Maintenance of efficacy and its predictors after discontinuation of eltrombopag in adults with primary immune thrombocytopenia / 中华血液学杂志

Objective: To determine the efficacy of eltrombopag for primary immune thrombocytopenia (ITP) in adults and the predictive factors for treatment-free response (TFR) . Methods: Clinical data of adults with ITP who received eltrombopag from June 14, 2013 to May 31, 2021 in the Hematology Department of Ruijin Hospital affiliated with Shanghai Jiao Tong University Medical College were retrospectively analyzed. The initial dose of eltrombopag was 25 mg/d, and the maximum dose was 75 mg/d; the dose was adjusted to maintain the platelet count to within 50-150×10(9)/L. Treatment was discontinued according either to the protocol, on the patient's wishes or doctor's judgment (prescription medication), or based on clinical trials. The efficacy of eltrombopag and factors for TFR among patients who achieved complete response and those who discontinued treatment were analyzed. Results: Overall, 106 patients with ITP (33 men and 73 women) were included in the study. The median age of patients was 50 (18-89) years. There were 2, 10, and 94 cases of newly diagnosed, persistent, and chronic ITP, respectively. The complete response rate was 44.3% (47/106), the response rate was 34.0% (36/106), and the overall response rate was 78.3% (83/106). Meanwhile, 83 patients who responded to treatment discontinued eltrombopag; of these, 81 patients were evaluated. Additionally, 17 patients (21.0%) achieved TFR. The median follow-up duration of patients who achieved TFR was 126 (30-170) weeks. The recurrence rate was 17.6% (3/17), and the relapse-free survival rate was 76.5%. The results of univariate analysis revealed that non-recurrence after discontinuation of other treatments for ITP (P=0.001), and platelet count and eltrombopag dose of ≥100×10(9)/L (P=0.007) and ≤25 mg/d (P=0.031), respectively, upon discontinuation of eltrombopag were predictors of TFR; these effects were attributed to prolonged effective duration of eltrombopag. Multivariate analysis showed that there was a correlation between non-recurrence and prolonged effective duration after discontinuation of other treatments for ITP (P=0.002) . Conclusion: Eltrombopag is effective for patients with ITP as it can result in TFR. Predictors for TFR include non-recurrence after discontinuation of concomitant ITP treatment, and platelet count and eltrombopag dose of ≥100 × 10(9)/L and ≤25 mg/d upon discontinuation of treatment, respectively.

Immune thrombocytopenic purpura following CoronaVac vaccination: a case report

Immune thrombocytopenic purpura (ITP), an autoimmune disorder, has been documented as a result of SARS-CoV-2 infection and a vaccination side effect. The COVID-19 pandemic has led to the creation of CoronaVac vaccine and has been widely administered in Brazil.Patient, in the case, is an 82-years-old female who received the vaccine two days before an acute episode of gingivorrhagia and diffuse cutaneous petechiae. Other exams were made to look for other causes of secondary thrombocytopenia and all the results were normal. The patient showed improvement on the platelet levels three day after the beginning of the treatment with high dosage methylprednisolone.Knowing that other kinds of vaccine can generate ITP, the SARS-CoV-2 vaccine could be related to the symptoms.

Diagnoses, outcomes, and chronicity predictors of patients with secondary immune thrombocytopenia: ten-year data from a hematology referral center

ABSTRACT Background: Secondary immune thrombocytopenia (ITP) is a heterogeneous and unpredictable disease associated with various underlying conditions. Objective: The objective of the study was to investigate clinical evolution and chronicity predictors in secondary ITP. Methods: Patients treated at an academic medical center during 2008-2019 were stratified by age as children <16 years and adults >16 years. Responses to steroids, intravenous immunoglobulin G (IVIG), rituximab, and eltrombopag were classified as response (R) and complete response (CR). Risk factors for chronic ITP were determined by multiple regression with uni- and multi-variate analysis. Results: Eighty-three patients were included, 37 children and 46 adults. The most frequent associated conditions were infections 53%, systemic lupus erythematosus (SLE) 24%, thyroid disease 9.6%, and Evans syndrome 3.6%. Response to first-line treatment in the whole cohort was 94%; CR 45.7%; and R 50.6%. Initial response to steroids alone was 91.3% (n = 21/23), rituximab plus high-dose dexamethasone (HDD) 93.3% (n = 14/15); children receiving IVIG alone 100% (n=12/12); and eltrombopag in adults 100% (n = 3/3). Relapse was documented in 19.4% of children and 34% of adults, at a median time of 15 and 2 months, respectively; 30.4% of adults (15.2% from the miscellaneous group, 10.9% SLE-associated, and 4.3% infection-associated) and 18.9% of children followed a chronic course; age ≥10 years and platelets ≥20 × 109/L were risk factors for chronic ITP in children. Conclusion: Evolution was heterogeneous: a better and more sustained response was documented in the infections group compared to SLE or the miscellaneous group. (REV INVEST CLIN. 2021;73(1):31-8)

