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.

Evaluation of oral-motor movements and facial mimic in patients with head and neck burns by a public service in Brazil

OBJECTIVES: The purpose of this study was to analyze the characteristics of oral-motor movements and facial mimic in patients with head and neck burns. METHODS: An observational descriptive cross-sectional study was conducted with patients who suffered burns to the head and neck and who were referred to the Division of Orofacial Myology of a public hospital for assessment and rehabilitation. Only patients presenting deep partial-thickness and full-thickness burns to areas of the face and neck were included in the study. Patients underwent clinical assessment that involved an oral-motor evaluation, mandibular range of movement assessment, and facial mimic assessment. Patients were divided into two groups: G1 - patients with deep partial-thickness burns; G2 - patients with full-thickness burns. RESULTS: Our final study sample comprised 40 patients: G1 with 19 individuals and G2 with 21 individuals. The overall scores obtained in the clinical assessment of oral-motor organs indicated that patients with both second- and third-degree burns presented deficits related to posture, position and mobility of the oral-motor organs. Considering facial mimic, groups significantly differed when performing voluntary facial movements. Patients also presented limited maximal incisor opening. Deficits were greater for individuals in G2 in all assessments. CONCLUSION: Patients with head and neck burns present significant deficits related to posture, position and mobility of the oral myofunctional structures, including facial movements. .

A Novel Case of Extreme Thrombocytosis in Acute Myeloid Leukemia Associated With Isochromosome 17q and Copy Neutral Loss of Heterozygosity

No abstract available.

A Rare Case of Chronic Myelogenous Leukemia and Plasma Cell Myeloma in the Same Patient

No abstract available.

Blood changes after splenectomy in portal hypertension. The "Amna model"

Splenectomy is a frequently performed operation for patients with portal hypertension and hypersplenism in areas endemic for schistosomiasis. Reactive thrombocytosis [RT] has been described following splenectomy. Nevertheless, data are lacking on the long term changes of platelet count and other blood cells and indices after splenectomy in such patients. To determine the hematological changes following splenectomy in portal hypertension due to schistosomiasis. This is a prospective, observational, case study in which we did a series of full blood counts before and after splenectomy on two patients. The periods of study were 12 days and 5 months, respectively. Platelets [RT>500x10[9]] occurred after the 7[th] post-splenectomy day in both patients. In the longer study, RT continued from day 8 to week10. Extreme thrombocytosis [> 1000x 10[9]] occurred from day 11 to day 21 postoperatively. The count was back to normal by week 17. Reactive leukocytosis occurred immediately after ligation of the splenic artery, but returned to normal levels during the 2[nd] postoperative week. Erythrocytes, however, showed changes different from both platelets and leukocytes. There was "persistent anaemia" for about one month postoperatively. This study provides data that were lacking on the long term haematological effects of splenectomy in Sudanese patients with portal hypertension. Knowing these changes can have important implications on the management and follow up of such patients, and provide evidence needed for discussing guidelines of treatment. RT and leukocytosis occur after splenectomy whereas anaemia persists. RT can reach extreme levels and it is wise to monitor the platelets after the discharge of patients from the hospital. It may also be wise to consider the use of antiplatelet drugs postoperatively till the count normalizes. The persistent anaemia, however, seems to correct itself without giving haematinics or blood transfusion.

Clinicohematological study of thrombocytosis.

Objective. To find out etiology and clinical course of thrombocytosis in Indian pediatric population. Methods. A total of 250 patients having thrombocytosis (defined as platelet count >500 x 109/L) on haematological investigations were studied over one yr period. All patients were evaluated clinically and were subjected to investigations, including complete blood counts (CBC) with peripheral smear examination. To elucidate the possible role of inflammatory cytokines in pathogenesis of RT, levels of Interleukin-6 (IL-6) and C - reactive protein (CRP) were estimated. Results. Infants and young children (<2 yr age) were most common group, contributing 60% of total cases. Out of total 250 cases, only 3 (1.2%) cases were found to have primary thrombocytosis and remaining 98.8% cases were having RT. Among RT patients, infections (alone or in association with iron deficiency anemia) were most common cause, accounting for 65% cases, while iron deficiency anemia (IDA) was second most common cause accounting for 41.3% cases (12.6% IDA alone and 28.7 % in association with infections). Other causes included nutritional dimorphic anemia and patients on treatment for megaloblastic anemia, acute lymphoblastic leukemia (during treatment) and lymphoma. Among various groups of RT, IL-6 and CRP levels were higher in patients with infection with or without IDA than IDA alone. One child with essential thrombocytosis and one child with RT had thrombotic complications. On follow up, platelet counts normalized in most of the patients with treatment of underlying conditions. Conclusions. Results of this study suggest that essential thrombocytosis is extremely rare in children. Infections and IDA (alone or in association with infections) are common causes of RT. IL-6 and CRP levels are increased in patients with RT, to a higher level in patients with infections than in patients with IDA. Most patients with RT have uneventful recovery of platelet counts to normal range with treatment for underlying condition.

