AL Amyloidosis with Polycythemia, Leucocytosis and Thrombocytosis : A Case Report

A young lady presented to us with clinical and biochemical evidence of Nephrotic Syndrome. Her laboratory investigations also revealed Erythrocytosis, Leucocytosis and Thrombocytosis. A renal biopsy revealed a diagnosis of Amyloidosis which was further characterized as AL amyloidosis with further investigations (kappa chain monoclonal gammopathy). She was started with appropriate therapy and she showed significant decline in her monoclonal Proteins on follow up. Her Erythrocytosis, Leucocytosis and Thrombocytosis also normalized with the decline in the levels of monoclonal light chains. We postulate a link between the monoclonal protein associated growth factors and inflammatory markers which were responsible for this unique association between AL Amyloidosis and tri-lineage hematopoietic cell proliferation.

Role and mechanism of platelet-derived growth factor BB in thrombocytosis in Kawasaki disease / 中国当代儿科杂志

OBJECTIVES@#To study the role and mechanism of platelet-derived growth factor BB (PDGF-BB) on platelet production in Kawasaki disease (KD) mice and human megakaryocytic Dami cells through in vitro and invivo experiments.@*METHODS@#ELISA was used to measure the expression of PDGF in the serum of 40 children with KD and 40 healthy children. C57BL/6 mice were used to establish a model of KD and were then randomly divided into a normal group, a KD group, and an imatinib group (30 mice in each group). Routine blood test was performed for each group, and the expression of PDGF-BB, megakaryocyte colony forming unit (CFU-MK), and the megakaryocyte marker CD41 were measured. CCK-8, flow cytometry, quantitative real-time PCR, and Western blot were used to analyze the role and mechanism of PDGF-BB in platelet production in Dami cells.@*RESULTS@#PDGF-BB was highly expressed in the serum of KD children (P<0.001). The KD group had a higher expression level of PDGF-BB in serum (P<0.05) and significant increases in the expression of CFU-MK and CD41 (P<0.001), and the imatinib group had significant reductions in the expression of CFU-MK and CD41 (P<0.001). In vitro experiments showed that PDGF-BB promoted Dami cell proliferation, platelet production, mRNA expression of PDGFR-β, and protein expression of p-Akt (P<0.05). Compared with the PDGF-BB group, the combination group (PDGF-BB 25 ng/mL + imatinib 20 μmol/L) had significantly lower levels of platelet production, mRNA expression of PDGFR-β, and protein expression of p-Akt (P<0.05).@*CONCLUSIONS@#PDGF-BB may promote megakaryocyte proliferation, differentiation, and platelet production by binding to PDGFR-β and activating the PI3K/Akt pathway, and the PDGFR-β inhibitor imatinib can reduce platelet production, which provides a new strategy for the treatment of thrombocytosis in KD.

Thrombocytosis in children

SUMMARY OBJECTIVE: We aimed to investigate the frequency and causes of thrombocytosis in patients admitted to the Department of Pediatric Hematology and Oncology of Elazig Fethi Sekin City Hospital, Elazig, Turkey. METHODS: Between 2019 and 2021, the laboratory parameters of 2,500 patients admitted to the Hematology Department were studied. During this examination, 319 (12.76%) patients were found to have thrombocytosis. Demographic characteristics (age and gender), hematologic parameters (hemoglobin, white blood cells, and platelets), and ultimate diagnoses ot the cases were recorded from their files. RESULTS: Of these 319 patients with thrombocytosis, 197 (1.8%) were male and 122 (38.2%) were female, and the mean age was 72.0±69.0 (1-216) months. The median platelet count of the patients was 590.43±280.12/μL (450,000-750,000). The most common cause of secondary thrombocytosis was infection, accounting for 37.9% of all patients. Other common causes were sickle cell anemia (21%), iron deficiency anemia (15.4%), colloid tissue disease (6.6%), hemolytic anemia (5.0%), splenectomy (4.5%), and other causes (9.7%). CONCLUSION: In our study, infections were the most common cause of thrombocytosis. In addition to infections, sickle cell anemia and iron deficiency anemia should also be considered in the differential diagnosis of thrombocytosis.

A rare case of thrombocytosis presentation in patient alcoholic liver disease with portal hypertension gastropathy: a stepwise evaluation

Thrombocytosis is rarely found in patient with chronic liver disease (CLD). The possibility of reactive thrombocytosis could be due to sustained process such as iron deficiency anemia (IDA) because of occult bleeding. Occult bleeding can happen in CLD patient because of portal hypertension gastropathy (PHG) as complication of portal hypertension. A carefully evaluation of anemia can lead to underlying cause of disease, even in limited of supportive evaluationand some other confounding presentation that is thrombocytosis.We report a case of 54 years-old male patient with severe anemia. He had same symptom previously and got transfusion. Peripheral blood smear showed microcytic hypochromic anemia, anisocytosis, and poikilocytosis even pencil cells (pencil cells or cigar cells) with thrombocytosis. No symptom of acute inflammation setting and no clear blood loss was founded. As patient admitted to smoking and heavy alcohol consumption in the past, Ultrasound was performed for screening of underlying disease that cause occult bleeding. Ultrasound of the liver showed generally increased echogenicity suggestive of liver cirrhosis, splenomegaly and minimal ascites. Thus, our patient clinically be suggestive of CLD with portal hypertension that cause PHG.

