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1.
Transfus Apher Sci ; 63(2): 103871, 2024 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-38245405

RESUMO

The histo-blood group antigens P, P1 and Pk are a closely related set of glycosphingolipid structures expressed by red blood cells and other tissues. None of these three characters is expressed on p cells, a null phenotype that arises in the context of homozygous mutation of the A4GALT gene. Subjects with p phenotype spontaneously develop a natural alloantibody named anti-PP1Pk, which is a mixture of IgG and IgM against P1, P and Pk. While anti-P1 is a weak cold antibody with poor clinical significance, anti-P and anti-Pk antibodies are potent haemolysins responsible for severe hemolytic transfusion reactions. The rare anti-PP1Pk alloantibodies are associated with recurrent spontaneous abortion in the first trimester of gestation. P and Pk antigens are expressed at high levels on the placenta and antibodies directed against both these structures are deleterious to placental trophoblasts. Here we describe the use of plasma exchange (PEX) in a nulliparous 39-year-old woman with anti-PP1Pk antibodies and a history of repeated spontaneous early abortions and hypofertility. The patient underwent apheresis starting from the third week throughout the pregnancy and a healthy child was delivered by cesarean section at 35 WG. The newborn required only phototherapy within a few days of life. We can state that an early treatment with the only PEX has proven to be effective and safe in the management of a fetomaternal P-incompatibility caused by a high anti-PP1Pk titer (256).


Assuntos
Aborto Habitual , Anemia Hemolítica Autoimune , Antígenos de Grupos Sanguíneos , Adulto , Feminino , Humanos , Recém-Nascido , Gravidez , Aborto Habitual/etiologia , Aborto Habitual/terapia , Anemia Hemolítica Autoimune/terapia , Cesárea/efeitos adversos , Isoanticorpos , Sistema do Grupo Sanguíneo P/genética , Placenta , Troca Plasmática/efeitos adversos , Gestantes
2.
Ann Med ; 55(1): 231-240, 2023 12.
Artigo em Inglês | MEDLINE | ID: mdl-36576329

RESUMO

OBJECTIVE: Autoimmune hemolytic anemia (AIHA) is rare heterogeneous disorder characterized by red blood cell (RBC) destruction via auto-antibodies, and after RBC is destroyed, proinflammatory danger-associated molecular patterns including extracellular hemoglobin, heme, and iron which causing cell injury. And oxidative stress represents one of the most significant effects of chronic hemolysis. Jianpishengxue keli can improve the symptoms of anemia patients with kidney disease and tumors and are beneficial in promoting recovery from chronic inflammation. Therefore, it is presumed that Jianpishengxue keli can improve the symptoms of AIHA. We aimed to investigate iron metabolism in AIHA and effects of Jianpishengxue keli on AIHA murine model. METHODS: Nineteen hemolytic episode AIHA patients, 10 remission patients and 10 healthy controls (HCs) were enrolled in this study. Serum hepcidin, ferritin and other related indicators of iron metabolism were measured. Mouse models of AIHA were established and received high, medium, or low doses of Jianpishengxue keli by gavage daily for 14 and 28 days respectively. The level of RBCs, Hb, bilirubin, LDH, hepcidin, and the expression level of hepcidin mRNA, and hepatic ferroportin 1(FPN1) protein were evaluated. RESULTS: Serum hepcidin in hemolytic episode AIHA patients and remission patients were significantly higher than that in HCs (p = 0.0083 and p = 0.0473, respectively). Serum ferritin in hemolytic AIHA patients was significantly higher than that in HCs (p = 0.008). Serum transferrin saturation levels are increased in patients with AIHA[ (57.21 ± 8.96) %]. EPO in hemolytic group was higher than that in healthy control (p<0.05). In AIHA mouse models, IBIL decreased after 14 days of high dose drug intervention. After 28 days, TBIL and IBIL both significantly decreased in all dose groups and LDH significantly decreased in the medium-and high-dose groups. Body weight improved, and the level of RBCs, Hb and hepcidin in the high-dose group returned to normal. After 14 and 28 days of intervention, hepatic hepcidin mRNA in all dose group significantly decreased. Hepatic FPN1 protein which were significantly lower in the AIHA mouse models, increased in all dose groups after drug intervention for 28 days. CONCLUSION: Iron metabolism abnormalities exists in AIHA patients and Jianpishengxue keli can ameliorate hemolysis and improve iron metabolism in AIHA mouse models.KEY MESSAGESIron metabolism abnormalities exists in hemolytic episode AIHA patients. Hepcidin and ferritin levels significantly elevated and also correlated with the severity of AIHA patients. Jianpishengxue keli can ameliorate hemolysis and prompt the recovery of AIHA.


