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1.
Medicine (Baltimore) ; 99(6): e18984, 2020 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-32028408

RESUMO

RATIONALE: Autoimmune hemolytic AQ5 anemia (AIHA) is an immune disorder caused by antibodies directed against unmodified autologous red blood cells. In rare cases, AIHA is comorbid with other immunological disorders; for instance, when AIHA is complicated with immunologic thrombocytopenic purpura (ITP) it is called Evans Syndrome (ES). These multiple autoimmune mechanisms are referred to as "immunological tolerance loss," which is known as a characteristic autoimmunity specific for AIHA. And there are no estimation of the risk for thromboembolism in the "immunological tolerance loss" case. PATIENT CONCERNS: A 66-year-old man was diagnosed with ES after autologous stem cell transplantation for malignant lymphoma. His background immunological status was complicated because AIHA was mixed-type (warm and cold antibody type). The direct/indirect Coombs tests were positive. The anticomplement antibody was positive and his cold hemagglutinin level had increased. Anticardiolipin antibodies were negative: anticardiolipin ß2GPI antibody ≤1.2 U/mL (<3.5), anticardiolipin immunoglobulin G antibody ≤8 U/mL (<10), and anticardiolipin immunoglobulin M antibody ≤5 U/mL (<8). DIAGNOSES: ITP and mixed-type AIHA. INTERVENTIONS: The patient achieved complete response by initial prednisolone therapy; however, he did not respond to corticosteroid therapy after AIHA recurrence. He required the red blood cell transfusion due to the progression of hemolytic anemia. OUTCOMES: On the fourth day of refractory treatment following AIHA recurrence, the patient had acute respiratory failure with severe hypoxia and died. The cause of death was identified as pulmonary embolism (PE) based on the laboratory data and echocardiography findings, and a literature search suggested rapidly progressive hemolysis-induced PE. LESSONS: Although infrequent, comorbid thromboembolism to AIHA is well documented; however, a mixed-type AIHA case complicated with thromboembolism has not been previously reported. The combined pathophysiology of AIHA and thromboembolism should be considered in the clinical course of hemolysis. Our case suggested multiple immunological background, ITP, and mixed type AIHA, could be associated to a risk for thromboembolism (TE).


Assuntos
Anemia Hemolítica Autoimune/complicações , Embolia Pulmonar/etiologia , Idoso , Anemia Hemolítica Autoimune/diagnóstico , Evolução Fatal , Humanos , Masculino , Embolia Pulmonar/diagnóstico , Púrpura Trombocitopênica Idiopática/complicações , Púrpura Trombocitopênica Idiopática/diagnóstico , Trombocitopenia/complicações , Trombocitopenia/diagnóstico
2.
Medicine (Baltimore) ; 99(4): e18887, 2020 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-31977897

RESUMO

INTRODUCTION: MYH9-related disease (MYH9-RD) is a rare autosomal dominant disorder caused by mutations in MYH9, which is responsible for encoding nonmuscle myosin heavy chains IIA (NMMHCIIA). MYH9-RD is clinically characterized by congenital macrothrombocytopenia, granulocyte inclusions variably associated with the risk of developing progressive sensorineural deafness, cataracts and nephropathy. PATIENT CONCERNS: A 5-year-old boy had a history of a mild bleeding tendency and chronic thrombocytopenia, first identified at four months of age. No other family members were noted to have similar clinical features or hematologic disorders. DIAGNOSES: The boy was diagnosed with MYH9-RD. Light microscopic examination of peripheral blood films (Wright-Giemsa stain) showed marked platelet macrocytosis with giant platelets and basophilic Döhle-like inclusions in 83% of the neutrophils. Immunofluorescence analysis disclosed a type II pattern, manifested by neutrophils which contained several circle-to-oval shaped cytoplasmic NMMMHCA-positive granules. Sequencing analysis of MYH9-RD genes was carried out and revealed a novel missense mutation of c.97T>G (p.W33G) in the patient but not in his parents. INTERVENTION: No treatment is necessary. Recognition of MYH9-RD is important to Avoiding unnecessary and potentially harmful treatments. OUTCOMES: The patient's condition remained stable during the follow-up. CONCLUSIONS: As a result of identifying this missense mutation in this particular case, we have added c.97T>G (p.W33G) to the broad spectrum of potential MYH9 mutations.


