RESUMO
Disseminated intravascular coagulopathy (DIC) occurred in a patient with hemolytic anemia and anasarca. Skin and muscle biopsy showed intravascular lymphomatosis (malignant angioendotheliomatosis). Combination chemotherapy resulted in resolution of the DIC and anasarca. After an unmaintained 8-month clinical remission, the patient had central nervous system relapse and died. Malignant angioendotheliomatosis is a rare disorder that should be considered among the occult causes of DIC.
Assuntos
Coagulação Intravascular Disseminada/etiologia , Hemangioendotelioma/diagnóstico , Linfoma Imunoblástico de Células Grandes/diagnóstico , Doenças Vasculares/diagnóstico , Idoso , Antígenos CD/análise , Vasos Sanguíneos/imunologia , Vasos Sanguíneos/patologia , Edema/etiologia , Feminino , Hemangioendotelioma/complicações , Humanos , Técnicas Imunoenzimáticas , Linfoma Imunoblástico de Células Grandes/complicações , Doenças Vasculares/complicaçõesRESUMO
A successfully managed case of posterior fossa subdural hematoma occurring in a hemophiliac is reported, and the rarity of this lesion is mentioned. The management of the hemophiliac who is a victim of craniocerebral trauma is discussed, with emphasis on the use of computerized tomographic scanning to obtain rapid diagnosis. The importance of prophylactic Factor VIII replacement is emphasized, and appropriate methods of Factor VIII administration are outlined.
Assuntos
Lesões Encefálicas/complicações , Hematoma Subdural/complicações , Hemofilia A/complicações , Adolescente , Lesões Encefálicas/diagnóstico por imagem , Lesões Encefálicas/terapia , Craniotomia , Fator VIII/uso terapêutico , Feminino , Hematoma Subdural/diagnóstico por imagem , Hematoma Subdural/terapia , Hemofilia A/tratamento farmacológico , Humanos , Masculino , Linhagem , Tomografia Computadorizada por Raios XRESUMO
The syndrome thalassemia intermedia can be the clinical expression of heterozygosity for different tyes of thalassemia, beta-thalassemia and hereditary persistence of fetal hemoglobin, beta-thalassemia and Hb-Lepore, and in blacks it may even represent a true beta-thalassemia homozygote. This report describes thalassemia intermedia in a white male due to beta-thalassemia and an unstable hemoglobin. Chain-synthesis studies showed an excess of alpha-chain production over beta-chain production in the propositus and his mother but balanced chain synthesis in the clinically normal father, who is heterozygous for the unstable hemoglobin. The unstable hemoglobin was found to be beta14 (A11) Leu leads to Pro, which has previously been described in a clinically normal African woman, and named Hb-Saki. This hemoglobin is not distinguishable from Hb-A on routine electrophoresis at alkaline or acid pH and tests for unstable hemoglobins are necessary for its detection. The increasing list of such hemoglobin variants and previous cases of heterozygosity for beta-thalassemia and unstable hemoglobins are reviewed.
