A Case of Thrombotic Thrombocytopenia Purpura Associated with Systemic Lupus Erythematosus: Diagnostic Utility of ADAMTS-13 Activity

Abstract

Thrombotic thrombocytopenia purpura (TTP) caused by a deficiency in ADAMTS-13 activity is considered to involve a subset of thrombotic microangiopathy (TMA). Although concept of TTP is included under the umbrella of TMA, discrimination of TTP from TMA is occasionally difficult in an autoimmune disorder. Herein, we report a case with TTP associated with systemic lupus erythematosus (SLE). In this case, it was difficult to discriminate TTP from TMA and the measurement of ADAMTS-13 activity was useful for obtaining an accurate diagnosis. SLE patients having thrombocytopenia in complication with anemia should be considered a monitoring of ADAMTS-13 activity even though the patients lacked symptoms of TTP related to the microvascular coagulation.

Thrombocytopenia-Associated Multiple Organ Failure and Acute Kidney Injury

“…41,43,44 These autoimmune diseases with their associated glomerulopathies and interstitial nephropathies can rapidly worsen the already fragile renal function during an acute TTP episode and manifest as TTP-induced AKI.…”

Rare association of a patient with multiple ischemic strokes with thrombotic thrombocytopenic purpura and systemic lupus erythematosus: A case report

“…However, given that the mechanism of stroke is not the common large vessel atherosclerosis or small vessel occlusion, treatment is dictated by the identified cause. TPE significantly improves the outcome in patients with TTP, and those left untreated have an increased probability of mortality by as high as 90% [9]. Literature dictates that TPE followed by high-dose steroid therapy are beneficial to the patient, but given that the patient had a stroke thrombolysis was not done due to thrombocytopenia and it was deemed more harmful than beneficial [4,9].…”

Post-partum management in a patient affected by thrombotic thrombocytopenic purpura: case report and review of literature

“…When ADAMTS13 activity rate is <10% the risk of relapse is 40%, while when it is >10%, the risk decreases to 4% [41]. The differential diagnosis must include haemolytic uraemic syndrome (HUS), disseminated intravascular coagulation (DIC), anti-phospholipid syndrome (APS), pre-eclampsia and haemolysis, elevated liver enzymes, and low platelet count (HELLP) syndrome [42]. Physiological changes and complications during pregnancy or puerperium can determine risk factors to trigger acute episodes of TTP, especially in women with family histories of thrombophilia or ADAMTS13 deficiency [27].…”