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1.
Medicine (Baltimore) ; 99(6): e18984, 2020 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-32028408

RESUMO

RATIONALE: Autoimmune hemolytic AQ5 anemia (AIHA) is an immune disorder caused by antibodies directed against unmodified autologous red blood cells. In rare cases, AIHA is comorbid with other immunological disorders; for instance, when AIHA is complicated with immunologic thrombocytopenic purpura (ITP) it is called Evans Syndrome (ES). These multiple autoimmune mechanisms are referred to as "immunological tolerance loss," which is known as a characteristic autoimmunity specific for AIHA. And there are no estimation of the risk for thromboembolism in the "immunological tolerance loss" case. PATIENT CONCERNS: A 66-year-old man was diagnosed with ES after autologous stem cell transplantation for malignant lymphoma. His background immunological status was complicated because AIHA was mixed-type (warm and cold antibody type). The direct/indirect Coombs tests were positive. The anticomplement antibody was positive and his cold hemagglutinin level had increased. Anticardiolipin antibodies were negative: anticardiolipin ß2GPI antibody ≤1.2 U/mL (<3.5), anticardiolipin immunoglobulin G antibody ≤8 U/mL (<10), and anticardiolipin immunoglobulin M antibody ≤5 U/mL (<8). DIAGNOSES: ITP and mixed-type AIHA. INTERVENTIONS: The patient achieved complete response by initial prednisolone therapy; however, he did not respond to corticosteroid therapy after AIHA recurrence. He required the red blood cell transfusion due to the progression of hemolytic anemia. OUTCOMES: On the fourth day of refractory treatment following AIHA recurrence, the patient had acute respiratory failure with severe hypoxia and died. The cause of death was identified as pulmonary embolism (PE) based on the laboratory data and echocardiography findings, and a literature search suggested rapidly progressive hemolysis-induced PE. LESSONS: Although infrequent, comorbid thromboembolism to AIHA is well documented; however, a mixed-type AIHA case complicated with thromboembolism has not been previously reported. The combined pathophysiology of AIHA and thromboembolism should be considered in the clinical course of hemolysis. Our case suggested multiple immunological background, ITP, and mixed type AIHA, could be associated to a risk for thromboembolism (TE).


Assuntos
Anemia Hemolítica Autoimune/complicações , Embolia Pulmonar/etiologia , Idoso , Anemia Hemolítica Autoimune/diagnóstico , Evolução Fatal , Humanos , Masculino , Embolia Pulmonar/diagnóstico , Púrpura Trombocitopênica Idiopática/complicações , Púrpura Trombocitopênica Idiopática/diagnóstico , Trombocitopenia/complicações , Trombocitopenia/diagnóstico
2.
Rev Port Cir Cardiotorac Vasc ; 26(3): 223-224, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31734976

RESUMO

Cold agglutinins (CA) are autoantibodies whose clinical significance depends upon titer and thermal amplitude. Patients, which undergo cardio-pulmonary bypass and especially hypothermic cardioplegia myocardial protection, represent a challenge regarding operative management, as tissue temperature should be maintained above the threshold of agglutination. We report on a case in which the presence of CA was discovered during elective aortic valve replacement surgery, and managed with normothermic cardiopulmonary bypass and continuous retrograde warm blood cardioplegia administration.


Assuntos
Anemia Hemolítica Autoimune/complicações , Ponte Cardiopulmonar/métodos , Parada Cardíaca Induzida/métodos , Doenças das Valvas Cardíacas/cirurgia , Hipotermia Induzida/efeitos adversos , Anemia Hemolítica Autoimune/imunologia , Valva Aórtica/cirurgia , Autoanticorpos/efeitos adversos , Ponte Cardiopulmonar/efeitos adversos , Crioglobulinas/efeitos adversos , Procedimentos Cirúrgicos Eletivos , Parada Cardíaca Induzida/efeitos adversos , Doenças das Valvas Cardíacas/complicações , Implante de Prótese de Valva Cardíaca/efeitos adversos , Implante de Prótese de Valva Cardíaca/métodos , Humanos
3.
Rinsho Ketsueki ; 60(10): 1418-1424, 2019.
Artigo em Japonês | MEDLINE | ID: mdl-31695001

