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1.
Clin Appl Thromb Hemost ; 26: 1076029620938149, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32677459

RESUMO

The novel coronavirus infection (COVID-19) is caused by the new coronavirus SARS-CoV-2 and is characterized by an exaggerated inflammatory response that can lead to severe manifestations such as adult respiratory syndrome, sepsis, coagulopathy, and death in a proportion of patients. Among other factors and direct viral effects, the increase in the vasoconstrictor angiotensin II, the decrease in the vasodilator angiotensin, and the sepsis-induced release of cytokines can trigger a coagulopathy in COVID-19. A coagulopathy has been reported in up to 50% of patients with severe COVID-19 manifestations. An increase in d-dimer is the most significant change in coagulation parameters in severe COVID-19 patients, and progressively increasing values can be used as a prognostic parameter indicating a worse outcome. Limited data suggest a high incidence of deep vein thrombosis and pulmonary embolism in up to 40% of patients, despite the use of a standard dose of low-molecular-weight heparin (LMWH) in most cases. In addition, pulmonary microvascular thrombosis has been reported and may play a role in progressive lung failure. Prophylactic LMWH has been recommended by the International Society on Thrombosis and Haemostasis (ISTH) and the American Society of Hematology (ASH), but the best effective dosage is uncertain. Adapted to the individual risk of thrombosis and the d-dimer value, higher doses can be considered, especially since bleeding events in COVID-19 are rare. Besides the anticoagulant effect of LMWH, nonanticoagulant properties such as the reduction in interleukin 6 release have been shown to improve the complex picture of coagulopathy in patients with COVID-19.


Assuntos
Anticoagulantes/uso terapêutico , Betacoronavirus , Infecções por Coronavirus/complicações , Pandemias , Pneumonia Viral/complicações , Trombofilia/etiologia , Trombose/prevenção & controle , Angiotensina II/metabolismo , Arteriopatias Oclusivas/sangue , Arteriopatias Oclusivas/epidemiologia , Arteriopatias Oclusivas/etiologia , Infecções por Coronavirus/sangue , Infecções por Coronavirus/epidemiologia , Síndrome da Liberação de Citocina/sangue , Síndrome da Liberação de Citocina/etiologia , Surtos de Doenças , Coagulação Intravascular Disseminada/etiologia , Coagulação Intravascular Disseminada/prevenção & controle , Produtos de Degradação da Fibrina e do Fibrinogênio/análise , Fibrinolíticos/uso terapêutico , Heparina de Baixo Peso Molecular/uso terapêutico , Humanos , Incidência , Inflamação , Pneumonia Viral/sangue , Pneumonia Viral/epidemiologia , Prognóstico , Embolia Pulmonar/etiologia , Embolia Pulmonar/prevenção & controle , Risco , Sepse/sangue , Sepse/complicações , Síndrome Respiratória Aguda Grave/sangue , Síndrome Respiratória Aguda Grave/complicações , Síndrome Respiratória Aguda Grave/epidemiologia , Trombofilia/sangue , Trombofilia/tratamento farmacológico , Trombose/sangue , Trombose/epidemiologia , Trombose/etiologia , Microangiopatias Trombóticas/etiologia , Microangiopatias Trombóticas/prevenção & controle , Ativador de Plasminogênio Tecidual/uso terapêutico
3.
Artigo em Inglês | MEDLINE | ID: mdl-32725056

RESUMO

Bothrops erythromelas are serpents that belong to the Viperidae family, which are the main species responsible for human snakebites in Ceara State, Northeast Brazil. Thrombotic microangiopathy (TMA) is an uncommon group of disorders characterized by microangiopathic hemolytic anemia (MAHA), thrombocytopenia and acute kidney injury (AKI), and occurrence after snakebites have been rarely reported. In this report, we described the case of a 57 year-old-man without comorbidities who was bitten by a Bothrops erythromelas on his right ankle. He presented with pain, edema and local bleeding. Symptomatology and laboratory tests were compatible with the diagnosis of TMA. He received specific antivenom and fluids replacement without any anaphylactic reaction. The conservative treatment was effective and there was no need for red blood cells transfusion or plasmapheresis. The aim of this report was to describe the first case of thrombotic microangiopathy following Bothrops erythromelas envenoming in the Northeast Brazil, providing insights about important mechanistic pathways of Bothrops snakebite-associated TMA and how to change the prognosis of the disease.


