Refractory autoimmune hemolytic anemia in a patient with systemic lupus erythematosus and ulcerative colitis:A case report

BACKGROUND Ulcerative colitis(UC)and systemic lupus erythematosus(SLE)are both systemic immunoreactive diseases,and their pathogenesis depends on the interaction between genes and environmental factors.There are no reports of UC with SLE in China,but six cases of SLE with UC have been reported in China.The combination of these two diseases has distinct effects on the pathogenesis of both diseases.CASE SUMMARY A female patient(30 years old)came to our hospital due to dull umbilical pain,diarrhea and mucous bloody stool in August 2018 and was diagnosed with UC.The symptoms were relieved after oral administration of mesalazine(1 g po tid)or folic acid(5 mg po qd),and the patient were fed a control diet.On June 24,2019,the patient was admitted for treatment due to anemia and tinnitus.During hospitalization,the patient had repeated low-grade fever and a progressively decreased Hb level.Blood tests revealed positive antinuclear antibody test,positive anti-dsDNA antibody,0.24 g/L C3(0.9-1.8 g/L),0.04 g/L C4(0.1-0.4 g/L),32.37 g/L immunoglobulin(8-17 g/L),and 31568.1 mg/24 h total 24-h urine protein(0-150 mg/24 h).The patient was diagnosed with SLE involving the joints,kidneys and blood system.Previously reported cases of SLE were retrieved from PubMed to characterize clinicopathological features and identify prognostic factors for SLE.CONCLUSION The patient was discharged in remission after a series of treatments,such as intravenous methylprednisolone sodium succinate,intravenous human immunoglobulin,cyclophosphamide injection,and plasma exchange.After discharge,the patient took oral prednisone acetate tablets,cyclosporine capsules,hydroxychloroquine sulfate tablets and other treatments for symptoms and was followed up regularly for 1 month,after which the patient's condition continued to improve and stabilize.

First case of IgG4-related sclerosing cholangitis associated with autoimmune hemolytic anemia

To our knowledge,patients with immunoglobulin G4-related sclerosing cholangitis(IgG4-SC)associated with autoimmune hemolytic anemia(AIHA)have not been reported previously.Many patients with IgG4-SC have autoimmune pancreatitis(AIP)and respond to steroid treatment.However,isolated cases of IgG4-SC are difficult to diagnose.We describe our experience with a patient who had IgG4-SC without AIP in whom the presence of AIHA led to diagnosis.The patient was a73-year-old man who was being treated for dementia.Liver dysfunction was diagnosed on blood tests at another hospital.Imaging studies suggested the presence of carcinoma of the hepatic hilus and primary sclerosing cholangitis,but a rapidly progressing anemia developed simultaneously.After the diagnosis of AIHA,steroid treatment was begun,and the biliary stricture improved.IgG4-SC without AIP was thus diagnosed.

Iguratimod in treatment of primary Sjögren’s syndrome concomitant with autoimmune hemolytic anemia:A case report

BACKGROUND Primary Sjögren's syndrome(pSS)concomitant with autoimmune hemolytic anemia(AIHA)but without eye and mouth dryness is exceedingly rare.Iguratimod(IGU)has been widely used in the treatment of pSS.However,there are few reports about the application of IGU in pSS concomitant with AIHA.CASE SUMMARY Here,we present the case of a patient with pSS concomitant with AIHA but without eye and mouth dryness.The patient was initially diagnosed with hyperplastic anemia and AIHA while pSS was missed,and was finally diagnosed with pSS concomitant with AIHA.The patient was treated with IGU along with prednisone and hydroxychloroquine,and her hemoglobin,reticulocytes and IgG returned to normal levels.CONCLUSION IGU was effective for and well tolerated by our patient with pSS concomitant with AIHA,and may be a promising therapy for the treatment of this disease.

