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2.
Acta Haematol ; 142(4): 239-243, 2019.
Artículo en Inglés | MEDLINE | ID: mdl-31132762

RESUMEN

Acquired amegakaryocytic thrombocytopenia (AAMT) is a rare disease that causes severe bleeding. The pathogenesis and treatment of AAMT have not yet been defined. We report the case of a 60-year-old woman diagnosed with AAMT, who presented with severe thrombocytopenia, gastroin-testinal bleeding, and significantly reduced bone marrow megakaryocytes. The patient was treated with methylprednisolone, cyclosporin, and intravenous immunoglobulin. After 2 weeks of treatment, her platelet count started to increase, and her bone marrow megakaryocyte count had normalized 3 months after diagnosis. At the time of diagnosis, the patient was seropositive for anti-c-mpl antibody but was seen to be seronegative once the platelet count recovered. In contrast, anti-c-mpl antibodies were not detected in the serum of 3 patients with idiopathic thrombocytopenic purpura. This case study suggests that anti-c-mpl antibody plays an important role in the development of AAMT, and that intensive immunosuppressive treatment is required for autoantibody clearance and recovery of megakaryocyte count.


Asunto(s)
Autoanticuerpos/sangre , Enfermedades de la Médula Ósea , Ciclosporina/administración & dosificación , Inmunoglobulinas Intravenosas/administración & dosificación , Metilprednisolona/administración & dosificación , Púrpura Trombocitopénica , Receptores de Trombopoyetina , Células de la Médula Ósea/metabolismo , Enfermedades de la Médula Ósea/sangre , Enfermedades de la Médula Ósea/diagnóstico , Enfermedades de la Médula Ósea/tratamiento farmacológico , Femenino , Hemorragia Gastrointestinal/sangre , Hemorragia Gastrointestinal/diagnóstico , Hemorragia Gastrointestinal/tratamiento farmacológico , Humanos , Megacariocitos/metabolismo , Persona de Mediana Edad , Recuento de Plaquetas , Púrpura Trombocitopénica/sangre , Púrpura Trombocitopénica/diagnóstico , Púrpura Trombocitopénica/tratamiento farmacológico
3.
Clin J Am Soc Nephrol ; 13(2): 300-317, 2018 02 07.
Artículo en Inglés | MEDLINE | ID: mdl-29042465

RESUMEN

Thrombotic microangiopathy can manifest in a diverse range of diseases and is characterized by thrombocytopenia, microangiopathic hemolytic anemia, and organ injury, including AKI. It can be associated with significant morbidity and mortality, but a systematic approach to investigation and prompt initiation of supportive management and, in some cases, effective specific treatment can result in good outcomes. This review considers the classification, pathology, epidemiology, characteristics, and pathogenesis of the thrombotic microangiopathies, and outlines a pragmatic approach to diagnosis and management.


Asunto(s)
Lesión Renal Aguda , Síndrome Hemolítico-Urémico , Púrpura Trombocitopénica , Lesión Renal Aguda/diagnóstico , Lesión Renal Aguda/epidemiología , Lesión Renal Aguda/terapia , Animales , Inactivadores del Complemento/uso terapéutico , Progresión de la Enfermedad , Síndrome Hemolítico-Urémico/diagnóstico , Síndrome Hemolítico-Urémico/epidemiología , Síndrome Hemolítico-Urémico/terapia , Humanos , Intercambio Plasmático , Pronóstico , Púrpura Trombocitopénica/diagnóstico , Púrpura Trombocitopénica/epidemiología , Púrpura Trombocitopénica/terapia , Medición de Riesgo , Factores de Riesgo
4.
World J Gastroenterol ; 23(35): 6540-6545, 2017 Sep 21.
Artículo en Inglés | MEDLINE | ID: mdl-29085203