RESUMO

Autoimmune hemolytic anemia (AIHA) is secondary to underlying diseases, such as autoimmune diseases and lymphoid malignancies. Recently, solid cancers have also been reported to be associated with AIHA, although there is not much information available. In this study, we retrospectively examined the correlation between AIHA and onset of malignancy in 100 patients diagnosed with AIHA based on the broad definition of AIHA at our hospital and cooperating institutions from January 1, 1995 to May 31, 2016. Malignancies were detected in 52 of the 100 patients (hematological malignancies: 39 patients; solid cancers: 22 patients; total malignancies including multiple primary malignancies: 67 patients). Of the 67 patients with malignancies, 28 were diagnosed with malignancies within 6 months of AIHA diagnosis. All patients with cold agglutinin disease (CAD) were associated with malignancies. Compared with warm AIHA, solid cancers were significantly more common among the patients with CAD. These findings emphasize the importance of investigating the malignancies upon diagnosis of AIHA.


Assuntos
Anemia Hemolítica Autoimune/complicações , Neoplasias/complicações , Humanos , Estudos Retrospectivos , Fatores de Risco
7.
Clin Nucl Med ; 44(5): e372-e374, 2019 05.
Artigo em Inglês | MEDLINE | ID: mdl-30888998

RESUMO

We report a case of splenic marginal zone B-cell lymphoma discovered in a 73-year-old man with cold agglutinin disease. PET/CT revealed splenomegaly with focally intense uptake of F-FDG and diffusely increased bone marrow uptake, which was considered to be secondary to hemolytic anemia. Splenectomy was performed. The histopathology of the spleen showed splenic marginal zone B-cell lymphoma with partial splenic infarction, which correlated with the area of focal intense FDG uptake. Depending on the time since onset, splenic infarctions can present as focal FDG accumulation.


Assuntos
Anemia Hemolítica Autoimune/diagnóstico por imagem , Linfoma de Zona Marginal Tipo Células B/diagnóstico por imagem , Tomografia Computadorizada com Tomografia por Emissão de Pósitrons , Infarto do Baço/diagnóstico por imagem , Neoplasias Esplênicas/diagnóstico por imagem , Idoso , Anemia Hemolítica Autoimune/complicações , Fluordesoxiglucose F18 , Humanos , Linfoma de Zona Marginal Tipo Células B/complicações , Masculino , Compostos Radiofarmacêuticos , Neoplasias Esplênicas/complicações
8.
Rev. cuba. hematol. inmunol. hemoter ; 35(1): e944, ene.-mar. 2019. graf
Artigo em Espanhol | LILACS | ID: biblio-1003892

RESUMO

La infección por parvovirus B19 humano, es la causa de la mayor parte de los casos de crisis aplásica transitoria que aparecen de forma brusca en pacientes con enfermedades hemolíticas crónicas, como es el caso de la drepanocitosis. Por otra parte, se han descrito unos pocos casos de infección aguda, por parvovirus B19 humano como causa de anemia hemolítica autoinmune, por medio de la formación de anticuerpos dirigidos contra los glóbulos rojos. La asociación entre drepanocitosis y anemias hemolíticas autoinmunes es poco frecuente. Se reporta un caso poco usual de una paciente adulta, con antecedentes de hemoglobinopatía S/C que presentó una crisis aplásica y posteriormente apareció una anemia hemolítica autoinmune diagnosticada en el Instituto de Hematología e Inmunología. Se trató con dosis inmunosupresoras de esteroide, con lo que se alcanzó la remisión de la anemia hemolítica autoinmune(AU)


Infection with human B19 parvovirus is the cause of most cases of transient aplastic crisis that appear in patients with chronic hemolytic diseases, as in the case of sickle cell disease. On the other hand, a few cases of acute infection by human parvovirus B19 have been described as a cause of autoimmune hemolytic anemia, through the formation of antibodies directed against red blood cells. The association between sickle cell disease and autoimmune hemolytic anemia is rare. We report an unusual case of an adult patient, with a history of S C hemoglobinopathy who presented an aplastic crisis and subsequently an autoimmune hemolytic anemia diagnosed at the Institute of Hematology and Immunology, treated with high steroids doses, reaching the remission of autoimmune hemolytic anemia and constitutes the first report in Cuba(AU)


Assuntos
Humanos , Feminino , Pessoa de Meia-Idade , Transfusão de Eritrócitos/métodos , Anemia Hemolítica Autoimune/complicações , Anemia Hemolítica Autoimune/tratamento farmacológico , Prednisona/uso terapêutico , Anemia Falciforme/complicações
9.
J Clin Neurosci ; 62: 226-228, 2019 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-30612916

RESUMO

Progressive multifocal leukoencephalopathy (PML) is caused by John Cunningham (JC) virus in immunocompromized patients such as those with human immunodeficiency virus (HIV) infection, hematological malignancy, autoimmune disorder, and immunodeficiency disorder as well as those undergoing chemotherapy or immunosuppressive therapy. No effective treatments have been established for PML, which commonly causes severe neurological sequelae. We describe the first case of PML in a patient without HIV infection who exhibited remarkable improvement following acute pyelonephritis with Escherichia coli bacteremia.


