A 20-year-old recruit, resident of north coastal Andhra Pradesh, a known sickle cell belt of India, was brought to the emergency with alleged history of collapse and sudden loss of consciousness following a 2.2-kilometre organised run during routine training activities. There was history of non-recordable blood pressure and pulse following which the regimental medical officer had administered chest compressions. On evaluation, patient was found to be unresponsive, hyperventilating with no spontaneous eye opening and no response to pain. Pupils were bilaterally equal with a sluggish response to light. Pulse was 100/min, BP was 110/60 and respiratory rate was 42/min.
Systemic examination revealed normal cardiovascular and chest findings. An arterial blood gas analysis showed profound anion gap metabolic acidosis (pH 6.61, lactate 24.59, anion gap 30.4 mmol/L, HCO3 4 mmol/L, pCO2 41.4 mmHg, sPO2 94%). Random blood sugar was 246 mg/dL. An electrocardiogram (ECG) showed features of hyperkalemia. Revaluation in the intensive care unit showed a disoriented patient with tense tender abdomen and decreased bowel sounds. Despite fluid resuscitation, patient continued to be anuric. A plain roentgenogram of abdomen showed grossly dilated stomach with distended bowel loops. Contrast enhanced computed tomogram of abdomen showed acute cortical necrosis of the kidneys with features of paralytic ileus. A urgent bedside 2D echogram was normal. Investigations revealed normal Hb, elevated total leucocyte counts, deranged coagulation profile, hyperkalemia, markedly elevated liver enzymes, lactate dehydrogenase and creatine kinase(Table 1). Patient was managed with continuous renal replacement therapy. However, he desaturated, had a cardiac arrest and died 18 hours after admission.
On perusal of medical documents of the individual, it was found that in the past, there had been history of two similar episodes. The patient had presented with presyncope following organised battle preparedness efficiency test(BPET) as a part of military training. He had been hospitalised following one such episode during which tall T waves on ECG and hyperkalemia had been documented. However, his cardiac evaluation including electrocardiogram, Holter and treadmill test had been normal.
Post mortem Findings
An autopsy revealed the body of a young male with a normal frame. Fine adhesions were seen between the lung and the pleura on the left side. The lower lobes of both lungs were congested and oozed pink fluid. The peritoneal cavity contained minimal blood stained fluid. The small intestine showed marked congestion with two gangrenous segments measuring 15 cm and 7 cm(Fig. 1). Stomach showed hemorrhagic mucosal surface. Bilateral iliopsoas hematomas were seen and a large left subdural haemorrhage was seen on opening the cranial cavity. Spleen was enlarged measuring 350 g and showed congested cut surface. No splenic infarcts were seen. Cut surface of the brain parenchyma, heart, both kidneys, liver, adrenals, pancreas, urinary bladder and testes and were unremarkable on gross.
On microscopy, acute congestion and microvascular occlusion by sickled red cells was seen in the glomerular capillaries, hepatic sinusoids, splenic red pulp, brain,(Figs. 2a-d), pancreatic vasculature, adrenals, intestines, lymph nodes, and skeletal muscle. Pleural fibrosis was seen. Alveolar spaces of lower lobes showed congestion. Kidneys showed proximal tubular necrosis. Sections of skeletal muscle showed myonecrosis. There was no histopathologic evidence of any inflammatory or infectious disease.
Postmortem high performance liquid chromatography (HPLC) performed on deceased blood revealed SCT(HbS 38.5%, HbA 58.2%, HbA2 2.4% and 0.9% HbF). A similar pattern was seen in his father’s blood. Death was attributed to multiorgan damage associated with massive intravascular sickling due to exertion induced hypoxia related with extreme physical activity and in an undiagnosed case of SCT.