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1.
Int J Hematol ; 115(6): 816-825, 2022 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-35275353

RESUMO

Acquired chronic pure red cell aplasia (PRCA) develops idiopathically or in association with other medical conditions, including T cell large granular lymphocytic leukemia (T-LGLL) and thymoma. T cell dysregulation is considered a cardinal pathogenesis of PRCA, but genetic-phenotypic associations in T cell abnormalities are largely unclear. We evaluated an extended cohort of 90 patients with acquired PRCA, including 26 with idiopathic, 36 with T-LGLL-associated and 15 with thymoma-associated PRCA, for their T cell immuno-phenotypes, clonalities and STAT3 mutations. TCR repertoire skewing of CD8+ T cells was detected in 37.5% of idiopathic, 66.7% of T-LGLL-associated and 25% of thymoma-associated PRCA patients, and restriction to Vß1 was most prominent (41%). Clonalities of TCRß or γ chain and STAT3 mutational status were statistically associated (P = 0.0398), and they were detected in all three subtypes. The overall response rate to cyclosporin A was 73.9%, without significant difference by subtypes nor STAT3 mutational status. The T cell dysregulations, such as TCR repertoire skewing with predominant Vß1 usage, clonality and STAT3 mutations, were frequently found across the subtypes, and the close associations between them suggest that these T cell derangements reflect a common pathophysiological mechanism among these PRCA subtypes.


Assuntos
Leucemia Linfocítica Granular Grande , Aplasia Pura de Série Vermelha , Fator de Transcrição STAT3 , Timoma , Neoplasias do Timo , Linfócitos T CD8-Positivos/patologia , Humanos , Leucemia Linfocítica Granular Grande/genética , Leucemia Linfocítica Granular Grande/imunologia , Leucemia Linfocítica Granular Grande/patologia , Mutação , Receptores de Antígenos de Linfócitos T/genética , Receptores de Antígenos de Linfócitos T/imunologia , Aplasia Pura de Série Vermelha/genética , Aplasia Pura de Série Vermelha/imunologia , Aplasia Pura de Série Vermelha/patologia , Fator de Transcrição STAT3/genética , Fator de Transcrição STAT3/imunologia , Timoma/genética , Timoma/imunologia , Neoplasias do Timo/imunologia
2.
Immunotherapy ; 14(2): 95-99, 2022 02.
Artigo em Inglês | MEDLINE | ID: mdl-34743591

RESUMO

We report on the presentation and outcome of a 28-year-old female who developed red cell aplasia following alemtuzumab therapy for relapsing remitting multiple sclerosis. The patient also developed synchronous immune thrombocytopenia and immune neutropenia, but not aplastic anemia. This patient received high dose steroids, intravenous immunoglobulin (iv.Ig), rituximab, red cell transfusions, vincristine, G-CSF, cyclosporin and mycophenolate to treat the combination of cytopenias over a period of 6 months with subsequent improvement in bone marrow function. While alemtuzumab has several recognized autoimmune complications, little is known about the potential hematological side effects. The combination of red cell aplasia, immune thrombocytic purpura and autoimmune neutropenia has not previously been described in the literature following alemtuzumab immunotherapy and highlights the importance of monthly blood monitoring post alemtuzumab administration.


Lay abstract We report a case of 28-year-old women with relapsing remitting multiple sclerosis who was treated with alemtuzumab and subsequently developed a series of autoimmune complications. Several months after completing her second course of alemtuzumab the patient became breathless and noticed bruising on her legs. On investigation she was found to be anemic and had a low platelet level (which predisposed her to bruising). In addition, her immune system was also impaired meaning she was more prone to developing opportunistic infections. The patient was treated with a variety of different medications and required blood transfusions for several months before she recovered. Despite the multiple complications the patient developed from alemtuzumab her multiple sclerosis remains stable with no new relapses 3 years following treatment.