Assuntos
Bothrops , Mordeduras de Serpentes , Microangiopatias Trombóticas , Animais , Antivenenos/administração & dosagem , Brasil , Humanos , Masculino , Pessoa de Meia-Idade , Mordeduras de Serpentes/complicações , Microangiopatias Trombóticas/tratamento farmacológico , Microangiopatias Trombóticas/etiologia , Microangiopatias Trombóticas/patologia , Resultado do Tratamento
5.
Int Heart J ; 61(2): 409-412, 2020 Mar 28.
Artigo em Inglês | MEDLINE | ID: mdl-32173712

RESUMO

We report here a 70-year-old female patient with a history of breast cancer who presented with dyspnea that had lasted for 2 weeks following a long-distance trip by bus. She was at first suspected of having a pulmonary embolism given the typical presentation, elevated D-dimer level, and enlargement of the right-side heart. However, her systemic condition deteriorated despite the initiation of anti-coagulation therapy. Given the absence of a major thrombus in the pulmonary major arteries but multiple low perfusion lesions in the periphery of the lungs, refractoriness to conventional therapy, an increase in tumor markers, and anaplastic cells demonstrated by aspiration cytology from the pulmonary artery, we diagnosed her as pulmonary tumor thrombotic microangiopathy (PTTM). She died on day 23 due to respiratory failure despite administration of inotropes and prostaglandin I2. The patient had an obvious history of malignancy, but we should emphasize that PTTM can develop even in patients with early-stage or completely cured malignancies. Although an early and definite diagnosis of PTTM is currently challenging, an optimal diagnostic and therapeutic strategy is warranted.


Assuntos
Neoplasias da Mama/complicações , Embolia Pulmonar/diagnóstico , Microangiopatias Trombóticas/diagnóstico , Idoso , Diagnóstico Diferencial , Feminino , Humanos , Microangiopatias Trombóticas/etiologia , Microangiopatias Trombóticas/terapia
6.
Intern Med ; 59(1): 93-99, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-31902910

RESUMO

Atypical hemolytic uremic syndrome (aHUS) is an extremely rare condition caused by an excessive activation of the complement pathway based on genetic or acquired dysfunctions in complement regulation, leading to thrombotic microangiopathy (TMA). A complement-amplifying condition (CAC) can trigger aHUS occurrence along with complement abnormality. We herein report a case of severe TMA after laparoscopic myomectomy in a healthy woman. This case was eventually diagnosed as complement-mediated TMA secondary to surgical invasive stress as a CAC, with no definitive diagnosis of aHUS despite a genetic test. The patient fully recovered after several eculizumab administrations.


Assuntos
Anticorpos Monoclonais Humanizados/uso terapêutico , Laparoscopia/efeitos adversos , Hemorragia Pós-Operatória/complicações , Microangiopatias Trombóticas/tratamento farmacológico , Miomectomia Uterina/efeitos adversos , Adulto , Inativadores do Complemento/uso terapêutico , Feminino , Humanos , Doenças Raras , Microangiopatias Trombóticas/diagnóstico , Microangiopatias Trombóticas/etiologia
7.
PLoS One ; 15(1): e0227445, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-31923282

RESUMO

INTRODUCTION: Thrombotic microangiopathy (TMA) in post-transplant setting has heterogeneous clinical manifestations. METHODS: We retrospectively studied data of 89 patients with post-transplant TMA, which was characterized by thrombi in at least one glomerulus and/or arteriole. Systemic TMA was defined by thrombocytopenia and microangiopathic anemia and early onset TMA, when occurred less than 90 days post transplant. RESULTS: The cumulative incidence was 0.93%. The majority of the recipients were young (mean age 39 years), female (52%) and Caucasian (48%) with primary kidney disease of unknown etiology (37%). Early TMA occurred in 51% of the patients and systemic TMA, in 25%. Underlying precipitating factors were: infection (54%), acute rejection (34%), calcineurin inhibitor toxicity (13%) and pregnancy (3%). 18% of the patients had several triggers. Glomerular TMA was observed in 50% of the biopsies and endothelial cell activation, in 61%. The 1-year patient survival was 97% and corresponding graft survival, 66%. Allograft survival was inferior when acute antibody mediated rejection (ABMR) occurred (with 41%; without 70%, p = 0.01), however no differences were determined by hemolysis, time of onset, thrombi location or endothelial cell activation. CONCLUSIONS: Our results suggest that post-transplant TMA is a rare but severe condition, regardless of its clinical and histological presentation, mainly when associated to ABMR.