Asociación plaquetas-factor de crecimiento epidérmico en pacientes con cáncer pulmonar de células no pequeñas tratados con quimioterapia / Platelet-epidermal growth factor association in patients with non-small cell lung cancer treated with chemotherapy

RESUMEN Introducción: la concentración plasmática del factor de crecimiento epidérmico pudiera encontrarse alterada en pacientes con cáncer de pulmón de células no pequeñas y trombocitopenia/trombocitosis por quimioterapia. Objetivo: determinar la asociación existente entre la concentración plasmática plaquetaria y la concentración plasmática de factor de crecimiento epidérmico en pacientes con cáncer de pulmón de células no pequeñas tratados con quimioterapia, entre marzo de 2019 y febrero de 2020 en el Hospital Provincial Saturnino Lora. Métodos: se realizó un estudio observacional descriptivo transversal en el Hospital Provincial Saturnino Lora, provincia Santiago de Cuba, Cuba, entre marzo de 2019 y febrero de 2020. El universo estuvo constituido por 54 pacientes con diagnóstico de cáncer pulmonar de células no pequeñas tratados con quimioterapia. Por muestreo probabilístico aleatorio simple se seleccionó una muestra de 12 pacientes. Se midieron las variables: concentración plasmática plaquetaria (pre y post-quimioterapia), concentración plasmática de factor de crecimiento epidérmico (pre y post-quimioterapia), y modificación de la concentración plasmática de factor de crecimiento epidérmico (castración, no castración). Para el procesamiento de los datos se empleó el test estadístico T student y la correlación lineal de Pearson, así como la media y desviación estándar como medidas de resumen y dispersión, respectivamente. Resultados: entre las concentraciones plasmáticas plaquetaria y del factor de crecimiento epidérmico se halló una relación lineal de -0,37 previo a la quimioterapia y de -0,51, posterior a esta; no se encontraron diferencias estadísticamente significativas. Conclusiones: se concluye que la modificación de la concentración plasmática del factor de crecimiento epidérmico no guarda relación aparente con la modificación plasmática plaquetaria, con posible relación espuria, dada por la quimioterapia.

ABSTRACT Introduction: the plasma concentration of epidermal growth factor may be altered in patients with non-small cell lung cancer and thrombocytopenia/thrombocytosis due to chemotherapy. Objective: to determine the association between platelet plasma concentration and epidermal growth factor plasma concentration in patients with non-small cell lung cancer treated with chemotherapy between March 2019 and February 2020 at Saturnino Lora Provincial Hospital. Methods: a cross-sectional, descriptive and observational study was conducted at Saturnino Lora Provincial Hospital, Santiago de Cuba province, Cuba, between March 2019 and February 2020. The target group comprised 54 patients diagnosed with non-small cell lung cancer treated with chemotherapy. A sample of 12 patients was chosen by simple probability-random sampling: platelet plasma concentration (pre- and post-chemotherapy), epidermal growth factor plasma concentration (pre- and post-chemotherapy), and modification of epidermal growth factor plasma concentration (castration, non-castration) were measured. For data processing, the statistical T-student test and Pearson's linear correlation were applied, as well as the mean and standard deviation as summary and dispersion measures, respectively. Results: a linear relationship of -0.37 before chemotherapy and -0.51 after the chemotherapy was found between platelet and epidermal growth factor plasma concentrations; no statistically significant differences were found. Conclusions: it is concluded that the modification of the plasma concentration of epidermal growth factor has no apparent relationship with the platelet plasma modification, with possible spurious relationship, given by chemotherapy.

