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1.
Medicina (Kaunas) ; 60(6)2024 May 26.
Artículo en Inglés | MEDLINE | ID: mdl-38929489

RESUMEN

Background and Objectives: Adult T-cell leukemia/lymphoma (ATLL) is a highly aggressive T-cell lymphoproliferative disease associated with the human T-cell lymphotropic virus type I (HTLV-1). ATLL is a rare disease, found more frequently in HTLV-1-endemic areas, Romania being one of them. Despite treatment advances, the prognosis remains dismal. We aimed to describe the clinical, biological, and survival outcome features of Romanian patients with aggressive-type ATLL. Materials and Methods: We report the data of a prospective, observational, and unicentric study of all 20 patients diagnosed with lymphoma and acute types of ATLL at our center over the past 12 years. Data were collected from the patients' medical records. Results: Lymphoma-type ATLL (60%) was more common than acute-type ATLL (40%). Median age at diagnosis was 40.5 years, and most patients were female. Laboratory data revealed significant differences between acute and lymphoma-type ATLL, namely, higher leukocyte (p = 0.02) and lymphocyte counts (p = 0.02) and higher levels of corrected calcium (p = 0.001) in acute-type ATLL. All patients received chemotherapy, and only two underwent allogeneic stem cell transplantation. Only six patients obtained a complete or partial response to chemotherapy, mostly the lymphoma-type ones. The median survival for all patients was 6.37 months, with higher survival in the lymphoma-type ATLL (8.16 months) than in the acute-type (3.60 months). Normal calcium levels (p = 0.011), uric acid (p = 0.005), BUN score (p = 0.000), JCOG-PI moderate risk (p = 0.038), and obtaining complete or partial response (p = 0.037) were associated with higher survival. Conclusion: Aggressive-type ATLL among Romanian patients presents distinct characteristics, including younger age at diagnosis, female predominance, and higher incidence of lymphoma-type ATLL compared to currently reported data. Survival remains very low, with all subtypes experiencing a median survival of less than one year.


Asunto(s)
Leucemia-Linfoma de Células T del Adulto , Humanos , Femenino , Leucemia-Linfoma de Células T del Adulto/mortalidad , Leucemia-Linfoma de Células T del Adulto/terapia , Leucemia-Linfoma de Células T del Adulto/epidemiología , Leucemia-Linfoma de Células T del Adulto/diagnóstico , Masculino , Adulto , Persona de Mediana Edad , Rumanía/epidemiología , Estudios Prospectivos , Virus Linfotrópico T Tipo 1 Humano , Infecciones por HTLV-I/mortalidad , Infecciones por HTLV-I/complicaciones , Anciano , Análisis de Supervivencia , Enfermedades Endémicas , Pronóstico
2.
Int J Mol Sci ; 25(4)2024 Feb 10.
Artículo en Inglés | MEDLINE | ID: mdl-38396839

RESUMEN

The management of immune thrombocytopenia (ITP) and the prediction of patient response to therapy still represent a significant and constant challenge in hematology. ITP is a heterogeneous disease with an unpredictable evolution. Although the pathogenesis of ITP is currently better known and its etiology has been extensively studied, up to 75% of adult patients with ITP may develop chronicity, which represents a significant burden on patients' quality of life. A major risk of ITP is bleeding, but knowledge on the exact relationship between the degree of thrombocytopenia and bleeding symptoms, especially at a lower platelet count, is lacking. The actual management of ITP is based on immune suppression (corticosteroids and intravenous immunoglobulins), or the use of thrombopoietin receptor agonists (TPO-RAs), rituximab, or spleen tyrosine kinase (Syk) inhibitors. A better understanding of the underlying pathology has facilitated the development of a number of new targeted therapies (Bruton's tyrosine kinase inhibitors, neonatal Fc receptors, strategies targeting B and plasma cells, strategies targeting T cells, complement inhibitors, and newer TPO-RAs for improving megakaryopoiesis), which seem to be highly effective and well tolerated and result in a significant improvement in patients' quality of life. The disadvantage is that there is a lack of knowledge of the predictive factors of response to treatments, which would help in the development of an optimized treatment algorithm for selected patients.