Assuntos
Anemia Hemolítica Autoimune , Hepcidinas , Humanos , Animais , Camundongos , Hepcidinas/metabolismo , Anemia Hemolítica Autoimune/tratamento farmacológico , Hemólise , Modelos Animais de Doenças , Ferro , Hemoglobinas , Ferritinas , RNA Mensageiro
3.
Transfus Clin Biol ; 30(1): 5-7, 2023 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-35944885

RESUMO

Red blood cell (RBC) alloimmunization which is the production of antibodies in response to foreign red cell antigen(s) may occur through exposure to cells or tissues from a genetically different member of same species via transfusion, transplantation or pregnancy. It may cause hemolytic disease of fetus and newborn (HDFN). Usually the incidence of HDFN due to irregular erythrocyte antibody is rare in primigravida. Here we report a primigravida pregnant woman who developed multiple alloantibodies and the neonate developed severe HDFN. A 36-year-old primigravida pregnant woman who had no history of significant medical issues except surgery done for severe endometriosis 1 year back and she had no history of previous blood transfusion presented to us for delivery. The antibody screening came out to be positive with a reaction in cell I and cell II of the antibody screening panel. Further, a mixture of anti D + anti C + anti E alloantibodies were identified using 16 cells panel, select cells and red cell phenotyping. The neonate developed severe HDFN which was managed with phototherapy, exchange transfusion and IvIg. There was no exposure history for sensitization except bleeding in early 2nd trimester. There was a significant discrepancy among mother, father and neonate Rh phenotype which was resolved with clinical history of Invitro fertilization (IVF) with sperm donation. This index case illustrates the need of antibody screening in primigravida antenatal women specially for Rh D negative high risk cases. It also shows importance of Rh Kell typing in sperm donors for future transfusion support of the child.


Assuntos
Anemia Hemolítica Autoimune , Eritroblastose Fetal , Feminino , Masculino , Gravidez , Humanos , Isoanticorpos , Sêmen , Eritroblastose Fetal/etiologia , Eritroblastose Fetal/terapia , Eritrócitos , Transfusão de Sangue , Anemia Hemolítica Autoimune/complicações
4.
Rev Med Interne ; 43(10): 608-616, 2022 Oct.
Artigo em Francês | MEDLINE | ID: mdl-35691756

RESUMO

Some common clinical situations, such as splenomegaly or lymphocytosis, or less common, such as autoimmune hemolytic anemia, cold agglutinin disease, or cryoglobulinemia can lead to the diagnosis of splenic lymphoma. Splenic lymphoma is rare, mainly of non-hodgkinian origin, encompassing very different hematological entities in their clinical and biological presentation from an aggressive form such as hepato-splenic lymphoma to indolent B-cell lymphoma not requiring treatment such as marginal zone lymphoma, the most frequent form of splenic lymphoma. These entities can be challenging to diagnose and differentiate. This review presents different clinical and biological manifestations suspicious of splenic lymphoma and proposes a diagnosis work-up. We extended the strict definition of splenic lymphoma (lymphoma exclusively involving the spleen) to lymphoma thant can be revealed by a splenomegaly and we discuss the differential diagnosis of splenomegaly.


Assuntos
Anemia Hemolítica Autoimune , Linfocitose , Linfoma de Zona Marginal Tipo Células B , Neoplasias Esplênicas , Anemia Hemolítica Autoimune/diagnóstico , Anemia Hemolítica Autoimune/terapia , Diagnóstico Diferencial , Humanos , Linfocitose/patologia , Linfoma de Zona Marginal Tipo Células B/diagnóstico , Linfoma de Zona Marginal Tipo Células B/patologia , Linfoma de Zona Marginal Tipo Células B/terapia , Neoplasias Esplênicas/diagnóstico , Neoplasias Esplênicas/patologia , Neoplasias Esplênicas/terapia , Esplenomegalia/diagnóstico , Esplenomegalia/etiologia
5.
Vet Rec ; 190(7): e946, 2022 04.
Artigo em Inglês | MEDLINE | ID: mdl-34580882

