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3.
Blood ; 141(1): 11-21, 2023 01 05.
Artículo en Inglés | MEDLINE | ID: mdl-36054922

RESUMEN

The risk of immune thrombocytopenia (ITP) worsening during pregnancy and neonatal ITP (NITP) have never been prospectively studied. We included 180 pregnant and 168 nonpregnant women with ITP in a prospective, multicenter, observational cohort study. A total of 131 pregnant women with ITP were matched to 131 nonpregnant women with ITP by history of splenectomy, ITP status (no response, response, complete response), and duration. Groups were followed for 15 months. The primary outcome was the first occurrence of ITP worsening defined by a composite end point including bleeding events and/or severe thrombocytopenia (<30 × 109/L) and/or ITP treatment modification. We also studied the recurrence of ITP worsening and the incidence of NITP and risk factors. The first occurrence of ITP worsening did not differ between pregnant and nonpregnant women with ITP (53.4 per 100 person-years [95% confidence interval {CI}, 40.8-69.9] vs 37.1 [95% CI, 27.5-50.0]; hazard ratio {HR}, 1.35 [95% CI, 0.89-2.03], P = .16). Pregnant women with ITP were more likely to have recurrence of severe thrombocytopenia and treatment modification (HR, 2.71 [95% CI, 1.41-5.23], P = .003; HR, 2.01 [95% CI, 1.14-3.57], P = .017, respectively). However, recurrence of severe bleeding events was not different between groups (P = .4). Nineteen (14%) neonates showed NITP <50 × 109/L. By multivariable analysis, NITP was associated with a previous offspring with NITP and maternal platelet count <50 × 109/L within 3 months before delivery (adjusted odds ratio, 5.55 [95% CI, 1.72-17.89], P = .004 and 4.07 [95% CI, 1.41-11.73], P = .009). To conclude, women with ITP do not increase their risk of severe bleeding during pregnancy. NITP is associated with NITP history and the severity of maternal ITP during pregnancy. These results will be useful for counseling women with ITP.


Asunto(s)
Complicaciones Hematológicas del Embarazo , Púrpura Trombocitopénica Idiopática , Trombocitopenia Neonatal Aloinmune , Recién Nacido , Femenino , Humanos , Embarazo , Púrpura Trombocitopénica Idiopática/epidemiología , Púrpura Trombocitopénica Idiopática/terapia , Púrpura Trombocitopénica Idiopática/complicaciones , Estudios de Cohortes , Estudios Prospectivos , Complicaciones Hematológicas del Embarazo/epidemiología , Complicaciones Hematológicas del Embarazo/terapia , Trombocitopenia Neonatal Aloinmune/terapia , Estudios Retrospectivos
8.
Autoimmun Rev ; 16(9): 963-969, 2017 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-28709761

RESUMEN

BACKGROUND: Although peripheral nervous system involvement is common in eosinophilic granulomatosis with polyangiitis (EGPA), central nervous system (CNS) manifestations are poorly described. This study aimed to describe CNS involvement in EGPA. PATIENTS AND METHODS: This retrospective, observational, multicenter study included patients with EGPA and CNS involvement affecting cranial nerves, brain and/or spinal cord. We also undertook a systematic literature review. RESULTS: We analyzed 26 personal cases and 62 previously reported cases. At EGPA diagnosis, asthma was noted in 97%, eosinophilia in 98%, peripheral neuropathy in 55% and cardiac involvement in 41%. 38/71 (54%) were ANCA-positive, with a perinuclear-labeling pattern and/or anti-MPO specificity. CNS was involved in 86% at EGPA diagnosis, preceded EGPA in 2%, and occurred during follow-up in 12% after a median of 24months. Main neurological manifestations were ischemic cerebrovascular lesions in 46 (52%), intracerebral hemorrhage and/or subarachnoid hemorrhage in 21 (24%), loss of visual acuity in 28 (33%) (15 with optic neuritis, 9 with central retinal artery occlusion, 4 with cortical blindness), and cranial nerves palsies in 18 (21%), with 25 patients having ≥1 of these clinical CNS manifestations. Among the 81 patients with assessable neurological responses, 43% had complete responses without sequelae, 43% had partial responses with long-term sequelae and 14% refractory disease. After a mean follow-up of 36months, 11 patients died including 5 from intracerebral hemorrhages. CONCLUSION: EGPA-related CNS manifestations form 4 distinct neurological pictures: ischemic lesions, intracerebral hemorrhages, cranial nerve palsies and loss of visual acuity. Such manifestation should prompt practitioners to consider EGPA in such conditions. Long-term neurological sequelae were common, and intracerebral hemorrhages had the worst prognostic impact.