Assuntos
Alemtuzumab/efeitos adversos , Antineoplásicos Imunológicos/efeitos adversos , Esclerose Múltipla Recidivante-Remitente/tratamento farmacológico , Neutropenia/induzido quimicamente , Púrpura Trombocitopênica Idiopática/induzido quimicamente , Aplasia Pura de Série Vermelha/induzido quimicamente , Corticosteroides/uso terapêutico , Adulto , Alemtuzumab/imunologia , Alemtuzumab/uso terapêutico , Antineoplásicos Imunológicos/imunologia , Antineoplásicos Imunológicos/uso terapêutico , Ciclosporina/uso terapêutico , Feminino , Fator Estimulador de Colônias de Granulócitos/uso terapêutico , Humanos , Imunoglobulinas Intravenosas/uso terapêutico , Fatores Imunológicos/uso terapêutico , Esclerose Múltipla Recidivante-Remitente/imunologia , Ácido Micofenólico/uso terapêutico , Neutropenia/tratamento farmacológico , Neutropenia/imunologia , Púrpura Trombocitopênica Idiopática/tratamento farmacológico , Púrpura Trombocitopênica Idiopática/imunologia , Aplasia Pura de Série Vermelha/tratamento farmacológico , Aplasia Pura de Série Vermelha/imunologia , Rituximab/uso terapêutico , Resultado do Tratamento , Vincristina/uso terapêutico
3.
Eur J Haematol ; 107(3): 324-332, 2021 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-34022082

RESUMO

OBJECTIVE: ABO mismatch between donor and recipient occurs in 40% of allogeneic hematopoietic stem cell transplantations (HCT). Different strategies have been described to reduce isohemagglutinins (IHA) before HCT. We describe the effect of selective ABO immunoadsorption (ABO IA) on erythrocyte transfusion rate and the development of post-transplant pure red cell aplasia (ptPRCA). METHODS: 63 patients with major ABO incompatibility were retrospectively analyzed. Nine patients with major ABO incompatibility and high-IHA titer were treated by ABO IA before HCT. We analyzed the need for transfusion and the occurrence of ptPRCA. We compared the outcome with patients treated by other methods to reduce IHA. RESULTS: In all nine patients treated by ABO IA, IHA decreased in a median four times. PtPRCA occurred in one patient. The median number of transfusions was 8 (range: 0-36) between d0 and d100. In 25 patients with high-IHA titer without treatment or treated by other methods to reduce IHA, the need for transfusions was comparable. No difference in the incidence of ptPRCA was observed. CONCLUSIONS: Selective ABO IA is a feasible, safe, and effective method to reduce IHA before HCT in major ABO incompatibility. No effect on transfusion rate or ptPRCA compared to other strategies could be observed.


Assuntos
Transplante de Células-Tronco Hematopoéticas/métodos , Leucemia/terapia , Síndromes Mielodisplásicas/terapia , Transtornos Mieloproliferativos/terapia , Plasmaferese/métodos , Aplasia Pura de Série Vermelha/prevenção & controle , Reação Transfusional/prevenção & controle , Sistema ABO de Grupos Sanguíneos/imunologia , Incompatibilidade de Grupos Sanguíneos/imunologia , Incompatibilidade de Grupos Sanguíneos/mortalidade , Incompatibilidade de Grupos Sanguíneos/terapia , Transfusão de Eritrócitos/efeitos adversos , Feminino , Transplante de Células-Tronco Hematopoéticas/efeitos adversos , Humanos , Leucemia/imunologia , Leucemia/mortalidade , Masculino , Pessoa de Meia-Idade , Síndromes Mielodisplásicas/imunologia , Síndromes Mielodisplásicas/mortalidade , Transtornos Mieloproliferativos/imunologia , Transtornos Mieloproliferativos/mortalidade , Aplasia Pura de Série Vermelha/etiologia , Aplasia Pura de Série Vermelha/imunologia , Aplasia Pura de Série Vermelha/mortalidade , Estudos Retrospectivos , Análise de Sobrevida , Doadores de Tecidos , Reação Transfusional/etiologia , Reação Transfusional/imunologia , Reação Transfusional/mortalidade , Transplante Homólogo , Resultado do Tratamento
5.
Sci Rep ; 11(1): 1491, 2021 01 15.
Artigo em Inglês | MEDLINE | ID: mdl-33452310