RESUMO

BACKGROUND: Folate deficiency in people can occur in conditions causing increased demand, including haemolytic anaemia. This has not been investigated in dogs with non-associative immune-mediated haemolytic anaemia (IMHA). METHODS: Cohort study of 15 dogs with non-associative IMHA. Haematocrit (HCT) and serum folate concentrations were measured at presentation and each subsequent venipuncture performed for monitoring. The relationship between serum folate concentrations and HCT was investigated using linear and logistic mixed-effects regression models and in paired samples using a one-tailed paired t-test. RESULTS: Low serum folate concentrations occurred in five of 15 dogs. In 126 samples, a significant positive relationship was found between HCT and corresponding serum folate concentrations. A significant relationship was found between dichotomised folate concentrations (below the reference interval or within/above the reference interval) and HCT and between serum folate concentrations and dichotomised HCT (less than or equal/above 0.30 L/L). For paired samples (available in eight dogs), the mean serum folate concentration of samples with the lowest HCT was significantly lower than that of samples in which the HCT first exceeded 0.30 L/L. CONCLUSIONS: Low serum folate concentrations were observed in some dogs with non-associative IMHA. Further studies are needed to determine the cause and investigate whether folate supplementation would be beneficial.


Assuntos
Anemia Hemolítica Autoimune , Anemia Hemolítica , Doenças do Cão , Anemia Hemolítica/veterinária , Anemia Hemolítica Autoimune/veterinária , Animais , Estudos de Coortes , Cães , Ácido Fólico , Humanos
6.
Br J Haematol ; 196(4): 1096-1104, 2022 02.
Artigo em Inglês | MEDLINE | ID: mdl-34862601

RESUMO

Red blood cell (RBC) alloimmunisation with anti-D and anti-K comprise the majority of cases of fetal haemolytic disease requiring intrauterine red cell transfusion (IUT). Few studies have investigated which haematological parameters can predict adverse fetal or neonatal outcomes. The aim of the present study was to identify predictors of adverse outcome, including preterm birth, intrauterine fetal demise (IUFD), neonatal death (NND) and/or neonatal transfusion. We reviewed the records of all pregnancies alloimmunised with anti-K and anti-D, requiring IUT over 27 years at a quaternary fetal centre. We reviewed data for 128 pregnancies in 116 women undergoing 425 IUTs. The median gestational age (GA) at first IUT was significantly earlier for anti-K than for anti-D (24·3 vs. 28·7 weeks, P = 0·004). Women with anti-K required more IUTs than women with anti-D (3·84 vs. 3·12 mean IUTs, P = 0·036) and the fetal haemoglobin (Hb) at first IUT was significantly lower (51.0 vs. 70.5 g/l, P = 0·001). The mean estimated daily decrease in Hb did not differ between the two groups. A greater number of IUTs and a slower daily decrease in Hb (g/l/day) between first and second IUTs were predictive of a longer period in utero. Earlier GA at first IUT and a shorter interval from the first IUT until delivery predicted IUFD/NND. Earlier GA and lower Hb at first IUT significantly predicted need for phototherapy and/or blood product use in the neonate. In the anti-K group, a greater number of IUTs was required in women with a higher titre. Furthermore, the higher the titre, the earlier the GA at which an IUT was required in both groups. The rate of fall in fetal Hb between IUTs decreased, as the number of transfusions increased. Our present study identified pregnancies at considerable risk of an unfavourable outcome with anti-D and anti-K RBC alloimmunisation. Identifying such patients can guide pregnancy management, facilitates patient counselling, and can optimise resource use. Prospective studies can also incorporate these characteristics, in addition to laboratory markers, to further identify and improve the outcomes of these pregnancies.


Assuntos
Anemia Hemolítica Autoimune/terapia , Transfusão de Sangue Intrauterina/métodos , Eritrócitos/imunologia , Isoimunização Rh/fisiopatologia , Imunoglobulina rho(D)/metabolismo , Adulto , Feminino , Feto , Humanos , Gravidez , Estudos Retrospectivos , Resultado do Tratamento
7.
BMJ Case Rep ; 14(7)2021 Jul 26.
Artigo em Inglês | MEDLINE | ID: mdl-34312140

RESUMO

A 70-year-old man with known cold autoimmune haemolytic anaemia was referred to the emergency department with increased shortness of breath on exertion. He had been confirmed positive for non-variant COVID-19 infection 1 week earlier based on nasopharyngeal swab PCR assay. CT thorax demonstrated diffuse patchy bilateral ground glass opacities, consistent with COVID-19 pneumonia. Bloodwork demonstrated severe cold agglutinin mediated haemolytic anaemia. To help stabilise the patient, he was transferred to a tertiary care hospital for urgent therapeutic plasma exchange. Key supportive therapy included folic acid supplementation, ensuring the patient was kept warm and warmed infusions including transfusions via the apheresis machine. The patient made a good recovery following plasma exchange, and his haemoglobin levels remained stable by discharge.