Assuntos
Transplante de Rim/efeitos adversos , Microangiopatias Trombóticas/etiologia , Adulto , Feminino , Rejeição de Enxerto/complicações , Rejeição de Enxerto/imunologia , Humanos , Incidência , Infecções/complicações , Nefropatias/complicações , Nefropatias/terapia , Masculino , Pessoa de Meia-Idade , Gravidez , Estudos Retrospectivos , Microangiopatias Trombóticas/patologia , Transplante Homólogo/efeitos adversos
9.
Pediatr Blood Cancer ; 67(3): e28070, 2020 03.
Artigo em Inglês | MEDLINE | ID: mdl-31774252

RESUMO

BACKGROUND: Transplant-associated thrombotic microangiopathy (TA-TMA) occurs after hematopoietic stem cell transplantation (HSCT) and is characterized by microvascular thrombosis and end-organ injury particularly of the kidneys. TA-TMA is challenging to diagnose and treat, which can lead to long-term complications and death in patients with severe disease. Studies have shown that genetic abnormalities of the alternative complement pathway (AP) are associated with TA-TMA. We hypothesized that patients with TA-TMA may generate elevated levels of the AP activation product, Ba, compared with HSCT patients without TA-TMA. PROCEDURE: We longitudinally measured plasma levels of complement activation products C3a, Ba, and C5a in 14 HSCT patients: 7 with TA-TMA and 7 without TA-TMA. We assessed renal function by calculating estimated glomerular filtration rate (eGFR) and correlated the extent of AP activation with renal dysfunction in both patient populations. RESULTS: The median days from HSCT to study enrollment were 154 (39-237) in the TA-TMA group and 84 (39-253) in the HSCT group without TA-TMA. Median Ba levels (ng/mL) at enrollment were 1096.9 (826.5-1562.0) in the TA-TMA group and 725.7 (494.7-818.9) in the HSCT group without TA-TMA (P = 0.007). Over the study duration, Ba levels inversely correlated with eGFR. There were no differences in C3a, C5a, or sC5b9 levels between the two populations at any measured interval. CONCLUSIONS: We conclude in this preliminary study that Ba protein may serve as a marker for TA-TMA, and furthermore, that components generated in the early phase of AP activation may be involved in the pathogenesis of renal endothelial injury in TA-TMA.


Assuntos
Biomarcadores/metabolismo , Complemento C3b/metabolismo , Fator B do Complemento/metabolismo , Via Alternativa do Complemento , Transplante de Células-Tronco Hematopoéticas/efeitos adversos , Microangiopatias Trombóticas/diagnóstico , Adolescente , Adulto , Estudos de Casos e Controles , Criança , Pré-Escolar , Ativação do Complemento , Feminino , Seguimentos , Humanos , Estudos Longitudinais , Masculino , Prognóstico , Microangiopatias Trombóticas/etiologia , Microangiopatias Trombóticas/metabolismo , Adulto Jovem
10.
Rinsho Ketsueki ; 60(11): 1560-1566, 2019.
Artigo em Japonês | MEDLINE | ID: mdl-31839635

RESUMO

In March 2009, a 17-year-old woman was first diagnosed with acute myelogenous leukemia and myelodysplasia-related changes. She underwent chemotherapy and allogeneic hematopoietic stem cell transplantation, which resulted in complete remission. However, she experienced relapse, and remission was achieved each time with repeated transplantation. In September 2014, a human leukocyte antigen (HLA)-haploidentical transplantation, which was the fifth allogeneic transplantation, was performed to treat the third relapse. Platelet transfusion refractoriness, hemolytic anemia with schistocytes, and renal dysfunction were observed from approximately the day of engraftment; therefore, transplantation-associated thrombotic microangiopathy (TA-TMA) was diagnosed. Recombinant human soluble thrombomodulin (rTM) was administered, and fresh-frozen plasma (FFP) was infused; this resulted in gradual improvement of TA-TMA. Treatment with rTM and FFP was discontinued on the 70th day after transplantation. Because the HLA-haploidentical transplantation was the fifth allogeneic transplantation, the risk of aggravation of TA-TMA was very high. Combined treatment with rTM and FFP, however, resulted in improvement of TA-TMA. Further investigation of similar cases is necessary for clarifying the usefulness of rTM for TA-TMA.