Assuntos
Hospedeiro Imunocomprometido , Leucoencefalopatia Multifocal Progressiva/imunologia , Pielonefrite/complicações , Adulto , Anemia Hemolítica Autoimune/complicações , Bacteriemia/complicações , Feminino , Humanos , Neoplasias Pulmonares/complicações , Linfoma de Zona Marginal Tipo Células B/complicações
10.
Medicine (Baltimore) ; 98(4): e14201, 2019 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-30681592

RESUMO

RATIONALE: Concomitant cryoglobulinemic vasculitis and cold agglutinin disease (CAD) is an extremely uncommon clinical scenario. The role of bortezomib in the treatment of cryoglobulinemic vasculitis needs further investigation. PATIENT CONCERNS: A 72-year-old Chinese woman presented with a 25-year history of cyanosis of the extremities after cold exposure, which worsened and was accompanied with purpuric skin lesions and proteinuria in recent years. Laboratory data demonstrated hemolysis. Cold agglutinin and cryoglobulin tests were positive. There was no evidence for malignancies after blood, image, and pathologic tests. DIAGNOSES: Concomitant cryoglobulinemic vasculitis and CAD. INTERVENTIONS: The patient was treated with bortezomib-based regimen, including bortezomib, cyclophosphamide, and dexamethasone. OUTCOMES: The patient responded well to the treatment. Both symptoms and laboratory tests significantly improved. The patient's condition was in a state of sustained remission in the 6-month follow-up. LESSONS: This rare case promotes further understanding of these 2 diseases and suggests that bortezomib is a promising treatment in type I cryoglobulinemic vasculitis.


Assuntos
Anemia Hemolítica Autoimune/tratamento farmacológico , Bortezomib/uso terapêutico , Crioglobulinemia/tratamento farmacológico , Vasculite/tratamento farmacológico , Idoso , Anemia Hemolítica Autoimune/complicações , Crioglobulinemia/complicações , Ciclofosfamida/uso terapêutico , Dexametasona/uso terapêutico , Feminino , Humanos , Resultado do Tratamento , Vasculite/complicações
12.
Blood ; 133(9): 893-901, 2019 02 28.
Artigo em Inglês | MEDLINE | ID: mdl-30559259

RESUMO

Cold agglutinin disease is a difficult-to-treat autoimmune hemolytic anemia in which immunoglobulin M antibodies bind to erythrocytes and fix complement, resulting in predominantly extravascular hemolysis. This trial tested the hypothesis that the anti-C1s antibody sutimlimab would ameliorate hemolytic anemia. Ten patients with cold agglutinin disease participated in the phase 1b component of a first-in-human trial. Patients received a test dose of 10-mg/kg sutimlimab followed by a full dose of 60 mg/kg 1 to 4 days later and 3 additional weekly doses of 60 mg/kg. All infusions were well tolerated without premedication. No drug-related serious adverse events were observed. Seven of 10 patients with cold agglutinin disease responded with a hemoglobin increase >2 g/dL. Sutimlimab rapidly increased hemoglobin levels by a median of 1.6 g/dL within the first week, and by a median of 3.9 g/dL (interquartile range, 1.3-4.5 g/dL; 95% confidence interval, 2.1-4.5) within 6 weeks (P = .005). Sutimlimab rapidly abrogated extravascular hemolysis, normalizing bilirubin levels within 24 hours in most patients and normalizing haptoglobin levels in 4 patients within 1 week. Hemolytic anemia recurred when drug levels were cleared from the circulation 3 to 4 weeks after the last dose of sutimlimab. Reexposure to sutimlimab in a named patient program recapitulated the control of hemolytic anemia. All 6 previously transfused patients became transfusion-free during treatment. Sutimlimab was safe, well tolerated, and rapidly stopped C1s complement-mediated hemolysis in patients with cold agglutinin disease, significantly increasing hemoglobin levels and precluding the need for transfusions. This trial was registered at www.clinicaltrials.gov as #NCT02502903.