Asunto(s)
Púrpura Trombocitopénica Idiopática , Trombocitopenia , Adulto , Recién Nacido , Humanos , Púrpura Trombocitopénica Idiopática/etiología , Púrpura Trombocitopénica Idiopática/terapia , Calidad de Vida , Trombocitopenia/tratamiento farmacológico , Recuento de Plaquetas , Inmunoglobulinas Intravenosas/uso terapéutico , Trombopoyetina , Proteínas Recombinantes de Fusión/uso terapéutico
3.
Medicina (Kaunas) ; 58(11)2022 Oct 28.
Artículo en Inglés | MEDLINE | ID: mdl-36363509

RESUMEN

ATL is a rare but a highly aggressive T-cell neoplasm associated with human T-cell leukemia virus-1 (HTLV-1) infection. Human T-cell lymphotropic virus type-1 (HTLV-1) is a oncogenic retrovirus responsible for the development of adult T-cell leukemia (ATL), but also for other non-malignant diseases, such as HTLV-1-associated myelopathy/tropical spastic paraparesis (HAM/TSP). HTLV-1 has a higher prevalence in Japan, the Caribbean, South America, intertropical Africa, Romania, and northern Iran. ATL patients can have an extensive spectrum of neurological manifestations. Numerous factors can be implicated, such as central nervous system infiltrates, neurolymphomatosis, complications to medication or allogeneic stem cell transplantation, HAM/TSP, infections, metabolic disturbances. The neurological complications are not always easy to recognize and treat. Thus, this review underlines the necessity of a multidisciplinary approach in ATL patients with neurological symptomatology.


Asunto(s)
Virus Linfotrópico T Tipo 1 Humano , Leucemia-Linfoma de Células T del Adulto , Paraparesia Espástica Tropical , Adulto , Humanos , Leucemia-Linfoma de Células T del Adulto/complicaciones , Leucemia-Linfoma de Células T del Adulto/terapia , Paraparesia Espástica Tropical/complicaciones , Paraparesia Espástica Tropical/epidemiología , Paraparesia Espástica Tropical/patología , Sistema Nervioso Central , África
4.
J Clin Med ; 11(3)2022 Jan 27.
Artículo en Inglés | MEDLINE | ID: mdl-35160118

RESUMEN

Taurine is a semi-essential, the most abundant free amino acid in the human body, with a six times higher concentration in platelets than any other amino acid. It is highly beneficial for the organism, has many therapeutic actions, and is currently approved for heart failure treatment in Japan. Taurine has been repeatedly reported to elicit an inhibitory action on platelet activation and aggregation, sustained by in vivo, ex vivo, and in vitro animal and human studies. Taurine showed effectiveness in several pathologies involving thrombotic diathesis, such as diabetes, traumatic brain injury, acute ischemic stroke, and others. As human prospective studies on thrombosis outcome are very difficult to carry out, there is an obvious need to validate existing findings, and bring new compelling data about the mechanisms underlying taurine and derivatives antiplatelet action and their antithrombotic potential. Chloramine derivatives of taurine proved a higher stability and pronounced selectivity for platelet receptors, raising the assumption that they could represent future potential antithrombotic agents. Considering that taurine and its analogues display permissible side effects, along with the need of finding new, alternative antithrombotic drugs with minimal side effects and long-term action, the potential clinical relevance of this fascinating nutrient and its derivatives requires further consideration.

5.
Exp Ther Med ; 22(4): 1096, 2021 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-34504550

RESUMEN

Primary immune thrombocytopenia (ITP) is characterized by isolated low platelet count and it is a diagnosis of exclusion, contrasting to secondary ITP. Therefore, a positive diagnosis is difficult and requires extensive investigation. Some of the underlying conditions that are associated with ITP are lymphoproliferative disorders and infections, especially viral ones. In the present study, the case of a patient diagnosed with diffuse large B-cell lymphoma, who received chemotherapy and autologous hematopoietic stem cell transplantation is presented. After a complete remission of four years, the patient presented with sudden intense hemorrhagic syndrome and severely decreased platelet count. The most frequent causes of secondary ITP were excluded, including lymphoma relapse, and intravenous corticosteroids were started. However, shortly after hospital admission, the patient developed neuro-psychiatric anomalies, fever and pancytopenia, and West-Nile encephalitis was diagnosed. Although the initial development was favorable, he started to complain of progressive severe muscle weakness and eventually succumbed to infectious complications in the setting of prolonged hospitalization, corticotherapy, and immobilization.