Oxidative stress as a potential causal factor for autoimmune hemolytic anemia and systemic lupus erythematosus

The kidneys and the blood system mutually exert influence in maintaining homeostasis in the body. Because the kidneys control erythropoiesis by producing erythropoietin and by supporting hematopoiesis, anemia is associated with kidney diseases. Anemia is the most prevalent genetic disorder, and it is caused by a deficiency of glucose 6-phosphate dehydrogenase(G6PD), for which sulfhydryl oxidation due to an insufficient supply of NADPH is a likely direct cause. Elevated reactive oxygen species(ROS) result in the sulfhydryl oxidation and hence are another potential cause for anemia. ROS are elevated in red blood cells(RBCs) under superoxide dismutase(SOD1) deficiency in C57BL/6 mice. SOD1 deficient mice exhibit characteristics similar to autoimmune hemolytic anemia(AIHA) and systemic lupus erythematosus(SLE) at the gerontic stage. An examination of AIHA-prone New Zealand Black(NZB) mice, which have normal SOD1 and G6 PD genes, indicated that ROS levels in RBCs are originally high and further elevated during aging. Transgenic overexpression of human SOD1 in erythroid cells effectively suppresses ROS elevation and ameliorates AIHA symptoms such as elevated anti-RBC antibodies and premature death in NZB mice. These results support the hypothesis that names oxidative stress as a risk factor for AIHA and other autoimmune diseases such as SLE. Herein we discuss the association between oxidative stress and SLE pathogenesis based mainly on the genetic and phenotypic characteristics of NZB and New Zealand white mice and provide insight into the mechanism of SLE pathogenesis.

Giant cell hepatitis with autoimmune hemolytic anemia in a nine month old infant

Giant cell hepatitis(GCH)with autoimmune hemolytic anemia is a rare entity,limited to young children,with an unknown pathogenesis.We report the case of 9-mo old who presented with fever,diarrhea and jaundice four days before hospitalization.Physical examination found pallor,jaundice and hepatosplenomegaly.The laboratory workup showed serum total bilirubin at 101 μmol/L,conjugated bilirubin at 84 μmol/L,hemolytic anemia,thrombocytopenia and immunoglobulin G(IgG)and anti-C3d positive direct Coombs' test.The antinuclear,anti-smooth muscle and liver kidney microsomes 1 non-organ specific autoantibodies,antiendomisium antibodies were negative.Serological assays for viral hepatitis B and C,cytomegalovirus,herpes simplex and Epstein Barr virus were negative.The association of acute liver failure,Evan's syndrome,positive direct Coomb's test of mixed type(IgG and C3)and the absence of organ and non-organ specific autoantibodies suggested the diagnosis of GCH.The diagnosis was confirmed by a needle liver biopsy.The patient was treated by corticosteroids,immunomodulatory therapy and azathioprine but died with septicemia.

Direct antiglobulin test-negative autoimmune hemolytic anemia in a patient withβ-thalassemia minor during pregnancy:A case report

BACKGROUND Severe refractory anemia during pregnancy can cause serious maternal and fetal complications.If the cause cannot be identified in time and accurately,blind symptomatic support treatment may cause serious economic burden.Thalassemia minor pregnancy is commonly considered uneventful,and the condition of anemia rarely progresses during pregnancy.Autoimmune hemolytic anemia(AIHA)is rare during pregnancy with no exact incidence available.CASE SUMMARY We report the case of a 30-year-oldβ-thalassemia minor multiparous patient experiencing severe refractory anemia throughout pregnancy.We monitored the patient closely,carried out a full differential diagnosis,made a diagnosis of direct antiglobulin test-negative AIHA,and treated her with prednisone and intravenous immunoglobulin.The patient gave birth to a healthy full-term baby.CONCLUSION Coombs-negative AIHA should be suspected in cases of severe hemolytic anemia in pregnant patients with and without other hematological diseases.

