{"id":59915,"date":"2026-03-28T11:00:00","date_gmt":"2026-03-28T10:00:00","guid":{"rendered":"https:\/\/inmuno.es\/index.php\/2026\/03\/28\/allogeneic-hematopoietic-cell-transplantation-for-congenital-athymia-a-nationwide-retrospective-study-in-japan\/"},"modified":"2026-03-28T11:00:00","modified_gmt":"2026-03-28T10:00:00","slug":"allogeneic-hematopoietic-cell-transplantation-for-congenital-athymia-a-nationwide-retrospective-study-in-japan","status":"publish","type":"post","link":"https:\/\/inmuno.es\/index.php\/2026\/03\/28\/allogeneic-hematopoietic-cell-transplantation-for-congenital-athymia-a-nationwide-retrospective-study-in-japan\/","title":{"rendered":"Allogeneic Hematopoietic Cell Transplantation for Congenital Athymia: A Nationwide Retrospective Study in Japan"},"content":{"rendered":"<div>\n<p><b>J Clin Immunol<\/b>. 2026 Mar 28. doi: 10.1007\/s10875-026-02008-y. Online ahead of print.<\/p>\n<p><b>ABSTRACT<\/b><\/p>\n<p>PURPOSE: Congenital athymia is a life-threatening condition characterized by thymic absence and profound T-cell immunodeficiency. Thymus implantation is the definitive treatment, but its availability is limited. This study aimed to evaluate the outcomes of hematopoietic cell transplantation (HCT) as an alternative therapy.<\/p>\n<p>METHODS: A nationwide, multicenter, retrospective study analyzed nine patients who underwent allogeneic HCT between 2000 and 2024 in Japan. Overall survival (OS) was estimated using the Kaplan-Meier method, and the cumulative incidence of immunodeficiency-related mortality was assessed using Gray&#8217;s test.<\/p>\n<p>RESULTS: Among the nine patients, eight (89%) received umbilical cord blood, and one (11%) received related peripheral blood. Seven patients (78%) underwent transplantation without conditioning. Engraftment with T-cell recovery was achieved in six patients (67%), with a median CD4<sup>+<\/sup> T-cell count of 0.352 \u00d7 10<sup>9<\/sup>\/L (range, 0.216-1.578 \u00d7 10<sup>9<\/sup>\/L) at the last follow-up. Acute graft-versus-host disease (GVHD) occurred in five patients (56%), all with Grade I-II skin involvement. No chronic GVHD was observed. The one-year OS rate was 66.7% (95% confidence interval: 28.2%-87.8%). Overall, six patients (67%) died: three early deaths within the first month from infections, and three late deaths beyond 1 year from congenital comorbidities. HCT before six months of age was associated with significantly lower immunodeficiency-related mortality (p = 0.02).<\/p>\n<p>CONCLUSION: HCT can allow immune reconstitution in congenital athymia, although long-term survival is affected by comorbidities. Early diagnosis and timely intervention are crucial in managing this condition. HCT can be a restricted alternative therapy for patients ineligible for thymus implantation.<\/p>\n<p>PMID:<a href=\"https:\/\/pubmed.ncbi.nlm.nih.gov\/41902846\/?utm_source=SimplePie&amp;utm_medium=rss&amp;utm_campaign=journals&amp;utm_content=8102137&amp;ff=20260329074109&amp;v=2.19.0.post6+133c1fe\">41902846<\/a> | DOI:<a href=\"https:\/\/doi.org\/10.1007\/s10875-026-02008-y\">10.1007\/s10875-026-02008-y<\/a><\/p>\n<\/div>","protected":false},"excerpt":{"rendered":"<p>J Clin Immunol. 2026 Mar 28. doi: 10.1007\/s10875-026-02008-y. Online ahead of print. ABSTRACT PURPOSE: Congenital athymia is a life-threatening condition characterized by thymic absence and profound T-cell immunodeficiency. Thymus implantation is the definitive treatment, but its availability is limited. This study aimed to evaluate the outcomes of hematopoietic cell transplantation (HCT) as an alternative therapy. &#8230; <a title=\"Allogeneic Hematopoietic Cell Transplantation for Congenital Athymia: A Nationwide Retrospective Study in Japan\" class=\"read-more\" href=\"https:\/\/inmuno.es\/index.php\/2026\/03\/28\/allogeneic-hematopoietic-cell-transplantation-for-congenital-athymia-a-nationwide-retrospective-study-in-japan\/\" aria-label=\"Read more about Allogeneic Hematopoietic Cell Transplantation for Congenital Athymia: A Nationwide Retrospective Study in Japan\">Read more<\/a><\/p>\n","protected":false},"author":1,"featured_media":0,"comment_status":"open","ping_status":"open","sticky":false,"template":"","format":"standard","meta":{"footnotes":""},"categories":[69,42],"tags":[],"class_list":["post-59915","post","type-post","status-publish","format-standard","hentry","category-journal-of-clinical-immunology","category-publicaciones"],"_links":{"self":[{"href":"https:\/\/inmuno.es\/index.php\/wp-json\/wp\/v2\/posts\/59915","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/inmuno.es\/index.php\/wp-json\/wp\/v2\/posts"}],"about":[{"href":"https:\/\/inmuno.es\/index.php\/wp-json\/wp\/v2\/types\/post"}],"author":[{"embeddable":true,"href":"https:\/\/inmuno.es\/index.php\/wp-json\/wp\/v2\/users\/1"}],"replies":[{"embeddable":true,"href":"https:\/\/inmuno.es\/index.php\/wp-json\/wp\/v2\/comments?post=59915"}],"version-history":[{"count":0,"href":"https:\/\/inmuno.es\/index.php\/wp-json\/wp\/v2\/posts\/59915\/revisions"}],"wp:attachment":[{"href":"https:\/\/inmuno.es\/index.php\/wp-json\/wp\/v2\/media?parent=59915"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/inmuno.es\/index.php\/wp-json\/wp\/v2\/categories?post=59915"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/inmuno.es\/index.php\/wp-json\/wp\/v2\/tags?post=59915"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}