RESUMEN

We report the first case of a patient with hepatitis C virus (HCV) infection and idiopathic thrombocytopenic purpura (ITP), who later developed acquired amegakaryocytic thrombocytopenia (AAMT), with autoantibodies to the thrombopoietin (TPO) receptor (c-Mpl). A 64-year-old woman, with chronic hepatitis C, developed severe thrombocytopenia and was diagnosed with ITP. She died of liver failure. Autopsy revealed cirrhosis and liver carcinoma. In the bone marrow, a marked reduction in the number of megakaryocytes was observed, while other cell lineages were preserved. Therefore, she was diagnosed with AAMT. Additionally, autoantibodies to c-Mpl were detected in her serum. Autoantibodies to c-Mpl are one of the causes of AAMT, acting through inhibition of TPO function, megakaryocytic maturation, and platelet formation. HCV infection induces several autoantibodies. HCV infection might also induce autoantibodies to c-Mpl, resulting in the development of AAMT. This mechanism may be one of the causes of thrombocytopenia in patients with HCV infection.


Asunto(s)
Autoanticuerpos/sangre , Enfermedades de la Médula Ósea/diagnóstico , Hepatitis C Crónica/inmunología , Fallo Hepático/sangre , Púrpura Trombocitopénica Idiopática/diagnóstico , Púrpura Trombocitopénica/diagnóstico , Autoanticuerpos/inmunología , Médula Ósea/patología , Células de la Médula Ósea/patología , Enfermedades de la Médula Ósea/sangre , Enfermedades de la Médula Ósea/inmunología , Enfermedades de la Médula Ósea/patología , Diagnóstico Diferencial , Resultado Fatal , Femenino , Hepacivirus/aislamiento & purificación , Hepatitis C Crónica/sangre , Hepatitis C Crónica/virología , Humanos , Hígado/inmunología , Hígado/patología , Fallo Hepático/etiología , Fallo Hepático/inmunología , Fallo Hepático/patología , Megacariocitos/patología , Persona de Mediana Edad , Púrpura Trombocitopénica/sangre , Púrpura Trombocitopénica/inmunología , Púrpura Trombocitopénica/patología , Púrpura Trombocitopénica Idiopática/sangre , Receptores de Trombopoyetina/inmunología , Trombopoyetina/metabolismo
5.
Pan Afr Med J ; 26: 32, 2017.
Artículo en Francés | MEDLINE | ID: mdl-28451010

RESUMEN

Acquired amegakaryocytic thrombocytopenic purpura is a very rare condition characterized by severe thrombocytopenia linked to the reduction or disappearance of megakaryocytes in the bone marrow. It may be primary idiopathic or secondary to many pathological conditions including hematologic disorders. We report the case of a 24-year-old patient admitted for haemorrhagic syndrome caused by immunological thrombocytopenic purpura. The diagnosis was acquired amegakaryocytosis after the failure of corticotherapy and the performance of myelography. The patient was treated with ciclosporin with rapid progression to acute myeloblastic leukemia. The progression of acquired amegakaryocytosis to acute leukemia is reported but it is generally not so rapid and above all it is preceded by myelodysplastic syndrome or medullary aplasia. This study highlights the importance of a close follow-up of these pathologies with a benign-like appearance.


Asunto(s)
Leucemia Mieloide Aguda/diagnóstico , Megacariocitos/patología , Púrpura Trombocitopénica/diagnóstico , Ciclosporina/administración & dosificación , Progresión de la Enfermedad , Humanos , Inmunosupresores/administración & dosificación , Leucemia Mieloide Aguda/patología , Masculino , Mielografía/métodos , Púrpura Trombocitopénica/etiología , Púrpura Trombocitopénica/patología , Adulto Joven
6.
Turk Kardiyol Dern Ars ; 45(1): 77-81, 2017 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-28106023

RESUMEN

Kabuki syndrome is a rare congenital malformation syndrome characterized by mental retardation, skeletal deformities, auditory dysfunction, cardiac defects, and distinctive facial appearance. Although complex cardiovascular malformations present in early childhood, rarely, atrioventricular septal defects may also present in young adults. Presently described is case of a 22-year-old female with KS who presented with ostium secundum atrial septal defect with deficient rim and idiopathic thrombocytopenic purpura. In this case, minimally invasive robotic surgery was preferred for closure of atrial septal defect.