Assuntos
Anemia Hemolítica Autoimune , COVID-19 , Idoso , Anemia Hemolítica Autoimune/terapia , Dispneia , Humanos , Masculino , Troca Plasmática , SARS-CoV-2
8.
Pediatr Transplant ; 25(7): e14045, 2021 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-34092010

RESUMO

BACKGROUND: Warm-antibody AIHA is known to complicate solid organ (SOT) and HSCT, the disease maybe refractory to standard therapy. Immunosuppressive therapies as well as IVIG, and rituximab have been the main stay of treatment. Over the past decade, B-lymphocyte targeted, anti-CD-20 antibody has been recognized in the treatment of autoimmune diseases and utilized in AIHA. Bortezomib, a proteasome inhibitor that causes apoptosis of plasma cells, is an appealing targeted therapy in secondary AIHA and has demonstrated efficacy in HSCT patients. From our experience, we advocate for early targeted therapy that combines B cell with plasma cell depletion. CASE REPORT: We describe a 4-year-old-girl with stage III neuroblastoma, complicated with intestinal necrosis needing multivisceral transplant developed warm AIHA 1-year after transplantation, and following an adenovirus infection. She received immunoglobulin therapy, rituximab, sirolimus, plasmapheresis, and long-term prednisolone with no sustained benefit while developing spinal fractures related to the latter therapy. She received bortezomib for intractable AIHA in combination with rituximab with no appreciable adverse effects. Three years later the child remains in remission with normal reticulocyte and recovered B cells. In the interim, she required chelation therapy for iron overload related to blood transfusion requirement during the treatment of AIHA. CONCLUSION: We propose early targeted anti-plasma cell therapy with steroid burst, IVIG, rituximab, and possible plasmapheresis may reduce morbidity in secondary refractory w-AIHA.


Assuntos
Anemia Hemolítica Autoimune/terapia , Neuroblastoma/cirurgia , Complicações Pós-Operatórias/terapia , Vísceras/transplante , Antineoplásicos/uso terapêutico , Bortezomib/uso terapêutico , Pré-Escolar , Terapia Combinada , Feminino , Humanos , Terapia de Imunossupressão/métodos , Necrose , Neuroblastoma/patologia , Plasmaferese , Rituximab/uso terapêutico
9.
J Oncol Pharm Pract ; 27(4): 1026-1028, 2021 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-32951522

RESUMO

INTRODUCTION: Atezolizumab is currently the only immunotherapy used in conjunction with nab-paclitaxel for locally advanced or triple negative breast cancer. Limited data is available regarding hemolytic anemia as a side effect of atezolizumab. CASE REPORT: We describe a 59-year-old female with a history of triple negative breast cancer with bone metastases presenting for follow up on Cycle 1, Day 15 of atezolizumab and nab-paclitaxel (100 mg/m2). Patient's complete blood count (CBC) showed macrocytic anemia, with further workup significant for autoimmune hemolytic anemia (AIHA) attributed to atezolizumab.Management and outcome: Patient was started on a high dose prednisone taper starting at 80 mg daily for 16 days, folic acid 1 mg three times daily, iron sucrose, and darbepoetin alfa. Patient's counts recovered, and she was able to start Cycle 2 and continued through Cycle 10 without any additional pre-medications. DISCUSSION: Hemolytic anemia induced by atezolizumab is a rare side effect that was successfully treated in this patient with a prednisone taper.


Assuntos
Anemia Hemolítica Autoimune/induzido quimicamente , Anemia Hemolítica Autoimune/diagnóstico , Anticorpos Monoclonais Humanizados/efeitos adversos , Anemia Hemolítica Autoimune/tratamento farmacológico , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Feminino , Humanos , Pessoa de Meia-Idade , Prednisona/uso terapêutico , Neoplasias de Mama Triplo Negativas/tratamento farmacológico
10.
Indian J Pediatr ; 87(12): 1018-1028, 2020 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-32607667

