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1.
J Assoc Physicians India ; 68(3): 77-79, 2020 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-32138492

RESUMO

Immune thrombocytopenia is a well-known complication of both HIV and Hepatitis C virus infections. Management becomes challenging when a patient with HCV-HIV co-infection presents with severe thrombocytopenia. Adverse drug reactions and drug interactions has to be considered while choosing treatment options for such patients. We report such a case which illustrates the difficulty in managing severe thrombocytopenia in HCV-HIV co-infected patients where evidence based clinical decision making helped in choosing the right therapy for the patient.


Assuntos
Antivirais , Coinfecção , Infecções por HIV , Hepatite C , Hepacivirus , Humanos , Púrpura Trombocitopênica Idiopática
3.
N Engl J Med ; 382(11): 1077-1078, 2020 03 12.
Artigo em Inglês | MEDLINE | ID: mdl-32160681
4.
Medicine (Baltimore) ; 99(7): e19096, 2020 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-32049816

RESUMO

INTRODUCTION: The assessment of bone marrow thrombopoietic activity in patients with thrombocytopenia is necessary to achieve an accurate diagnosis and administer effective treatment. We evaluated the discriminatory power of the immature platelet fraction (IPF) in differentiating hyperdestructive/consumptive thrombocytopenia from hypoproductive thrombocytopenia and its potential use as a predictive marker for platelet recovery. METHODS: In this observational study, platelet indices, including IPF, were measured in 105 healthy individuals, 27 patients with hyperdestructive/consumptive thrombocytopenia (all with immune thrombocytopenic purpura [ITP]), and 35 patients with hypoproductive thrombocytopenia (5 with aplastic anemia and 30 with cancer who were undergoing chemotherapy) using a Sysmex XN-3000 hematology analyzer. RESULTS: The platelet distribution width, mean platelet volume, platelet large cell ratio, IPF, and absolute immature platelet count (AIPC) were significantly higher in the hyperdestructive/consumptive thrombocytopenia group than in the hypoproductive thrombocytopenia group (P < .001). The IPF showed the highest difference between the two patient groups (200%). Receiver operating characteristics analysis that showed the IPF had the largest area under the curve among all the platelet indices analyzed; its cut-off value was 2.3%. The IPF decreased 3 to 4 days in advance of platelet count elevation in patients with ITP, whereas the delta AIPC increased 3 days in advance. Furthermore, the IPF and delta AIPC increased 5.5 days and 8.5 days, respectively, before platelet counts increased up to 130.0 × 10/L in cancer patients receiving chemotherapy. CONCLUSION: These data demonstrated that the IPF and delta AIPC are both excellent indicators of the etiology of thrombocytopenia and predictive markers for platelet recovery.


Assuntos
Anemia Aplástica/diagnóstico , Volume Plaquetário Médio , Contagem de Plaquetas , Púrpura Trombocitopênica Idiopática/diagnóstico , Adulto , Idoso , Anemia Aplástica/sangue , Anemia Aplástica/complicações , Biomarcadores/sangue , Estudos de Casos e Controles , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Neoplasias/complicações , Valor Preditivo dos Testes , Púrpura Trombocitopênica Idiopática/sangue , Curva ROC
5.
Gene ; 736: 144419, 2020 Apr 30.
Artigo em Inglês | MEDLINE | ID: mdl-32018016

RESUMO

OBJECTIVES: To evaluate the relationship between two common single nucleotide polymorphisms (SNPs) of CD40 gene (rs1883832 C/T and rs4810485 G/T) and the risk of immune thrombocytopenia (ITP) in the Egyptian population. METHODS: A case-control study was conducted retrospectively on 101 cases with ITP and 97 healthy subjects. Two SNPs of CD40 gene (rs1883832 C/T and rs4810485 G/T) were genotyped via Taqman allele discrimination real-time PCR. The frequencies of different genetic models of both SNPs were calculated and compared between ITP cases and controls. Linkage analysis was performed between the studied SNPs. Odds ratio (OR) and 95% confidence interval (CI) were assessed using multinomial logistic regression analysis to determine the association of CD40 gene SNPs genotypes, alleles, and haplotypes with the risk of ITP. The odds ratio was further adjusted to the confounders for risk stratification. RESULTS: CD40 (rs1883832) TT genotype carriers have a significantly higher risk of ITP when compared to CC genotype carriers (adjusted OR: 3.792, 95%CI: 1.252-11.49, P = 0.018). T allele also represents 1.711-fold increased risk of ITP which is more evident in males (P = 0.016). No significant difference was observed in the frequency of CD40 (rs4810485 G/T) genetic models between cases and controls. Linkage disequilibrium was found between the two SNPs and revealed four main haplotypes (C-G; C-T; T-G; T-T) with a significantly higher frequency of T-G haplotype in ITP cases than in healthy controls which confers an increased risk of ITP development (OR: 2.349, 95%CI: 1.271-4.339, P = 0.006). CONCLUSIONS: CD40 gene SNP rs1883832 is associated with an increased risk of ITP development in the Egyptian population, while the SNP rs4810485 has no association. Moreover, T-G haplotype is a risk genetic model for ITP.