6.
Medicina (Kaunas) ; 57(5)2021 Apr 30.
Artículo en Inglés | MEDLINE | ID: mdl-33946377

RESUMEN

Background: Emerging evidence suggests that patients with metabolic (dysfunction) associated fatty liver disease (MAFLD) are prone to severe forms of coronavirus disease (COVID-19), especially those with underlying liver fibrosis. The aim of our study is to assess the association of an increased FIB-4 score with COVID-19 disease prognosis. Methods: We performed a prospective study on hospitalized patients with known type II diabetes mellitus (T2DM) and confirmed COVID-19, with imaging evidence of liver steatosis within the last year or known diagnosis of MAFLD. All individuals were screened for liver fibrosis with a FIB-4 index. We evaluated the link between FIB-4 and disease prognosis. Results: Of 138 participants, 91.3% had MAFLD and 21.5% patients had a high risk of fibrosis. In the latter group of patients, the number of severe forms of disease, the hospital stay length, the rate of ICU admissions and the number of deaths reported registered a statistically significant increase. The independent predictors for developing severe forms of COVID-19 were obesity (odds ratio (OR), 3.24; 95% confidence interval (CI), p = 0.003), higher values of ferritin (OR-1.9; 95% CI, 1.17-8.29, p = 0.031) and of FIB-4 ≥ 3.25 (OR-4.89; 95% CI, 1.34-12.3, p = 0.02). Conclusions: Patients with high scores of FIB-4 have poor clinical outcomes and liver fibrosis may have a relevant prognostic role. Although the link between liver fibrosis and the prognosis of COVD-19 needs to be evaluated in further studies, screening for liver fibrosis with FIB-4 index, particularly in patients at risk, such as those with T2DM, will make a huge contribution to patient risk stratification.


Asunto(s)
COVID-19 , Diabetes Mellitus Tipo 2 , Enfermedad del Hígado Graso no Alcohólico , Diabetes Mellitus Tipo 2/complicaciones , Diabetes Mellitus Tipo 2/epidemiología , Humanos , Pronóstico , Estudios Prospectivos , SARS-CoV-2
7.
J Clin Med ; 10(1)2021 Jan 04.
Artículo en Inglés | MEDLINE | ID: mdl-33406783

RESUMEN

Anabolic androgenic steroids (AAS), simply called "androgens", represent the most widespread drugs used to enhance performance and appearance in a sporting environment. High-dosage and/or long-term AAS administration has been associated frequently with significant alterations in the cardiovascular system, some of these with severe endpoints. The induction of a prothrombotic state is probably the most life-threatening consequence, suggested by numerous case reports in AAS-abusing athletes, and by a considerable number of human and animal studies assessing the influence of exogenous androgens on hemostasis. Despite over fifty years of research, data regarding the thrombogenic potential of exogenous androgens are still scarce. The main reason is the limited possibility of conducting human prospective studies. However, human observational studies conducted in athletes or patients, in vitro human studies, and animal experiments have pointed out that androgens in supraphysiological doses induce enhanced platelet activity and thrombopoiesis, leading to increased platelet aggregation. If this tendency overlaps previously existing coagulation and/or fibrinolysis dysfunctions, it may lead to a thrombotic diathesis, which could explain the multitude of thromboembolic events reported in the AAS-abusing population. The influence of androgen excess on the platelet activity and fluid-coagulant balance remains a subject of debate, urging for supplementary studies in order to clarify the effects on hemostasis, and to provide new compelling evidence for their claimed thrombogenic potential.