RESUMO

Recombinant human erythropoietin (rHuEPO) is a biopharmaceutical drug given to patients who have a low hemoglobin related to chronic kidney disease, cancer or anemia. However, some patients repeatedly receiving rHuEPO develop anti-rHuEPO neutralizing antibodies leading to the development of pure red cell aplasia (PRCA). The immunogenic antibody response activated by rHuEPO is believed to be triggered by T-cells recognizing EPO epitopes bound to MHC molecules displayed on the cell surface of APCs. Previous studies have reported an association between the development of anti-rHuEpo-associated PRCA and the HLA-DRB1*09 gene, which is reported to be entrenched in the Thai population. In this study, we used computational design to screen for immunogenic hotspots recognized by HLA-DRB1*09, and predicted seventeen mutants having anywhere between one through four mutations that reduce affinity for the allele, without disrupting the structural integrity and bioactivity. Five out of seventeen mutants were less immunogenic in vitro while retaining similar or slightly reduced bioactivity than rHuEPO. These engineered proteins could be the potential candidates to treat patients who are rHuEpo-dependent and express the HLA-DRB1*09 allele.


Assuntos
Eritropoetina/imunologia , Eritropoetina/metabolismo , Alelos , Anemia/tratamento farmacológico , Formação de Anticorpos/genética , Técnicas de Cultura de Células , Linhagem Celular , Eritropoetina/genética , Humanos , Complexo Principal de Histocompatibilidade/genética , Engenharia de Proteínas/métodos , Proteínas Recombinantes/genética , Proteínas Recombinantes/imunologia , Proteínas Recombinantes/metabolismo , Aplasia Pura de Série Vermelha/tratamento farmacológico , Aplasia Pura de Série Vermelha/imunologia , Aplasia Pura de Série Vermelha/fisiopatologia , Diálise Renal
7.
Blood Cells Mol Dis ; 88: 102464, 2021 05.
Artigo em Inglês | MEDLINE | ID: mdl-32653327

RESUMO

Pure red cell aplasia is a known complication after ABO incompatible stem cell transplant. Due to rarity of disease, no established treatment guidelines are available for PRCA. Daratumumab is a monoclonal antibody against CD38 expressed by plasma cells. In this report we present our experience of successfully managing a patient of post-transplant PRCA with daratumumab. Our patient had failed multiple lines of therapy prior to receiving daratumumab. Response was seen after the 3rd weekly dose of daratumumab.


Assuntos
Sistema ABO de Grupos Sanguíneos/imunologia , Anticorpos Monoclonais/uso terapêutico , Antineoplásicos Imunológicos/uso terapêutico , Incompatibilidade de Grupos Sanguíneos/complicações , Transplante de Células-Tronco Hematopoéticas/efeitos adversos , Aplasia Pura de Série Vermelha/tratamento farmacológico , ADP-Ribosil Ciclase 1/antagonistas & inibidores , ADP-Ribosil Ciclase 1/imunologia , Adolescente , Anemia Aplástica/imunologia , Anemia Aplástica/terapia , Incompatibilidade de Grupos Sanguíneos/imunologia , Feminino , Humanos , Aplasia Pura de Série Vermelha/etiologia , Aplasia Pura de Série Vermelha/imunologia , Transplante Homólogo/efeitos adversos
8.
BMC Nephrol ; 21(1): 275, 2020 07 14.
Artigo em Inglês | MEDLINE | ID: mdl-32664894