Cambio de diagnóstico y de categoría diagnóstica en pacientes con artritis idiopática juvenil en 7 años de seguimiento / Change of diagnosis and diagnostic category in patients with juvenile idiopathic arthritis within 7 years of follow-up

Resumen: Introducción: Al menos 50% de los pacientes pediátricos portadores de artritis idiopática juvenil (AIJ) continuará control en reumatología adulto. La clasificación de la Liga Internacional de Asociaciones de Reumatología (ILAR) vigente, actualmente en revisión, difiere de la clasificación de las artritis inflamatorias del adulto. Se ha reportado cambios de categoría en 10,8% de los pacientes durante el seguimiento. Objetivo: Analizar los pacientes con AIJ seguidos al menos 7 años para objetivar cambios de diagnós tico en la transición, e identificar factores de mal pronóstico funcional. Pacientes y Método: Estudio retrospectivo en base a registros clínicos. Se incluyó a la totalidad de los pacientes con AIJ controla dos en policlínico pediátrico del Hospital de Puerto Montt entre el año 2005 y 2017, que cumplieron siete o más años de seguimiento. Se realizó análisis descriptivo en base a variables clínicas: categoría diagnóstica, tiempo de evolución al diagnóstico, actividad clínica y serológica, y tiempo de evolución al inicio de la terapia farmacológica. Resultados: Se evaluaron 18 pacientes, 3 Oligo-articular (OA) persistente, 1 OA extendida, 4 Poli-articular (PA) factor reumatoide (FR) negativo, 4 PA FR positivo, 5 Sistémicas, 1 Psoriática, todos con seguimiento mayor a 7 años. Once de 18 niños fueron transfe ridos a adultos. Tres de 11 cambiaron de diagnóstico a Artritis Reumatoide (AR) más otra enferme dad autoinmune: Síndrome de Sjögren + Lupus eritematoso sistémico, Púrpura trombocitopénico inmune, Enfermedad autoinmune no clasificada y cinco de 11 niños de categoría ILAR: OA a Artritis reumatoide juvenil, OA extendida a PA FR negativo, 3 Sistémicas a PA FR negativo. Edad de inicio, formas poli-articulares, retrasos en diagnóstico y comienzo de terapia se asociaron a secuelas e infla mación persistente. Conclusiones: Ocho de once pacientes transferidos cambiaron denominación diagnóstica y/o presentaron otras enfermedades autoinmunes. Algunos factores de mal pronóstico deben mejorar.

