Assuntos
Gamopatia Monoclonal de Significância Indeterminada/complicações , Aplasia Pura de Série Vermelha/etiologia , Biópsia , Contagem de Células Sanguíneas , Medula Óssea/patologia , Feminino , Hemoglobinas/análise , Humanos , Pessoa de Meia-Idade , Gamopatia Monoclonal de Significância Indeterminada/sangue , Gamopatia Monoclonal de Significância Indeterminada/patologia , Aplasia Pura de Série Vermelha/sangue , Aplasia Pura de Série Vermelha/diagnóstico , Aplasia Pura de Série Vermelha/patologiaRESUMO
Management of intermediate and high risk acute pulmonary embolism (PE) is challenging. The role of multidisciplinary teams for the care of these patients is emerging. Herein, we report our experience with a pulmonary embolism response team (PERT). We conducted a retrospective chart review on all patients admitted to the Cleveland Clinic main campus who required activation of the (PERT) from October 1, 2014 to September 1, 2016. We extracted data pertaining to clinical presentation, bleeding complications, and pre- and post-discharge imaging. Patients were classified as low, intermediate or high risk PE. Descriptive and continuous variables were collected and analyzed. There were 134 PERT activations. PE was confirmed by CT-PA in 118 patients. Fifteen (13%) patients were classified as low risk, 80 (68%) intermediate risk PE and 23 (19%) high risk PE. Fourteen (12%) patients were treated with catheter directed rtPA, 6 (5%) received full dose (100 mg rtPA), 16 (13%) received systemic half-dose (50 mg rtPA), 6 (5%) underwent a surgical embolectomy and 4 (3%) underwent mechanical thrombectomy. 65 (55%) patients received anticoagulation only, and 8 (7%) patients were managed conservatively without any anticoagulation or advanced therapy. 11 (9%) patients died while during the hospitalization. Fourteen patients had major bleeding events. There were no bleeding events among patients who received systemic low dose or full dose rtPA. A multidisciplinary approach to cases of intermediate risk and high risk PE can be implemented successfully. We saw a relatively low rate of bleeding events with use of rtPA.
Assuntos
Equipe de Assistência ao Paciente/normas , Embolia Pulmonar/terapia , Adulto , Idoso , Anticoagulantes/uso terapêutico , Gerenciamento Clínico , Embolectomia , Hemorragia/induzido quimicamente , Hemorragia/etiologia , Humanos , Pessoa de Meia-Idade , Embolia Pulmonar/complicações , Estudos Retrospectivos , Medição de Risco , Trombectomia , Terapia Trombolítica , Ativador de Plasminogênio Tecidual/uso terapêuticoRESUMO
A 62-year-old woman with prosthetic mitral valve was admitted for explant of an infected prosthetic knee. Perioperatively, she was bridged with heparin and started on empiric vancomycin and piperacillin-tazobactam. Platelet counts dropped precipitously within 2 days reaching a nadir of 6000/µL, without any bleeding. Decline persisted despite substituting heparin with bivalirudin. Antiplatelet factor 4 and anti-PLA1 antigen were negative. Schistocytes were absent. Antibiotics were substituted with daptomycin for suspected drug-induced thrombocytopenia. Pulse dose of intravenous immunoglobulin was initiated with rapid normalization of platelet count. She tested positive for IgG antiplatelet antibodies to vancomycin and piperacillin-tazobactam thereby confirming the diagnosis. Drug-induced immune-mediated thrombocytopenia is an underrecognized cause of thrombocytopenia in the intensive care units. Clinicians should be cognizant of this entity, and a definitive diagnosis should be sought if feasible.
Assuntos
Ácido Penicilânico/análogos & derivados , Púrpura Trombocitopênica Idiopática/induzido quimicamente , Vancomicina/efeitos adversos , Remoção de Dispositivo , Feminino , Humanos , Imunoglobulina G/imunologia , Unidades de Terapia Intensiva , Pessoa de Meia-Idade , Ácido Penicilânico/administração & dosagem , Ácido Penicilânico/efeitos adversos , Piperacilina/administração & dosagem , Piperacilina/efeitos adversos , Combinação Piperacilina e Tazobactam , Infecções Relacionadas à Prótese/cirurgia , Púrpura Trombocitopênica Idiopática/diagnóstico , Púrpura Trombocitopênica Idiopática/imunologia , Vancomicina/administração & dosagemAssuntos
Artroplastia do Joelho , Linfo-Histiocitose Hemofagocítica/etiologia , Linfoma Difuso de Grandes Células B/complicações , Idoso , Protocolos de Quimioterapia Combinada Antineoplásica , Biópsia , Medula Óssea/patologia , Humanos , Linfo-Histiocitose Hemofagocítica/diagnóstico , Linfo-Histiocitose Hemofagocítica/tratamento farmacológico , Linfoma Difuso de Grandes Células B/diagnóstico , Linfoma Difuso de Grandes Células B/tratamento farmacológico , Macrófagos/metabolismo , Macrófagos/patologia , Masculino , Estadiamento de NeoplasiasAssuntos
Anemia Sideroblástica/genética , Testes Genéticos/métodos , Mutação , Fosfoproteínas/genética , Ribonucleoproteína Nuclear Pequena U2/genética , Adulto , Idoso , Anemia Sideroblástica/classificação , Anemia Sideroblástica/diagnóstico , Criança , Análise Mutacional de DNA , Diagnóstico Diferencial , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Fatores de Processamento de RNA , Sensibilidade e Especificidade , Adulto JovemRESUMO
Spontaneous perinephric hematoma (SPH) is a rare entity whose diagnosis is challenging because of its varied clinical presentation and lack of any specific etiology. We report a 34-year-old African-American male who presented with left flank pain and was found to have a large left perinephric hematoma, in the setting of undiagnosed AL amylodosis. The case illustrates that while a SPH due to the vascular angiopathy of amyloid is rare, when amyloidosis is associated with abnormal coagulation studies or bleeding at multiple sites, it should be considered because of its protean systemic manifestations and potential response to chemotherapy.
