The spectrum of Evans syndrome in adults: new insight into the disease based on the analysis of 68 cases

Michel, Marc; Chanet, V.; Dechartres, Agnès; Morin, Anne‐Sophie; Piette, Jean‐Charles; Cirasino, Lorenzo; Emilia, Giovanni; Zaja, Francesco; Ruggeri, Marco; Andrès, Emmanuel; Bierling, Philippe; Godeau, Bertrand; Rodeghiero, Francesco

doi:10.1182/blood-2009-04-215368

Cited by 239 publications

(310 citation statements)

References 37 publications

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“…Patients with a history of simultaneous or sequential immune thrombocytopenia (ITP) defining Evans' syndrome [12] were included if the aforementioned criteria for wAIHA were fulfilled. We excluded patients with a C 3d -type only positive DAT result due to solely the presence of cold agglutinins.…”

Section: Methodsmentioning

confidence: 99%

Characteristics and outcome of warm autoimmune hemolytic anemia in adults: New insights based on a single‐center experience with 60 patients

et al. 2014

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Warm autoimmune hemolytic anemia (wAIHA) is a rare autoimmune disease with poorly known natural history and management remaining mainly empirical. To better describe the characteristics and outcome of wAIHA in adults, we performed a single-center cohort study of patients diagnosed with wAIIHA from 2001 to 2012 in our center. Sixty patients (50% women) were included, the mean age at the time of wAIHA onset was 54 6 23 years. wAIHA was considered "primary" for 21 patients (35%) and was associated with an underlying disorder in 39 (65%), including mainly lymphoproliferative disorders and systemic lupus. All patients but two needed treatment and received corticosteroids, with an overall initial response rate of 87%. However, 63% of the patients were corticosteroid-dependent and 56% required at least one second-line treatment including mainly rituximab (n 5 19). At the time of analysis, after a mean follow-up of 46 months, 28 patients (47%) were in remission and off treatment and 5 (8%) had died. The presence of an underlying lymphoproliferative disorder was associated with reduced response to corticosteroids and increased need for second-line therapy. In conclusion, in the last decade and compared to a previous series from our center, the rate of secondary wAIHA has increased and the use of rituximab has emerged as the preferred second-line treatment and corticosteroid-sparing strategy; the overall mortality has significantly decreased (8 vs. 18%).Am. J. Hematol. 89:E150-E155,

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Section: Methodsmentioning

confidence: 99%

Characteristics and outcome of warm autoimmune hemolytic anemia in adults: New insights based on a single‐center experience with 60 patients

et al. 2014

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“…Michel et al analyzed the data from 68 patients with Evans syndrome including 3 DAT-negative cases (4). Half of the patients were associated with an underlying disorder including lymphoproliferative disorders and two patients with B-cell non-Hodgkin lymphoma was included (4).…”

Section: Discussionmentioning

confidence: 99%

“…Among autoimmune diseases, combination of immune thrombocytopenia and autoimmune hemolytic anemia, which is known as Evans syndrome, may precede lymphoma (3,4). Treatment of these cytopenias is the same for primary cases although coincident presence of lymphoma is a clinical problem.…”

Section: Introductionmentioning

confidence: 99%

Efficacy of bendamustine on thrombocytopenia and hemolytic anemia secondary to CD5-positive B-cell lymphoma with massive splenomegaly in a patient with rheumatoid arthritis

Hosoda

Hagino

Hino

et al. 2017

Molecular and Clinical Oncology

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Abstract. Chemotherapy for lymphoma may be avoided in the presence of coincident cytopenia. In case of immune cytopenia secondary to lymphoma, treatment of cytopenia is the same for primary cases, however, chemotherapy for lymphoma may be effective at the cost of severe hematological toxicity. The present study reports a complex case of thrombocytopenia and direct antiglobulin test-negative hemolytic anemia, thus mimicking Evans syndrome, secondary to cluster of differentiation 5-positive B-cell lymphoma with massive splenomegaly, in a patient suffering from rheumatoid arthritis for two decades. Treatment with prednisolone, high-dose dexamethasone, eltrombopag and rituximab for cytopenia were not effective. Chemotherapy with bendamustine subsequently resolved the cytopenia, additionally resulting in a complete remission of lymphoma. Thus, bendamustine may have a role in the management of lymphoma complicated with severe cytopenia.

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“…A second cytopenia may emerge over the course of 10 years [17]. Patient 14 presented with AIHA and also had ITP 3 years later.…”

Section: Sarper Et Al Autoimmune Hemolytic Anemia In Children and Admentioning

confidence: 99%

“…ES is more than a coincidental combination of immune cytopenias; rather, it is a chronic state of profound dysregulation of the immune system [17]. Despite discrepancies in descriptions, all researchers conclude that cytopenias are recurrent, often refractory to IVIG, corticosteroids, and splenectomy, and that the syndrome may be fatal [18].…”

Section: Sarper Et Al Autoimmune Hemolytic Anemia In Children and Admentioning

confidence: 99%

Management of autoimmune hemolytic anemia in children and adolescents: A single center experience

Sarper¹,

Kılıç²,

Zengin³

et al. 2011

Turk J Hematol

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Objective: To present and discuss the treatment of autoimmune hemolytic anemia (AIHA). Materials and Methods: The medical records of all patients (n=19) diagnosed in a tertiary hematology center between 1999 and 2010 were retrospectively reviewed. Results: Median age at diagnosis of AIHA was 5 years (range: 4 months-17 years). In all, 13 patients had primary (idiopathic) AIHA, whereas 2 had primary Evans Syndrome (ES), 2 had autoimmune lymphoproliferative syndrome (ALPS)+ES, and 1 had Wiskott-Aldrich syndrome (WAS)+AIHA. Among the 13 primary idiopathic AIHA patients, 9 recovered following a 4-8-week course of prednisolone treatment without relapses, whereas 3 patients required a longer course of prednisolone. One AIHA patient that was very resistant to prednisolone recovered after cyclosporine A was added to the treatment. All patients with primary idiopathic AIHA were in remission for a median of 3 years (range: 4 months-10 years) at the time this manuscript was written. Among the patients with primary ES, 2 had relapses similar to the ALPS patients. Splenectomy was performed in 1 primary ES patient, who at the time this report was written was also in remission. One ALPS patient required the addition of mycophenolate mofetil due to prednisolone resistance. The WAS patient was treatment resistant and died due to septicemia. Conclusions: Primary AIHA in pediatric patients generally has an acute onset and good response to corticosteroids. Primary or secondary ES has a chronic or relapsing course, and treatment may require other immunosuppressive agents in addition to corticosteroids. Complications of splenectomy must not be underestimated in patients with underlying immunodeficiency. AIHA often causes considerable morbidity and mortality in WAS. (Turk J Hematol 2011; 28: 198-205)

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The spectrum of Evans syndrome in adults: new insight into the disease based on the analysis of 68 cases

Cited by 239 publications

References 37 publications

Characteristics and outcome of warm autoimmune hemolytic anemia in adults: New insights based on a single‐center experience with 60 patients

Characteristics and outcome of warm autoimmune hemolytic anemia in adults: New insights based on a single‐center experience with 60 patients

Efficacy of bendamustine on thrombocytopenia and hemolytic anemia secondary to CD5-positive B-cell lymphoma with massive splenomegaly in a patient with rheumatoid arthritis

Management of autoimmune hemolytic anemia in children and adolescents: A single center experience

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