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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.
Am J Med Sci ; 359(5): 296-302, 2020 05.
Artigo em Inglês | MEDLINE | ID: mdl-32265009

RESUMO

Thrombotic microangiopathy (TMA) is characterized by microangiopathic hemolytic anemia with thrombocytopenia. In addition to the primary TMA syndromes, microangiopathic hemolytic anemia with thrombocytopenia can be seen in many systemic diseases. Transplant associated TMA (TA-TMA) affects patients following stem cell or solid organ transplant. A 48-year-old male who underwent autologous stem cell transplant for nonsecretory multiple myeloma was admitted to our hospital with worsening anemia, thrombocytopenia, renal dysfunction and hepatosplenomegaly. Initial blood work revealed rare schistocytes and normal lactate dehydrogenase and haptoglobin levels. He underwent an extensive workup looking for an infectious, inflammatory or malignant etiology but a definitive diagnosis could not be reached. Over his prolonged stay at the hospital, he suffered from multiorgan failure and eventually passed away. An autopsy revealed TMA involving all clinically affected organ systems and was deemed to be the cause of his demise. The absence of typical blood work suggestive of hemolysis does not rule out a diagnosis of TA-TMA. Knowledge of this rare disease entity will help physicians identify and treat this life-threatening condition early and effectively.


Assuntos
Eritrócitos Anormais , Hemólise , Microangiopatias Trombóticas/complicações , Biópsia , Evolução Fatal , Hepatomegalia/complicações , Humanos , Inflamação , Integrina beta3/metabolismo , L-Lactato Desidrogenase/metabolismo , Fígado/patologia , Pulmão/patologia , Masculino , Microcirculação , Pessoa de Meia-Idade , Esplenomegalia/complicações , Transplante de Células-Tronco , Trombocitopenia/complicações , Trombose/metabolismo , Microangiopatias Trombóticas/terapia , Transplante Autólogo
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.
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
8.
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
10.
Cancer Treat Res ; 179: 151-158, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31317486

RESUMO

Thrombotic microangiopathy (TMA) is a syndrome involving fragmentation haemolysis, thrombocytopenia, and thrombosis. A range of disorders including cancer may have TMA as a clinical manifestation. TMA in cancer may be caused by several mechanisms, including systemic microvascular metastases, but may also be due to extensive bone marrow involvement with cancer or secondary necrosis. Chemotherapeutic agents may also cause associated TMA through a range of different mechanisms. Gemcitabine, platinum-based drugs, mitomycin C, and proteasome inhibitors are known to cause TMA in cancer patients. Transplant-associated TMA (TA-TMA) may affect either solid organ or HSCT patients. TA-TMA remains a difficult complication to address due to its high mortality rate, lack of standard diagnostic criteria, and limited therapeutic options. The challenge of cancer-associated TMA is furthered by the fact that plasma exchange is ineffective in its management.


Assuntos
Antineoplásicos/efeitos adversos , Neoplasias/complicações , Microangiopatias Trombóticas/terapia , Antineoplásicos/uso terapêutico , Humanos , Neoplasias/tratamento farmacológico , Microangiopatias Trombóticas/etiologia
12.
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
14.
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
16.
Mod Pathol ; 32(8): 1147-1157, 2019 07.
Artigo em Inglês | MEDLINE | ID: mdl-30936425

RESUMO

Complement factor C4d was recently observed in renal biopsies from patients who had IgA nephropathy and a poor prognosis. We previously reported that C4d is a common denominator in microangiopathies. In this retrospective cohort study, we investigated whether C4d is a marker of microangiopathy in both IgA nephropathy and IgA vasculitis with nephritis, and whether patients with C4d and microangiopathy have poor renal outcome. We examined 128 renal biopsies from adult and pediatric patients, including normotensive and hypertensive patients, who presented with IgA nephropathy or IgA vasculitis with nephritis. Biopsies were re-evaluated in accordance with the Oxford classification, scored for additional lesions, and stained for complement proteins using immunohistochemistry, including C4d and C5b-9. Clinical data were collected with a mean (±SD) follow-up period of 51 ± 39 months. Changes in estimated glomerular filtration rate over time were compared using linear mixed-effects models. Renal survival was analyzed using multivariable Cox regression. Microangiopathic lesions were present in 20% of all biopsies (23% and 9% of patients with IgA nephropathy and IgA vasculitis with nephritis, respectively). Microangiopathy was associated with C4d and C5b-9 deposits, a higher number of chronic lesions, and hypertension (all p < 0.05). Patients with C4d and microangiopathic lesions had significantly poorer renal survival than patients without these findings, corrected for hypertension (p < 0.01). In conclusion, patients with IgA nephropathy or IgA vasculitis with nephritis with a combination of C4d positivity and microangiopathy comprise a clinical subgroup with an increased number of chronic lesions, lower estimated glomerular filtration rate, and poorer renal survival, even when corrected for hypertension. These data suggest that complement activation is involved in the development of microangiopathy in patients with IgA nephropathy and IgA vasculitis with nephritis, and that complement-mediated microangiopathy contributes to disease progression.


