Articles

Diagnosis and treatment of thrombotic thrombocytopenic purpura

BJH - volume 11, issue 6, october 2020

E. Roose PhD, S. Deconinck , C. Dekimpe , A. Curie , SF. De Meyer PhD, K. VanHoorelbeke PhD, D. Dierickx MD, PhD

SUMMARY

Thrombotic thrombocytopenic purpura (TTP) is a rare and life-threatening thrombotic microangiopathic disorder (TMA) due to a severe deficiency of ADAMTS13 (A Disintegrin And Metalloprotease with Thrombo-Spondin type 1 repeats, member 13). The deficiency in ADAMTS13 can either be caused by mutations in ADAMTS13 (congenital TTP or Upshaw-Schulman syndrome, cTTP) or by anti-ADAMTS13 autoantibodies (immune-mediated TTP, iTTP). Diagnosis of TTP is challenging but crucial for the survival of the patient. TTP should be suspected when microangiopathic haemolytic anaemia and severe thrombocytopenia are observed. A severely decreased ADAMTS13 activity (activity <10%) should confirm the diagnosis of TTP. Standard treatment of TTP is plasma therapy (plasma exchange for iTTP, while plasma infusion for cTTP), but novel therapeutics like rituximab, caplacizumab and recombinant ADAMTS13 show promising results regarding the recovery and sustained remission of TTP patients. However, although major advances have been made in the management of TTP, TTP is a chronic disease and patients still relapse, careful and stringent patient follow-up is needed to improve the patients’ quality of life.

(BELG J HEMATOL 2020;11(6):253-60)

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P30 VWF DEFICIENCY IS ASSOCIATED WITH RETICULOCYTOSIS AND INCREASED PARASITE ACCUMULATION IN EXPERIMENTAL MALARIA-ASSOCIATED ACUTE RESPIRATORY DISTRESS SYNDROME

BJH - 2019, issue ?, february 2019

S. Kraisin , S. Verhenne , T.-T. Pham , K. Martinod , I. Portier , N. VanDePutte , H. Deckmyn PhD, K. VanHoorelbeke PhD, P.E. Van Den Steen , S.F. De Meyer

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P28 THE MECHANISM OF THE LOSS OF HMW VWF MULTIMERS AFTER LEFT VENTRICULAR ASSIST DEVICE IMPLANTATION IS DIFFERENT BETWEEN HUMANS AND SHEEP

BJH - 2019, issue ?, february 2019

S. Deconinck , A.-S. Schelpe , S. Jacobs , C. Nix , S. Barth , H.B. Feys PhD, N. VanDePutte , C. Tersteeg PhD, H. Deckmyn PhD, S.F. De Meyer , B. Meyns , K. VanHoorelbeke PhD

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P27 VON WILLEBRAND FACTOR DEFICIENCY DOES NOT INFLUENCE ANGIOTENSIN I I-INDUCED ABDOMINAL AORTIC ANEURYSM FORMATION IN MICE

BJH - 2019, issue ?, february 2019

I. Portier , K. Martinod , L. Desender , N. VanDePutte , H. Deckmyn PhD, K. VanHoorelbeke PhD, S.F. De Meyer

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P22 ANTI-CUB1 OR ANTI-SPACER ANTIBODIES THAT INCREASE ADAMTS13 ACTIVITY ACT BY ALLOSTERICALLY ENHANCING METALLOPROTEASE DOMAIN FUNCTION

BJH - 2019, issue ?, february 2019

A.-S. Schelpe , A. Petri , N. VanDePutte , H. Deckmyn PhD, S.F. De Meyer , J.t.b. Crawley , K. VanHoorelbeke PhD

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P21 A NOVEL ADAMTS13 CONFORMATION ELISA SHOWS CONFORMATIONAL ACTIVATION OF RAT ADAMTS13 WITH EXPOSURE OF CRYPTIC EPITOPES IN VITRO

BJH - 2019, issue ?, february 2019

C. Dekimpe , L.c. VelÁsquez Pereira , J. Voorberg , H. Deckmyn PhD, S.F. De Meyer , K. VanHoorelbeke PhD

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O07 THE VWF-GPIB INTERACTION MEDIATES THROMBO-INFLAMMATION IN EXPERIMENTAL STROKE VIA RECRUITMENT OF MONOCYTES, NEUTROPHILS AND T-CELLS TO THE BRAIN

BJH - 2019, issue ?, february 2019

F. Denorme , K. Martinod , A. Vandenbulcke , C.v. Denis , P.j. Lenting , H. Deckmyn PhD, K. VanHoorelbeke PhD, S.F. De Meyer

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