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Editorial
. 2021 Jun 30;5(7):e607.
doi: 10.1097/HS9.0000000000000607. eCollection 2021 Jul.

The EHA Research Roadmap: Anemias

Achille Iolascon  1   2 , Stefano Rivella  3   4   5   6   7 , Nicholas P Anagnou  8 , Clara Camaschella  9 , Dorine Swinkels  10 , Martina U Muckenthaler  11 , Graça Porto  12 , Wilma Barcellini  13 , Immacolata Andolfo  1   2 , Antonio M Risitano  14 , Antonis Kattamis  15 , Maria Domenica Cappellini  16 , Ali T Taher  17 , Lucia De Franceschi  18 , David Rees  19 , Roberta Russo  1   2 , Hannah Tamary  20 , Reinhard Stauder  21 , Domenico Girelli  22
Affiliations
Editorial

The EHA Research Roadmap: Anemias

Achille Iolascon et al. Hemasphere. .

Erratum in

No abstract available

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Figures

Figure 1.
Figure 1.
Graphical representations of the aims of the Research Roadmap for Anemias in Europe during the period 2020–2025.
Figure 2.
Figure 2.
Schematic representation of pathogenic mechanism of DHS and new discovery on Plasmodium infection. (A) Recent study demonstrated that RBCs of DHS patients with PIEZO1 gain-of-function (GOF) mutations show a low rate of infection by Plasmodium falciparum. (B) RBCs of DHS patients show dehydration that is caused by the increased potassium efflux and increased calcium influx of PIEZO1 GoF mutations or KCNN4/Gardos GoF mutations. Senicapoc, a Gardos antagonist, is able to restore the hydration status in both PIEZO1 and KCNN4 mutants. (C) RBCs of DHS patients with GoF mutations in PIEZO1 show delayed terminal differentiation and reticulocytes maturation. (D) At the hepatic level, PIEZO1 GoF mutations in DHS result in increased intracellular Ca2+ concentrations. The hyperactivation of the mechanoreceptor leads to amplification of the phosphorylation of ERK1/2, which acts in turn by inhibiting the BMP/SMADs pathway. The lack of phosphorylation of SMAD1/5/8 inactivates HAMP transcription.
Figure 3.
Figure 3.
Standard and target therapies for warm autoimmune hemolytic anemia (wAIHA) and cold agglutinin disease (CAD). The figure shows the several immunologic mechanisms involved in pathogenesis, including macrophages, T and B lymphocytes, cytokines, activation of the complement cascade, antibody-dependent cellular cytotoxicity (ADCC) in the spleen and/or complement-dependent cytotoxicity (CDC) in the liver, and possible inadequate bone marrow compensation. Standard therapies include steroids and immunosuppressors that act not specifically on the various mechanisms, and splenectomy. Target therapies are directed against specific immunological mechanisms either inhibiting the immune attack or stimulating bone marrow compensation. APC = antigen presenting cell; BAFF = B-cell activating factor; BM = bone marrow; BTK = Bruton tyrosine kinase; CAD = cold agglutinin disease; EPO = erythropoietin; FcRn = neonatal Fc receptor; MMF = mycophenolate mofetil; PI3K = phosphoinositide 3-kinase; Syk = spleen tyrosine kinase.
See this image and copyright information in PMC

References

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