Analysis of risk factors for thrombocytosis after splenectomy in patients with traumatic splenic rupture and construction of nomogram model / 中国医师进修杂志

Objective:To construct a nomogram model that can be used to predict thrombocytosis after splenectomy in patients with traumatic splenic rupture.Methods:The clinical data of 54 patients with splenectomy in Lu′an People′s Hospital from November 2016 to November 2021 were retrospectively analyzed. Among them, postoperative thrombocytosis occurred in 33 cases (postoperative thrombocytosis group), and 21 cases had no significant changes in platelets (postoperative platelet normal group). The general clinical data were recorded; the platelet parameters 14 d after operation were measured, including platelet count, mean platelet volume (MPV), procalcitonin (PCT) and platelet distribution width (PDW). The receiver operating characteristic (ROC) curve was drawn to analyze the predictive value of platelet parameters for thrombocytosis after splenectomy in patients with traumatic splenic rupture. Multivariate Logistic regression analysis was used to analyze independent risk factors of thrombocytosis after splenectomy in patients with traumatic splenic rupture. A nomogram model to predict thrombocytosis after splenectomy in patients with traumatic splenic rupture was established with R language software 4.0.2 package, internal validation of the nomogram model was performed using correction curves, and the prediction efficiency of the nomogram model was evaluated using decision curves.Results:The incidence of shock, rate of blood transfusion volume≥2 000 ml, platelet count and PCT in postoperative thrombocytosis group were significantly higher than those in postoperative platelet normal group: 69.70% (23/33) vs. 19.05% (4/21), 66.67% (22/33) vs. 38.10% (8/21), (823.56 ± 129.81) ×10 9/L vs. (521.92 ± 85.89) ×10 9/L, (0.87 ± 0.11)% vs. (0.54 ± 0.09)%, the MPV and PDW were significantly lower than those in postoperative platelet normal group: (10.23 ± 1.03) fl vs. (11.57 ± 0.92) fl and 0.113 ± 0.012 vs. 0.125 ± 0.020, and there were statistical differences ( P<0.01 or <0.05). ROC curve analysis results show that the area under curve of platelet count, MPV, PCT and PDW in for predicting the thrombocytosis after splenectomy in patients with traumatic splenic rupture were 0.973, 0.835, 0.987 and 0.734, and the optimal cut-off values were 642.29 ×10 9/L, 11.02 fl, 0.7% and 0.120. Multivariate Logistic regression analysis result showed that the platelet count, MPV, PCT, PDW, shock and blood transfusion volume were independent risk factors for thrombocytosis after splenectomy in patients with traumatic splenic rupture ( OR = 1.571, 1.243, 1.042, 1.413, 1.436 and 1.726; 95% CI 0.014 to 1.762, 0.743 to 2.862, 0.954 to 2.563, 0.584 to 2.389, 0.045 to 2.643 and 0.154 to 2.143; P<0.01 or <0.05). When platelet count, MPV, PCT, PDW, shock and blood transfusion volume were included as predictors for constructing the nomogram model, the internal validation results showed that the C-index of the nomogram model for predicting thrombocytosis after splenectomy in patients with traumatic splenic rupture was 0.793 (95% CI 0.267 to 2.311); the threshold value of the nomogram model for predicting thrombocytosis after splenectomy in patients with traumatic splenic rupture was >0.067, and the nomogram model provided a net clinical benefit; the clinical net benefit of the nomogram model was greater than that of platelet count, MPV, PCT, PDW, shock and blood transfusion volume. Conclusions:The nomogram model based on platelet count, MPV, PCT, PDW, shock and blood transfusion volume that affect the high risk of thrombocytosis after splenectomy in patients with traumatic splenic rupture has great clinical value in screening and identifying high risk patients.

Linfoma no Hodgkin primario de ovario: reporte de caso y revisión de la bibliografía / Primary ovarian non-Hodgkin lymphoma: case report and review of literature

Resumen INTRODUCCIÓN: Los linfomas no Hodgkin son un grupo heterogéneo de neoplasias hematolinfoides, de manifestación principal ganglionar; un pequeño porcentaje tiene un origen extra-ganglionar. Se han descrito algunos casos de linfomas no Hodgkin primarios de ovario y, en general, del conducto genitourinario femenino. Debido a su poca frecuencia suponen un reto diagnóstico, clínico y patológico. Se informa el caso de una paciente con linfoma no Hodgkin primario de ovario, a quien se le practicó citorreducción quirúrgica primaria y tratamiento coadyuvante con quimioterapia sistémica. Se revisa la bibliografía en torno a los criterios diagnósticos y el pronóstico de la enfermedad. CASO CLÍNICO: Paciente de 65 años, que acudió a consulta debido a un síndrome constitucional y síntomas irritativos urinarios; al ingreso tuvo signos vitales dentro de los parámetros de referencia y en el examen físico se encontraron: edema grado 2, bilateral, en los miembros inferiores y marcada palidez mucocutánea. Los estudios complementarios evidenciaron anemia microcítica hipocrómica heterogénea y trombocitosis. La resonancia magnética reportó la existencia de una masa anexial derecha, compleja, por lo que se practicó la citorreducción quirúrgica primaria. El estudio anatomopatológico reveló que se trataba de un linfoma no Hodgkin B primario de ovario. El tratamiento consistió en quimioterapia, con esquema R-CHOP. Diez meses después se estableció la curación de la enfermedad mediante estudios de imagen y pruebas de laboratorio. CONCLUSIONES: El linfoma no Hodgkin primario de ovario es una causa poco frecuente de afectación extraganglionar y se trata de un diagnóstico de exclusión, que requiere confirmación histopatológica. El pronóstico sugiere que la enfermedad confinada al ovario muestra una tasa de supervivencia global de 79% a 10 años. Se requieren estudios adicionales, que caractericen el pronóstico y el procedimiento diagnóstico de esta enfermedad.