Infantile giant cell hepatitis with autoimmune hemolytic anemia

Giant cell hepatitis(GCH)is characterized by large and multinucleated(syncytial)hepatocytes in the context of liver inflammation.Infantile GCH is typically associated with autoimmune hemolytic anemia in the absence of any other systemic or organ-specific autoimmune comorbidity.The etiology is unknown;concomitant viral infections(as potential trigger factors)have been identified in a few patients.The pathogenesis reportedly relies upon immune-mediated/autoimmune mechanisms.This condition should be considered in any infant developing Coombs-positive anemia;indeed,anemia usually precedes the development of hepatitis.The clinical course is usually aggressive without the appropriate immunosuppressive therapy,which may include steroids,conventional immunosuppressors(e.g.,azathioprine and cyclophosphamide as first-line treatments),intravenous immunoglobulin,and biologics(rituximab).Improvements in medical management(including the availability of rituximab)have significantly reduced the mortality of this condition in the last decade.

Acute kidney injury following autoimmune hemolytic anemia due to simultaneous use of ciprofloxacin and hydrochlorothiazide:A case report and review of the literature

Introduction:Rare cases of autoimmune hemolytic anemia(AHA)associated with using ciprofloxacin or hydrochlorothiazide(HCTZ)alone have been reported before.However,simultaneous use of both drugs could lead to a severe clinical condition.This study presents the combination of acute kidney injury(AKI)and AHA following the simultaneous use of ciprofloxacin and HCTZ for three days.Case Description:A 42-year-old Iranian woman presented to the emergency department with symptoms of fatigue,lethargy,nausea and vomiting,ataxia,oliguria,dark urine,and jaundice.The patient reported using HCTZ due to high blood pressure and ciprofloxacin for a urinary tract infection three days before presentation.Early laboratory findings revealed hemolytic anemia with a hemoglobin of 7 g/dl,the strongly positive direct and indirect Coombs test,high level of lactate dehydrogenase(820 IU/L),and hyperbilirubinemia(total:3 mg/dL and direct:1.2 mg/dL).Furthermore,hyperkalemia(5.2 mEq/L),hyperphosphatemia(6.2 mg/dL),high levels of BUN(100 mg/dL),and creatinine rise(6.8 mg/dL)were found.Urine analysis showed 2+blood,4-6 red blood cells,and cola-colored urine.Based on the findings,druginduced AHA,followed by AKI,was diagnosed.Following,the drugs were stopped and steroid therapy was initiated.The patient underwent four sessions of hemodialysis to improve the AKI.Conclusion:Healthcare providers should be aware of the life-threatening adverse effects of commonly used drugs such as ciprofloxacin or HCTZ.The timely diagnosis of the offending drugs leads to avoiding the persistence of the risk factor and the deterioration of the patient's clinical condition.

Autoimmune Diseases and Acquired Von Willebrand Disease in Two Cases of Progeria

Ammar A., a 23-year-old male patient, who lives in Babylon, Haswa District, and his mother describes symptoms of growth retardation, skin changes, hair changes early graying and alopecia. These manifestation started early during his childhood period. There is canseguanity between the patient’s mother & father also one of the patient’s sister has similar illness and one male brother died few months following his birth. We admit the patient to hospital due acute pulmonary infection in Jan 2009, which is controlled after a course of antibiotic and after 5 months he develops generalised mucocuteneous bullous eruption which shows partial response to oral prednisolone 2 mg/Kg. The patient has normal IQ and he is in the secondary school and he has normal blood picture and the only abnormal biochemical abnormalities is mild hyperlipidemia Serum cholestrol of 5.8 mmol/L and Serum Triglyceride of 260 mg/dl. Ammar’s Sister Qawthar A., who has a similar phenotypic manifestations, presented skin vitiligo and hepatosplenomegaly associated with sever anemia and jaundice and her presentation suggestive of autoimmune haemolytic anemia improved following blood transfusion, corticosteroid and azothioprim. In February 2014 Ammer presented with multiple and diffuse cuteneous ecchymymosis with markedly prolonged PTT and slightly proloned bleeding time highly consistent with acquired Von Willebrand’s disease. In conclusion premature aging is a predisposing factor for disturbed immunity and development of autoimmune diseases.

