RESUMEN
Von Willebrand disease is an inherited disorder characterized by deficiency of von Willebrand factor, which contributes to platelet adhesion to the endothelium. Patients with coagulation disorders present a challenge at the time of surgery due to the high risk of presenting heavy bleeding within the procedure or postoperative hematomas. We present a case of a 56-year-old woman with Type 1 von Willebrand's disease who was scheduled for breast explantation with autologous reconstruction, due to the presence of long-standing breast implants. The case was satisfactorily managed by a multidisciplinary team formed by plastic surgery, hematology, and anesthesiology, individualizing the management for the patient's case, obtaining good results and a safe procedure.
RESUMEN
Resumen Introducción: Un espectro de enfermedad hemorrágica intracraneal se puede presentar con síntomas neurológicos focales transitorios; aunque las enfermedades congénitas del fibrinógeno son inusuales y rara vez se manifiestan de esta manera, a continuación se presenta un caso de microsangrados cerebrales por hipofibrinogenemia congénita con síntomas neurológicos focales transitorios. Presentación del caso: Paciente masculino de 29 años de edad con microsangrados cerebrales por hipofibrinogenemia congénita con síntomas neurológicos focales transitorios. Discusión: La hemorragia intracerebral de vasos pequeños es una causa de síntomas neurológicos focales transitorios. La prevalencia de microhemorragias cerebrales en la población adulta mayor es un problema altamente reconocido, sin embargo, la incidencia de estos microsangrados en personas más jóvenes es baja y desconocida. Las discrasias sanguíneas, como el trastorno hereditario del fibrinógeno, son una causa de anomalías hereditarias de la coagulación sanguínea, donde este tiene una función importante en el control del sangrado por agregación plaquetaria y en la cascada de coagulación. La ausencia de fibrinógeno normal conduce a una altercación en la hemostasia y, por tanto, a complicaciones hemorrágicas. Conclusiones: Los síntomas neurológicos focales transitorios pueden ser causados por una enfermedad vascular cerebral hemorrágica de vasos pequeños, donde las discrasias sanguíneas congénitas son una causa rara de este tipo de enfermedad cerebrovascular.
Abstract Introduction: Intracranial hemorrhagic disease can present with transient focal neurological symptoms. Congenital fibrinogen diseases are unusual and can rarely manifest in this way. Below we present a case of cerebral microbleeds due to congenital hypofibrinogenemia with transient focal neurological symptoms. Case presentation: This is a 29-year-old man with cerebral microbleeds due to congenital hypofibrino-genemia with transient focal neurological symptoms. Discussion: Cerebral microbleed and intracerebral hemorrhage is a cause of transient focal neurological symptoms. The prevalence of cerebral microbleeds in the elderly population is a highly recognized problem. However, the incidence of these microbleeds in younger people is low and unknown. Blood dyscrasias, such as inherited fibrinogen disorder, are a cause of inherited abnormalities of blood clotting. Fibrinogen has an important role in the control of bleeding due to platelet aggregation and is part of the coagulation cascade. The absence of normal fibrinogen induces alteration in platelet and coagolation hemostasis and, therefore, causes hemorrhagic complications. Conclusions: Transient focal neurological symptoms may be caused by small vessel hemorrhagic cere-brovascular disease. Congenital blood dyscrasias are a rare cause of this type of cerebrovascular disease.
RESUMEN
Pancytopenia is seen in late HIV infection; it is associated with medical complications and with decreased survival. We determined the prevalence of pancytopenia at baseline in a cohort of HIV-positive Hispanics living in Puerto Rico, and compared their socio-demographic, immunological and clinical characteristics. A total of 1202 patients enrolled between 2000 and 2010 were included. They were grouped according to pancytopenia status, defined by having: platelets <150,000 µL, white cell count <4000 µL, and hemoglobin <12 g/dL (women) or <13 g/dL (men). Differences were evaluated using Student's t-test, Chi-square test and Kaplan-Meier method. The prevalence of pancytopenia was 8.7%. Patients with pancytopenia had lower BMI and lower CD4 count, as well as higher HIV viral load and higher proportions of unemployment, clinical AIDS and antiretroviral treatment (ART) use (p < 0.05). One-year mortality rate was significantly higher in patients with pancytopenia (18.1% vs. 5.1%, p < 0.001). When stratifying for ART this association persisted for patients who did not receive ART (41.4% vs. 5.2%, p < 0.001), but it was not seen in patients who received treatment (9.2% vs. 5.6%, p = 0.196). Pancytopenia was associated with elements of advanced stages of HIV. ART could reduce the mortality of HIV-patients with pancytopenia to levels comparable to patients without the disorders.