Abstract: Introduction: At least 50% of pediatric patients with Juvenile Idiopathic Arthritis (JIA) will require continued fo llow-up in adult rheumatology. The present International League of Associations for Rheumatology (ILAR) classification, currently under revision, differs from its classification of inflammatory arthritis in adults. Category changes have been reported in 10.8% of patients during follow-up. Objective: To analyze JIA patients in follow-up for at least 7 years to detect diagnosis changes during transition to adult care, identifying factors of poor functional prognosis. Patients and Method: Retrospective study based on medical records of JIA patients seen at the pediatric polyclinic of the Puerto Montt Hospital between 2005 and 2017, who were monitored for at least 7 years. Descriptive analysis was performed according to clinical variables: diagnostic category, evolution before diagnosis, clinical and serological activity, and evolution before starting drug therapy. Results: We evaluated 18 pa tients, corresponding to 3 patients with persistent oligoarticular arthritis (OA), 1 with extended OA, 4 with polyarticular arthritis (PA) rheumatoid factor (RF) negative, 4 with PA RF positive, 5 with syste mic JIA, and 1 with psoriatic arthritis, all have had follow-up more than 7 years. 11 out of 18 patients transitioned to adult care. Three out of 11 patients changed diagnosis to Rheumatoid Arthritis (RA) plus another autoimmune disease such as Sjögren's Syndrome + Systemic Lupus Erythematosus, Immune thrombocytopenia, or unclassified autoimmune disease, and 5 out of 11 children changed ILAR category from OA to Juvenile Rheumatoid Arthritis, extended OA to PA RF negative, and 3 from Systemic arthritis to PA RF negative. Age of onset, polyarticular forms, delay in diagnosis, and the start of therapy were associated with sequelae and persistent inflammation. Conclusions: Eight of the eleven JIA patients who transitioned to adult care changed their diagnosis or presented other autoimmune diseases. Some factors of poor prognosis must improve.

Trombocitopenia inmune. Guía de diagnóstico y tratamiento / Guideline for diagnosis and treatment of immune thrombocytopenia

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.

High Remission Rate of Chronic Immune Thrombocytopenia in Children: Result of 20-Year Follow-Up

PURPOSE: This study examined the outcomes of children with chronic immune thrombocytopenia (ITP). MATERIALS AND METHODS: We retrospectively analyzed the medical records of all patients diagnosed with ITP from January 1992 to December 2011 at our institution. RESULTS: A total of 128 patients (64%) satisfied the criteria for newly diagnosed ITP, 31 (15%) for persistent ITP, and 41 (21%) for chronic ITP. The median age at diagnosis was 4.5 years (range, 1 month to 18 years). The median platelet count at diagnosis was 32x109/L. A comparison of the initial treatment data from 2001 to 2011 with those from 1992 to 2000 showed that the number of bone marrow examinations decreased, whereas observation increased. Chronic ITP presented at an older age than newly diagnosed and persistent ITP (6.6 years vs. 3.8 years vs. 4.1 years, respectively); however, the difference did not reach statistical significance (p=0.17). The probability of complete remission of chronic ITP was 50% and 76% at 2 and 5 years after diagnosis, respectively. Patients aged <1 year at diagnosis had a significantly better prognosis than did older patients (hazard ratio, 3.86; p=0.02). CONCLUSION: Children with chronic ITP showed a high remission rate after long-term follow-up. This study suggests that invasive treatments such as splenectomy in children with chronic ITP can be delayed for 4 to 5 years if thrombocytopenia and therapeutic medication do not affect the quality of life.

Síndrome de aglutininas frías y púrpura trombocitopénica autoinmune: Un caso inusual de síndrome de Evans / Cold agglutinins syndrome and autoimmune thrombocytopenic purpura: An unusual case of Evans syndrome

El síndrome de Evans es un trastorno poco frecuente en el que se observan trombocitopenia y anemia, ambas de etiología autoinmune; las que pueden ocurrir de manera simultánea o sucesiva. Se presenta un caso poco usual de anemia hemolítica autoinmune por anticuerpos fríos asociada a púrpura trombocitopénica autoinmune. Paciente femenina de 22 años de edad con diagnóstico de púrpura trombocitopénica autoinmune, después de 7 años de evolución y un año en remisión, presentó una anemia hemolítica autoinmune por anticuerpos fríos, refractaria al tratamiento con esteroides y alcaloides de la Vinca, que requirió transfusiones de concentrado de eritrocitos y logró la remisión con la administración de anticuerpo monoclonal anti CD 20. Los restantes estudios de autoinmunidad fueron negativos. Actualmente se mantiene asintomática y sin tratamiento inmunosupresor(AU)

Evans syndrome is a rare disorder in which thrombocytopenia and anemia are observed, both of autoimmune aetiology, which may occur simultaneously or successively. A rare case of cold autoimmune hemolytic anemia associated to autoimmune thrombocytopenic purpura is presented. A 22-year-old female patient with diagnosis of autoimmune thrombocytopenic purpura, after 7 years of evolution and one year in remission, has a cold autoimmune hemolytic anemia, refractory to steroid treatment and vinca alkaloids, which requires transfusions of packed erythrocytes and achieves remission with anti CD 20 monoclonal antibody. The remaining studies of autoimmunity are negative. Currently the patient is asymptomatic and without immunosuppressive therapy(AU)

Place of Bone Marrow Biopsy as Compared to Bone Marrow Aspiration in Different Haematological Disorders.

