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1.
J Med Case Rep ; 15(1): 584, 2021 Dec 13.
Artigo em Inglês | MEDLINE | ID: mdl-34903283

RESUMO

BACKGROUND: Leptospirosis is a common zoonotic infection caused by the spirochete Leptospira. The disease is more prevalent in the tropics, causing subclinical to severe illness leading to high morbidity and mortality. CASE PRESENTATION: A 77-year-old healthy Sri Lankan man presented to the Teaching Hospital Peradeniya with severe leptospirosis complicated with acute kidney injury, pulmonary hemorrhages, myocarditis, and severe thrombocytopenia. He was deteriorating despite treatment with intravenous antibiotics and methylprednisolone boluses. He made a dramatic improvement with two cycles of plasma exchange. CONCLUSION: Therapeutic plasma exchange is a life-saving treatment modality in severe leptospirosis with multiorgan failure.


Assuntos
Leptospirose , Miocardite , Idoso , Animais , Humanos , Leptospirose/complicações , Leptospirose/tratamento farmacológico , Masculino , Metilprednisolona , Troca Plasmática , Zoonoses
2.
Ital J Pediatr ; 47(1): 236, 2021 Dec 14.
Artigo em Inglês | MEDLINE | ID: mdl-34906202

RESUMO

BACKGROUND: Although kidney transplantation (KTX) is the treatment of choice for pediatric end stage kidney disease (ESKD); concerns for recurrence in cases of focal segmental glomerulosclerosis (FSGS) are still present. This study aimed to investigate the outcome of KTX in children with ESKD secondary to FSGS, with implementation of preemptive perioperative plasma exchange (PE) for non-genetically proven patients. METHODS: Forty FSGS pediatric kidney transplant recipients were studied. Of them: 12 patients (30%) had genetically proven NPHS2 mutations/familial and 28 (70%) were sporadic FSGS patients. All sporadic patients electively received 6 perioperative PE sessions. Patients with recurrence of proteinuria (n = 13; including 3 patients with genetic/familial and 10 patients with sporadic FSGS) were managed with PE and Rituximab (RTX). Kaplan-Meier curves were used to analyze graft and recurrence free survival data. RESULTS: The mean follow-up duration after KTX was 3.8 ± 2.86 years. Recurrence of proteinuria was encountered early postoperative in 11 patients (27.5%) and late (1.6 and 2.9 years after KTX) in 2 patients (5%). All patients with early recurrence achieved complete remission, while patients with late recurrence developed graft failure. Current serum creatinine and proteinuria levels were not different in patients received PE (n = 31) and patients did not PE (n = 9) (p = 0.308 and 0.287 respectively). Current serum creatinine and proteinuria levels in sporadic patients (n = 28) after prophylactic perioperative PE were not different from those of genetic/ familial patients (n = 12) (p = 0.303 and 0.144 respectively). Proteinuria was less in patients underwent native nephrectomy than others immediately postoperative and at assessment (p = 0.002 & 0.0031 respectively). One-year graft and patient survival was 93.8% with a mean 1-year serum creatinine of 0.67 ± 0.25 mg/dl. Three graft losses (7.5%) were due to chronic rejection 3.3, 3.75 and 4.17 years after KTX and 2 patients' mortality (5%) occurred early postoperative (first 2 weeks). CONCLUSION: FSGS transplanted children have favorable outcomes with perioperative PE for non-genetically proven cases. Early recurrence after KTX can be successfully managed with PE and RTX.


Assuntos
Glomerulosclerose Segmentar e Focal/terapia , Falência Renal Crônica/terapia , Transplante de Rim , Troca Plasmática , Criança , Estudos de Coortes , Creatinina/sangue , Feminino , Seguimentos , Rejeição de Enxerto , Sobrevivência de Enxerto , Humanos , Falência Renal Crônica/etiologia , Masculino , Proteinúria/terapia , Recidiva , Indução de Remissão , Estudos Retrospectivos
3.
Iran J Kidney Dis ; 15(6): 419-425, 2021 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-34930853

