BMJ  2007;334:73 (13 January), doi:10.1136/bmj.39091.590093.47

Observations

Nightmare on NEJM Street

Richard Lehman

Your Christmas Day came to a blurry end with quantities of port wine and Stilton cheese. You don't really remember going to bed, but soon afterwards you are aware that you have become a junior doctor working in an intensive care unit, trying to put in a central line. A sharp American voice from behind you snaps, "Doctor, your patient appears to be septicaemic. Are you aware that 20 000 US citizens die every year from catheter-related bloodstream infections?" In your panic, your hand jerks and the line flies out. Blood spurts over you, your patient, and the attending physician.

You wake up, dry mouthed and sweating. After groping for a couple of indigestion tablets you turn over and hope for better dreams. But now you are in front of a large audience in the Massachusetts General Hospital. The same sharp American voice asks you, "So, doctor, what is your diagnosis?" You see before you the attending physician, with a few remaining traces of blood in his distinguished white hair. A vast audience looks down at you. Every face wears the same expression of earnest disdain. You feel in your pocket for the clinical notes which you must have brought with you, but they are missing: your pocket is full of congealing blood. "Lymphoplasmacytic lymphoma with Waldenström's macroglobulinaemia and biclonal gammopathy," barks the attending, "and you missed it! So, doctor, can you tell us how you would go about investigating monoclonal gammopathy of uncertain significance?" You decide to confess humbly that you have never come across a case. The audience sniggers. "Maybe that's because it's only present in only over 3% of people over the age of 50," says the attending with an ominous smile. "You may leave the hall, doctor."

You wake again, with the hooting of the audience in your ears. This time you resolve to wake yourself properly and get up for a glass of water. Settling back groggily, you find that you are in an examination room. A number of solemn inquisitors sit before you, dressed in gowns the colour of blood. The central figure, a white haired attending physician whom you quickly recognise, begins the interrogation. "If a patient with HER-2 positive breast cancer has disease progression despite a taxane, an anthracycline, and trastuzumab, which chemotherapeutic agents should you use?"

"Err . . ."

"No doctor, not err, you will get a better response if you use lapatinib plus capecitabine. Next question: explain why simple mendelian inheritance patterns are not seen in the long-QY syndrome."

"Could it be because . . . actually, I'm sorry I haven't a clue."

"Because survival bias leads to female predominance. OK, a simple one then. Lack of which apolipoprotein predisposes to infection with Trypanosoma evansi?"

"Oh 'ell . . ."

"I will allow that. L-1 is the full answer. Now, what can you tell us about the biochemical defect which underlies recessive lethal osteogenesis imperfecta?"

"In all its gristly detail?"

"Hah, you are on the right lines again. Deficiency in cartilage-associated protein. And now, doctor, for which condition might you consider the use of eculizumab?"

At this you break down and shout, "You must be taking the bloody p . . .!"

"Excellent, doctor," smiles the attending. "Paroxysmal nocturnal haemoglobinuria!"

You wake up a last time and go to the loo, putting the light on to check the colour . . .

Then, a couple of days later, the New England Journal arrives and you check the contents:

2725: IV catheter-related bloodstream infections in the ICU
2733: Lapatinib plus capecitabine for HER-2 positive advanced breast cancer
2744 Female predominance and transmission distortion in the long Q-T syndrome
2752 Trypanosoma evansi infection linked to a lack of apolipoprotein L-1
2757 Deficiency of cartilage-associated protein and recessive lethal osteogenesis imperfecta
2765 Monoclonal gammopathy of undetermined significance
2772 Case Records of the Massachusetts General Hospital: lymphoplasmacytic lymphoma with Waldenström's macroglobulinemia and biclonal gammopathy
2786 Correspondence: eculizumab in paroxysmal nocturnal hemoglobinuria.

You had had no ordinary nightmare—you had entered a parallel universe.


Richard Lehman's weekly review of medical journals is at http://blogs.bmj.com/category/comment/medical-journals-review.


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