Bone marrow aspiration (BMA) is much more frequently used than bone marrow biopsy (BMB) in the diagnosis of different haematological disorders. BMB is performed primarily in cases where bone marrow aspiration either fails or gives insufficient information. It is the sole modality of diagnosis in some situations and may give useful information of prognostic importance. The present study was done to find comparative usefulness of each of these procedures in various hematological disorders. Methods: Study subjects included all 45 patients attending the hematology section of pathology department of College of Medicine & Jawaharlal Nehru Memorial Hospital, Kalyani for bone marrow aspiration and who also consented for bone marrow biopsy during the period from 1st July 2011 to 30th June 2012. BMA smears and BMB specimens were obtained from posterior superior iliac spine in the same sitting and stained with Leishman’s stain and Haematoxylin & Eosin stain respectively. Findings of BMA and BMB were compared. Results: BMA was diagnostic in all cases of iron deficiency anemia (IDA), acute myeloid leukemia (AML), chronic myeloid leukemia (CML), chronic lymphocytic leukemia (CLL), idiopathic thrombocytopenic purpura (ITP) and essential thrombocythemia (ET). On the whole, BMA was diagnostic in 82.2% of cases and could accurately reveal hypercellularity in all cases. BMB was the only diagnostic method in myelofibrosis (MF) and provided additional information like fibrosis in cases of AML, CML and MF & pattern of involvement in CLL and multiple myeloma (MM). BMB, however, was poor in assessing iron stores due to leaching of iron during decalcification. Conclusions: BMA alone is sufficient for IDA and ITP. BMB is mandatory for diagnosis of MF and provides additional prognostic information in other cases.

A Case of Refractory Thrombocytopenia with 5q Deletion: Myelodysplastic Syndrome Mimicking Idiopathic Thrombocytopenic Purpura

No abstract available.

Características clínicas, epidemiológicas y de tratamiento de niños con púrpura trombocitopénica inmune, hospitalizados en el Instituto Nacional de Salud del Niño. Año 2011-2012 / Clinical characteristics, epidemiological and treatment of the children with immune thrombocytopenic purpura, hospitalized at the National Institute of Child Health. Year 2011-2012

O Describir las características clínicas, epidemiológicas y de tratamiento de niños con Púrpura Trombocitopénica Inmune, hospitalizados en el Instituto Nacional de Salud del Niño durante el periodo 2011-2012. Metodología: Estudio observacional, descriptivo, retrospectivo. Se realizó un muestreo no probabilístico por conveniencia, conformada por 54 niños hospitalizadas con el diagnóstico de Púrpura Trombocitopénica Inmune en Instituto Nacional de Salud del Niño durante el año 2011 a 2012. Para describir las variables cualitativas se usó frecuencias absolutas y porcentajes. Resultados: Del estudio entre las características clínicas el 55.6 por ciento de los pacientes presentó púrpura trombocitopénica inmune de tipo aguda, siendo el inicio de la enfermedad principalmente insidiosa (88.9 por ciento), de curso progresivo (81.5 por ciento), con menos de 10 días de duración (70.4 por ciento). El 27.8 por ciento de la aparición de la enfermedad se dio entre los meses de octubre a diciembre, el 64.8 por ciento tuvo enfermedades previas. Las manifestaciones clínicas más frecuentes fueron petequias de color rojo (85.2 por ciento), la equimosis superficial (61.1 por ciento), la epistaxis (53.7 por ciento) y la hemorragia nasal (51.9 por ciento). Más de la mitad de pacientes tuvieron 2 episodios de hemorragia. Respecto a las pruebas de diagnóstico, más de la tres cuartas partes presentó menos de 12 gr/dl de hemoglobina la cuarta parte de su tiempo de sangría fue menor a 5 minutos, 79.6 por ciento tuvo un tiempo de protombina de 13 seg. a más. Asimismo, al 72.2 por ciento de los pacientes su tiempo de tromboplastina fue entre 36 seg. a más, la cuarta parte presento un tiempo de coagulación menor a 7 min y el 51.9 por ciento de pacientes tuvieron valores de 200 a 400 mg/dl de fibrinógeno. Al 14.8 por ciento de pacientes que se le realizó la prueba de velocidad corpuscular media tuvo valores superiores a 25 mm/hora. Con respecto al recuento plaquetario...