Assuntos
Anemia Hemolítica Autoimune/tratamento farmacológico , Anemia Hemolítica/prevenção & controle , Anticorpos Monoclonais Humanizados/uso terapêutico , Complemento C1s/antagonistas & inibidores , Hemólise/efeitos dos fármacos , Índice de Gravidade de Doença , Idoso , Anemia Hemolítica/etiologia , Anemia Hemolítica Autoimune/complicações , Complemento C1s/imunologia , Feminino , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Prognóstico , Estudos Prospectivos
13.
PLoS One ; 13(11): e0207218, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-30408135

RESUMO

Thrombotic manifestations are a hallmark of many auto-immune diseases (AID), specially of warm autoimmune hemolytic anemia (wAIHA), as 15 to 33% of adults with wAIHA experience venous thromboembolic events (VTE). However, beyond the presence of positive antiphospholipid antibodies and splenectomy, risk factors for developing a VTE during wAIHA have not been clearly identified. The aim of this retrospective study was to characterize VTEs during wAIHA and to identify risk factors for VTE. Forty-eight patients with wAIHA were included, among whom 26 (54%) had secondary wAIHA. Eleven (23%) patients presented at least one VTE, that occurred during an active phase of the disease for 10/11 patients (90%). The frequency of VTE was not different between primary and secondary AIHA (23.7 vs. 19.2%; p = 0.5). The Padua prediction score based on traditional risk factors was not different between patients with and without VTE. On multivariate analysis, total bilirubin ≥ 40 µmol/L [odds ratio (OR) = 7.4; p = 0.02] and leucocyte count above 7x10(9)/L (OR = 15.7; p = 0.02) were significantly associated with a higher risk of thrombosis. Antiphospholipid antibodies were screened in 9 out the 11 patients who presented a VTE and were negative. Thus, the frequency of VTE is high (23%) during wAIHA and VTE preferentially occur within the first weeks of diagnosis. As no clinically relevant predictive factors of VTE could be identified, the systematic use of a prophylactic anticoagulation should be recommended in case of active hemolysis and its maintenance after hospital discharge should be considered. The benefit of a systematic screening for VTE and its procedure remain to be determined.


Assuntos
Anemia Hemolítica Autoimune/complicações , Tromboembolia Venosa/etiologia , Adulto , Idoso , Anemia Hemolítica Autoimune/sangue , Anemia Hemolítica Autoimune/terapia , Anticoagulantes/uso terapêutico , Bilirrubina/sangue , Feminino , Humanos , Contagem de Leucócitos , Masculino , Pessoa de Meia-Idade , Análise Multivariada , Estudos Retrospectivos , Fatores de Risco , Tromboembolia Venosa/sangue , Tromboembolia Venosa/prevenção & controle
14.
Transfusion ; 58(12): 2777-2781, 2018 12.
Artigo em Inglês | MEDLINE | ID: mdl-30291762

RESUMO

BACKGROUND: Evans syndrome is a rare autoimmune disorder that is defined by the simultaneous or sequential presence of two or more cytopenias without an obvious underlying precipitating cause. Evans syndrome usually follows a chronic relapsing and remitting course and is quite rare, making it difficult to evaluate in clinical studies. CASE REPORT: A 66-year-old male patient with a 17-year history of Evans syndrome presented with fulminant autoimmune hemolytic anemia (AIHA). He presented with a markedly elevated C-reactive protein (CRP; 46 mg/L [normal, 0-5 mg/L]) before onset of a decrease in hemoglobin. He required the transfusion of 20 units of red blood cells while awaiting response to aggressive immunosuppressive therapy including high-dose corticosteroids, intravenous immunoglobin therapy, and rituximab. He achieved a complete hematologic response. RESULTS: His postdischarge course was complicated by acute cholecystitis requiring laparoscopic cholecystectomy. In addition, his transfusional iron overload requiring 16 phlebotomies to reduce his ferritin level from 4933 µg/L to 326 µg/L, with phlebotomies ongoing every 2 weeks to achieve a ferritin level of less than 100 µg/L. CONCLUSION: Neither transfusional iron overload nor acute cholecystitis are well-recognized complications of a severe episode of AIHA. An elevated CRP has been recently recognized as an important prognostic marker in patients with immune thrombocytopenic purpura and this case suggests a need to evaluate its utility in AIHA.


