ADAMTS13 activity and inhibitor

Doldan-Silvero, Adriana; Acevedo-Gadea, Carlos Rodrigo; Habib, Clandine; Freeman, Jonathan; Johari, Vandita

doi:10.1002/ajh.21257

Cited by 9 publications

(1 citation statement)

References 32 publications

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“…Given that without active therapy, relapse is common, (12) especially within the first 18 months, (17, 18) and that relapses are still associated with high morbidity and mortality rates, our prior protocol favored a slow stepwise taper of PE. While plasma therapy may have been safely discontinued earlier in some patients treated by our previous protocol, we ascribed a relatively high value to prevention of early relapse, especially in the absence of a biomarker of such relapse.…”

Section: Discussionmentioning

confidence: 99%

Long‐term management of acquired thrombotic thrombocytopenic purpura using serial plasma ADAMTS13 measurements

Knovich

Farland

Owen

2012

European J of Haematology

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Although significant advances in the understanding of TTP pathophysiology have been made in the last 15 yr, none have yet impacted the empiric treatment paradigm for this disease for which plasmapheresis is the mainstay. Laboratory assays for ADAMTS13 activity and inhibitors can be used to confirm a clinical diagnosis, but the assays are not routinely used to guide treatment. The routine availability of ADAMTS13 testing has allowed our group to tailor plasmapheresis and immunosuppressive therapy in patients under active treatment for TTP. In addition, the concept of establishing immune tolerance, similar to the eradication of a factor VIII inhibitor in patients with congenital or acquired hemophilia, has emerged as an important strategy to prevent early relapse of TTP. With the expected incorporation of recombinant ADAMTS13 into the treatment algorithm over the next several years, we anticipate that readily available ADAMTS13 testing will play an important role in individualized therapy that incorporates enzyme replacement and establishment of immune tolerance.

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Section: Discussionmentioning

confidence: 99%

Long‐term management of acquired thrombotic thrombocytopenic purpura using serial plasma ADAMTS13 measurements

Knovich

Farland

Owen

2012

European J of Haematology

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Cyclosporine therapy in refractory/relapsed patients with thrombotic thrombocytopenic purpura

et al. 2009

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To the editor: The interesting article by van Beers et al. states that functional studies does not offer clues as to the presence of mild pulmonary hypertension (PH) in patients with sickle cell disease (SCD) [1]. However, they did show that although not statistically significant, lung function findings do vary in hemoglobin-SS patients.Studies examining lung function in adults with SCD conclude that pulmonary function is abnormal in 90% of patients and gradually declines over time [2,3]. The most common patterns observed were restrictive lung disease and low diffusing capacity (D LCO ). Few publications include data on both the D LCO and the transfer coefficient (K CO ). However, measuring K CO provides information on pathophysiology, which cannot be obtained just from the D LCO . These measurements help us understand when lung disease is due to a loss of lung units (e.g., pneumonectomy), a process interfering with the alveolar surface (e.g., fibrosis) or the capillaries (e.g., PH).Studies on patients with pulmonary arterial hypertension show that a low D LCO can be used as an early marker of PH, before echocardiographic signs develop [4]. When patients with SCD are screened with echocardiogram up to 36% have evidence of PH depending on the hemoglobin genotype [5,6]. However, in autopsy studies there is histological evidence of PH in up to 75% of patients [7]. D LCO may therefore be a more reliable tool than echocardiogram in predicting PH. Van Beers study shows both the D LCO and K CO were reduced in patients with mild PH [1].We examined lung function results from 32 adults with SCD, average age was 34 years and 30 patients had hemoglobin-SS. The mean forced expiratory volume in 1 sec (FEV 1 ) was 81.8% predicted and the mean forced vital capacity (FVC) was 80.9% predicted. Two patients had an obstructive defect (FEV 1 /FVC < 70%) and 18 patients had a FEV 1 /FVC >80%. The mean D LCO (82.6% AE 3.2%) was lower than predicted but the mean K CO (110.4% AE 3.2%) was higher than predicted. There was no statistical difference between patients with or without a history of acute chest syndrome. The results suggest that in these patients the pathophysiological process may be the loss of lung units.To our knowledge, only two other studies on adults with SCD included data on K CO [8,9]. These studies had similar patient numbers, characteristics and spirometry results. Unlike our study both these studies found the K CO lower than predicted. This implies in these patients a different pathophysiological mechanism was operating. The difference with our patient group may be one of disease severity. The high K CO we found is consistent with an increased blood flow to normal lung. A lower than predicted K CO could imply the patients had developed PH, as suggested by the findings in van Beers study, or diffuse alveolar damage. Only two studies performed in children examined D LCO and K CO and found results similar to our study, perhaps reflecting their lower exposure to pulmonary complications [10,11].We feel both our study and ...

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High Frequency of Acquired ADAMTS13 Deficiency After Hemolysis in Hemiscorpius Lepturus (Scorpion) Stung Children

et al. 2013

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ADAMTS13 activity and inhibitor

Cited by 9 publications

References 32 publications

Long‐term management of acquired thrombotic thrombocytopenic purpura using serial plasma ADAMTS13 measurements

Long‐term management of acquired thrombotic thrombocytopenic purpura using serial plasma ADAMTS13 measurements

Cyclosporine therapy in refractory/relapsed patients with thrombotic thrombocytopenic purpura

High Frequency of Acquired ADAMTS13 Deficiency After Hemolysis in Hemiscorpius Lepturus (Scorpion) Stung Children

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