8.
J Clin Med ; 11(1)2021 Dec 26.
Artículo en Inglés | MEDLINE | ID: mdl-35011859

RESUMEN

BACKGROUND AND OBJECTIVES: In acute myeloid leukemia (AML), extensive bleeding is one of the most frequent causes of death. Impaired activation and aggregation processes were identified in previous studies on platelet behaviour associated with this disease. This study's aim was to examine platelet function in correlation with other haemorrhage risk factors (fever, sepsis, recent bleeding, uraemia, leucocytosis, haematocrit value, treatment). DESIGN AND METHODS: The analysis of platelet surface proteins (Glycoprotein Ib-IX (CD42b, CD42a), Glycoprotein IIb-IIIa (CD41, CD61), p-selectin (CD62P), granulophysin (CD63)) was conducted by flowcytometry from samples of whole blood in patients with acute myeloid leukaemia in different stages of diagnosis and therapy (n = 22) in comparison with healthy human controls (n = 10). RESULTS AND INTERPRETATIONS: Our results show a significant decrease in fluorescence level associated with platelet activation markers (CD63 (14.11% vs. 40.78 % p < 0.05); CD62P (15.26% vs. 28.23% p < 0.05)); adhesion markers (CD42b (69.08% vs. 84.41% p < 0.05)) and aggregation markers (CD61 (83.79% vs. 98.62% p < 0.001)) in patients compared to controls. The levels of CD41 (80.62% vs. 86.31%, p = 0.290) and CD42a (77.98% vs. 94.15%, p = 0.99) demonstrate no significant differences in the two groups. CONCLUSION: The AML patients present changes in adhesion receptors and activation markers, suggesting a functional defect or denatured intracellular signalling in platelets. The exposed data indicate that flow cytometry can effectively identify multiple functional platelet impairments in AML pathogenesis.

9.
Medicina (Kaunas) ; 56(5)2020 May 11.
Artículo en Inglés | MEDLINE | ID: mdl-32403360

RESUMEN

Background and Objectives: Current recommendations and treatment regimens in breast cancer are a reflection of its heterogeneity on multiple levels including histological subtypes, grading, molecular profiling, and numerous prognostic indices. Although based on extensive research, current guidelines are not explicit in the case of surgical specimens showing various degrees of mismatch between different parts of the same tumor and even more so between multicentric lesions. Synchronous breast cancer is the ideal prototype for studying inter- and intra-tumoral heterogeneity, therefore we envisaged that a study on patients with multicentric and multifocal lesions could contribute to the reshaping of the staging, prognosis, and treatment of breast malignancies. Material and Methods: A prospective observational study was conducted between January 2013 and May 2017 on 235 patients diagnosed with breast cancer (BC) and surgically treated at Emergency University Hospital, Bucharest. Thirty-seven patients had multiple breast tumors and were eligible for assessment of the heterogeneity of their lesions. Results: 6 were multicentric and 31 multifocal. The number of foci varied from 2 to 11. We encountered numerous mismatches between the index and the secondary tumors, as follows: 3 cases (8.1%) with histopathological mismatch, 13 (35.1%) with different grades of differentiation, 11 (29.8%) with ER (Estrogen Receptors) status mismatch, 12 (32.4%) with PR (Progesterone Receptors) status mismatch, 8 (21.6%) with molecular phenotype mismatch, and 17 (45.9%) cases with variable Ki-67. After careful analysis of index and secondary tumors, apart from the mismatches reported above, we discovered that the secondary tumors were actually dominant in 5 cases (13.5%), and therefore at least those cases had to be reclassified/restaged, as the supplementary data commanded changes in the therapeutic decision. Conclusions: For synchronous breast tumors, the current Tumor-Node-Metastasis (TNM) staging system ignores not only the histopathological and immunohistochemical characteristics of the secondary foci, but also their size. When secondary lesions are more aggressive or their cumulative mass is significantly bigger than that of the index tumor, the treatment plan should be adapted accordingly. We believe that information obtained from examining secondary foci in synchronous breast cancer and assessment of the cumulative tumoral mass should be reflected in the final staging and definitive treatment. The clinical benefit of staging the patients based on the most aggressive tumor and the cumulative tumoral burden rather than according to the biggest single tumor, will avoid under-treatment in cases with multifocal/multicentric BC displaying intertumoral mismatch.


