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.

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.

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.

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.

Subclasses of warm autoantibody Ig Gin patients with autoim m unehemolytic anemia and their clinicalim plications

Objective To explore the mechanism of autoimmune hemolysis and establish a more sensitive test for autoimmune hemolytic anemia (AIHA) Methods IgG subclasses were tested in 40 patients with idiopathic or secondary AIHA by Coombs test with monoclonal antibodies Hb, TBil, RC and FHb, as hemolytic parameters, were used to analyze the clinical implications of subclasses of AIHA warm autoantibody IgG Results In most patients IgG incomplete warm autoantibody was IgG1(27 patients), but IgG3(20 patients), rarely IgG2(14 patients) and IgG4(9 patients) also occurred Three groups were analyzed: Group A included 20 patients with IgG3; Group B included 14 patients with IgG1 but without IgG3; Group C included 6 patients without IgG1 and IgG3 There were significant differences ( P <0 01) between groups in Hb, TBil, RC and FHb Moreover, we also found that the sensitivity of Coombs test with polyclonal antiserums was 90 0%, while that of Coombs test with monoclonal antibodies was 97 5% The effect of treatment was worst in patients with positive IgG3 autoantibody, whose hemolysis recurred frequently Conclusion IgG3 autoantibody was the most effective in bringing about red cell destruction, IgG1 autoantibody was less effective, IgG2 even less, whereas IgG4 autoantibody was shown to hardly affect red cell survival Coombs test with monoclonal antibodies was more sensitive than that with polyclonal antiserums Patients’ respondence to treatment correlated with the type of IgG subclasses