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3.
Platelets ; 31(1): 62-67, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-30759044

RESUMO

Plasma thrombopoietin (Tpo) levels distinguish thrombocytopenia resulting from increased platelet destruction or decreased platelet production. We investigated whether measuring plasma Tpo levels in thrombocytopenic newborns is of diagnostic value to establish the underlying mechanism of thrombocytopenia.Tpo levels were measured with in-house developed ELISA in samples referred to our center because of thrombocytopenia noticed in the first 10 days of life. Clinical data were collected.Plasma Tpo levels <128 AU/ml were found in the majority (92%) of 121 newborns with immune-mediated thrombocytopenia (n = 104) and thrombocytopenia due to bacterial infections (n = 7); increased plasma Tpo levels (≥128 AU/ml) were found in thrombocytopenic newborns with severe asphyxia (n = 24). Highly increased plasma Tpo levels (>200 AU/ml) were found in thrombocytopenic neonates with congenital viral infections (n = 22) or amegakaryocytosis (n = 6). A plasma Tpo level <128 AU/ml excludes (negative predictive value 96%, 95% CI 90-99) severe asphyxia, congenital viral infections and amegakaryocytosis as the cause for thrombocytopenia in newborns.Increased plasma Tpo levels indicate that thrombocytopenia in newborns, as a result of various nonimmune disorders, is often caused by (temporary) bone marrow suppression/failure. Measurement of plasma Tpo levels provides the clinician with an additional tool to decide on the differential diagnosis, the necessity for subsequent diagnostics and treatment in neonates with thrombocytopenia.


Assuntos
Trombocitopenia/sangue , Trombocitopenia/diagnóstico , Trombopoetina/sangue , Biomarcadores , Diagnóstico Diferencial , Gerenciamento Clínico , Humanos , Imunoglobulinas Intravenosas/uso terapêutico , Recém-Nascido , Isoanticorpos/imunologia , Contagem de Leucócitos , Contagem de Plaquetas , Trombocitopenia/etiologia , Trombocitopenia/terapia
4.
Anticancer Res ; 39(12): 6895-6901, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31810959

RESUMO

BACKGROUND: Lenvatinib, a newly developed oral multi-tyrosine kinase inhibitor, has amazing potential in the multidisciplinary treatment of advanced or metastatic hepatocellular carcinoma. Thrombocytopenia is a serious adverse event that causes drug dose reduction or withdrawal. Partial splenic embolization is currently being used as a non-surgical treatment for thrombocytopenia caused by various pharmacotherapies. CASE REPORT: Partial splenic embolization was performed for three patients with hepatocellular carcinoma receiving lenvatinib therapy with/without transarterial chemoembolization. Partial splenic embolization was advantageous for various situations, including the induction of lenvatinib for patients with thrombocytopenia, application of lenvatinib after multiple transarterial chemoembolization using cisplatin and radiotherapy, and re-administration of lenvatinib after lenvatinib therapy-induced thrombocytopenia. In all cases, lenvatinib therapy was completed without need for cessation due to thrombocytopenia. CONCLUSION: We strongly recommend the new concept of combining partial splenic embolization and lenvatinib therapy for hepatocellular carcinoma.


Assuntos
Carcinoma Hepatocelular/tratamento farmacológico , Neoplasias Hepáticas/tratamento farmacológico , Compostos de Fenilureia/efeitos adversos , Quinolinas/efeitos adversos , Trombocitopenia/terapia , Idoso , Embolização Terapêutica , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Baço , Trombocitopenia/induzido quimicamente , Resultado do Tratamento
5.
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
6.
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.
BMJ Case Rep ; 12(9)2019 Sep 16.
Artigo em Inglês | MEDLINE | ID: mdl-31527216

RESUMO

A middle-aged woman with history of antiphospholipid syndrome, admitted to our hospital for lethargy and persistent chest pain, developed during hospitalisation intracerebral transverse sinus and sigmoid vein thromboses, retinal thromboses, acute renal failure and cardiac involvement associated with thrombocytopaenia. Diagnosis of catastrophic antiphospholipid syndrome was made and treated with IV steroids, heparin infusion and double filtration plasmapheresis (DFPP) without fresh frozen plasma. Heparin-induced thrombocytopaenia was second diagnosed because of persistent severe thrombocytopaenia after 2 weeks of treatment associated with positive conversion of antibodies against platelet factor 4, and a positive functional assay. The clinical course was complicated by cerebellar haematomas that appeared a few days after the last session of DFPP. Heparin-induced thrombocytopaenia was managed by administration of argatroban, a direct thrombin inhibitor with an increase in platelet count. Neurologically, the patient recovered completely and was discharged on vitamin K antagonist treatment and regular dialysis.


