Evans syndrome after autologous peripheral blood stem cell transplantation for recurrent Hodgkin lymphoma

Kato, Ikuko; Okada, Hitoshi; Nishida, Tomoko; Konishi, Yukihiko; Kondo, Sonoko; Nishisho, Sae; Igarashi, Takashi; Itoh, Shinji; Kusaka, Takashi

doi:10.1111/ped.13030

Cited by 2 publications

(3 citation statements)

References 9 publications

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“… 146 A child with recurrent Hodgkin lymphoma underwent an autologous blood stem cell transplantation, after 15 months the patient developed ES refractory to steroids and IVIG; cyclosporine A was effective in this case. 147 A female patient with recurrent Hodgkin disease treated with an autologous bone marrow transplant with a conditioning regimen of cyclophosphamide, etoposide and carmustine developed ES. 148 A patient with non-Hodgkin lymphoma with multiple relapses received a peripheral blood stem cell transplant with a conditioning regimen of etoposide, carboplatin and cyclophosphamide; 49 days after transplantation the patient developed ES, non-Hodgkin lymphoma relapsed and died.…”

Section: Third-line Treatmentsmentioning

confidence: 99%

Evans syndrome: clinical perspectives, biological insights and treatment modalities

Jaime‐Pérez¹,

Aguilar-Calderón²,

Salazar‐Cavazos³

et al. 2018

JBM

113

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Evans syndrome (ES) is a rare and chronic autoimmune disease characterized by autoimmune hemolytic anemia and immune thrombocytopenic purpura with a positive direct anti-human globulin test. It is classified as primary and secondary, with the frequency in patients with autoimmune hemolytic anemia being 37%–73%. It predominates in children, mainly due to primary immunodeficiencies or autoimmune lymphoproliferative syndrome. ES during pregnancy is associated with high fetal morbidity, including severe hemolysis and intracranial bleeding with neurological sequelae and death. The clinical presentation can include fatigue, pallor, jaundice and mucosal bleeding, with remissions and exacerbations during the person’s lifetime, and acute manifestations as catastrophic bleeding and massive hemolysis. Recent molecular theories explaining the physiopathology of ES include deficiencies of CTLA-4, LRBA, TPP2 and a decreased CD4/CD8 ratio. As in other autoimmune cytopenias, there is no established evidence-based treatment and steroids are the first-line therapy, with intravenous immunoglobulin administered as a life-saving resource in cases of severe immune thrombocytopenic purpura manifestations. Second-line treatment for refractory ES includes rituximab, mofetil mycophenolate, cyclosporine, vincristine, azathioprine, sirolimus and thrombopoietin receptor agonists. In cases unresponsive to immunosuppressive agents, hematopoietic stem cell transplantation has been successful, although it is necessary to consider its potential serious adverse effects. In conclusion, ES is a disease with a heterogeneous course that remains challenging to patients and physicians, with prospective clinical trials needed to explore potential targeted therapy to achieve an improved long-term response or even a cure.

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Section: Third-line Treatmentsmentioning

confidence: 99%

Evans syndrome: clinical perspectives, biological insights and treatment modalities

Jaime‐Pérez¹,

Aguilar-Calderón²,

Salazar‐Cavazos³

et al. 2018

JBM

113

View full text Add to dashboard Cite

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“…There are only five cases of Evans syndrome after autologous HSCT. [3][4][5][6][7] The mechanism of post-HSCT AIC is unclear but may be the result of impaired or altered immune reconstitution and failure or loss of self-tolerance. Immunological dysregulation can occur in both post-allogeneic and post-autologous HSCT cases.…”

Section: Discussionmentioning

confidence: 99%

“…2 There are only five cases of Evans syndrome after autologous HSCT, and only one pediatric case of pancytopenia after autologous HSCT has been reported. [2][3][4][5][6][7] We herein report the first adult case of autoimmune pancytopenia that developed after autologous HSCT for nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL).…”

Section: Introductionmentioning

confidence: 99%

Severe autoimmune pancytopenia after autologous hematopoietic stem cell transplantation for Hodgkin lymphoma

Fukui

Honda

Takano

et al. 2022

J Clin Exp Hematopathol

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Autoimmune pancytopenia is rarely seen with Hodgkin lymphoma, and only one pediatric case of pancytopenia after autologous hematopoietic stem cell transplantation (HSCT) has been reported. We herein report a case of autoimmune pancytopenia that developed after autologous HSCT for nodular lymphocyte predominant Hodgkin lymphoma (NLPHL). A 56-year-old Japanese woman underwent autologous HSCT for NLPHL. She developed autoimmune pancytopenia seven months after autologous HSCT. In this case, PSL was effective, and the blood cell counts normalized completely. However, the patient suffered from a fatal infection, probably because of immunosuppression caused by prolonged administration of PSL, as well as a history of several chemotherapies and autologous HSCT. To our knowledge, this is the first adult case of autoimmune pancytopenia after autologous HSCT for Hodgkin lymphoma. To further validate the optimal treatment strategy for autoimmune cytopenia after autologous HSCT, more cases are necessary.

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Evans syndrome after autologous peripheral blood stem cell transplantation for recurrent Hodgkin lymphoma

Cited by 2 publications

References 9 publications

Evans syndrome: clinical perspectives, biological insights and treatment modalities

Evans syndrome: clinical perspectives, biological insights and treatment modalities

Severe autoimmune pancytopenia after autologous hematopoietic stem cell transplantation for Hodgkin lymphoma

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