Cholestatic Hepatitis and Thrombocytosis in a Secondary Syphilis Patient

The incidence of acute hepatitis in syphilis patient is rare. First of all, our patient presented with hepatitis comorbid with thrombocytosis. To our knowledge, this is only the second report of syphilitic hepatitis with thrombocytosis. The 42-yr-old male complained of flulike symptoms and skin eruptions on his palms and soles. Laboratory findings suggested an acute hepatitis and thrombocytosis. Serologic test results were positive for VDRL. He recovered from his symptoms and elevated liver related enzymes with treatment. Because syphilitic hepatitis can present without any typical signs of accompanying syphilis, syphilis should be considered as a possible cause in acute hepatitis patients.

Thrombocytosis in childhood.

Thrombocytosis is a frequent finding in hemograms obtained from hospitalized and ambulatory children due to the widespread use of automated blood cell counters. Pediatricians are commonly puzzled in cases of thrombocytosis to determine the underlying cause and the need for therapy. The purpose of this review is to assist the general pediatrician into dealing with this common hematological finding in every day clinical practice. Fortunately, primary thrombocytosis or essential thrombocythemia, a clonal disease, is exceedingly rare in childhood, but may be associated with thromboembolic and hemorrhagic complications. On the other hand, secondary or reactive thrombocytosis is very common and is due to a variety of conditions, such as acute and chronic infections, iron deficiency, bleeding, hemolytic anemias, collagen vascular diseases, malignancies, drugs and splenectomy. Treatment of reactive thrombocytosis should be directed to the underlying problem alone. Administration of platelet aggregation inhibitors such as aspirin is unwarranted. Consultation is necessary only for the rare child with extreme thrombocytosis who has clinical and/or laboratory criteria consistent with essential thrombocythemia, or in whom a hemorrhagic or thrombotic complication has developed.

interest of automated parameters of the platelet count in the diagnosis of essential thrombocythemia

Essential thrombocythemia is an increase of the initial platelet, which frequency tends to increase with the generalization of platelet counts on automat, allowing a positive diagnosis. The objective of this study is to compare platelet parameters of the ADVIA 120@ [Bayer Diagnostic, Tarrytown USA] between two populations of essential thrombocythemia [ET] and Secondary Thrombocythemia [ST] and to establish threshold values for these analyzed platelet parameters. This study include 210 patients, hospitalized at service of internal medicine, and whose selection criteria were an platelet count > 400G/L and/or diagnosis of Essential thrombocythemia. Then, the formula composed with threshold values of platelet parameters that we have found was applied on other groups of patients with a hyper-platelet count >400G/L and whose diagnosis was undetermined. Six platelet parameters out of nine present a significant difference between Et and ST [Mean platelet volume [VPM], variation Coefficient of the platelet of the platelet volume [CV Plat], Mean Platelet, Concentration [CPM], Standard deviation of the platelet concentration [ETCP], big platelet and Aggregate count], and the combination of at least four parameters, gives a sensitive [65 percent] and specific [95 percent] diagnosis Key for ET. These preliminary results show a major interest for ETCP parameters and big platelet parameters. Their association with other platelet parameters and the platelet count higher than 600 G/L should allow to better direct the diagnosis of ET

Cancer colique revele par une thrombocytose

We report a case of a 60-year-old woman who presented thrombocytosis at 2.400.000 m[3] associated with a tumor of the colon. This article describes a paraneoplastic thrombocytosis higher a 2.000.000 mm [3] probably secondary to the elevation of the serum interleukin 6 levels of the thrombocytosis

