Autoimmune hemolytic anemia combined with idiopathic thrombocytopenia (Evans Syndrome)

1992 ◽  
Vol 70 (1) ◽  
pp. 38-39 ◽  
Author(s):  
P. E. Petrides ◽  
E. Hiller
Keyword(s):  
Hemolytic Anemia ◽  
Autoimmune Hemolytic Anemia ◽  
Evans Syndrome ◽  
Idiopathic Thrombocytopenia

A CASE OF EVANS SYNDROME WITH MIXED-TYPE AUTOIMMUNE HEMOLYTIC ANEMIA

2012 ◽  
Vol 58 (4) ◽  
pp. 539-546
Author(s):  
Takahiro Amamoto ◽  
Kouichi Egashira ◽  
Hiroyuki Kawano ◽  
Takanori Higashitani ◽  
Ken Ishimaru ◽  
...  
Keyword(s):  
Hemolytic Anemia ◽  
Autoimmune Hemolytic Anemia ◽  
Mixed Type ◽  
Evans Syndrome

scholarly journals Autoimmune Cytopenias and Covid-19 Vaccination: Relapse and Suggested Treatment

Blood ◽  
2021 ◽  
Vol 138 (Supplement 1) ◽  
pp. 4141-4141
Author(s):  
Gregorio Campos-Cabrera ◽  
Francisco-Gerardo Torres-Salgado ◽  
Salvador Campos-Cabrera ◽  
Jose-Luis Campos-Villagomez ◽  
Virginia Campos-Cabrera
Keyword(s):  
Complete Remission ◽  
Hemolytic Anemia ◽  
Autoimmune Hemolytic Anemia ◽  
De Novo ◽  
Conflicts Of Interest ◽  
High Dose ◽  
Evans Syndrome ◽  
Autoimmune Thrombocytopenia ◽  
Platelet Counts ◽  
Autoimmune Cytopenias

Abstract Introduction: There are "de novo" and relapsed autoimmune diseases in patients with COVID-19 that includes autoimmune thrombocytopenia, Evans syndrome and autoimmune hemolytic anemia among others (Hematology 2021;26:225-239 and Curr Rheumatol Rev 2021;17:193-204). There is scanty material about relapse of autoimmune hematological diseases after vaccination for COVID-19 (Blood Adv 2021;13:2794-2798). Material and methods: Adult patients 18 years or older with autoimmune thrombocytopenia, Evans syndrome and autoimmune hemolytic anemia who completed SARS-Cov2 vaccination. Results: Between December 2020 and June 2021 there were identified 53 patients with autoimmune hematological disease that completed SARS-Cov2 vaccination. Thirty-six with autoimmune thrombocytopenia, all were preexisting. Twelve with autoimmune hemolytic anemia, 5 secondaries to previous COVID-19 and 7 preexisting. Five with Evans syndrome, all preexisting. Twenty-three patients with autoimmune thrombocytopenia did not develop any fall in the platelet count. Ten patients had a fall of 50 % from basal counts and recovered spontaneously. Three patients developed counts below 30,000 with purpuric symptoms and needed treatment that consisted in two courses of dexamethasone 40 mg daily for four days every three weeks; all patients reached complete remission without any further treatment. All patients with Evans syndrome developed hemolysis and low platelet counts. Two patients maintained Hb levels above 10 and platelet counts above 50,000; both patients had spontaneously recovery. Three patients developed Hb levels below 7 with anemic syndrome and platelets below 50,000 but without purpuric syndrome; they received the same treatment as patients with autoimmune thrombocytopenia and reached complete remission too. All five patients with autoimmune hemolytic anemia secondary to COVID-19 developed Hb levels below 7 with anemic symptoms and needed treatment as described. The remaining 7 patients with preexisting autoimmune anemia developed hemolysis; five with Hb levels above 7 and recovery without any treatment; two had Hb levels below 7 and received the same treatment with full recovery and complete remission. Conclusions: Autoimmune cytopenias can be trigger by vaccines and viral infections by involving molecular mimicry and circulating immune complexes, including SARS-Cov2. The viral protein spike from SARS-Cov2 has mimicry between the Ankyrin-1 in the erythrocyte surface, and has been linked as one of the pathogenesis pathways of autoimmune hemolytic anemia secondary to COVID-19 (Br J Haematol 2020;190:e92-e93 and Blood 2020;136:suppl8,138001). Relapse of autoimmune cytopenias after vaccination with SARS-Cov2 involves stimulation of autoantibodies production from preexisting B cells. Although relapses were observed in the three kinds of patients, all with hemolytic component developed a drop in the hemoglobin levels, most of them needed treatment. It is important to notice that patients with hemolytic autoimmune anemia secondary COVID-19 had severe relapse, event that support the mimicry mentioned lines above. It is important to follow up closely this kind of patients after SARS-CoV2 vaccination, we suggest weekly complete blood counts, and a short courses of high dose dexamethasone can induce curable responses if treatment is advised. Disclosures No relevant conflicts of interest to declare.