RESUMO

INTRODUCTION: Anti-Compliment Factor H antibody hemolytic uremic syndrome (AFH-HUS) is a common cause of paediatric atypical HUS in India. We wanted to study the outcome of patients receiving less than recommended plasma exchange (PLEX) but adequate immunosuppression, with respect to hypertension, preservation of renal function and proteinuria. METHODS: A retrospective study was performed in 15 children admitted from 2016 to 2018 with AFH-HUS. Follow up details including physical examination, hematological parameters, renal function test and urine examination performed at 3, 6, and 12 months were noted. Risk stratification and staging for chronic kidney disease (CKD) were done according to the Kidney Disease Improving Global Outcomes (KDOQI) guidelines. Standard statistical tests which were appropriate were used. RESULTS: Mean age of our study cohort was 7.8 ± 1.9 years. 14 children had hypertension. Mean nadir hemoglobin was 5.8 ± 1.0 g/dL and platelet = 58 ± 37.7 × 109 cells/L. Median anti factor H (AFH) level was 316 AU/mL (150 to 452). Hemodialysis was required in 7 children. Fourteen children received PLEX with a mean of 11 ± 6 cycles. Thirteen children received 6 cycles of intravenous cyclophosphamide. After six months, therapy was switched to mycophenolate mofetil in 4 children, steroids alone in 2 children and 9 children with azathioprine. On follow-up, risk of CKD reduced from 80% at 3 months to 26% at 12 months (P = .01). Only 40% patients had CKD stage 2 at the end of 12 months (mean eGFR = 95.0 ± 19.4 mL/min/1.73m2). CONCLUSION: The adequate number of PLEX needed in AFH-HUS needs further studies. Till such reports come, PLEX in recommended strategies or lesser, if not available, with immunosuppression in AFH-HUS can decrease progression to CKD. DOI: 10.52547/ijkd.6507.


Assuntos
Fator H do Complemento , Síndrome Hemolítico-Urêmica , Criança , Pré-Escolar , Seguimentos , Síndrome Hemolítico-Urêmica/diagnóstico , Síndrome Hemolítico-Urêmica/terapia , Humanos , Troca Plasmática , Estudos Retrospectivos
4.
J Infect Dev Ctries ; 15(11): 1607-1614, 2021 Nov 30.
Artigo em Inglês | MEDLINE | ID: mdl-34898486

RESUMO

Novel coronavirus infections 2019 (COVID-19) associated hyperinflammatory syndromes are well-defined clinical conditions and have a potential risk for severe infection. Hemophagocytic lymphohistiocytosis (HLH), a rare type of acute progressive hyperinflammatory syndrome, has been reported in a limited number of COVID-19 cases. In this article, we aimed to present a patient with HLH secondary to COVID-19 diagnosed by bone marrow biopsy, and to summarize and review HLH cases associated with COVID-19 in the literature. A 47-year-old male patient presented with complaints of fever, cough, abdominal discomfort, and nausea-vomiting. He had recovered from COVID-19 a month ago and was readmitted to the hospital due to the re-appearance of clinical symptoms after a two-week interval. The patient was diagnosed with HLH secondary to COVID-19 on sixth day of admission and fully recovered with systemic pulse steroid, intravenous immunoglobulin, and plasma exchange therapy. Analysis of literature searches revealed that 22 cases were definitely diagnosed with COVID-19-associated HLH, 16 of them were male. They had been treated with different anti-cytokine drugs, of which nine had died. The increasing number of HLH cases, which have high mortality rates, shows the importance of hyperinflammatory syndromes in COVID-19 patients. Some patients may experience hemophagocytosis in the late period of COVID-19, even while in recovery. Increased awareness and early treatment for HLH triggered by COVID-19 can be a life-saving effort for reducing mortality in severe COVID-19 cases.