RESUMO

BACKGROUND: Erythropoietin-stimulating agents (ESAs) are used to treat anemia in patients with chronic kidney disease, enabling maintenance of stable hemoglobin levels and eliminating the need for multiple transfusions. Epoetin-beta pegol (C.E.R.A.) is a continuous erythropoietin receptor activator created by integrating a large methoxy-polyethylene-glycol-polymer chain into the erythropoietin molecule, which provides it with a longer half-life. On rare occasions, cases of antibody-mediated pure red cell aplasia (PRCA) related to ESAs are reported. They are characterized by abrupt onset of severe transfusion-dependent anemia, despite ESA therapy. We herein report a case of antibody-mediated PRCA in a dialysis patient receiving C.E.R.A. CASE PRESENTATION: A 44-year-old man with end-stage renal failure had been receiving continuous ambulatory peritoneal dialysis for 2 years. C.E.R.A. was administered subcutaneously as a sole ESA once a month at the hospital since 4 years ago for the treatment of renal anemia and his hemoglobin level was well controlled at 12 g/dl. From 10 months before diagnosis, however, his hemoglobin level suddenly declined, necessitating frequent transfusions. Based on the results of a bone marrow examination and detection of anti-C.E.R.A. antibodies, the patient was diagnosed with antibody-mediated PRCA. After successful elimination of the antibodies using oral prednisolone plus cyclosporine, the patient was re-administrated C.E.R.A. intravenously, as there are few reports of antibody-mediated PRCA related to ESA using that administration route. He responded to the C.E.R.A., and his anemia dramatically improved, eliminating the need for blood transfusions. CONCLUSIONS: This is the first reported case of recovery from an antibody-mediated PRCA with C.E.R.A. after its re-administration following a reversal of the antibody. It has been suggested that the additional large pegylation chain makes C.E.R.A. less likely to trigger antibody generation than other ESAs. Following successful treatment of antibody-mediated PRCA using immunosuppressive therapy, C.E.R.A. can be re-administered intravenously to treat renal anemia.


Assuntos
Anemia/tratamento farmacológico , Anticorpos/imunologia , Eritropoetina/imunologia , Hematínicos/imunologia , Falência Renal Crônica/terapia , Aplasia Pura de Série Vermelha/imunologia , Adulto , Anemia/etiologia , Ciclosporina/uso terapêutico , Eritropoetina/administração & dosagem , Glucocorticoides/uso terapêutico , Hematínicos/administração & dosagem , Humanos , Imunossupressores/uso terapêutico , Injeções Intravenosas , Injeções Subcutâneas , Falência Renal Crônica/complicações , Masculino , Polietilenoglicóis/administração & dosagem , Prednisolona/uso terapêutico , Aplasia Pura de Série Vermelha/tratamento farmacológico , Diálise Renal
9.
Transplant Proc ; 52(8): 2530-2532, 2020 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-32276841

RESUMO

Acquired pure red cell aplasia (PRCA) is characterized by severe normocytic (rarely macrocytic) and normochromic anemia, a low reticulocytes count in peripheral blood, and near absence of erythroid precursors in the bone marrow, with a normal level of erythropoietin. We describe a case of the kidney transplant recipient, diagnosed with PRCA induced with parvovirus B19 infection. Our case demonstrates that although this complication is rare, it should be considered in a differential diagnosis of anemia diagnostics in immunocompromised patients. In our case reduced immune response resulted from post-transplant immunosuppressive therapy. In our patient, apart from infection by parvovirus B19, graft dysfunction due to polyomavirus BK virus infection was also detected together with histologic and serologic features of antibody-mediated renal graft rejection. Considering the entire clinical picture, intravenous immunoglobulin therapy (IVIg) was successfully introduced.


Assuntos
Rejeição de Enxerto/imunologia , Hospedeiro Imunocomprometido/imunologia , Transplante de Rim/efeitos adversos , Complicações Pós-Operatórias/imunologia , Aplasia Pura de Série Vermelha/imunologia , Aplasia Pura de Série Vermelha/virologia , Anticorpos Antivirais/imunologia , Eritema Infeccioso/imunologia , Eritema Infeccioso/virologia , Eritropoetina , Feminino , Rejeição de Enxerto/tratamento farmacológico , Rejeição de Enxerto/virologia , Humanos , Imunoglobulinas Intravenosas/administração & dosagem , Masculino , Pessoa de Meia-Idade , Parvovirus B19 Humano/imunologia , Complicações Pós-Operatórias/tratamento farmacológico , Complicações Pós-Operatórias/virologia , Aplasia Pura de Série Vermelha/tratamento farmacológico
10.
Clin Rheumatol ; 39(7): 2227-2234, 2020 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-32277368