To describe the clinical, epidemiological and treatment characteristics of children with immune thrombocytopenic purpura, hospitalized at the National Institute ofChild Health during the period 2011-2012. Methodology: Observational, descriptive, retrospective study. It was performed a non-probability convenience sample, consisting of 54 children hospitalized with the diagnosis of Immune Thrombocytopenic Purpura in National Institute of Child Health during 2011-2012. To describe qualitative variables, absolute frequencies and percentages were used. Results: Among clinical features, 55.6 per cent of patients had immune thrombocytopenic purpura type acute, being the beginning of the disease mainly insidious (88.9 per cent), progressive course (81.5 per cent), less than 10 days (70.4 per cent). The 27.8 per cent of the onset of illness occurred between the months of October to December, 64.8 per cent had previous illnesses. The most frequent clinical manifestations were red petechiae (85.2 per cent), superficial ecchymosis (61.1 per cent), epistaxis (53.7 per cent) and nasal hemorrhage (51.9 per cent). More than half had 2 episodes of bleeding. Regarding diagnostic tests, more than three quarters had less than 12 g/dl of hemoglobin, the fourth bleeding time was less than 5 minutes, and 79.6 per cent had a prothrombin time of 13 sec more. Also, 72.2 per cent of patients, thromboplastin time was between 36 sec more, a quarter presented a clotting time less than 7 min and 51.9 per cent of patients had values of 200 to 400 mg/dl of fibrinogen. In 14.8 per cent of patients whom performed the mean corpuscular velocity test had values above 25 mm/hour. Respect to platelet count, 44.5 per cent had between 10000 and 20000 platelets/mrn3. Among the treatments received, 1.8 per cent had expectant treatment. Of those who received no expectant treatment, 1.9 per cent was applied anti-D immunoglobulin, 61.1 per cent and 73.6 per cent of patients were administered...

Octaploidy in idiopathic thrombocytopenic purpura.

We report a case of an elderly 68-year-old male who presented in our hospital with chief complaints of petechial rashes and ecchymosis over extremities and bleeding from the oral cavity since 3–4 days prior to hospitalization. He saw a physician before coming to our hospital and received one dose of IV methylprednisolone and oral wysolone. He had come to our hospital for further management. Bone marrow karyotyping was done and chromosomal analysis revealed two cell lines. Eighty percent of the cells analyzed revealed apparently normal male karyotype. However, 20% cells analyzed revealed a total of 184 chromosomes, suggesting octaploidy.

Púrpura trombocitopénico inmunológico (PTI): a propósito de un caso / Idiopathic thrombocytopenic purpura: a case report

Paciente de sexo masculino de 22 años que ingresa por cuadro de inicio agudo de hemorragia muco cutánea. Al ingreso se pesquisa severa trombocitopenia asociada a anemia normocítica. Se realiza estudio, concluyéndose diagnóstico de Purpura trombocitopénico inmunológico y anemia secundaria at sangrado concomitante. Se inicia tratamiento con corticoides 1 mg/kg/día, con mala respuesta a los 15 días de tratamiento, por lo que se realiza esplenectomía laparoscópica con respuesta favorable.

Spontaneous bilateral peripapillary, subhyaloid and vitreous hemorrhage with severe anemia secondary to idiopathic thrombocytopenic purpura.

