The German sickle cell disease registry reveals a surprising risk of acute splenic sequestration and an increased transfusion requirement in patients with compound heterozygous sickle cell disease HbS/β‐thalassaemia and no or low HbA expression

Patients with sickle cell disease (SCD) in Germany exhibit a substantial genetic diversity in the β‐globin genotype. Data collected by the national German SCD registry reflect this diversity and allowed us to analyze the phenotypes associated with different SCD genotypes. Our study focused on 90 pat...

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Published in:	European journal of haematology Vol. 113; no. 4; pp. 501 - 509
Main Authors:	Allard, Pierre, Tagliaferri, Laura, Weru, Vivienn, Cario, Holger, Lobitz, Stephan, Jarisch, Andrea, Kopp‐Schneider, Annette, Lassay, Lisa, Kontny, Udo, Frühwald, Michael, Westphal, Silke, Schulte, Johannes, Oevermann, Lena, Hakimeh, Dani, Eckert, Maike, Khurana, Claudia, Calaminus, Gabriele, Eberl, Wolfgang, Mudler, Astrid, Scheer‐Preis, Johanna, Eberling, Torsten, Pekrun, Arnulf, Fröhling, Stefan, Bernbeck, Benedikt, Lara‐Villacanas, Eusebia, Westkemper, Marco, Brummel, Bastian, Naumann‐Bartsch, Nora, Zierk, Jakob, Aramayo‐Singelmann, Carmen, Erlacher, Miriam, Mauz‐Körholz, Christine, Meinhardt, Andrea, Ströter, Natascha, Körholz, Dieter, Hauch, Holger, Kullmann, Silke, Kühnle, Ingrid, Ebert, Sabine, Asemissen, Anne Marie, Beilken, Andreas, Lamottke, Britta, Maecker‐Kolhoff, Britta, Sander, Annette, Sauer, Martin, Kunz, Joachim, Full, Hermann, Simon, Arne, Krenn, Thomas, Leipold, Alfred, Lacroix, Jeannine, Hartel, Simone, Nathrath, Michaela, Rodehüser, Martina, Vieth, Simon, Heydrich‐Karsten, Christiane, Behr, Ümmügül, Ziehe, Christine, Balzer, Stephan, Belke, Luisa, Simon, Thorsten, Hero, Barbara, Christiansen, Holger, Fischer, Lars, Starke, Sven, Faber, Jörg, Wingerter, Arthur, El Malki, Khalifa, Otto, Henrike, Robinson, Abigale, Theisen‐Riedel, Marie, Dürken, Matthias, Karremann, Michael, El‐Hilali, Marie‐Luise, Albert, Michael, Meilbeck, Rita, Schenk, Daniela, Wawer, Angela, Rössig, Claudia, Corbacioglu, Selim, Föll, Jürgen, Kramer, Sonja, Tröger, Anja, Kietz, Silke, Classen, Carl Friedrich, Schütte, Peter, Reinhard, Harald, Blattmann, Claudia, Knirsch, Stephanie, Ebinger, Martin, Holzer, Ursula, Brecht, Ines, Lang, Peter, Döring, Michaela, Pritschow, Yvonne
Format:	Journal Article
Language:	English
Published:	England Wiley Subscription Services, Inc 01-10-2024
Subjects:	Adolescent Adult anemia Anemia, Sickle Cell - complications Anemia, Sickle Cell - diagnosis Anemia, Sickle Cell - therapy beta-Thalassemia - diagnosis beta-Thalassemia - genetics beta-Thalassemia - therapy Blood diseases Blood levels Blood Transfusion Child Child, Preschool Erythrocytes Female Gene Expression Genetic diversity Genotype Genotypes Germany - epidemiology Hemoglobin A - analysis Hemoglobin A - metabolism Hemoglobin, Sickle - genetics Hemoglobin, Sickle - metabolism Heterozygote Humans Male Middle Aged Phenotype Phenotypes Phenotypic variations Registries sickle cell Sickle cell disease Spleen Thalassemia Young Adult Germany anemia thalassemia sickle cell
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