Abstract Number: PO156
Meeting: ISTH 2021 Congress
Theme: Platelet Disorders, von Willebrand Disease and Thrombotic Microangiopathies » ADAMTS13 and TTP
Background: The clinical spectrum of immune-mediated thrombotic thrombocytopenic purpura (iTTP) is based on microvascular occlusion caused by platelet-rich thrombi.
Aims: We present a refractory iTTP case complicated by hepatic sinusoidal obstruction syndrome (SOS) and managed with defibrotide.
Methods: Case:
Results: A 36-year-old woman was admitted to the emergency department with weakness and bruises. She was pale, subicteric, and had ecchymoses. She had mild anemia (hemoglobin,9.2×109/L) and severe thrombocytopenia (platelet count,7×109/) with normal hemorrhagic diathesis tests. With increased serum lactate dehydrogenase and indirect bilirubin level associated with decreased haptoglobin and red cell fragmentation on peripheral smear, iTTP was diagnosed. The direct antiglobulin test was negative. Daily therapeutic plasma exchange (TPE), and steroid (methylprednisolone: 1g/day for 3 days and then 1 mg/kg/day) were started. On the second day during TPE, she developed tonic-clonic seizures and transferred to ıntensive care unit as intubated. On the 5th day of TPE and steroid, serum LDH was persistently elevated with a mild increase in platelet count (16×109/L). Rituximab was started (375mg/m2/week). Hepatomegaly and generalized edema as pleural effusion and ascites with an increase in serum direct bilirubin (2.94mg/dL) added to the clinical picture. Hepatic SOS was suspected and defibrotide infusion (25mg/kg/day) was started. At that time the ADAMTS-13 activity was achieved as being 0.00IU/mL with an inhibitör level of >90U/mL. TPE was continued twice-daily with cryopoor plasma. In one-week, fluid retention gradually disappeared. On the 11th day, the platelet count reached the normal level. The patient could be extubated and transferred to the hematology ward. She is still confused and has disorientation and cooperation problems.
Conclusions: The pathogenesis of SOS is multifactorial but begins with the activation of sinusoidal endothelial cells. The disease can be mortal. In our case, it could be the consequence of iTTP or had an undetermined trigger. Defibrotide is given protective effects toward microvascular injury.
To cite this abstract in AMA style:
Erdem S, Mastanzade M, Altay-Dadin S, Yanasik M, Kalayoglu-Besisik S. A Refractory Thrombotic Thrombocytopenic Purpura Case Complicated by Hepatic Sinusoidal Obstruction Syndrome and Successful Management with Defibrotide [abstract]. Res Pract Thromb Haemost. 2021; 5 (Suppl 2). https://abstracts.isth.org/abstract/a-refractory-thrombotic-thrombocytopenic-purpura-case-complicated-by-hepatic-sinusoidal-obstruction-syndrome-and-successful-management-with-defibrotide/. Accessed March 22, 2024.« Back to ISTH 2021 Congress
ISTH Congress Abstracts - https://abstracts.isth.org/abstract/a-refractory-thrombotic-thrombocytopenic-purpura-case-complicated-by-hepatic-sinusoidal-obstruction-syndrome-and-successful-management-with-defibrotide/