Clinical and serological characterization of autoimmune hemolytic anemia after allogeneic hematopoietic stem cell transplantation

Background Autoimmune hemolytic anemia （AIHA） is an uncommon complication of allogeneic hematopoietic stem cell transplantation （allo-HSCT） which has only been reported in a few cases.We here aimed to explore its mechanism.Methods We retrospectively analyzed 296 patients who underwent allo-HSCT in our center from July 2010 to July 2012.Clinical manifestations were carefully reviewed and the response to currently available treatment approaches were evaluated.The survival and risk factors of AIHA patients after allo-HSCT were further analyzed.Results Twelve patients were diagnosed with AIHA at a median time of 100 days （15-720 days） after allo-HSCT.The incidence of AIHA after allo-HSCT was 4.1％.IgG antibody were detected in ten patients and IgM antibody in two patients.The two cold antibody AIHA patients had a better response to steroid corticoid only treatment and the ten warm antibody AIHA patients responded to corticosteroid treatment and adjustment of immunosuppressant therapy.Rituximab was shown to be effective for AIHA patients who failed conventional therapy.Survival analysis showed that the combination of AIHA in allo-HSCT patients hinted at poor survival.Cytomegalovirus （CMV） infection,graft-versus-host disease （GVHD） and histocompatibility leukocyte antigen （HLA） mismatch seemed to increase the risk of developing AIHA.Conclusions Patients who develop AIHA after allo-HSCT have poor survival compared to non-AIHA patients.Possible risk factors of AIHA are CMV infection,GVHD,and HLA mismatch.Rituximab is likely to be the effective treatment choice for the refractory patients.

洗涤红细胞和去白悬浮红细胞输注治疗自身免疫性溶血性贫血的效果

目的:探讨洗涤红细胞和去白悬浮红细胞输注治疗自身免疫性溶血性贫血(AIHA)的效果。方法:回顾性选取2020年3月—2023年3月蚌埠医学院第一附属医院收治的64例AIHA患者作为研究对象。根据治疗方法的不同将其分为试验组和对照组,各32例。对照组输注去白悬浮红细胞,试验组输注洗涤红细胞。比较两组输血后24 h临床疗效,输血前、输血后24 h相关指标,输血反应。结果:试验组总有效率高于对照组,差异有统计学意义(P<0.05)。输血后24 h,两组血红蛋白(Hb)、红细胞计数(RBC)升高,网织红细胞百分比(Ret)、总胆红素(TBIL)水平降低,试验组Hb、RBC高于对照组,差异有统计学意义(P<0.05)。两组输血反应发生率比较,差异无统计学意义(χ^(2)=1.016,P>0.05)。结论:对AIHA患者而言,在输血治疗中输注洗涤红细胞的总体有效率高于输注去白悬浮红细胞,并且Hb和RBC的提升效果更加明显。

自身免疫性溶血性贫血患者临床护理研究进展

自身免疫性溶血性贫血(AIHA)是临床上较难治疗的贫血疾病。本文基于AIHA患者院内及院外护理展开综述研究,涵盖健康教育、心理护理、病情观察、延续护理等方面,以期为临床AIHA的治疗及护理提供参考。

Clinical profile of autoimmune hemolytic anemia with monoclonal gammopathy IgMk

Objective To explore the clinical features of autoimmune hemolytic anemia(AIHA)with monoclonal gammopathy IgMk.Methods The clinical and laboratory features of 12 AIHA with monoclonal gammopathy Ig Mκwere retrospectively analyzed.Results 12 cases with monoclonal immunoglobulin Igmκwere found in 85 pa-

沙利度胺导致自身免疫性溶血性贫血1例

本文报道1例15岁的重型β地中海贫血女性患儿,从出生6个月开始依赖输血治疗,且输血需求随着年龄增长而增加。患儿15岁开始接受沙利度胺治疗,治疗1个月后,完全脱离输血,血红蛋白水平维持在98~109 g·L^(-1),但在沙利度胺治疗第11个月时出现头晕和疲劳症状、血红蛋白下降,间接抗球蛋白试验和直接抗球蛋白试验阳性,诊断为新发的自身免疫性溶血性贫血,考虑与沙利度胺相关,不良反应关联性评价为“很可能”。经停药、糖皮质激素治疗后,间接抗球蛋白试验和直接抗球蛋白试验转为阴性。本病例提示,使用沙利度胺治疗地中海贫血患者应定期进行直接抗球蛋白试验和间接抗球蛋白试验,监测自身免疫性溶血性贫血的发生。