Asunto(s)
Neoplasias de la Mama/patología , Estadificación de Neoplasias/métodos , Neoplasias Primarias Secundarias/patología , Anciano , Neoplasias de la Mama/cirugía , Femenino , Humanos , Masculino , Persona de Mediana Edad , Estadificación de Neoplasias/normas , Neoplasias Primarias Secundarias/cirugía , Estudios Prospectivos
10.
Am J Blood Res ; 10(6): 311-319, 2020.
Artículo en Inglés | MEDLINE | ID: mdl-33489439

RESUMEN

Patients with chronic lymphocytic leukemia (CLL) treated with Ibrutinib often present hemorrhagic complications. Platelets dysfunction is well documented by aggregometry and flow cytometry, but the mechanisms by which Ibrutinib treatment influences the platelets status is yet to be evaluated. The aim of this study is to identify platelet membrane parameters in chronic lymphocytic leukemia (CLL) that could be altered by Ibrutinib administration. In this paper we propose a set of fluorescence measurements of the following parameters: membrane fluidity, resting membrane potential, and reactive oxygen species production of platelets suspensions obtained from CLL patients treated or not with Ibrutinib as markers for platelets status in this pathological situation. Platelets from CLL patients treated with Ibrutinib have higher membrane fluidity, lower resting membrane potential and higher level of reactive oxygen species production compared to the untreated CLL patients. These patients are also presenting higher membrane fluidity and lower resting membrane potential compared to healthy volunteers.

11.
Rom J Intern Med ; 57(4): 273-283, 2019 Dec 01.
Artículo en Inglés | MEDLINE | ID: mdl-31199777

RESUMEN

Immune thrombocytopenia is an autoimmune hematological disorder characterized by severely decreased platelet count of peripheral cause: platelet destruction via antiplatelet antibodies which may also affect marrow megakaryocytes. Patients may present in critical situations, with cutaneous and/or mucous bleeding and possibly life-threatening organ hemorrhages (cerebral, digestive, etc.) Therefore, rapid diagnosis and therapeutic intervention are mandatory. Corticotherapy represents the first treatment option, but as in any autoimmune disorder, there is a high risk of relapse. Second line therapy options include: intravenous immunoglobulins, thrombopoietin receptor agonists, rituximab or immunosuppression, but their benefit is usually temporary. Moreover, the disease generally affects young people who need repeated and prolonged treatment and hospitalization and therefore, it is preferred to choose a long term effect therapy. Splenectomy - removal of the site of platelet destruction - represents an effective and stable treatment, with 70-80% response rate and low complications incidence. A challenging situation is the association of ITP with pregnancy, which further increases the risk due to the immunodeficiency of pregnancy, major dangers of bleeding, vital risks for mother and fetus, potential risks of medication, necessity of prompt intervention in the setting of specific obstetrical situations - delivery, pregnancy loss, obstetrical complications, etc. We present an updated review of the current clinical and laboratory data, as well as a detailed analysis of the available therapeutic options with their benefits and risks, and also particular associations (pregnancy, relapsed and refractory disease, emergency treatment).


Asunto(s)
Púrpura Trombocitopénica Idiopática/terapia , Diagnóstico Diferencial , Tratamiento de Urgencia , Femenino , Humanos , Embarazo , Complicaciones del Embarazo , Púrpura Trombocitopénica Idiopática/diagnóstico , Púrpura Trombocitopénica Idiopática/etiología
12.
Medicina (Kaunas) ; 56(1)2019 Dec 27.
Artículo en Inglés | MEDLINE | ID: mdl-31892196

