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How would you treat a patient with autoimmune hepatitis, grade 4 cirrhosis, and declining platelets? Readers' Response

December 30, 2021

Here’s how readers responded to a You Make the Call question about a 22-year-old patient presenting with epistaxis, a platelet count of zero, abnormal liver function tests, and autoimmune hepatitis with grade 4 cirrhosis.

Disclaimer: ASH does not recommend or endorse any specific tests, physicians, products, procedures, or opinions, and disclaims any representation, warranty, or guaranty as to the same. Reliance on any information provided in this article is solely at your own risk.

Yes, liver transplant is an option. I guess that she has been screened for other autoimmune antibodies already. The platelet count of approximately 30×109/L should be enough for survival. She will need a platelet transfusion if going for surgery. It is better to keep her on a minimum dose of steroids if she is comfortable. Splenectomy should be a last option in my opinion.

Mala Tudawe, MD Durdans Hospital Colombo, Sri Lanka

Continue rituximab or eltrombopag.

Anastasia Skandali, MD Hygeia Hospital Athens, Greece

First of all, if she is being considered for liver transplantation, it is possible that the patient has portal hypertension, so the splenectomy is a very risky alternative (because of post-surgery bleeding). I would prefer maintaining the prednisone in 1 mg/kg daily for at least one month, then initiate a slow, six-month taper. If the platelet count drops below 30×109/L, I would give another dose of rituximab (or IVIg depending on the clinical scenario). If the patient is already waiting for a liver donor, I would prefer adding eltrombopag to the steroid therapy, with the aim of inducing a complete response. After the transplant, I would continue with eltrombopag (added to the immunosuppressive therapy used with the transplant), trying to reduce the dose, very carefully, keeping the patient in complete response.

José Luis Viñuela Cox Hospital del Salvador Santiago, Chile

Thrombopoietin receptor agonists.

Mixalis Mixail, MD Nicosia General Hospital Nicosia, Cyprus

Splenectomy is my advice.

David M. Baer, MD Kaiser Permanente Oakland Medical Center Oakland, CA

I think that splenectomy at the same time of liver transplant is the way to go since she has had relapsed ITP after steroids and rituximab. I would consider thrombopoietin agents after that, if necessary.

David Sumoza, MD John H. Stroger, Jr. Hospital Chicago, IL

It takes a while to find a transplant. I would give her romiplostim or eltrombopag until an appropriate donor liver is found. She probably does not need a splenectomy as she will be on immunosuppression after her liver transplant.

Steven Sandler, MD Advocate Illinois Masonic Medical Center Skokie, IL

Rituximab has proven useful in this kind of patient. Splenectomy can be considered when liver transplant is decided.

Arturo Mario Musso, MD Hospital Militar Central Buenos Aires, Argentina


  January 2022


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