RESUMO
The review discusses approaches to treatment of congenital thrombotic thrombocytopenic purpura (TTP) or Upshaw-Schulman syndrome. In congenital TTP, plasma transfusions are sufficient. Such treatment options as plasma exchange, administration of clotting factor VIII concentrate, recombinant ADAMTS13, are also used. Separately discussed issues of management of patients with TTP during pregnancy, and pediatric patients with TTP.
Assuntos
Púrpura Trombocitopênica Trombótica , Gravidez , Feminino , Humanos , Criança , Púrpura Trombocitopênica Trombótica/diagnóstico , Púrpura Trombocitopênica Trombótica/terapia , Proteína ADAMTS13 , Troca Plasmática , Fatores de Coagulação SanguíneaRESUMO
The review discusses approaches to treatment of acquired thrombotic thrombocytopenic purpuгa (aTTP). In patients with aTTP plasma exchanges, glucocorticosteroids allow to stop an acute attack of TTP, and use of rituximab allows to achieve remission. In recent years, caplacizumab has been used. Treatment options such as cyclosporin A, bortezomib, splenectomy, N-acetylcysteine, recombinant ADAMTS13 are also described. Separately discussed issues of management of patients with TTP during pregnancy, and pediatric patients with TTP.
Assuntos
Púrpura Trombocitopênica Trombótica , Humanos , Criança , Púrpura Trombocitopênica Trombótica/diagnóstico , Púrpura Trombocitopênica Trombótica/tratamento farmacológico , Rituximab/uso terapêutico , Bortezomib , Ciclosporina , Acetilcisteína , Troca PlasmáticaRESUMO
Сytokine release syndrome is the common complication of CAR-T therapy. We report a case of patient with B-cell acute lymphoblastic leukemia developing Ñytokine release syndrome with shock and multiple organ failure and requiring cytokine removal and hemodiafiltration. Remission of the disease was achieved after CAR-T therapy.
Assuntos
Leucemia-Linfoma Linfoblástico de Células Precursoras , Receptores de Antígenos Quiméricos , Humanos , Síndrome da Liberação de Citocina/diagnóstico , Síndrome da Liberação de Citocina/etiologia , Síndrome da Liberação de Citocina/terapia , Antígenos CD19 , Citocinas , Leucemia-Linfoma Linfoblástico de Células Precursoras/complicações , Leucemia-Linfoma Linfoblástico de Células Precursoras/terapia , Doença Aguda , Terapia Baseada em Transplante de Células e TecidosRESUMO
Thrombotic thrombocytopenic purpura (TTP) is a rare, life-threatening disease, disease, characterised by microangiopathic hemolytic anaemia, consumption thrombocytopenia, and organ dysfunction. The pathogenesis of TTP is attributed to the deficiency in the activity of the metalloproteinase ADAMTS13, specific von Willebrand factor cleaving protease. TTP is suspected when detecting microangiopathic hemolytic anemia, thrombocytopenia, damage to various organs. Diagnosis of TTP is confirmed by the detection of ADAMTS13 activity in plasma less than 10%. Plasma samples for the study of ADAMTS13 activity should be taken before the start of plasma transfusions or plasma exchange. In patients with severe ADAMTS-13 deficiency autoantibodies anti-ADAMTS13 and inhibitor ADAMTS13 should be investigated. Anti-ADAMTS13 antibodies belonging to IgG not always have inhibitory effects. The inhibitory effect of anti-ADAMTS13 antibodies is confirmed by mixing test. All patients with the first established diagnosis of TTP should be examined for mutations of the ADAMTS13 gene.