Assuntos
Perda Auditiva Neurossensorial/genética , Trombocitopenia/congênito , Pré-Escolar , Imunofluorescência , Perda Auditiva Neurossensorial/diagnóstico , Sequenciamento de Nucleotídeos em Larga Escala , Humanos , Masculino , Mutação de Sentido Incorreto , Neutrófilos/patologia , Trombocitopenia/diagnóstico , Trombocitopenia/genética
3.
Rev Med Liege ; 74(11): 616-619, 2019 Nov.
Artigo em Francês | MEDLINE | ID: mdl-31729852

RESUMO

Inherited platelet disorders (IPD) include a set of rare diseases whose diagnosis is often difficult because it requires the use of complex biological assays in specialized centers. They are probably under-diagnosed. Clinicians should consider an IPD when facing a chronic thrombocytopenia resistant to intravenous immunoglobulins (IVIG) and steroids together with a family history of thrombocytopenia. A syndromic thrombocytopenia will be suspected by the family survey and specific clinical signs. The confirmation of the diagnosis will then require the use of specialized biological assays such as platelet aggregation, flow cytometry, electron microscopy, platelet secretion assays, karyotype and molecular biology.


Assuntos
Trombocitopenia , Humanos , Imunoglobulinas Intravenosas , Anamnese , Contagem de Plaquetas , Trombocitopenia/diagnóstico , Trombocitopenia/genética , Trombocitopenia/terapia
4.
FP Essent ; 485: 32-43, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-31613566

RESUMO

Platelets have an important role in hemostasis. Platelet disorders occur when too few or too many platelets are present, or when platelet functions are abnormal. Thrombocytopenia, defined as a platelet count less than 150,000/mcL, can be acute or chronic and congenital or acquired. Severe thrombocytopenia is associated with life-threatening bleeding and thrombotic complications. A comprehensive history and physical examination are central to the diagnostic approach. These elements should focus on identification of concurrent conditions associated with thrombocytopenia and differentiation among three mechanisms: decreased platelet production, increased platelet consumption, and platelet sequestration. Although previously thought to be the result of a single process, thrombocytopenia often is due to a combination of factors. Thrombocytosis is present when the platelet count is elevated. The principal types are essential (primary) thrombocythemia and reactive (secondary) thrombocytosis. Essential thrombocythemia is a myeloproliferative neoplasm associated with mutations of genes that regulate thrombopoiesis (eg, JAK2). It can lead to thrombotic and hemorrhagic complications. Reactive thrombocytosis frequently is encountered in the family medicine setting. It rarely causes vascular complications or requires management beyond that required for the underlying condition. Written permission from the American Academy of Family Physicians is required for reproduction of this material in whole or in part in any form or medium.


Assuntos
Anemia , Trombocitopenia , Trombocitose , Anemia/diagnóstico , Anemia/terapia , Plaquetas , Humanos , Contagem de Plaquetas , Trombocitopenia/diagnóstico , Trombocitopenia/terapia , Trombocitose/diagnóstico , Trombocitose/terapia
7.
Res Vet Sci ; 126: 45-50, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-31430579

RESUMO

The study objectivs were to evaluate the correlation between platelet count (PLT) and rotational thromboelastometry (ROTEM) parameters and to determine ROTEM cut-off values for identification of thrombocytopenia in dogs. Medical records of 113 dogs with concurrent EXTEM (ROTEM activated by proprietary tissue factor), FIBTEM (EXTEM with added cytochalasin D) analysis and PLT were retrospectively reviewed. Signalment, treatment prior to analysis, hematocrit (HCT), EXTEM/FIBTEM maximum clot firmness (MCFEXTEM, MCFFIBTEM), EXTEM/FIBTEM maximum clot elasticity (MCEEXTEM, MCEFIBTEM) and EXTEM maximum lysis (MLEXTEM) were extracted from patient records and ROTEM database. Delta (Δ) MCF was calculated as MCFEXTEM-MCFFIBTEM and ΔMCE as MCEEXTEM-MCEFIBTEM. The PLT was correlated to MCFEXTEM, MCEEXTEM, ΔMCF and ΔMCE using Spearman-Rho analysis. Correlations were further analyzed in thrombocytopenic dogs. The ability to predict thrombocytopenia was evaluated with receiver operating characteristics (ROC). Thirty-seven samples (32.7%) showed thrombocytopenia (<130 × 109/L) and 19 samples (17%) severe thrombocytopenia (<60* x 109/L). The PLT significantly correlated with MCFEXTEM (r = 0.545, P < .001), MCEEXTEM (r = 0.547, P < .001), ΔMCF (r = 0.441, P < .001) and ΔMCE (r = 0.559, P < .001). MCFEXTEM < 49 mm, MCEEXTEM < 93, ΔMCF <42 mm and ΔMCE <90 predicted thrombocytopenia <60 × 109/L with a sensitivity of 90% and a specificity of 78% with a negative predictive value >97% for all 4 parameters. In conclusion, PLT in dogs correlated moderately but significantly with all evaluated ROTEM parameters. All parameters were able to rule out thrombocytopenia <60 × 109/L with a high negative predictive value, while the sensitivity to predict thrombocytopenia was only moderate and the positive predictive value was low.


