Dip into ASH 2020

Article No. 397

28 November 2020

 

The second major conference at this time of year with research results which matter to aHUS patients is the  American Society of Hematologists’ Conference.

ASN 2020 is to be held in the first week in December, (5 to 8th).

There is a sprinkling of talks and posters presentations about aHUS and related TMA topics.

A quick search finds a talk  is to be given by Prof. Fadi Fakhouri . Fadi  is no stranger to aHUS Global Action and is probably the world’s top authority on aHUS and pregnancy  and that it is what his talk is to be about:

-AHUS in Pregnancy

Program: Scientific Workshops @ ASH
Session: Complement Activation and Thrombotic Microangiopathies in Pregnancy
Friday, December 4, 2020, 8:50 AM-9:50 AM

Fadi Fakhouri, MBBS

ITUN, Dept Nephrology Chu Nantes, Nantes, France

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-1579: Triggers in Patients with Atypical Hemolytic Uremic Syndrome: An Observational Cohort Study Using a US Claims Database

Secondly another topic of interest to aHUS patients is that of what triggers the aHUS? A poster is to be presented by the Tomazos Group from Alexion . The abstract can be read at this LINK. Of the 147 patients studied in the USA , 92 had infections,  28 were on chemotherapy 26 had Lupus (SLE) 10 were pregnant , 9 had a bone marrow stem cell transplant and 6 had graft versus host disease(GVHD)

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-379: Outcomes of a Clinician-Directed Protocol for Discontinuation of Complement Inhibition Therapy in Atypical Hemolytic Uremic SyndromeClinically Relevant Abstract

Discontinuation of eculizumab treatment is an important research topic for aHUS patients. In this study by an India  researcher the results of what happened to 32 aHUS patients are reported  The Abstract can viewed HERE.

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-852 :Defects in Sialic Acid Biosynthesis Cause Dysregulation of the Alternative Pathway of Complement

Another factor which hampers the control of the Complement System has been discovered by the Chen Group of John Hopkins University ( see abstract HERE) . Genetic defects in Sialic acid biosynthesis cause an aHUS patient’s effective CFH not to work properly and the alternative pathway is unregulated as a result . Another explanation of idiopathic aHUS may have been found.

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-Infection Risk, Immunization Recommendations, and Antimicrobial Prophylaxis Needs when Treating Non-Malignant Hematologic Disorders – Wash Your Hands and What Else?

Susceptibility to infection is a side effect of eculizumab treatment. In an educational session Dr Johan Walter will talk about the risks of land ways to mitigate such side effects in those treated with eculizumab. Some information about her talk can be found in this introduction to the session , click HERE

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-831: Von Willebrand Factor Activates the Alternative Complement Pathway In Vivo in a Mouse Model of Complement Thrombotic Microangiopathy

Some research, by Jacobi and Sartain from Texas, on Von Willebrand factor reveals the role it plays in the Complement Systems alternative pathway activation which when excessive can result in aHUS. More information at this link.

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-1860: A Novel pH Dependent C5 Monoclonal Antibody with Better Developability

A new C5 blockade drug candidate was revealed by the Cheng Group of Nanjing China. The research funded by F Hoffmann-La Roche Ltd was undertaken on the novel humanized anti-C5 monoclonal antibody, named LP-005. Results suggest an effectiveness which is long lasting. More information can be read HERE.

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-756 : Rapid Turnaround ADAMTS13 Testing Is Likely to Improve Diagnosis of Thrombotic Thrombocytopenic Purpura and Avoid Unnecessary Plasma Exchange

Although not directly related to aHUS, this research by the McGinnis Group of British Columbia,  Canada finds that by improving the testing for TTP an earlier recognition of it could mean that plasma exchange can be stopped sooner if it is not TTP. Ruling out TTP quicker could therefore speed up diagnosing aHUS as the cause of the TMA.

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So a quick dip into ASH 2020 reveals more answers about aHUS triggers (including pregnancy), treatment side effects and discontinuation, new therapies, and its diagnosis process. All of which helps those with aHUS or yet to experience it.