Assuntos
Púrpura Trombocitopênica Trombótica , Autoanticorpos , Humanos , Mutação , Troca Plasmática , Plasmaferese , Púrpura Trombocitopênica Trombótica/diagnóstico , Púrpura Trombocitopênica Trombótica/terapiaRESUMO
AIM: Remission induction can be associate, with the life threatening complications and transfer to ICU of de novo acute myeloid leukemia (AML) patients (pts). We evaluate influence of transfer to ICU and life threatening complication on early mortality and long - tram survival of de novo AML pts. MATERIALS AND METHODS: Retrospective study. All de novo AML pts younger than 60 years old admitted in the National Research Center for Hematology from 2013 to 2016 years were enrolled in the study. Patients were divided into 2 groups: pts who were required ICU admission during remission induction (ICU-pts) and pts who did not require ICU admission and received chemotherapy only in hematology ward (non-ICU pts). The reasons for ICU admissions and results of life support were analyzed. Overall survival (OS) were assessed by the Kaplan-Meier method, long rank value p.
Assuntos
Leucemia Mieloide Aguda , Protocolos de Quimioterapia Combinada Antineoplásica , Cuidados Críticos , Humanos , Pessoa de Meia-Idade , Indução de Remissão , Estudos RetrospectivosRESUMO
The case report of botulinum toxin treatment of gastroparesis in a patient following allogenic bone marrow transplantation is described. The causes of gastroparesis and methods of prevention and treatment are discussed. It was noted that pyloric injection of botulinum toxin can improve symptoms and gastric emptying.
Assuntos
Toxinas Botulínicas , Gastroparesia , Medula Óssea , Transplante de Medula Óssea , Toxinas Botulínicas/uso terapêutico , Esvaziamento Gástrico , Gastroparesia/tratamento farmacológico , HumanosRESUMO
The article describes a rare clinical case of a patient with previously undiagnosed von Willebrand disease and basal meningioma; an intracranial neurosurgical intervention was complicated by delayed intracranial hematomas, both at the resected tumor site and distantly. The diagnosis of von Willebrand disease was established only after special hematology tests and only after surgery. Despite the use of specific therapy, the patient died due to intracranial hemorrhagic complications in the postoperative period. The paper discusses the problem of preoperative diagnosis of asymptomatic hemostasis disorders in neurosurgical patients and potential ways of its solution.
Assuntos
Hemorragias Intracranianas , Neoplasias Meníngeas , Meningioma , Doenças de von Willebrand , Humanos , Complicações Pós-Operatórias , Doenças de von Willebrand/complicaçõesRESUMO
Gaucher disease is the most common form of hereditary enzymopathies combined into a group of lysosomal storage diseases. The basis for the disease is a hereditary deficiency of the activity of acid ß-glucosidase, a lysosomal enzyme involved in the catabolism of lipids, which results in the accumulation of nonutilized cellular metabolism products in the macrophage lysosomes. The main clinical manifestations of type 1 Gaucher disease are cytopenia, hepatomegaly, and splenomegaly, and bone lesion. One of the atypical clinical manifestations of Gaucher disease is damage to the lungs with the development of pulmonary hypertension, which is usually considered within the underlying disease - the development of pneumosclerosis due to macrophage dysfunction. The paper describes a case of progressive pulmonary hypertension in a patient with type 1 Gaucher disease.
Assuntos
Doença de Gaucher , Cardiopatias Congênitas , Insuficiência Cardíaca , Comunicação Interventricular , Hipertensão Pulmonar , Septo Interventricular/patologia , Adulto , Diagnóstico , Progressão da Doença , Evolução Fatal , Feminino , Doença de Gaucher/complicações , Doença de Gaucher/diagnóstico , Doença de Gaucher/genética , Doença de Gaucher/fisiopatologia , Glucosilceramidase/genética , Cardiopatias Congênitas/complicações , Cardiopatias Congênitas/patologia , Cardiopatias Congênitas/fisiopatologia , Insuficiência Cardíaca/etiologia , Insuficiência Cardíaca/fisiopatologia , Comunicação Interventricular/complicações , Comunicação Interventricular/patologia , Comunicação Interventricular/fisiopatologia , Humanos , Hipertensão Pulmonar/etiologia , Hipertensão Pulmonar/fisiopatologiaRESUMO
Primary central nervous system (CNS) lymphomas account for 13-20% of the posttransplant lymphoproliferative disorders (PTLD) and rank among the most aggressive conditions. Reduction of immunosuppressive therapy should be mandatory to treat PTLD, but this is rarely used as the only therapy option. Chemotherapy regimens for PTLD involving the CNS most commonly include high-dose rituximab and high-dose methotrexate and/or cytarabine. The efficiency only of discontinuation of immunosuppressive therapy for PTLD does not exceed 5-10%, but there are no literature data on its efficiency for PTLD involving the CNS. The paper describes a clinical case of achieving long-term remission in a female patient with Epstein-Barr virus (EBV)-positive diffuse large B-cell lymphoma involving the central nervous system, associated with immunosuppression after kidney transplantation from a related donor, in the absence of chemotherapy during immunosuppressive therapy discontinuation and transplantectomy.
