RESUMEN
This study presents a series of 16 carriers of hemoglobin S (HbS) who died during various circumstances. Many of the cases were associated with mild to moderate exertion. The onset and/or duration of symptoms varied from a few minutes to several hours with many displaying a prolonged lucid interval with stable vital signs. Despite seeking medical treatment, sickle cell trait-related micro-occlusive crisis was never considered in the differential diagnosis. Several cases were associated with sudden death. In those deaths which were delayed, high anion gap and uncompensated metabolic acidosis were typical and were not heat related. Also characteristic were large increases in creatine kinase, alanine aminotransferase, and aspartate aminotransferase along with myoglobinemia. Although the antemortem diagnosis of rhabdomyolysis was made, the underlying cause was never deduced by the clinicians. The sickling found at autopsy is not always a postmortem artifact, and in the right circumstances can be diagnostic.
Asunto(s)
Rasgo Drepanocítico/complicaciones , Acidosis/etiología , Adolescente , Adulto , Alanina Transaminasa/sangre , Aspartato Aminotransferasas/sangre , Niño , Creatina Quinasa/sangre , Muerte Súbita/etiología , Coagulación Intravascular Diseminada/etiología , Eritrocitos/patología , Femenino , Patologia Forense , Humanos , Masculino , Mioglobina/sangre , Esfuerzo Físico , Rabdomiólisis/diagnóstico , Rabdomiólisis/etiología , Rasgo Drepanocítico/diagnóstico , Estrés Fisiológico , Trastornos Relacionados con Sustancias , Adulto JovenRESUMEN
Hemoglobin electrophoresis is the method most commonly used to diagnose sickle cell trait (SCT) at autopsy. However, in some cases, this accepted technique is unable to be used due to either insufficient sampling, sample degradation, or lack of forethought; histology samples and/or gross tissue are not subject to these sampling errors and are routinely taken during autopsies. In this study, we attempted to determine whether one can reliably diagnose SCT using histology only. Histology sections of commonly sampled tissues (primarily heart, lung, and liver) from 9 decedents with SCT, 3 decedents with hemoglobin SC disease, and 18 control cases were examined in a blinded fashion as single slides and then as slide sets. When evaluating slide sets, the reviewers were able to identify the cases with SCT (sensitivity = 95%, specificity = 100%). Such samples could be used to diagnose SCT even decades after the original death certification and long after samples necessary for other techniques have degraded or been discarded.