Successful outcome with eculizumab treatment in a patient with antiphospholipid syndrome presenting with an unusual thro
- PDF / 704,774 Bytes
- 4 Pages / 595.276 x 790.866 pts Page_size
- 104 Downloads / 209 Views
Successful outcome with eculizumab treatment in a patient with antiphospholipid syndrome presenting with an unusual thrombotic storm Jones T. Nauseef1 · Hana I. Lim1 · Maria T. DeSancho1 Accepted: 10 November 2020 © Springer Science+Business Media, LLC, part of Springer Nature 2020
Abstract Catastrophic Antiphospholipid Syndrome (CAPS) is a life-threatening complication of APS requiring complex management to optimize patient outcome. We describe a 54-year-old man with APS with history of splanchnic vein thrombosis, a Factor II G20210A heterozygote, autoimmune hemolytic anemia and thrombocytopenia. He developed sudden onset of severe flank pain due to spontaneous bilateral adrenal hemorrhage while on warfarin with a therapeutic INR. Despite unfractionated heparin and initial clinical improvement, severe thrombocytopenia developed requiring dexamethasone, rituximab, and romiplostim. Hospitalization was complicated further by thrombosis of the inferior vena cava, pulmonary embolism, and painful violaceous patches on his neck and ear cartilages. Punch biopsy of lesions revealed C5b-C9 deposition of small vessel thromboses. Although the inciting event for his thrombotic storm remains uncertain, anti-complement therapy with eculizumab provided rapid and durable lesion resolution. Eculizumab was discontinued after 6 months and patient remains in remission without recurrent thrombosis. This case provides insight on the management of CAPS, including the use of eculizumab. Keywords Thrombotic Storm · Antiphospholipid syndrome · Anticomplement therapy
Highlights • Micro thrombosis in the ear cartilage is an unusual pres-
entation of catastrophic antiphospholipid syndrome.
• Biopsy of affected area showed marked C5b-C9 comple-
ment deposition.
• Rapid initiation of anticomplement therapy led to a suc-
cessful outcome.
• This case report underscores the importance of recogniz-
ing complement activation in the pathogenesis of catastrophic antiphospholipid syndrome.
This case was presented as a poster at the ISTH 2019 congress in Melbourne, Australia. * Maria T. DeSancho [email protected] 1
Division of Hematology‑Oncology, Department of Medicine, Weill Cornell Medicine, New York Presbyterian Hospital, New York, NY, USA
The antiphospholipid syndrome (APS) is an autoimmune clinico-pathologic disorder characterized by thrombosis and/or pregnancy morbidity along with persistent antiphospholipid (aPL) antibodies. Patients with APS can experience venous, arterial, and/or microvascular thrombosis [1]. Catastrophic antiphospholipid syndrome (CAPS) is an unusual but often fatal complication of APS distinguished by microvascular thrombosis manifesting as multiorgan failure in three or more organs. Severity correlates with extent of thrombosis and the organs involved, and the damage occurring within one week, or up to one month after initial onset [2]. There is increasing evidence that complement is activated in patients with antiphospholipid antibodies (aPL) and may function as a cofactor in the pathogenesis of aPL r
Data Loading...