Assuntos
Transplante de Células-Tronco Hematopoéticas , Trombomodulina/uso terapêutico , Microangiopatias Trombóticas , Transplante Haploidêntico/efeitos adversos , Adolescente , Feminino , Antígenos HLA , Transplante de Células-Tronco Hematopoéticas/efeitos adversos , Humanos , Microangiopatias Trombóticas/tratamento farmacológico , Microangiopatias Trombóticas/etiologia
11.
Transplant Proc ; 51(9): 3159-3162, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31711585

RESUMO

BACKGROUND: Allogenic hematopoietic stem cell transplantation may be the best currently available method to treat relapsed hemophagocytic lymphohistiocytosis (HLH) related to Epstein-Barr virus. The high rate of transplantation-related complications was initially the main obstacle preventing the wider adoption of this protocol; however, the previously more common complications, such as infection and graft failure, have fallen to very low levels with the development of new drugs and methods. Some other complications, such as veno-occlusive disease and transplantation associated thrombotic microangiopathy, are rare after allogenic hematopoietic stem cell transplantation, but the morbidity and mortality associated with them are very high. CASE PRESENTATION: A patient with relapsed HLH related to Epstein-Barr virus showed the sequential severe complications of veno-occlusive disease, transplantation-associated thrombotic microangiopathy, and acute graft-vs-host disease after haploidentical transplantation. This patient was successfully treated by stopping administration of calcineurin inhibitors and instead treating with defibrotide, rituximab, CD25 monoclonal antibody, atorvastatin calcium tablets, methylprednisolone, budesonide, continuous plasma exchange, and bedside ultrafiltration. At the last follow-up, the patient had been living disease free for 2 years without any other complications. CONCLUSION: Epstein-Barr virus related-HLH patients have severe clinical features and currently poor prognosis. Allogenic hematopoietic stem cell transplantation may be the best way to treat this disease; however, the management of related complications is vital in the improvement of long-term survival.


Assuntos
Doença Enxerto-Hospedeiro/etiologia , Transplante de Células-Tronco Hematopoéticas/efeitos adversos , Hepatopatia Veno-Oclusiva/etiologia , Linfo-Histiocitose Hemofagocítica/cirurgia , Microangiopatias Trombóticas/etiologia , Criança , Infecções por Vírus Epstein-Barr/complicações , Feminino , Doença Enxerto-Hospedeiro/terapia , Transplante de Células-Tronco Hematopoéticas/métodos , Hepatopatia Veno-Oclusiva/terapia , Herpesvirus Humano 4 , Humanos , Linfo-Histiocitose Hemofagocítica/virologia , Recidiva , Microangiopatias Trombóticas/terapia
12.
BMJ Case Rep ; 12(11)2019 Nov 28.
Artigo em Inglês | MEDLINE | ID: mdl-31780612

RESUMO

A 48-year-old woman presented with severe abdominal pain, bilious vomiting and bloody diarrhoea for 1 day. On examination, she was haemodynamically unstable, febrile and clinically had an acute surgical abdomen. She had markedly raised inflammatory markers, neutrophils and deranged renal function. A CT abdominal scan revealed severe colitis and thickening throughout the length of the colon. The patient was stabilised and underwent emergency laparotomy resulting in total colectomy and end ileostomy formation. Postoperatively, she required several units of human albumin solution, red blood cell transfusions and octaplex (prothrombin complex) to prevent further bleeding. An inpatient haematology review revealed a hypocomplementaemia (C3/C4), low immunoglobulin (IgG, IgM, IgA) and peripheral blood films revealed schistocytosis indicating microangiopathic haemolytic anaemia. Bowel histology supported this, demonstrating circumferential lymphocytic phlebitis with thrombi and mucosal haemorrhage, necrosis and ulceration. The patient went on to suffer multiple ischaemic strokes before undergoing plasmapheresis, subsequent rehabilitation and making a successful recovery.