Assuntos
Antígenos CD40/genética , Polimorfismo de Nucleotídeo Único/genética , Púrpura Trombocitopênica Idiopática/genética , Adulto , Alelos , Estudos de Casos e Controles , Egito , Feminino , Frequência do Gene/genética , Predisposição Genética para Doença/genética , Genótipo , Haplótipos/genética , Humanos , Desequilíbrio de Ligação/genética , Masculino , Estudos Retrospectivos
6.
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
7.
Medicine (Baltimore) ; 99(1): e18624, 2020 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-31895820

RESUMO

The purpose of this study was to evaluate neutropenia following intravenous immunoglobulin (IVIG) therapy in adults with immune thrombocytopenic purpura (ITP).Our analysis included 88 patients with ITP, who received IVIG from January 2006 to March 2016, at Pusan National University Hospital in Korea. Their white blood cell (WBC) count and absolute neutrophil count (ANC) before and after IVIG treatment were analyzed.Of 88 patients, 24 patients (27.3%) were male, and 64 patients (72.7%) were female. Neutropenia developed in 8 patients (18.7%) after IVIG treatment. In patients with a decrease in WBC count and ANC compared to baseline, median WBC count decreased from 6280/µL to 4530/µL after IVIG therapy, and median ANC decreased from 3840/µL to 2840/µL after IVIG therapy. The neutropenia induced by IVIG had resolved spontaneously after several days, and the mean recovery time was 8.72 days after the completion of the IVIG treatment. During the neutropenic episodes, only one patient developed neutropenic fever, which subsided soon without any treatment.The results of this study suggest that IVIG may cause neutropenia commonly in adults with ITP, and it seems to be transient and self-limited. This study is meaningful as the first report that not only pediatric ITP patients may develop neutropenia post IVIG administration, but also adult patients suffering ITP.


Assuntos
Imunoglobulinas Intravenosas/efeitos adversos , Neutropenia/etiologia , Púrpura Trombocitopênica Idiopática/terapia , Adolescente , Adulto , Idoso , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Púrpura Trombocitopênica Idiopática/sangue , Púrpura Trombocitopênica Idiopática/imunologia , Estudos Retrospectivos , Adulto Jovem
8.
Lakartidningen ; 1172020 01 23.
Artigo em Sueco | MEDLINE | ID: mdl-31990364

RESUMO

A program for care of women with immune thrombocytopenic purpura (ITP) with the recommendation to avoid treatment if platelets were >20 × 109/l during pregnancy, with the target level 100 × 109/l at delivery, was introduced. Treatment should be given with intravenous immunoglobulin (IVIG) or corticosteroids. Out of 75 pregnancies with ITP, 39 percent were treated and the treatment period was shorter with IVIG. Blood loss at delivery was similar as the reference population. Epidural analgesia was given in only 17 percent of the vaginal deliveries. Twenty-three percent of the infants had platelet counts less than 50 × 109/l during the first days after birth. If the women had prior neonatal trombocytopenia 63 percent got a child with thrombocytopenia and 40 percent of those with platelets <20 × 109/l during pregnancy had a child with thrombocytopenia. Multidisciplinary care of pregnant women with ITP including obstetricians, hematologists and neonatologists is recommended.