Assuntos
Contagem de Plaquetas/veterinária , Tromboelastografia/veterinária , Trombocitopenia/veterinária , Animais , Cães , Feminino , Hematócrito/veterinária , Humanos , Masculino , Curva ROC , Estudos Retrospectivos , Sensibilidade e Especificidade , Tromboelastografia/métodos , Trombocitopenia/diagnóstico
8.
J Korean Med Sci ; 34(30): e208, 2019 Aug 05.
Artigo em Inglês | MEDLINE | ID: mdl-31373186

RESUMO

BACKGROUND: Performing transarterial chemoembolization (TACE) is difficult with the occurrence of thrombocytopenia in cirrhotic patients with hepatocellular carcinoma (HCC). We aimed to evaluate the long-term efficacy and safety of partial splenic embolization (PSE) combined with TACE in patients with HCC with severe thrombocytopenia related to splenomegaly. METHODS: We conducted a case-control study consisting of 18 HCC patients with severe thrombocytopenia (< 50 × 109/L) who underwent PSE concurrently with TACE (PSE group) and 72 controls who underwent TACE alone (non-PSE group). RESULTS: Mean platelet counts at 1 month and 1, 3, and 5 years after concurrent PSE and TACE significantly increased compared with baseline (all P < 0.05), whereas the platelet count did not significantly increase after TACE alone. In addition, the platelet count at several time points after treatment in the PSE group was significantly higher than that in the non-PSE group, although the baseline platelet count in the PSE group was significantly lower than that in the non-PSE group. The platelet increase after PSE significantly reduced the need for platelet transfusions (P = 0.040) and enabled the subsequent TACE procedures in time (P = 0.046). The leukocyte counts and hemoglobin concentrations after concurrent PSE and TACE were also significantly increased, without deterioration of Child-Turcotte-Pugh score and unexpected side effects. CONCLUSION: PSE combined with TACE is effective in inducing and maintaining long-term thrombocytopenia improvement which reduces the need for the platelet transfusion and helps to perform initial and serial TACE, and is well-tolerated in patients with HCC and thrombocytopenia. PSE may be a promising treatment option for HCC patients with severe thrombocytopenia associated with splenomegaly who will undergo TACE.


Assuntos
Carcinoma Hepatocelular/terapia , Embolização Terapêutica/métodos , Neoplasias Hepáticas/terapia , Trombocitopenia/diagnóstico , Adulto , Idoso , Carcinoma Hepatocelular/complicações , Carcinoma Hepatocelular/mortalidade , Estudos de Casos e Controles , Quimioembolização Terapêutica , Feminino , Hemoglobinas/análise , Humanos , Contagem de Leucócitos , Neoplasias Hepáticas/complicações , Neoplasias Hepáticas/mortalidade , Masculino , Pessoa de Meia-Idade , Contagem de Plaquetas , Estudos Retrospectivos , Artéria Esplênica/cirurgia , Esplenomegalia/complicações , Esplenomegalia/diagnóstico , Taxa de Sobrevida , Trombocitopenia/complicações
9.
Ann Hematol ; 98(10): 2299-2302, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-31444663

RESUMO

Iron deficiency anemia (IDA) is often associated with mild to moderate thrombocytosis, and iron deficiency-associated thrombocytopenia (IDAT) is much more uncommon and often misdiagnosed as immune thrombocytopenia (ITP). To better describe the features of IDAT, we conducted a retrospective multicenter case-control study. We identified 10 patients (9 women) with a definite diagnosis IDAT, with a median age of 43.5 [range, 16-72] years and a median platelet count of 30.5 × 109/L [range, 21-80], and 7 patients with a possible diagnosis of IDAT. Bleeding manifestations were absent in all patients but one. All the patients recovered (platelet count ≥ 150 × 109/L) upon iron therapy ± red blood cell transfusion after a median time of 6 [4-39] days. When compared with 30 randomly newly diagnosed ITP patients matched on age, the baseline platelet count was significantly lower in ITP (median = 7 × 109/L [4-59], p < 0.001) whereas MPV was higher (10.5 fL [9,4-13,8] vs 8.2 fL, for IDAT p < 0.001). The median platelet count on day 7 was 337 × 109/L [113-1000] for IDAT cases vs 72 × 109/L [13-212] for ITP controls (p < 0.001). IDAT is potentially an under-recognized cause of thrombocytopenia that may be easily managed with iron therapy.