Abstract INTRODUCTION: Non-Hodgkin's lymphomas are a heterogeneous group of neoplasms hematolymphoid, with main lymph node manifestation; a small percentage have an extra-nodal origin. Some cases of non-Hodgkin's lymphomas have been described.Primaries of the ovary and, in general, of the female genitourinary duct. Because of his they infrequently pose a diagnostic, clinical and pathological challenge. We report the case of a patient with primary ovarian non-Hodgkin's lymphoma, who underwent primary surgical cytoreduction and adjuvant treatment with systemic chemotherapy. The bibliography around the criteria is reviewed diagnosis and prognosis of the disease. CLINICAL CASE: A 65-years-old patient who presented constitutional syndrome and urinary irritative symptoms, her vital signs at the admission were normal, at the physical examination bilateral grade 2 edema in lower limbs and mucocutaneous paleness was observed, heterogeneous hypochromic microcytic anemia, thrombocytosis and also a right complex ovarian mass were documented during the hospitalization, for this reason was treated with primary surgical reduction, the histopathological result was Ovarian Primary Non Hodgkin B-cell Lymphoma, for that reason systemic adjuvant chemotherapy with R-CHOP scheme was initiated, at the ten month follow-up radiological and nuclear examinations were performed confirming the remission of the disease. CONCLUSIONS: Primary ovarian non-Hodgkin lymphoma is an unusual extra-nodular hematolymphoid neoplasm that requires a histopathological confirmation. The prognosis according to the literature review for the disease limited to the ovary has an overall survival rate of 79% to 10 years after diagnosis. More studies are required to characterize the prognosis and diagnostic approach of this entity.

Myelodysplastic syndrome/myeloproliferative neoplasm with ring sideroblasts and thrombocytosis: report of 2 cases and review of literature / 白血病·淋巴瘤

Objective:To deepen the understanding of myelodysplastic syndrome/myeloproliferative neoplasm with ring sideroblasts and thrombocytosis (MDS/MPN-RS-T), and to improve the levels of precise diagnosis and individualized treatment.Methods:The clinical data and next-generation sequencing molecular cloning results of two MDS/MPN-RS-T patients who were admitted to the First People's Hospital of Chuzhou in October 2017 and November 2019 were retrospectively analyzed, and the related literature was reviewed.Results:Case 1 was a 76-year-old female. The mutation loads from high to low were DNMT3A, JAK2 V617F and SF3B1. After administration of hydroxyurea, this patient acquired amelioration in anemia, and the platelet count improved. The clinical course was indolent. Case 2 was a 66-year-old male, who was initially diagnosed with essential thrombocythemia but failed to acquire response after hydroxyurea treatment. MDS/MP-RS-T was diagnosed after comprehensive examination. The mutation loads from high to low were SF3B1, ASXL1, JAK2 V617F and SRSF2. Pancytopenia occurred after disease progression, and the JAK2 V617F mutation finally turned negative. Administration of erythropoietin and lenalidomide failed to improve the condition, but low-dose decitabine treatment (10 mg/d, 3-5 d, once a month) improved the hematopoiesis.Conclusions:The co-mutation of JAK2 V617F and SF3B1 has a suggestive effect on the diagnosis of MDS/MPN-RS-T, and dynamic next-generation sequencing is helpful to elucidate the molecular nature of clinical heterogeneity of the disease. Low-dose decitabine has a certain curative effect on MDS/MPN-RS-T.

Gene mutation-negative essential thrombocytosis in children: report of 1 case and review of literature / 白血病·淋巴瘤

Objective:To investigate the clinical characteristics, diagnosis and treatment methods of children with gene mutation-negative essential thrombocytosis (ET).Methods:The clinical data of a child with gene mutation-negative ET in the Blood Diseases Hospital of Chinese Academy of Medical Sciences were collected, and the related literature was reviewed.Results:The epistaxis was the main clinical symptom of this child. He was diagnosed as ET (gene mutation-negative) by bone marrow aspiration and gene detection. After hydroxyurea treatment, the platelet count increased and the clinical symptoms were improved.Conclusions:The incidence rate of ET in children is low, and the frequency of gene mutation-negative ET in children reported in the literature is different. The large number of samples and long-term follow-up studies are needed.