A 42-year-old female presented to us with a complaint of sudden painless loss of vision in both eyes of three days duration. Visual acuity was 20/100 for distance in both eyes. Fundus examination showed bilateral peripapillary hemorrhages, with subhyaloid and vitreous hemorrhage in both eyes. Hematological investigations revealed hemoglobin (HB 7 gm %) and severe thrombocytopenia (12,000/ ul). She was referred to a hematologist where a diagnosis of idiopathic thrombocytopenic purpura (ITP) was made. She was treated for systemic condition with regular ophthalmic follow-up. Over the next nine months, retinal hemorrhages completely resolved and the patient regained her visual acuity. The purpose of this case report is to highlight the clinical presentation of severe anemia, which is different from previous reports and the role of an ophthalmologist in first detecting the Idiopathic thrombocytopenic purpura (ITP), which led to successful recovery.

Idiopathic thrombocytopenic purpura and oral surgery: case report / Púrpura trombocitopênica idiopática e cirurgia bucal: relato de caso

OBJECTIVES: To review the function of platelets in the blood clotting mechanism. To address,conceptualize and classify thrombocytopenic purpura, especially idiopathic thrombocytopenic purpura (ITP),emphasizing the immunological aspects involved in the etiology of the disease. To address the clinicalmanifestations of the disease and the appropriate therapy. To present the case study of a patient with ITPwho requires oral surgical intervention. DISCUSSION: Leukopenia in ITP can be subclinical. The firstmanifestation of the disease can be severe hemorrhaging due to small lacerations or minor medical anddental surgical procedures. The cause of the platelet reduction is idiopathic; an autoimmune reaction in whichthe antibodies destroy the platelets appears to participate in the process. A variety of situations can occur,leading to mild to severe thrombocytopenia. One frequent aspect is the instability of the platelet count,which oscillates inconsistently and may be related to infections and other factors that have not been clearlydetermined, including stress. In the case presented here, the leukopenia in the patient was mild in presentation.However, it was decided that the patient should be referred to a hematologist for preparation and clearance.Oral surgery that compromises the bone, such as exodontia, can present significant difficulties in localizingand clamping intraosseous vessels, which does not only occur in small soft tissue surgeries. CONCLUSIONS:Platelet destruction in ITP occurs from a complex process that is comprised of multiple components of theimmune system. The platelets are prematurely destroyed by antibodies that are aimed at the platelet glycoprotein,which can results in serious, even fatal consequences. It is important to emphasize the significance of themedical history and the appropriate physical examination during the diagnostic process, as well as collaborationwith the patient’s medical...

OBJETIVOS: Revisar a função das plaquetas no mecanismo de coagulação sanguínea. Conceituare classificar a púrpura trombocitopênica, especialmente a púrpura idiopática, enfatizando osaspectos imunológicos envolvidos na etiologia da doença. Enfocar as manifestações clínicas dadoença e a terapia apropriada. Apresentar um caso de manifestação clínica da doença e a terapiaapropriada. Apresentar o caso de uma paciente com púrpura que necessitava de intervençãocirúrgica bucal. DISCUSSÃO: A leucopenia na púrpura trombocitopênica idiopática pode sersubclínica. A primeira manifestação da doença pode ser uma hemorragia severa por causa depequenas lacerações ou pequenos procedimentos médicos e odontológicos. A causa da reduçãodas plaquetas é idiopática; uma reação autoimune na qual os anticorpos destroem as plaquetasparece participar do processo. Uma variedade de situações pode ocorrer, levando àtrombocitopenia discreta a severa. Um aspecto frequente é a instabilidade da contagem plaquetária,que oscila inconsistentemente e pode relacionar-se com infecções e outros fatores ainda nãoclaramente determinados, incluindo estresse. No presente caso, o paciente apresentava discretaleucopenia. Entretanto, decidiu-se que o paciente deveria ser avaliado por um hematologista,para preparação e liberação para cirurgia, pois a cirurgia bucal que compromete osso, como aexodontia, pode apresentar dificuldades significativas na localização e pinçamento de vasosintraósseos, o que não acontece em pequenas cirurgias de tecidos moles. CONCLUSÕES: Adestruição plaquetária na púrpura trombocitopênica ocorre por meio de um complexo processo,com múltipos componentes do sistema imune. As plaquetas são destruídas prematuramente poranticorpos dirigidos contra as glicoproteínas plaquetárias, o que pode resultar em sérias – emesmo fatais – consequências. É importante enfatizar o significado da história médica e o examefísico adequado no processo diagnóstico...

