Generalized edema and ecchymoses were present. Evidence of DIC included bilateral serosanguineous pleural effusions and a similar peritoneal effusion, along with extensive pelvic soft-tissue hemorrhage extending from catheter sites in the thigh. Also, a small, thin interhemispheric subdural hemorrhage was grossly noted on the brain. The heart weighed 425 g (ULN about 400 g) and the left ventricular wall and interventricular septum were 15 mm thick (ULN about 13 mm). Lungs were moderately congested with thickened bronchial basement membranes and occasional eosinophils, consistent with mild asthma. The 1400-g liver was congested with developing centrilobular necrosis and many sickled red cells. The kidneys showed patchy coagulative necrosis, vacuolization and hydropic change of tubular epithelium, and sickled red cells. Mild lymphocytic thyroiditis was present. Adrenal glands were normal. The spleen was enlarged at 250 g and showed red pulp congestion and focal packing of sickled cells (Fig. 1). The brain had no other abnormalities on careful sectioning; however, on microscopic examination, it showed focal ischemic neuronal changes and rare perivascular pigmented macrophages and calcification suggesting possible sickling bouts in the past. Skeletal muscle showed evidence of the acute rhabdomyolysis that in our opinion was ischemic, from exertional sickling, and led to the collapse of the player: focal vacuolization and disruption of the normal banding pattern, contraction bands, and decreased to absent staining for myoglobin and desmin in the cytoplasm of muscle cells, along with loss of staining of cell membranes for dystrophin (Fig. 2). Antemortem toxicologic testing and postmortem testing on pretransfusion antemortem samples were both negative. The heart was examined also by an expert in cardiopathology and was judged as normal, with physiologic cardiomegaly ("athlete's heart"), normal atrioventricular and sinoatrial nodes with hypertrophic myocytes, contraction band necrosis, and vacuolization of some myocytes. Most of the sickling was judged to be antemortem, not agonal, because 1) it was extensive despite considerable transfusion with normal red cells that would never sickle, and 2) the spleen was enlarged with focal packing with sickled red cells. Hemoglobin electrophoresis showed SCT with 55.3% hemoglobin A, 41.3% hemoglobin S, and 3.4% hemoglobin A2, whereas a normal electrophoresis would be about 97% hemoglobin A, 2.5% hemoglobin A2, and no hemoglobin S. His newborn screening record showed SCT. The cause of death was acute exertional rhabdomyolysis associated with SCT.
Focal packing of sickled red blood cells in the spleen. A, Hematoxylin and eosin (H&E), low magnification. B, H&E, higher magnification.
Skeletal muscle with microscopic features of acute rhabdomyolysis. A, Disruption of normal banding pattern and contraction bands, H&E. B, Decreased to absent cytoplasmic immunostaining for desmin. C, Decreased to absent cytoplasmic immunostaining for myoglobin. D, Loss of cell membrane immunostaining for dystrophin.
Med Sci Sports Exerc. 2010;42(1):3-7. © 2010
Cite this: Sickle Cell Trait and Fatal Rhabdomyolysis in Football Training: A Case Study - Medscape - Jan 01, 2010.