Assuntos
Corticosteroides/administração & dosagem , Anemia Hemolítica Autoimune , Colecistite , Transfusão de Eritrócitos , Imunoglobulinas Intravenosas/administração & dosagem , Sobrecarga de Ferro , Rituximab/administração & dosagem , Trombocitopenia , Reação Transfusional , Idoso , Anemia Hemolítica Autoimune/sangue , Anemia Hemolítica Autoimune/complicações , Anemia Hemolítica Autoimune/terapia , Colecistite/sangue , Colecistite/complicações , Colecistite/patologia , Colecistite/terapia , Gangrena , Humanos , Sobrecarga de Ferro/sangue , Sobrecarga de Ferro/tratamento farmacológico , Sobrecarga de Ferro/etiologia , Sobrecarga de Ferro/patologia , Masculino , Trombocitopenia/sangue , Trombocitopenia/complicações , Trombocitopenia/terapia , Reação Transfusional/sangue , Reação Transfusional/tratamento farmacológico
15.
Ann Dermatol Venereol ; 145(12): 761-764, 2018 Dec.
Artigo em Francês | MEDLINE | ID: mdl-30197053

RESUMO

BACKGROUND: Digital necrosis is rarer than lower limb necrosis and constitutes a medical or surgical emergency. Etiological evaluation is required. Cold agglutinin disease is a cause of digital necrosis but diagnosis is difficult. PATIENTS AND METHODS: Herein we report the case of a 57-year-old man presenting recent paroxysmal acrosyndrome of the left hand subsequently complicated by digital necrosis following occupational exposure to cold in his work as a forklift driver. After etiological evaluation, a diagnosis of primary cold agglutinin disease was made. Intravenous rituximab and topical treatment resulted in complete healing. DISCUSSION: Cold agglutinin disease is a rare type of auto-immune hemolytic anemia. Following exposure to cold, paroxysmal cutaneous signs are frequent. The disease may be either primary or secondary with B-cell lymphoproliferative disorder, auto-immune disease or infection. A thorough workup is required. To date, the treatment combining the best positive response rate and good safety is rituximab in weekly perfusions over a 1-month period.


Assuntos
Anemia Hemolítica Autoimune/diagnóstico , Dedos/patologia , Deformidades Adquiridas da Mão/etiologia , Imunossupressores/uso terapêutico , Isquemia/etiologia , Doença de Raynaud/etiologia , Rituximab/uso terapêutico , Amputação , Anemia Hemolítica Autoimune/complicações , Anemia Hemolítica Autoimune/tratamento farmacológico , Anemia Hemolítica Autoimune/cirurgia , Temperatura Baixa , Terapia Combinada , Angiografia por Tomografia Computadorizada , Crioglobulinas/análise , Dedos/irrigação sanguínea , Dedos/diagnóstico por imagem , Dedos/cirurgia , Humanos , Cadeias kappa de Imunoglobulina/sangue , Isquemia/cirurgia , Masculino , Pessoa de Meia-Idade , Necrose , Doenças Profissionais/etiologia , Doença de Raynaud/diagnóstico por imagem , Fumar/efeitos adversos
16.
Rinsho Ketsueki ; 59(8): 997-1001, 2018.
Artigo em Japonês | MEDLINE | ID: mdl-30185717

RESUMO

A 26-year-old man presented with fever, multiple lymphadenopathies, polyclonal hypergammaglobulinemia, and an elevated serum interleukin-6 (IL-6) level. Multicentric Castleman disease (MCD) was diagnosed by lymph node biopsy. Treatment with prednisolone (PSL) was initiated; however, its efficacy was limited. During PSL tapering, rapidly progressive anemia and thrombocytopenia developed concurrently with increased reticulocyte level, elevated serum LDH level, decreased haptoglobin level, and positive direct Coombs test. Based on these findings, Evans syndrome, which is a concurrent development of autoimmune hemolytic anemia and immune thrombocytopenia, was confirmed. The PSL dose was increased but was ineffective. Therefore, treatment with tocilizumab was initiated, and the clinical findings of both MCD and Evans syndrome improved. The clinical course of this case suggests that tocilizumab could be a treatment option for Evans syndrome complicated with MCD. Three other cases of Evans syndrome complicated with MCD have also been reported; however, this is the first case that shows the efficacy of tocilizumab as treatment for both MCD and Evans syndrome.