RESUMO

In spite of advances in medical science, Rh alloimmunisation remains one of the leading causes of preventable neuro-morbidities and significant neonatal hyperbilirubinemia in lower-middle income countries. Despite availability of effective antenatal preventive strategy (Anti-D), its uptake in antenatal period is low due to ignorance. Further, once diagnosed, there is lack of adequate antenatal follow up in health care facility. Some of these cases even remain undiagnosed in antenatal period only to present as a case of severe hyperbilirubinemia and kernicterus in late neonatal period. Thus, there is an urgent need for creating awareness and educating health care professionals for early detection and timely management in both antenatal and postnatal period. Following two doses of anti-D prophylaxis (one in antenatal period and one in immediate postnatal period) the incidence of Rh alloimmunisation can reduce to <1%. It is recommended to follow all Rh alloimmunised pregnancies antenatally with serial indirect Coombs test titre (till critical titre is reached) followed by serial Doppler velocimetry of middle cerebral artery in a perinatal centre where facility for intrauterine transfusion as well as advanced neonatal care is available. Postnatal management of these infants comprises of confirmation of diagnosis, aggressive phototherapy and in selective cases, double volume exchange transfusion. With appropriate antenatal and postnatal management, the prognosis of Rh alloimmunised pregnancy remains favourable and long term outcome of Rh alloimmunised infants remain comparable with their normal counterparts.


Assuntos
Anemia Hemolítica Autoimune , Isoimunização Rh , Transfusão Total , Feminino , Humanos , Hiperbilirrubinemia Neonatal , Recém-Nascido , Fototerapia , Gravidez , Isoimunização Rh/diagnóstico , Isoimunização Rh/prevenção & controle
11.
Zhongguo Shi Yan Xue Ye Xue Za Zhi ; 28(3): 904-908, 2020 Jun.
Artigo em Chinês | MEDLINE | ID: mdl-32552956

RESUMO

OBJECTIVE: To evaluate the characteristics of autoimmune hemolytic anemia caused by salvianolate by antibody detection and clinical index monitoring. METHODS: Micro-column gel anti-human globulin method was used for irregular antibody screening and antibody identification. Salvianolate, sodium creatine phosphate and levocarnitine were used to sensitize red blood cells that were compatible with the patient's plasma, and the RBCs were used to test drug antibody in patient plasma respectively. The patient's clinical examination of hemolysis index and blood transfusion effect were analyed retrospectively. RESULTS: The patients were positive for irregular antibody screening, and there were antoanti-Ce antibodies in serum. The erythrocytes sensitized with salvianolate in the patient's serum were positive, while those sensitized with sodium creatine phosphate and levocarnitine were negative. CONCLUSION: Salvianolate causes drug-induced autoimmune hemolytic anemia in this patient.


Assuntos
Anemia Hemolítica Autoimune , Transfusão de Sangue , Eritrócitos , Humanos , Extratos Vegetais , Estudos Retrospectivos
12.
Vox Sang ; 115(6): 515-524, 2020 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-32249930

RESUMO

BACKGROUND: Warm autoantibodies (WAA) are antibodies that react with an antigen on a patient's own red-blood-cells and can complicate compatibility testing whether or not they cause clinical haemolysis. The goal of this study was to understand the overall prevalence of WAA, the risk of RBC alloimmunization and determine whether RBC selection practices have an impact on alloimmunization. MATERIALS AND METHODS: Records of patients (>1 year of age) with an indirect antibody detection test (IAT) and serologic evidence of WAA over a 10-year-period were included. Eight centres from 5 countries collectively reviewed 1 122 245 patients who had an IAT. RESULTS: Of patients having IAT, 1214 had WAA (0·17%). Transfusion information for 1002 of the patients was available; 631 were transfused after identification of the WAA (63%); of the transfused patients, 390 received prophylactic antigen-matched (PAM) RBCs and 241 did not. Of the 372 patients with WAA who were transfused and had serologic testing 30+ days following transfusion (30-2765 days), 56 developed new RBC alloimmunization (15·1%). Patients who were transfused using a PAM strategy were not protected from new RBC alloimmunization [14·6% (31 of 212 patients) having PAM transfusion approach compared with those not receiving PAM approach (15·6%, 25 of 160 patients, P = 0·8837)]. CONCLUSIONS: The prevalence of WAA in patients having an IAT is low (<1%). A significant portion of patients with WAA form new RBC alloimmunization (15·1%); however, the use of PAM approach for RBC selection was not found to be protective against new alloimmunization.