Assuntos
Microangiopatias Trombóticas/diagnóstico , Abdome Agudo/etiologia , Feminino , Humanos , Pessoa de Meia-Idade , Microangiopatias Trombóticas/complicações , Microangiopatias Trombóticas/etiologia
13.
Rheumatology (Oxford) ; 58(12): 2099-2106, 2019 12 01.
Artigo em Inglês | MEDLINE | ID: mdl-31566243

RESUMO

Categorization of scleroderma renal crisis (SRC) as hypertensive or normotensive can potentially overlook the underlying pathophysiology that might be unique in each patient, as they often exhibit a mixture of distinct pathological characteristics of narrowly defined SRC (nd-SRC) and systemic sclerosis associated thrombocytic micro-angiopathy (SSc-TMA). In this review, we provide evidence suggesting that better categorization of patients presenting with certain clinical features of both nd-SRC and TMA will improve treatment approaches. Based on our clinical experience and literature review, distinguishing between nd-SRC and SSc-TMA is important because the association of SSc-TMA with prior steroid administration and poor prognosis was stronger than that of nd-SRC. Although the two pathological entities cannot be easily distinguished based on blood pressure, we suggest that the detailed clinical course is helpful. Typically, nd-SRC exhibits prominently elevated blood pressure and worsening of renal function initially, followed by mild thrombocytopenia. Conversely, SSc-TMA presents first with severe thrombocytopenia, followed by elevated blood pressure and renal function deterioration. The degree of involvement in each pathological condition should be considered for determination of appropriate therapeutic interventions and prognostic prediction.


Assuntos
Nefropatias/classificação , Escleroderma Sistêmico/metabolismo , Microangiopatias Trombóticas/metabolismo , Idoso , Creatinina/metabolismo , Feminino , Hematúria , Humanos , Hipertensão/etiologia , Hipertensão/fisiopatologia , Nefropatias/etiologia , Nefropatias/metabolismo , Nefropatias/fisiopatologia , Masculino , Pessoa de Meia-Idade , Proteinúria , Escleroderma Sistêmico/complicações , Escleroderma Sistêmico/fisiopatologia , Trombocitopenia/sangue , Trombocitopenia/etiologia , Microangiopatias Trombóticas/etiologia , Microangiopatias Trombóticas/fisiopatologia
14.
Int J Hematol ; 110(5): 529-532, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31586304

RESUMO

Transplant-associated thrombotic microangiopathy (TA-TMA) is a severe complication of allogeneic hematopoietic cell transplantation (allo-HCT) with multisystem involvement. Cases of TMA in the intestinal vasculature (intestinal TMA/iTMA) have been reported. We hypothesized that iTMA is a distinct entity from TA-TMA. To test this hypothesis, we prospectively recruited allo-HCT recipients with an indication for endoscopy. Among 20 patients, histological features of iTMA, including loss of glands, total denudation of mucosa, apoptosis and detachment of endothelial cells, mucosal hemorrhage, intraluminal fibrin and microthrombi were found in six. Only 2/6 were classified as GVHD/TA-TMA, while the other 4 as GVHD/no TA-TMA. Gastro-intestinal symptoms were similar between the patients with or without iTMA. With a median follow-up of 11.1 (2.1-67.5) months, 1-year overall survival was 22.2% for iTMA, 55% for GVHD and 60% for TA-TMA. On multivariate analysis, independent unfavorable predictors of OS were iTMA (p = 0.048), HLA mismatched donors (p = 0.008) and gastro-intestinal bleeding (p = 0.021). In conclusion, iTMA emerges as a novel distinct entity in patients with GVHD and/or TA-TMA. Distinct histological features may be useful in differential diagnosis of these severe HCT complications. The higher mortality rates of iTMA than TA-TMA highlight the need for further investigation of this condition.