Assuntos
Complicações Hematológicas na Gravidez , Púrpura Trombocitopênica Idiopática , Criança , Feminino , Humanos , Imunoglobulinas Intravenosas , Contagem de Plaquetas , Gravidez , Complicações Hematológicas na Gravidez/terapia , Púrpura Trombocitopênica Idiopática/terapia
9.
Einstein (Sao Paulo) ; 18: eRC5111, 2020.
Artigo em Inglês, Português | MEDLINE | ID: mdl-31939527

RESUMO

Situs inversus totalis is a rare recessive autosomal congenital abnormality in which the mediastinal and abdominal organs are in a mirrored position when compared to the usual topography. The literature reports some cases of situs inversus totalis and concomitant conditions: spinal abnormalities, cardiac malformations and hematological diseases, such as idiopathic thrombocytopenic purpura, which is an autoimmune disease that causes thrombocytopenia due to platelet destruction or suppression of its production. This article aimed to report the coexistence of situs inversus totalis and idiopathic thrombocytopenic purpura.


Assuntos
Púrpura Trombocitopênica Idiopática/complicações , Situs Inversus/complicações , Situs Inversus/diagnóstico por imagem , Humanos , Masculino , Radiografia Panorâmica , Situs Inversus/patologia , Tomografia Computadorizada por Raios X , Adulto Jovem
12.
J Surg Res ; 245: 643-648, 2020 01.
Artigo em Inglês | MEDLINE | ID: mdl-31536907

RESUMO

BACKGROUND: As medical therapy improves, splenectomy has been relegated to third- or fourth-line therapy for immune thrombocytopenic purpura (ITP) in many hematologic practices. However, these medications have well-known associated morbidity and changes in treatment algorithms may affect the timing and degree of response to splenectomy as well as complications in heavily treated ITP patients. MATERIALS AND METHODS: This is a retrospective study of consecutive patients who underwent ITP splenectomy from January 1994 to June 2017. Nonresponders after splenectomy and those with recurrent disease were compared to complete responders. RESULTS: The cohort included 84 patients. Median number of medications received before splenectomy was 3 (1-6). 14.3% of patients had a medication-related complication, including heart failure, adrenal insufficiency, diabetes mellitus, infection, and osteoporosis. After splenectomy, 83.5% had a complete response, 7.5% partial response, and 9% no response. Complete response was associated with response to steroids before surgery (P < 0.01). Among responders, 19% had recurrent disease, which was associated with lower platelet count at diagnosis (P < 0.01). Forty-four patients (52.0%) had nonelective splenectomies for persistent bleeding or dangerously low platelets despite maximal medical therapy. Ten patients had Clavien-Dindo grade II or higher surgical complications (11.9%). Seven of these complications were related to recurrent or refractory ITP. CONCLUSIONS: Many ITP patients have complications related to medication use, and 52.0% required nonelective splenectomy despite maximal medical therapy. Earlier splenectomy may avoid medication-related complications and may reduce the complications from splenectomy. Splenectomy remains an effective and safe treatment for ITP.


Assuntos
Púrpura Trombocitopênica Idiopática/cirurgia , Esplenectomia/métodos , Corticosteroides/uso terapêutico , Adulto , Idoso , Feminino , Humanos , Laparoscopia , Masculino , Pessoa de Meia-Idade , Púrpura Trombocitopênica Idiopática/tratamento farmacológico , Estudos Retrospectivos , Esplenectomia/efeitos adversos
13.
Ann Hematol ; 99(1): 49-55, 2020 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-31853702

RESUMO

Immune thrombocytopenia (ITP) has been reported to be associated with thrombotic events. The incidence of thrombosis in 303 newly diagnosed ITP patients in our institute between 2000 and 2016 was retrospectively reviewed. During a median follow-up of 3.6 years, 16 thrombotic events (12 arterial and four venous) occurred. The median platelet count at thrombotic events was 102 × 109/l. At 10 years, the cumulative thrombosis incidence was 10%. A univariate analysis showed that smoking, hypertension, male gender, a history of thrombosis, and atrial fibrillation (Af) were significantly associated with the occurrence of thrombosis, and a multivariate analysis identified smoking and Af as independent risk factors. The thrombotic risk was not increased by lupus anticoagulant positivity or ITP treatment. At 5 years, the cumulative incidence of bleeding and overall survival probability was 5.6% and 92%, respectively. This study demonstrates that smoking and Af were associated with an increased risk of thrombosis. Previously identified risk factors were not confirmed in these Japanese ITP patients.


