title: Autoimmune cytopaenia after paediatric intestinal transplantation: a case series.
abstract: Autoimmune cytopaenia is a rare, but severe complication after solid organ transplantation. We retrospectively analysed 57 paediatric intestinal transplants performed in 49 patients between 1999 and 2009. Autoimmune cytopaenia was observed in six patients; it appeared after an average of 10 months post-transplant. Warm autoimmune haemolytic anaemia was developed in three patients, cold autoimmune haemolytic anaemia in one and two presented a mixed type. Incidence and causes for haematological cytopaenia such as the following were investigated: immunosuppression, major blood mismatch, viral infection, malignancy, passenger lymphocyte syndrome and lymphoproliferative disorders. Initial treatment included high-dose steroids, intravenous immunoglobulin, plasmapheresis and maintenance of body temperature above 37°C in those with cold autoantibodies. Inclusion of the spleen in multivisceral transplants seems to be an important risk factor. All patients, except one, relapsed after classic therapy, requiring additional treatments. Sirolimus conversion was performed in four patients. One died after infection. The immunosuppressive therapies associated with other concomitant factors, such as viral infections, lymphoproliferative disorders, graft-versus-host disease, passenger lymphocyte syndrome and the inclusion of the spleen as part of multivisceral graft seem to play an important part in the development of autoimmune processes after intestinal transplantation. Therapy is not well established, especially in those resistant to first-line treatment.
authors.affiliation: Department of Pediatric Gastroenterology and Nutrition, Pediatric Intestinal Rehabilitation Unit, Hospital Infantil La Paz, Madrid, Spain.
authors.forename: Gonzalo
authors.initials: G
authors.lastname: Botija
authors.forename: Marta
authors.initials: M
authors.lastname: Ybarra
authors.forename: Esther
authors.initials: E
authors.lastname: Ramos
authors.forename: Manuel
authors.initials: M
authors.lastname: Molina
authors.forename: Jesús
authors.initials: J
authors.lastname: Sarría
authors.forename: Eva
authors.initials: E
authors.lastname: Martínez-Ojinaga
authors.forename: Ane Miren
authors.initials: AM
authors.lastname: Andrés
authors.forename: Manuel
authors.initials: M
authors.lastname: López-Santamaría
authors.forename: Gerardo
authors.initials: G
authors.lastname: Prieto
mesh_terms: D000074323:Alemtuzumab; D000744:Anemia, Hemolytic, Autoimmune / Q000188:drug therapy / Q000209:etiology* / Q000401:mortality; D000911:Antibodies, Monoclonal / Q000627:therapeutic use; D061067:Antibodies, Monoclonal, Humanized; D000912:Antibodies, Neoplasm / Q000627:therapeutic use; D002648:Child; D005260:Female; D006084:Graft Rejection; D006801:Humans; D007165:Immunosuppression Therapy / Q000009:adverse effects*; D007422:Intestines / Q000637:transplantation*; D008232:Lymphoproliferative Disorders / Q000209:etiology; D008297:Male; D012189:Retrospective Studies; D020123:Sirolimus / Q000627:therapeutic use; D013154:Spleen / Q000637:transplantation; D014184:Transplantation, Homologous / Q000009:adverse effects*; D014777:Virus Diseases / Q000150:complications
chemical_list: D000911:Antibodies, Monoclonal; D061067:Antibodies, Monoclonal, Humanized; D000912:Antibodies, Neoplasm; D000074323:Alemtuzumab; D020123:Sirolimus