Manejo anestésico de pacientes con trombocitemia esencial / Anesthetic management of a patient with essential trombocythemia

La trombocitemia esencial forma parte del grupo de neoplasias mieloproliferativas. Se caracteriza por síntomas microvasculares y vasomotores, recuento plaquetario superior a 450 x 109/l, proliferación megacariocítica con morfología grande y madura, ausencia de proliferación eritroide y granulocítica, demostración de JAK2V617F u otro marcador clonal y ausencia de evidencia de trombocitosis reactiva. Se reporta el manejo anestésico en una paciente donde las principales consideraciones están relacionadas con la prevención de eventos hemorrágicos y trombóticos. La suspensión de la aspirina, el mantenimiento del tratamiento con hidroxiurea, la preparación con ácido tranexámico, el uso pre y posoperatorio de fraxiparina, hidratación adecuada, uso de medias elásticas en miembros inferiores, deambulación precoz, buena hemostasia quirúrgica y disponibilidad de concentrados de plaquetas son los elementos fundamentales en la conducción anestésica de esta paciente(AU)

Essential thrombocythemia is part of the group of myeloproliferative neoplasms. It is characterized by microvascular and vasomotor symptoms, platelet count over 450x109/L, megakaryocytic proliferation with large and mature morphology, absence of erythroid and granulocytic proliferation, demonstration of JAK2V617F or other clonal marker, and absence of evidence of reactive thrombocytosis. Anesthetic management is reported in a patient, whose case's main considerations are related to the prevention of hemorrhagic and thrombotic events. Aspirin suspension, maintenance of hydroxyurea treatment, preparation with tranexamic acid, pre- and post-operative use of fraxiparin, adequate hydration, use of elastic stockings in lower limbs, early ambulation, good surgical hemostasis, as well as availability of platelet concentrates are the fundamental elements in the anesthetic management of this patient(AU)

Artrite reumatoide do tipo EORA com trombocitose significativa: relato de caso / Elderly-onset rheumatoid arthritis with significant thrombocytosis: a case report

Este relato teve como objetivo apresentar um caso de elderly onset rheumatoid arthritis associada à trombocitose reacional significativa. À admissão, o paciente apresentava quadro de poliartrite de pequenas e grandes articulações associado à rigidez matinal. Após exames solicitados, evidenciaram-se trombocitose de 1.697.000 cel./mm³ e anticorpos antipeptídeos citrulinados positivos, sendo diagnosticado com artrite reumatoide do tipo elderly onset rheumatoid arthritis.

This report aimed at presenting a case of elderly-onset rheumatoid arthritis associated with significant reactive thrombocytosis. On admission, the patient presented polyarthritis of small and large joints associated with morning stiffness. After the performance of the requested tests, thrombocytosis of 1,697,000 cells/mm3 and positive anti-CCP were evidenced, and the patient was diagnosed with elderly-onset rheumatoid arthritis.

Diagnostic value of the neutrophil/lymphocyte ratio, platelet/lymphocyte ratio, and thrombocytosis in the preoperative investigation of ovarian masses / Valor diagnóstico das razões neutrófilo/linfócito, plaqueta/ linfócito, e trombocitose na investigação pré-operatória de massas ovarianas

Abstract Objective To evaluate the diagnostic accuracy of cancer antigen 125 (CA125) and complete blood count (CBC) parameters, such as the neutrophil to lymphocyte ratio (NLR), the platelet to lymphocyte ratio (PLR), and thrombocytosis in patients with ovarian masses. Methods The present is a retrospective study conducted at a single tertiary hospital from January 2010 to November 2016. We included consecutive women referred due to suspicious adnexal masses. The CBC and CA125 were measured in the serum of 528 women with ovarian masses before surgery or biopsy. We evaluated the diagnostic performance of the NLR, PLR, platelets (PLTs), CA125, and the associations between them. We tested the clinical utility of the CBC parameters and CA125 in the discrimination of ovarian masses through decision curve analysis (DCA). Results The best balance between sensitivity and specificity was obtained by the associations of CA125 or PLTs ≥ 350/nL, with 70.14% and 71.66%, CA125 or PLTs ≥ 400/ nL, with 67.30% and 81.79%, CA125 or PLR, with 76.3% and 64.87%, and CA125 or NLR, with 71.09% and 73.89% respectively. In the DCA, no isolated CBC parameter presented a higher clinical utility than CA125 alone. Conclusion We showed that no CBC parameter was superior to CA125 in the prediction of the malignancy of ovarian tumors in the preoperative scenario.