Assuntos
Doença Enxerto-Hospedeiro/etiologia , Transplante de Células-Tronco Hematopoéticas/efeitos adversos , Enteropatias/etiologia , Microangiopatias Trombóticas/etiologia , Adulto , Células Endoteliais/patologia , Feminino , Hemorragia Gastrointestinal/complicações , Doença Enxerto-Hospedeiro/diagnóstico , Doença Enxerto-Hospedeiro/mortalidade , Doença Enxerto-Hospedeiro/patologia , Humanos , Enteropatias/diagnóstico , Enteropatias/mortalidade , Enteropatias/patologia , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos , Trombose/etiologia , Microangiopatias Trombóticas/diagnóstico , Microangiopatias Trombóticas/mortalidade , Microangiopatias Trombóticas/patologia , Transplante Homólogo/efeitos adversos , Adulto Jovem
15.
BMJ Case Rep ; 12(9)2019 Sep 16.
Artigo em Inglês | MEDLINE | ID: mdl-31527221

RESUMO

A 42-year-old woman was referred from a primary care centre for severe hypertension, stage 3A chronic kidney disease and proteinuria. This was associated with a significant obstetric history of pre-eclampsia during her previous two pregnancies. Secondary hypertension was suspected and autoimmune workup was positive for anticardiolipin IgG and lupus anticoagulant. A renal biopsy showed evidence of chronic thrombotic microangiopathy, with electron microscopy features suggestive of fibrillar glomerulonephritis. The diagnosis of antiphospholipid syndrome with antiphospholipid-associated nephropathy was made. She was started on anticoagulation with warfarin, and her hypertension was controlled with lisinopril and amlodipine with subsequent improvement in proteinuria. She remains on regular follow-up to monitor for possible development of malignancy or connective tissue disease.


Assuntos
Síndrome Antifosfolipídica/complicações , Hipertensão/etiologia , Nefropatias/etiologia , Microangiopatias Trombóticas/etiologia , Varfarina/uso terapêutico , Adulto , Anlodipino/uso terapêutico , Anticoagulantes/uso terapêutico , Anti-Hipertensivos/uso terapêutico , Diagnóstico Diferencial , Quimioterapia Combinada , Feminino , Humanos , Hipertensão/tratamento farmacológico , Lisinopril/uso terapêutico , Microangiopatias Trombóticas/tratamento farmacológico
16.
BMJ Case Rep ; 12(9)2019 Sep 04.
Artigo em Inglês | MEDLINE | ID: mdl-31488441

RESUMO

Pulmonary tumour thrombotic microangiopathy (PTTM) and pulmonary tumour emboli (PTE) are distinct but related complications of malignancy. The incidence of each is exceedingly rare, unfortunately often being diagnosed postmortem. Patients with PTTM and PTE typically present with dyspnoea associated with a rapid onset of hypoxia due to pulmonary hypertension (PH), and respiratory failure that is almost certain to be fatal. The prognosis is grim due to the rapidity of the clinical decline and difficulty in establishing an ante-mortem diagnosis. We present a case of new-onset severe PH in a young woman with a recently discovered breast mass. She presented with shortness of breath and experienced rapid deterioration of her cardiopulmonary status which we attributed to PTTM. With early initiation of chemotherapy, systemic steroids and sildenafil, the patient dramatically improved. Case reports have identified early use of steroids, phosphodiesterase inhibitors and other alternative therapies as providing possible benefit in PTTM.


Assuntos
Neoplasias da Mama/complicações , Hipertensão Pulmonar/etiologia , Microangiopatias Trombóticas/etiologia , Adulto , Neoplasias da Mama/diagnóstico , Neoplasias da Mama/terapia , Quimiorradioterapia , Feminino , Humanos
18.
Clin Nephrol ; 92(3): 155-158, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31262399

RESUMO

BACKGROUND: Prostate cancer is the second most common solid tumor leading to membranous nephropathy (MN). Thrombotic microangiopathy (TMA) has been reported to be related to prostate cancer. Nonetheless, the association between prostate cancer and MN and TMA has not been well established. CASE REPORT: A 73-year-old man presented with nephritic syndrome 40 days after implantation of iodine-125 seed for stage II T2N0M0 prostatic carcinoma. The prostatic-specific antigen (PSA) was normalized, and the tumor disappeared after the initial brachytherapy. The circulating autoantibody to phospholipase A2 receptor (PLA2R) and thrombospondin type 1 domain containing 7A (THSD7A) was undetectable. Kidney biopsy revealed MN and TMA in glomerulus. Staining of PLA2R, THSD7A, prostate-specific membrane antigen, and prostate acid phosphatase in glomeruli were all negative. The diagnosis of MN and TMA was made, and a combination of steroid therapy and tacrolimus was prescribed. Two weeks after immunosuppressive treatment with prednisone 30 mg/d and tacrolimus 2 mg/d, the patient achieved partial remission in terms of proteinuria. CONCLUSION: This case study was the first report of MN with TMA as manifestations in patients with prostate cancer after I-125 seeds implantation. We hypothesize that prostate cancer may cause MN and TMA, and the mechanism behind this relationship merits further study.
.


