The role of splenectomy in autoimmune hematological disorders: Outdated or still worth considering?

Sys, Judith; Schauwvlieghe, Alexander; Vanderschueren, Steven; Dierickx, Daan

doi:10.1016/j.blre.2017.01.001

Cited by 16 publications

(15 citation statements)

References 141 publications

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“…Splenectomy is the classic second-line therapy in w-AIHA [24]. In our group, seven (10.8%) patients were treated this way, similar to 13% reported in other centers [6].…”

Section: Discussionsupporting

confidence: 84%

Treatment of autoimmune hemolytic anemia: real world data from a reference center in Mexico

et al. 2019

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Background Warm autoimmune hemolytic anemia (w-AIHA) is an uncommon disease with heterogeneous response to treatment. Steroids are the standard treatment at diagnosis, whereas rituximab has recently been recommended as the second-line therapy of choice. Our main objective was to document the response to treatment in patients with newly diagnosed w-AIHA, including the effectiveness of low-dose rituximab as frontline treatment and for refractory disease. Methods Patients with w-AIHA from 2002 to 2017 were included. Relapse-free survival (RFS), probability of maintained response (MR), and time-to-response were analyzed using the Kaplan–Meier method. Response was classified as complete, partial, and no response. Results We included 64 adults with w-AIHA (39 women and 25 men). The median age was 37 (16–77) years. Response rates to steroids alone were 76.7%, rituximab plus steroids, 100%; and cyclophosphamide, 80%. RFS with steroids at 6, 36, and 72 months was 86.3%, 65.1%, and 59.7%, respectively. Eighteen patients received rituximab at 100 mg/wk for 4 weeks plus high-dose dexamethasone as first-line therapy, with RFS at 6, 36, and 72 months of 92.3%, 58.7% and 44.1%, respectively. Eight patients refractory to several lines of therapy were treated with low-dose rituximab, and all achieved a response (three complete response and five partial response) at a median 16 days (95% confidence interval, 14.1–17.8), with a 75% probability of MR at 103 months; the mean MR was 81.93±18 months. Conclusion Outcomes of w-AIHA treatment were considerably heterogeneous. Low rituximab doses plus high dexamethasone doses were effective for refractory disease.

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“…Splenectomy is the classic second-line therapy in w-AIHA [24]. In our group, seven (10.8%) patients were treated this way, similar to 13% reported in other centers [6].…”

Section: Discussionsupporting

confidence: 84%

Treatment of autoimmune hemolytic anemia: real world data from a reference center in Mexico

et al. 2019

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“…A study involving physicians from Oklahoma showed that experienced hematologists-oncologists were more likely to choose splenectomy as second-line treatment 30 . Thus, splenectomy remains the standard second-line option, although rituximab tends to achieve similar results 2, 27. In our service, if the platelet count is still under 20 × 10 9 /L, we propose splenectomy in persistent and chronic ITP (Figure 1, Figure 2).…”

Section: Discussionmentioning

confidence: 89%

“…In one study, the overall initial response rate of rituximab was 57%, while 21% of responders maintained treatment-free response for at least 5 years without significant toxicity 32 . Rituximab also had an influence on the different T-cell subsets, including restoration of the Th1/Th2 balance and elevation of immune regulatory T cells (Tregs), which suppress cell-mediated and antibody-mediated responses and protect the host against autoimmunity 2, 33, 34. The combination of rituximab and corticoids improves immunomodulation and the effect is more persistent 35 and platelet counts >50 × 10 9 /L are sustained longer than when the corticoid is used alone.…”

Section: Discussionmentioning

confidence: 99%

“…However, the pathogenesis of ITP is complex, as it involves multiple aspects of immune deregulation and a range of different cell types (T and B lymphocytes, macrophages, dendritic cells, plasma cells), resulting in shorter platelet survival and impaired platelet production. Serum thrombopoetin levels are also reduced in ITP 1, 2. The immune nature of ITP is clear and a platelet count of 100 × 10 9 /L was proposed as the threshold level to consider the diagnosis of ITP 3 …”

Section: Introductionmentioning

confidence: 99%

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Proposal of treatment algorithm for immune thromocytopenia in adult patients of a hematology service at a referral center in Northeastern Brazil

