Familial isolated congenital asplenia
MONDO:0010066Familial isolated congenital asplenia is a rare, non-syndromic, potentially life-threatening visceral malformation characterized by the absence of normal spleen function, resulting in a primary immunodeficiency. Typically, the condition manifests with severe, recurrent, overwhelming infections (especially pneumococcal sepsis) in otherwise apparently healthy infants. In adults with no history of severe sepsis in infancy, thrombocytosis may be the presenting sign. Howell-Jolly bodies on blood smears and an absent spleen on abdominal ultrasound examination are highly suggestive associated findings.
Also known as: ICAS, asplenia, familial, asplenia, isolated congenital, hyposplenia, isolated congenital, splenic hypoplasia
47 clinical trials for this condition and its sub-types, 6 tagged with Familial isolated congenital asplenia itself.
Follow this condition to get notified about new trialsWhere it sits in the disease tree
Browse by category →-
Can a vaccine protect spleen injury patients from deadly infections?
Disease control Recruiting nowPeople without a working spleen are at high risk of severe infections from pneumococcus bacteria. This study looks at how well the pneumococcal vaccine works in adults aged 18-65 who have a spleen injury and are treated with monitoring, embolization, or spleen removal. Researcher…
Phase 2 • Sponsor: University of California, Davis • Aim: Disease control
Last updated Aug 20, 2026 00:00 UTC
-
New vaccine strategy could shield spleenless patients from deadly infection
Disease control Recruiting nowThis phase 3 trial is testing three different ways to give the meningococcal B vaccine to adults who have had their spleen removed. People without a spleen are at higher risk for serious infections. The study will compare how well each vaccine schedule triggers the immune system …
Phase 3 • Sponsor: Assistance Publique - Hôpitaux de Paris • Aim: Disease control
Last updated Jun 27, 2026 07:58 UTC
-
Spleenless patients under the microscope: can we predict their biggest health threats?
Knowledge-focused Recruiting nowThis study follows 6,000 adults in France who have had their spleen removed or rendered non-functional due to surgery, radiation, or embolization. Researchers aim to identify risk factors for serious complications such as infections, cancer, and blood clots. By comparing differen…
Sponsor: Poitiers University Hospital • Aim: Knowledge-focused
Last updated Jul 18, 2026 00:00 UTC
-
New survey aims to boost infection safety for Spleen-Free patients
Knowledge-focused Recruiting nowThis study is developing a questionnaire in Italian to find out how much people who have had their spleen removed know about preventing infections. A team of 20 healthcare experts will use a structured method to agree on the best questions. The final tool will help researchers an…
Sponsor: Fondazione Policlinico Universitario Agostino Gemelli IRCCS • Aim: Knowledge-focused
Last updated Jun 27, 2026 12:05 UTC
-
Can a simple chat save lives? new study educates spleen-removed patients on infection dangers
Knowledge-focused Recruiting nowThis study looks at whether giving splenectomy patients a structured lesson about infection risks and recommended vaccines helps them understand and remember better. About 100 adults who had their spleen removed at one hospital will take a quiz before and after the lesson. The go…
Sponsor: Fondazione Policlinico Universitario Agostino Gemelli IRCCS • Aim: Knowledge-focused
Last updated Jun 27, 2026 09:01 UTC