Splenectomy and the incidence of venous thromboembolism and sepsis in patients with autoimmune hemolytic anemia

Ho, Gwendolyn; Brunson, Ann; Keegan, Theresa H.M.; Wun, Ted

doi:10.1016/j.bcmd.2019.102388

Cited by 22 publications

(24 citation statements)

References 36 publications

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“…Splenectomy is as effective as rituximab, with somewhat longerlasting remissions (Table 1). 8,9,16,[20][21][22] Use of splenectomy has gradually declined, however, to ,10% of cases, mainly due to the increased infection risk, particularly in the first year. This risk is not eliminated by vaccination and antibiotic prophylaxis.…”

Section: Commentary On Patientmentioning

confidence: 99%

How I treat warm autoimmune hemolytic anemia

Barcellini

Fattizzo

2021

Blood

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Warm autoimmune hemolytic anemia (wAIHA) is caused by increased erythrocyte destruction by IgG autoantibodies, with or without complement activation. Antibody-dependent cell-mediated cytotoxicity by macrophages/activated lymphocytes occurs in the lymphoid organs and spleen (extravascular hemolysis). The ability of the bone marrow to compensate determines clinical severity. The different pathogenic mechanisms, their complex interplay, and changes over time may explain wAIHA's great clinical heterogeneity and unpredictable course. The disease may be primary, drug-induced, or associated with lymphoproliferative neoplasms, autoimmune and infectious diseases, immunodeficiencies, solid tumors, or transplants. Therapeutic interventions include steroids, splenectomy, immunosuppressants, and rituximab; the latter is increasingly used in steroid refractory cases based on evidence from the literature and a few prospective trials. We present five patient case studies highlighting important issues: 1) the diagnosis and proper use of steroid therapy; 2) the concerns about the choice between rituximab and splenectomy in second-line treatment; 3) the need of periodical re-evaluation of the disease to assess the possible evolution of relapsed/refractory cases in myelodysplastic and bone marrow failure syndromes; and 4) the difficulties in managing cases of severe/acute disease which are at high risk of relapse. Incorporating novel targeted therapies into clinical practice will be an exciting challenge in the future.

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Section: Commentary On Patientmentioning

confidence: 99%

How I treat warm autoimmune hemolytic anemia

Barcellini

Fattizzo

2021

Blood

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“…Thrombosis was associated with severe hyperhemolytic disease (i.e. Hb<6 g/dL and LDH>1.5 xULN), but not to previous splenectomy as described for primary AIHA [11,16,20]. Thrombotic risk in ES appears also higher than that observed in isolated ITP, where it is only slightly augmented as compared to the general population but increases by three folds if TPO-RA are given [21].…”

Section: Discussionmentioning

confidence: 77%

“…Finally, patient education is fundamental, along with prompt referral to medical attention/emergency room, in case of severe infections [17]. Thrombotic complications, observed in 20% of cases, were fairly more frequent than in AIHA alone, where have been reported in about 10-15% of cases [11,16,[18][19][20]. Thrombosis was associated with severe hyperhemolytic disease (i.e.…”

Section: Discussionmentioning

confidence: 99%

Evans syndrome in adults: an observational multicenter study

Fattizzo

Michel²,

Giannotta

et al. 2021

Blood Advances

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Evans syndrome (ES) is a rare condition, defined as the presence of two autoimmune cytopenias, more frequently autoimmune hemolytic anemia and immune thrombocytopenia, and rarely autoimmune neutropenia. ES can be classified as primary or secondary to various conditions, including lymphoproliferative disorders, other systemic autoimmune diseases, and primary immunodeficiencies, particularly in children. In adult ES, little is known about clinical features, disease associations and outcome. In this retrospective international study, we analyzed 116 adult patients followed at 13 European tertiary centers, focusing on treatment requirement, occurrence of complications and death. ES was secondary to or associated with an underlying condition in 24 cases (21%), mainly other autoimmune diseases and hematologic neoplasms. Bleeding occurred in 42% of subjects, mainly low grade and at ITP onset. Almost all patients received first line treatment (steroids+/-IVIG), and 23% needed early additional therapy for primary refractoriness. Further therapy lines included rituximab, splenectomy, immunosuppressants, thrombopoietin receptor agonists, and others, with response rates greater than 80%. However, a remarkable number of relapses occurred, requiring ≥3 therapy lines in 54% of cases. Infections and thrombotic complications occurred in 33% and 21% of subjects, respectively, mainly grade ≥3, and correlated with the number of therapy lines. Besides age, other factors negatively impacting on survival were severe anemia at onset and occurrence of relapse, infections and thrombosis. These data show that adult ES is often severe and marked by a relapsing clinical course and potentially fatal complications, pinpointing the need for high clinical awareness, prompt therapy, and anti-infectious/anti-thrombotic prophylaxis.

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“…However, it has been moved to further than third‐line therapy in current guidelines for adult primary wAIHA, due to the introduction of new effective treatments and the increased awareness of complications, namely infections and thrombosis 9,38 . A recent large study on 4 766 adults, affected mainly by primary AIHA, reported an increased risk of venous thrombosis, abdominal thrombosis and sepsis for those who received a splenectomy 44 . In post‐HSCT AIHA, response rates to splenectomy are lower: in the largest series, only one out of seven cases responded 16 .…”

Section: Treatment Of Aiha Post Hsctmentioning

confidence: 99%

Pathogenesis, risk factors and therapeutic options for autoimmune haemolytic anaemia in the post‐transplant setting

et al. 2021

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Summary Autoimmune haemolytic anaemia (AIHA) is a rare complication of allogeneic haematopoietic stem cell transplantation (HSCT), observed with an incidence of 1–5%. Paediatric age, diagnosis of non‐malignant disease, lympho‐depleting agents in the conditioning regimen, use of unrelated donor, graft versus host disease and infections have been associated with a higher risk of AIHA post HSCT. Post‐HSCT AIHA is associated with high mortality and morbidity, and it is often very difficult to treat. Steroids and rituximab are used with a response rate around 30–50%. These and other therapeutic strategies are mainly derived from data on primary AIHA, although response rates in post‐HSCT AIHA have been generally lower. Here we review the currently available data on risk factors and therapeutic options. There is a need for prospective studies in post‐HSCT AIHA to guide clinicians in managing these complex patients.

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Splenectomy and the incidence of venous thromboembolism and sepsis in patients with autoimmune hemolytic anemia

Cited by 22 publications

References 36 publications

How I treat warm autoimmune hemolytic anemia

How I treat warm autoimmune hemolytic anemia

Evans syndrome in adults: an observational multicenter study

Pathogenesis, risk factors and therapeutic options for autoimmune haemolytic anaemia in the post‐transplant setting

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