Assuntos
Herpesvirus Humano 4/isolamento & purificação , Terapia de Imunossupressão/efeitos adversos , Imunossupressores , Falência Renal Crônica/terapia , Transplante de Rim , Linfoma Difuso de Grandes Células B , Adulto , Encéfalo/diagnóstico por imagem , Feminino , Humanos , Terapia de Imunossupressão/métodos , Imunossupressores/administração & dosagem , Imunossupressores/efeitos adversos , Transplante de Rim/efeitos adversos , Transplante de Rim/métodos , Linfoma Difuso de Grandes Células B/etiologia , Linfoma Difuso de Grandes Células B/patologia , Linfoma Difuso de Grandes Células B/terapia , Linfoma Difuso de Grandes Células B/virologia , Nefrectomia/métodos , Procedimentos Neurocirúrgicos , Tomografia Computadorizada por Raios X/métodos , Transplantes/diagnóstico por imagem , Transplantes/fisiopatologia , Transplantes/cirurgia , Resultado do Tratamento , Suspensão de TratamentoRESUMO
The paper describes 4 clinical cases of thrombotic events (pulmonary embolism, deep vein thrombophlebitis, acute myocardial infarction, ischemic stroke) that have occurred in patients with hemophilia. It discusses the possible causes of their development and methods for their prevention and treatment. Controlled natural hypocoagulation, in which the dose of an administered deficient factor decreases to such an extent that in order to maintain the safe level of hypocoagulation (plasma factor activity is 15-20%; activated partial thromboplastin time is 1.5-2 times normal values), is proposed as one of the treatment options.
Assuntos
Isquemia Encefálica , Fator VIII , Hemofilia A , Infarto do Miocárdio , Embolia Pulmonar , Acidente Vascular Cerebral , Tromboflebite , Adulto , Coagulação Sanguínea/fisiologia , Testes de Coagulação Sanguínea/métodos , Isquemia Encefálica/etiologia , Gerenciamento Clínico , Fator VIII/administração & dosagem , Fator VIII/análise , Hemofilia A/complicações , Hemofilia A/diagnóstico , Hemofilia A/fisiopatologia , Hemofilia A/terapia , Humanos , Masculino , Pessoa de Meia-Idade , Infarto do Miocárdio/diagnóstico , Infarto do Miocárdio/etiologia , Infarto do Miocárdio/fisiopatologia , Infarto do Miocárdio/terapia , Embolia Pulmonar/diagnóstico , Embolia Pulmonar/etiologia , Embolia Pulmonar/fisiopatologia , Embolia Pulmonar/terapia , Acidente Vascular Cerebral/diagnóstico , Acidente Vascular Cerebral/etiologia , Acidente Vascular Cerebral/fisiopatologia , Acidente Vascular Cerebral/terapia , Tromboelastografia/métodos , Tromboflebite/diagnóstico , Tromboflebite/etiologia , Tromboflebite/fisiopatologia , Tromboflebite/terapia , Resultado do TratamentoRESUMO
PURPOSE: To compare the accuracy of bedside lung ultrasound (LUS) and chest computed tomography (CT) for the de- tection of lung lesions in patients with hematological malignancies and acute respiratory failure (ARF). MATERIALS AND METHODS: 39 patients with hematological malignancies and ARF were enrolled in prospective study. The investigation of the patients included LUS, chest C, extravascular lung water index (EVLW) by transpulmonary ther- modilution, and bronchoalveolar lavage (BAL). RESULTS: There was correlation between the total number of B-lines and E VLW index (r = 0,40; p <0,05). The sensitivity, specificity of LUS in the total number of B-lines were 78% and 70%, respectively (and A UC 0,7). There were correla- tions between A-lines and volume of hyperaerated lung regions (r = 0,40; p <0,05) and normally ventilated (r = 0,60; p = 0,001) regions, between A-lines and the total lung volume (r = 0,50; p = 0,001), between A-lines and volume of poorlyventilated lung regions (r = -0,40; p = 0,001), A-lines and weight of normally ventilated lung regions (r = 0,50; p = 0,001), A-lines and weight of poorly ventilated regions (r = -0,35; p <0,05), total