Resumo Objetivo Avaliar a acurácia diagnóstica do antígeno de câncer 125 (cancer antigen 125, CA125, em inglês) e dos parâmetros do hemograma como as razões neutrófilo/linfócito (RNL), plaqueta/linfócito (RPL), e trombocitose em pacientes com massas ovarianas. Métodos Este é um estudo retrospectivo realizado em um hospital terciário no período de janeiro de 2010 a novembro de 2016. Foram incluídas de forma consecutiva mulheres encaminhadas por massas anexiais suspeitas. Foram dosados hemogramas e CA125 no soro de 528 mulheres com massas ovarianas antes da cirurgia ou biópsia. Foram avaliados os desempenhos diagnósticos da RNL, da RPL, das plaquetas (PLQs) e do CA125, considerando-os isoladamente e associados entre si. Testamos a utilidade clínica dos parâmetros do hemograma e do CA125 na discriminação das massas ovarianas por análise de curva de decisão (ACD). Resultados Os melhores equilíbrios entre sensibilidade e especificidade foram obtidos por meio das associações do CA125 ou PLQs ≥ 350/nL, com 70,14% e 71,66%, CA125 ou PLQs ≥ 400/nL, com 67,30% e 81,79%, CA125 ou RPL, com76,3% e 64,87%, e CA125 ou RNL, com 71,09% e 73,89%, respectivamente. Conclusão Na ACD, nenhum parâmetro do hemograma isolado se mostrou superior ao CA125 na predição de malignidade de tumores ovarianos no pré-operatório.

Características citomorfológicas de las alteraciones plaquetarias cuantitativas y su relación con otras alteraciones celulares / Cytomorphological characteristics of quantitative platelet alterations and its association with other cell alterations

Introducción: Las alteraciones cuantitativas de plaquetas son producidas por el incremento o disminución de los conteos globales de plaquetas. El incremento o trombocitosis se produce por redistribución o aumento de la producción medular; la disminución puede ser el resultado de una reducción de la producción, redistribución o acortamiento de la sobrevida de las plaquetas en circulación. Objetivo: Describir los hallazgos citomorfológicos más importantes en las alteraciones cuantitativas de plaquetas. Métodos: Se realizó una revisión de la literatura, en inglés y español, en la base de datos PubMed y el motor de búsqueda Google Académico de artículos publicados en los últimos 10 años. Se hizo un análisis y resumen de la bibliografía revisada. Análisis y síntesis de la información: Las alteraciones cuantitativas de plaquetas se caracterizan por variaciones en el número y morfología de estas células. Estas se asocian a causas congénitas o adquiridas, en la que la detallada anamnesis de los pacientes es un elemento importante en el diagnóstico. En la trombocitosis se debe diferenciar una trombocitosis reactiva de una enfermedad medular primaria; mientras que en la trombocitopenia se debe considerar el origen étnico de los pacientes y la morfología de los leucocitos. Son numerosas las causas hereditarias de trombocitopenia con anomalías morfológicas de plaquetas y granulocitos. Conclusiones: Las alteraciones cuantitativas de plaquetas son un amplio número de entidades con semejanzas y diferencias en cuanto a presentación y manifestaciones clínicas. Los exámenes de laboratorio constituyen una herramienta importante en el diagnóstico, pronóstico y el seguimiento de los pacientes afectados(AU)

Introduction: Quantitative platelet alterations are produced by the increase or decrease in global platelet counts. Platelet count increase or thrombocytosis is produced by redistribution or increased marrow production. Platelet decrease may result from production, redistribution, or shortened survival of circulating platelets. Objective: To describe the most significant cytomorphological findings in quantitative platelet alterations. Methods: A literature review was carried out, in English and in Spanish, in the database PubMed and with the search engine of Google Scholar, of articles published in the last ten years. An analysis and summary of the revised bibliography was made. Information analysis and synthesis: Quantitative platelet alterations are characterized by variations in the number and morphology of these cells. These are associated with congenital or acquired causes, in which detailed anamnesis of patients is an important element in the diagnosis. In thrombocytosis, reactive thrombocytosis must be differentiated from primary marrow disease; while in thrombocytopenia, the ethnic origin of the patients and the morphology of the leukocytes must be considered. Hereditary causes of thrombocytopenia with morphological abnormalities of platelets and granulocytes are numerous. Conclusions: Quantitative platelet alterations are a large number of entities with similarities and differences in terms of presentation and clinical manifestations. Laboratory tests are an important tool for diagnosis, prognosis, and follow-up of affected patients(AU)

Incidence of reactive thrombocytosis in serious bacterial infections in children aged two months to twelve years and its usefulness as a predictor of serious bacterial infections

