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Getting back to basics
Sometimes the most complex problems have the simplest solutions.
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“Go see Mrs. X. She has Coombs’-negative hemolytic anemia and needs a bone marrow aspiration.” This was my partner’s sign-out information, which started my day a few weeks ago.
“OK, no problem,” I said.
The facts
I walked to the patient’s floor and started leafing through her chart. She was a black woman, 88 years old. Doing a bone marrow aspiration in the hospital as opposed to the office is always such a chore. There’s a new assistant each time, and everything seems to take three times as long. I examined the lab data. Yes, the patient certainly had anemia. Her hemoglobin was 9.5 g/dL, but it was 6.3 g/dL on admission prior to transfusion. Her complaint had been syncope. Did she really need this marrow? The medical resident, the internist, the cardiologist, the neurologist and my partner had already been there.
The patient’s serum haptoglobin was less than 6.0 mg/dL, and the reticulocyte count was 6%. This was all very positive for a hemolytic state. The Coombs’ test was truly negative. My partner had ordered glucose-6-phosphate dehydrogenase and pyruvate kinase assays. The results were pending. Pertinent negatives in the chart included no history of heart disease, stroke or diabetes. I looked through the radiographic reports. The CAT scans of the chest, abdomen and pelvis were, at first glance, unrevealing.
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The patient
I went to see the patient. She was a little old lady, curled up in bed at 3 p.m. She was awake.
“Good afternoon, I’m Dr. Topilow, a blood specialist called in by your doctors to see if we can find the cause of your fainting spell and anemia. This may involve doing a bone marrow test, but first I’d like to ask you some questions. Can you tell me why you came to the hospital?”
“I’m not exactly sure. I think I passed out,” she said.
“Anything else?”
“I don’t think so.”
I examined her heart, lungs and abdomen. Nothing seemed awry. She seemed pleasantly demented, making my job both easier and more difficult at the same time.
I returned to the chart. Her hemoglobin had responded appropriately after the transfusions. Once again I reviewed the radiographic studies — this time more thoroughly. The pelvic CT was not normal. It read: “There is a collection of what appears to be blood tracking down into the groin on the left side.” That’s odd, I thought. Maybe this woman really has an occult hemorrhage masquerading as hemolytic anemia. I had actually used this very same hematologic situation in question-and-answer format for a book that I had written while studying for my hematology boards 35 years ago. I decided to talk to the patient again and reexamine her.
“Hello again. Mrs. X, did you have any injury or accident recently?” I asked.
“No, not that I can recall.”
“Are you sure?”
“Well, I did have a fall two weeks ago. I hurt myself pretty badly, but I didn’t break anything.”
“What did you do?”
“Well, I fell onto my hip and seemed to twist it.”
“OK, let’s take a look.”
I thought I might see a little swelling of the left thigh. Turning on the light in Mrs. X’s darkened room, I was surprised to see that her left thigh was more than a little swollen. It was twice the size of the right one. Medially, there was a lump the size of a grapefruit.
“Does this bother you or hurt you?” I asked.
“No, not really,” she said.
The diagnosis
So there was the diagnosis. This woman didn’t need a hematologist and a bone marrow. She didn’t need the cardiologist who had ordered a Holter monitor and echocardiogram. She didn’t need the neurologist who had ordered MRI and an electroencephalogram. She needed someone to examine and talk to her. I almost missed the diagnosis myself, especially as I did not initially ask the patient to get up and walk. She had injured herself seriously enough to have had pelvic and intramuscular bleeding, resulting in a syncopal episode and her subsequent hospitalization.
The concealed hemorrhage had led to extravascular free hemoglobin being reabsorbed into the circulation, where it was bound to haptoglobin. The hemoglobin–haptoglobin complex had been taken out of circulation by the liver, and the serum haptoglobin subsequently had fallen to an abnormally low level, mimicking hemolysis. A haptoglobin level, though a very good test for the presence of hemolysis, only has a true predictive value of 87%. The patient’s elevated reticulocyte count was a response to the blood loss from hemorrhage. It would certainly have been a stretch to discover a congenital hemolytic anemia in an 88-year-old woman.
It was an old lesson learned once again. A good history and physical exam is always important and often provides the needed clues to a patient’s diagnosis, which may or may not require more extensive or invasive studies. Without it, we risk getting off the track. It was a close call for my patient and for me. We all need to go “back to basics” from time to time.
Arthur Topilow, MD, is in private practice at Atlantic Hematology & Oncology in Manasquan, N.J.
For more information:
- Topilow A, Wilson M. Medical Examination Review Book, Volume 32, Hematology. Flushing, NY: Medical Examination Publishing Company; 1974:54, question 342.
- Marchand A, Galen RS, Van Lente F. The predictive value of serum haptoglobin in hemolytic disease. JAMA. 1980;243:1909-1911.