Assuntos
Anemia Hemolítica Autoimune/epidemiologia , Autoanticorpos/imunologia , Transfusão de Sangue Autóloga/métodos , Adulto , Anemia Hemolítica Autoimune/etiologia , Transfusão de Sangue Autóloga/efeitos adversos , Eritrócitos/imunologia , Feminino , Humanos , Masculino
13.
Transfusion ; 60(1): 175-183, 2020 01.
Artigo em Inglês | MEDLINE | ID: mdl-31850521

RESUMO

BACKGROUND: Rhesus D (RhD) incompatibility is still the most important cause of hemolytic disease of the fetus and newborn (HDFN) worldwide. The aim of this study was to investigate the incidence, causes, and consequences of anti-D alloimmunizations in pregnancy in Iceland, prior to implementation of targeted routine antenatal anti-D prophylaxis (RAADP) in 2018. STUDY DESIGN AND METHODS: This was a nation-wide cohort study of 130 pregnancies affected by RhD alloimmunization in Iceland in the period from 1996 through 2015. Data were collected from transfusion medicine databases, medical records, and the Icelandic Medical Birth Register. RESULTS: Of 130 RhD alloimmunizations, 80 cases (61.5%) represented new RhD immunization in the current pregnancy. Sensitization was discovered in the third trimester in 41 (51.3%) and occurred in the first pregnancy in 14 cases (17.5%). The most likely causative immunization event was the index pregnancy for 45 (56.25%), a previous pregnancy/birth for 26 (32.5%), abortion for 3 (3.75%), and unknown for 6 women (7.5%). Higher anti-D titers were associated with shorter gestational length, cesarean sections, positive direct antiglobulin test (DAT), and severe HDFN. Intrauterine transfusion (IUT) was performed in five pregnancies (3.8%), and 35 of 132 (26.5%) live-born neonates received treatment for HDFN; 32 received phototherapy (24.2%), 13 exchange transfusion (9.8%), and seven simple blood transfusion (5.3%). CONCLUSION: In about half of cases, RhD alloimmunization was caused by the index pregnancy and discovered in the third trimester. Thus, the newly implemented RAADP protocol should be effective in reducing the incidence of RhD immunization in Iceland in the future.


Assuntos
Transfusão de Sangue Intrauterina , Nascido Vivo , Diagnóstico Pré-Natal , Isoimunização Rh , Imunoglobulina rho(D)/sangue , Adulto , Anemia Hemolítica Autoimune/sangue , Anemia Hemolítica Autoimune/diagnóstico , Anemia Hemolítica Autoimune/epidemiologia , Anemia Hemolítica Autoimune/prevenção & controle , Feminino , Humanos , Islândia , Recém-Nascido , Gravidez , Estudos Retrospectivos , Isoimunização Rh/sangue , Isoimunização Rh/diagnóstico , Isoimunização Rh/epidemiologia , Isoimunização Rh/prevenção & controle
14.
Artigo em Chinês | WPRIM | ID: wpr-827187

RESUMO

OBJECTIVE@#To evaluate the characteristics of autoimmune hemolytic anemia caused by salvianolate by antibody detection and clinical index monitoring.@*METHODS@#Micro-column gel anti-human globulin method was used for irregular antibody screening and antibody identification. Salvianolate, sodium creatine phosphate and levocarnitine were used to sensitize red blood cells that were compatible with the patient's plasma, and the RBCs were used to test drug antibody in patient plasma respectively. The patient's clinical examination of hemolysis index and blood transfusion effect were analyed retrospectively.@*RESULTS@#The patients were positive for irregular antibody screening, and there were antoanti-Ce antibodies in serum. The erythrocytes sensitized with salvianolate in the patient's serum were positive, while those sensitized with sodium creatine phosphate and levocarnitine were negative.@*CONCLUSION@#Salvianolate causes drug-induced autoimmune hemolytic anemia in this patient.


Assuntos
Humanos , Anemia Hemolítica Autoimune , Transfusão de Sangue , Eritrócitos , Extratos Vegetais , Estudos Retrospectivos
15.
J Otolaryngol Head Neck Surg ; 48(1): 52, 2019 Oct 22.
Artigo em Inglês | MEDLINE | ID: mdl-31640785

RESUMO

Cold agglutinin disease (CAD) is a rare condition leading to blood agglutination and autoimmune hemolytic anemia. Cutaneous ischemia resulting from CAD in the head and neck is uncommon. Treatment regimens and outcomes vary widely in the literature and no clear protocol exists. This manuscript describes a patient with CAD who developed severe ischemia of the nose that resolved completely without sequellae following a medical regimen of aspirin, low molecular weight heparin, nitroglycerin ointment and hyperbaric oxygen therapy (HBOT). To our knowledge, this is the first reported case where nitroglycerin ointment or HBOT was successfully employed in the treatment of this complication.