Trombocitosis en la edad pediátrica / Thrombocytosis in paedriatric patients

El valor normal de plaquetas varía entre 150,000 y 450,000 x mm al cubo. Se define trombocitosis como un recuento mayor de 600,000. Pueden ser primarias, por un trastorno mieloproliferativo o secundarias a un gran número de patologías. Objetivos: conocer en nuestro medio la frecuencia de ambos tipos de trombocitosis, describir las patologías asociadas y relacionar la magnitud de la trombocitosis con los diferentes diagnósticos. Material y métodos: se analizaron 18,000 hemogramas realizados entre enero y diciembre de 1998, en el Hospital Roberto del Río. Se evaluó sexo, recuento de plaquetas y leucocitos, hematocrito, hemoglobina, VCM, CHCM y diagnósticos. Resultados: se encontró trombocitosis en 584 exámenes (3,24 por ciento). Se evaluaron 334 fichas, el 62 por ciento eran de sexo masculino. El 0,9 por ciento presentó cifras de plaquetas > 1,000,000 x mm al cubo (dos casos fueron trombocitosis primarias: trombocitemia esencial y leucemia mieloide crónica y un caso de meningitis bacteriana). Las trombocitosis secundarias se asociaron a: infecciones (48,8 por ciento), principalmente respiratorias, deficiencia de hierro (18,6 por ciento) y daño tisular (12,6 por ciento). Conclusiones: la frecuencia de trombocitosis en niños es baja. Cuando la trombocitosis es menor de 1,000,000 x mm al cubo debe sospecharse una etiología secundaria

Epidemiología de la trombocitosis / Thrombocytosis epidemiology

En los últimos años la trombocitosis (T) es reportada con más frecuencia gracias a la utilización de los sistemas automaticos de conteo. Definimos como Trombocitosis (T) a cifras de plaquetas superiores a 500.000. Con el objetivo de conocer la epidemiología de la (T) en los pacientes internados, se revisaron las historias clínicas de los niños con T internados en el Hospital J. P. Garrahan en cuatro meses de distintos períodos estacionales. Método estadístico: chi cuadrado. Presentaron T el 17 por ciento (n=201) de los pacientes a los que se les realizó hemograma. Edad promedio: 2,9 años. (DS: 4 años). 48 por ciento eran menores de 1 año (p<005). La etiología más frecuente fue la infecciosa (63 por ciento) seguida por la anemia (13 por ciento), el postquirúrgico inmediato (12.4 por ciento), la patología respiratoria (8.9 por ciento), los quemados (7.5 por ciento) y la enfermedad de Kawasaki (1.5 por ciento). Según el grupo etáreo, la etiología infecciosa fue la más frecuente en todos ellos. Como segunda causa predominó la anemia en el grupo de 1 á 5 años y los postquirúrgicos en los mayores de 10 años, igualándose estas dos causas en los otros grupos etáreos (<1 año y 5-10 años). Clasificamos la T en a) leve: 500-750.000 plaquetas, la presentaron el 73 por ciento, b) moderada: 750-1.000.000 plaquetas, el 22 por ciento y c) grave: >1.000.000 el 5 por ciento. La severidad de la T no tuvo relación con la etiología. Se observó mayor incidencia de leucocitosis en la T grave. Se concluye que la T es una manifestación hematológica frecuente en los pacientes internados, el grupo etáreo más afectado son los menores de 1 año y su presencia debe hacer pensar en patología infecciosa.

Postoperative thrombocytosis after coronary artery bypass grafting: a potential danger even after hospital discharge

Thrombocytosis [platelet counts >400 ' 103/mm3] following coronary artery bypass grafting has been described to occur frequently [20-30%] and to be associated with thrombotic complications postoperatively. The purpose of the present study is to establish when the peak value of platelet count occurs, and how high it is, as well as to determine the duration of thrombocytosis. Thirty consecutive patients undergoing elective coronary artery bypass grafting, who subsequently developed postoperative thrombocytosis [group 1] were considered for the study. Another 30 patients with platelet counts within normal limits postoperatively served as controls [group 2]. Platelet count was monitored on a weekly basis during 5 weeks postoperatively. Patient characteristics, operation data and cardiopulmonary bypass data in group 1 did not differ from group 2 patients, except for a higher incidence of hyperlipidemia, i.e., 97% [29/30 patients] in group 1 compared to 40% [12/30] in group 2 [p < 0.001]. Neither deaths nor nonfatal myocardial infarctions occurred during the study period. Postoperative thrombocytosis was diagnosed on 6.1 +/- 1.5 days postoperatively, and peak platelet count reached 14 +/- 4.0 days postoperatively [6-21 days]. The highest platelet count observed was 905,000/mm3. Platelet counts returned to normal values within 5 weeks. Three late vein graft occlusions occurred in all groups, and thus coinciding with the maximum platelet count observed. Conclusions: Postoperative thrombocytosis is a potentially dangerous complication, with an increased risk for vein graft occlusion. Postoperative thrombocytosis, when it occurs, is diagnosed around the 6th postoperative day, reaches its peak 2 weeks postoperatively and may last as long as 5 weeks. Close surveillance of patients with postoperative thrombocytosis is emphasized