Assuntos
Púrpura Trombocitopênica Idiopática , Trombose , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Intervalo Livre de Doença , Feminino , Seguimentos , Humanos , Incidência , Masculino , Pessoa de Meia-Idade , Púrpura Trombocitopênica Idiopática/complicações , Púrpura Trombocitopênica Idiopática/mortalidade , Estudos Retrospectivos , Taxa de Sobrevida , Trombose/etiologia , Trombose/mortalidade
14.
Zhongguo Shi Yan Xue Ye Xue Za Zhi ; 27(6): 1949-1954, 2019 Dec.
Artigo em Chinês | MEDLINE | ID: mdl-31839065

RESUMO

OBJECTIVE: To investigate the changes of mean platelet volume (MPV), platelet distribution width (PDW) and platelet associated antibodies (PAIg) in children with acute immune thrombocytopenic purpura (aITP), and to explore the diagnostic value of MPV, PDW, PAIg and their combination for megakaryocyte dysmaturity in aITP children. METHODS: Plt count, MPV and PDW of 36 aITP children were measured by using Sysmex XN automatic blood cell analyzer, and 33 children with acquired thrombocytopenic purpura (ATP) without megakaryocyte dysmaturity. The expression of PAIg was detected by flow cytometry, and the number and classification of megakaryocytes in the bone marrow were performed by marrow cytology. The diagnostic significances of MPV, PDW, PAIg and their combination as well as the sensitivity and specificity for megakaryocytes dysmaturity in aITP were assessed through calculating the area under ROC curve (AUC), after determining the influence of each parameters on the megakaryocyte dysmaturity by Logistic regression. RESULTS: MPV, PDW and PAIg of aITP children were significantly higher than those of the ATP children (P<0.05), while the Plt count and number of thromocytogenic megakaryocytes per area (1.5 cm×3 cm) were less than those of the controls (P<0.05). Count of RBC and WBC, percentages of neutrophil granulocytes and lymphocydes in aITP were similar to those in the controls(P>0.05). The results of Logistic regression showed that Plt count, MPV, PDW and PAIg were the factors influencing megakaryocyte dysmaturity in aITP children, and the regression model has a high statistical significance (χ2=65.491,P=0.001) and r square (R2=0.713). The AUC of the combined detection of Plt count, MPV, PDW and PAIg was 0.863, which was much higher than that of Plt count, MPV, PDW, PAIg individually or in pairs. The sensitivity and specificity of the combined detection were 79.167% and 89.697%, which were higher than those of Plt count, MPV, PDW, PAIg individually or in pairs. CONCLUSION: The diagnostic significance of MPV and PDW for megakaryocyte dysmaturity in aITP are insufficient, but the diagnostic efficacy can be improved by combined examination with PAIg.


Assuntos
Volume Plaquetário Médio , Púrpura Trombocitopênica Idiopática , Anticorpos , Plaquetas , Criança , Humanos , Megacariócitos , Contagem de Plaquetas , Púrpura Trombocitopênica Idiopática/diagnóstico
15.
Zhongguo Shi Yan Xue Ye Xue Za Zhi ; 27(6): 1955-1961, 2019 Dec.
Artigo em Chinês | MEDLINE | ID: mdl-31839066

RESUMO

OBJECTIVE: To establish a flow cytometric immunobead array assay (FCIA) to quantify platelet antibodies and to explore its application in the diagnosis and treatment of ITP. METHODS: The guantitative standard curve was established by binding the human IgG of known concentration on antibody-coated microbeads; at the same time, the platelet-specific antigen and antibody complex was captured and levels of platelet antibodies were detected using the microbeads coated by 5 kinds of antibodies against platelets suca as GPIX (SZ1), GPⅠb (SZ2), GpⅢa (SZ21), GPⅡb (SZ22) and p-selection (SZ51). The fluorescence signal detected by flow cytometry were transformed into the conentration of platelet antibodies in samples through the quantitative standard curve, thereby establishing the method for quantititive detection of platelet-specific antibodies in plasm samples (FCIA), moreover the property, efficiency and clinical application of establishod FCIA method were evaluated. RESULTS: The FCIA could detect 5 kinds of antibodies against GPIX, GPⅠb, GpⅢa, GPⅡb and ß-selection within a broad range of 33.29-1280 ng/ml, 45.17-1280 ng/ml, 42.07-1280 ng/ml, 46.40-1280 ng/ml, 42.48-1280 ng/ml and 42.48-1280 ng/ml respectively, and their recovery rates were 115.23%, 112.58%, 117.47%, 107.64% and 112.67% respectively. The intra-assay coefficient of variation (CV) for anti- GPIX, -GPⅠb, -GpⅢa, -GPⅡb and p-selection antibodies was 3.54%, 3.63%, 4.66%, 6.43% and 6.67% respectively, and the inter-assay CV for above mentioned antibodies were 10.89%, 7.57%, 10.34%, 6.95% and 10.72% respectively. The detection showed that the levels of 5 kinds of platelet-specific antibodies in ITP group all were higher than those in non-ITP and healthy control groups (P<0.01). The sensitivity, specificity and accuracy of quantitatively detecting 5 kinds of antibodies for diagnosis of ITP by FCIA were 68.29%, 84.98% and 78.95% respectively, while the sensitivity, specificity and accuracy of detecting 5 kinds of antibodies by modified indirect MAIPA were 41.46%, 90.41% and 72.81% respectively. CONCLUSION: The established quantitative FCIA for detection of antibodies provides a powerful tool for diaghosis and evaluation of therapeutic efficacy and prognosis of ITP patients.


