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2.
Haematologica ; 89(6): 704-9, 2004 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-15194538

RESUMO

BACKGROUND AND OBJECTIVES: Inherited factor VII (FVII) deficiency is a rare bleeding disorder characterized by a poor relationship between reported FVII clotting activity (FVII:C) and bleeding tendency. Our study was aimed at defining biological parameters that are possibly predictive for bleeding risk in this condition. DESIGN AND METHODS: Forty-two FVII-deficient patients (FVII:C <30%) were classified into two opposite clinical groups defined as severe and non-or-mild bleeders. For each patient, plasma samples were collected and then investigated for FVII:C (using a sensitive method and human recombinant thromboplastin as the reagent), FVII antigen, activated FVII coagulant activity (FVIIa:C) and the free-form of tissue factor pathway inhibitor. RESULTS: None of these tests could be used as highly accurate predictors of bleeding. Nevertheless, both FVII:C and FVIIa:C differed significantly between the two clinical groups. Using ROC-curve analysis, two critical values of 8% and 3mIU/mL for FVII:C and FVIIa:C, respectively, could be proposed to discriminate between severe bleeders and non-or-mild bleeders. INTERPRETATION AND CONCLUSIONS: A highly accurate diagnostic test for predicting bleeding tendency in inherited FVII deficiency still eludes definition, highlighting the fact that factors other than FVII itself interfere with the expression of bleeding phenotypes in this condition. Nevertheless, potential critical values using sensitive FVII:C and FVIIa:C methods may be useful in clinical laboratories for FVII-deficient patients. Those patients with FVII:C levels higher than 8% FVII:C or FVIIa:C higher than 3 mIU/mL, with no other hemostatic defect, seem to have a minimal risk of severe bleeding. Extended clinical studies are needed to support these findings.


Assuntos
Deficiência do Fator VII/diagnóstico , Fator VII/análise , Adolescente , Adulto , Transtornos Herdados da Coagulação Sanguínea , Criança , Pré-Escolar , Deficiência do Fator VII/sangue , Feminino , Hemorragia/sangue , Humanos , Masculino , Pessoa de Meia-Idade , Valor Preditivo dos Testes , Risco , Índice de Gravidade de Doença
3.
Br J Haematol ; 92(1): 244-6, 1996 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-8562404

RESUMO

We studied activated protein C sensitivity ratio (APC-SR), factors V and VIII activity and von Willebrand antigen in control women, women using oral contraceptives, and pregnant women at delivery. The mean APC-SR of 2.4 in pregnant women was significantly lower than the mean APC-SR value of 3.5 for both the other groups and 45% of pregnant women had a ratio below the 5th percentile of the control group. None of them carried the R506-->Q mutation. This decreased ratio at delivery appeared to be connected, at least in part, with increased VIII activity. Thus, APC-SR at delivery should not be used to detect APC resistance.


Assuntos
Anticoncepcionais Orais/farmacologia , Parto Obstétrico , Gravidez/sangue , Proteína C/fisiologia , Adulto , Coagulação Sanguínea , Fator V/metabolismo , Fator VIII/metabolismo , Feminino , Humanos , Fator de von Willebrand/metabolismo
4.
Nouv Rev Fr Hematol (1978) ; 32(6): 409-14, 1990.
Artigo em Inglês | MEDLINE | ID: mdl-2101874

RESUMO

Splenic B cell lymphoma with circulating villous lymphocytes (SLVL) is a lymphoproliferative disorder characterized by the presence in the peripheral blood of atypical B-lymphocytes with hairy appearance. Although the clinical features with massive splenomegaly, absence of peripheral lymphadenopathy and blood cytopenia may mimic hairy cell leukemia (HCL), precise analysis of the morphologic and immunologic features allow differential diagnosis between these two entities. Bone marrow and spleen histology resemble the pattern in chronic lymphocytic leukemia (CLL). We studied 8 patients with this entity illustrating the difficulty of diagnosis between SLVL and HCL.


Assuntos
Linfócitos B/ultraestrutura , Leucemia de Células Pilosas/diagnóstico , Linfoma de Células B/diagnóstico , Células-Tronco Neoplásicas/ultraestrutura , Neoplasias Esplênicas/diagnóstico , Idoso , Idoso de 80 Anos ou mais , Anticorpos Antineoplásicos/sangue , Antígenos de Diferenciação de Linfócitos B/sangue , Antígenos de Neoplasias/sangue , Biomarcadores Tumorais , Medula Óssea/patologia , Diagnóstico Diferencial , Feminino , Humanos , Linfoma de Células B/sangue , Linfoma de Células B/patologia , Masculino , Pessoa de Meia-Idade , Receptores de Antígenos de Linfócitos B/análise , Baço/patologia , Neoplasias Esplênicas/sangue , Neoplasias Esplênicas/patologia , Esplenomegalia/diagnóstico , Esplenomegalia/etiologia
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