Assuntos
Síndrome Antifosfolipídica/complicações , Síndrome Antifosfolipídica/terapia , Heparina/efeitos adversos , Trombocitopenia/induzido quimicamente , Trombocitopenia/terapia , Anticoagulantes/uso terapêutico , Doença Catastrófica , Dor no Peito , Terapia Combinada , Feminino , Glucocorticoides/uso terapêutico , Humanos , Pessoa de Meia-Idade , Plasmaferese
8.
Rev. esp. anestesiol. reanim ; 66(7): 385-389, ago.-sept. 2019. tab
Artigo em Espanhol | IBECS | ID: ibc-187552

RESUMO

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


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


Assuntos
Humanos , Feminino , Gravidez , Adulto , Trombocitopenia/complicações , Anestésicos/administração & dosagem , Hemorragia Pós-Parto/etiologia , Lesão Renal Aguda/etiologia , Microangiopatias Trombóticas/diagnóstico , Corioamnionite/diagnóstico , Complicações Hematológicas na Gravidez/diagnóstico , Trombocitopenia/terapia , Cuidados Críticos/métodos , Diagnóstico Diferencial , Febre/etiologia , Complicações do Trabalho de Parto/diagnóstico
9.
Transfus Apher Sci ; 58(4): 505-507, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-31387833

RESUMO

Lung transplantation surgery often relies on the use of intraoperative extracorporeal membrane oxygenation (ECMO) and necessitates the need for high dose anticoagulation. Heparin induced thrombocytopenia complicates intraoperative anticoagulation management during lung transplant surgery requiring ECMO. Though other anticoagulants such as argatroban and bivalrudin are utilized for the treatment of Heparin Induced Thrombocytopenia (HIT), the lack of reversal agents makes it difficult to use these agents intraoperatively in cases with high bleeding risk. This is especially true in patients with end stage fibrotic lung disease with calcified mediastinal lymphadenopathy and pulmonary hypertension undergoing lung transplantation. Here we describe a case of HIT in a patient with Sarcoidosis listed for lung transplant who was treated with Therapeutic Plasma Exchange and Intravenous Immune globulin preoperatively and successfully underwent lung transplantation with the use of intraoperative venoarterial ECMO and heparin anticoagulation.


Assuntos
Heparina/efeitos adversos , Imunoglobulinas Intravenosas/administração & dosagem , Transplante de Pulmão , Troca Plasmática , Cuidados Pré-Operatórios , Trombocitopenia , Heparina/administração & dosagem , Humanos , Masculino , Pessoa de Meia-Idade , Sarcoidose Pulmonar/sangue , Sarcoidose Pulmonar/terapia , Trombocitopenia/sangue , Trombocitopenia/induzido quimicamente , Trombocitopenia/terapia
10.
Einstein (Sao Paulo) ; 17(4): eAO4720, 2019 Aug 19.
Artigo em Inglês, Português | MEDLINE | ID: mdl-31433009