Assuntos
Anemia Hemolítica Autoimune/tratamento farmacológico , Anticorpos Monoclonais Humanizados/uso terapêutico , Hiperplasia do Linfonodo Gigante/tratamento farmacológico , Trombocitopenia/tratamento farmacológico , Adulto , Anemia Hemolítica Autoimune/complicações , Hiperplasia do Linfonodo Gigante/complicações , Humanos , Masculino , Trombocitopenia/complicações
17.
BMJ Case Rep ; 20182018 Jul 10.
Artigo em Inglês | MEDLINE | ID: mdl-29991541

RESUMO

Cold agglutinin-mediated autoimmune haemolytic anaemia is associated with the development of autoantibodies that can agglutinate red blood cells at cold temperatures. While primary cold agglutinin disease is an idiopathic lymphoproliferative disorder, secondary cold agglutinin syndrome (CAS) complicates other diseases such as infections, autoimmune diseases and cancers, mostly low-grade lymphomas. Early recognition, treatment of CAS and treatment of its associated underlying diseases are crucial to a successful outcome. We report a case of CAS in a setting of diffuse large B cell lymphoma, in which the treatment course was complicated by worsened anaemia due to chemotherapy-induced myelosuppression. We reviewed previously reported cases and discussed diagnosis and treatment strategies, including novel complement inhibitors, as potential future therapy.


Assuntos
Anemia Hemolítica Autoimune/complicações , Linfoma Difuso de Grandes Células B/complicações , Idoso , Anemia Hemolítica Autoimune/sangue , Anemia Hemolítica Autoimune/diagnóstico , Feminino , Humanos , Linfoma Difuso de Grandes Células B/sangue , Linfoma Difuso de Grandes Células B/terapia
19.
Intern Med ; 57(22): 3261-3265, 2018 Nov 15.
Artigo em Inglês | MEDLINE | ID: mdl-29984743

RESUMO

Renal hemosiderosis occurs in the context of severe intravascular hemolysis, with the most common cause being paroxysmal nocturnal hematuria. Patients with cold agglutinin disease (CAD) have relatively mild hemolysis, and acute kidney injury (AKI) due to renal hemosiderosis has not been reported. We encountered a patient with CAD caused by lymphoplasmacytic lymphoma who developed AKI secondary to renal hemosiderosis after an excessive alcohol intake.


Assuntos
Lesão Renal Aguda/etiologia , Alcoolismo/complicações , Anemia Hemolítica Autoimune/complicações , Hemossiderose/complicações , Rim/diagnóstico por imagem , Lesão Renal Aguda/diagnóstico , Idoso , Anemia Hemolítica Autoimune/diagnóstico , Biópsia , Diagnóstico Diferencial , Hemossiderose/diagnóstico , Humanos , Imagem por Ressonância Magnética , Masculino , Tomografia Computadorizada por Raios X
20.
BMJ Case Rep ; 20182018 Jul 06.
Artigo em Inglês | MEDLINE | ID: mdl-29982182

RESUMO

An 85-year-old man presented with a pale appearance and generalised pruritic papules. Laboratory investigations disclosed eosinophilia, autoimmune haemolytic anaemia, mixed hyperbilirubinaemia, cholestasis and elevated serum IgG4 levels. Abdominal sonography and CT showed progressive dilatation of biliary trees, with diffuse pancreatic enlargement and a subtle capsule-like low-density rim around the pancreatic head and body. Endoscopic retrograde cholangiopancreatography found no stone-related biliary obstruction, while endoscopic transpapillary biopsy demonstrated chronic inflammation only. Nevertheless, the diagnosis of IgG4-related disease with coexisting autoimmune haemolytic anaemia was presumed. The clinical picture and laboratory abnormalities improved after administration of moderate dose of methylprednisolone.


Assuntos
Anemia Hemolítica Autoimune/complicações , Colangite/sangue , Colangite/complicações , Hipergamaglobulinemia/complicações , Imunoglobulina G/sangue , Idoso de 80 Anos ou mais , Anti-Inflamatórios/administração & dosagem , Ductos Biliares Intra-Hepáticos/diagnóstico por imagem , Ductos Biliares Intra-Hepáticos/patologia , Colangiopancreatografia Retrógrada Endoscópica , Colangite/tratamento farmacológico , Exantema/etiologia , Humanos , Masculino , Metilprednisolona/administração & dosagem , Pâncreas/diagnóstico por imagem , Pâncreas/patologia , Tomografia Computadorizada por Raios X
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