RESUMO

The thymus plays an integral role in immune system regulation, modulating the development, diversity, and selection of T lymphocytes, a critical feature for the prevention of T cell-mediated autoimmunity. Thymoma is a rare tumor of the thymus. Autoimmune diseases and paraneoplastic syndromes such as myasthenia gravis, pure red blood cell aplasia, and systemic lupus erythematosus, although relatively uncommon, have been described in association with thymomas. Rare cases of post-thymectomy autoimmune related diseases, including systemic lupus erythematosus and pure red cell aplasia, have been reported in the literature. Here, we present the case of a 65-year-old male who developed systemic lupus erythematosus 2 years after thymectomy in the setting of thymoma-associated pure red cell aplasia.


Assuntos
Lúpus Eritematoso Sistêmico/etiologia , Aplasia Pura de Série Vermelha/etiologia , Linfócitos T/imunologia , Timo/imunologia , Idoso , Autoimunidade , Humanos , Lúpus Eritematoso Sistêmico/imunologia , Masculino , Radiografia , Aplasia Pura de Série Vermelha/imunologia , Linfócitos T/patologia , Timectomia/efeitos adversos , Timoma/patologia , Timoma/cirurgia , Neoplasias do Timo/patologia , Neoplasias do Timo/cirurgia , Tomografia Computadorizada por Raios X
11.
Ann Hematol ; 99(3): 443-449, 2020 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-31970447

RESUMO

This current study retrospectively analyzed the clinical characteristics of 69 adult patients with acquired pure red cell aplasia (PRCA) including 40 elderly and 29 non-elderly patients from September 2009 to June 2019. The remission induction therapy regimens included cyclosporine A (CsA), corticosteroids (CS), or other immunosuppressive agents. The overall response rate was 55% (22/40) in the elderly group compared with 75.9% (22/29) in non-elderly patients (P = 0.075). In elderly patients, the best remission was achieved in the group treated with CsA than those treated with CS or other immunosuppressive agents (83.3% vs 26.7% vs 42.9%%, P = 0.004). However, outcomes of remission were similar among different treatment groups (P = 0.458) in non-elderly patients. CS induced a higher response rate in the non-elderly than that in the elderly (88.9% vs 26.7%, P = 0.009). By univariate and multivariate analysis, the clinical efficacy of elderly patients with acquired PRCA was closely associated with an induction regimen of CsA (P = 0.009; P = 0.017). In conclusion, CsA might produce higher response rate than CS and other drugs in elderly patients with acquired PRCA.


Assuntos
Ciclosporina/administração & dosagem , Terapia de Imunossupressão , Aplasia Pura de Série Vermelha/tratamento farmacológico , Indução de Remissão , Adulto , Fatores Etários , Idoso , Idoso de 80 Anos ou mais , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Aplasia Pura de Série Vermelha/imunologia , Aplasia Pura de Série Vermelha/patologia
14.
Exp Clin Transplant ; 17(Suppl 1): 195-197, 2019 01.
Artigo em Inglês | MEDLINE | ID: mdl-30777553

RESUMO

Parvovirus B19 is a single-stranded DNA virus that typically has an affinity for erythroid progenitor cells in bone marrow and leads to pure red cell aplasia. This is a common pathogen in humans, and the expression of the infection depends on the host's hematologic and immunologic status. Here, we report a female patient who developed severe and persistent anemia after kidney transplant while being on immunosuppressive therapy. The parvovirus B19 immunoglobulin M test was positive, and the virus was detected by polymerase chain reaction as parvovirus B19 (23.5 million copies/mL) in the blood sample. Bone marrow examination revealed giant pronormoblasts. She responded well to intravenous immunoglobulin without adverse event. Hemoglobin levels gradually increased, and normal levels were achieved at 3 months posttreatment. Although her renal function did not deteriorate, severe anemia (with hemoglobin level 5 g/dL) recurred 3 times during 12 months posttransplant.