Assuntos
Anemia Ferropriva , Trombocitopenia , Adolescente , Adulto , Fatores Etários , Idoso , Anemia Ferropriva/sangue , Anemia Ferropriva/complicações , Anemia Ferropriva/diagnóstico , Anemia Ferropriva/epidemiologia , Erros de Diagnóstico , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Contagem de Plaquetas , Púrpura Trombocitopênica Idiopática/sangue , Púrpura Trombocitopênica Idiopática/diagnóstico , Púrpura Trombocitopênica Idiopática/epidemiologia , Púrpura Trombocitopênica Idiopática/etiologia , Estudos Retrospectivos , Trombocitopenia/sangue , Trombocitopenia/diagnóstico , Trombocitopenia/epidemiologia , Trombocitopenia/etiologia
10.
Emerg Microbes Infect ; 8(1): 1122-1125, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31347462

RESUMO

The Xinjiang Uygur Autonomous Region locating in Northwest of China was not considered the epidemic area of severe fever with thrombocytopenia syndrome (SFTS). Here we report the first laboratory-confirmed SFTS case that a female patient had tick bite in Xinjiang and illness onset after returning to Hainan Province. Laboratory tests identified SFTS virus (SFTSV) infection, and the virus was isolated from the patient's serum sample. Furthermore, SFTSV prevalence among tick groups was identified, and IgM response to SFTSV from febrile patients was identified. The findings suggested that there have been risks of SFTSV infection due to exposure to ticks in Xinjiang.


Assuntos
Infecções por Bunyaviridae/diagnóstico , Phlebovirus/isolamento & purificação , Trombocitopenia/diagnóstico , Animais , Infecções por Bunyaviridae/sangue , Infecções por Bunyaviridae/transmissão , Infecções por Bunyaviridae/virologia , China , Feminino , Humanos , Pessoa de Meia-Idade , Phlebovirus/classificação , Phlebovirus/genética , Phlebovirus/fisiologia , Filogenia , Trombocitopenia/sangue , Trombocitopenia/virologia , Carrapatos/fisiologia , Carrapatos/virologia , Viagem
11.
Gene ; 713: 143964, 2019 Sep 10.
Artigo em Inglês | MEDLINE | ID: mdl-31279707

RESUMO

This study aimed to investigate single-nucleotide polymorphisms (SNPs) associated with lobaplatin-induced thrombocytopenia in patients with advanced lung cancer in China. Thirty-nine patients who received lobaplatin-based chemotherapy in the 307 Hospitals of Chinese People's Liberation Army from April 2017 to March 2018 were enrolled as study subjects. Peripheral blood DNA was extracted, and 79 candidate SNP positions were selected. A Sanger sequencing platform was employed to measure genotypes for locating the SNP positions associated with lobaplatin-induced thrombocytopenia. Of the 79 candidate genes, SNPs rs342275 and rs7694379 were significantly associated with lobaplatin-induced decrease in platelet (PLT) count (P < 0.05). SNPs rs342275, rs342293, rs11789898, and rs17824620 showed significant association with lobaplatin-induced lowest PLT counts (P < 0.05). SNPs rs342275, rs342293, rs11789898, rs17824620, and rs7694379 can be used as predictors of thrombocytopenia induced by lobaplatin-based chemotherapy in patients with advanced lung cancer in China.


Assuntos
Adenocarcinoma/tratamento farmacológico , Biomarcadores Tumorais/genética , Carcinoma de Células Escamosas/tratamento farmacológico , Ciclobutanos/efeitos adversos , Compostos Organoplatínicos/efeitos adversos , Polimorfismo de Nucleotídeo Único , Carcinoma de Pequenas Células do Pulmão/tratamento farmacológico , Trombocitopenia/diagnóstico , Adenocarcinoma/patologia , Adulto , Idoso , Carcinoma de Células Escamosas/patologia , China , Feminino , Seguimentos , Humanos , Neoplasias Pulmonares/tratamento farmacológico , Neoplasias Pulmonares/patologia , Masculino , Pessoa de Meia-Idade , Prognóstico , Carcinoma de Pequenas Células do Pulmão/patologia , Trombocitopenia/induzido quimicamente , Trombocitopenia/genética
12.
Mayo Clin Proc ; 94(9): 1753-1768, 2019 09.
Artigo em Inglês | MEDLINE | ID: mdl-31256854