RESUMEN

Background and Objectives: Hematological malignancies are usually systemic diseases of life-threatening impact, and frequently require prompt and energetic therapeutic intervention. Due to systemic involvement, the role of surgery is generally limited to diagnostic approaches, and it is very rarely employed as a therapeutic modality. Splenectomy represents an exception to this paradigm, being used both as a diagnostic and tumor debulking procedure, notably in splenic lymphomas. Materials and Methods: We investigated the role of splenectomy in a single center prospective study of splenectomy outcome in patients with splenic involvement in the course of lymphoproliferative disorders. In the present study, we included all patients treated in our department for lymphoid malignancies over a period of six years, who underwent splenectomy as a diagnostic or debulking procedure after referral and workup, or had been referred to our department after first being splenectomized and diagnosed with splenic lymphoma. Patient characteristics and treatment outcome were investigated. Results: We enrolled 54 patients, with 34 (63%) splenectomized patients: 12 splenectomies (22.2%) for diagnostic purposes and 22 (40.7%) for treatment. Special attention was given to the 28 (51.85%) patients diagnosed with splenic marginal zone lymphoma (SMZL), a subtype with a clear therapeutic indication for splenectomy. Average age of patients was 57.5 (±13.1) years with a higher prevalence of feminine gender (66.67%). Age above 60 years old (p = 0.0295), ECOG (Eastern Cooperative Oncology Group) > 2 (p = 0.0402) and B-signs (p nonsignificant (NS)) were most frequently found in SMZL patients. Anemia, and notably autoimmune anemia, was more frequent in SMZL versus other small-cell lymphomas and also in splenectomized patients, as was leukocytosis and lymphocytosis. Treatment of patients with lymphoproliferative disorders consisted of chemotherapy and/or splenectomy. Most SMZL patients received chemotherapy as first line treatment (61.5%) and had only partial response (57.7%). Second treatment line was splenectomy in 80% of patients who required treatment, followed by a 60% rate of complete response (CR). Splenectomy offered a higher complete response rate (twice as high than in non-splenectomized, regardless of histology type, p = NS), followed by a survival advantage (Overall Survival (OS)~64 versus 59 months, p = NS). Particularly, SMZL patients had a 4.8 times higher rate of CR than other non-Hodgkin lymphoma (NHL) patients (p = 0.04), a longer progression free survival (73 months vs. 31 months for other small-cell NHLs p = NS) and a 1.5fold lower death rate (p = NS). The procedure was rather safe, with a 38.5% frequency of adverse reactions, mostly minor and manageable. Conclusions: Our data suggest that splenectomy is an effective and safe therapeutic option in patients with lymphoid malignancies and splenic involvement, particularly splenic marginal zone lymphoma.


Asunto(s)
Trastornos Linfoproliferativos/complicaciones , Esplenectomía/efectos adversos , Adulto , Anciano , Femenino , Humanos , Trastornos Linfoproliferativos/epidemiología , Masculino , Persona de Mediana Edad , Estudios Prospectivos , Rumanía/epidemiología , Esplenectomía/métodos , Resultado del Tratamiento
13.
Onco Targets Ther ; 11: 6271-6276, 2018.
Artículo en Inglés | MEDLINE | ID: mdl-30310290

RESUMEN

POEMS syndrome (acronym consisting of: polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes) is an uncommon disorder associated with an underlying plasma cell dyscrasia. There is no single specific test for POEMS, and due to its rarity and heterogeneity, patients are often mis- or underdiagnosed. Castleman disease (CD) is a rare lymphoproliferative disorder, closely related to POEMS syndrome; ~11%-30% of POEMS patients are associated with concomitant CD. In contrast to frequently published reports on vascular events in POEMS syndrome affecting coronary arteries or lower limbs, cases of cerebrovascular events are rarely mentioned in literature. We hereby report a patient with POEMS syndrome accompanied by CD who presented recurrent strokes and splenic infarction.

14.
Case Rep Hematol ; 2018: 1791627, 2018.
Artículo en Inglés | MEDLINE | ID: mdl-29785310

RESUMEN

Kikuchi-Fujimoto disease (KFD) or histiocytic necrotizing lymphadenitis is a rare disease that is frequently underdiagnosed due to clinical features that are similar to those of non-Hodgkin lymphomas, systemic lupus erythematosus (SLE), or infectious reactive lymphadenopathy. An excisional biopsy is required. We report a young Caucasian female diagnosed with KFD with skin lesions, complicating with SLE. The clinical course, laboratory, and CT findings are described, as are histopathologic features, for a better recognition of this rare disorder in clinical practice.

15.
Blood Coagul Fibrinolysis ; 26(7): 830-3, 2015 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-26196190

RESUMEN

Essential thrombocytemia--a classic myeloproliferative neoplasm characterized by persistent thrombocytosis--may associate both thrombotic and hemorrhagic events, as well as platelet dysfunction. Of all myeloproliferative neoplasms, essential thrombocytemia is more likely to be associated with pregnancy, because of a higher comparative incidence in younger patients. This association significantly increases the risk of pregnancy loss and of various pregnancy complications. We present a case of early severe preeclampsia with a critical and unusual evolution and life-threatening complications.