Assuntos
Imunoglobulinas Intravenosas/administração & dosagem , Fatores Imunológicos/administração & dosagem , Transplante de Rim/efeitos adversos , Infecções Oportunistas/tratamento farmacológico , Infecções por Parvoviridae/tratamento farmacológico , Parvovirus B19 Humano/efeitos dos fármacos , Aplasia Pura de Série Vermelha/tratamento farmacológico , Adulto , Esquema de Medicação , Feminino , Humanos , Hospedeiro Imunocomprometido , Imunossupressores/administração & dosagem , Imunossupressores/efeitos adversos , Infecções Oportunistas/diagnóstico , Infecções Oportunistas/imunologia , Infecções Oportunistas/virologia , Infecções por Parvoviridae/diagnóstico , Infecções por Parvoviridae/imunologia , Infecções por Parvoviridae/virologia , Parvovirus B19 Humano/imunologia , Parvovirus B19 Humano/patogenicidade , Recidiva , Aplasia Pura de Série Vermelha/diagnóstico , Aplasia Pura de Série Vermelha/imunologia , Aplasia Pura de Série Vermelha/virologia , Resultado do Tratamento , Ativação Viral
15.
Int Immunopharmacol ; 63: 14-18, 2018 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-30056258

RESUMO

We herein report a unique case of type B2 thymoma-associated myasthenia gravis which was ameliorated by immunosuppressive therapy in combination with chemotherapy. However, the patient subsequently developed pure red cell aplasia and marked lymphocytosis after additional chemotherapy aimed at improvement of thymoma. While a separate immunosuppressive regimen was effective for anemia, lymphocytosis was exacerbated. The biopsied thymoma specimen contained CD4+, CD8+, and CD4+/CD8+ T cells, some of which were CD3-, suggesting immature thymocytes. In contrast, majority of the peripheral lymphocytes were polyclonal CD3+/CD8+/T cell receptor (TCR)αß+ T cells. The CD4/CD8 ratio in the present patient might be affected by immunosuppressive agents, resulting in CD8+ T cell expansion associated with pure red cell aplasia. Although several cases of thymoma accompanied by peripheral T cell lymphocytosis were reported, marked CD8+ T cell proliferation is extremely rare.


Assuntos
Linfócitos T CD8-Positivos/imunologia , Linfocitose/imunologia , Miastenia Gravis/imunologia , Aplasia Pura de Série Vermelha/imunologia , Timoma/imunologia , Neoplasias do Timo/imunologia , Adulto , Protocolos de Quimioterapia Combinada Antineoplásica/efeitos adversos , Relação CD4-CD8 , Feminino , Humanos , Terapia de Imunossupressão , Imunossupressores/efeitos adversos , Miastenia Gravis/terapia , Aplasia Pura de Série Vermelha/terapia , Timoma/terapia , Neoplasias do Timo/terapia
16.
Ann Hematol ; 97(11): 2047-2054, 2018 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-29982851

RESUMO

Acquired pure red cell aplasia (aPRCA) is a kind of anemia characterized by severe reticulocytopenia and obvious bone marrow erythroblastic cells decreased. Some patients are refractory or intolerant to the first-line therapy (cyclosporine A with/without steroids). The effects of the second-line therapy are not satisfactory and sometimes not available. In this study, we analyzed the efficacy and side effect of sirolimus on refractory/relapsed aPRCA and investigated the possible mechanism of sirolimus on immune regulation. Twenty-one patients with refractory/relapsed aPRCA were enrolled in this study and were administered with sirolimus. Totally, 76.2% of patients responded to the sirolimus with 42.9% complete response during the experimental period. The median time for reaction was 4 months. Side effects were tolerable including infections; mild oral mucositis; sinus tachycardia, the increase of creatinine, transaminase, triglyceride, or cholesterol; and thrombocytopenia. Most patients stayed in remission or remained stable during the follow-up period. Early drug withdrawal may lead to quick relapse. Compared with healthy control, Treg levels in patients with aPRCA reduced significantly before sirolimus but recovered after successful treatment. Level of Treg cells correlated with hemoglobin level after effective sirolimus treatment. Thus, sirolimus was effective and tolerable for refractory/relapsed aPRCA. Effective sirolimus treatment may lead to the upregulation of Treg cells which may partly explain the underlying mechanism.