Asunto(s)
Anomalías Múltiples , Cara/anomalías , Defectos del Tabique Interatrial/diagnóstico , Defectos del Tabique Interatrial/cirugía , Enfermedades Hematológicas , Púrpura Trombocitopénica/diagnóstico , Enfermedades Vestibulares , Diagnóstico Diferencial , Ecocardiografía Transesofágica , Procedimientos Endovasculares , Femenino , Defectos del Tabique Interatrial/complicaciones , Defectos del Tabique Interatrial/diagnóstico por imagen , Humanos , Púrpura Trombocitopénica/complicaciones , Procedimientos Quirúrgicos Robotizados , Resultado del Tratamiento , Adulto Joven
7.
Prague Med Rep ; 118(4): 147-155, 2017.
Artículo en Inglés | MEDLINE | ID: mdl-29324222

RESUMEN

Acquired amegakaryocytic thrombocytopenic purpura (AATP) is a rare hematological disorder characterized by severe thrombocytopenia and a complete or near-to complete absence of megakaryocytes in the bone marrow, while granulopoiesis, as well as erythropoiesis are usually preserved. Although autoimmune mechanisms are believed to be causative, the exact underlying pathogenesis is not known. To date, only few cases have been reported and management of this disease remains controversial with immunosuppression being the treatment modality of choice in the majority of patients. In this article, we report a case of newly acquired AATP without an associated autoimmune disease, refractory to corticoids, intravenous immunoglobulin (IVIG) and second-generation TPO (thrombopoietin) agonists, which have recently been approved for the treatment of thrombocytopenia. Finally, in accordance with other reports, disease progression into aplastic anemia has occurred.


Asunto(s)
Anemia Aplásica/diagnóstico , Anemia Aplásica/etiología , Púrpura Trombocitopénica/complicaciones , Púrpura Trombocitopénica/diagnóstico , Anemia Aplásica/tratamiento farmacológico , Antibacterianos/uso terapéutico , Humanos , Inmunosupresores/uso terapéutico , Masculino , Persona de Mediana Edad , Púrpura Trombocitopénica/tratamiento farmacológico , Resultado del Tratamiento
8.
J Clin Virol ; 83: 61-2, 2016 10.
Artículo en Inglés | MEDLINE | ID: mdl-27596376

RESUMEN

We report here two cases of thrombocytopenic purpura at onset of Zika virus infection. A 26-year-old woman and a 21-year-old man had thrombocytopenia above 5×10(9) platelets/L. Hemorrhagic symptoms were mucosal and subcutaneous bleeding and gross hematuria and they reported episode of conjunctivitis. In both cases blood and bone marrow analysis suggested thrombocytopenic purpura, blood PCR tests for Dengue (DENV), Chikungunya (CHIKV) and Zika virus (ZIKV) were negative. In both cases urinary PCR for ZIKV was positive, Prednisolone yielded early remission. Only three similar cases have been reported so far. In the Caribbean, DENV is also epidemic and responsible for severe thrombocytopenia. Coinfections can occur. Our report underlines the need to include a ZIKV assay in the diagnostic work-up of thrombocytopenic purpura in epidemic areas.


Asunto(s)
Púrpura Trombocitopénica , Infección por el Virus Zika , Virus Zika , Adulto , Femenino , Humanos , Masculino , Púrpura Trombocitopénica/complicaciones , Púrpura Trombocitopénica/diagnóstico , Adulto Joven , Infección por el Virus Zika/complicaciones , Infección por el Virus Zika/diagnóstico
11.
Indian J Chest Dis Allied Sci ; 58(3): 189-190, 2016 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-30152654

RESUMEN

We report the case of a 28-year-old resident doctor with no past history of having taken rifampicin, who presented with thrombocytoapaenic purpura occurring after the initiation of anti-tuberculosis therapy (isoniazid, rifampicin, pyrazinamide and ethambutol) for tubercular lymphadenopathy.