count of B-lines and volume of poorly ventilated lung regions (r = 0,4; p = 0,001), between total count of B-lines and weight poorly ventilated lung regions (r = 0,4; p = 0,001). There were associations between USfeathers and etiology ofpneumonia. A-lines were often detected in patients with gram-negative bacterial pneumonia and fungal pneumonia more than in patients with pneu- mocystis pneumonia. B-lines were detected often in patients with Pneumocystis pneumonia. Sensitivity ofLUS pleural effusion assessment was 95%, specificity was 90%. CONCLUSION: LUS is high sensitivity and specificity method to detect lung lesions in patients with ARF.
Assuntos
Água Extravascular Pulmonar/diagnóstico por imagem , Neoplasias Hematológicas/complicações , Pulmão/diagnóstico por imagem , Cavidade Pleural/diagnóstico por imagem , Pneumonia/diagnóstico por imagem , Insuficiência Respiratória/diagnóstico por imagem , Doença Aguda , Adulto , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Pneumonia/complicações , Estudos Prospectivos , Insuficiência Respiratória/etiologia , Sensibilidade e Especificidade , UltrassonografiaRESUMO
AIM: to study the nature of pain in hemophilic arthropathy and its prevention ways used by patients with hemophilia. SUBJECTS AND METHODS: A prospective, multicenter, cross-sectional study was conducted to interview 136 patients with hemophilia A or B. The survey included 32 points, including questions about drug and nondrug pain treatment, as well as questionnaires to assess the severity of pain and its impact on daily life (Brief Pain Inventory) and those to identify a neuropathic pain component (PainDetect questionnaire). RESULTS: 83 (75%) patients with hemophilia experienced acute pain associated with bleeding into the large joints; 44 (39%) patients had chronic pain that had lasted longer than six months; 33% assessed the moderate pain experienced in the past 24 hours as severe (more than 5-8 of the 10 scores). In addition to the above, only 32% of the respondents indicated that they had painless intervals within the last 24 hours. 75% of the hemophilia patients mentioned to have limited daily activities. 74% reported their partial or complete disability when pain occurred. 77% of the respondents pointed out that when having pain, they had experienced great difficulty walking. 55% of the hemophilia patients had sleep disorders because of pain. When acute pain occurred, only 91 (81%) respondents injected a factor VIII or IX preparation, 37% of the respondents used narcotic analgesics; 51% received different nonsteroidal anti-inflammatory drugs; 13% took paracetamol. Some patients indicated that they used alcohol and illegal narcotic drugs to relieve pain. Attention is drawn to the fact that the hemophilia patients very frequently took painkillers: 60% of the respondents used analgesics every month; of them 49% had them every week, 11% every day. Most patients (n=83 (74%)) stated that they treated pain (prescribed drugs) themselves; 49 (44%) patients held they were dissatisfied with their pain treatment. When the question as to how the analgesic you used could "remove' pain was asked, only 12 (10%) patients answered that this could fully relieve the pain; 31 (26%) patients told that the pain was not reduced even by half. The study has shown that a neuropathic pain component is very common in hemophilia patients (31%). CONCLUSION: Pain in patients with hemophilia is a serious problem that negatively affects their quality of life, including their working capacity. The prevention and treatment of pain in hemophilia patients should involve the following: organization of educational activities and pain services; adequate treatment of acute pain; detection and prevention of a neuropathic pain component.