RESUMO

OBJECTIVE: To demonstrate experience and feasibility of a precision medicine approach for patients with unexplained cytopenias, defined as low blood counts in one or more cell lineages, persistent for 6 months or longer, in the absence of known nutritional, autoimmune, infectious, toxic, and neoplastic (secondary) causes. PATIENTS AND METHODS: Patients were evaluated in our clinic between November 8, 2016, and January 12, 2018. After a thorough evaluation of known causes, family history, and appropriate clinical assays, genomic evaluation was performed in a stepwise manner, through Sanger, targeted, and/or whole-exome sequencing. Variants were analyzed and discussed in a genomics tumor board attended by clinicians, bioinformaticians, and molecular biologists. RESULTS: Sixty-eight patients were evaluated in our clinic. After genomic interrogation, they were classified into inherited bone marrow failure syndromes (IBMFS) (n=24, 35%), cytopenias without a known clinical syndrome which included idiopathic and clonal cytopenias of undetermined significance (CCUS) (n=30, 44%), and patients who did not fit into the above two categories ("others," n=14, 21%). A significant family history was found in only 17 (25%) patients (9 IBMFS, 2 CCUS, and 6 others), whereas gene variants were found in 43 (63%) patients (34 [79%] pathogenic including 12 IBMFS, 17 CCUS, and 5 others]. Genomic assessment resulted in a change in clinical management in 17 (25%) patients, as evidenced by changes in decisions with regards to therapeutic interventions (n=8, 47%), donor choice (n=6, 35%), and/or choice of conditioning regimen for hematopoietic stem cell transplantation (n=8, 47%). CONCLUSION: We show clinical utility of a real-world algorithmic precision medicine approach for unexplained cytopenias.


Assuntos
Contagem de Células Sanguíneas/métodos , /terapia , Sintomas Inexplicáveis , Medicina de Precisão/métodos , Medicina de Precisão/estatística & dados numéricos , Centros Médicos Acadêmicos , Adolescente , Adulto , Anemia/diagnóstico , Anemia/terapia , /mortalidade , Criança , Estudos de Coortes , Feminino , Genômica , Doenças Hematológicas/diagnóstico , Doenças Hematológicas/terapia , Humanos , Leucopenia/diagnóstico , Leucopenia/terapia , Masculino , Pessoa de Meia-Idade , Neutropenia/diagnóstico , Neutropenia/terapia , Pancitopenia/diagnóstico , Pancitopenia/terapia , Prognóstico , Estudos Retrospectivos , Medição de Risco , Índice de Gravidade de Doença , Taxa de Sobrevida , Trombocitopenia/diagnóstico , Trombocitopenia/terapia , Resultado do Tratamento , Adulto Jovem
13.
Med J Aust ; 210(11): 509-516, 2019 06.
Artigo em Inglês | MEDLINE | ID: mdl-31155728

RESUMO

INTRODUCTION: Heparin-induced thrombocytopenia (HIT) is a prothrombotic disorder that occurs following the administration of heparin and is caused by antibodies to platelet factor 4 and heparin. Diagnosis of HIT is essential to guide treatment strategies using non-heparin anticoagulants and to avoid unwanted and potential fatal thromboembolic complications. This consensus statement, formulated by members of the Thrombosis and Haemostasis Society of Australia and New Zealand, provides an update on HIT pathogenesis and guidance on the diagnosis and management of patients with suspected or confirmed HIT. MAIN RECOMMENDATIONS: A 4Ts score is recommended for all patients with suspected HIT prior to laboratory testing. Further laboratory testing with a screening immunoassay or confirmatory functional assay is not recommended in individuals with a low 4Ts score. However, if there are missing or unreliable clinical data, then laboratory testing should be performed. A positive functional assay result confirms the diagnosis of HIT and should be performed to confirm a positive immunoassay result. Heparin exposure must be ceased in patients with suspected or confirmed HIT and initial treatment with a non-heparin alternative instituted. Non-heparin anticoagulants (danaparoid, argatroban, fondaparinux and bivalirudin) used to treat HIT should be given in therapeutic rather than prophylactic doses. Direct oral anticoagulants may be used in place of warfarin after patients with HIT have responded to alternative parenteral anticoagulants with platelet count recovery. CHANGES IN MANAGEMENT AS A RESULT OF THIS STATEMENT: These are the first Australasian recommendations for diagnosis and management of HIT, with a focus on locally available diagnostic assays and therapeutic options. The importance of examining both clinical and laboratory data in considering a diagnosis of HIT cannot be overstated.