Assuntos
COVID-19/complicações , Linfo-Histiocitose Hemofagocítica/diagnóstico , SARS-CoV-2 , Diagnóstico Diferencial , Humanos , Linfo-Histiocitose Hemofagocítica/etiologia , Linfo-Histiocitose Hemofagocítica/terapia , Masculino , Pessoa de Meia-Idade , Troca Plasmática
5.
Medicine (Baltimore) ; 100(41): e27351, 2021 Oct 15.
Artigo em Inglês | MEDLINE | ID: mdl-34731107

RESUMO

BACKGROUND: Guillain-Barre syndrome (GBS) is a disease with the features of acuteness, paralysis, inflammation, and in peripheral nerves. There are many current treatment options with varying efficacy, and to assess their effectiveness, we performed a network meta-analysis (NMA). The study protocol was registered at PROSPERO (CRD: 42019119178). Posted history: this manuscript was previously posted to medRxiv: doi: https://doi.org/10.1101/2020.06.03.20121780. METHODS: The literature search database includes Web of Science, PubMed, Embase, and the Cochrane library that meets the requirements. We performed the NMA using controlled trials with 2 kinds of outcomes. We used the gemtc R package to perform the NMA to evaluate different GBS treatments' relative results. The consistency of direct and indirect evidence was also assessed by R software with gemtc package. RESULTS: This NMA study included a total of 2474 subjects from 28 trials with 15 kinds of therapies. No improvement was observed in methylprednisolone and prednisolone compared with placebo. Conversely, plasma exchange (PE) and intravenous immunoglobulin (IVIg) were more effective than placebo. There was no significant difference between different doses and courses of PE and IVIg. For combination treatment, such as IVIg+eculizumab, immunoadsorption followed by IVIg and PE followed by IVIg, they didn't show significant advantages than IVIg and PE in NMA. On the consistency examination between direct and indirect evidence, there was no apparent heterogeneity between them. Funnel plots indicated there was little possibility of publication bias in this study. CONCLUSION: PE or IVIg has significant efficacy for GBS patients. The effects of several kinds of therapies should be further explored. Corticosteroids have no considerable impact on GBS.


Assuntos
Síndrome de Guillain-Barré/terapia , Avaliação de Resultados em Cuidados de Saúde , Terapia Combinada , Humanos , Imunoglobulinas Intravenosas/uso terapêutico , Metilprednisolona/uso terapêutico , Metanálise em Rede , Troca Plasmática
7.
Am J Case Rep ; 22: e931877, 2021 Oct 10.
Artigo em Inglês | MEDLINE | ID: mdl-34628462

RESUMO

BACKGROUND Immune thrombocytopenic purpura (ITP) is primarily caused by antibody-mediated destruction of platelets. Alterations in immune homeostasis can induce loss of peripheral tolerance and promote the development of self-reactive antibodies. Primary ITP is the diagnosis of exclusion made after the extensive work-up rules out other possible causes of thrombocytopenia. The association between the ITP and other autoimmune disorders is well-established. In recent years, increasing attention has been directed toward the association between celiac disease (CD) and ITP. CASE REPORT A 27-year-old man with a history of primary ITP presented with an occasional nosebleed, 1 episode of rectal bleeding, and easy bruising. The patient was later found to have high titers of TTG-IGA and endomysial IGA levels consistent with CD. Our patient not only failed to improve with the gluten-free diet, but also failed multiple lines of treatment including steroids, IVIG, rituximab, eltrombopag, and even a non-traditional treatment for ITP (azathioprine and plasma exchange). The patient's CD-related antibody titers remained elevated. CONCLUSIONS It is possible that in certain cases the alteration of immune response caused by CD with a concurrent elevation of CD-related antibodies can make ITP refractory to all medical management. Whether or not this refractoriness to treatment is related to the persistently elevated antibody titers of CD or unknown genetic relationship between ITP and CD remains not entirely clear and warrants further molecular, immunologic, and genetic analysis.


Assuntos
Doença Celíaca , Púrpura Trombocitopênica Idiopática , Adulto , Doença Celíaca/complicações , Doença Celíaca/diagnóstico , Dieta Livre de Glúten , Humanos , Masculino , Troca Plasmática , Púrpura Trombocitopênica Idiopática/complicações , Púrpura Trombocitopênica Idiopática/diagnóstico , Púrpura Trombocitopênica Idiopática/terapia , Rituximab
8.
Clin Nephrol ; 96(1): 96-100, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34643499