Asunto(s)
Encéfalo/patología , Eosinofilia/patología , Granulomatosis con Poliangitis/patología , Adulto , Anciano , Asma/diagnóstico por imagen , Asma/patología , Encéfalo/diagnóstico por imagen , Eosinofilia/diagnóstico por imagen , Eosinofilia/tratamiento farmacológico , Femenino , Granulomatosis con Poliangitis/diagnóstico por imagen , Granulomatosis con Poliangitis/tratamiento farmacológico , Humanos , Masculino , Persona de Mediana Edad , Pronóstico , Oclusión de la Arteria Retiniana/diagnóstico por imagen , Estudios Retrospectivos , Resultado del Tratamiento
9.
Autoimmun Rev ; 13(10): 1055-63, 2014 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-25183241

RESUMEN

INTRODUCTION: B-cell depletion with rituximab (RTX) is widely used to treat autoimmune diseases, especially as second-line therapy for immune thrombocytopenia (ITP). The incidence of RTX-induced hypogammaglobulinemia is unknown because of heterogeneous follow-up and confounding factors such as concomitant immunosuppressive treatments in most patients. We describe 3 cases and attempted to determine the incidence of RTX-induced hypogammaglobulinemia by a systematic review of the literature. METHODS: We retrospectively analyzed 189 ITP patients receiving RTX in 3 referral centers in France and conducted a systematic review of 32 studies (results published 2001-2014) reporting the use of RTX for ITP, particularly searching for symptomatic secondary hypogammaglobulinemia. We also searched for case reports of hypogammaglobulinemia after RTX initiation for ITP. RESULTS: Of the 189 patients, 3 showed symptomatic hypogammaglobulinemia more than 2years after RTX infusion (initial immunoglobulin level was normal). All 3 presented recurrent severe infections. In 2, the outcome suggested common variable immunodeficiency. In patient 3, the peripheral blood lacked CD19(+)CD20(+) B cells and the bone-marrow B-cell precursor level was impaired. Among 1245 ITP patients in the literature who received RTX for ITP, gammaglobulin level was monitored before and after RTX initiation for 351 (28%). For 192 (55%), dosages were available and we identified 21 patients with secondary hypogammaglobulinemia, usually not symptomatic, 14 of whom had received concomitant dexamethasone. Finally, we found 4 case reports of ITP and symptomatic hypogammaglobulinemia possibly related to RTX according to the authors. CONCLUSIONS: This large analysis led us to recommend monitoring serum immunoglobulin level before and repeatedly after RTX initiation for ITP. Physicians should be aware of hypogammaglobulinemia as a rare but severe complication of RTX.


Asunto(s)
Agammaglobulinemia/inducido químicamente , Anticuerpos Monoclonales de Origen Murino/efectos adversos , Trombocitopenia/tratamiento farmacológico , Adolescente , Adulto , Linfocitos B/inmunología , Femenino , Francia , Humanos , Hidroxicloroquina/uso terapéutico , Inmunosupresores/efectos adversos , Persona de Mediana Edad , Prednisona/uso terapéutico , Estudios Retrospectivos , Rituximab , Trombocitopenia/complicaciones
10.
Br J Haematol ; 166(6): 929-35, 2014 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-24957165

RESUMEN

In women with pre-existing immune thrombocytopenic purpura (ITP), the effect of pregnancy on the course of the disease is poorly known. We performed a dual-centre retrospective cohort study of 118 pregnancies in 82 women with primary ITP. In early pregnancy, the platelet count was <100 × 10(9) /l in 35·6% of pregnancies. During pregnancy the median platelet count nadir was 66 × 10(9) /l (25th-75th percentile: 42-117), with platelet count <30 × 10(9) /l for 26 pregnancies (22%). In 49% of pregnancies, a significant decrease of the platelet count required treatment at least transiently in preparation for delivery. At the time of delivery, the median platelet count was 110 × 10(9) /l (77-155). Compared to before pregnancy, at 3 months post-partum, only 11% of pregnancies [95% confidence interval (95% CI): 6·8-20·2] showed disease worsening. Previous splenectomy was the only factor significantly associated with ITP worsening after pregnancy (53·9% vs. 10·3%, P < 0·001). For 8·3% of the pregnancies (95% CI: 3·8-15·1), neonatal thrombocytopenia required treatment, especially in case of previous maternal splenectomy (adjusted odds ratio 16·7, 95% CI: 2·61-106). The overall risk of exacerbation of ITP and severe thrombocytopenia during pregnancy is acceptable.