Soluble CD30 (sCD30), a Marker of Th2 Polarization, Is Elevated in Patients with Autoimmune Hemolytic Anemia (AIHA) but Not with Idiopathic Thrombocytopenia (ITP).

Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 1248-1248
Author(s):  
Eugene R. Ahn ◽  
Eckhard Podack ◽  
Wenche Jy ◽  
Carlos J. Bidot ◽  
Lawrence Horstman ◽  
...  
Keyword(s):  
Red Blood Cells ◽  
Hemolytic Anemia ◽  
Lupus Erythematosus ◽  
Autoimmune Hemolytic Anemia ◽  
Blood Cells ◽  
Large Cell ◽  
Mhc I ◽  
Idiopathic Thrombocytopenia ◽  
Soluble Cd30 ◽  
Active Disease

Abstract Introduction. CD30, member of the TNF-receptor family, is expressed on activated T and B cells. Upon CD30 signaling and the downstream event of cytokine release of IL-4 and IL-13, CD30 is cleaved by a metalloprotease, resulting in soluble CD30 (sCD30). sCD30 has been shown to be a reliable proxy for Th2 activity and is elevated in Th2-polarized disorders such as atopic dermatitis, rheumatoid arthritis, and systemic lupus erythematosus (SLE) but is subnormal in patients with Th1-polarized disease such as X-linked agammaglobulinemia (Eur J Immunol2001; 31:1927–1934). Elevated sCD30 levels were also found in patients with Hodgkin’s lymphoma or anaplastic large cell lymphoma. We investigated sCD30 levels in patients with chronic idiopathic thrombocytopenia (ITP) and autoimmune hemolytic anemia (AIHA). Method. We measured plasma sCD30 levels in 35 patients with ITP, 12 with AIHA, 8 with SLE, 9 with lymphoma and 18 healthy controls. sCD30 was measured by ELISA (Bender Medsystems) in platelet poor plasma from citrated blood. Results. Compared to controls (19.0 ± 6.2 units/mL), sCD30 levels were significantly higher in AIHA (mean 145.6, p=0.03) but not in ITP (22.7, p=0.1). sCD30 levels were also elevated in SLE (mean 73.6, p=0.001) and lymphoma (mean 107.4, p=0.04), confirming previously published studies. In AIHA, sCD30 levels were not significantly different in patients with active/chronic disease versus those in clinical remission, although levels trended higher during the acute episodes of hemolysis. In ITP, sCD30 levels were also similar between active disease versus remission and splenectomized versus unsplenectomized patients. Conclusion. Our results support the hypothesis that AIHA and SLE are Th2-polarized disorders, whereas ITP is not. Lack of significant differences in sCD30 between active disease and remission in AIHA and ITP may suggest that imbalances of Th1/Th2 immunity persist regardless of clinical course, consistent with Panitsas et al (BMC Blood Disord2004; 4:4). Discussion. Prior studies have examined serum cytokine concentrations such as IFN-γ and IL-4 in patients with ITP and reached inconclusive results. But recent studies using more sensitive techniques such as RT-PCR indicate that ITP is a Th1-polarized disorder (Blood2004; 103:2645–2647). Our findings of elevated sCD30 levels in AIHA but not ITP lead to a provocative question: what is it about the immunological destruction of platelets that makes it associated with a markedly different Th1/Th2 milieu in comparison to the destruction of red blood cells? We propose that the key differences are 1) platelets are more potent in modulating immunity via mechanisms including CD40L (CD154) release upon platelet activation (Immunity2003; 19:9–19) and 2) platelets express MHC I molecules whereas red blood cells do not and hence they are processed by macrophages in a different manner (Transfusion2002; 24:958–961).