血抗人球蛋白试验阴性的自身免疫性溶血性贫血临床误诊分析

目的总结血抗人球蛋白试验阴性的自身免疫性溶血性贫血(AIHA)患者的临床特点、诊治方法及误诊原因、防范措施。方法回顾性分析2019年6月—2023年9月收治的曾误诊的血抗人球蛋白试验阴性的AIHA 11例的临床资料。结果6例因黑便就诊,误诊为急性上消化道出血;2例因腹痛、黄疸就诊,腹部CT提示胆总管结石,误诊为胆总管结石伴急性胆管炎;3例因晕厥就诊,查叶酸、维生素B 12降低,血常规示大细胞性贫血,误诊为巨幼细胞性贫血。误诊时间(4.82±1.67)d。11例血抗人球蛋白试验均阴性,6例经诊断性治疗和综合评估病情,2例通过骨髓单个核细胞抗人球蛋白试验阳性,3例转诊至外院通过微柱凝胶法查自身抗体阳性确诊血抗人球蛋白试验阴性AIHA。11例给予相应治疗后9例预后良好,1例死亡;1例疗效不佳放弃进一步治疗出院。结论血抗人球蛋白试验阴性AIHA临床少见,缺乏特异性症状、体征,易误诊。加强临床医生对该病认识,提高警惕性;对高度怀疑AIHA但血抗人球蛋白试验阴性患者经综合病情评估后可予诊断性治疗,必要时筛查骨髓抗人球蛋白试验或转诊至上级医院。

自身免疫性溶血性贫血(AIHA)患者意外抗体类型与输血疗效相关性分析

目的分析广州地区AIHA患者抗体类型并跟踪输血疗效,为AIHA患者临床输血治疗策略提供参考依据。方法在2021年1月—2023年10月本地区医院输血科(血库)送检至本站的96份疑难交叉配血标本分析鉴定出25份AIHA患者标本,再对其进行血型鉴定、Rh系统的抗原表型分型、抗体筛查及交叉配血,分析AIHA患者抗体类型与输血疗效的相关性。结果25份AIHA患者标本中,ABO正反定型相符17份,8份正反定型不符。主侧配血不相合19(19/25,76%)份,其中直接Coombs试验阳性+自身抗体阳性+无特异性抗体阳性18(18/19,94.7%)份;自身抗体+同种抗体阳性1(1/19,5.3%)份。主侧配血相合6(6/25,24%)份,其中自身抗体阳性3(3/6,50%)份;自身抗体+同种抗体阳性3(3/6,50%)份。25名AIHA患者中20名患者有输血治疗并能追踪到输血疗效,5名患者没有输血治疗或转院而无法追踪。输血有效11(11/20,55%)例;输血部分有效6(6/20,30%)例;输血无效3(3/20,15%)例。配血不合病例中,输血有效或部分有效17(17/20,85%)例。结论AIHA患者的输血疗效与交叉配血结果并无直接联系,在调节干预自身免疫内环境并排除同种意外抗体导致的配血不相合前提下,配血不合的AIHA患者在充分权衡利弊的情况下,必要时可输血,选择ABO、Rh系统抗原同型血液,可提高输血安全性和有效率。

黏多糖贮积症脐带血造血干细胞移植术后并发自身免疫性溶血性贫血的研究

目的 探讨黏多糖贮积症脐带血造血干细胞移植术(UCBT)后发生自身免疫性溶血性贫血(AIHA)的情况。方法 回顾性分析2019年3月—2023年6月在本中心收治的黏多糖贮积症患者行UCBT发生AIHA的临床特征及治疗方法。结果 6例患者发生AIHA,均为男性。其中4例为单份脐血移植,2例为双份脐血移植中;6例患者给予激素、静注人免疫球蛋白、利妥昔单抗治疗后,5例治愈,1例死于感染。结论 AIHA一般发生在移植后3-6个月;血型不合更易发生移植后AIHA;激素、静注人免疫球蛋白、利妥昔单抗治疗有效。