Assuntos
Plaquetas , Púrpura Trombocitopênica Idiopática , Anticorpos , Autoanticorpos , Citometria de Fluxo , Humanos
16.
Zhongguo Shi Yan Xue Ye Xue Za Zhi ; 27(6): 1962-1965, 2019 Dec.
Artigo em Chinês | MEDLINE | ID: mdl-31839067

RESUMO

OBJECTIVE: To study the changes of Th1 and Th2 type cytokines and B lymphocyte level and their clinical significance in idiopathic thrombocytopenic purpura (ITP) patients treated by recombinant human thrombopoietin (rhTPO). METHODS: The peripheral blood levels of Th1 and Th2 type of cytokines and B lymphocyte were estimated by CBA in 48 patients with ITP, and compared with those in 35 control persons of heath examination. RESULTS: Before treatment, the levels of Th1 type cytokines and B lymphocyte in 48 patients with ITP were higher, and the levels of Th2 type cytokines were lower than those of healthy controls (P<0.05). The levels of the peripheral blood CD19+ cells, CD5+CD19+ cells, IL-2 expression negatively correlated with Plt counts in ITP patients (P<0.05), the levels of IL-4 positively correlated with Plt counts (P<0.05). After treatment with rhTPO, the levels of Th1 type cytokines and B lymphocytes in 48 patients with ITP significantly decreased, and the levels of Th2 type cytokines significantly increased in comparison with those before treatment (P<0.05). CONCLUSION: Peripheral blood Th1 and Th2 type cytokines express abnormally and level of B lymphocytes increases significantly in ITP patinets. The disease severity correlats with the levels of Th1 and Th2 type cytokines and B lymphocytes. Platelets increase after rhTPO treatment, showing that rhTPO can play an important role in regulating Th1 and Th2 immunologic balance and B lymphocyte level in ITP patients.


Assuntos
Púrpura Trombocitopênica Idiopática , Linfócitos B , Humanos , Células Th1 , Células Th2 , Trombopoetina
17.
Arch. argent. pediatr ; 117(6): S243-S254, dic. 2019. Tab, graf
Artigo em Espanhol | LILACS, BINACIS | ID: biblio-1051620

RESUMO

El manejo de la trombocitopenia inmune es motivo de discusión en lo concerniente a evolución,diagnóstico, pronóstico y tratamiento. Se han publicado arias guías que expresan distintas opiniones de expertos, pero no existe aún consenso mundial sobre cuál es el manejo más adecuado de la enfermedad. Esta guía establece los criterios para definir el diagnóstico; detalla el plan de estudios de laboratorio por realizar inicialmente; plantea los distintos diagnósticos diferenciales; desarrolla aspectos relativos a evolución y pronóstico, y enumera los tratamientos disponibles para las formas agudas y las crónicas, así como para el manejo de las emergencias y en algunas situaciones especiales.


Management, outcome, diagnosis, prognosis and treatment of immune thrombocytopenia are controversial. Several guidelines stating different experts' opinions have been published; however, no worldwide consensus regarding the management of the disease has still been reached. This guideline defines diagnostic criteria, states initial laboratory tests, establishes differential diagnosis, develops topics concerning outcome and prognosis, and enumerates available treatments for acute and chronic disease, as well as for management of life-threatening bleeding.