RESUMO
This case report describes a case of congenital sideroblastic anaemia, one of the prototype disorders of erythroid haem biosynthesis. In this instance it was not recognised until after the patient had undergone splenectomy and developed refractory thromboembolic disease.
Assuntos
Anemia Sideroblástica/congênito , Esplenectomia/efeitos adversos , Tromboembolia/etiologia , Anemia Sideroblástica/complicações , Anemia Sideroblástica/cirurgia , Anticoagulantes/uso terapêutico , Diagnóstico Diferencial , Enoxaparina/uso terapêutico , Humanos , Masculino , Tromboembolia/diagnóstico , Tromboembolia/prevenção & controle , Adulto JovemAssuntos
Doença Enxerto-Hospedeiro/tratamento farmacológico , Transplante de Fígado/efeitos adversos , Proteínas Recombinantes de Fusão/uso terapêutico , Alefacept , Fármacos Dermatológicos/uso terapêutico , Humanos , Transplante de Fígado/métodos , Masculino , Pessoa de Meia-Idade , Indução de RemissãoRESUMO
Thrombotic thrombocytopenic purpura (TTP) is an uncommon but severe disorder that classically presents with microangiopathic hemolytic anemia (MAHA), thrombocytopenia, and fluctuating neurological changes. Previously, it was impossible to make a diagnosis of TTP in the absence of thrombocytopenia or microangiopathic hemolysis (MAHA). We describe two cases of relapsing TTP that presented with acute cerebrovascular accident (CVA) without concurrent thrombocytopenia or MAHA after initial classical presentation of TTP. In both cases, the diagnosis of TTP as the cause of the CVA was attributed to severe deficiency of the von Willebrand factor cleaving protease, ADAMTS13 in plasma (11 and 12%, normal 79-127%). Each patient had a dramatic clinical improvement in response to therapeutic plasma exchange. The experience in these two cases suggests that TTP should be considered as a potential cause among patients presenting with a CVA, particularly if the patients have a history of TTP.
Assuntos
Púrpura Trombocitopênica Trombótica/diagnóstico , Acidente Vascular Cerebral/diagnóstico , Proteínas ADAM , Proteína ADAMTS13 , Adulto , Diagnóstico Diferencial , Feminino , Humanos , Metaloendopeptidases/deficiência , Púrpura Trombocitopênica Trombótica/patologia , Recidiva , Trombocitopenia/diagnóstico , Fatores de TempoRESUMO
Deficiency of von Willebrand factor (VWF) cleaving protease ADAMTS13 has been demonstrated to be the proximate cause of a subset of thrombotic microangiopathic haemolytic anaemias (MAHA) typical for thrombotic thrombocytopenic purpura (TTP). ADAMTS13 gene mutations cause the hereditary form; acquired deficiency has been attributed to presence of an autoantibody, which may represent a specific subset of MAHA best termed 'autoimmune thrombotic thrombocytopenic purpura'. We describe a patient with relapsing TTP because of ADAMTS13 inhibitors, who failed to achieve sustained remission despite therapies with plasma exchange, steroids, vincristine, staphylococcal protein A and splenectomy. The ADAMTS13 inhibitor titre remained elevated and clinical stability was only maintained by plasma exchange every 2-3 d over a period of 268 d. The patient then received rituximab therapy (eight doses of 375 mg/m2 weekly), during which she required five plasma exchanges in the first 10 d, two exchanges in the next 3 weeks, and none thereafter for 450 d and ongoing. The ADAMTS13 inhibitor titre decreased and enzyme activity increased. We compared this case with that of seven previously reported TTP cases also treated with rituximab; experience suggests that rituximab therapy deserves further investigation for patients with either refractory or relapsing TTP caused by ADAMTS13 inhibitors.
Assuntos
Anticorpos Monoclonais/uso terapêutico , Púrpura Trombocitopênica Trombótica/tratamento farmacológico , Proteínas ADAM , Proteína ADAMTS13 , Adulto , Anticorpos Monoclonais Murinos , Doença Crônica , Terapia Combinada , Feminino , Humanos , Metaloendopeptidases/sangue , Metaloendopeptidases/genética , Troca Plasmática , Púrpura Trombocitopênica Trombótica/genética , Recidiva , RituximabRESUMO
PRIMARY OBJECTIVE: This paper examined the use of an atypical neuroleptic medication, risperidone, in reducing the excessive motor activity of an adolescent with an anoxic brain injury following cardiac arrest from a lightning strike. Lower extremity restlessness caused the patient to develop skin breakdown and interfered with healing of existing burns. RESEARCH DESIGN: Single-blind, placebo-controlled single-subject experimental design. EXPERIMENTAL INTERVENTION: Escalating doses of risperidone up to 1 mg and in combination with methylphenidate (10 mg) and amantidine (100 mg). MAIN OUTCOMES AND RESULTS: The patient demonstrated a reduction in restlessness in response to the use of risperidone, which permitted wound healing. The addition of methlphenidate to risperidone led to a slight increase in attention to task. CONCLUSIONS: The use of the atypical neuroleptic medication, risperidone, may be considered as part of the armamentarium available to physicians treating restlessness in severe brain injuries.