RESUMEN
Between July 1992 and July 2001, 81 patients with de novo adult acute lymphoblastic leukemia (ALL) treated according to the German Multicenter Study Group for Adult ALL (GMALL) 01/81 protocol were evaluated in order to analyze the effect of aberrant myeloid antigen expression on prognosis. We observed myeloid antigen aberrant expression in 21 of the adult ALL cases. We did not observe any effect of aberrant myeloid antigen expression on the time to achieve remission, relapse rate, and death rate. After 5 years of follow-up, cumulative disease-free survival of myeloid antigen (My) (+) and My (-) adult ALL patients was 67% and 43%, respectively. These data were not found to be statistically significant (P=0.29), but we did find a statistically significant difference in overall survivals between these two groups (85% vs 50%) (P=0.05). Twenty-nine patients died and the remaining 52 patients were followed for a median of 31 months. We could not find any special effect of the known prognostic factors on prediction of relapse in multivariate analysis. However, myeloid antigen expression was the most significant factor, which affected long-term survival in our patients (P=0.01). These data indicate that myeloid antigen expression is useful for predicting a favorable outcome of adult patients with ALL.
Asunto(s)
Antígenos de Superficie/metabolismo , Células Mieloides/inmunología , Leucemia-Linfoma Linfoblástico de Células Precursoras/patología , Adolescente , Adulto , Análisis de Varianza , Biomarcadores/análisis , Femenino , Estudios de Seguimiento , Humanos , Inmunofenotipificación , Masculino , Persona de Mediana Edad , Leucemia-Linfoma Linfoblástico de Células Precursoras/diagnóstico , Leucemia-Linfoma Linfoblástico de Células Precursoras/mortalidad , Pronóstico , Factores de Riesgo , Análisis de Supervivencia , Resultado del TratamientoRESUMEN
UNLABELLED: The purpose of this study is to evaluate the ocular findings in patients with the primary antiphospholipid syndrome (APS). PATIENTS AND METHODS: Twenty-two patients (44 eyes) with primary APS (17 women, 5 men) were examined. All patients were younger than 50 years (median age; 37.5 years). In 18 patients, fundus flourescein angiography was performed in addition to the ophthalmologic examination. RESULTS: Sixteen patients (72.7%) described visual symptoms. Anterior segment was normal in 19 patients (86.4%). Posterior segment abnormalities were observed in 15 patients (68.2%). Venous dilatation and tortuosity were the most common ocular findings. Retinal vascular occlusive disease was detected in 5 patients (22.7%). Flourescein angiography abnormalities were noted in 14 of the 18 patients (77.8%). The most common angiographic finding was pigment epithelial window defects. CONCLUSIONS: Our results indicate that posterior eye segment involvement is relatively common in the primary APS. It also seems that the screening for APS is important in young patients with retinal vascular occlusion, especially in those without conventional risk factors.
Asunto(s)
Síndrome Antifosfolípido/complicaciones , Epitelio Pigmentado Ocular/patología , Oclusión de la Arteria Retiniana/etiología , Oclusión de la Vena Retiniana/etiología , Adulto , Anticuerpos Anticardiolipina/análisis , Anticuerpos Antinucleares/análisis , Síndrome Antifosfolípido/diagnóstico , Síndrome Antifosfolípido/genética , Síndrome Antifosfolípido/inmunología , ADN/genética , ADN/inmunología , Diagnóstico Diferencial , Ensayo de Inmunoadsorción Enzimática , Factor V/genética , Femenino , Angiografía con Fluoresceína , Fondo de Ojo , Humanos , Masculino , Persona de Mediana Edad , Mutación Puntual , Oclusión de la Arteria Retiniana/diagnóstico , Oclusión de la Arteria Retiniana/genética , Oclusión de la Arteria Retiniana/inmunología , Oclusión de la Vena Retiniana/diagnóstico , Oclusión de la Vena Retiniana/genética , Oclusión de la Vena Retiniana/inmunología , Estudios RetrospectivosRESUMEN
Glanzmann's thrombasthenia was reported and described as a bleeding diathesis seen in children and characterized by diminished clot retraction. It is an autosomal recessive bleeding disorder. The disease is marked by frequent mucocutaneous hemorrhages either due to defective function of the platelets or lack of fibrinogen binding membrane receptor glycoproteins IIb/IIIa which are located on the surface of the platelets. Case reports on 3 siblings, a girl of 11, and 2 boys of 12 and 16 years old with Glanzmann's thrombasthenia are reviewed. The major complaint of the patients was gingival bleeding. Periodontal treatment was performed under platelet transfusion and proper oral hygiene instruction was given. The patients were followed for 6 months and no periodontal complications developed during this time. Proper periodontal care for such patients is essential both for local and systemic health.
Asunto(s)
Hemorragia Gingival/terapia , Gingivitis/terapia , Trombastenia/genética , Adolescente , Plaquetas/fisiología , Niño , Femenino , Estudios de Seguimiento , Genes Recesivos , Humanos , Masculino , Higiene Bucal , Educación del Paciente como Asunto , Complejo GPIIb-IIIa de Glicoproteína Plaquetaria/fisiología , Transfusión de Plaquetas , Trombastenia/terapiaRESUMEN
Severe periodontitis in a patient with infantile genetic agranulocytosis (Kostmann syndrome) is presented. This rare syndrome is inherited as an autosomal recessive pattern and characterized by severe neutropenia. The periodontal status and treatment of the patient is described. It is concluded that periodontal therapy including scaling, root planning, soft tissue curettage, and the use of selected antimicrobial agents can be successful in this particular syndrome.