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Abstract: TH-PO668

A Do-Not-Miss Diagnosis: Thrombotic Thrombocytopenic Purpura in Systemic Lupus Erythematosus

Session Information

Category: Glomerular Diseases

  • 1402 Glomerular Diseases: Clinical, Outcomes, and Trials

Authors

  • Yanik, Andrew, University of Utah Health, Salt Lake City, Utah, United States
  • Abraham, Josephine, University of Utah Health, Salt Lake City, Utah, United States
Introduction

Thrombotic thrombocytopenic purpura (TTP) is a rare complication of systemic lupus erythematosus (SLE) associated with high mortality. We report the case of a patient with SLE complicated by the development of fatal TTP.

Case Description

A 42-year-old male with history of SLE presented to an outside hospital endorsing three days of hematuria, hematemesis, dysarthria, malaise, and cutaneous changes for one week. His objective data there was pertinent for platelets (PLT) of 2 k/uL, creatinine of 2.38 mg/dL (baseline 0.8), and CT evidence of perinephric stranding. On arrival to our hospital, he was tachycardic, tachypneic, afebrile, and normotensive. Examination revealed jaundice with diffuse ecchymoses, and a normal neurological status. Laboratory studies on arrival were pertinent for undetectably low PLT count, hemoglobin 8.4 g/dL, creatinine 2.55 mg/dL, total bilirubin 3.6 mg/dL (direct 1.0), haptoglobin 20 mg/dL (ref 30-200), schistocytes on blood smear, LDH 3,433 U/L, D-dimer 38.5 ug/mL, fibrinogen 603 mg/dL, PT of 15.2 s, PTT of 34 s, and a normal vitamin B12. The patient’s mental status rapidly worsened in the ensuing hours, and he developed hypoxic respiratory failure with a serum lactate of 12 mmol/L. He was moved to the intensive care unit to start empiric therapeutic plasma exchange (TPE). His ADAMTS13 activity level resulted at <5% (ref >61%) and notably the ADAMTS13 inhibitor activity was elevated. Prior to initiation of TPE, however, the patient was found unresponsive and in cardiac arrest. After forty minutes of advanced cardiovascular life support effort, the patient was pronounced deceased just twelve hours after admission. Autopsy showed platelet-rich microthrombi in multiple organs, severe thrombocytopenia, and no deep vein thrombi most consistent with TTP.

Discussion

Thrombotic microangiopathies (TMA) are rare clinical syndromes characterized by thrombocytopenia, microangiopathic hemolytic anemia, and microvascular thrombus formation leading to end-organ damage. TTP is a rare and often lethal complication of SLE (sTTP) and its diagnosis is challenging due to the significant overlap in signs and symptoms. As illustrated by this case, severe thrombocytopenia in a patient with SLE should raise concern for sTTP as any delay in its diagnosis can be fatal. Early diagnosis and prompt initiation of treatment including therapeutic plasma exchange is crucial.