Assuntos
Ativação do Complemento , Complemento C4b/análise , Glomerulonefrite por IGA/imunologia , Imunoglobulina A/análise , Rim/imunologia , Nefrite/imunologia , Fragmentos de Peptídeos/análise , Microangiopatias Trombóticas/imunologia , Vasculite/imunologia , Adulto , Complexo de Ataque à Membrana do Sistema Complemento/análise , Progressão da Doença , Feminino , Glomerulonefrite por IGA/patologia , Glomerulonefrite por IGA/terapia , Humanos , Rim/patologia , Masculino , Pessoa de Meia-Idade , Nefrite/patologia , Nefrite/terapia , Prognóstico , Estudos Retrospectivos , Microangiopatias Trombóticas/patologia , Microangiopatias Trombóticas/terapia , Vasculite/patologia , Vasculite/terapia , Adulto Jovem
17.
Biol Blood Marrow Transplant ; 25(3): 570-576, 2019 03.
Artigo em Inglês | MEDLINE | ID: mdl-30940363

RESUMO

Transplant-associated thrombotic microangiopathy (TA-TMA) after allogeneic hematopoietic cell transplantation (HCT) has not been well characterized in large population studies with clinically adjudicated cases. We performed a retrospective cohort study of adults who underwent allogeneic HCT between 2006 and 2015 to determine the incidence of and risk factors for TA-TMA and to describe its natural history and response to immunosuppressant withdrawal management. Among 2145 patients in this study, 192 developed TA-TMA with a cumulative incidence of 7.6% by 100days post-transplant. Independent pretransplant risk factors included the receipt of a second (or third) allogeneic HCT, HLA-mismatched donor, and myeloablative conditioning with or without total body irradiation; post-transplant risk factors included the antecedent development of acute graft-versus-host disease, diffuse alveolar hemorrhage, bacteremia, invasive aspergillosis, BK viremia, and higher sirolimus trough level. Among TA-TMA patients 27% achieved hematologic resolution and 57% remained alive as of 90days after diagnosis. Antecedent risk factors stratified patients into different survival groups, and immunosuppressant withdrawal alone did not improve patient outcomes. In conclusion, TA-TMA is a heterogenous disease that occurs after allogeneic transplantation. Management with immunosuppressant withdrawal does not impact patient outcomes. Until further evidence becomes available, the management of TA-TMA should focus on the treatment of underlying diseases.


Assuntos
Transplante de Células-Tronco Hematopoéticas/efeitos adversos , Microangiopatias Trombóticas/etiologia , Adulto , Estudos de Coortes , Feminino , Humanos , Imunossupressores , Masculino , Estudos Retrospectivos , Fatores de Risco , Microangiopatias Trombóticas/terapia , Transplante Homólogo/efeitos adversos
18.
Transfus Apher Sci ; 58(3): 262-265, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-31029612

RESUMO

Plasma exchange had been proposed to treat many kidney diseases. A few controlled studies, observational studies, and mechanistic evidence have specified the better indications, which are thrombotic microangiopathies, kidney transplantation and ANCA-associated vasculitides. Plasma removal can be realized by filtration, centrifugation, or immunoadsorption, with differences in efficacy, but these also impact on volume load and anticoagulation in patients with renal failure.


Assuntos
Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/terapia , Nefropatias/terapia , Troca Plasmática , Microangiopatias Trombóticas/terapia , Humanos
19.
BMC Nephrol ; 20(1): 91, 2019 03 14.
Artigo em Inglês | MEDLINE | ID: mdl-30871486

RESUMO

BACKGROUND: Microangiopathic hemolytic anemias and thrombocytopenias in pregnant or postpartum women constitute an interdisciplinary diagnostic and therapeutic challenge in the evaluation of thrombotic microangiopathies (TMA), where urgent care must be considered. CASE PRESENTATION: We here report the case of a 21-year-old Somali woman, who was delivered by emergency caesarean section at 35 weeks of gestational age with acute dyspnea, placental abruption and gross edema due to severe preeclampsia/HELLP syndrome. After delivery, she developed acute kidney failure and thrombotic microangiopathy as revealed by kidney biopsy. The lack of early response to plasma exchange prompted extensive laboratory workup. Ultimately, the patient completely recovered with negative fluid balance and control of severe hypertension. CONCLUSIONS: This case report emphasizes the importance to differentiate between primary TMA syndromes and microangiopathic hemolytic anemias due to systemic disorders. Delayed recovery from preeclampsia/HELLP syndrome and malignant hypertension can clinically mimic primary TMA syndromes in the postpartum period.


Assuntos
Lesão Renal Aguda/diagnóstico , Lesão Renal Aguda/terapia , Gerenciamento Clínico , Cuidado Pós-Natal/métodos , Microangiopatias Trombóticas/diagnóstico , Microangiopatias Trombóticas/terapia , Lesão Renal Aguda/complicações , Cesárea/efeitos adversos , Cesárea/tendências , Feminino , Humanos , Troca Plasmática/métodos , Troca Plasmática/tendências , Gravidez , Adulto Jovem
20.
J Clin Apher ; 34(4): 507-509, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-30779435

RESUMO

Post snake bite renal failure due to thrombotic microangiopathy (TMA) is often overlooked and not considered as a separate entity while managing the patient. This case report highlights the efficacy of Therapeutic Plasma Exchange in managing the post envenomation TMA. Anti-snake venom was administered following severe hematotoxic envenomation, but later on developed acute kidney injury and hence hemodialysis was done for 2 days without much improvement. On third day of bite, diagnosis of TMA was made from microangiopathic hemolytic picture in peripheral blood smear, thrombocytopenia and renal failure. Therapeutic plasma exchange with human albumin solution was started on a daily basis and after 3 cycles, patient's condition improved as shown by the laboratory parameters. Though this entity is not well defined, the supporting evidence is found in few reports of published literature. Hence, we propose plasma exchange as an adjunctive therapeutic option in post snake TMA.


Assuntos
Troca Plasmática/métodos , Mordeduras de Serpentes/complicações , Microangiopatias Trombóticas/terapia , Lesão Renal Aguda/etiologia , Albuminas , Humanos , Microangiopatias Trombóticas/diagnóstico , Microangiopatias Trombóticas/etiologia , Resultado do Tratamento
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