RESUMO
Ascites is rare in patients with multiple myeloma (MM). It may be due to diverse mechanisms, most frequently because of an increased permeability of the peritoneum or because of portal hypertension due to liver infiltration. Myelomatous ascites occurs more frequently in patients having Ig-G or Ig-A paraprotein and their prognosis is poor. It is submitted the case of a female patient aged 50 years with IgA-kappa MM, who evolved with cardiac failure (CF), plasma cells leukemia and ascites of mixed cause, because of peritoneal infiltrate of myelomatous cells, hepatic compromise and CF. A review of the different causes of ascites in patients with MM is performed. There are also summarized all myelomatous ascites cases published in the literature. Our report presents the first case of myelomatous ascites in a patient with plasma cells leukemia.
Assuntos
Ascite/etiologia , Mieloma Múltiplo/complicações , Feminino , Humanos , Pessoa de Meia-IdadeAssuntos
Humanos , Fator VIII/análise , Fator VIII/genética , Fator de von Willebrand/análise , Fator de von Willebrand/genética , Sistema ABO de Grupos Sanguíneos/genética , Sistema ABO de Grupos Sanguíneos/imunologia , Antígenos/sangue , Preservação de Sangue/métodos , Criopreservação , Fator de von Willebrand/metabolismo , Genótipo , Valores de ReferênciaAssuntos
Humanos , Adulto , Feminino , Trombocitopenia/complicações , Trombocitopenia/diagnóstico , Trombocitopenia/etiologia , Veia Porta/anormalidades , Veia Porta/diagnóstico por imagem , Diagnóstico por Imagem , Análise Química do Sangue , Ecocardiografia Doppler em Cores , Diagnóstico DiferencialAssuntos
Humanos , Adulto , Feminino , Trombocitopenia/complicações , Trombocitopenia/diagnóstico , Trombocitopenia/etiologia , Veia Porta/anormalidades , Veia Porta/diagnóstico por imagem , Diagnóstico por Imagem , Análise Química do Sangue , Ecocardiografia Doppler em Cores , Diagnóstico DiferencialRESUMO
La subunidad A del Factor XIII (FXIII), parte activa del FXIII, es sintetizada casi exclusivamente por megacariocitos y precursores de monocitos-macrófagos. Determinándose la actividad del FXIII en pacientes sometidos a transplante de médula ósea autólogo (TAMO) y alegeneico (TMO). En 52 pacientes, 30 TAMO y 22 TMO, la actividad del FXIII fue estudiada en días fijos (basal, mitad de régimen condicionante, día 0, día +7, día +15 y día +30). Un descenso del FXIII fue encontrado en todos los casos, cuando se comparó con los niveles basales, alcalzando el nadir de la actividad al día +7. La caída del FXIII fué significativamente más pronunciada en TMO que en TAMO. Se demostró correlación entre la actividad del FXIII y el tiempo de recuperación hematopoyética. Siete de los 52 ptes. (13, 4 porciento) tuvieron niveles de actividad del FXIII inferiores al 10 porciento, sólo 3 sufrieron sangrados asociados. En TMO, los niveles de PAI estaban significativamente aumentados en los días +7, +15 y +30. En conclusión, la actividad del FXIII está siempre desminuída post-trasplante y el restablecimiento de los niveles basales se relaciona directamente con la velocidad de reconstitución hematopoyética. Diferencias en los tiempos de "engraftment" podrían explicar las diferencias encontradas entre TAMO y TMO. Finalmente, la ausencia de sangrado en ptes. con actividad del FXIII por debajo de niveles hemostáticos(<10 porciento) podría deberse a hipofibrinolisis concominante asociada a aumento del PAI (AU)
Assuntos
Transplante de Medula Óssea , Fator XIIIRESUMO
La subunidad A del Factor XIII (FXIII), parte activa del FXIII, es sintetizada casi exclusivamente por megacariocitos y precursores de monocitos-macrófagos. Determinándose la actividad del FXIII en pacientes sometidos a transplante de médula ósea autólogo (TAMO) y alegeneico (TMO). En 52 pacientes, 30 TAMO y 22 TMO, la actividad del FXIII fue estudiada en días fijos (basal, mitad de régimen condicionante, día 0, día +7, día +15 y día +30). Un descenso del FXIII fue encontrado en todos los casos, cuando se comparó con los niveles basales, alcalzando el nadir de la actividad al día +7. La caída del FXIII fué significativamente más pronunciada en TMO que en TAMO. Se demostró correlación entre la actividad del FXIII y el tiempo de recuperación hematopoyética. Siete de los 52 ptes. (13, 4 porciento) tuvieron niveles de actividad del FXIII inferiores al 10 porciento, sólo 3 sufrieron sangrados asociados. En TMO, los niveles de PAI estaban significativamente aumentados en los días +7, +15 y +30. En conclusión, la actividad del FXIII está siempre desminuída post-trasplante y el restablecimiento de los niveles basales se relaciona directamente con la velocidad de reconstitución hematopoyética. Diferencias en los tiempos de "engraftment" podrían explicar las diferencias encontradas entre TAMO y TMO. Finalmente, la ausencia de sangrado en ptes. con actividad del FXIII por debajo de niveles hemostáticos(<10 porciento) podría deberse a hipofibrinolisis concominante asociada a aumento del PAI