Background: The aims of the study were to estimate the incidence of reactive thrombocytosis(RT) among febrile children aged 2 months to 12 years and to identify any differences in age group and gender in mounting thrombocytosis as a response to infection, to identify if thrombocytosis occurred preferentially in any particular group of serious bacterial infections(SBI), to compare thrombocytosis with other parameters like total white cell count, C-reactive protein(CRP), cultures etc. and to assess the utility of platelet count as a potential predictor of serious bacterial infection.Methods: This was a prospective study done in Institute of Child Health and Hospital for children, Madras Medical College from September 2015 to July 2016. Inclusion criteria: Children aged 2 months to 12 years with symptoms of fever less than 6 days admitted in the paediatric wards and those seen at the outpatient department. Exclusion criteria: Children having received parenteral antibiotics.Results: Of the 500 children, 142 (28.4%) had reactive thrombocytosis. RT was mild in 120 children (24%), moderate in 16 children (3.2%), severe in 4(0.8%) and extreme in two children (0.4%). This study showed that 36.48% (85 / 233) of children under 1 year had RT (p = 0.0002).There was no significant sex related difference in mounting RT. Out of 500 children included in the study, serious bacterial infection was diagnosed among 171 children (34.2%).Pneumonia (n=100) was the most common SBI followed by urinary tract infections (n=33), meningitis (n=23), sepsis (n=15). This study showed that RT has a moderate ability to predict serious bacterial infections (AUC=0.78; PPV-75.35%).Conclusions: Incidence of reactive thrombocytosis in febrile children aged 2 months to 12 years is 28.4%. It occurs more frequently in infants and without any sex predilection. RT is associated with leucocytosis, positive CRP, positive Chest X ray findings, positive urine culture and positive CSF findings. This shows that RT has a moderate ability in predicting SBI in children.

Trombose essencial: uma revisão da literatura / Essential thrombosis: a literature review

Trombose essencial é uma das doenças mieloproliferativas crônicas, rara e de etiologia ainda desconhecida, mas que apresenta risco alto de eventos trombóticos e/ou hemorrágicos, uma vez que acomete as células megacariocíticas e, consequentemente, as plaquetas. O objetivo deste trabalho foi realizar uma revisão das publicações sobre o tema abordado. O estudo caracteriza-se como revisão bibliográfica de artigos das bases de dados da Literatura Latino-Americana e do Caribe em Ciências da Saúde (Lilacs), National Library of Medicine (PubMed), Scientific Electronic Library Online (SciELO) e Google acadêmico. Foram incluídos artigos disponíveis e em qualquer idioma de publicação, com a finalidade de aumentar o referencial teórico. Posteriormente à seleção e análise dos artigos, foram encontrados alguns pontos em comum, como a dificuldade em se diagnosticar a doença. De acordo com os estudos, a maioria dos doentes é assintomática, mas pode apresentar desde eventos trombóticos até mesmo embolia pulmonar. Atualmente, o tema vem crescendo, principalmente abordando técnicas moleculares mais específicas para a descoberta da doença em seu estágio inicial. A análise dos artigos demonstrou a dificuldade do diagnóstico da trombose essencial, sendo a sua identificação crucial nos estágios iniciais.

Essential thrombosis is one of the chronic myeloproliferative diseases, rare and of unknown etiology, but which presents a high risk of thrombotic and / or hemorrhagic events. Once it attacks the megakaryocytic cells and consequently the platelets. The objective of this work is to review the publications on the subject. The study is characterized as a bibliographical review of articles from the Latin American and Caribbean Literature in Health Sciences (LILACS), National Library of Medicine (PubMed), Scientific Electronic Library Online (SciELO) and Google Acadêmico. Articles were included and in any language of publication, in order to increase the theoretical reference. Subsequent to the selection and analysis of the articles, some common points were found such as the difficulty in diagnosing the disease. According to the studies, the majority of patients are asymptomatic but may present from thrombotic events to even pulmonary embolism. Currently, the topic has been growing, mainly addressing molecular techniques more specific to the discovery of the disease in its initial stage. The analysis of the articles demonstrated the difficulty of diagnosing essential thrombosis, which is crucial in the initial stages.

Clinico-hematological study of abnormalities of platelet count in children with iron deficiency anemia

Background: Iron deficiency anemia is a major cause of morbidity in developing countries like India. The aim of the study was to assess abnormalities of platelet count in iron deficiency anemia and to relate the severity of thrombocytosis with severity of anemia and its association with erythropoietin (EPO) level.Methods: A prospective observational study comprising of 200 children below 18 years confirmed to have IDA. Erythropoietin (EPO) level was done in patients who had thrombocytosis. Degree of thrombocytosis was correlated with EPO and also with ferritin, haematological indices like hemoglobin and MCV (mean corpuscular volume) and blood counts were followed up while on iron therapy for one month.Results: Thrombocytosis was noted in 24.5%. In 75.5% thrombocytosis was mild. Platelet had negative correlation with Hb (hemoglobin). EPO was elevated in 67.35% of thrombocytosis. EPO showed negative correlation with Hb and Ferritin and positive correlation with platelet however, these were non-significant. All patients were treated with standard preparation of ferrous fumarate (33mg elemental iron every 5 ml) in a dose of 3mg/kg/day of elemental iron along with appropriate dietary advice.' On one month follow up 92% of the study population showed normalization of platelet count.Conclusions: Nearly One-fourth of children had thrombocytosis. Platelet count was inversely related to Hb and ferritin level. EPO was increased in two-third cases of thrombocytosis and showed positive correlation with platelet count. As authors excluded patients with severe IDA requiring blood transfusion, authors did not get any thrombocytopenia in present study.