Assuntos
Braquiterapia , Glomerulonefrite Membranosa/etiologia , Radioisótopos do Iodo/uso terapêutico , Neoplasias da Próstata/complicações , Microangiopatias Trombóticas/etiologia , Idoso , Glomerulonefrite Membranosa/tratamento farmacológico , Humanos , Imunossupressores/uso terapêutico , Masculino , Neoplasias da Próstata/patologia , Neoplasias da Próstata/radioterapia , Proteinúria/etiologia , Receptores da Fosfolipase A2/análise , Trombospondinas/análise , Microangiopatias Trombóticas/tratamento farmacológico
19.
Pediatr Blood Cancer ; 66(10): e27912, 2019 10.
Artigo em Inglês | MEDLINE | ID: mdl-31264793

RESUMO

Preexisting endothelial dysfunction and vascular injury sustained during allogeneic hematopoietic cell transplantation (HCT) increases risk for endothelial injury-related complications such as posterior reversible encephalopathy syndrome (PRES) and transplant-associated thrombotic microangiopathy (TA-TMA) in patients with sickle cell disease (SCD). We report two patients with SCD who developed PRES following allogeneic HCT. In both patients, PRES-related symptoms resolved only after a diagnosis of TA-TMA was established and eculizumab therapy was initiated. Renal manifestations at diagnosis included non-nephrotic range proteinuria and hypertension. This report highlights the importance of screening PRES-affected SCD HCT recipients for TA-TMA as usual treatment strategies may be inadequate.


Assuntos
Anemia Falciforme/terapia , Anticorpos Monoclonais Humanizados/uso terapêutico , Inativadores do Complemento/uso terapêutico , Transplante de Células-Tronco Hematopoéticas/efeitos adversos , Síndrome da Leucoencefalopatia Posterior/etiologia , Microangiopatias Trombóticas/tratamento farmacológico , Adulto , Criança , Feminino , Humanos , Masculino , Microangiopatias Trombóticas/etiologia
20.
Thromb Haemost ; 119(9): 1433-1440, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31266080

RESUMO

Transplant-associated thrombotic microangiopathy (TA-TMA) is a severe and life-threatening complication of hematopoietic cell transplantation (HCT) that often coincides with graft-versus-host-disease (GVHD). Although endothelial damage seems to be the common denominator for both disorders, the role of complement system, neutrophils, and coagulation has not been clarified. In an effort to distinguish the pathogenesis of TA-TMA from GVHD, we evaluated markers of complement activation, neutrophil extracellular trap (NET) release, endothelial damage, and activation of coagulation cascade in the circulation of patients with these two disorders, as well as control HCT recipients without TA-TMA or GVHD. We observed that the terminal complement product C5b-9 levels, the levels of markers of NET formation, and thrombin-antithrombin complex levels were significantly increased in the TA-TMA group compared with patients without complications, whereas there was no significant difference between the GVHD and the control group. On the other hand, the levels of circulating thrombomodulin, an endothelial damage marker, were significantly increased in both TA-TMA and GVHD patients. These findings propose a role for the interplay between complement system, neutrophil activation through NET release, and activation of the coagulation cascade in TA-TMA.


Assuntos
Endotélio Vascular/patologia , Armadilhas Extracelulares/metabolismo , Doença Enxerto-Hospedeiro/imunologia , Transplante de Células-Tronco Hematopoéticas , Neutrófilos/imunologia , Complicações Pós-Operatórias/imunologia , Microangiopatias Trombóticas/imunologia , Adulto , Idoso , Antitrombina III , Biomarcadores/metabolismo , Coagulação Sanguínea , Estudos de Casos e Controles , Ativação do Complemento , Complexo de Ataque à Membrana do Sistema Complemento/metabolismo , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Peptídeo Hidrolases/sangue , Trombomodulina/sangue , Microangiopatias Trombóticas/etiologia , Adulto Jovem
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