Here’s how readers responded to a You Make the Call question about a patient with aplastic anemia who refuses transfusions for religious reasons.
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I would treat with ATG and cyclosporine and add erythropoietin plus eltrombopag. If baseline ferritin is less than 100 ng/ml, I would add intravenous sodium ferric gluconate complex 125 mg daily for five days. Since there is no infection and neutrophil count is near 500/μL, I would not give granulocyte colony-stimulating factor; steroids and cyclosporine may increase the WBC.
William Caceres, MD
San Juan VA Medical Center
Río Piedras, Puerto Rico
This patient is unlikely to get real benefit from colony stimulating factors. Supportive hospice care might be appropriate.
Michael Rooney, MD
St. Joseph Regional Medical Center
I suggest adding eltrombopag based on recent data. I also recommend HLA typing.
Joseph W. Fay, MD
Baylor University Medical Center
I would start eltrombopag 150 mg twice-daily immediately as per the report by Townsley DM, et al (N Engl J Med 2017; 376:1540-1550 April 20, 2017).
I would also invite a senior member of the patient’s religious organization to a meeting to help him reconsider his treatment options, as the most effective and appropriate curative treatment for aplastic anemia is an allogeneic hematopoietic stem cell transplant from an HLA-matched donor. In my experience, the decision to refuse a transfusion may come from a lack of knowledge/understanding and trust.
George Georges, MD
Fred Hutchinson Cancer Research Center
It is my understanding that Jehovah’s Witnesses do allow BM transplant, as they do not consider this blood. If the immunosuppressive treatment is not effective, I suggest that the patient and his spiritual advisor meet with the physician to discuss the situation more fully.
Alexandra M. Levine, MD
City of Hope
With the platelets so low, I believe that epsilon aminocaproic acid would be helpful to try to prevent serious bleeding.
Fred Hardwicke, MD
Texas Tech University Health Sciences
What are the cytogenetics? Any genomic sequencing performed to rule out hypoplastic myelodysplastic syndromes? What about HLA-DR15 and telomerase studies? He has some likelihood of responding to immunosuppressive therapy based on age and no increase in blasts. Any nutritional deficiencies?
Pavan Kumar Bhamidipati, MD
Washington University School of Medicine
St. Louis, MO
I would certainly consider eltrombopag as this may speed platelet recovery. I would give extra B12, folate, and iron if needed. I would not consider growth factors initially as he needs some stem cells to stimulate. If there are issues with infection, I might consider granulocyte colony-stimulating factor. It is important to explore why he is opposed to blood products because with knowledge he may reconsider his choice.
Loree M. Larratt, MD
University of Alberta
Some Jehovah’s Witnesses would rather die than receive blood and trying to talk them out of their belief only creates a poor patient/physician relationship. This is their choice and they accept the consequences. That does not mean that they do not want to get well and have the best medical care available. They want a physician who is willing to accept their inability to accept any blood or blood products.
So rather than spending your time thinking of ways to change his beliefs, you and the patient have to deal with the situation at hand. All those ideas you have are good. I would also use intravenous iron with higher doses of epoetin and minimize blood draws and use pediatric tubes for blood collection. I would also use tranexamic acid or aminocaproic acid.
I would use your favorite immunosuppressive regimen, at the normal doses. With bedrest and oxygen, this otherwise healthy person may survive with a hemoglobin that is less than 2 g/dl.
Steven Sandler, MD
I would treat with eltrombopag. It could work along with ATG, steroids, and cyclosporine.
A. Saifudeen, MBBS, MD
Sultan Qaboos Hospital
I would add eltrombopag along with immunosuppression.
Naureen Saeed, MBBS
Rawalpindi Medical College
Androgens and growth factors should also be added.
You can add erythropoietin and eltrombopag to this patient.
Manzoor Hussain Wandar Sr., MD
Shahbaz Sharif Hospital
I think it is time to start erythropoietin, granulocyte colony-stimulating factor, and eltrombopag, eventually increasing dosages if significant results are not evident.
Giulio De Rossi, MD
I would add eltrombopag to the current regimen.
Babette B. Weksler, MD
New York Presbyterian Hospital
New York, NY
I think that the administered treatment is the right choice.
Erythropoietin administered in combination with granulocyte colony-stimulating factor has good results in patients diagnosed with aplastic anemia, especially in those with non-severe forms. However, you may not obtain a rapid response; it usually appears after long-term treatment. Moreover, I recommend testing the serum erythropoietin levels and serum transferrin receptors and performing a reticulocyte count before starting treatment with erythropoietin. If these show low values, you have a higher chance of obtaining a response to treatment.
The serum folate level should also be measured, and administration of folic acid 1 mg daily should be considered.
Monica Pescaru, MD
Municipality Emergency Hospital