RESUMO

OBJECTIVE: To verify the adequacy of platelet concentrate prescription by pediatricians in different pediatric sectors of a general hospital. METHODS: A cross-sectional study evaluating 218/227 platelet concentrate records in children and adolescents (zero to 13 years old), from January 2007 to April 2015, by the pediatricians of the emergency room, sick bay and intensive care unit. The requisitions were excluded in patients with hematological diseases and those without the number of platelets. RESULTS: Children under 12 months received 98 platelet concentrates (45.2%). Most of the transfusions were prophylactic (165; 79%). Regarding the transfusion site, 39 (18%) were in the emergency room, 27 (12.4%) in the sick bay and 151 (69.6%) in the intensive care unit. The trigger, prescribed volume and platelet concentrate subtype were adequate in 59 (28.2%), 116 (53.5%) and 209 (96.3%) of the transfusions, respectively. Patients with hemorrhage presented adequacy in 42 (95.5%), while children without bleeding presented in 17 (10.3%). The most common inadequacy related to volume was the prescription above recommendation (95; 43.8%). Eight platelet concentrates were prescribed with subtype requests without indication. CONCLUSION: The results obtained in this study showed that transfusion of platelet concentrate occurred more adequately in children with active bleeding compared to prophylactic transfusion. There was a tendency to prescribe high volumes and platelet subtypes not justified according to current protocols. The teaching of transfusion medicine should be more valued at undergraduate and medical residency.


Assuntos
Transfusão de Plaquetas/estatística & dados numéricos , Prescrições/normas , Trombocitopenia/terapia , Adolescente , Criança , Pré-Escolar , Estudos Transversais , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Centros de Atenção Terciária , Trombocitopenia/prevenção & controle
11.
Cancer Treat Res ; 179: 139-150, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31317485

RESUMO

Chemotherapy-induced thrombocytopenia (CIT) is a frequent complication of cancer therapy, leading to increased risk of bleeding, when the thrombocytopenia is severe (<10,000/mcL). However, the major clinical relevance of CIT is the subsequent delay or dose reduction in chemotherapy. CIT, therefore, leads to reduced relative dose intensity (RDI) of cancer therapy. Reduced RDI has been shown in several studies to impact progression-free survival and other cancer outcomes. While there are a number of factors leading to reduced RDI, CIT is a common cause. We review the causes and clinical manifestations of CIT, the current recommendations for management, and the status of research to develop targeted therapies to treat CIT.


Assuntos
Antineoplásicos/efeitos adversos , Neoplasias/tratamento farmacológico , Trombocitopenia/terapia , Antineoplásicos/uso terapêutico , Humanos , Neoplasias/complicações , Estudos Retrospectivos , Trombocitopenia/induzido quimicamente , Trombocitopenia/etiologia
12.
Intern Med ; 58(21): 3153-3156, 2019 Nov 01.
Artigo em Inglês | MEDLINE | ID: mdl-31292386

RESUMO

A 53-year-old woman presented at our hospital due to nasal bleeding and petechiae with profound thrombocytopenia (0.4×109/L). Her platelet count returned to the normal range immediately following a platelet transfusion. In this case, tea brewed from Taxus yunnanensis was the only suspected agent ingested prior to the onset of thrombocytopenia while all other etiologies for thrombocytopenia were excluded. A re-exposure test to Taxus yunnanensis resulted in the recurrence of acute thrombocytopenia. The association of thrombocytopenia with substances other than drugs has so far only been rarely described and to the best of our knowledge, this is the first reported case of thrombocytopenia caused by Taxus yunnanensis.


Assuntos
Bebidas/efeitos adversos , Extratos Vegetais/efeitos adversos , Taxus/efeitos adversos , Trombocitopenia/etiologia , Feminino , Humanos , Pessoa de Meia-Idade , Contagem de Plaquetas , Transfusão de Plaquetas , Trombocitopenia/terapia
13.
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
14.
Transfus Apher Sci ; 58(4): 525-528, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-31327731

RESUMO

We present important laboratory testing and clinical management strategies used to safely discharge home a 69-year old woman with heparin-induced thrombocytopenia (HIT) from the hospital. She was admitted for a coronary artery bypass graft procedure for which she was anticoagulated with heparin. Shortly after the procedure she developed thrombocytopenia and was diagnosed with HIT using the 4Ts scoring system, a latex-enhanced immunoassay (LEI) screen and confirmatory serotonin release assay. Her anticoagulation was switched from heparin to argatroban, and response to treatment was monitored in the laboratory using LEI. Unfortunately, she also received platelet transfusions and subsequently developed multifocal deep vein thrombosis with worsening platelet counts with nadir less than 10 x 10^3/µL. After five therapeutic plasma exchange procedures we noted an improvement in platelet counts, which plateaued into the 50s x 10^3/µL. Furthermore, the LEI remained positive. At this juncture we decided to transition from argatroban to fondaparinux so that she could leave the hospital in stable condition. Upon follow-up with hematology she exhibited no worsening clinical signs or symptoms of disease, and platelet counts markedly improved to within normal limits of detection. In this report we examine the utility of LEI in monitoring patients with HIT, therapeutic plasma exchange in the management of severe HIT (with thrombosis), and the use of subcutaneous fondaparinux in managing HIT in the outpatient setting.