Assuntos
Transplante de Medula Óssea , Fator XIIIRESUMO
In a previous study we demonstrated that the incidence of fibroblast colony-forming units (CFU-F) was very low in bone marrow primary cultures from the majority of untreated advanced non-small lung cancer patients (LCP) compared to normal controls (NC). For this reason, we studied the ability of bone marrow stromal cells to achieve confluence in primary cultures and their proliferative capacity following four continuous subcultures in consecutive untreated LCP and NC. We also evaluated the production of interleukin-1ß (IL-1ß) and prostaglandin E2 (PGE2) by pure fibroblasts. Bone marrow was obtained from 20 LCP and 20 NC. A CFU-F assay was used to investigate the proliferative and confluence capacity. Levels of IL-1ß and PGE2 in conditioned medium (CM) of pure fibroblast cultures were measured with an ELISA kit and RIA kit, respectively. Only fibroblasts from 6/13 (46 percent) LCP confluent primary cultures had the capacity to proliferate following four subcultures (NC = 100 percent). Levels of spontaneously released IL-1ß were below 10 pg/ml in the CM of LCP, while NC had a mean value of 1,217 + or - 74 pg/ml. In contrast, levels of PGE2 in these CM of LCP were higher (77.5 + or - 23.6 pg/ml) compared to NC (18.5 + or - 0.9 pg/ml). In conclusion, bone marrow fibroblasts from LCP presented a defective proliferative and confluence capacity, and this deficiency may be associated with the alteration of IL-1ß and PGE2 production
Assuntos
Humanos , Masculino , Feminino , Adulto , Pessoa de Meia-Idade , Carcinoma Pulmonar de Células não Pequenas , Células da Medula Óssea/patologia , Fibroblastos , Neoplasias Pulmonares , Estudos de Casos e Controles , Células da Medula Óssea/química , Ensaio de Unidades Formadoras de Colônias , Meios de Cultivo Condicionados , Dinoprostona , Ensaio de Imunoadsorção EnzimáticaRESUMO
In a previous study we demonstrated that the incidence of fibroblast colony-forming units (CFU-F) was very low in bone marrow primary cultures from the majority of untreated advanced non-small lung cancer patients (LCP) compared to normal controls (NC). For this reason, we studied the ability of bone marrow stromal cells to achieve confluence in primary cultures and their proliferative capacity following four continuous subcultures in consecutive untreated LCP and NC. We also evaluated the production of interleukin-1beta (IL-1beta) and prostaglandin E2 (PGE2) by pure fibroblasts. Bone marrow was obtained from 20 LCP and 20 NC. A CFU-F assay was used to investigate the proliferative and confluence capacity. Levels of IL-1beta and PGE2 in conditioned medium (CM) of pure fibroblast cultures were measured with an ELISA kit and RIA kit, respectively. Only fibroblasts from 6/13 (46%) LCP confluent primary cultures had the capacity to proliferate following four subcultures (NC = 100%). Levels of spontaneously released IL-1beta were below 10 pg/ml in the CM of LCP, while NC had a mean value of 1,217 +/- 74 pg/ml. In contrast, levels of PGE2 in these CM of LCP were higher (77.5 +/- 23.6 pg/ml) compared to NC (18.5 +/- 0.9 pg/ml). In conclusion, bone marrow fibroblasts from LCP presented a defective proliferative and confluence capacity, and this deficiency may be associated with the alteration of IL-1beta and PGE2 production.
Assuntos
Células da Medula Óssea/patologia , Carcinoma Pulmonar de Células não Pequenas/patologia , Fibroblastos/patologia , Neoplasias Pulmonares/patologia , Adulto , Células da Medula Óssea/química , Estudos de Casos e Controles , Ensaio de Unidades Formadoras de Colônias , Meios de Cultivo Condicionados , Dinoprostona/análise , Ensaio de Imunoadsorção Enzimática , Feminino , Humanos , Masculino , Pessoa de Meia-IdadeRESUMO
Severe aplastic anemia is a hematological disease with a high mortality rate, for which bone marrow transplantation is the treatment of choice, specially in children and young adults. The number of transfusions undergone before the transplant is the most important factor to predict the possibility of graft failure. Twenty patients with severe aplastic anemia, most of them already multiple transfused, were transplanted utilizing cyclophosphamide combined with antilymphocyte globulin as a conditioning regiment. All the evaluable patients engrafted and there were no episodes of graft failure. Three patients died, and 17 (85%) are alive with hematopoietic recovery at a median of 27.7 months post-transplant. Bone marrow transplantation was an excellent therapeutic option in this series of patients with severe aplastic anemia and the conditioning regiment appeared to be sufficiently myeloablative and immunosuppressive to avoid early or late graft failure.