Assuntos
Humanos , Púrpura Trombocitopênica Idiopática/diagnóstico , Púrpura Trombocitopênica Idiopática/terapia , Pediatria
19.
Mayo Clin Proc ; 94(11): 2199-2208, 2019 11.
Artigo em Inglês | MEDLINE | ID: mdl-31685150

RESUMO

OBJECTIVE: To evaluate the impact of the sequence of treatment with rituximab and/or splenectomy on time to relapse for patients with steroid-refractory immune thrombocytopenia (ITP). PATIENTS AND METHODS: Patients 18 years or older with steroid-refractory immune thrombocytopenia who underwent treatment with splenectomy or rituximab from January 1, 2002, through December 31, 2015, at Mayo Clinic. Evaluation included freedom from relapse (FFR) and response rates after treatment with rituximab or splenectomy as single or sequential interventions. RESULTS: A total of 218 eligible patients with ITP who were treated according to standard of care were included in this analysis. Patients failing steroids treated with splenectomy had a higher 5-year FFR than did those treated with rituximab (67.4% vs 19.2%; P<.001, propensity-score matched). Patients who failed splenectomy and were then treated with rituximab had a 2-year FFR similar to that of patients who failed rituximab and were then treated with splenectomy (73.4% vs 59.9%; P=.52). Patients treated with rituximab after splenectomy had a longer 2-year FFR than did patients treated with rituximab as a second-line treatment (73.4% vs 29.0%; P<.001). CONCLUSION: For patients with ITP that relapse after treatment with steroids, splenectomy provides longer FFR than rituximab as a second-line therapy. Among patients who fail second-line treatment with splenectomy or rituximab, those who end up receiving sequential splenectomy-rituximab or rituximab-splenectomy therapy seem to derive similar benefit in the long term. Patients who received rituximab after splenectomy seem to derive superior benefit than do those who are treated with rituximab with an intact spleen.


Assuntos
Imunossupressores/uso terapêutico , Púrpura Trombocitopênica Idiopática/terapia , Rituximab/uso terapêutico , Esplenectomia/métodos , Esteroides/uso terapêutico , Adulto , Terapia Combinada , Feminino , Humanos , Pessoa de Meia-Idade , Púrpura Trombocitopênica Idiopática/fisiopatologia , Análise de Sobrevida , Resultado do Tratamento
20.
Medicine (Baltimore) ; 98(44): e17766, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31689837

RESUMO

RATIONALE: Although essential thrombocythemia (ET) and immune thrombocytopenia (ITP) have different etiologies, 3 previous reports have described ET development in ITP patients, all of whom were positive for the JAK2 V617F mutation. Here, we report the first published case of ITP following ET in the absence of other platelet disorders. PATIENT CONCERNS: A 70-year-old woman with a five-year history of ET with JAK2 V617F mutation treated with hydroxycarbamide for five months presented with petechiae on her limbs. DIAGNOSIS: Her platelet count was 3 × 10/L, with the immature platelet fraction being 29%. White blood cell count and hemoglobin level were normal. Bone marrow examination showed increased number of megakaryocytes, but no morphologic dysplasia in any lineage. G-band analysis revealed no abnormalities. Platelet transfusion and cessation of hydroxycarbamide did not affect the platelet count. Thrombocytopenia was unlikely to have been induced by drugs, heparin, systemic lupus erythematosus, or human immunodeficiency virus. Hence, a diagnosis of ITP was made. INTERVENTIONS: The patient received oral prednisolone combined with intravenous immunoglobulin. OUTCOMES: Her platelet count rose to 310 × 10/L and remained stable, while her steroid dose was reduced. Further blood tests revealed the presence of antibodies against Helicobacter pylori, and appropriate treatment was administered. Resumption of hydroxycarbamide did not induce thrombocytopenia. LESSONS: Although ET and ITP have different etiologies, chronic inflammation and immune deregulation underlie both and may play an important role in the progression from one to the other. Further research is warranted to understand the relationship between ET and ITP.


Assuntos
Janus Quinase 2/sangue , Púrpura Trombocitopênica Idiopática/genética , Trombocitemia Essencial/genética , Idoso , Plaquetas , Feminino , Humanos , Contagem de Leucócitos , Mutação , Contagem de Plaquetas , Púrpura Trombocitopênica Idiopática/etiologia , Trombocitemia Essencial/sangue , Trombocitemia Essencial/complicações
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