Predictors of Outcome and Response to Therapy in Primary Autoimmune Hemolytic Anemia: A Gimema Study of 307 Patients

Blood ◽  
2014 ◽  
Vol 124 (21) ◽  
pp. 1346-1346
Author(s):  
Barcellini Wilma ◽  
Bruno Fattizzo ◽  
Anna Zaninoni ◽  
Tommaso Radice ◽  
Ilaria Nichele ◽  
...  
Keyword(s):  
Renal Failure ◽  
Acute Renal Failure ◽  
Hemolytic Anemia ◽  
Relapse Rate ◽  
Autoimmune Hemolytic Anemia ◽  
Response To Therapy ◽  
Clinical Severity ◽  
Predictors Of Outcome ◽  
Evans Syndrome ◽  
Serological Type

Abstract Autoimmune hemolytic anemia (AIHA) is a greatly heterogeneous condition both in terms of clinical presentation and response to treatment, usually classified as warm (WAIHA), cold (CAD), mixed, and atypical forms. The aim of this study was to identify predictors of outcome and response to therapy considering in particular the serological characteristics and the severity of anemia at onset. We evaluatedretrospectively 307 patients (112 M and 195 F, median age at diagnosis 63, range 1-97), diagnosed between 1978 and 2013 and followed-up for a median of 33 months (range 12-372); 60% of cases were WAIHA, 27% CAD, 8% mixed, and 5% atypical (14 DAT- and 1 DAT+ for IgA only). Hemoglobin values were lower in mixed (median 5.8, range 2-10.7 g/dL) atypical (6.2, 3-9), and in IgG+C3 DAT+ WAIHA (6.9, 2.9-11.5). Twenty-one subjects were diagnosed with Evans’ syndrome, the majority of them WAIHA, with a severe onset. Considering anemia at onset, 27% of cases had Hb levels <6, 36% Hb 6.1-8, 24% Hb 8.1-10, and 13% Hb>10 g/dL; the most severe cases were mainly mixed and atypical forms (P=0.0001). Regarding therapy, 47% of cases were treated with one therapy line only, 26% with two, 13% with three, and 4% with four or more lines. Sixty % of WAIHA received first line steroid therapy only, 20 CAD required no treatment, and patients with IgG+C DAT+ WAIHA, mixed, and atypical forms were more frequently treated with 2 or more therapy lines (P<0.0001); the gender- and age-adjusted cumulative incidence of relapse was significantly increased in more severe cases by Fine and Gray model (Figure). Response to steroids was observed in ~75% of cases, with lower rates in CAD and generally observed at high steroid dosages. Splenectomy (32 cases, mostly WAIHA or severe forms) had a response rate of 75%, but was ineffective in 2/3 CAD; the relapse rate was 8/24 (33%) after a median of 41 months. Regarding immunosuppressants (31 cases azathioprine, 40 cyclophosphamide, and 12 cyclosporine) the OR was 50-70% (PR 20-40), irrespective of serological type and severity of anemia, although the simultaneous administration of steroid in most cases may weaken these results; the relapse rate was 8/60 (13%) after a median of 11 months. Rituximab (55 cases at conventional, and in 19 at low doses (LD) of 100 mg /weekly x 4) had an 80% OR (35% PR). Predictors of response to LD were WAIHA, younger age, and shorter interval between diagnosis and rituximab therapy; at variance, OR to conventional doses occurred irrespectively of age, serological type, clinical severity