scholarly journals Alternate‐day dosing of caplacizumab for immune‐mediated thrombotic thrombocytopenic purpura

2022 ◽  
Author(s):  
Lucas Kühne ◽  
Jessica Kaufeld ◽  
Linus A. Völker ◽  
Ralph Wendt ◽  
Ulf Schönermarck ◽  
...  
Keyword(s):  
Thrombotic Thrombocytopenic Purpura ◽  
Thrombocytopenic Purpura ◽  
Immune Mediated

Dasatinib-induced immune mediated-thrombotic thrombocytopenic purpura

2018 ◽  
Vol 57 (2) ◽  
pp. 222-224 ◽  
Author(s):  
Esra Terzi Demirsoy ◽  
Ozgur Mehtap ◽  
Elif Birtas Atesoglu ◽  
Pinar Tarkun ◽  
Necmi Eren ◽  
...  
Keyword(s):  
Thrombotic Thrombocytopenic Purpura ◽  
Thrombocytopenic Purpura ◽  
Immune Mediated

scholarly journals The HLA Variant rs6903608 Is Associated with Disease Onset and Relapse of Immune-Mediated Thrombotic Thrombocytopenic Purpura in Caucasians

2020 ◽  
Vol 9 (10) ◽  
pp. 3379
Author(s):  
Ilaria Mancini ◽  
Elisa Giacomini ◽  
Silvia Pontiggia ◽  
Andrea Artoni ◽  
Barbara Ferrari ◽  
...  
Keyword(s):  
Thrombotic Thrombocytopenic Purpura ◽  
Risk Allele ◽  
Absolute Risk ◽  
Disease Onset ◽  
Close Monitoring ◽  
Reference Allele ◽  
Thrombocytopenic Purpura ◽  
Immune Mediated ◽  
The Impact ◽  
Risk Of Relapse

Immune-mediated thrombotic thrombocytopenic purpura (iTTP) is a rare, life-threatening thrombotic microangiopathy caused by severe ADAMTS13 (a disintegrin and metalloproteinase with thrombospondin motifs 13) deficiency, recurring in 30–50% of patients. The common human leukocyte antigen (HLA) variant rs6903608 was found to be associated with prevalent iTTP, but whether this variant is associated with disease relapse is unknown. To estimate the impact of rs6903608 on iTTP onset and relapse, we performed a case-control and cohort study in 161 Italian patients with a first iTTP episode between 2002 and 2018, and in 456 Italian controls. Variation in rs6903608 was strongly associated with iTTP onset (homozygotes odds ratio (OR) 4.68 (95% confidence interval (CI) 2.67 to 8.23); heterozygotes OR 1.64 (95%CI 0.95 to 2.83)), which occurred over three years earlier for each extra risk allele (β −3.34, 95%CI −6.69 to 0.02). Of 153 survivors (median follow-up 4.9 years (95%CI 3.7 to 6.1)), 44 (29%) relapsed. The risk allele homozygotes had a 46% (95%CI 36 to 57%) absolute risk of relapse by year 6, which was significantly higher than both heterozygotes (22% (95%CI 16 to 29%)) and reference allele homozygotes (30% (95%CI 23 to 39%)). In conclusion, HLA variant rs6903608 is a risk factor for both iTTP onset and relapse. This newly identified biomarker may help with recognizing patients at high risk of relapse, who would benefit from close monitoring or intensified immunosuppressive therapy.

scholarly journals Presenting ADAMTS13 antibody and antigen levels predict prognosis in immune-mediated thrombotic thrombocytopenic purpura

Blood ◽  
2017 ◽  
Vol 130 (4) ◽  
pp. 466-471 ◽  
Author(s):  
Ferras Alwan ◽  
Chiara Vendramin ◽  
Karen Vanhoorelbeke ◽  
Katy Langley ◽  
Vickie McDonald ◽  
...  
Keyword(s):  
Thrombotic Thrombocytopenic Purpura ◽  
Fold Increase ◽  
Thrombocytopenic Purpura ◽  
Immune Mediated ◽  
Key Points

Key Points High anti-ADAMTS13 antibody and low ADAMTS13 antigen levels adversely affect outcome in immune-mediated TTP with greater mortality seen. A raised troponin at presentation confers a sixfold increase and reduced GCS a nine-fold increase in mortality in acute TTP.

scholarly journals Systemic Lupus Erythematosus Presenting as Thrombotic Thrombocytopenia Purpura: How Close Is Close Enough?

2011 ◽  
Vol 2011 ◽  
pp. 1-3 ◽  
Author(s):  
Cesar A. Perez ◽  
Nabil Abdo ◽  
Anuj Shrestha ◽  
Edgardo S. Santos
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Thrombotic Thrombocytopenic Purpura ◽  
Lupus Erythematosus ◽  
Systemic Lupus ◽  
Laboratory Findings ◽  
Thrombocytopenic Purpura ◽  
Double Stranded Dna ◽  
Immune Mediated ◽  
Healthy Female ◽  
Life Threatening

Thrombotic thrombocytopenic purpura (TTP) is an uncommon life-threatening disease characterized by microangiopathic hemolytic anemia and thrombocytopenia, commonly associated with infections, malignancy, drugs, and autoimmune diseases. We report a case of 19-year-old previously healthy female that presents with anemia and thrombocytopenia diagnosed with thrombotic thrombocytopenic purpura that was treated successfully with plasmapheresis and corticosteroids. Laboratory findings also revealed antinuclear antibodies and antibodies to double-stranded DNA. Two weeks after presentation developed inflammatory arthritis, fulfilling diagnostic criteria for systemic lupus erythematosus (SLE). Prompt diagnosis and treatment with plasma exchange and corticosteroids should be instituted as soon as the diagnosis of TTP is suspected, even if other diagnoses, including lupus, are possible. When present, the coexistence of these two etiologies can have a higher mortality than either disease alone. An underlying diagnosis of SLE should be considered in all patients presenting TTP and the study of this association may provide a better understanding of their immune-mediated pathophysiology.

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