Asunto(s)
Plaquetas/metabolismo , Preeclampsia/etiología , Trombocitemia Esencial/complicaciones , Adulto , Plaquetas/citología , Femenino , Humanos , Preeclampsia/fisiopatología , Embarazo
16.
Blood Coagul Fibrinolysis ; 25(2): 142-50, 2014 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-24346354

RESUMEN

Patients with chronic myeloproliferative leukemia (CML) have frequent haemorrhage and/or thrombosis in their medical history. The mechanisms of these major and life-threatening complications remain unclear. Membrane organization influences many of the unique cellular functions and is strongly correlated, among other factors, to the membrane lipid composition; it may be evaluated by following up the membrane fluidity and aggregation properties of the platelet. In this study, we evaluated the platelet aggregation, the expression of platelet surface receptors, the membrane fluidity (as evaluated by fluorescence anisotropy) and its correlation to reactive oxygen species (ROS) production, in patients with chronic myeloid leukaemia (CML). It was found that the patients in accelerated and blastic phase of CML present an altered platelet aggregation response to all reagents except for ristocetin as compared with chronic phase group, which shows only epinephrine-altered response. We also found that BCR/ABL transcript leads to higher levels of ROS in accelerated and blastic CML phases. Patients without molecular remission have lower platelet membrane fluidity. We obtained a positive correlation between ROS level and membrane fluorescence anisotropy changes. The CD41 expression was decreased in CML patients and P selectin expression was found to be higher in these patients than in healthy volunteers. Platelets of CML patients have altered aggregation parameters in accelerated and blastic phases, in which BCR/ABL transcript level is increased. The increased level of ROS in CML patients without molecular remission is associated with a decrease in fluidity of platelet membrane and expression of CD41/CD61 receptors. These findings may contribute to understanding the mechanism of the altered platelet response reported in CML patients.


Asunto(s)
Plaquetas/patología , Leucemia Mielógena Crónica BCR-ABL Positiva/sangre , Anciano , Plaquetas/metabolismo , Femenino , Humanos , Leucemia Mielógena Crónica BCR-ABL Positiva/patología , Masculino , Persona de Mediana Edad , Estadificación de Neoplasias , Agregación Plaquetaria/fisiología , Pronóstico , Estudios Prospectivos , Especies Reactivas de Oxígeno/metabolismo
17.
Maedica (Bucur) ; 8(2): 182-8, 2013 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-24371483

RESUMEN

ABSTRACT: The incidence of ITP during pregnancy is low. When ITP is suspected it is necessary to perform an extended set of clinical and biological investigations in order to determine the etiology of thrombocytopenia, as the diagnosis of ITP is a process of exclusion, because there is no sensitive and specific diagnostic test so far. The treatment for ITP during pregnancy represents a challenge, being necessary in the cases selected according to the obstetrical indications, to the degree of maternal thrombocytopenia and to the extent of the hemorrhagic syndrome, as well as according to the adverse reactions of the treatment on the mother and fetus.

18.
Rom J Morphol Embryol ; 53(3): 645-50, 2012.
Artículo en Inglés | MEDLINE | ID: mdl-22990561

RESUMEN

"Refractory anemia with ring sideroblasts and thrombocytosis" (RARS-T) is a rare disease, a provisional entity, with a controversial status in the 2008 revised WHO classification. Even at present time, RARS-T is a matter of debate whether it is a distinct clinicopathological entity or more likely a constellation of clinical and pathological features of two well-defined myeloid neoplasms, myelodysplastic syndrome and myeloproliferative neoplasm. Perhaps none of the clonal disorders illustrates better the challenges presented by the current classification of myeloid neoplasms, than this clinical entity with overlapping features of both refractory anemia with ring sideroblasts and essential thrombocythemia. The purpose of this study is to present the evolution of such a case, with difficulties in establishing not only the correct diagnosis, but also the appropriate therapeutic approach. For this reported case, we present documented details regarding persistent thrombocytosis, slightly increased number of leukocytes and analysis of Janus kinase 2 (JAK2) genes that revealed a V617F mutation, confirming the presence of an underlying myeloproliferative neoplasm, followed later in the evolution by occurrence of myelodysplastic features as ring sideroblasts. This case might interest pathologists, but especially clinicians, for at least two reasons: the rarity of this disease and the lack of data on prognosis of these patients, probably because of relatively recent established diagnosis criteria and existence of few studies with small number of patients. The third interesting aspect for practitioners would be the absence of consensus on optimal clinical treatment for this disorder, because there are few cases that meet the rigorous diagnostic criteria.


