{"id":1552,"date":"2023-11-19T21:34:45","date_gmt":"2023-11-20T02:34:45","guid":{"rendered":"http:\/\/wordpress.forensicpath.us\/?p=1552"},"modified":"2023-11-19T21:39:12","modified_gmt":"2023-11-20T02:39:12","slug":"pathology-cases-exertional-sickle-trait-death","status":"publish","type":"post","link":"https:\/\/wordpress.forensicpath.us\/index.php\/2023\/11\/19\/pathology-cases-exertional-sickle-trait-death\/","title":{"rendered":"Pathology cases — Exertional Sickle Trait Death"},"content":{"rendered":"

Here is a case of a young man who collapsed after sprinting.\u00a0 At autopsy, he was noted to have numerous sickled cells, sometimes clumping and filling vessels.\u00a0 Further workup revealed sickle trait.<\/p>\n

Because the gene that makes sickle cell hemoglobin (Hemoglobin S) is autosomal, there are two copies in each cell.\u00a0 \u00a0If both copies have the HgbS mutation, then the person has “sickle cell disease.”\u00a0 If the person has one normal and one HgbS, he or she has “sickle cell trait.”\u00a0 Sickle cell disease is life-threatening, painful, and debilitating.\u00a0 Sickle trait is largely asymptomatic.\u00a0 In fact, until the 1980s, it was believed that sickle trait was completely benign.\u00a0 Then folk noticed that people with sickle trait tended to collapse and die under very specific circumstances — when they exercise past the point of exhaustion.\u00a0 Thus, there was greatly increased mortality among people with sickle trait going through military training, athletic training, and when running from the police.<\/p>\n

The problem is that sickle cells have a roughly normal shape when there’s lots of oxygen in the blood.\u00a0 When oxygen gets low, the cells collapse into shapes that easily clump, resulting in blockage of blood vessels and damage to tissues downstream of the blockage.\u00a0 Young cells exhibit “reversible” sickling — the collapse when oxygen starved, and expand into a more normal shape when oxygen is replenished.\u00a0 Older sickle cells are “irreversibly” sickled, and will not expand when exposed to oxygen.<\/p>\n

For a more complete description, I discuss it a bit more in my evaluation of the George Floyd case<\/a> .<\/p>\n

In this case, the decedent was a young man who collapsed after exertion and went into multiorgan failure — a common presentation.\u00a0 At autopsy, he demonstrated minimal pathology, but had abundant sickle cells in many organs.\u00a0 Here’s an example from the kidney.<\/p>\n

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Here’s a side-by-side.\u00a0 On the left is a section from the decedent’s kidney showing thin, spiked red cells.\u00a0 On the right is a section from a decedent without sickle trait who died of pneumonia.\u00a0 Notice how round and plump the red cells are on the right.\u00a0 This is a large image and is scaled down automatically by WordPress for this web page.\u00a0 Right click on the image and open it in a new tab to see higher resolution.<\/p>\n

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Here’s a picture of a renal glomerulus.\u00a0 You can see the sickled cells stacking up and blocking vessels.\u00a0 This is a trichrome stain.<\/p>\n

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One of the conundrums in postmortem evaluation of these cases is interpreting the presence of sickled cells in tissues.\u00a0 Some reports say that it’s not meaningful because the cells sickle in low oxygen environments, such as being dead.\u00a0 Thus, the presence of sickled cells does not itself imply a sickle crisis.\u00a0 On the other hand, other studies indicate that sickled cells can reverse upon tissue processing or drawing blood because the cells are often exposed to air in processing, and thus reversibly sickled cells can plump up. Thus, the absence of sickled cells does not rule it out.\u00a0 The diagnosis of exertional sickle trait death in the absence of obvious ischemic changes in affected organs is based primarily on history in the context of a diagnosis of sickle trait.<\/p>\n

As always, free for use with or without attribution, though attribution is appreciated.\u00a0 If you need higher resolution copies, contact me.<\/p>\n

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 <\/p>\n","protected":false},"excerpt":{"rendered":"

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