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Characterization of bleeding in thrombotic thrombocytopenic purpura in the precaplacizumab era: a retrospective nationwide analysis.
Res Pract Thromb Haemost
January 2025
Division of Hematology, Department of Medicine, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.
Background: The addition of caplacizumab to immune thrombotic thrombocytopenia (iTTP) treatment options has led to a renewed interest in characterizing the epidemiology and risk factors for bleeding in iTTP. Limited data exist on the bleeding risk in iTTP due to systemic underreporting in earlier cohorts.
Objectives: To describe the incidence, patterns, and predictors of bleeding in hospitalized iTTP patients independent of caplacizumab use.
Case report: Rituximab combined with plasma exchange treatment for systemic lupus erythematosus complicated with thrombotic microangiopathy and non-cirrhotic portal hypertension.
Front Immunol
January 2025
Department of Rheumatology and Immunology, the Second Affiliated Hospital of Xiamen Medical College, Xiamen, China.
Introduction: Systemic lupus erythematosus (SLE) complicated by thrombotic microangiopathy (TMA) and non-cirrhotic portal hypertension (NCPH) is rare. We present a case of a female patient with SLE who developed TMA and NCPH and responded positively to rituximab and plasma exchange treatment.
Case Description: A 53-year-old woman was admitted with 6 h of confusion.
Safety and Efficacy of DPMAS in Patients With Acute-on-Chronic Liver Failure Based on Different Platelet Count Levels.
J Clin Apher
February 2025
Department of Gastroenterology and Hepatology, Tianjin Union Medical Center, Tianjin Medical University, Tianjin, China.
To investigate the effect of different platelet (PLT) counts on the safety and efficacy of the double-plasma molecular absorption system (DPMAS) in patients with acute-on-chronic liver failure (ACLF). A total of 156 patients with ACLF receiving DPMAS were divided into the observed group (40 × 10/L ≤ PLT < 50 × 10/L) and the control group (PLT ≥ 50 × 10/L) according to PLT count level. The safety and efficacy indices of bleeding-related complications, PLT reduction rate, total bilirubin (TBIL) reduction rate, and 28-days survival rate after DPMAS were analyzed and compared between the two groups.
View Article and Find Full Text PDFEfficacy of apheresis in antibody-negative progressive encephalomyelitis with rigidity and myoclonus.
Ther Apher Dial
January 2025
Department of Neurology, Wakayama Medical University, Wakayama, Japan.
Introduction: Progressive encephalomyelitis with rigidity and myoclonus (PERM) is characterized by brainstem symptoms, muscle rigidity, and myoclonus. While autoantibodies to inhibitory neurons have been associated with the pathology, about 30% of cases are negative for autoantibodies. There are few reported cases of antibody-negative PERM and its clinical course and prognosis are unknown.
View Article and Find Full Text PDFTherapeutic plasmapheresis as an effective treatment for refractory anti-3-hydroxy-3-methylglutaryl coenzyme A reductase-positive immune-mediated necrotising myopathy.
BMJ Case Rep
January 2025
Faculty of Health and Medical Sciences, The University of Adelaide, Adelaide, South Australia, Australia.
Immune-mediated necrotising myopathy (IMNM) can be associated with autoantibodies to 3-hydroxy-3-methylglutaryl coenzyme A reductase (anti-HMGCR). We present a case of a man in his 60s with a 13-year history of relapsing anti-HMGCR-positive IMNM, intermittently partially responsive to various treatments including corticosteroids, methotrexate, mycophenolate, intravenous immunoglobulin, abatacept and rituximab. After a repeat presentation with severe weakness, plasmapheresis was commenced, resulting in rapid and significant improvement in muscle strength and biochemical markers, which was sustained for several months.
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