Asunto(s)
Anemia Refractaria/patología , Anemia Sideroblástica/patología , Trombocitosis/patología , Anciano , Anemia Refractaria/sangre , Anemia Sideroblástica/sangre , Humanos , Masculino , Trombocitosis/sangre
19.
J Matern Fetal Neonatal Med ; 25(9): 1622-6, 2012 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-22220678

RESUMEN

INTRODUCTION: Thrombocytopenia affects about 10% of all pregnancies. Preeclampsia/HELLP syndrome induced thrombocytopenia may associate perinatal morbidity, preterm delivery, or low-birth-weight newborns. OBJECTIVE: To assess perinatal outcome and complications of pregnancy in women presenting with thrombocytopenia. METHODS: We retrospectively analyzed 936 consecutive pregnant women admitted during a 6-month period. RESULTS: Incidence of thrombocytopenia in pregnancy was 11.11% (104/936). Thrombocytopenia represented a risk factor for premature delivery - highest risk for severe thrombocytopenia (RR=8.69, p<0.01). Thrombocytopenic preeclampsia or HELLP syndrome associated the highest rates of prematurity (RR=7.97, p=0.00, respectively 12.32). Thrombocytopenia also represented a risk factor for low-birth-weight newborns, especially severe thrombocytopenia - 2047.50 ± 938.98 g (p=0.02) versus 3224.86 ± 496.00 g in controls. Again, thrombocytopenic preeclampsia was significantly associated with low-birth-weight newborns (RR=11.94, p=0.00), with medium weight of 2462.05 ± 794.54 g versus 2932.37 ± 708.91 g in thrombocytopenic pregnancies, respectively 3224.86 ± 496.00 g (p=0.00) in normal pregnancies. CONCLUSIONS: Thrombocytopenia in pregnancy was associated with perinatal morbidity, with the strongest association for preeclampsia and HELLP syndrome - for both prematurity and low-birth-weight: the lower the platelet count, the higher the risks for the fetus/newborn. Therefore, we strongly recommend close surveillance of thrombocytopenic mothers and their babies, in order to establish the etiology and the best moment for intervention.


Asunto(s)
Complicaciones Hematológicas del Embarazo/epidemiología , Resultado del Embarazo/epidemiología , Trombocitopenia/epidemiología , Adolescente , Adulto , Estudios de Casos y Controles , Femenino , Síndrome HELLP/epidemiología , Síndrome HELLP/mortalidad , Humanos , Recién Nacido , Enfermedades del Prematuro/epidemiología , Enfermedades del Prematuro/mortalidad , Morbilidad , Trabajo de Parto Prematuro/epidemiología , Trabajo de Parto Prematuro/mortalidad , Mortalidad Perinatal , Preeclampsia/epidemiología , Preeclampsia/mortalidad , Embarazo , Complicaciones Hematológicas del Embarazo/mortalidad , Estudios Retrospectivos , Trombocitopenia/mortalidad , Adulto Joven
20.
Maedica (Bucur) ; 7(4): 339-43, 2012 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-23483840

RESUMEN

The incidence of venous thromboembolism is significantly increased during pregnancy, recurrent venous thromboembolism being a serious complication because it is potentially life-threatening. According to recent ACCP guidelines, women with "high-risk" thrombophilias (e.g., homozygosity for factor V Leiden) who had a single prior episode of VTE treated with oral anticoagulants, should receive LMWH or UFH during pregnancy and puerperium, followed by resumption of long-term anticoagulants postpartum.We present the case of a young woman with a history of severe deep vein thrombosis of the inferior vena cava, occurring during oral contraceptive use. Subsequent investigation revealed homozygosity for Leiden mutation. She was treated with enoxaparin throughout gestation and 6 weeks postpartum and no complications appeared.

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