不同红细胞制剂输注对自身免疫性溶血性贫血患者输血疗效的影响

目的:探讨自身免疫性溶血性贫血(AIHA)患者输注不同红细胞制剂的疗效。方法:选取96例AIHA患者作为研究对象,依据输注红细胞制剂不同分为对照组(n=47,接受去白细胞悬浮红细胞输注)和观察组(n=49,接受洗涤红细胞输注)。比较两组患者的疗效、临床症状积分、血液指标及输注不良反应发生情况。结果:治疗后,观察组患者总有效率为93.88%,高于对照组的78.72%(P<0.05);观察组患者心悸、头晕、气短、乏力及胸闷症状评分分别为[(1.44±0.18)、(1.78±0.23)、(1.55±0.18)、(1.06±0.17)及(1.15±0.18)分],均低于对照组的[(1.92±0.23)、(2.04±0.25)、(1.74±0.21)、(1.43±0.18)及(1.24±0.17)分](P<0.05);观察组患者血红蛋白(Hb)、红细胞计数(RBC)水平分别为[(97.42±9.83)g/L、(4.72±0.48)×1012/L]高于对照组的[(80.54±8.35)g/L、(4.01±0.45)×1012/L](P<0.05),网织红细胞比例(Ret)、总胆红素(TBIL)为[(18.12±1.94)%、(26.33±2.75)μmol/L],低于对照组的[(21.33±2.56)%、(40.15±4.29)μmol/L](P<0.05)。两组患者输注不良反应总发生率无统计学差异(P>0.05)。结论:洗涤红细胞输注疗效更佳,可显著改善AIHA患者临床症状、血液指标,且安全性良好。

自身免疫性溶血性贫血合并淋巴瘤的临床特点及预后因素

目的分析伴有自身免疫性溶血性贫血(autoimmune hemolytic anemia,AIHA)的淋巴瘤患者的临床特征,探讨治疗效果、预后影响因素及临床治疗方案。方法收集2011年1月至2023年7月在徐州医科大学附属医院血液科治疗的18例淋巴瘤合并AIHA患者的临床资料,回顾性分析其临床特点、治疗效果及预后生存情况。结果18例患者的中位发病年龄为61岁,其中男性12例,女性6例,Ann Arbor分期为Ⅱ期4例,Ⅲ期8例,Ⅳ期6例。根据免疫表型来源不同,T细胞淋巴瘤4例,B细胞淋巴瘤14例,其中弥漫大B细胞淋巴瘤10例。18例患者AIHA抗体类型皆为温抗体型,其中IgG型4例,补体C3型5例,IgG+补体C3型7例,2例患者类型未定。IgG水平对淋巴瘤患者是否合并AIHA有统计学意义(P=0.042)。国际预后评分(International Prognostic Index,IPI)高低对淋巴瘤患者是否合并AIHA有统计学意义(P=0.011)。利用受试者工作特征(receiver operating characteristic,ROC)曲线分析出乳酸脱氢酶(lactate dehydrogenase,LDH)最佳截断值为400 U/L(P=0.002)后,对其进行分组并利用Kaplan⁃Meier法进行生存分析发现:LDH>400 U/L是影响患者总生存(overall survival,OS)的不利预后因素。患者性别为女性是影响OS和无进展生存(progression⁃free survival,PFS)的不利预后因素。IPI评分为高中危/高危组(3~5分)是影响OS的不利预后因素。18例患者2年OS率为50%,2年PFS率为62.9%。结论淋巴瘤合并AIHA临床较少见,好发于老年,多数为Ⅲ~Ⅳ期患者,治疗上应根据患者临床特征采取个性化的治疗方案,预后主要取决于淋巴瘤的缓解程度,患者总体预后较差。