RESUMO

AIMS: In therapeutic plasma exchange (TPE), large amounts of plasma with all components, including antibodies, albumin, coagulation factors and inhibitors, are removed and usually replaced with replacement fluid without coagulation factors. Hemostatic parameters should be closely monitored in patients at risk of bleeding or with large volume exchanges with a short recovery interval. In this prospective study, we compared standard coagulation parameters and the rotational thromboelastometry (ROTEM) point-of-care test to identify hemostatically severely compromised patients treated with TPE. MATERIALS AND METHODS: 22 patients without recent or planned invasive procedures received 63 TPE treatments with regional citrate anticoagulation. One plasma volume was exchanged with replacement fluid containing albumin and electrolytes. Standard coagulation tests, fibrinogen concentration, and rotational thromboelastometry (ROTEM, including EXTEM test, INTEM test, and FIBTEM test) were performed before and after each TPE treatment. RESULTS: Fibrinogen concentration decreased significantly and international normalized ratio increased slightly after TPE. Activated partial thromboplastin time, EXTEM, INTEM, and FIBTEM clotting times as well as clot formation times were prolonged, and maximum clot firmness decreased after TPE procedures. No serious adverse events occurred during TPE treatment. CONCLUSION: Our study showed that ROTEM parameters changed significantly after TPE performed with replacement fluid without coagulation factors. Among all parameters, FIBTEM clotting time showed the highest percentual change after TPE. According to this data, the ROTEM point-of-care test may have a potential to guide TPE therapy, particularly in patients at high risk for bleeding.


Assuntos
Troca Plasmática , Tromboelastografia , Testes de Coagulação Sanguínea , Hemostasia , Humanos , Estudos Prospectivos
9.
Clin Nephrol ; 96(1): 101-106, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34643500

RESUMO

AIMS: Different forms of apheresis have been proposed as potential therapeutic approaches to remove soluble Fms-like tyrosine kinase-1 (sFlt-1) and allow safe pregnancy prolongation in cases of extremely preterm preeclampsia. This is a follow-up study presenting our experiences with therapeutic plasma exchange (TPE) in 5 women with preeclampsia at < 28 weeks of gestational age. MATERIALS AND METHODS: All women received standard treatment for preeclampsia and 2 - 3 TPE treatments per week. Blood samples for sFlt-1 and placental growth factor (PlGF) measurements were collected before and after each TPE. RESULTS: Seventeen TPE procedures were performed, 2 - 5 per patient. TPE significantly reduced sFlt-1 (by 35 ± 6%), sFlt-1/PlGF ratio (by 24 ± 13%), and to a lesser degree also PlGF (by 12 ± 16%), with a rebound observed on day 1 post procedure. TPE procedures were well tolerated by pregnant women and fetuses. Stabilization of sFlt-1 allowed pregnancy prolongation for a median of 8 (range 2 - 14) days from first TPE and for a median of 10 (range 4 - 17) days from hospital admission. There were no signs of increased risks of adverse neonatal outcome associated with TPE. One neonate died due to extreme prematurity 3 days after delivery, 2 had bronchopulmonary dysplasia, and 1 had retinopathy of prematurity. CONCLUSION: This study provides new evidence of effective reduction in sFlt-1 and sFlt-1/PlGF ratio with TPE treatment, which seems to allow a clinically meaningful prolongation of pregnancy. Controlled studies are necessary to convincingly show the potential benefit of apheresis treatment in preeclampsia at extremely preterm gestation.


Assuntos
Pré-Eclâmpsia , Biomarcadores , Feminino , Seguimentos , Humanos , Lactente Extremamente Prematuro , Recém-Nascido , Fator de Crescimento Placentário , Troca Plasmática , Pré-Eclâmpsia/diagnóstico , Pré-Eclâmpsia/terapia , Gravidez , Receptor 1 de Fatores de Crescimento do Endotélio Vascular
10.
Reumatol Clin (Engl Ed) ; 17(8): 431-436, 2021 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-34625144

RESUMO

The novel SARS-CoV-2 human coronavirus in Wuhan, China, has triggered a worldwide respiratory disease outbreak (COVID-19). Acute respiratory distress syndrome (ARDS), multiorgan dysfunction and thrombotic events are among the leading causes of death in critically ill patients with COVID-19. The elevated inflammatory cytokines suggest that a "cytokine storm", also known as cytokine release syndrome (CRS), may play a major role in the pathology of COVID-19. In addition to anti-viral therapy and supportive treatment in critically ill patients, unique medications for this condition are also under investigation. Here we reviewed therapeutic options, including the antibody therapy that might be an immediate strategy for SARS-CoV-2 therapy.


