RESUMO
Thrombosis and hemorrhage are two opposing manifestations of multiple myeloma. These hemostatic disorders are present in less than 12% of patients at diagnosis and involve various pathophysiological mechanisms. We report the case of a 39-year-old patient with multiple myeloma revealed by the association of a hemorrhagic syndrome and deep vein thrombosis related to a hypoprothrombinemia-anticoagulant lupus syndrome.
Assuntos
Mieloma Múltiplo/diagnóstico , Adulto , Hemorragia/etiologia , Humanos , Hipoprotrombinemias/etiologia , Cadeias Leves de Imunoglobulina , Inibidor de Coagulação do Lúpus , Masculino , Mieloma Múltiplo/complicações , Síndrome , Trombose/etiologiaRESUMO
BACKGROUND: Pyoderma gangrenosum (PG) is a rare form of neutrophilic dermatosis and is a potential complication in a number of systemic diseases. These include blood diseases, which represent 3.5% of cases, with the main forms being monoclonal gammopathy and acute myeloid leukemia. PATIENTS AND METHODS: Herein we report a case of pyoderma gangrenosum in a female patient who had undergone haematopoietic stem cell allograft six months earlier as part of her treatment for acute T-cell leukemia. DISCUSSION: This condition forms one of the general disorders potentially associated with PG and is a dermatological disorder that can occur in marrow graft patients.
Assuntos
Transplante de Células-Tronco Hematopoéticas/efeitos adversos , Complicações Pós-Operatórias/etiologia , Pioderma Gangrenoso/etiologia , Feminino , Humanos , Adulto JovemAssuntos
Candidíase Invasiva/diagnóstico por imagem , Radioisótopos de Flúor , Fluordesoxiglucose F18 , Hepatite/diagnóstico por imagem , Imagem Multimodal , Tomografia por Emissão de Pósitrons , Compostos Radiofarmacêuticos , Esplenopatias/diagnóstico por imagem , Tomografia Computadorizada por Raios X , Anfotericina B/uso terapêutico , Antifúngicos/uso terapêutico , Protocolos de Quimioterapia Combinada Antineoplásica/efeitos adversos , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Candidíase Invasiva/tratamento farmacológico , Candidíase Invasiva/etiologia , Neutropenia Febril Induzida por Quimioterapia/complicações , Citarabina/administração & dosagem , Citarabina/efeitos adversos , Daunorrubicina/administração & dosagem , Daunorrubicina/efeitos adversos , Hepatite/tratamento farmacológico , Hepatite/etiologia , Humanos , Hospedeiro Imunocomprometido , Leucemia Mieloide Aguda/complicações , Leucemia Mieloide Aguda/tratamento farmacológico , Masculino , Pessoa de Meia-Idade , Esplenopatias/tratamento farmacológico , Esplenopatias/etiologiaRESUMO
Current salvage therapies for relapsed acute myeloid leukemia (AML) are unsatisfactory. We retrospectively evaluated the efficacy and toxicity of fractionated doses of gemtuzumab ozogamicin (GO) combined with a standard 3 + 7 induction regimen in young patients with AML in first relapse. Salvage regimen consisted of GO 3 mg/m² on days 1, 4, and 7; cytarabine, 200 mg/m² on days 1-7; and daunorubicine, 60 mg/m²; or idarubicine, 12 mg/m² on days 1-3. Fourteen patients were treated between April 2008 and April 2011. Median age was 46 years (29-58), median white blood cell count is 3.4 109/L (0.9-19), and median first complete remission (CR) duration is 11 months (1-42). All the patients had previously received high or intermediate doses of cytarabine as consolidation therapy. Salvage treatment was performed as scheduled for the 14 patients, using daunorubicine in 12 patients and idarubicine in two. Overall response rate was 79 % with six CR and five CR with incomplete platelet recovery. Median times to neutrophil (>0.5 109/L) and platelet (>20 109/L) recovery were 29 days (23-32) and 36 days (28-48), respectively. Allogeneic transplantation was performed in the 11 responding patients within a median time of 4 months (3-10). Three mild and one moderate veno-occlusive disease (VOD) occurred after salvage and two moderate VOD after transplantation. Median and 2-year overall survival (OS) were 10 months and 42 %, respectively. For responders, estimated 2-year OS was 53 % (median OS not reached). This salvage regimen seems safe and effective in younger patients with AML in first relapse allowing allogeneic transplantation in CR2 for most patients.
Assuntos
Aminoglicosídeos/administração & dosagem , Anticorpos Monoclonais Humanizados/administração & dosagem , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Quimioterapia de Indução , Leucemia Mieloide Aguda/tratamento farmacológico , Terapia de Salvação , Adulto , Aminoglicosídeos/efeitos adversos , Aminoglicosídeos/uso terapêutico , Anticorpos Monoclonais Humanizados/efeitos adversos , Anticorpos Monoclonais Humanizados/uso terapêutico , Antineoplásicos/administração & dosagem , Antineoplásicos/efeitos adversos , Antineoplásicos/uso terapêutico , Protocolos de Quimioterapia Combinada Antineoplásica/efeitos adversos , Terapia Combinada , Citarabina/administração & dosagem , Citarabina/efeitos adversos , Citarabina/uso terapêutico , Daunorrubicina/administração & dosagem , Daunorrubicina/efeitos adversos , Daunorrubicina/uso terapêutico , Esquema de Medicação , Feminino , Seguimentos , Gemtuzumab , Transplante de Células-Tronco Hematopoéticas/efeitos adversos , Hepatopatia Veno-Oclusiva/induzido quimicamente , Hepatopatia Veno-Oclusiva/etiologia , Hepatopatia Veno-Oclusiva/fisiopatologia , Hepatopatia Veno-Oclusiva/prevenção & controle , Humanos , Idarubicina/administração & dosagem , Idarubicina/efeitos adversos , Idarubicina/uso terapêutico , Quimioterapia de Indução/efeitos adversos , Leucemia Mieloide Aguda/terapia , Masculino , Pessoa de Meia-Idade , Recidiva , Estudos Retrospectivos , Terapia de Salvação/efeitos adversos , Índice de Gravidade de Doença , Análise de SobrevidaRESUMO
INTRODUCTION: Evans syndrome (ES) is characterized by the coexistence of an autoimmune hemolytic anemia (AIHA) and immune thrombocytopenic purpura (ITP). Despite being frequently evocated in the simultaneous presence of anemia and thrombocytopenia, this rare disease only accounts for 0.8 to 3.7% of patients with ITP or AIHA. CASE REPORTS: We report three suspected cases of ES, diagnosed in the presence of thrombocytopenia and hemolytic anemia association, with a positive direct Coombs test in two patients. Standard ES treatment failure and occurrence of additional features subsequently led to correct diagnosis to thrombotic thrombocytopenic purpura, myelodysplastic syndrome with AIHA, and ITP with hemorrhagic anemia, respectively. CONCLUSION: Bicytopenias, even in an immunological context, are not sufficient to ascertain ES diagnosis. Our cases illustrate the diagnostic difficulties that may arise in daily practice, and induce over-diagnosis of this rare disease.