Assuntos
Heparina/efeitos adversos , Trombocitopenia , Idoso , Ponte de Artéria Coronária , Feminino , Fondaparinux/administração & dosagem , Heparina/administração & dosagem , Humanos , Ácidos Pipecólicos/administração & dosagem , Contagem de Plaquetas , Transfusão de Plaquetas , Trombocitopenia/sangue , Trombocitopenia/induzido quimicamente , Trombocitopenia/terapia , Trombose Venosa/sangue , Trombose Venosa/induzido quimicamente , Trombose Venosa/terapia
15.
Am J Case Rep ; 20: 1075-1079, 2019 Jul 23.
Artigo em Inglês | MEDLINE | ID: mdl-31332158

RESUMO

BACKGROUND Platelet transfusion is a common clinical practice required for therapeutic purposes in the setting of symptomatic thrombocytopenia, and, in some cases, prophylactically for asymptomatic thrombocytopenia. Crossmatch compatibility is not routinely done for platelet transfusions, and transfusion of ABO non-identical platelets has been adapted as an acceptable clinical practice. Acute intravascular hemolysis due to ABO non-identical platelets is a rare but clinically significant entity. Our case report reinforces the importance of a vigilant clinical approach in case of ABO non-identical platelet transfusions. CASE REPORT We report the case of 61-year-old woman with blood group A, with chemotherapy-induced asymptomatic thrombocytopenia, who developed acute intravascular hemolysis following transfusion of group O single-donor platelets (SDPs). The patient was transfused 1 unit of single-donor platelets for bleeding prophylaxis, as her platelet count dropped to less than 10×109/L due to chemotherapy that she was receiving for acute myeloid leukemia (AML). Immediately after transfusion, the patient noticed cherry-colored urine; and within 12 h of transfusion, her hemoglobin dropped by more than 2.5 g/dL. A post-transfusion immunohematology work-up showed positive DAT and high titers of anti-A1 isohemagglutinins in the platelet donor, supporting the diagnosis of acute intravascular hemolysis due to ABO non-identical platelets. CONCLUSIONS The possibility of acute intravascular hemolysis should be kept in mind in cases of transfusion of group O single donor platelets to non-group O recipients. ABO non-identical platelets, even with low isohemagglutinin titers, can cause significant adverse effects, particularly in newborns, children, and immunosuppressed and transfusion-dependent patients; therefore, a cautious clinical approach is recommended.


Assuntos
Sistema ABO de Grupos Sanguíneos , Incompatibilidade de Grupos Sanguíneos , Hemólise , Transfusão de Plaquetas , Feminino , Humanos , Hospedeiro Imunocomprometido , Pessoa de Meia-Idade , Trombocitopenia/terapia
16.
Blood ; 134(8): 663-667, 2019 08 22.
Artigo em Inglês | MEDLINE | ID: mdl-31248877

RESUMO

Germ line mutations in ETV6 are responsible for a familial thrombocytopenia and leukemia predisposition syndrome. Thrombocytopenia is almost completely penetrant and is usually mild. Leukemia is reported in ∼30% of carriers and is most often B-cell acute lymphoblastic leukemia. The mechanisms by which ETV6 dysfunction promotes thrombocytopenia and leukemia remain unclear. Care for individuals with ETV6-related thrombocytopenia and leukemia predisposition includes genetic counseling, treatment or prevention of excessive bleeding and surveillance for the development of hematologic malignancy.