Assuntos
Anticoagulantes/efeitos adversos , Heparina/efeitos adversos , Trombocitopenia/diagnóstico , Trombocitopenia/tratamento farmacológico , Anticoagulantes/uso terapêutico , Austrália , Consenso , Hemorragia/induzido quimicamente , Heparina/imunologia , Humanos , Nova Zelândia , Fator Plaquetário 4/imunologia , Receptores de IgG/imunologia , Trombocitopenia/induzido quimicamente , Trombose/etiologia
14.
Chin Med J (Engl) ; 132(12): 1441-1447, 2019 Jun 20.
Artigo em Inglês | MEDLINE | ID: mdl-31205102

RESUMO

BACKGROUND: Diagnosis of heparin-induced thrombocytopenia (HIT) is challenging. This study aimed to compare the diagnostic performance of HIT expert probability (HEP) and 4T scores, and to evaluate the inter-observer reliability for the 4T score in a clinical setting. METHODS: This prospective study included HIT-suspected patients between 2016 and 2018. Three hematologists assessed the HEP and 4T scores. Correlations between scores and anti-platelet factor 4 (anti-PF4)/heparin antibodies were evaluated. Receiver operating characteristic curves and area under the curve (AUC) were used to assess the predictive accuracy of these two scoring models. The intraclass correlation coefficient (ICC) was used to assess the inter-observer agreement of 4T scores between residents and hematologists. RESULTS: Of the 89 subjects included, 22 (24.7%) were positive for anti-PF4/heparin antibody. The correlations between antibody titer and either HEP or 4T scores were similar (r = 0.392, P < 0.01 for the HEP score; r = 0.444, P < 0.01 for the 4T score). No significant difference in the diagnostic performance was displayed between these two scores (AUC for the HEP score: 0.778 vs. AUC for the 4T score: 0.741, P = 0.357). Only 72 4T scores were collected from the residents, with a surprisingly low percentage of observers (43.1%) presenting the four individual item scores which made up their 4T score. The AUC of 4T score assessed by residents and hematologists was 0.657 (95% confidence interval [CI]: 536-0.765) and 0.780 (95% CI: 0.667-0.869, P < 0.05), respectively. The ICC of 4T score between residents and hematologists was 0.49 (95% CI: 0.29-0.65, P < 0.01), demonstrating a fair inter-observer agreement. CONCLUSIONS: The HEP score does not display a better performance for predicting HIT than the 4T score. With the unsatisfactory completion rate, the inter-observer agreement of 4T score in a tertiary hospital is fair, underscoring the necessity for continuing education for physicians.


Assuntos
Heparina/toxicidade , Trombocitopenia/induzido quimicamente , Trombocitopenia/diagnóstico , Adulto , Idoso , Ensaio de Imunoadsorção Enzimática , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Observacionais como Assunto , Estudos Prospectivos , Curva ROC , Centros de Atenção Terciária/estatística & dados numéricos
15.
AACN Adv Crit Care ; 30(2): 165-180, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31151947

RESUMO

Evaluating, diagnosing, and managing patients with consumptive thrombocytopenia is challenging because of the overlapping nature of many of the diseases that reduce platelet counts. Immune thrombocytopenia (and its variations), drug-induced immune thrombocytopenia, and heparin-induced thrombocytopenia result from autoimmune antibody-mediated destruction of platelets. Thrombotic thrombocytopenia (both congenital and acquired) and the hemolytic uremic syndromes (both typical and atypical) are thrombotic microangiopathies associated with platelet aggregation and consumption along with anemia and renal dysfunction. Rapid history taking, physical assessment, and laboratory evaluation are crucial to accurately managing patients with these disorders. Platelet-associated coagulopathies are infrequently encountered by most providers, and limited exposure to these types of patients, combined with the wide variety of treatment options for reversing bleeding or thrombotic sequelae, makes management difficult. This article reviews the pathophysiology, patient presentation, diagnostic testing, and specific management strategies and challenges of these thrombocytopenias.


Assuntos
Enfermagem de Cuidados Críticos , Trombocitopenia/diagnóstico , Trombocitopenia/enfermagem , Enfermagem Baseada em Evidências , Humanos , Diagnóstico de Enfermagem
16.
BMC Infect Dis ; 19(1): 498, 2019 Jun 07.
Artigo em Inglês | MEDLINE | ID: mdl-31174484

RESUMO

BACKGROUND: Cases of severe fever with thrombocytopenia syndrome (SFTS) have increasingly been observed in Miyazaki, southwest Japan. It is critical to identify and elucidate the risk factors of infection at community level. In the present study, we aimed to identify areas with a high risk of SFTS virus infection using a geospatial dataset of SFTS cases in Miyazaki. METHODS: Using 10 × 10-km mesh data and a geographically weighted logistic regression (GWLR) model, we examined the statistical associations between environmental variables and spatial variation in the risk of SFTS. We collected geospatial and population census data as well as forest and agriculture mesh information. Altitude and farmland were selected as two specific variables for predicting the presence of SFTS cases in a given mesh area. RESULTS: Using GWLR, the area under the receiver operating characteristic curve (AUC) was estimated at 73.9%, outperforming the classical logistic regression model (72.4%). The sensitivity and specificity of the GWLR model were estimated at 90.9 and 58.7%, respectively. We identified altitude (odds ratio (OR) = 0.996, 95% confidence interval (CI): 0.994-0.999 per one-meter elevation) and farmland (OR = 0.999, 95% CI: 0.998-1.000 per % increase) as useful negative predictors of SFTS cases in Miyazaki. CONCLUSIONS: Our study findings revealed that the risk of SFTS is high in geographic areas where farmland area begins to diminish and at mid-level altitudes. Our findings can help to improve the efficiency of ecological and animal surveillance in high-risk areas. Using finer geographic resolution, such surveillance can help raise awareness among local residents in areas with a high risk of SFTS.