Assuntos
COVID-19/terapia , Síndrome da Liberação de Citocina/terapia , Anti-Inflamatórios/uso terapêutico , Anticorpos Monoclonais Humanizados/uso terapêutico , Antivirais/uso terapêutico , COVID-19/complicações , COVID-19/diagnóstico , COVID-19/imunologia , Cloroquina/uso terapêutico , Terapia Combinada , Síndrome da Liberação de Citocina/diagnóstico , Síndrome da Liberação de Citocina/imunologia , Síndrome da Liberação de Citocina/virologia , Fibrinolíticos/uso terapêutico , Glucocorticoides/uso terapêutico , Humanos , Hidroxicloroquina/uso terapêutico , Imunização Passiva , Imunoglobulinas Intravenosas/uso terapêutico , Fatores Imunológicos/uso terapêutico , Troca Plasmática , Inibidores de Proteínas Quinases/uso terapêutico , Terapia Respiratória , Reumatologia , Índice de Gravidade de Doença , Trombose/tratamento farmacológico , Trombose/virologia
11.
BMJ Case Rep ; 14(10)2021 Oct 08.
Artigo em Inglês | MEDLINE | ID: mdl-34625443

RESUMO

A 50-year-old Indian woman presented with acute dysphasia, left upper limb numbness and thrombocytopenia 12 days after receiving the ChAdOx1 nCoV-19 vaccine (AstraZeneca/Vaxzevria). MRI of the brain was unremarkable. Microangiopathic haemolytic anaemia with thrombocytopenia was noted on her peripheral blood film. A diagnosis of thrombotic thrombocytopenic purpura (TTP) was confirmed through the findings of absent ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) activity and markedly raised titre of ADAMTS13 autoantibodies. Prompt treatment with plasma exchange, adjunctive steroids and rituximab was commenced. A remission of TTP was achieved and she was discharged 3 weeks after admission. While other immune-mediated conditions have been documented after receipt of the vaccine, this report highlights the first case of immune-mediated TTP diagnosed after administration of the ChAdOx1 nCoV-19 vaccine.


Assuntos
Púrpura Trombocitopênica Trombótica , Vacinas , Vacinas contra COVID-19 , Feminino , Humanos , Pessoa de Meia-Idade , Troca Plasmática , Púrpura Trombocitopênica Trombótica/induzido quimicamente , Púrpura Trombocitopênica Trombótica/diagnóstico , Púrpura Trombocitopênica Trombótica/tratamento farmacológico , Rituximab/efeitos adversos
12.
J Zhejiang Univ Sci B ; 22(9): 701-717, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34514751

RESUMO

The coronavirus disease 2019 (COVID-19) pandemic is a major public health event caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). COVID-19 has spread widely all over the world. A high proportion of patients become severely or critically ill, and suffer high mortality due to respiratory failure and multiple organ dysfunction. Therefore, providing timely and effective treatment for critically ill patients is essential to reduce overall mortality. Convalescent plasma therapy and pharmacological treatments, such as aerosol inhalation of interferon-α (IFN-α), corticosteroids, and tocilizumab, have all been applied in clinical practice; however, their effects remain controversial. Recent studies have shown that extracorporeal therapies might have a potential role in treating critically ill COVID-19 patients. In this review, we examine the application of continuous renal replacement therapy (CRRT), therapeutic plasma exchange (TPE), hemoadsorption (HA), extracorporeal membrane oxygenation (ECMO), and extracorporeal carbon dioxide removal (ECCO2R) in critically ill COVID-19 patients to provide support for the further diagnosis and treatment of COVID-19.