at onset, and disease duration. The relapse rate was 5% (2/42, of whom 1 CAD) for standard and 38% (6/16, of whom 5 CAD) for LD, and relapses occurred mostly within the first year after treatment. As regards complications, infections occurred in 26 cases (10 grade 3, 11 grade 4, and 5 grade 5), irrespective of serological AIHA type and severity at onset, and of the number of therapy lines; on the contrary, they were observed more frequently in splenectomized cases. Acute renal failure was recorded in 6 cases and was not associated with AIHA clinical or serological characteristics. A thrombotic event was recorded in 11% and was associated with severe onset, higher median LDH levels, and previous splenectomy. At the time of the analysis 63 cases (21%) have died, of whom 11 because of AIHA (3.6%); death was not associated with the severity of anemia at onset, nor with the serological type of AIHA; at variance, it was associated with infections (HR 11.47, 95% CI 3.43-38.4, p=0.0004), acute renal failure (HR 17.99, 95% CI 4.73-68.40, p=0.001), Evans’ syndrome (HR 6.8, 95% CI 1.99-23.63, P=0.0074), previous splenectomy (HR 3.21, 95% CI 0.92-11.25), and multi-treatment (4 or more lines of therapy; HR 9.1, 95% CI 2.41-34.36, p=0.0076). Death was not associated with thrombotic events, nor with the type of treatment, in particular immunosuppressants or rituximab. In conclusion, we showed that AIHA cases with a severe onset, mostly mixed and atypical forms, are frequently refractory to different therapies. Although obtained retrospectively, our results suggest to put forward rituximab among second line options, given its efficacy and safety. In addition, standard rituximab doses should be preferred in CAD, whereas lower doses may be equally effective in WAIHA and mixed forms. Finally, we suggest to defer splenectomy after rituximab, given the increased risk of thromboembolism, infections and fatal outcome in splenectomized patients. Figure 1 Figure 1. Disclosures No relevant conflicts of interest to declare.

miR-150 regulates B lymphocyte in autoimmune hemolytic anemia/Evans syndrome by c-Myb

2018 ◽  
Vol 107 (6) ◽  
pp. 666-672 ◽  
Author(s):  
Limin Xing ◽  
Wenyan Xu ◽  
Yingying Qu ◽  
Manjun Zhao ◽  
Hongli Zhu ◽  
...  
Keyword(s):  
Hemolytic Anemia ◽  
Autoimmune Hemolytic Anemia ◽  
B Lymphocyte ◽  
Evans Syndrome

High-dose intravenous intact IgG infusion in refractory autoimmune hemolytic anemia (Evans syndrome)

1985 ◽  
Vol 107 (5) ◽  
pp. 744-746 ◽  
Author(s):  
Hideaki Oda ◽  
Akihito Honda ◽  
Katsuo Sugita ◽  
Akira Nakamura ◽  
Hironori Nakajima
Keyword(s):  
Hemolytic Anemia ◽  
Autoimmune Hemolytic Anemia ◽  
High Dose ◽  
Evans Syndrome

scholarly journals Scrotal Pyoderma Gangrenosum Associated with Evans Syndrome

2018 ◽  
Vol 7 (9) ◽  
pp. 230
Author(s):  
Deng-Ho Yang ◽  
Meng-Yin Yang
Keyword(s):  
Emergency Room ◽  
Hemolytic Anemia ◽  
Pyoderma Gangrenosum ◽  
Autoimmune Hemolytic Anemia ◽  
Severe Pain ◽  
Immune Thrombocytopenia ◽  
Steroid Treatment ◽  
Evans Syndrome ◽  
History Of ◽  
Cutaneous Ulcer