Asunto(s)
Complicaciones Hematológicas del Embarazo/sangre , Púrpura Trombocitopénica Idiopática/sangre , Adulto , Parto Obstétrico , Femenino , Humanos , Recuento de Plaquetas , Hemorragia Posparto/sangre , Hemorragia Posparto/etiología , Embarazo , Complicaciones Hematológicas del Embarazo/terapia , Resultado del Embarazo , Atención Prenatal , Púrpura Trombocitopénica Idiopática/terapia , Estudios Retrospectivos , Trombocitopenia Neonatal Aloinmune/sangre , Trombocitopenia Neonatal Aloinmune/etiología , Adulto Joven
11.
Am J Hematol ; 89(9): E150-5, 2014 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-24847759

RESUMEN

Warm autoimmune hemolytic anemia (wAIHA) is a rare autoimmune disease with poorly known natural history and management remaining mainly empirical. To better describe the characteristics and outcome of wAIHA in adults, we performed a single-center cohort study of patients diagnosed with wAIIHA from 2001 to 2012 in our center. Sixty patients (50% women) were included, the mean age at the time of wAIHA onset was 54 ± 23 years. wAIHA was considered "primary" for 21 patients (35%) and was associated with an underlying disorder in 39 (65%), including mainly lymphoproliferative disorders and systemic lupus. All patients but two needed treatment and received corticosteroids, with an overall initial response rate of 87%. However, 63% of the patients were corticosteroid-dependent and 56% required at least one second-line treatment including mainly rituximab (n = 19). At the time of analysis, after a mean follow-up of 46 months, 28 patients (47%) were in remission and off treatment and 5 (8%) had died. The presence of an underlying lymphoproliferative disorder was associated with reduced response to corticosteroids and increased need for second-line therapy. In conclusion, in the last decade and compared to a previous series from our center, the rate of secondary wAIHA has increased and the use of rituximab has emerged as the preferred second-line treatment and corticosteroid-sparing strategy; the overall mortality has significantly decreased (8 vs. 18%).


Asunto(s)
Anemia Hemolítica Autoinmune/tratamiento farmacológico , Anemia Hemolítica Autoinmune/etiología , Anemia Hemolítica Autoinmune/inmunología , Anticuerpos Monoclonales de Origen Murino/uso terapéutico , Glucocorticoides/uso terapéutico , Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Anemia Hemolítica Autoinmune/mortalidad , Estudios de Cohortes , Supervivencia sin Enfermedad , Transfusión de Eritrocitos , Femenino , Hemoglobinas/análisis , Humanos , Lupus Eritematoso Sistémico/complicaciones , Lupus Eritematoso Sistémico/tratamiento farmacológico , Lupus Eritematoso Sistémico/inmunología , Lupus Eritematoso Sistémico/mortalidad , Trastornos Linfoproliferativos/complicaciones , Trastornos Linfoproliferativos/tratamiento farmacológico , Trastornos Linfoproliferativos/inmunología , Trastornos Linfoproliferativos/mortalidad , Masculino , Persona de Mediana Edad , Rituximab , Esplenectomía , Resultado del Tratamiento , Adulto Joven
12.
Expert Rev Hematol ; 5(2): 229-41, 2012 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-22475291

RESUMEN

Hemolytic anemia is not an exceptional situation in adults. Although establishing the hemolytic mechanism of an anemia is usually rather easy, finding the etiology may be quite difficult as both hereditary (corpuscular) and acquired causes of hemolytic anemia may occur during adulthood. The diagnosis of hemolytic anemia, therefore, requires a multistep procedure taking into account both the patient's and family history, a careful analysis of the blood smear and a direct antiglobulin test. Based on these first data, the diagnostic procedure may then require more specific tests whose indications are discussed in this review.


Asunto(s)
Anemia Hemolítica/diagnóstico , Anemia Hemolítica/etiología , Prueba de Coombs , Efectos Colaterales y Reacciones Adversas Relacionados con Medicamentos , Eritrocitos/enzimología , Eritrocitos Anormales , Humanos , Malaria/complicaciones , Proteínas de la Membrana/genética , Proteínas de la Membrana/metabolismo , Microangiopatías Trombóticas/etiología
13.
Presse Med ; 40(5): 470-85, 2011 May.
Artículo en Francés | MEDLINE | ID: mdl-21295436

RESUMEN

Hemolytic anemia (HA) is not an exceptional situation in adults. While establishing the hemolytic mechanism of an anemia is usually rather easy, finding the etiology may be quite difficult as both some hereditary (corpuscular) and acquired causes of HA may occur during adulthood. The diagnosis of HA therefore requires a multiple step procedure taking into account both patient's and family history, a careful analysis of the blood smear and a direct antiglobulin test. Based on these first data, the diagnosis procedure may then require more specific tests whose indications are discussed in this review.


Asunto(s)
Anemia Hemolítica/diagnóstico , Anemia Hemolítica/etiología , Adulto , Factores de Edad , Árboles de Decisión , Humanos
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