Assuntos
COVID-19/terapia , Circulação Extracorpórea/métodos , Oxigenação por Membrana Extracorpórea , SARS-CoV-2 , COVID-19/complicações , Dióxido de Carbono/isolamento & purificação , Estado Terminal , Síndrome da Liberação de Citocina/terapia , Hemoperfusão , Humanos , Imunização Passiva , Troca Plasmática , Terapia de Substituição Renal
13.
Rinsho Ketsueki ; 62(8): 1222-1228, 2021.
Artigo em Japonês | MEDLINE | ID: mdl-34497210

RESUMO

Thrombotic thrombocytopenic purpura (TTP) is a rare and potentially life-threatening disease that is characterized by microangiopathic hemolytic anemia, consumptive thrombocytopenia, and ischemic organ damage resulting from the formation of platelet-rich thrombi in the microvasculature. It is especially related to a severe deficiency of ADAMTS13, the specific von Willebrand factor-cleaving protease. Thus, <10% of the normal activity of ADAMTS13 is an essential diagnostic marker for establishing a diagnosis of TTP. TTP can be of congenital form that is termed the Upshaw-Schulman syndrome (USS) that is caused by genetic abnormality of ADAMTS13 and acquired form that is caused by autoantibodies against ADAMTS13. The congenital form is treated with the infusion of fresh frozen plasma, and the treatment of the acquired form involves plasma exchange combined with immunosuppressive therapy that uses corticosteroids and rituximab. Recently, the efficacy and safety of new drugs caplacizumab, single-domain antibody against ADAMTS13, and recombinant ADAMTS13 products have been reported in large-scale clinical trials conducted in other countries. These results suggested the better outcome in the treatment for the patients with TTP in the near future.


Assuntos
Anemia Hemolítica , Púrpura Trombocitopênica Trombótica , Proteína ADAMTS13 , Autoanticorpos , Humanos , Troca Plasmática , Plasmaferese , Púrpura Trombocitopênica Trombótica/diagnóstico , Púrpura Trombocitopênica Trombótica/terapia
14.
Neurologist ; 26(5): 196-224, 2021 Sep 07.
Artigo em Inglês | MEDLINE | ID: mdl-34491938

RESUMO

BACKGROUND: Central nervous system complications are reported in an increasing number of patients with Coronavirus Disease 2019 (COVID-19). COVID-19-related Guillain-Barré syndrome (GBS) is of particular importance given its association with higher mortality rates and prolonged respiratory failure. REVIEW SUMMARY: We conducted a systematic review of published cases for COVID-19-related GBS, and provide a summary of clinical management strategies for these cases. Sixty-three studies, including 86 patients, were included. Seventy-six cases with reported outcome data were eligible for the outcome analysis. Ninety-nine percent of patients were diagnosed with COVID-19 before diagnosis of GBS (median: 14 d prior, interquartile range: 7 to 20). Intravenous immunotherapy (intravenous immunoglobulin: 0.4 g/kg/d for 5 d) was the most frequently used treatment approach. The review indicated that the outcome was not favorable in 26% of cases (persistent neurological deficits). A mortality rate of 3.5% was observed in patients with COVID-19-related GBS. CONCLUSIONS: Although evidence to support specific treatments is lacking, clinicians should consider the benefits of immunotherapy and plasma exchange in addition to the standard antimicrobial and supportive therapies for patients who meet the diagnostic criteria for acute sensory and motor polyradiculoneuritis. Intravenous immunoglobulin treatment alone is not shown to result in improved outcomes or mortality. More extensive studies aimed at exploring the neurological manifestations and complications of COVID-19 and distinctive treatment options for COVID-19-related GBS are warranted.


Assuntos
COVID-19/tratamento farmacológico , Síndrome de Guillain-Barré/tratamento farmacológico , Imunoglobulinas Intravenosas/uso terapêutico , SARS-CoV-2/efeitos dos fármacos , Neoplasias da Glândula Tireoide/tratamento farmacológico , Síndrome de Guillain-Barré/diagnóstico , Humanos , Troca Plasmática/métodos , Plasmaferese/efeitos adversos , Plasmaferese/métodos
15.
Radiology ; 301(1): 242-246, 2021 10.
Artigo em Inglês | MEDLINE | ID: mdl-34543144