Trombocitosis

Con la incorporación generalizada de los contadores electrónicos al laboratorio clínico es frecuente el hallazgo de trombocitosis. Como en la anemia u otro signo clínico, el aumento de plaquetas no es una enfermedad sino que debe considerarse como la expresión de varias situaciones clínicas y en este caso el médico debe identificar la enfermedad de fondo. Se definen dos tipos de trombocitosis: la trombocitosis maligna o mieloproliferativa y la trombocitosis secundaria o relativa. Se hace un análisis detallado del cuadro clínico de la trombocitosis esencial primaria y se enfatiza en la necesidad y en los pasos para lograr su diagnóstico adecuado y oportuno, como punto de partida para el tratamiento. Se analizan las principales causas de trombocitosis secundaria y se definen los principales criterios para su diagnóstico. Se llama la atención para no minimizar el problema y a no administrar tratamientos empíricos sin diagnóstico etiológico, a través del estudio integral del paciente y de la utilización racional de los recursos de laboratorio.

Hemostatic profile in nephrotic syndrome.

OBJECTIVE: To evaluate the coagulation profile and its relation to steroid therapy, and the frequency of thromboembolic complications and its correlation with coagulation parameters in nephrotic syndrome (NS). SETTING: Hospital based. SUBJECTS AND METHODS: Forty children with NS were subdivided into four groups, namely, fresh cases, steroid dependent, remission after therapy and steroid resistant. An equal number of age and sex matched children served as controls. In all the study and control subjects, detailed clinical examination, liver function tests, renal function tests and detailed coagulation profile were done. Evaluation of renal veins and inferior vena cava for the presence of thrombosis was also done by abdominal ultrasonography. RESULTS: Thrombocytosis was detected in 57.5% and the degree of thrombocytosis was directly related to the amount of proteinuria. The mean prothrombin and thrombin times were within normal range in the study children. The activated partial thromboplastine time (APTT) was prolonged in six cases (15%) and three out of these six children had thromboembolic complications. Antithrombin-III level was significantly lower (p < 0.001) whereas protein C and S were significantly elevated (p < 0.001) as compared to controls. The levels became normal with remission of the disease. Steroid therapy significantly increased the levels of proteins C, protein S. AT-III and fibrinogen as compared to controls. Thromboembolic complications were seen in 3 cases (7.6%) and were associated with very low levels of AT-III and protein C and all three had serum albumin below 2 g/dl. CONCLUSIONS: The importance of coagulation profile in nephrotic syndrome is highlighted and a high index of suspicion for thromboembolic complications is warranted in patients with thrombocytosis, hyper fibrinogenemia, prolonged APTT and in children with low levels of AT-III, protein C and protein S.

Serum interleukin-6 in Kawasaki disease

Kawasaki disease (KD) is an acute febrile illness of infancy and early childhood. In spite of extensive studies, the cause of KD is not known. Interleukin 6 (IL-6) has manyfold biological functions involved in the immune or inflammatory responses of the host to various stimuli. Here the author investigated whether IL-6 might be responsible for manifestations of KD, such as immunoglobulin hypersecretion, lymphocyte activation and systemic vasculitis. Serum IL-6 levels in KD were determined by ELISA. Usually sera from healthy children contained only negligible levels of IL-6. Serum IL-6 was markedly elevated in all patients with acute KD, which gradually decreased during the course of the disease. Serum IL-6 correlated with serum concentration of C-reactive protein and with serum soluble interleukin-2 receptor level, but did not show any correlation with peak platelet count during subacute phase of the disease. Increased serum IL-6 level did not show any relation to development of coronary aneurysms and dilatation. Further studies will be needed to examine the source and the pathogenetic roles of increased serum IL-6 in KD.

Renal cell carcinoma: presenting with congestive hepatosplenomegaly and rare haematological complications.

We report here a case of right-sided renal cell carcinoma who presented with hypertension and multi-organ metastases. Haematological manifestations noted were erythrocytosis, thrombocytosis and leukaemoid reaction. Of these leukemoid reaction and thrombocytosis are very rare. The patient had hepatosplenomegaly which was found to be congestive in origin due to the pressure of the tumour on the hepatic vein and the inferior vena cava. These rare features make it an unusual case.