Ribeiro

Neto

Furtado

et al. 2019

Hematology, Transfusion and Cell Therapy

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Introduction The management of adult (≥18 years) immune thrombocytopenia patients relies on platelet count, the risk of bleeding and presence of bleeding. Objective Confirming the diagnosis of immune thrombocytopenia and the start of therapy, our hematology service, a referral center, favors the establishment of this algorithm to treat those patients. Results Presentation, recently diagnosed or recurrence – group 1: life-threatening bleeding: high-dose intravenous immunoglobulins with methylprednisolone or dexamethasone. Hospitalization and platelet transfusion are considered. Group 2: Platelets <30 × 10 9 /L with bleeding or risk factor for bleeding, or platelets <20 × 10 9 /L: prednisone or dexamethasone. No response, platelets <20 × 10 9 /L: replace corticoid or increase doses. If platelets continue <20 × 10 9 /L: immunization and splenectomy. Investigation of Helicobacter pylori , if positive: treatment for H. pylori. Chronic immune thrombocytopenia with platelets <20 × 10 9 /L we propose two new groups (A and B): Group A: <65 years, no or low surgical risk, patient declines maintenance therapy or patient intends to get pregnant: immunization and splenectomy. Group B: failure of splenectomy (refractory) or no splenectomy indication or history of exposure to malaria or babesiosis and no response to corticoids or corticoid dependence: choose thrombopoietin receptor agonists: eltrombopag or romiplostim. Patient at high risk for arterial or venous thrombosis: recommend rituximab. After rituximab or thrombopoietin receptor agonists, if platelets continue <20 × 10 9 /L: indicate immunosuppressants (azathioprine or cyclophosphamide), dapsone or mycophenolate mofetil or vinca alkaloids. The goals of treatment for chronic or refractory immune thrombocytopenia are to keep platelets >20 × 10 9 /L and stop bleeding.

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“…However, the potential for surgical morbidity, including bleeding and arterial or venous thrombosis, must be taken into account when considering treatment options for patients with autoimmune cytopenias. 11 Moreover, splenectomy is an invasive and irreversible procedure and patients may express a preference to avoid it.…”

mentioning

confidence: 99%

Long‐term remission rates after splenectomy in adults with Evans syndrome compared to immune thrombocytopenia: A single‐center retrospective study

Sulpizio

Raghunathan

Shatzel

et al. 2019

European J of Haematology

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Objective Evans syndrome, the combination of immune thrombocytopenia (ITP) and autoimmune hemolytic anemia (AIHA) or autoimmune neutropenia, is associated with a high rate of relapsed/refractory disease. There are limited data on the efficacy of splenectomy for this condition. We reviewed patient outcomes after splenectomy for Evans syndrome compared to ITP at our institution. Methods We performed a retrospective analysis of patients who underwent splenectomy for autoimmune cytopenias over a 23‐year period with the intention of comparing disease relapse rates after splenectomy in patients with Evans syndrome and in those with ITP. Results During the study period, 77 patients underwent splenectomy for ITP and seven underwent splenectomy for Evans syndrome. In the Evans cohort, splenectomy led to an 85.7% initial response rate with a 42.8% rate of relapse within one year and a long‐term (one‐year) response rate of 42.8%. In the ITP cohort, the initial response rate was 90.9% with a long‐term response rate of 70.1%. Conclusion Our data suggest that long‐term remission rates after splenectomy are lower in adults with Evans syndrome compared to those with ITP, although splenectomy may still be an acceptable treatment for certain patients with Evans syndrome. Our findings underscore the need for further research and development of additional therapeutic strategies for this patient population.

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The role of splenectomy in autoimmune hematological disorders: Outdated or still worth considering?

Cited by 16 publications

References 141 publications

Treatment of autoimmune hemolytic anemia: real world data from a reference center in Mexico

Treatment of autoimmune hemolytic anemia: real world data from a reference center in Mexico

Proposal of treatment algorithm for immune thromocytopenia in adult patients of a hematology service at a referral center in Northeastern Brazil

Long‐term remission rates after splenectomy in adults with Evans syndrome compared to immune thrombocytopenia: A single‐center retrospective study

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