Assuntos
Dor Aguda , Analgésicos/uso terapêutico , Artralgia , Dor Crônica , Hemofilia A/complicações , Neuralgia , Dor Aguda/tratamento farmacológico , Dor Aguda/epidemiologia , Dor Aguda/etiologia , Adulto , Idoso , Artralgia/tratamento farmacológico , Artralgia/epidemiologia , Artralgia/etiologia , Dor Crônica/tratamento farmacológico , Dor Crônica/epidemiologia , Dor Crônica/etiologia , Estudos Transversais , Hemofilia A/epidemiologia , Humanos , Masculino , Pessoa de Meia-Idade , Neuralgia/tratamento farmacológico , Neuralgia/epidemiologia , Neuralgia/etiologia , Estudos Prospectivos , Adulto JovemRESUMO
The paper describes 4 cases of laparoscopic splenectomy in pregnant women with immune thrombocytopenic purpura. No complications of surgery were noted in all the patients. The postoperative period was marked by sustained clinical and hematological remission that made it possible to discontinue prednisolone therapy and to ensure an uncomplicated course of pregnancy and labor.
Assuntos
Laparoscopia/métodos , Complicações na Gravidez/cirurgia , Púrpura Trombocitopênica Idiopática/cirurgia , Esplenectomia/métodos , Adulto , Feminino , Humanos , Gravidez , Resultado da GravidezRESUMO
AIM: to analyze well-known risk factors (RFs), such as age, immunophenotype, baseline leukocytosis, enhanced lactate dehydrogenase (LDH) activity, time to achieve complete remission, a risk group, and cytogenetic abnormalities) in patients with acute lymphoblastic leukemia (ALL) in the use of the ALL-2009 protocol. SUBJECTS AND METHODS: The protocol covered 298 patients (137 women (including 13 pregnant women) and 161 men) aged 15 to 55 years (median age 28 years) with Ph-negative ALL. The phenotype was unknown in 6 patients. Three (1%) were ascertained to have a biphenotypic variant. 182 (62.4%) patients were found to have B-cell ALL (early pre-B ALL (n=51); common ALL (n=92), and pre-B ALL (n=39); 107 (36.6%) patients had T-cell ALL (early T-ALL (n=56); thymic T-ALL (n=41), and mature T-ALL (n=10). According to the baseline clinical and laboratory parameters (leukocytosis of 30·109/l and more for B-ALL; and that of 100·109/l and more for T-ALL; phenotype Ð-I for B-ALL, phenotype Т-I-II-IV for T-ALL; LDH activity was more than twice the normal values; the presence of translocation t(4;11)), the high-risk group included most patients with B-ALL (n=110 (72.8%)) and T-ALL (n=76 (76%)). Thirty-five patients with T-ALL underwent autologous bone marrow transplantation (BMT). Allogeneic BMT was performed in 18 (7%) of the 258 patients who had undergone an induction phase. RESULTS: Five-year overall survival for all the patients included in the investigation was 59%; relapse-free survival was 65%, which was significantly different in the patients with B-ALL and in those with T-ALL: the overall survival rates were 53.3 and 67.5% (p=0.1); the relapse-free survival was 56 and 79% (p=0.005), respectively. Multivariate analysis including the well-known RFs demonstrated that the latter for T-ALL were of no independent prognostic value and only the patient's age was identified for B-ALL (p=0.013). CONCLUSION: A lower chemotherapeutic load and a small number of allogeneic BMTs did not affect total positive treatment results in adult patients with ALL, by complying with the principle achieving the continuity of cytostatic effects and by preserving the total cytostatic loading dose. The results of the Russian investigation casts some doubt on the necessity of using very intensive consolidation cycles and performing a large number of allogeneic BMTs in adult patients with ALL.