Assuntos
Febre/etiologia , Modelos Teóricos , Trombocitopenia/diagnóstico , Animais , Área Sob a Curva , Humanos , Japão/epidemiologia , Modelos Logísticos , Razão de Chances , Curva ROC , Trombocitopenia/epidemiologia
17.
Thromb Res ; 180: 55-61, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-31220752

RESUMO

BACKGROUND: Heparin-induced thrombocytopenia (HIT) is caused by platelet-activating antibodies that recognize platelet factor 4/heparin (PF4/hep)-complexes. The in vitro demonstration of PF4/hep antibodies using functional assays is essential for an optimal management of patients suspected to have HIT. However, conventional functional assays are technically challenging and limited to specialized laboratories. In contrast, flow cytometers are commonly used in routine laboratories. The aim of this study is to investigate the performance characteristics of a commercially available, flow cytometer based assay in the diagnosis of HIT. STUDY DESIGN: Sera of consecutive patients with suspected HIT were investigated using the Emo-test HIT Confirm® assay and compared to the standard method consisting of an IgG-specific enzyme immunoassay (EIA) for anti-PF4/hep antibodies and the heparin induced platelet aggregation (HIPA) test. RESULTS: 390 sera were included in the study, 164 sera tested IgG EIA-positive, of which 33 also tested HIPA-positive. No HIPA-positive samples were EIA-negative. In the Emo-test HIT Confirm® assay, 112 sera revealed positive results (%Hepla > 13); however, 51 (45.5%) were EIA-negative. Of the 33 HIPA-positive/EIA-positive HIT sera, 23 tested positive in the Emo-test HIT Confirm® assay, 2 gave ambiguous results, and 8 sera yielded false-negative results. Accordingly, the HIT Confirm® assay showed a sensitivity of 69.7% with a slightly better specificity of 75.4% compared to the EIA (sensitivity 100%, specificity 63.3%). An increase in diagnostic specificity for HIT to 85% was found when positive results were obtained in both the Emo-test HIT Confirm® assay and EIA. CONCLUSION: The Emo-Test HIT Confirm® assay may improve the specificity of laboratory investigations of HIT. However, the assay can only be recommended in combination with an immunoassay due to the high rate of false negativity. Our observation indicates a need to establish external quality assessment for functional assays to avoid such clinically relevant pitfalls.


Assuntos
Anticoagulantes/efeitos adversos , Heparina/efeitos adversos , Trombocitopenia/induzido quimicamente , Trombocitopenia/diagnóstico , Idoso , Reações Falso-Negativas , Feminino , Citometria de Fluxo/métodos , Humanos , Técnicas Imunoenzimáticas , Masculino , Pessoa de Meia-Idade , Agregação Plaquetária/efeitos dos fármacos , Plasma Rico em Plaquetas/efeitos dos fármacos
18.
Eur J Haematol ; 103(3): 225-233, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31206215

RESUMO

OBJECTIVES: Reliable diagnosis of heparin-induced thrombocytopenia and thrombosis (HIT) is mandatory for patient management, yet prompt determination of pathogenic antibodies remains an unmet clinical challenge. Common immunoassays carry inherent limitations and functional assays which detect antibody-mediated platelet activation are not usually readily available to routine laboratories, especially the serotonin release assay (SRA), being technically demanding, time consuming, and requires high level expertise. To overcome some of these limitations, we have developed a practical functional flow cytometric assay (FCA) for routine clinical use. METHODS: A simple FCA is described which avoids platelet manipulation, is highly specific and sensitive compared with SRA, and provides rapid results. RESULTS: Of the 650 consecutive samples, from HIT-suspected patients, 99 (15.3%) were positive by the PaGIA Heparin/PF4 immunoassay and 31 (4.8%) by FCA. Average platelet activation was 11-fold higher in PaGIA+/FCA+ vs PaGIA-/FCA- samples. Of 21 SRA-positive samples, 19 were FCA-positive (relative sensitivity 90.5%), and of 42 SRA-negative samples, 40 were FCA-negative (relative specificity 95.2%). The FCA showed significantly higher correlation with the clinical presentation of HIT (4Ts score) performed on 182 patients, compared with PaGIA Heparin/PF4 (ROC-plot analysis, AUC 0.93 vs 0.63, P < 0.001). At a 92% sensitivity, the assay specificity was 96%. CONCLUSIONS: The present FCA is practical for routine testing, providing prompt reliable results for initial diagnosis and confirmation, to effectively assist in HIT patient management.


