Fatal Evans’ syndrome after matched unrelated donor transplantation for hyper‐IgM syndrome

Urban, C; Benesch, Martin; Sovinz, Petra; Schwinger, Wolfgang; Lackner, Herwig

doi:10.1111/j.1600-0609.2004.00256.x

Cited by 20 publications

(15 citation statements)

References 18 publications

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“…In contrast, although none of our patients died after transplant, the overall clinical course of post-HCT AIC indicated a transplant-related complication that failed to resolve despite prolonged treatment, a characteristic supported by other studies [222]. As well as MMF, second line therapies that have been reported to be effective in the post-HCT setting include romiplostim for ITP and sirolimus for AIHA [2324].…”

Section: Discussionsupporting

confidence: 73%

Treatment and response of autoimmune cytopenia occurring after allogeneic hematopoietic cell transplantation in children

et al. 2017

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BackgroundAutoimmune cytopenia (AIC) is a rare complication of allogeneic hematopoietic cell transplantation (HCT). In this study, we reviewed the diagnosis, treatment and response to therapy for pediatric patients with post-HCT AIC at our institution.MethodsOf the 292 allogeneic HCTs performed from January, 2011 to December, 2015 at the Department of Pediatrics, The Catholic University of Korea, seven were complicated by post-HCT AIC, resulting in an incidence of 2.4%.ResultsAll seven patients with post-HCT AIC had received unrelated donor transplant. Six of seven patients had a major donor-recipient blood type mismatch. The subtypes of AIC were as follows: immune thrombocytopenia (ITP) 2, autoimmune hemolytic anemia (AIHA) 2, Evans syndrome 3. Median time from HCT to AIC diagnosis was 3.6 months. All but one patient responded to first line therapy of steroid±intravenous immunoglobulin (IVIG), but none achieved complete response (CR) with this treatment. After a median duration of treatment of 15.3 months, two patients with ITP achieved CR and five had partial response (PR) of AIC. Five patients were treated with rituximab, resulting in the following response: 2 CR, 2 PR, 1 no response (NR). Median time to response to rituximab was 26 days from first infusion. All patients are alive without event.ConclusionPost-HCT AIC is a rare complication that may not resolve despite prolonged therapy. Rapid initiation of second line agents including but not limited to B cell depleting treatment should be considered for those that fail to achieve CR with first line therapy.

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Section: Discussionsupporting

confidence: 73%

Treatment and response of autoimmune cytopenia occurring after allogeneic hematopoietic cell transplantation in children

et al. 2017

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“…5 In addition, Evans syndrome has been described after allogeneic HCT in children and associated with evidence of disseminated CMV infections. 6,7 Indeed, AIHA has been reported in 2.6% of patients after bone marrow and/or peripheral blood progenitor cell transplantation and typically occurs 2 to 25 months after transplant. 8,9 The frequency of AIHA was higher (20%) in SCID children who received T-cell-depleted haploidentical HCT from their parents.…”

Section: Evans Syndrome After Unrelated Cord Blood Transplantation Fomentioning

confidence: 99%

Evans Syndrome After Unrelated Cord Blood Transplantation for Disseminated Langerhans Cell Histiocytosis in a Child

Chen¹,

Hsu³

et al. 2007

Journal of Pediatric Hematology/Oncology

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“…Additional reports have included several cases following cord blood transplants, with resultant AIHA and Evans syndrome. 25,26 There have been reports of successful therapy of AIHA following hematopoietic stem cell transplantation with rituximab.…”

Section: Association Of Immune Hemolysis With Allogeneic Hematopoietimentioning

confidence: 99%

Immune hemolytic anemia—selected topics

Hoffman¹

2009

Hematology

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Autoimmune hemolytic anemia (AIHA) is most often idiopathic. However, in recent years, AIHA has been noted with increased incidence in patients receiving purine nucleoside analogues for hematologic malignancies; it has also been described as a complication of blood transfusion in patients who have also had alloimmunization. As the technology of hematopoietic stem cell transplantation has become more widespread, immune hemolysis in the recipients of ABO-mismatched products has become better recognized. The syndrome is caused by passenger lymphocytes transferred from the donor and, although transient, can be quite severe. A similar syndrome has been observed in recipients of solid organ transplants when there is ABO-incompatibility between donor and recipient. Venous thromboembolism is a little-recognized, though likely common, complication of AIHA, and may in some instances be related to coexistent antiphospholipid antibodies. While AIHA is a well-documented complication of malignant lymphoproliferative disorders, lymphoproliferative disorders may also paradoxically appear as a consequence of AIHA. A number of newer options are available for treatment of AIHA in patients refractory to corticosteroids and splenectomy. Newer immunosuppressives such as mycophenolate mofetil may have a role in such cases. Considerable experience has been accumulating in the last few years with monoclonal antibody therapy, mainly rituximab, in difficult AIHA cases; it appears to be a safe and effective option.

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Fatal Evans’ syndrome after matched unrelated donor transplantation for hyper‐IgM syndrome

Cited by 20 publications

References 18 publications

Treatment and response of autoimmune cytopenia occurring after allogeneic hematopoietic cell transplantation in children

Treatment and response of autoimmune cytopenia occurring after allogeneic hematopoietic cell transplantation in children

Evans Syndrome After Unrelated Cord Blood Transplantation for Disseminated Langerhans Cell Histiocytosis in a Child

Immune hemolytic anemia—selected topics

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