Infectious Complications in Autoimmune Hemolytic Anemia

Giannotta, Juri Alessandro; Fattizzo, Bruno; Cavallaro, Francesca; Barcellini, Wilma

doi:10.3390/jcm10010164

Cited by 23 publications

(25 citation statements)

References 150 publications

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“…Although infections under CyA are less frequently reported than under other immunosuppressants (i.e. cyclophosphamide, mycophenolate mofetil), 41 we observed two serious infectious complications (5%, one fatality in an elderly) both in patients concomitantly receiving steroids. Thus, particular attention is warranted for elderly, heavily pretreated patients, including anti-pneumocystis prophylaxis in patients receiving >25 mg/day prednisone or equivalent for >4 weeks.…”

Section: Discussionmentioning

confidence: 49%

Efficacy and safety of cyclosporine A treatment in autoimmune cytopenias: the experience of two Italian reference centers

Fattizzo

Cantoni

Giannotta

et al. 2022

Therapeutic Advances in Hematology

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Background: Immune thrombocytopenia (ITP) and autoimmune hemolytic anemia (AIHA) show good responses to frontline steroids. About two-third of cases relapse and require second-line treatment, including rituximab, mainly effective in AIHA, and thrombopoietin-receptor agonists (TPO-RAs) in ITP, while the use of splenectomy progressively decreased due to concerns for infectious/thrombotic complications. For those failing second line, immunosuppressants may be considered. Objectives: The aim of this study was to evaluate the efficacy of cyclosporine treatment in patients with ITP and AIHA. Design: In this retrospective study, we evaluated the efficacy and safety of cyclosporine A (CyA) in ITP ( N = 29) and AIHA ( N = 10) patients followed at two reference centers in Milan, Italy. Methods: Responses were classified as partial [Hb > 10 or at least 2 g/dl increase from baseline, platelets (PLT) > 30 × 109/l with at least doubling from baseline] and complete (Hb > 12 g/dl or PLT > 100 × 109/l) and evaluated at 3, 6, and 12 months. Treatment emergent adverse events were also registered. Results: The median time from diagnosis to CyA was 35 months (3–293), and patients had required a median of 4 (1–8) previous therapy lines. Median duration of CyA was 28 (2–140) months and responses were achieved in 86% of ITP and 50% of AIHA subjects. Responders could reduce or discontinue concomitant treatment and resolved PLT fluctuations on TPO-RA. CyA was generally well tolerated, and only two serious infectious complications in elderly patients on concomitant steroids suggesting caution in this patient population. Conclusion: CyA may be advisable in ITP, which is not well controlled under TPO-RA, and in AIHA failing rituximab, particularly if ineligible in clinical trial.

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Section: Discussionmentioning

confidence: 49%

Efficacy and safety of cyclosporine A treatment in autoimmune cytopenias: the experience of two Italian reference centers

Fattizzo

Cantoni

Giannotta

et al. 2022

Therapeutic Advances in Hematology

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“…It is well documented that patients often have infectious complications due to the immunosuppressive nature of their treatments; additionally, those that undergo splenectomy are further susceptible to serious infections. 12,13 Clinician's attention toward infectious complications through vigilant vaccinations and antimicrobial prophylaxis is crucial. Additionally, (as observed with our patient) those with AIHA often experience venous thromboembolic events.…”

Section: Discussionmentioning

confidence: 99%

“…Additionally, these patients suffer from multitudes of other problems stemming from their medications and physiologic states. It is well documented that patients often have infectious complications due to the immunosuppressive nature of their treatments; additionally, those that undergo splenectomy are further susceptible to serious infections 12,13 . Clinician's attention toward infectious complications through vigilant vaccinations and antimicrobial prophylaxis is crucial.…”

Section: Discussionmentioning

confidence: 99%

Refractory autoimmune hemolytic anemia in a systemic lupus erythematosus patient: A clinical case report

Crawford

Neparidze

2022

Clinical Case Reports

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Warm autoimmune hemolytic anemia (AIHA) is a hematologic disorder with an incidence of 1-3 per 10 5 individuals/year. Patients with systemic lupus erythematosus (SLE) develop AIHA in 3% of adult cases and 14% of pediatric cases. We report a case of AIHA refractory to multiple lines of treatment in a patient with SLE, who eventually responded to a proteasome inhibitor-based combination. A patient with systemic lupus erythematous was diagnosed with symptomatic autoimmune hemolytic anemia. The patient was refractory to multiple lines of treatment including prednisone, intravenous immune globulin, methylprednisolone, rituximab, cyclophosphamide, mycophenolate mofetil, and splenectomy. She eventually had a beneficial response to a proteasome inhibitor-based combination with bortezomib plus mycophenolate mofetil. The treatment of refractory autoimmune hemolytic anemia can be challenging. Patients with AIHA refractory to primary or secondary treatments must resort to receiving novel therapeutic modalities including combinations targeting plasma cell, T-and B-cell proliferation. K E Y W O R D S autoimmune hemolytic anemia, proteasome inhibitor, systemic lupus erythematosus How to cite this article: Crawford LR, Neparidze N. Refractory autoimmune hemolytic anemia in a systemic lupus erythematosus patient: A clinical case report.

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“…If tuberculosis is suspected, the infective diseases specialist may indicate specific prophylaxis. 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].…”

Section: Discussionmentioning

confidence: 99%

“…Finally, patient education is fundamental, along with prompt referral to medical attention/emergency room in case of severe infection. 16 …”

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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Infectious Complications in Autoimmune Hemolytic Anemia

Cited by 23 publications

References 150 publications

Efficacy and safety of cyclosporine A treatment in autoimmune cytopenias: the experience of two Italian reference centers

Efficacy and safety of cyclosporine A treatment in autoimmune cytopenias: the experience of two Italian reference centers

Refractory autoimmune hemolytic anemia in a systemic lupus erythematosus patient: A clinical case report

Evans syndrome in adults: an observational multicenter study

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