Wednesday, April 16, 2008

Page 3

“No. He has a patient precisely similar.”

“Remarkable. Like London buses. You had better begin at the beginning and tell me the whole story.”

“My patient,” she began, “is a 35 year old man who presented to the emergency department with fever and tiredness. He had bruises on his arms and legs and scattered crepitations in both lungs. Otherwise there was nothing to find clinically. He had no living relatives and no family history of leukaemia. When asked about his occupation he said he did a little of this and a little of that. He had been completely well until three weeks before he came to the hospital.”

“Anything else about his skin?”

“No, I don’t think so.” She took a notebook from her handbag. “Oh, he had tattoos and a patch of psoriasis at both elbows.”

“Go on.”

“We admitted him to Kinross ward, did a bone marrow and, just like you, we made a diagnosis of M3, promyelocytic leukaemia. Then the chromosomes came back and he didn’t have the 15;17 translocation as he should have done. So no PML/retinoic acid receptor conjoined gene and I suppose we should not have been surprised that he didn’t respond to ATRA. We added in daunorubicin; still no response. We changed to arsenic and he goes straight into complete remission. The bleeding was a problem as you might expect, but we stuffed him full of platelets and fresh frozen plasma and we managed.”

“And how is he now?”

“Goodness knows. Last Thursday night he did a runner. Sometime between ten at night and six in the morning he left the hospital and hasn’t been seen since. I went round to his address and it turned out to be bogus. He gave the name of a doctor in South London who doesn’t exist. The NHS number he gave is also bogus.”

“He was a white, Anglo-Saxon, I suppose?”

“Certainly seemed like one. He had a London accent. The name he gave, Harry Fisher, certainly sounds English, though there is no Harry Fisher on the National Database, not with the date of birth that he gave. “

“What about the Chediak Higashi granules in the lymphocytes? Did they disappear when he went into remission?”

“Yes. What about your case, Prof? Was he similar?”

“Hardly! He was an 87-year old who had atypical TB .” The professor consulted his blue notebook again. “Yes, he was though to be too old to warrant chemotherapy and died after four days of a cerebral haemorrhage.” He read on. “There follows a little bit more on what we knew about Chediak Higashi in 1974. Did you know that it used to be called the Chediak-Steinbrinck-Higashi anomaly? Large peroxidase-positive granules in myeloid cells, normally seen as part of a congenital disease. Did you know that it also occurs in mice, Hereford cattle and Aleutian mink? In 1974 there had been only 59 human cases worldwide. Most died in infancy, with only a few surviving to early adult life. This was the first time it had been seen in an old man and the first time the granules had been seen in lymphocytes. Since it occurred in a patient with leukaemia I believed that this was part of the dysmyelopoietic process. We know that lymphocytes and myeloid cells drive from the same stem cell. It follows therefore that in my patient there had been a acquired genetic defect in the common stem cell that manifested both as the bizarre giant granules and the leukaemic process. I see that I have a note here that says ‘there will be others’.”

“And now you have seen another.”

“I have seen one other case. It was presented at a meeting of the British Society for Haematology. I rather think it was the year that it was held at Lancaster University. I think the doctor may have come from Hereford though that may be the association with Hereford cattle. Sometimes my mind begins to play tricks with me.”

“Let’s look it up. The proceedings of the meeting will be published in the British Journal.”

“Alas, would that we could, but Lancaster was the meeting when something went awry and no abstracts were delivered to the Journal. I was on the Editorial Board at the time. You might say that Lancaster bombed.”

“Prof, was that one of your awful jokes?”

“Forgive me, it slipped out. But now we have four cases with your friend’s in Northern Ireland. Tell me about that case.”

“The Irish case was very similar to ours. A man of 34, sudden onset, M3 with Chediak granules in the lymphocytes, no chromosomal abnormality and unresponsive to ATRA and chemotherapy.”

“And did their case do a runner too, as you so entertainingly put it.”

“No, their patient died. They didn’t have access to arsenic. But what is really strange is that the patient didn’t disappear, but his body did.”

“It would seem that the running here was involuntary.”

No comments: