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2.
Ceska Gynekol ; 85(1): 18-28, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32414281

RESUMO

OBJECTIVE: The aim of this study is to draw attention to a nosological unit called thrombotic microangiopathy (TMA). This syndrome represents a serious pathological condition characterized by microangiopathic haemolytic anemia (MAHA), thrombocytopenia and various organ dysfunction. Patients are most often presented with symptoms of the HELLP syndrome but if the clinical picture is not restituted within 48-72 hours after delivery, other TMAs should be considered. SETTING: Department of Obstetrics and Gynecology, 1st Medical Faculty and General Teaching Hospital Prague; Clinic of Nephrology, 1st Medical Faculty and General Teaching Hospital Prague; Department of Obstetrics and Gynecology, Regional Hospital Kolín. DESIGN: Review article and case reports. METHODS: Review of the literature and description of two cases of TMA. CONCLUSION: The authors present a basic overview of the issue of TMA, which requires interdisciplinary cooperation of obstetricians, anesthesiologists, nephrologists and hematologists. In the second part of the article, we present two TMA case reports and finally show the differential diagnostic and therapeutic scheme as agreed by the authorities in the field.


Assuntos
Complicações Cardiovasculares na Gravidez/diagnóstico , Microangiopatias Trombóticas/diagnóstico , Feminino , Humanos , Equipe de Assistência ao Paciente , Gravidez , Complicações Cardiovasculares na Gravidez/terapia , Púrpura Trombocitopênica Trombótica , Microangiopatias Trombóticas/terapia
3.
Ceska Gynekol ; 85(1): 30-34, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32414282

RESUMO

OBJECTIVE: Case of a primigravid woman who suffered from severe PTMS (postpartum thrombotic microangiopathy syndrome) in the 26th week of pregnancy. DESIGN: Case report. SETTING: Department of Gynecology and Obstetrics, Hospital Nový Jičín; Department of Gynecology and Obstetrics, University Hospital Ostrava; Department of Hematooncology, University Hospital Ostrava; Department of Anaesthesiology and Resuscitation, University Hospital Ostrava. RESULTS: A thirty-one-year old primigravid woman was admitted to a secondary level institution due to epigastric pain and spontaneous rupture of membranes at 26th week of pregnancy. On admission her blood pressure was 140/90 mm Hg and an intrauterine fetal death was confirmed. The patients condition deteriorated quickly, resulting in a hypertensive crisis (220/120 mm Hg), which did not respond to medication over a two hour period. Emergency caesarean section was performed, but the patients condition progressed to HELLP syndrome class I, DIC and MOF. She was transferred to the intensive care unit (ICU) of the district referral hospital 38 hours postpartum. On admission to ICU, liver rupture was diagnosed which was managed conservatively. Therapeutic plasma exchange (TPEX) was initiated on day 2 postpartum in response to falling platelets and continued for 6 days. Due to acute kidney injury (AKI), the patient required dialysis for 21 days. The patients condition improved gradually and at 28 days after admission to ICU she was transferred back to the referring hospital. The consensus reached by the treating teams was that PTMS was the most likely diagnosis. CONCLUSION: This case demonstrates that PTMS improves (usually rapidly) after TPEX is initiated. It also emphasises the importance of maintaining a high index of suspicion for PTMS so that life-saving TPEX can be initiated, because it does not respond to classical treat-ment used in the management of HELLP syndrome. Other research suggests patients may also require a terminal complement blockade with the anti-C5 monoclonal antibody (eculizumab). Further research could focus on diagnostic tests to distinguish PTMS from HELLP to identify which patients would most benefit from these treatments.


Assuntos
Troca Plasmática/métodos , Microangiopatias Trombóticas/diagnóstico , Microangiopatias Trombóticas/terapia , Adulto , Cesárea , Feminino , Síndrome HELLP , Humanos , Gravidez , Complicações Hematológicas na Gravidez , Resultado da Gravidez , Resultado do Tratamento
4.
Zhonghua Nei Ke Za Zhi ; 59(3): 250-252, 2020 Mar 01.
Artigo em Chinês | MEDLINE | ID: mdl-32146758

RESUMO

The 21-year-old male patient was admitted to the Department of Rheumatology and Immunology at Peking Union Medical College Hospital with chief complaints of "skin rash for 1 year and edema for 2 months". He was diagnosed with systemic lupus erythematosus (SLE) with renal, cardiac and hematological involvement. Remission was not achieved after glucocorticoid pulse treatment. The patient experienced oliguria, malignant hypertension, accompanied by thrombocytopenia and low serum complements, and elevated lactate dehydrogenase and serum creatinine. Schistocytes were seen in the peripheral blood smear. Thrombotic microangiopathy (TMA) secondary to SLE was diagnosed. Though plasma exchange was partially effective, TMA could not be controlled yet. The activity of serum von Willebrand factor -cleaving protease (ADAMTS 13) was 100%, and ADAMTS 13 inhibitor was negative. Finally, remission of the disease was achieved after second glucocorticoid pulse therapy and rituximab treatment. At the 3-month follow-up, the patient's condition was stable with mild anemia and normal platelet count. Patients with TMA secondary to SLE are heterogenous, while normal ADAMT 13 activity indicates poor prognosis. Early and aggressive treatment is important for disease control, and plasma exchange is helpful as a supportive care.


Assuntos
Lúpus Eritematoso Sistêmico/diagnóstico , Microangiopatias Trombóticas/diagnóstico , Proteína ADAMTS13/genética , Anemia , Edema , Exantema , Glucocorticoides/uso terapêutico , Humanos , Lúpus Eritematoso Sistêmico/complicações , Masculino , Troca Plasmática , Rituximab/uso terapêutico , Microangiopatias Trombóticas/complicações , Adulto Jovem
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.
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
8.
Am J Kidney Dis ; 75(4): 513-516, 2020 04.
Artigo em Inglês | MEDLINE | ID: mdl-31866228

RESUMO

Thrombotic microangiopathy (TMA) is an emerging complication of oncologic therapy. Cancer-related causes of renal endothelial cell damage include cytotoxic chemotherapies, radiation given for myeloablation, and direct involvement of renal vasculature by tumor cells. Another class of therapeutic agents that has been implicated in TMA is the vascular endothelial growth factor (VEGF) pathway inhibitors, including the anti-VEGF monoclonal antibody bevacizumab and the VEGF receptor tyrosine kinase inhibitor sunitinib. These TMAs have been termed type II cancer drug-induced TMA and are distinguished from those associated with some cytotoxic chemotherapies (ie, type I) in that they are not dose dependent and patients are more likely to demonstrate some recovery of kidney function. Determination of the cause of TMA in oncologic patients often presents a significant challenge because patients frequently receive multiple chemotherapeutic agents simultaneously and clinicopathologic features often demonstrate substantial overlap, regardless of cause. We present a case of TMA with predominantly chronic features in a 70-year-old patient being treated for adenoid cystic carcinoma of the breast with a single agent, a short interfering RNA targeted against Myc (DCR-MYC).


Assuntos
Proteínas Proto-Oncogênicas c-myc/genética , RNA Interferente Pequeno/efeitos adversos , RNA Interferente Pequeno/genética , Microangiopatias Trombóticas/induzido quimicamente , Microangiopatias Trombóticas/genética , Idoso , Antineoplásicos/administração & dosagem , Antineoplásicos/efeitos adversos , Doença Crônica , Sistemas de Liberação de Medicamentos/efeitos adversos , Sistemas de Liberação de Medicamentos/métodos , Evolução Fatal , Feminino , Marcação de Genes/efeitos adversos , Marcação de Genes/métodos , Humanos , Proteínas Proto-Oncogênicas c-myc/antagonistas & inibidores , RNA Interferente Pequeno/administração & dosagem , Microangiopatias Trombóticas/diagnóstico
9.
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
10.
F1000Res ; 82019.
Artigo em Inglês | MEDLINE | ID: mdl-31598213

RESUMO

The thrombotic microangiopathies (TMAs) are a group of diseases characterised by microangiopathic haemolysis, thrombocytopenia, and thrombus formation leading to tissue injury. Traditionally, TMAs have been classified as either thrombotic thrombocytopenic purpura (TTP) or haemolytic uremic syndrome (HUS) based on the clinical presentation, with neurological involvement predominating in the former and acute kidney injury in the latter. However, as our understanding of the pathogenesis of these conditions has increased, it has become clear that this is an over-simplification; there is significant overlap in the clinical presentation of TTP and HUS, there are different forms of HUS, and TMAs can occur in other, diverse clinical scenarios. This review will discuss recent developments in the diagnosis of HUS, focusing on the different forms of HUS and how to diagnose and manage these potentially life-threatening diseases.


Assuntos
Lesão Renal Aguda , Síndrome Hemolítico-Urêmica , Púrpura Trombocitopênica Trombótica , Microangiopatias Trombóticas , Lesão Renal Aguda/diagnóstico , Lesão Renal Aguda/terapia , Hemólise , Síndrome Hemolítico-Urêmica/diagnóstico , Síndrome Hemolítico-Urêmica/terapia , Humanos , Púrpura Trombocitopênica Trombótica/diagnóstico , Púrpura Trombocitopênica Trombótica/terapia , Microangiopatias Trombóticas/diagnóstico , Microangiopatias Trombóticas/terapia
11.
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
12.
J Med Case Rep ; 13(1): 281, 2019 Sep 05.
Artigo em Inglês | MEDLINE | ID: mdl-31484586

RESUMO

BACKGROUND: Renal involvement in idiopathic hypereosinophilic syndrome is uncommon. The mechanism of kidney damage can be explained as occurring via two distinct pathways: (1) thromboembolic ischemic changes secondary to endocardial disruption mediated by eosinophilic cytotoxicity to the myocardium and (2) direct eosinophilic cytotoxic effect to the kidney. CASE PRESENTATION: We present a case of a 63-year-old Caucasian man who presented to our hospital with 2 weeks of progressively generalized weakness. He was diagnosed with idiopathic hypereosinophilic syndrome with multiorgan involvement and acute kidney injury with biopsy-proven thrombotic microangiopathy. Full remission was achieved after 8 weeks of corticosteroid therapy. CONCLUSION: Further studies are needed to investigate if age and absence of frank thrombocytopenia can serve as a prognostic feature of idiopathic hypereosinophilic syndrome, as seen in this case.


Assuntos
Lesão Renal Aguda/etiologia , Síndrome Hipereosinofílica/diagnóstico , Microangiopatias Trombóticas/diagnóstico , Dispneia/etiologia , Humanos , Masculino , Pessoa de Meia-Idade , Debilidade Muscular/etiologia
13.
Rev. esp. anestesiol. reanim ; 66(7): 385-389, ago.-sept. 2019. tab
Artigo em Espanhol | IBECS | ID: ibc-187552

RESUMO

La trombocitopenia es una alteración común durante el embarazo, y las diversas etiologías posibles hacen que su diagnóstico diferencial sea un desafío. Los cambios fisiológicos en el embarazo pueden enmascarar la gravedad de la situación hasta etapas avanzadas. Este caso se refiere a una embarazada que presentó fiebre y trombocitopenia durante el parto. En el posparto desarrolló inestabilidad hemodinámica rápidamente progresiva, requiriendo ingreso en la UCI. Además, presentó lesión renal aguda, trombocitopenia y disfunción plaquetaria persistentes. El diagnóstico diferencial incluyó CID en el contexto de sepsis, HPP y otras causas de microangiopatías trombóticas en el embarazo


Thrombocytopenia is a common alteration during pregnancy and the multiple possible aetiologies make the differential diagnosis challenging. Physiological changes of pregnancy can mask severe conditions until advanced stages. This case refers to a pregnant woman who presented fever and thrombocytopenia during labour. In the postpartum period she developed rapidly progressive hemodynamic instability requiring admission to the ICU. There was progression to acute kidney injury, persistent thrombocytopenia and platelet dysfunction. The differential diagnosis included DIC in the context of sepsis, PPH and other causes of thrombotic microangiopathies in pregnancy


Assuntos
Humanos , Feminino , Gravidez , Adulto , Trombocitopenia/complicações , Anestésicos/administração & dosagem , Hemorragia Pós-Parto/etiologia , Lesão Renal Aguda/etiologia , Microangiopatias Trombóticas/diagnóstico , Corioamnionite/diagnóstico , Complicações Hematológicas na Gravidez/diagnóstico , Trombocitopenia/terapia , Cuidados Críticos/métodos , Diagnóstico Diferencial , Febre/etiologia , Complicações do Trabalho de Parto/diagnóstico
15.
Turk J Haematol ; 36(4): 222-229, 2019 11 18.
Artigo em Inglês | MEDLINE | ID: mdl-31337190

RESUMO

Thrombotic microangiopathies (TMAs) are multiple disease entities with different etiopathogeneses, characterized by thrombocytopenia, microangiopathic hemolytic anemia (MAHA) with schistocytosis, variable symptoms including fever, and multi-organ failure such as mild renal impairment and neurological deficits. The two paradigms of TMAs are represented on one hand by acquired thrombotic thrombocytopenic purpura (TTP) and on the other by hemolytic uremic syndrome (HUS). The differential diagnosis between these two paradigmatic forms of TMA is based on the presence of either frank renal failure in HUS or a severe deficiency (<10%) of the zinc-protease ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) in TTP. ADAMTS13 is an enzyme involved in the proteolytic processing of von Willebrand factor (vWF), and its deficiency results in formation of high-molecular-weight vWF-rich microthrombi in the environment of the microvasculature. The presence of these ultra-large vWF multimers in the microcirculation can recruit platelets, promoting multi-organ ischemic lesions. The presence of ADAMTS13 activity at >10% could rule out the presence of a TTP form. However, it is often difficult to differentiate either a TTP or HUS clinical scenario presenting with typical symptoms of TMA. There are in fact several additional diagnoses that should be considered in patients with ADAMTS13 activity of >10%. Widespread inflammation with endothelial damage and adverse reactions to drugs play a central role in the pathogenesis of several forms of TMA, and in these cases, the differential diagnosis should be directed at the underlying disease. Hence, a correct etiologic diagnosis of TMA should involve a critical illness, cancer-associated TMA, drug-induced TMA, and hematopoietic transplant-associated TMA. A complete assessment of all the possible etiologies for TMA symptoms, including acquired or congenital TTP, will allow for a more accurate diagnosis and application of a more appropriate treatment.


Assuntos
Testes Diagnósticos de Rotina/métodos , Microangiopatias Trombóticas/diagnóstico , Humanos , Microangiopatias Trombóticas/epidemiologia , Microangiopatias Trombóticas/patologia
16.
Eur J Haematol ; 103(4): 307-318, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-31251415

RESUMO

OBJECTIVES: Describe the clinical presentation, treatment, and outcomes of postsurgical thrombotic microangiopathy (TMA). METHODS: In this retrospective study, records of individuals diagnosed with TMA developing within 30 days of a surgical procedure at Mayo Clinic from 2000 to 2016 were reviewed. Available literature regarding postsurgical TMA was comparatively reviewed. RESULTS: Twenty patients were diagnosed with TMA developing within 30 (median 6.5, range (1-28)) days) following a procedure. Preceding procedures included orthopedic (n = 4), vascular (n = 4), abdominal (n = 8), thoracic (n = 2), and other (n = 2). Review of the literature identified 65 patients with postsurgical TMA and cardiovascular procedures were the most common preceding surgery. The majority of patients in the current cohort and literature were treated with therapeutic plasma exchange (TPE). Among the evaluable patients in the current cohort, 100% demonstrated response to TPE; however, 25% required the addition of other therapy including eculizumab to maintain a response 80% of patients in the literature demonstrated a response to TPE. CONCLUSIONS: Although rare, early recognition and treatment of postsurgical TMA can lead to good outcomes. More research is necessary to determine the underlying pathophysiology and optimal treatment for postsurgical TMA.


Assuntos
Complicações Pós-Operatórias , Microangiopatias Trombóticas/diagnóstico , Microangiopatias Trombóticas/etiologia , Adolescente , Adulto , Biomarcadores , Criança , Pré-Escolar , Terapia Combinada , Gerenciamento Clínico , Feminino , Humanos , Lactente , Masculino , Prognóstico , Estudos Retrospectivos , Avaliação de Sintomas , Microangiopatias Trombóticas/mortalidade , Microangiopatias Trombóticas/terapia , Fatores de Tempo , Resultado do Tratamento , Adulto Jovem
19.
J. bras. nefrol ; 41(2): 296-299, Apr.-June 2019. graf
Artigo em Inglês | LILACS | ID: biblio-1012529

RESUMO

ABSTRACT Introduction: Purpura fulminans (PF) is a rapid progressive thrombotic disease in which hemorrhagic infarction of the skin and disseminated intravascular coagulation (DIC) occurs. It can potentially cause acute kidney injury (AKI). However, there is no description in the medical literature of renal histological findings of PF. Case report: A 20-year-old female patient, previously healthy, was admitted to the emergency department (ED) with odynophagia, fever, generalized myalgia and anuria, which evolved with the appearance of purpuric plaques on the face and limbs. She required dialysis on admission. Laboratorial tests showed anemia, leukocytosis, thrombocytopenia, and elevation of lactic dehydrogenase (LDH). The purpuric lesions became bullous with ruptures and then necrotic and erosive, reaching the dermis, subcutaneous tissue and musculature, until bone exposure. There was no improvement with initial antibiotic therapy aimed at the treatment of meningococcemia. Thrombotic microangiopathy (TMA) and PF were then suspected. The patient remained in daily dialysis, requiring plasmapheresis. After sustained improvement of the thrombocytopenia, she underwent renal biopsy, which was not compatible with TMA, characterizing possible PF. A complete recovery of the renal function was achieved and cutaneous sequels were treated with grafts. Conclusion: When thrombotic and hemorrhagic phenomena overlap, obtaining a renal biopsy can be difficult. However, in the presented case, the biopsy allowed the exclusion of AKI caused by TMA, presenting for the first time, histological findings compatible with PF.


RESUMO Introdução: Purpura Fulminans (PF) é uma doença trombótica de rápida progressão, com infarto hemorrágico da pele e coagulação intravascular disseminada (CIVD). É potencialmente causadora de injúria renal aguda (IRA). Porém, não há descrição na literatura médica dos achados histológicos renais causados por PF. Relato de caso: Mulher, 20 anos, previamente hígida, hospitalizada por odinofagia, febre, mialgia generalizada e anúria, evoluiu com aparecimento de placas purpúricas em face e membros. Necessitou de hemodiálise (HD) já na admissão. Exames laboratoriais mostravam anemia, leucocitose, plaquetopenia e elevação de desidrogenase lática. As lesões purpúricas tornaram-se bolhosas com rompimento e progressão para necrose, se aprofundaram, atingindo derme, subcutâneo e musculatura, até a exposição óssea. Não houve melhora com antibioticoterapia inicial voltada para tratamento de meningococemia. Suspeitou-se, então, de microangiopatia trombótica (MAT) e PF. A paciente permaneceu em HD diária e necessitou também de plasmaférese, após melhora sustentada da plaquetopenia, foi submetida à biópsia renal, que não foi compatível com MAT, possivelmente caracterizando PF. Houve recuperação completa da função renal e as sequelas cutâneas foram tratadas com enxerto. Conclusão: Em casos nos quais os fenômenos trombóticos e hemorrágicos se sobrepõem, a obtenção da biópsia renal se torna difícil. Neste caso, a biópsia permitiu excluir IRA causada por MAT e mostrar, pela primeira vez, achados compatíveis com PF.


Assuntos
Humanos , Feminino , Adulto Jovem , Púrpura Fulminante/complicações , Púrpura Fulminante/diagnóstico , Microangiopatias Trombóticas/complicações , Microangiopatias Trombóticas/diagnóstico , Lesão Renal Aguda/complicações , Lesão Renal Aguda/patologia , Rim/patologia , Biópsia , Diálise Renal , Plasmaferese , Transplante de Pele , Resultado do Tratamento , Lesão Renal Aguda/terapia , Tempo de Internação
20.
Transfus Apher Sci ; 58(3): 347-350, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-31097308

RESUMO

Transplant-associated thrombotic microangiopathy (TA-TMA) is one of the early endothelial complications post Hematopoietic Stem Cell Transplant (HSCT). Several mechanisms during HSCT can contribute to systemic capillary endothelial damage which can lead to TA-TMA among other complications as capillary leak syndrome or engraftment syndrome. Early diagnosis of TA-TMA contributes a challenge due to overlapping clinical manifestations and the absence of specific diagnostic criteria. Incidence is greatly variable between 1-76% according to risk factors of patients and the definition used to confirm the diagnosis. The mortality rates in patients who develop severe TA-TMA are in excess of 80%. Early treatment improves the outcome. This review outlines the diagnostic challenges and therapeutic options for TA-TMA.


Assuntos
Síndrome de Vazamento Capilar , Transplante de Células-Tronco Hematopoéticas , Microangiopatias Trombóticas , Aloenxertos , Síndrome de Vazamento Capilar/diagnóstico , Síndrome de Vazamento Capilar/etiologia , Síndrome de Vazamento Capilar/metabolismo , Síndrome de Vazamento Capilar/terapia , Humanos , Microangiopatias Trombóticas/diagnóstico , Microangiopatias Trombóticas/etiologia , Microangiopatias Trombóticas/metabolismo , Microangiopatias Trombóticas/terapia
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