Assuntos
Protocolos Clínicos , Avaliação de Resultados em Cuidados de Saúde , Leucemia-Linfoma Linfoblástico de Células Precursoras , Complicações na Gravidez , Adolescente , Adulto , Transplante de Medula Óssea , Intervalo Livre de Doença , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Leucemia-Linfoma Linfoblástico de Células Precursoras/classificação , Leucemia-Linfoma Linfoblástico de Células Precursoras/epidemiologia , Leucemia-Linfoma Linfoblástico de Células Precursoras/metabolismo , Leucemia-Linfoma Linfoblástico de Células Precursoras/terapia , Gravidez , Complicações na Gravidez/epidemiologia , Complicações na Gravidez/metabolismo , Complicações na Gravidez/terapia , Fatores de Risco , Transplante Autólogo , Adulto JovemRESUMO
The described case of FIP1L1-PDGFRÐ-positive myeloproliferative disease is characterized by an atypical aggressive course to develop severe specific complications as injuries to the brain, heart, lung, and intestine. Pathogenetic therapy with imatinib could stabilize a patient's state, but failed to produce a complete hematological response. Switching from imatinib to dasatinib could produce sustained clinical, hematological, and molecular remissions.
Assuntos
Eosinofilia/tratamento farmacológico , Insuficiência de Múltiplos Órgãos/tratamento farmacológico , Transtornos Mieloproliferativos/tratamento farmacológico , Proteínas de Fusão Oncogênica , Inibidores de Proteínas Quinases/farmacologia , Proteínas Tirosina Quinases/antagonistas & inibidores , Receptor alfa de Fator de Crescimento Derivado de Plaquetas , Fatores de Poliadenilação e Clivagem de mRNA , Adulto , Dasatinibe/administração & dosagem , Dasatinibe/farmacologia , Feminino , Humanos , Mesilato de Imatinib/administração & dosagem , Mesilato de Imatinib/farmacologia , Transtornos Mieloproliferativos/genética , Inibidores de Proteínas Quinases/administração & dosagemRESUMO
AIM: To estimate the informative value of ultrasonography (USG) in the diagnosis of lung injuries in pregnant women with blood system tumors. SUBJECTS AND METHODS: Lung ultrasound was performed in 5 pregnant patients with blood cancers. The women's age was 29-38 years; gestational age was 14-33 weeks. Four women had different types of acute leukemia; one had primary mediastinal large B-cell lymphoma. All the women received chemotherapy for blood cancer. When there were signs of lung injury, USG was conducted, the results of which necessitated therapy or bronchoalveolar lavage (BAL). RESULTS: Three patients developed acute respiratory failure; 2 of them required noninvasive ventilation. Based on the detection of consolidation with a dynamic air bronchogram and pleural effusion, the authors diagnosed bilateral pneumonia and alveolar-interstitial syndrome in 1 patient, right-sided pneumonia in 1, left-sided one in 1, and transfusion-related pulmonary edema in 1. Lung ultrasound did not verify the diagnosis of pneumonia in 1 patient. According to USG data, BAL procedures were performed in 2 patients; one of them was diagnosed as having Pneumocystis pneumonia; the other was found to have no pathogens in lavage fluid. Treatment resulted in clinical improvements and normalization of the lung ultrasound pattern in all the pregnant women. Later on, 4 women delivered via cesarean section done at 32-34 weeks' gestation and gave birth to healthy babies. One patient died from infectious complications after chemotherapy. CONCLUSION: Lung sound may be used to diagnose lung injury in pregnant women with blood cancers.