Assuntos
Anemia Aplástica/terapia , Soro Antilinfocitário/uso terapêutico , Transplante de Medula Óssea , Ciclofosfamida/uso terapêutico , Imunossupressores/uso terapêutico , Condicionamento Pré-Transplante , Adolescente , Adulto , Criança , Pré-Escolar , Intervalo Livre de Doença , Quimioterapia Combinada , Feminino , Doença Enxerto-Hospedeiro/tratamento farmacológico , Doença Enxerto-Hospedeiro/prevenção & controle , Humanos , Masculino , Índice de Gravidade de DoençaRESUMO
We report our experience with total lymphoid irradiation (TLI) in three patients with GVHD which had failed to respond to standard drug treatment. The clinical manifestations of GVHD markedly improved with TLI treatment.
Assuntos
Doença Enxerto-Hospedeiro/radioterapia , Tecido Linfoide/efeitos da radiação , Adolescente , Adulto , Transplante de Medula Óssea/efeitos adversos , Doença Crônica , Resistência a Medicamentos , Feminino , Doença Enxerto-Hospedeiro/tratamento farmacológico , Doença Enxerto-Hospedeiro/etiologia , Humanos , Leucemia Mieloide de Fase Crônica/cirurgia , Masculino , Leucemia-Linfoma Linfoblástico de Células Precursoras/cirurgiaRESUMO
Joints and larynx are uncommonly involved by non-Hodgkin's lymphoma (NHL). Synovial involvement has been reported in only 7 cases, mainly located in the knees. When this is the first location of NHL it is usually misdiagnosed. The treatment of choice is local radiotherapy followed by systemic chemotherapy. Laryngeal lymphoma can be either primary or forming part of multifocal disease. The prognosis of the primary form is usually good only with radiotherapy, whereas the prognosis of the laryngeal location of advanced disease is rather poor. The symptoms include dysphonia and slowly progressive dyspnea. A case of NHL is presented who showed initial arthritis of the knee, later evolving into severe laryngeal obstruction, an association not previously reported.
Assuntos
Artrite/etiologia , Laringoestenose/etiologia , Linfoma não Hodgkin/complicações , Neoplasias de Tecidos Moles/complicações , Doença Aguda , Obstrução das Vias Respiratórias/etiologia , Humanos , Masculino , Pessoa de Meia-IdadeRESUMO
Thrombotic thrombocytopenic purpura (TTP) is a rare disorder of unknown etiology, clinically characterized by a diagnostic pentad (thrombocytopenia, microangiopathic hemolytic anemia, neurologic signs and symptoms, fever and renal damage). Recent reports in the medical literature have described its association with the human immunodeficiency virus (HIV). We report such a case in a woman admitted with TTP in whom HIV seropositivity was found. The histopathologic findings in biopsies and autopsy confirmed the clinical diagnosis of TTP: disseminated microthrombosis in arterioles and capillaries.
Assuntos
Soropositividade para HIV/complicações , Púrpura Trombocitopênica Trombótica/complicações , Biópsia , Feminino , Humanos , Linfonodos/patologia , Pessoa de Meia-Idade , Púrpura Trombocitopênica Trombótica/patologia , Pele/patologiaRESUMO
Thrombotic thrombocytopenic purpura (TTP) is a rare disorder of unknown etiology, clinically characterized by a diagnostic pentad (thrombocytopenia, microangiopathic hemolytic anemia, neurologic signs and symptoms, fever and renal damage). Recent reports in the medical literature have described its association with the human immunodeficiency virus (HIV). We report such a case in a woman admitted with TTP in whom HIV seropositivity was found. The histopathologic findings in biopsies and autopsy confirmed the clinical diagnosis of TTP: disseminated microthrombosis in arterioles and capillaries.
RESUMO
We report results of allogeneic bone marrow transplantation in an 8-year-old boy with acute megakaryoblastic leukemia characterized by intense fibrosis together with 20% blast cells in the bone marrow, who was transplanted without preceding chemotherapy for remission induction. Conditioning comprised cytosine arabinoside, cyclophosphamide and total body irradiation. The donor was his HLA-identical sister. The patient is well with minor chronic graft-versus-host disease and normal hematologic values 670 days post-transplant.