Essential thrombocythemia - a predisponent factor for stroke

SUMMARY The essential thrombocythemia is one of the seven described forms of myeloproliferative neoplasms. It is characterized by megakaryocytic hyperplasia with consequent thrombocytosis maintained in the peripheral blood, favoring the occurrence of thrombo-hemorrhagic phenomena. We present the case of an 81-year-old woman with a history of ischemic stroke in the context of a sustained thrombocytosis, which led to a spinal study and a search for the V617F mutation in the JAK2 gene, which was positive. The patient started cytoreductive therapy with hydroxyurea with favorable current evolution.

Association of pretreatment thrombocytosis with prognosis in ovarian cancer: a systematic review and meta-analysis / 부인종양

OBJECTIVE: To investigate the association between pre-treatment thrombocytosis and prognosis in patients with ovarian cancer (OC). METHODS: PubMed, EMBASE, and the Cochrane Library were searched for articles regarding the prognosis of OC patients with pre-treatment thrombocytosis by the end of March 2018. Pooled estimates for overall survival (OS) and progression-free survival (PFS) events were calculated as hazard ratios (HRs) either on a fixed or random effect model by Stata 13.0 software. Funnel plot and Egger's test were applied to evaluate publication bias and sensitivity analyses were undertaken to estimate the strength of outcomes. RESULTS: Eleven studies that met the inclusion criteria were enrolled, including a total of 4,953 patients. Pooled results showed that pre-treatment thrombocytosis was significantly associated with OS (HR=1.722; 95% confidence interval [CI]=1.437–2.064) and PFS (HR=1.452; 95% CI=1.323–1.593) in the cohort. Significant correlation was found in OS and PFS between pre-treatment thrombocytosis and both epithelial OC (all stages and differentiation degrees of OC) and advanced epithelial OC (III or IV) by subgroup analyses, which were performed according to publication year, country, case numbers, OC category, International Federation of Gynecology and Obstetrics stage, and cut-off value. However, subgroup analyses indicated no significant correlation between pre-treatment thrombocytosis and OS for patients with high-grade serous (poorly differentiated or undifferentiated) OC (HR=1.220; 95% CI=0.946–1.573; p=0.125). Egger's test demonstrated no obvious publication bias in the articles enrolled in this study (OS: p=0.226; PFS: p=0.071). CONCLUSION: Pre-treatment thrombocytosis might be taken as an independent prognostic indicator for patients with OC.

Gene mutation spectrum and clinical characteristics analysis of 178 patients with essential thrombocytosis / 中华血液学杂志

Objective@#To analyze the gene mutation spectrum, clinical features, and the factors of disease progression and prognosis in patients with essential thrombocytosis (ET) .@*Methods@#A retrospective analysis was conducted on 178 newly diagnosed ET patients admitted from February 1st, 2009 to November 1st, 2018.@*Results@#Of the 178 patients, 89 were male and 89 female, and the median diagnosis age was 49.5 (3-86) years old. JAK2V617F, CALR and MPL mutations frequencies were 16.45% (1.67%-43.90%) , 40.00% (10.00%-49.15%) and 25.10% (25.00%-40.00%) , respectively. Compared with patients with CALR mutations, patients with JAK2V617F mutation had higher diagnosis age (P=0.035) , higher white blood cell count (P=0.040) , higher hemoglobin concentration (P=0.001) , and lower platelet count (P=0.002) , respectively. Of them, 47 patients (27.01%) developed thrombotic events before diagnosis, and 3 ones (1.72%) experienced thrombotic events after diagnosis. Multivariate analysis revealed age >60 years (P=0.013, OR=4.595, 95%CI 1.382-15.282) and cardiovascular risk factors (CVF) (P<0.001, OR=8.873, 95%CI 2.921-26.955) as risk factors for thrombotic events, CALR mutation (P=0.032, OR=0.126, 95%CI 0.019-0.838) as a protective factor for thrombotic events. Age >60 years (P=0.042, OR=4.045, 95%CI 1.053-15.534) was found to be a risk factor for the overall survival (OS) of ET patients. OS of age ≤60 years and age>60 years were calculated by Kaplan-Meier analysis to be (115.231±1.899) months and (83.291±4.991) months (χ2=6.406, P=0.011) , respectively.@*Conclusion@#Age>60 years and CVF were risk factors for thrombotic event. CALR mutation was a protective factor for thrombotic event. Age >60 years was a risk factor for OS in ET patients.