Refractory Evans Syndrome after Autologous Stem Cell Transplantation for Multiple Myeloma: Management with a Second Transplantation

Chihara, Dai; Sakamoto, Takashi; Arimoto-Miyamoto, Kazue; Kishimoto, Wataru; Takeoka, Tomoharu; Tsuji, Masaaki; Ohno, Tatsuharu

doi:10.2169/internalmedicine.49.2922

Cited by 5 publications

(9 citation statements)

References 25 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“… 149 Also, development of refractory ES in a patient with multiple myeloma treated with an autologous stem cell transplantation has been documented. 150 …”

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

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.

show abstract

“… 149 Also, development of refractory ES in a patient with multiple myeloma treated with an autologous stem cell transplantation has been documented. 150 …”

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

show abstract

“…Evans syndrome is found in only 0.8–3.7% of patients with ITP or AIHA at the onset of these conditions, and only four cases of post‐autologous HSCT ES have been reported (Table ) . In the first case, a 28‐year‐old woman developed ES 3 years after receiving autologous bone marrow transplant (ABMT) for recurrent Hodgkin lymphoma.…”

Section: Discussionmentioning

confidence: 96%

“…She underwent four rounds of plasma exchange and was treated with prednisone, azathioprine, and cyclosporine A, which produced a good response, but subsequently died of bronchopneumonia . In the second case, a patient with multiple myeloma who received a second PBSCT for ES also died of infection . In the third case, the patient underwent autologous HSCT for recurrent non‐Hodgkin lymphoma (NHL).…”

Section: Discussionmentioning

confidence: 99%

“…4 Evans syndrome is found in only 0.8-3.7% of patients with ITP or AIHA at the onset of these conditions, 3 and only four cases of post-autologous HSCT ES have been reported (Table 1). [5][6][7][8] In the first case, a 28-year-old woman developed ES 3 years after receiving autologous bone marrow transplant (ABMT) for recurrent Hodgkin lymphoma. She underwent four rounds of plasma exchange and was treated with prednisone, azathioprine, and cyclosporine A, which produced a good response, but subsequently died of bronchopneumonia.…”

Section: Discussionmentioning

confidence: 99%

“…5 In the second case, a patient with multiple myeloma who received a second PBSCT for ES also died of infection. 6 In the third case, the patient underwent autologous HSCT for recurrent non-Hodgkin lymphoma (NHL). She developed ES together with NHL bone marrow relapse on day 49 of the transplantation.…”

Section: Discussionmentioning

confidence: 99%

See 2 more Smart Citations

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

Kato

Okada

Nishida

et al. 2016

Pediatrics International

View full text Add to dashboard Cite

There have been a number of recent reports on the occurrence of autoimmune conditions after autologous hematopoietic stem cell transplantation. We describe a rare case of Evans syndrome (ES) that developed in a 16-year-old patient >1 year after autologous peripheral blood stem cell transplantation for recurrent Hodgkin lymphoma. ES is a rare and frequently refractory condition. No therapy for the condition has been established, and it can often be fatal. In the present case, i.v. cyclosporine A injection was significantly effective against the ES, which has not recurred.

show abstract

A Rare Case of Multiple Myeloma Presenting as Evan’s Syndrome

Karapetians

Bajaj

Valdes

et al. 2019

Journal of Investigative Medicine High Impact Case Reports

View full text Add to dashboard Cite

Multiple myeloma is defined as the neoplastic proliferation of plasma cells resulting in a monoclonal gammopathy. The classic presentation of a patient is someone who presents with bone pain, osteopenia, or new onset fractures. We present a case of multiple myeloma presenting as Evan’s syndrome (ES). Evan’s syndrome is autoimmune hemolytic anaemia with autoimmune thrombocytopenia. A 44-year-old female was referred from her primary physician to the hospital as laboratory testing revealed haemoglobin of 5 gm/dL. The patient reported a two-month history of fatigue and a sixty-pound weight loss. Laboratory results demonstrated autoimmune hemolytic anaemia, C3 positivity, elevated immunoglobulin (Ig)G, elevated lactate dehydrogenase (LDH), low haptoglobin, elevated reticulocyte count, elevated RDW-CV (red blood cell distribution width-corpuscular volume), positive direct Coombs test, thrombocytopenia, and proteinuria, all of which led to an underlying ES. The patient was started on intravenous steroids followed by oral steroids. A flow cytometry, serum protein electrophoresis, and cytogenetics were obtained. A bone marrow biopsy revealed multiple myeloma and she was started on Bortezomib treatment. We present the fifth reported case of Evan’s syndrome and multiple myeloma.

show abstract

Refractory Evans Syndrome after Autologous Stem Cell Transplantation for Multiple Myeloma: Management with a Second Transplantation

Abstract: Abstract

Cited by 5 publications

References 25 publications

Evans syndrome: clinical perspectives, biological insights and treatment modalities

Evans syndrome: clinical perspectives, biological insights and treatment modalities

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

A Rare Case of Multiple Myeloma Presenting as Evan’s Syndrome

Contact Info

Product

Resources

About