Assuntos
Neoplasias Hematológicas/diagnóstico por imagem , Pulmão/diagnóstico por imagem , Pneumonia Bacteriana/diagnóstico por imagem , Complicações Hematológicas na Gravidez/diagnóstico por imagem , Complicações Infecciosas na Gravidez/diagnóstico por imagem , Adulto , Antibacterianos/administração & dosagem , Antibacterianos/uso terapêutico , Protocolos de Quimioterapia Combinada Antineoplásica/administração & dosagem , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Feminino , Idade Gestacional , Neoplasias Hematológicas/complicações , Neoplasias Hematológicas/tratamento farmacológico , Humanos , Pneumonia Bacteriana/complicações , Pneumonia Bacteriana/microbiologia , Gravidez , Complicações Hematológicas na Gravidez/tratamento farmacológico , Complicações Infecciosas na Gravidez/tratamento farmacológico , Complicações Infecciosas na Gravidez/microbiologia , Resultado do Tratamento , UltrassonografiaRESUMO
UNLABELLED: aim: To study the factors influencing the results of treatment for candidemia (CE) in patients with blood system tumors. SUBJECTS AND METHODS: The investigation enrolled patients with hemoblastoses and CE. 30-day all-cause mortality was analyzed. RESULTS: In an 8-year period (2006-2013), CE was diagnosed in 55 patients (median age, 50 years); there was a preponderance of patients with lymphomas (47%) and acute leukemias (27%). The causative agents of CE were C. albicans (38%), C. parapsilosis (17%), C. krusei (11%), C. guilliermondii (11%), C. lusitaniae (6%), C. tropicalis (6%), C. glabrata (3%), C. famata (3%), C. pelliculosa (3%), and C. kefyr (2%). 30-day all-cause mortality was 43.6%. Recovery was statistically significantly more frequently seen following removal of a central venous catheter (67% versus 13%; p=0.004; odds ratio (OR), 14); after use of an antifungal drug on day 1 of isolation of Candida spp. from blood cultures (62% versus 13%; p=0.01; OR, 12); and that of echocandin as a first-line agent (86% versus 42%; p=0.005; OR, 8.4). The poor predictors were septic shock (5% recovery rate versus 86% in the patients without this factor; p<0.0001; OR, 0.01), granulocytopenia (42% versus 88%; p=0.001; OR, 0.1); use of amphotericin B as a first-line drug (26% versus 71%; p=0.002; OR, 0.15); hemoblastosis recurrence or resistance (39% versus 73%; p=0.01; OR, 0.24). Multivariate analysis showed the positive impact of antifungal administration on day 1 of isolation of Candida spp. from blood cultures on treatment results (p=0.03; OR, 27). CONCLUSION: High mortality rates were noted in the patients with hemoblastoses and CE. The recovery rates were statistically significantly higher after use of echinocandin as a first-line agent, after that of an antifungal agent on day 1 of positive blood cultures, after removal of a central venous catheter, and hemoblastosis remission.
Assuntos
Antifúngicos/uso terapêutico , Candida/isolamento & purificação , Candidemia/tratamento farmacológico , Neoplasias Hematológicas/complicações , Adolescente , Adulto , Idoso , Candidemia/complicações , Candidemia/diagnóstico , Feminino , Humanos , Masculino , Testes de Sensibilidade Microbiana , Pessoa de Meia-Idade , Estudos Retrospectivos , Fatores de Risco , Resultado do Tratamento , Adulto JovemRESUMO
AIM: To evaluate the efficiency of the treatment policy for patients with acute myeloid leukemia (AML) and hyperleukocytosis (HL), which is aimed at preventing rapid hypercytolysis and massive tumor lysis (cytolysis) syndrome and/or at reducing the degree of the latter at the start of induction polychemotherapy. SUBJECTS AND METHODS: In 2010 to 2014, the Hematology Research Center, Ministry of Health of Russia, treated 92 patients with AML, out of them 18 patients were found to have white blood cell counts of 100 to 408-10(9)/1 (median, 130-10(9)/l) at the onset of the disease. All the examinees received cytoreductive therapy with hydroxyurea and, in presence of leukostasis and/or leukocytosis (≥150-10(9)/1), with leukocytapheresis. In case of reduced leukocytosis, plasmapheresis was carried out to prevent (treat) cytolysis. Daunorubicin was injected on days 3-5 of the 7+3 induction cycle. RESULTS: The signs of leukostases were detected in more than half of the 18 patients with higher white blood cell counts: 13 (72%) with lung injury, including 5 of them with signs of respiratory distress syndrome, 6 (27.8%) with neurological symptomatology, 7 (38.9%) with disseminated intravascular coagulation syndrome, including 1 with intracranial hemorrhage. Cytoreduction therapy with hydroxyurea (10 mg/kg/day) was performed 1-5 (median 2) days before initiating induction chemotherapy in 17 patients; 9 patients underwent 1-2 (median 2) leukocytapheresis sessions. Sixteen patients received 1-4 (median 2) plasmapheresis sessions prior to and within the first days of the 7+3 treatment regimen. Daunorubicin (60 mg/m2) was administered to 16 patients on days 5-7 of the 7+3 cycle and to 2 patients on days 3-5 of the cycle. There were no signs of severe cytolysis with the development of multiple organ dysfunction in any patient. 50% (9/18) achieved remission after the first 7+3 cycle and 7 more examinees did after the second cycle. Thus, the remission rate was 89%; early mortality was 5.5% (1/18), three-year overall and relapse-free survival rates were 50%. CONCLUSION: Adequate cytoreductive and accompanying therapies for AML with HL can virtually completely prevent massive tumor cytolysis syndrome and early mortality during the first days of induction chemotherapy.