RESUMO

History A 50-year-old woman presented to the emergency department of our hospital with a 2-day history of lower limb pain associated with unusual asthenia and diffuse arthralgia over the past 3 weeks. She was a native of Guinea and had lived in France for most of her life, working as a personal care assistant. Her only medical history of note was an occurrence of fetal death at 12 weeks gestation when she was 35 years old. She had bilateral lower limb swelling, without changes in skin temperature or color. All proximal and distal arterial pulses were felt. General physical examination findings were otherwise unremarkable. Her laboratory tests showed a decreased hemoglobin concentration of 8.9 g/dL (normal range, 12-16 g/dL), a decreased platelet count of 45 × 109/L (normal range, [150-400] × 109/L), a C-reactive protein level of 158 mg/L (normal range, <5 mg/L), and a d-dimer level of 2000 mg/L (normal range, <500 mg/L]). Compression US of the lower limbs revealed bilateral calf vein thrombosis involving the fibular and posterior tibial veins. Curative anticoagulation using low-molecular-weight heparin (enoxaparin, subcutaneous injection of 100 units per kilogram of body weight twice a day) was started. The day after the start of anticoagulation therapy, the patient reported dyspnea and acute chest and abdominal pain. Her vital signs were assessed, and she had elevated blood pressure and increased heart rate and respiratory rate, but she remained afebrile. Her cardiac auscultation was unremarkable, besides tachycardia. Skin examination revealed small areas of necrosis on the fingertips of her right hand. Laboratory studies were repeated and showed an increase in serum creatinine level from a baseline value of 0.49 mg/dL to a new value of 1.01 mg/dL (normal range, 0.6-1.1 mg/dL), an apparition of low-grade proteinuria of 0.43 g per day (normal range, <0.3 g/ day), and a high serum troponin level of 1066 ng/L (normal range, <14 ng/L), whereas electrocardiography showed no ST segment modification and echocardiography revealed a moderately altered left ventricular ejection fraction (45%). There was no coronary occlusion seen at emergency coronarography. Contrast-enhanced CT of the chest, abdomen, and pelvis was performed (Figs 1, 2) together with cardiac MRI (Figs 3, 4).


Assuntos
Síndrome Antifosfolipídica/complicações , Síndrome Antifosfolipídica/diagnóstico , Trombose Venosa/diagnóstico por imagem , Trombose Venosa/etiologia , Anticoagulantes/uso terapêutico , Síndrome Antifosfolipídica/terapia , Aspirina/uso terapêutico , Enoxaparina/uso terapêutico , Feminino , Humanos , Extremidade Inferior/diagnóstico por imagem , Pulmão/diagnóstico por imagem , Imageamento por Ressonância Magnética/métodos , Metilprednisolona/uso terapêutico , Pessoa de Meia-Idade , Troca Plasmática/métodos , Tomografia Computadorizada por Raios X/métodos , Ultrassonografia/métodos , Trombose Venosa/terapia
16.
BMJ Case Rep ; 14(8)2021 Aug 11.
Artigo em Inglês | MEDLINE | ID: mdl-34380671

RESUMO

Thrombotic thrombocytopenic purpura (TTP) is a life-threatening disease characterised by thrombocytopenia, microangiopathic haemolytic anaemia and microvascular thrombosis. Congenital TTP accounting for less than 5% of all TTP cases can have a late presentation in adulthood mostly triggered by predisposing factors such as infection, pregnancy and inflammation. We present a case of a 23-year-old woman who presented to us in the postpartum period with mesenteric artery thrombosis with infarcts and later was diagnosed as a case of TTP based on congenital a disintegrin and metalloproteinase with thrombospondin type 1 repeats 13 (ADAMTS-13) deficiency detected on ADAMTS-13 levels and gene sequencing. She was successfully managed initially with therapeutic plasma exchanges and is now on prophylactic fortnightly fresh frozen plasma infusions at 15 mL/kg body weight and continues to be in remission.