Evans syndrome is a rare disorder with presentations of autoimmune hemolytic anemia and immune thrombocytopenia, in the absence of any underlying cause. Here, we reported a case with a history of Evans syndrome for seven years. A persistent scrotal ulcer with severe pain occurred for two weeks. He called at our emergency room because of a painful, necrolytic cutaneous ulcer over the scrotal region. A biopsy showed sterile dermal neutrophilia with lymphocytic vasculitis, and pyoderma gangrenosum was impressed. The patient received steroid treatment and recovery after one month.

scholarly journals Most CD5+ B Lymphocytes Secrete IL-10 in Autoimmune Hemolytic Anemia/Evans Syndrome Patients

Blood ◽  
2018 ◽  
Vol 132 (Supplement 1) ◽  
pp. 4887-4887
Author(s):  
Limin Xing ◽  
Manjun Zhao ◽  
Hongli Zhu ◽  
Zonghong Shao
Keyword(s):  
B Cells ◽  
Serum Level ◽  
B Lymphocytes ◽  
Hemolytic Anemia ◽  
Autoimmune Hemolytic Anemia ◽  
Cell Activation ◽  
Conflicts Of Interest ◽  
Enzyme Linked Immunosorbent Assay ◽  
Newly Diagnosed ◽  
Evans Syndrome

Abstract Objective: Autoimmune hemolytic anemia (AIHA)/Evans syndrome is a form of immune-related cytopenia characterized by autoantibodies directed against red blood cells. A subset of B lymphocytes expressing CD5 is found in patients with AIHA/Evans syndrome. Previous studies have shown that the number of CD5+ B lymphocytes is correlated with the severity of AIHA/Evans syndrome. IL-10 can be induced in various types of human B cells, including naive, memory, and CD5+B cells, following B-cell activation by Th-cell-dependent and TLR-dependent signals. This study is to investigate the secretion function of CD5+ B lymphocytes in AIHA/Evans syndrome (ES) patients. Methods: 25 untreated AIHA/ES patients, 28 remission AIHA/ES patients and 25 healthy controls (HCs) were enrolled in this study. The quantity of CD5+B lymphocytes which produce interleukin-10 (IL-10) (CD5+IL-10+) were detected by flow cytometry. CD5+ B lymphocytes were sorted from Peripheral blood (PB) by CytoFLEX Flow Cytometer. The expression of IL-10 mRNA in CD5+ B cells were measured by real-time PCR (RT-PCR). The concentrations of IL-10 in serum were detected by Enzyme-linked Immunosorbent assay (ELISA). Results: The percentage of CD5+IL-10+B cells in CD5+ B lymphocytes in newly diagnosed patients was 82.18±14.78%, which being significantly higher than that of remission AIHA/ES patients (56.68±24.39%) and HC (51.90±22.95%)(p<0.05). The percentage of CD5+IL-10+ B cells in CD5+ B lymphocytes in newly diagnosed patients was negatively correlated with their serum level of hemoglobin, C3 (P<0.05) and positively correlated with their serum level of LDH, TBIL and IBIL (P<0.05). The expression level of CD5+ B lymphocytes IL-10 mRNA in newly diagnosed patients (49.34±22.84) was higher than that of remission patients (3.97±3.83) and HC (1.78±1.66) (P<0.05). The concentration of IL-10 in serum in newly diagnosed patients (4.01±1.54 pg/ml) was lower than that of remission patients (5.08±1.72pg/ml) and HC (5.70±1.60pg/ml) (P<0.05). Conclusions: Most CD5+ B lymphocytes secrete IL-10 in AIHA/Evans patients and are positively correlated with the severity of the disease. The increased quantity of CD5+IL-10+ B cells and the decreased cytokines-secreted level maybe involved in the occurrence of AIHA/Evans syndrome. Disclosures No relevant conflicts of interest to declare.

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