Púrpura trombocitopénica inmunitaria. Guía de diagnóstico y tratamiento / Guideline for diagnosis and treatment of immune thrombocytopenia

El manejo de la púrpura trombocitopénica inmunitariaes motivo de discusión en lo concerniente a evolución, diagnóstico, pronóstico y tratamiento.Se han publicado varias guías con 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 a realizar inicialmente, plantealos distintos diagnósticos diferenciales, desarrolla aspectos relativos a evolución y pronóstico, y enumeralos tratamientos disponibles para las formas agudas y las crónicas, así como para el manejo de las emergencias.

Púrpura trombocitopênica idiopática: importância do cirurgião-dentista no diagnóstico / Idiopathic thrombocytopenic purpura: importance of the dentist in this diagnosis

Esse trabalho descreve um caso de púrpura trombocitopênica idiopática (PTI) diagnosticado durante exame odontológico de rotina. A PTI é uma desordem caracterizada pela destruição anormal das plaquetas circulantes e geralmente apresenta manifestações bucais. Dessa forma, destacamos a importância de o cirurgião-dentista estar apto a reconhecer os sinais e sintomas da PTI, principalmente mudanças bucais que não têm origem dental, possibilitando tratamento imediato. Esta patologia quando não tratada, pode ser irreversível e fatal.

A Comparison of Fasting Glucose and HbA1c for the Diagnosis of Diabetes Mellitus Among Korean Adults / 예방의학회지

OBJECTIVES: The American Diabetes Association (ADA) has recently recommended the HbA1c assay as one of four options for making the diagnosis of diabetes mellitus, with a cut-point of > or =6.5%. We compared the HbA1c assay and the fasting plasma glucose level for making the diagnosis of diabetes among Korean adults. METHODS: We analyzed 8710 adults (age 45-74 years), who were not diagnosed as having diabetes mellitus, from the Namwon study population. A fasting plasma glucose level of > or =126 mg/dL and an A1c of > or =6.5% were used for the diagnosis of diabetes. The kappa index of agreement was calculated to measure the agreement between the diagnosis based on the fasting plasma glucose level and the HbA1c. RESULTS: The kappa index of agreement between the fasting plasma glucose level and HbA1c was 0.50. CONCLUSIONS: The agreement between the fasting plasma glucose and HbA1c for the diagnosis of diabetes was moderate for Korean adults.

Myelodysplastic Syndrome Mimicking Idiopathic Thrombocytopenic Purpura / 대한진단검사의학회지

BACKGROUND: In patients with isolated thrombocytopenia, but without significant dysplasia, diagnosis of idiopathic thrombocytopenic purpura (ITP) rather than myelodysplastic syndrome (MDS) may be taken into account. It is important to make an accurate diagnosis because different treatments are used for ITP and MDS. The purpose of this study was to investigate the clinical and hematologic features of patients who were initially diagnosed as ITP but had cytogenetic abnormalities. METHODS: We retrospectively reviewed cytogenetic studies of 100 patients who were diagnosed as ITP from 2004 to 2009 at Mokdong Hospital of Ewha Womans University based on clinical features and hematologic studies. Bone marrow pathology was re-evaluated based on 2008 WHO classification. Cytogenetic analysis was performed by 24-48 hr culture of bone marrow aspirates without using mitogens and 20 metaphases were analyzed. RESULTS: Of the 100 patients diagnosed as ITP initially, three patients (3%) had cytogenetic abnormalities. They had no thrombocytopenia-related symptoms and thrombocytopenia was found accidentally. The numbers of megakaryocytes in bone marrow were increased and dysplasia was not found in megakaryocyte, erythroid, and myeloid cell lineages. The proportion of blasts was within normal limits. Clonal chromosomal abnormalities found were der(1;7)(q10;p10), add(9)(q12), or t(7;11)(p22;q12). Presumptive diagnosis of MDS or diagnosis of idiopathic cytopenia of undetermined significance (ICUS) was made according to 2008 WHO classification. During the follow up, disease progression was not found. CONCLUSIONS: In patients with suspected ITP, cytogenetic analysis should be done. If specific clonal chromosomal abnormality is found, presumptive diagnosis of MDS has to be considered and close follow up is needed.