Assuntos
Proteína ADAMTS13/deficiência , Anemia Hemolítica , Púrpura Trombocitopênica Trombótica , Trombose , Proteína ADAMTS13/genética , Feminino , Humanos , Troca Plasmática , Gravidez , Púrpura Trombocitopênica Trombótica/diagnóstico , Púrpura Trombocitopênica Trombótica/terapia , Adulto Jovem
18.
Adv Chronic Kidney Dis ; 28(1): 59-73, 2021 01.
Artigo em Inglês | MEDLINE | ID: mdl-34389138

RESUMO

Therapeutic plasma exchange (TPE) is frequently the most common Apheresis Medicine technique used for extracorporeal therapy of a wide variety of renal, neurological, hematological, and other clinical indications. Many of these clinical indications require intensive care during critical illness. Conventional TPE uses one of two main technical methods to achieve the goal of removing known disease mediators from the plasma: using centrifugal forces to separate and remove components of blood, or a membrane filtration method that separates plasma from the cellular components of blood. The following review discusses the basic principles of TPE, the technological aspects, and relevant clinical scenarios encountered in the intensive care unit, including relevant guidelines and recommendations from the American Society for Apheresis.


Assuntos
Estado Terminal , Troca Plasmática , Humanos , Unidades de Terapia Intensiva , Plasmaferese , Tecnologia
19.
Hematology ; 26(1): 590-593, 2021 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-34396933

RESUMO

Methods: We report a case of a 20-year-old Nigerian male who presented with acquired thrombotic thrombocytopenic purpura (aTTP) and sickle cell trait. The coexistence of published cases of TTP and sickle cell hemoglobinopathies is rare.Results: Despite the initial treatment with plasma exchange and glucocorticoids, our patient relapsed and also required caplacizumab which resulted in successful remission.Discussion: We conclude by reviewing the cases of TTP in patients with sickle cell hemoglobinopathies and review how vaso-occlusive crises with multiorgan injury can mimic TTP.Conclusion: Ours is the first published case of aTTP with confirmed ADAMTS13 autoantibodies in a patient with a sickle cell hemoglobinopathy and contributes to the literature on the successful use of caplacizumab in clinical practice.


Assuntos
Fibrinolíticos/uso terapêutico , Púrpura Trombocitopênica Trombótica/complicações , Púrpura Trombocitopênica Trombótica/tratamento farmacológico , Traço Falciforme/complicações , Anticorpos de Domínio Único/uso terapêutico , Adulto , Humanos , Masculino , Troca Plasmática , Púrpura Trombocitopênica Trombótica/terapia , Adulto Jovem
20.
Medicine (Baltimore) ; 100(29): e26587, 2021 Jul 23.
Artigo em Inglês | MEDLINE | ID: mdl-34398013

RESUMO

ABSTRACT: Poor availability and a lack of affordability of bypassing agents (recombinant activated factor VII and activated prothrombin complex concentrate) in west China prompted us to investigate an alternative cost-effective combination therapy. We aimed to explore the feasibility of therapeutic plasma exchange (TPE)-based combination therapy in the treatment of acquired hemophilia A (AHA).We retrospectively investigated the clinical features of AHA in 6 patients who were treated with a combination of TPE, corticosteroids, and rituximab in our department for 9 years between January, 2011 and December, 2019.We examined 1 male and 5 female patients. The median age at diagnosis of AHA was 51 years (18-66 years). In all patients, FVIII activity levels were low (median: 1.5%; 1-3%), FVIII inhibitor titers were high (median: 24.5 BU/mL; 13.2-48.6 BU/mL), and activated partial thromboplastin time was markedly prolonged (median: 99.4 s; 60.9-110.1 s). They underwent 2 to 8 cycles of plasma exchange and were given varying combinations of dexamethasone, methylprednisolone, prednisone, and rituximab. After TPE bleeding gradually stopped, and activated partial thromboplastin time decreased. After 3 months of treatment, FVIII inhibitors completely disappeared.TPE when combined with corticosteroids and rituximab, as adjunctive immunosuppressive agents, may be an effective and reliable treatment for AHA. When there is no alternative, intensive first-line treatment including TPE may be lifesaving.


Assuntos
Hemofilia A/terapia , Troca Plasmática/normas , Adulto , China , Quimioterapia Combinada/normas , Quimioterapia Combinada/estatística & dados numéricos , Estudos de Viabilidade , Feminino , Humanos , Imunossupressores/uso terapêutico , Masculino , Troca Plasmática/métodos , Troca Plasmática/estatística & dados numéricos , Estudos Retrospectivos
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