July 25, 2008
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A not-so-routine thrombocytopenia

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“Oh doctor, would you mind seeing the patient in 513A? She’s about to be transferred to a nursing home and she needs you to clear her.”

My patient, Mrs C, now in her late 80s, had developed cardiac problems and had been admitted by the cardiology team.

Arthur Topilow, MD
Arthur Topilow

“OK,” I thought to myself, “no problem.” Mrs. C had a pacemaker inserted two days before and was going to a rehab facility. Her hemoglobin was around 9.5 g/dL. Not too bad, I thought. I’ll take a look, and send her on her way.

I had been treating this woman for several years for primary refractory anemia. She was originally treated fairly successfully with epoetin alpha (Procrit, Ortho Biotech) in the office. She had sustained a hip fracture about a year ago, and had come through her surgery without any significant incident. When Mrs. C started to require multiple red cell transfusions for her refractory anemia due to myelodysplasia, I felt it was time for azacitadine (Vidaza, Pharmion Corporation), the hypomethylating wonder drug.

Although some patients on it failed miserably, many were helped, and in fact, some patients had remarkable successes. I had the opportunity to use the drug before it was released and was quite positive about it. I told the patient and her daughter that this was the time to try the drug and get off the transfusion habit. We started the drug, not anticipating a cardiac hospitalization.

The surgeon called me before the pacemaker insertion. “You know, Mrs. C’s platelet count is 15,000, but we really need to put in a pacemaker.” I realized that she had just recently completed Vidaza, and, of course, this was the cause of her thrombocytopenia. She had not been on heparin. Her prothrombin time, partial thromboplastin time and fibrinogen tests were normal. There were no other obvious causes. As luck would have it, the Vidaza had been given just three weeks before the cardiac events took place and the need for a pacemaker developed. “Just give her two units of single donor platelets and she’ll be fine,” I said.

Two days later, I was being asked to clear her for transfer. Her hemoglobin had dropped to 7.0 g/dL and her platelets were around 15,000. I walked into the room. A large bandage over her chest was soaked with blood and was dripping onto the bed sheets. I couldn’t believe it. The woman had been seen by three specialists that morning and no one had mentioned that she was bleeding, much less bleeding vigorously. I called the surgeon.

“Look Topilow,” he said. “I was careful about hemostasis. Everywhere I touched she bled. I put in sutures and she bled.”

“OK, no problem. Let’s re-pack the wound, give her blood and platelets, and she’ll be OK.” There was no clinical history of bleeding prior to the pacemaker insertion and all her bleeding tests, except for her platelets, had been normal. I wrote the orders and left. Although she was signed out, none of my partners saw her on rounds the next day. The following day I didn’t get to see her until late in the day on a Friday. The nurses again asked, “Can we send her to rehab?” I walked into her room.

She now seemed even older than her real age of 87. She was pale, frightened and mute. The bandage was soaked and still dripping blood. A slow, but steady stream was coming from under the bandages. I couldn’t believe that no one had called.

“Now what?” I thought. The single donor platelets hadn’t worked at all. She was obviously refractory to platelets due to prior blood transfusions. Do we give blood type–specific platelets or human leukocyte antigen–type specific platelets? This approach was not practical and would take too much time.

“What about novo-seven?” I mused. “Too dangerous,” I said, giving myself my own opinion. She was bleeding and it refused to stop. “No wait, what about Amicar?” I thought. I hadn’t used Amicar (aminocaproic acid) in years, but it had worked great in a patient of mine who had bled severely after a prostate biopsy. I had never heard it being used in thrombocytopenic bleeding, but when I looked it up it was described as an off-label indication. Would it work on my patient?

I called the patient’s daughter. “Look, we’ve got a real problem here,” and I described the situation. I included the risk of thrombosis and failure to stop the bleeding. We decided to proceed with the Amicar every six hours. I also ordered packed cells.

When I came into the hospital the next morning I went directly to Mrs. C’s bedside. The bandage was soaked with blood. Once again, there had been no calls. “Let’s take off the bandage,” I said to the nurse. The nurse removed the bandage put on the day before. I watched it drip blood, but underneath the bandage the wound was not bleeding!

“Well, isn’t this interesting. How about one more dose of Amicar and then stop,” I said to the nurse. I called the daughter and was cautiously optimistic. The second dose was given. The bandage remained dry. There was no further bleeding from the wound. The patient left the hospital two days later. When she came to the office two weeks later her platelets had completely recovered from the Vidaza–induced thrombocytopenia. The patient looked at me and said, “I always thought that surgeon was too cocky and cavalier. I can’t thank you enough.”

I think I was the one who was too cavalier about her low platelets. We decided to stay away from Vidaza in the future. So far, so good; there has been little need for transfusion. In medical school, we used to joke about how luck could beat science every day. Some days, both you and the patient get lucky.

Arthur Topilow, MD, is in private practice at Atlantic Hematology & Oncology in Manasquan, N.J.