Assuntos
Anemia Hemolítica Autoimune/complicações , Oxigenoterapia Hiperbárica , Isquemia/terapia , Nariz/irrigação sanguínea , Administração Tópica , Idoso de 80 Anos ou mais , Anticoagulantes/uso terapêutico , Terapia Combinada , Enoxaparina/administração & dosagem , Feminino , Humanos , Isquemia/tratamento farmacológico , Isquemia/etiologia , Nitroglicerina/administração & dosagem , Pomadas , Vasodilatadores/administração & dosagem
16.
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
17.
Transfusion ; 58(9): 2122-2127, 2018 09.
Artigo em Inglês | MEDLINE | ID: mdl-30179262

RESUMO

BACKGROUND: After hematopoietic stem cell transplantation (HSCT) autoimmune hemolytic anemia (AIHA) is a known and fairly common complication. It is often refractory to conventional therapies including corticosteroids, intravenous immunoglobulin, splenectomy, and the more recently described use of monoclonal antibodies. The high morbidity associated with these severe persistent cases elucidates the gaps in alternative therapies available for treatment. STUDY DESIGN AND METHODS: We described the successful use of abatacept for severe refractory AIHA after HSCT in three patients. RESULTS: Three pediatric patients with refractory AIHA after allogeneic stem cell transplantation were observed to be unresponsive to multitude immunosuppressive therapies, resulting in persistent transfusion dependency. Treatment with abatacept, a fusion protein that inhibits T-cell activation by binding to CD80/CD86 on antigen-presenting cells (APCs), thus blocking the required CD28 interaction between APCs and T cells, resulted in the resolution of hemolysis. CONCLUSION: Abatacept may provide significant clinical benefit in the management of AIHA after HSCT.


Assuntos
Abatacepte/uso terapêutico , Anemia Hemolítica Autoimune/tratamento farmacológico , Transplante de Células-Tronco Hematopoéticas/efeitos adversos , Imunossupressores/uso terapêutico , Adolescente , Anemia Hemolítica Autoimune/etiologia , Anemia Falciforme/terapia , Bacteriemia/complicações , Tipagem e Reações Cruzadas Sanguíneas , Criança , Pré-Escolar , Resistência a Medicamentos , Substituição de Medicamentos , Feminino , Fatores de Troca do Nucleotídeo Guanina/deficiência , Humanos , Síndrome de Job/complicações , Linfo-Histiocitose Hemofagocítica/terapia , Masculino , Staphylococcus aureus Resistente à Meticilina , Pneumonia por Pneumocystis/complicações , Indução de Remissão , Estudos Retrospectivos , Infecções Estafilocócicas/complicações , Viroses/complicações
18.
Medicine (Baltimore) ; 96(49): e9041, 2017 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-29245301

RESUMO

RATIONALE: We report a complicated case of cholestatic hepatitis with suspected autoimmune hemolytic anemia (AIHA) and copper toxicity syndrome after HSCT and donor lymphocyte infusion (DLI). PATIENT CONCERNS: A 19-year-1-month-old girl presented with a history of CML. She underwent matched unrelated donor HSCT and donor lymphocyte infusion subsequently. Three months later, yellowish discoloration of the skin was found, which was accompanied by progressive itchy skin, easy fatigability, insomnia, and dark urine output. After admission, liver function disorders were observed. INTERVENTION: Methylprednisolone was administered for suspected hepatic GVHD. Although abdominal sonography revealed no evidence of biliary tract obstruction and the viral hepatitis survey disclosed unremarkable findings; silymarin and ursodeoxycholic acid were administered to preserve the liver function. In addition, rituximab was prescribed for suspected AIHA. Because hyperbilirubinemia was progressive, mycophenolate and high-dose intravenous immunoglobulin were accordingly administered. As drug-induced liver injury cannot be excluded, all potential unconfirmed causes of drug-related hepatoxicity were discontinued. DIAGNOSIS: In this case, the patient's history of shrimps and chocolate consumption led us to strongly suspect cholestatic hepatitis associated with copper toxicity syndrome. High 24-hour urine copper excretion and low serum zinc levels were also confirmed. Accordingly, D-penicillamine and zinc gluconate were administered. OUTCOMES: She succumbed to progressive hepatic failure and eventual multisystem organ failure 14 months after HSCT. No autopsy was performed. LESSONS: This report described the combined effects of hepatic GVHD, AIHA, drugs, and copper toxicity on liver damage, and demonstrated the potential diagnostic challenges and treatment dilemmas associated with this disease.