Assuntos
Mutação em Linhagem Germinativa , Leucemia/genética , Proteínas Proto-Oncogênicas c-ets/genética , Proteínas Repressoras/genética , Trombocitopenia/genética , Medula Óssea/metabolismo , Medula Óssea/patologia , Gerenciamento Clínico , Predisposição Genética para Doença , Hemorragia/etiologia , Hemorragia/prevenção & controle , Humanos , Leucemia/complicações , Leucemia/patologia , Leucemia/terapia , Proteínas Proto-Oncogênicas c-ets/análise , Proteínas Repressoras/análise , Trombocitopenia/complicações , Trombocitopenia/patologia , Trombocitopenia/terapia
17.
BMJ Case Rep ; 12(6)2019 Jun 21.
Artigo em Inglês | MEDLINE | ID: mdl-31229978

RESUMO

A rare case of acquired amegakaryocytic thrombocytopenia (AATP) in a 35-year-old woman who presented with anaemia and thrombocytopenia at 22 weeks gestation. The first diagnostic impression was of an evolving aplastic anaemia; however, the patient was simultaneously diagnosed with severe vitamin B12 deficiency in the setting of vegetarianism. Once the cyanocobalamin deficiency was corrected, a repeat bone marrow biopsy revealed an isolated depletion of megakaryocytes, which suggested the diagnosis of AATP. Supportive care was provided for her anaemia and thrombocytopenia and she delivered a healthy baby girl with a normal platelet count. The patient was subsequently started on romiplostim with steady improvement in her platelet counts. This rare AATP case presentation highlights the importance of a well-structured diagnostic approach to thrombocytopenia during pregnancy and supports the successful use of thrombopoietin agonists for the management of AATP.


Assuntos
Doenças da Medula Óssea/complicações , Complicações Hematológicas na Gravidez/fisiopatologia , Púrpura Trombocitopênica/complicações , Receptores Fc/uso terapêutico , Proteínas Recombinantes de Fusão/uso terapêutico , Trombocitopenia/etiologia , Trombopoetina/uso terapêutico , Adulto , Doenças da Medula Óssea/fisiopatologia , Doenças da Medula Óssea/terapia , Cesárea , Feminino , Humanos , Contagem de Plaquetas , Gravidez , Complicações Hematológicas na Gravidez/terapia , Púrpura Trombocitopênica/fisiopatologia , Púrpura Trombocitopênica/terapia , Trombocitopenia/fisiopatologia , Trombocitopenia/terapia , Resultado do Tratamento
18.
Int J Lab Hematol ; 41 Suppl 1: 131-141, 2019 May.
Artigo em Inglês | MEDLINE | ID: mdl-31069978

RESUMO

Advances in molecular genetic sequencing techniques have contributed to the elucidation of previously unknown germline mutations responsible for inherited thrombocytopenia (IT). Regardless of age of presentation and severity of symptoms related to thrombocytopenia and/or platelet dysfunction, a subset of patients with IT are at increased risk of developing myeloid neoplasms during their life time, particularly those with germline autosomal dominant mutations in RUNX1, ANKRD26, and ETV6. Patients may present with isolated thrombocytopenia and megakaryocytic dysmorphia or atypia on baseline bone marrow evaluation, without constituting myelodysplasia (MDS). Bone marrow features may overlap with idiopathic thrombocytopenic purpura (ITP) or sporadic MDS leading to misdiagnosis. Progression to myelodysplastic syndrome/ acute myeloid leukemia (MDS/AML) may be accompanied by progressive bi- or pancytopenia, multilineage dysplasia, increased blasts, cytogenetic abnormalities, acquisition of bi-allelic mutations in the underlying gene with germline mutation, or additional somatic mutations in genes associated with myeloid malignancy. A subset of patients may present with MDS/AML at a young age, underscoring the growing concern for evaluating young patients with MDS/AML for germline mutations predisposing to myeloid neoplasm. Early recognition of germline mutation and predisposition to myeloid malignancy permits appropriate treatment, adequate monitoring for disease progression, proper donor selection for hematopoietic stem cell transplantation, as well as genetic counseling of the affected patients and their family members. Herein, we describe the clinical and diagnostic features of IT with germline mutations predisposing to myeloid neoplasms focusing on mutations involving RUNX1, ANKRD26, and ETV6.