Assuntos
Aplasia Pura de Série Vermelha/tratamento farmacológico , Aplasia Pura de Série Vermelha/imunologia , Sirolimo/administração & dosagem , Linfócitos T Reguladores/imunologia , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Feminino , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Aplasia Pura de Série Vermelha/patologia , Indução de Remissão , Sirolimo/efeitos adversos , Linfócitos T Reguladores/patologia
17.
Intern Med ; 57(21): 3175-3177, 2018 Nov 01.
Artigo em Inglês | MEDLINE | ID: mdl-29877260

RESUMO

We herein report a case of long-lasting pure red cell aplasia (PRCA) after major ABO-incompatible allogeneic stem cell transplantation (SCT) for acute lymphoblastic leukemia. The patient needed red blood cell (RBC) transfusion every week after SCT. On day 236, he was diagnosed with odontogenic infection, and the serum levels of Interleukin (IL)-6 were elevated to 12.1 pg/mL. After that, the numbers of reticulocyte rapidly began to increase, and RBC support was not needed from day 251. No standard care for PRCA following SCT has been established. The IL-6 elevation caused by the odontogenic infection therefore appears to have been affected by the improvement in PRCA.


Assuntos
Infecções Bacterianas/complicações , Transplante de Células-Tronco Hematopoéticas , Interleucina-6/sangue , Leucemia-Linfoma Linfoblástico de Células Precursoras/complicações , Leucemia-Linfoma Linfoblástico de Células Precursoras/terapia , Aplasia Pura de Série Vermelha/sangue , Doenças Dentárias/complicações , Sistema ABO de Grupos Sanguíneos/imunologia , Antibacterianos/uso terapêutico , Infecções Bacterianas/tratamento farmacológico , Infecções Bacterianas/imunologia , Incompatibilidade de Grupos Sanguíneos , Transplante de Células-Tronco Hematopoéticas/efeitos adversos , Humanos , Masculino , Pessoa de Meia-Idade , Aplasia Pura de Série Vermelha/etiologia , Aplasia Pura de Série Vermelha/imunologia , Aplasia Pura de Série Vermelha/terapia , Doenças Dentárias/tratamento farmacológico , Doenças Dentárias/imunologia
19.
Blood Transfus ; 16(4): 397-404, 2018 07.
Artigo em Inglês | MEDLINE | ID: mdl-28488966

RESUMO

BACKGROUND: Acute and delayed haemolysis, alloimmunisation and pure red cell aplasia (PRCA) are potential complications after ABO incompatible haematopoietic stem cell transplantation (HSCT). The aims of this study were to investigate acute and delayed red blood cell (RBC) antibody-associated complications, including haemolysis, PRCA and alloimmunisation in major and bidirectional ABO incompatible HSCT. MATERIALS AND METHODS: We retrospectively examined the transplant courses of 36 recipients of bone marrow or peripheral blood stem cells from ABO incompatible donors and evaluated the current practice of performing plasmapheresis in patients with higher isoagglutinin titres. We investigated the role of ABO incompatibility in haematopoietic recovery, transfusion requirements, alloimmunisation and PRCA. RESULTS: Laboratory signs of acute haemolysis were noted in five (14%) patients, one (3%) of whom had clinically overt haemolysis. Patients with haemolysis had IgM titres ≥1:8 and received >16 mL of RBC in the HSCT. In patients with higher titres, plasmapheresis performed prior to the transplant prevented acute haemolysis. Delayed haemolysis was not recorded in the follow up. Haematopoietic recovery and transfusion requirements did not differ notably between patients with and without haemolysis. De novo RBC antibodies were detected in two (5.5%) patients after HSCT, and PRCA was noted in one (3%) patient. DISCUSSION: Carried out with adequate graft processing, plasmapheresis and blood component support, haemolysis is not a common complication after HSCT. Our results confirm that the occurrence of haemolysis depends on larger RBC volumes and higher isoagglutinin titres. Despite the reduction of patients' isoagglutinin titres by plasmapheresis, we still noted a critical combination for the development of laboratory signs of haemolysis (IgM titre ≥1:8 and RBC volume >16 mL). De novo immunisation to RBC antigens and PRCA are rare events following ABO incompatible HSCT.