Assuntos
Quimioterapia de Indução/métodos , Leucemia Mieloide Aguda/terapia , Leucocitose/etiologia , Plasmaferese/métodos , Adolescente , Adulto , Feminino , Humanos , Leucemia Mieloide Aguda/sangue , Leucemia Mieloide Aguda/complicações , Contagem de Leucócitos , Leucocitose/sangue , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Resultado do Tratamento , Adulto JovemRESUMO
AIM: To evaluate the efficiency and toxicity of the intensive Burkitt's lymphoma (BL) therapy protocol BL-M-04. SUBJECTS AND METHODS: A total of 70 patients diagnosed with BL, including 45 men and 25 women whose age was 15 to 62 years (median age 31 years), were followed up in 2003 to 2014. Stage I (according to S. Murphy) was diagnosed in 4 (5.7%) patients; II in 9 (12.9%), III in 25 (35.7%), IV in 11 (15.7%), and Burkitt's leukemia in 21 (30%). There were tumor involvements of the bone marrow and central nervous system in 23 (32.9%) and 15 (21.4%) patients, respectively. B symptoms were detected in 56 (80%) patients; enhanced lactate dehydrogenase (LDH) activity was found in 50 (78.1%) out of 64 patients; moreover, in 34 (56.2%) out of 64 patients, LDH activity was more than twice as high as the reference values. The median LDH activity was 2398 (238-20,300) U/I. Acute renal failure at disease onset was identified in 17 (24.2%) patients; chemotherapy was initiated in 8 patients during renal replacement therapy. The treatment was performed using the BL-M-04±R protocol (4 successive blocks of A-C-A-C±R). Six blocks of A-C-A-C-A-C with rituximab has been carried out in patients with bone marrow involvement since 2011. RESULTS: Sixty-two (89%) patients achieved complete remission. At this time, 6 patients died from therapy complications during remission induction; 2 patients were observed to have disease progression; 3 developed disease recurrence (2 patients had early recurrence; 1 patient developed recurrence 2 years after treatment). Five-year overall survival (OS) was 85%; 5-year relapse-free survival (RFS) was 95%. The Cox multivariate regression analysis revealed that Burkitt's leukemia and bone marrow involvement were independent factors that influenced OS and RFS. The poor somatic status (3-4 ECOC scores versus 0-2 scores) proved to be statistically significant for OS rather than RFS. CONCLUSION: Despite the optimistic results obtained by our study group, there is a need to further improve BL treatment protocols and to elaborate novel approaches to therapy particularly for older patients and patients with Burkitt's leukemia.
Assuntos
Antineoplásicos/uso terapêutico , Linfoma de Burkitt/tratamento farmacológico , Previsões , Adolescente , Adulto , Intervalo Livre de Doença , Feminino , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Resultado do Tratamento , Adulto JovemRESUMO
The article deals with description of multipotent mesenchymal stromal cells (MMSCs), the mechanisms, the effects and uses of MMSCs for the treatment of sepsis and septic shock, and overview of the experimental data on the use of MMSCs in sepsis obtained in studies in cell cultures and in different models of sepsis in animals.