Assuntos
Plaquetas/metabolismo , Citometria de Fluxo , Heparina/efeitos adversos , Trombocitopenia/diagnóstico , Trombocitopenia/etiologia , Trombose/diagnóstico , Trombose/etiologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Autoanticorpos/imunologia , Gerenciamento Clínico , Feminino , Citometria de Fluxo/métodos , Humanos , Imunoensaio/métodos , Masculino , Pessoa de Meia-Idade , Ativação Plaquetária , Contagem de Plaquetas , Curva ROC , Avaliação de Sintomas , Trombocitopenia/sangue , Trombose/sangue , Adulto Jovem
19.
Zhonghua Er Ke Za Zhi ; 57(6): 429-433, 2019 Jun 02.
Artigo em Chinês | MEDLINE | ID: mdl-31216799

RESUMO

Objective: To explore the clinical value of genetic screening for early identification of WAS gene-related disorders in newborns. Methods: This was a retrospective study. Neonatal Genome Project from Children's Hospital of Fudan University collected 5 800 high-risk newborns in the neonatal intensive care unit to study the patients' genetic causes using high-throughput sequencing from January 2016 to December 2017. Eleven newborns (all were boys) with pathogenic or likely pathogenic variants in WAS gene were enrolled. Data of clinical characteristics,gene variants and genotype-phenotype correlation were collected and summarized. Results: Eleven patients included 5 cases with Wiskott-Aldrich syndrome (WAS) and 6 cases with X-linked thrombocytopenia (XLT).Two patients with WAS developed clinical manifestations in the early neonatal period,and 3 patients in 5-8 weeks after birth. Three neonates with XLT were hospitalized for other diseases in the first place.Their platelet count was found to be reduced after admission to hospital, and diagnosis was made after genetic testing. Eleven pathogenic or likely pathogenic variants in WAS gene were identified. Among them, 7 were first reported in this study, including 2 frame shift variants c.138delG and c.388_390del, 4 splicing variants c.1453+1G>A,c.734+1G>C,c.135G>A and c.1453+3G>C, and 1 missense variant c.1118C>T. The other 4 reported variants were c.777+1G>A,c.107_108delTT, c.436delC and c.1509_*3delAGTG. Conclusions: The clinical features of WAS gene-related disorders in neonatal period lack specificity. Genetic screening in newborns plays an important role in the early diagnosis of diseases and provides providing evidence for the early intervention.


Assuntos
Doenças Genéticas Ligadas ao Cromossomo X , Testes Genéticos/métodos , Trombocitopenia/diagnóstico , Proteína da Síndrome de Wiskott-Aldrich/genética , Síndrome de Wiskott-Aldrich/diagnóstico , Criança , Análise Mutacional de DNA , Diagnóstico Precoce , Humanos , Recém-Nascido , Masculino , Mutação , Estudos Retrospectivos , Trombocitopenia/genética , Síndrome de Wiskott-Aldrich/genética
20.
Semin Thromb Hemost ; 45(4): 348-353, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-31041804

RESUMO

Thrombotic microangiopathy (TMA) is a rare but often devastating complication of cancer and cancer treatment. The syndrome is defined by thrombocytopenia (i.e., a platelet count of < 150,000/mcL or > 30% decrease from baseline), microangiopathic hemolytic anemia, and some evidence of organ damage. Among the nine recognized groups of disorders causing TMA, the focus of this article will be on cancer and cancer treatment-related causes of TMA. This review will discuss the pathophysiology of TMA in cancer, chemotherapy-associated TMA, transplant-associated TMA, and newer therapeutic modalities.


Assuntos
Antineoplásicos/efeitos adversos , Transplante de Células-Tronco Hematopoéticas/efeitos adversos , Neoplasias/terapia , Microangiopatias Trombóticas/diagnóstico , Anticorpos Monoclonais Humanizados/uso terapêutico , Antineoplásicos/uso terapêutico , Complemento C5a/antagonistas & inibidores , Complemento C5a/metabolismo , Inativadores do Complemento/uso terapêutico , Transplante de Células-Tronco Hematopoéticas/métodos , Humanos , Neoplasias/complicações , Contagem de Plaquetas , Trombocitopenia/diagnóstico , Trombocitopenia/tratamento farmacológico , Trombocitopenia/etiologia , Microangiopatias Trombóticas/tratamento farmacológico , Microangiopatias Trombóticas/etiologia
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