascular damage in the lung tissue, platelet activation, and procoagulatory activity could lead to such pathology.…”