Asunto(s)
Ganglios Linfáticos/diagnóstico por imagen , Mediastino , Rifampin , Tuberculosis Pulmonar , Adulto , Antibióticos Antituberculosos/administración & dosificación , Antibióticos Antituberculosos/efectos adversos , Humanos , Masculino , Púrpura Trombocitopénica/inducido químicamente , Púrpura Trombocitopénica/diagnóstico , Radiografía Torácica/métodos , Rifampin/administración & dosificación , Rifampin/efectos adversos , Resultado del Tratamiento , Tuberculosis Pulmonar/diagnóstico , Tuberculosis Pulmonar/tratamiento farmacológico , Privación de Tratamiento
12.
Clin Appl Thromb Hemost ; 22(2): 115-20, 2016 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-25115760

RESUMEN

Nowadays, we have a relatively sophisticated standard approach to a patient with acute ischemic stroke, including the sequence of diagnostic methods and treatment modalities. In practice, however, we are occasionally confronted with a patient whose medical history or comorbidities force us to make a decision without the support of guidelines. One such situation is the occurrence of acute ischemic stroke in a patient with known idiopathic thrombocytopenic purpura, where a tendency to use thrombolysis, anticoagulants, or antiplatelet agents collides with the fear of life-threatening bleeding. In this review, we try to outline current understanding of the pathophysiology of "paradoxical" ischemic events in this illness characterized by thrombocytopenia and to summarize clinical experience from case reports dealing with this topic, which could help us to rely on more than individual opinion seen through a purely "neurological" or "hematological" prism.


Asunto(s)
Anticoagulantes/uso terapéutico , Isquemia Encefálica , Inhibidores de Agregación Plaquetaria/uso terapéutico , Púrpura Trombocitopénica , Accidente Cerebrovascular , Terapia Trombolítica/métodos , Isquemia Encefálica/diagnóstico , Isquemia Encefálica/etiología , Isquemia Encefálica/terapia , Humanos , Púrpura Trombocitopénica/complicaciones , Púrpura Trombocitopénica/diagnóstico , Púrpura Trombocitopénica/terapia , Accidente Cerebrovascular/diagnóstico , Accidente Cerebrovascular/etiología , Accidente Cerebrovascular/terapia
13.
Rev. méd. hered ; 26(4): 246-255, oct.-dic.2015. ilus, tab
Artículo en Español | LILACS, LIPECS | ID: lil-781707

RESUMEN

Desde siempre los episodios de sangrado muco-cutáneos, al ser tan evidentes, causan mucha preocupación y zozobra, más aún si ocurren sin causa aparente. La púrpura trombocitopénica suele ser la responsable de estos desagradables sucesos. Esta revisión tiene por objetivo actualizar los conocimientos acerca de la fisiopatología, el diagnóstico y el tratamiento de la púrpura trombocitopénica inmunológica (PTI), una patología hematológica que afecta tanto a niños como a adultos y que se ve con relativa frecuencia en la actividad diaria de un hospital general...


Thrombocytopenic purpura is mostly responsible for episodes of muco-cutaneous bleeding. This review updates topics on the pathophysiology, diagnosis and treatment of immunologic thrombocytopenic purpura (IPT), an hematologic condition that afects both childern and adults, which is seen relatively frequent in daily practice in a general hospital...


