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 A case of pregnancy complicated with Evance syndrome and heparin-induced thrombocytopenia

2021 ◽  
pp. 91-99
Author(s):  
O.M. Naumchik ◽  
Keyword(s):  
Hemolytic Anemia ◽  
Autoimmune Hemolytic Anemia ◽  
Immune Thrombocytopenia ◽  
Underlying Disease ◽  
Evans Syndrome ◽  
Thrombotic Risk ◽  
Heparin Induced Thrombocytopenia ◽  
First Line Therapy ◽  
Successful Termination ◽  
Neonatal Thrombocytopenia

The simultaneous development of autoimmune hemolytic anemia and immune thrombocytopenia is known as Evans syndrome. This pathology is extremely rare during pregnancy, requires careful differential diagnosis and is associated with a high risk of hemorrhagic and thromboembolic complications. Treatment approaches for autoimmune hemolytic anemia and immune thrombocytopenia are similar, but in the case of autoimmune hemolytic anemia, thromboprophylaxis is mandatory. Heparin-induced thrombocytopenia is a serious adverse event of anticoagulation. One presents with a decrease in platelets but a paradoxical increase in thrombotic risk and mandates switching of the anticoagulant agent to a non-heparin one. Clinical case. We describe the case of pregnancy complicated with Evans syndrome and development of heparin-induced thrombocytopenia at 28 weeks. Treatment of the underlying disease was effective on the use of first-line therapy. Fondaparinux and vitamin K antagonist (warfarin) have been used to treat heparin-induced thrombocytopenia. Monitoring of the fetal condition is carried out with careful control of Doppler ultrasound of the middle cerebral artery. Successful change of anticoagulant, close surveillance and carefully selected therapy allowed to achieve a successful termination of pregnancy. A healthy female newborn with body weight 2450 g, 8–8 Apgar scores was vaginally delivered uneventful at the 38th week. There were neither neonatal thrombocytopenia nor anemia. During the year after birth, the woman's condition is not worse, the child develops according to age. An analysis of similar cases of pregnancy from the literature is described. The research was carried out in accordance with the principles of the Helsinki Declaration. The informed consent of the patient was obtained for conducting the studies. No conflict of interest was declared by the author. Key words: thrombocytopenia, hemolytic anemia, pregnancy, Evans syndrome, heparin-induced thrombocytopenia.

scholarly journals NUDT15 polymorphism explains serious toxicity to azathioprine in Indian patients with chronic immune thrombocytopenia and autoimmune hemolytic anemia: a case series

2020 ◽  
Vol 0 (0) ◽  
Author(s):  
Anup J. Devasia ◽  
Raveen Stephen Stallon Illangeswaran ◽  
Infencia Xavier Raj ◽  
Biju George ◽  
Poonkuzhali Balasubramanian
Keyword(s):  
Hemolytic Anemia ◽  
Autoimmune Hemolytic Anemia ◽  
Immune Thrombocytopenia ◽  
Case Series ◽  
Severe Toxicity ◽  
Toxic Side Effect ◽  
Case Presentation ◽  
Oral Ulcers ◽  
Asian Patients ◽  
Life Threatening

AbstractObjectivesAzathioprine (AZA) is a commonly used immunosuppressant in patients with autoimmune diseases. The toxic side effect to AZA (myelosuppression, hair loss, and oral ulcers) are highly unpredictable which can be life threatening if not identified earlier and dose adjustments made or the drug is withdrawn.Case presentationHere we report a case series of five patients with severe toxicity while on treatment with AZA for autoimmune hemolytic anemia (n=1) and Immune thrombocytopenia (n=4). The common thiopurine methyltransferase (TPMT) variants (TPMT*2, *3A, *3B) were not present in these patients. However, all these patients had the NUDT15 415C>T variant that has been reported to explain serious toxicity to thioguanine in Asian patients.ConclusionsOur report suggests pre-emptive genotype-based dosing of AZA could reduce adverse toxicity and hence better outcome.

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 A Case of Warm Autoimmune Hemolytic Anemia and a Pulmonary Embolus in a Patient Treated with Triple Therapy

2019 ◽  
Vol 2019 ◽  
pp. 1-3
Author(s):  
Gurchetan Randhawa ◽  
Chia-Yu Chiu ◽  
Thanunthorn Suban Na Ayutthaya
Keyword(s):  
Medical History ◽  
Triple Therapy ◽  
Hemolytic Anemia ◽  
Autoimmune Hemolytic Anemia ◽  
Pulmonary Embolus ◽  
Hodgkin's Lymphoma ◽  
Pulmonary Emboli ◽  
Past Medical History ◽  
Non Hodgkin's Lymphoma ◽  
History Of

Autoimmune hemolytic anemia (AIHA) can be caused by a variety of etiologies. AIHA is associated with the development of coagulopathy, leading to potentially fatal pulmonary emboli. Here, we present a case of a 66-year-old female with a past medical history of non-Hodgkin’s lymphoma and gastritis treated with triple therapy that developed warm AIHA. The patient later succumbed to a suspected pulmonary embolus.

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.

scholarly journals Difficulties of anemia diagnosis in patients with B-cell chronic lymphocytic leukemia

2021 ◽  
Vol 16 (2) ◽  
pp. 40-47
Author(s):  
I. S. Piskunova ◽  
T. N. Moiseeva ◽  
L. S. Al-Radi ◽  
L. V. Plastinina ◽  
S. R. Goryacheva
Keyword(s):  
Chronic Lymphocytic Leukemia ◽  
B Cell ◽  
Hemolytic Anemia ◽  
Autoimmune Hemolytic Anemia ◽  
Immune Thrombocytopenia ◽  
Lymphocytic Leukemia ◽  
Red Cell ◽  
Red Cell Aplasia ◽  
Cell Chronic Lymphocytic Leukemia

Cytopenia commonly occurs in case of chronic lymphocytic leukemia. It can either precede the diagnosis of chronic lymphocytic leukemia or appear at any time during the disease. Autoimmune hemolytic anemia, immune thrombocytopenia, and partial red cell aplasia are most often found among cytopenias in chronic lymphocytic leukemia. At the same time, the development of cytopenia may be associated with the displacement of normal hematopoiesis cells by tumor lymphocytes. It is very important to accurately diagnose and identify the cause of cytopenia in chronic lymphocytic leukemia, since the prognosis and therapy differ significantly.

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
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