Assuntos
Doença Hepática Induzida por Substâncias e Drogas/etiologia , Cobre/toxicidade , Doença Enxerto-Hospedeiro/etiologia , Transplante de Células-Tronco Hematopoéticas/efeitos adversos , Leucemia Mielogênica Crônica BCR-ABL Positiva/complicações , Anemia Hemolítica Autoimune/etiologia , Feminino , Humanos , Adulto Jovem
19.
J Vet Intern Med ; 31(5): 1430-1440, 2017 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-28804921

RESUMO

BACKGROUND: Relapses of immune-mediated hemolytic anemia (IMHA), thrombocytopenia (ITP), or polyarthropathy (IMPA) occur despite normal hematologic and cytologic parameters. Thymidine kinase 1 (TK1), canine C-reactive protein (c-CRP), haptoglobin (HPT), and 25-Hydroxyvitamin-D (25(OH)D) might be adjunct to current monitoring strategies. HYPOTHESIS/OBJECTIVES: Compare serum concentrations of TK1, c-CRP, HPT, and 25(OH)D in dogs with well- and poorly controlled primary IMHA, ITP, or IMPA. ANIMALS: Thirty-eight client-owned dogs. METHODS: Prospective descriptive study. Dogs diagnosed with IMHA, ITP, or IMPA had serum biomarker concentrations measured commercially. Disease control was assessed by hematocrit/PCV and reticulocyte count, platelet count, and synovial fluid cytology for IMHA, ITP, and IMPA, respectively. Statistical analysis performed by Mann-Whitney rank-sum tests and receiver operating characteristic curves. RESULTS: TK1 and c-CRP, but not HPT significantly decreased with well- versus poorly controlled IMHA (P = 0.047, P = 0.028, P = 0.37). C-CRP, but not TK or HPT was significantly lower with well- versus poorly controlled IMPA (P = 0.05, P = 0.28, P = 0.84). Sensitivity and specificity of TK and c-CRP (simultaneously) for detecting dogs with poorly controlled IMHA were 88 and 100%, respectively. Sensitivity and specificity of c-CRP for detecting poorly controlled dogs with IMPA were 13 and 100%, respectively. 92% of dogs were vitamin D insufficient (<100 ng/mL) regardless of disease control. CONCLUSIONS AND CLINICAL IMPORTANCE: Combining TK1 and c-CRP might act markers of disease control in dogs with IMHA. Canine-CRP cannot be recommended as an independent marker of disease control in IMPA. 25(OH)D insufficiency in immune-mediated disorders might benefit from further study to determine if supplementation could improve therapeutic response or reduce disease risk.


Assuntos
Anemia Hemolítica Autoimune/veterinária , Artrite/veterinária , Proteína C-Reativa/análise , Doenças do Cão/sangue , Haptoglobinas/análise , Trombocitopenia/veterinária , Timidina Quinase/sangue , Vitamina D/sangue , Anemia Hemolítica Autoimune/sangue , Animais , Artrite/sangue , Cães , Feminino , Masculino , Estudos Prospectivos , Trombocitopenia/sangue
20.
Tex Heart Inst J ; 43(4): 363-6, 2016 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-27547154

RESUMO

Cold hemagglutinin disease with broad thermal amplitude and high titers presents challenges in treating cardiac-surgery patients. Careful planning is needed to prevent the activation of cold agglutinins and the agglutination of red blood cells as the patient's temperature drops during surgery. We describe our approach to mitigating cold agglutinin formation in a 77-year-old man with severe cold hemagglutinin disease who underwent off-pump coronary artery bypass surgery without the use of preoperative plasmapheresis. This experience shows that the use of an intravascular warming catheter can maintain normothermia and prevent the activation and subsequent formation of cold agglutinins. To our knowledge, this is the first reported use of this technique in a patient with cold hemagglutinin disease. The chief feature in this approach is the use of optimal thermal maintenance-rather than the more usual decrease in cold-agglutinin content by means of therapeutic plasma exchange.


Assuntos
Anemia Hemolítica Autoimune/complicações , Ponte de Artéria Coronária sem Circulação Extracorpórea , Doença da Artéria Coronariana/cirurgia , Hemaglutininas/sangue , Hipertermia Induzida/instrumentação , Dispositivos de Acesso Vascular , Idoso , Anemia Hemolítica Autoimune/sangue , Anemia Hemolítica Autoimune/diagnóstico , Anemia Hemolítica Autoimune/imunologia , Doença da Artéria Coronariana/complicações , Doença da Artéria Coronariana/diagnóstico por imagem , Desenho de Equipamento , Humanos , Hipertermia Induzida/métodos , Masculino , Índice de Gravidade de Doença , Resultado do Tratamento
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