Asunto(s)
Humanos , Púrpura Trombocitopénica , Púrpura Trombocitopénica/diagnóstico , Púrpura Trombocitopénica/fisiopatología , Púrpura Trombocitopénica/terapia
14.
Transfusion ; 55(11): 2738-41, 2015 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-26098194

RESUMEN

BACKGROUND: Drug-induced immune thrombocytopenia (DITP) is a rare clinical disorder characterized by accelerated platelet (PLT) clearance in the presence of drug-dependent antibodies. Distinguishing DITP from other immune-mediated disorders such as posttransfusion purpura (PTP) and autoimmune thrombocytopenia can represent a clinical challenge. CASE REPORT: A 68-year-old male with no prior transfusion history presented to the emergency department (ED) with dyspnea, epistaxis, and severe thrombocytopenia (<10 × 10(9)/L) 12 days after discharge from a hospital admission for a coronary artery bypass graft. Evaluation of the degree of thrombocytopenia and the temporal association between the peri- and postoperative receipt of multiple transfusions and the acute decrease in PLT count indicated PTP as a possible cause of the severe thrombocytopenia. Treatment with 1 g/kg intravenous immunoglobulin (IVIG) was initiated and followed by a rapid 48-hour increase in the PLT count. PLT antibodies lacking serologic specificity were subsequently identified in a sample collected upon presentation. Two weeks later he again presented to the ED with epistaxis and severe thrombocytopenia (<10 × 10(9)/L). Clinical history now revealed that the patient had been treated with trimethoprim-sulfamethoxazole by his primary care physician after his first hospitalization for a "cellulitic-appearing" leg and again before his final presentation for surgical site erythema and edema. IVIG was administered again with a rapid return of PLT count to baseline. Sulfamethoxazole-dependent PLT antibodies were subsequently identified in the original patient sample. CONCLUSION: This case report documents a case of IVIG-responsive DITP initially misdiagnosed as PTP, highlighting the clinical overlap of these immunologic-mediated phenomena.


Asunto(s)
Púrpura Trombocitopénica/diagnóstico , Sulfametoxazol/efectos adversos , Trombocitopenia/inducido químicamente , Trombocitopenia/diagnóstico , Anciano , Humanos , Masculino , Transfusión de Plaquetas/efectos adversos , Reacción a la Transfusión , Combinación Trimetoprim y Sulfametoxazol/efectos adversos
16.
Methods Mol Biol ; 1310: 149-65, 2015.
Artículo en Inglés | MEDLINE | ID: mdl-26024633

RESUMEN

Human platelet antigen (HPA) typing plays a critical role in the diagnosis of fetal/neonatal alloimmune thrombocytopenia, and the prevention of posttransfusion purpura and refractoriness to platelet transfusions. The recent development of high-throughput genotyping methods, allowing simultaneous genotyping of as many as 17 HPAs, is of utmost interest for saving time and money. Here, we describe a microarray technology named "BeadChip," designed for HPA-1 to -9, -11, and -15 genotyping of up to 96 individuals, in approximately 5 h. This technology was used to study allele frequencies in Brazilian blood donors, considering the heterogeneous ethnic composition.


Asunto(s)
Antígenos de Plaqueta Humana/genética , Frecuencia de los Genes , Técnicas de Genotipaje/métodos , Secuenciación de Nucleótidos de Alto Rendimiento/métodos , Análisis de Secuencia por Matrices de Oligonucleótidos/métodos , Brasil , Diseño de Equipo , Genotipo , Técnicas de Genotipaje/instrumentación , Secuenciación de Nucleótidos de Alto Rendimiento/instrumentación , Humanos , Análisis de Secuencia por Matrices de Oligonucleótidos/instrumentación , Reacción en Cadena de la Polimerasa/instrumentación , Reacción en Cadena de la Polimerasa/métodos , Púrpura Trombocitopénica/diagnóstico , Púrpura Trombocitopénica/genética , Análisis de Secuencia de ADN/instrumentación , Análisis de Secuencia de ADN/métodos , Trombocitopenia/diagnóstico , Trombocitopenia/genética , Reacción a la Transfusión
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