Here’s how readers responded to a You Make the Call question about treatment for a patient with sickle cell trait and end-stage renal disease.
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I assume the patient is of African origin/descent, and that he was non-transfused when the Hb phenotype studies were done. Or, that if the SCT was diagnosed by DNA, that the beta thalassemia mutation common in African populations was specifically excluded. Some African American patients with sickle beta thalassemia are asymptomatic, with little or no anemia.
If he has SCT only, the case is highly unusual, as there must be many SCT patients with renal failure and renal anemia, and only rare, if any, reports of vaso-occlusion problems such as pain and priapism. If he has significant EPO resistance, that would be another similarity to a sickling disorder.
If he responds normally to EPO, then it is possible that when his Hb level gets back to normal, the sickling-like symptoms may abate as they did after renal transplant.
It would be nice to know his transfusion history, if any. If there were peri-transplant transfusions, they may have contributed to the improvement in pain/priapism. If so, they could be considered, at least on a trial basis, until transplant.
I agree that there is no indication for hydroxyurea, especially with normal or hypo-plastic marrow.
Oswaldo Castro, MD
If a man has priapism and ESRD, he doesn’t have just SCT. This shouldn’t have been written up without a print-out of the hemoglobin electrophoresis results or a review of the peripheral blood smear. I have seen many patients incorrectly diagnosed with SCT when they actually have sickle beta thalassemia, or something similar.
Kelty R. Baker, MD
The cause of anemia is not clear. What is the mean corpuscular volume? What are the iron stores? How about vitamin B12 level, thyroid-stimulating hormone, and immunoelectrophoresis results? What is the reason for the patient’s ESRD? All this info is needed before I can formulate a treatment plan.
Dana Shani, MD
New York, NY
The patient may have concomitant alpha thalassemia to account for his symptomatic disease. Not shown were his red cell indices and the percentage of Hb A and Hb S in his red cells; those findings alone will usually suffice to make that diagnosis.
George Honig, PhD