A Dull Day At Work...?: clinical condition

Showing posts with label clinical condition. Show all posts

12 January 2026 (Monday) - T.A.M.O.F. (wassat then?)

No massive bleeding. No dramatic hypotension. But test results drifting in a direction that feels wrong: platelets falling, creatinine creeping, LDH elevated, hemoglobin sliding just enough to notice.

Organ dysfunction without a single unifying explanation…

Here’s a condition that is new to me. Thrombocytopenia-associated multiple organ failure (TAMOF)

Here’s a little write-up about it.

As I always say, a day when you learn nothing is a day wasted.

23 July 2025 (Wednesday) - Wiskott-Aldrich Syndrome

I saw a diagnosis today - Wiskott–Aldrich syndrome. I couldn’t remember that much about it... so I looked it up.

As always, people might sneer at it, but Wikipedia is a good place to start. There's a more detailed review here.

These days there's a genetic marker which is considered definitive for the condition

There never used to be genetic markers back in the day.

14 February 2025 (Friday) - ITP

The nice people at TouchHaematology sent me this link today. The latest thinking on the treatment of ITP.

This is why I need to do CPD. Back in the day the patients either had a massive dose of steroids, and if that didn’t work, out came the spleen. Both were rather effective; as I remember my mate’s mother was rather ill with ITP but soon recovered.

Treatment these days is a lot less like taking a sledgehammer to crack a nut.

5 September 2024 (Thursday) - TACO

I watched a little video about TACO today…

Transfusion-Associated Circulatory Overload (TACO) is defined as acute or worsening respiratory compromise and/or acute or worsening pulmonary oedema during or up to 12 hours of transfusion, with additional features including cardiovascular system changes not explained by the patient’s underlying medical condition.

It’s not rocket science – if you stuff anything into a system of fluids being pumped about, that pump is going to have to work harder.

There’s an interesting article about the matter here.

23 August 2024 (Friday) - Long Long Ago

As I peered into Facebook this morning there was something on one of the haematology-related pages that made me think. Someone was talking about LE cell preparations…

Compared to the technology of today, LE cell preparations were somewhat laughable.

Back in the day we would take ten millilitres of blood into a glass jar which contained several glass beads. We would then seal the jar with a tight cap and shake. I say “shake” – we would thrash the thing as vigorously as we could with all of our might. And at the point of exhaustion we would pass the jar to a colleague who would carry on thrashing the thing. And when they were worn out someone else would take over.

After fifteen minutes it would go into an incubator for half an hour or so. The contents would then be poured through a muslin cloth (which made a real mess and spilled blood everywhere) into a tube, gently spun, and a blood film prepared from the buffy coat.

We would then look under the microscope for neutrophils which had engulfed other cells – these were the LE cells. Seeing the cells was diagnostic of systemic lupus erythematosus, and not seeing them excluded the condition. In the three years that I did this procedure (whilst working in a long-since bulldozed hospital) we never had a positive result. Not one. And neither had anyone else with whom I spoke about them at day release college.

It would seem that lupus is on the rise. And has been for the last few decades Is this because the disease is really becoming more prevalent, or because nowadays there’s a much more involved diagnostic process which employs tests which are nowhere near so medieval.

Did we miss cases of lupus back in the day because they really were less common? Are we finding more these days because the condition is more prevalent. Or because the technology is better? I don’t know, but I can remember doing at least one LE cell preparation a week for three years and never getting a positive result. At the time I was only a trainee, and the frankly bullying atmosphere in which I worked actively discouraged comment, but surely someone would have thought there was something wrong in a test which was *always* negative.

Unless it really was always negative?

15 July 2024 (Monday) - Gaucher's Disease

#LearningMonday… continuing being a reactionary old fart, I hate hashtags. But I do like the Learning Monday idea. This one was a good one… which is the most unlikely diagnosis. Initially I plumped for PNH as it is a disease of older people.

But… Gaucher’s disease… what’s that?

There’s a Wikipedia entry on the condition here. I know that proper science sneers at Wikipedia, but as a starting point it is hard to beat. Apparently Gaucher’s disease is one in which glucocerebroside accumulates in cells and certain organs… one of the places it accumulates is in macrophages which have a distinctive appearance in bone marrow smears… and to complicate the issue there are also pseudo-Gaucher cells.

I learned something here…

27 May 2023 (Saturday) - Lysosomal Storage Diseases

Mott cells? In a seven year old?

No.

These are characteristic of lysosomal storage diseases; a group of over seventy rare inherited metabolic disorders that result from defects in lysosomal function. This came up in an article posted on one of the Facebook groups I follow.

But personally I found the entry on Wikipedia to be far more on my level…

23 May 2023 (Tuesday) - Pearson's Syndrome

According to Wikipedia “Pearson syndrome is a mitochondrial disease characterized by sideroblastic anemia and exocrine pancreas dysfunction. Other clinical features are failure to thrive, pancreatic fibrosis with insulin-dependent diabetes and exocrine pancreatic deficiency, muscle and neurologic impairment, and, frequently, early death. It is usually fatal in infancy. The few patients who survive into adulthood often develop symptoms of Kearns–Sayre syndrome. It is caused by a deletion in mitochondrial DNA. Pearson syndrome is very rare, less than a hundred cases have been reported in medical literature worldwide”.

That would be why I hadn’t heard of the condition before…

3 January 2023 (Tuesday) - DRESS

We had an interesting case on the lead-up to Christmas. A patient with increasing eosinophilia…. What was that all about?

The patient had a rash and was quite unwell too – we don’t see that in the lab. Eventually a diagnosis was made.

Drug rash with eosinophilia and systemic symptoms (DRESS) syndrome is a severe reaction to a drug after a prolonged latency period.

There can be a variety of clinical symptoms including fever rash lymphadenopathy eosinophilia, and a wide range of mild-to-severe systemic presentations.

That’s rather vague isn’t it? But DRESS illustrates an interesting point. The introduction of new drugs has often led to a wide range of systemic and cutaneous reactions.

When hydantoin was introduced in the 1940s, reports of lymphadenopathy soon followed. The lymph node biopsies in these cases demonstrated a lymphomatous appearance, which was termed drug-induced pseudolymphoma.

Another drug, carbamazepine, was found to induce a reaction consisting of a rash, fever, and lymphadenopathy.

Such a reaction was termed anticonvulsant hypersensitivity syndrome.

Shortly thereafter, multiple drugs with a similar range of manifestations were observed. Hence the term drug-induced hypersensitivity also known as hypersensitivity syndrome was coined.

The term DRESS was eventually introduced, and looks to be possibly superceded by the term drug-induced delayed multiorgan hypersensitivity syndrome.

All of these different terms describe what is effectively the same condition…

Here’s some articles on DRESS:

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3718748/

https://www.aaaai.org/conditions-treatments/related-conditions/dress

https://www.medsafe.govt.nz/profs/puarticles/dresssyndromejune2011.htm

https://www.karger.com/Article/Fulltext/509712