Assuntos
Doenças Genéticas Inatas , Predisposição Genética para Doença , Mutação em Linhagem Germinativa , Leucemia Mieloide Aguda , Síndromes Mielodisplásicas , Proteínas de Neoplasias , Trombocitopenia , Aloenxertos , Genes Dominantes , Aconselhamento Genético , Doenças Genéticas Inatas/sangue , Doenças Genéticas Inatas/diagnóstico , Doenças Genéticas Inatas/genética , Doenças Genéticas Inatas/terapia , Neoplasias Hematológicas/sangue , Neoplasias Hematológicas/diagnóstico , Neoplasias Hematológicas/genética , Neoplasias Hematológicas/terapia , Transplante de Células-Tronco Hematopoéticas , Leucemia Mieloide Aguda/sangue , Leucemia Mieloide Aguda/diagnóstico , Leucemia Mieloide Aguda/genética , Leucemia Mieloide Aguda/terapia , Mutação , Síndromes Mielodisplásicas/sangue , Síndromes Mielodisplásicas/diagnóstico , Síndromes Mielodisplásicas/genética , Síndromes Mielodisplásicas/terapia , Trombocitopenia/sangue , Trombocitopenia/diagnóstico , Trombocitopenia/genética , Trombocitopenia/terapia
19.
J Clin Apher ; 34(5): 545-554, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-31116461

RESUMO

INTRODUCTION: Anti-heparin/platelet factor 4 antibody immune complexes resulting from heparin-induced thrombocytopenia (HIT) are removed by therapeutic plasma exchange (TPE). We sought to define TPE in HIT practice patterns using an international survey. METHODS: A 31-item online survey was disseminated through the American Society for Apheresis. After institutional duplicate responses were eliminated, a descriptive analysis was performed. RESULTS: The survey was completed by 94 respondents from 78 institutions in 18 countries. Twenty-nine institutions (37%) used TPE for HIT (YES cohort) and 49 (63%) did not (NO cohort). Most NO respondents (65%) cited "no requests received" as the most common reason for not using TPE. Of the 29 YES respondents, 10 (34%) gave incomplete information and were excluded from the final analysis, leaving 19 responses. Of these, 18 (95%) treated ≤10 HIT patients over a 2-year period. The most common indications were cardiovascular surgery (CS; 63%) and HIT-associated thrombosis (HT; 26%). The typical plasma volume processed was 1.0 (63% CS and 58% HT). For CS, the typical replacement fluid was plasma (42%) and for HT, it was determined on an individual basis (32%). For CS, patients were treated with a set number of TPE procedures (37%) or laboratory/clinical response (37%). For HT, the number of TPE procedures typically depended on laboratory/clinical response (42%). CONCLUSION: In a minority of responding institutions, TPE is most commonly used in HIT to prophylactically treat patients who will undergo heparin re-exposure during CS. Prospective studies are needed to more clearly define the role of TPE in HIT.


Assuntos
Troca Plasmática/métodos , Guias de Prática Clínica como Assunto , Trombocitopenia/terapia , Procedimentos Cirúrgicos Cardiovasculares/métodos , Gerenciamento Clínico , Heparina/uso terapêutico , Humanos , Pré-Medicação , Inquéritos e Questionários , Trombocitopenia/induzido quimicamente
20.
Chin Med Sci J ; 34(1): 60-64, 2019 Mar 30.
Artigo em Inglês | MEDLINE | ID: mdl-30961783

RESUMO

Heparin-induced thrombocytopenia (HIT) is a relatively infrequent complication of heparin administration. HIT can cause devastating thrombosis, making it one of the most serious adverse drug reactions encountered in clinical practice. We successfully treated a case of severe HIT presenting with thrombosis and life-threatening bleeding complications with intravenous immunoglobulin (IVIG), platelet transfusion and oral anticoagulant Rivaroxaban. In this case, we considered that IVIG played the most important role by preventing further thrombosis, increasing the platelet count, and ensuring the efficacy of Rivaroxaban. We therefore suggest that IVIG might be the optimal treatment for patients with this urgent condition.


Assuntos
Heparina/efeitos adversos , Imunoglobulinas Intravenosas/administração & dosagem , Transfusão de Plaquetas , Rivaroxabana/administração & dosagem , Trombocitopenia/induzido quimicamente , Trombocitopenia/terapia , Idoso de 80 Anos ou mais , Feminino , Heparina/administração & dosagem , Humanos
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