Assuntos
Sistema ABO de Grupos Sanguíneos , Transplante de Células-Tronco Hematopoéticas/efeitos adversos , Isoanticorpos , Aplasia Pura de Série Vermelha , Adolescente , Adulto , Aloenxertos , Criança , Pré-Escolar , Feminino , Hemólise , Humanos , Isoanticorpos/sangue , Isoanticorpos/imunologia , Masculino , Pessoa de Meia-Idade , Aplasia Pura de Série Vermelha/sangue , Aplasia Pura de Série Vermelha/etiologia , Aplasia Pura de Série Vermelha/imunologia
20.
Am J Hematol ; 93(3): 424-429, 2018 03.
Artigo em Inglês | MEDLINE | ID: mdl-29247540

RESUMO

The aim of the study was to assess the efficacy and safety of rituximab (RTX) for treating systemic lupus erythematosus (SLE)-associated immune cytopenias. This multicenter retrospective cohort study of adults from French referral centers and networks for adult immune cytopenias and SLE involved patients ≥18 years old with a definite diagnosis of SLE treated with RTX specifically for SLE-associated immune cytopenia from 2005 to 2015. Response assessment was based on standard definitions. In total, 71 patients, 61 women (85.9%), with median age 36 years [interquartile range 31-48], were included. The median duration of SLE at the time of the first RTX administration was 6.1 years [2.6-11.6] and the reason for using RTX was immune thrombocytopenia (ITP) for 44 patients (62.0%), autoimmune hemolytic anemia (AIHA) for 16 (22.5%), Evans syndrome for 10 (14.1%), and pure red cell aplasia for one patient. Before receiving RTX, patients had received a mean of 3.1 ± 1.3 treatments that included corticosteroids (100%), and hydroxychloroquine (88.5%). The overall initial response rate to RTX was 86% (91% with ITP, 87.5% with AIHA, and 60% with Evans syndrome), including 60.5% with complete response. Median follow-up after the first injection of RTX was 26.4 months [14.3-71.2]. Among 61 initial responders, relapse occurred in 24 (39.3%); for 18, RTX retreatment was successful in 16 (88.8%). Severe infections occurred after RTX in three patients, with no fatal outcome. No cases of RTX-induced neutropenia were observed. In conclusion, RTX seems effective and relatively safe for treating SLE-associated immune cytopenias.


Assuntos
Anemia Hemolítica Autoimune/tratamento farmacológico , Imunossupressores/uso terapêutico , Lúpus Eritematoso Sistêmico/complicações , Púrpura Trombocitopênica Idiopática/tratamento farmacológico , Rituximab/uso terapêutico , Adulto , Anemia Hemolítica Autoimune/etiologia , Anemia Hemolítica Autoimune/imunologia , Suscetibilidade a Doenças , Intervalo Livre de Doença , Avaliação de Medicamentos , Quimioterapia Combinada , Feminino , Febre/induzido quimicamente , Seguimentos , Humanos , Imunossupressores/efeitos adversos , Infecções/etiologia , Estimativa de Kaplan-Meier , Lúpus Eritematoso Sistêmico/tratamento farmacológico , Lúpus Eritematoso Sistêmico/imunologia , Masculino , Pessoa de Meia-Idade , Púrpura Trombocitopênica Idiopática/etiologia , Púrpura Trombocitopênica Idiopática/imunologia , Aplasia Pura de Série Vermelha/tratamento farmacológico , Aplasia Pura de Série Vermelha/etiologia , Aplasia Pura de Série Vermelha/imunologia , Estudos Retrospectivos , Rituximab/efeitos adversos , Trombocitopenia/tratamento farmacológico , Trombocitopenia/etiologia , Trombocitopenia/imunologia , Resultado do Tratamento
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