Allogeneic hematopoietic stem cell transplantation for adult HLH: a retrospective study by the chronic malignancies and inborn errors working parties of EBMT

Machowicz, Rafal; Suarez, Felipe; Wiktor-Jedrzejczak, Wieslaw; Eikema, Diderik-Jan; Wreede, Liesbeth C. de; Blok, Henric-Jan; Isaksson, Cecilia; Einsele, Hermann; Poire, Xavier; Dorp, Suzanne van; Nikolousis, Emmanouil; Johansson, Jan-Erik; Kobbe, Guido; Zecca, Marco; Arnold, Renate; Gerbitz, Armin; Finke, Jürgen; Diez-Martin, Jose Luis; Bonifazi, Francesca; McQuaker, Grant; Lenhoff, Stig; Rohrlich, Pierre-Simon; Theobald, Matthias; Ljungman, Per; Collin, Matthew; Albert, Michael H.; Ehninger, Gerhard; Carlson, Kristina; Halaburda, Kazimierz; Lehmberg, Kai; Schoenland, Stefan; Yakoub-Agha, Ibrahim; Gennery, Andrew R.; Lankester, Arjan C. und Kröger, Nicolaus (2022): Allogeneic hematopoietic stem cell transplantation for adult HLH: a retrospective study by the chronic malignancies and inborn errors working parties of EBMT. In: Bone Marrow Transplantation, Bd. 57, Nr. 5: S. 817-823

Abstract

Hemophagocytic lymphohistiocytosis (HLH;hemophagocytic syndrome) is a rare syndrome of potentially fatal, uncontrolled hyperinflammation. Allogeneic hematopoietic stem cell transplantation (allo-HSCT) is indicated in primary, recurrent or progressive HLH, but information about its outcomes in the adult population is limited. We obtained data about 87 adult (>= 18 years of age) patients retrospectively reported to the EBMT. The median survival time was 13.9 months. The three and five-year overall survival (OS) was 44% (95% CI 33-54%). Among 39 patients with a follow-up longer than 15 months, only three died. Relapse rate was 21% (95% CI 13-30%), while NRM reached 36% (95% CI 25-46%). Younger patients (<30 years of age) had better prognosis, with an OS of 59% (95% CI 45-73%) at three and five years vs 23% (95% CI 8-37%) for older ones. No difference in survival between reduced and myeloablative conditioning was found. To our knowledge, this is the largest report of adult HLH patients who underwent allo-HSCT. Patients who survive the first period after this procedure can expect a long disease-free survival. Both reduced intensity and myeloablative conditioning have therapeutic potential in adult HLH.

• Dokumententyp: Zeitschriftenartikel
• Fakultät: Medizin
• Themengebiete: 600 Technik, Medizin, angewandte Wissenschaften > 610 Medizin und Gesundheit
• ISSN: 0268-3369
• Sprache: Englisch