HomeAnswersHematologyliver infectionHow to treat spleen enlargement due to repeated blood transfusion?

How to treat the problem in the liver accompanied by spleen enlargement in a young patient?

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The following is an actual conversation between an iCliniq user and a doctor that has been reviewed and published as a Premium Q&A.

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Published At February 18, 2023
Reviewed AtOctober 13, 2023

Patient's Query

Hello doctor,

My nephew who is about to become a teenager has been suffering for more than seven years. Two years after his birth he used to have a poor appetite and constipation. Back overseas he underwent many laboratory tests like myelogram, biopsy, and ultrasound. He has a medical history of fever, severe anemia, a big and hurting tummy, chest pain, and vomiting blood. He recently moved to another country overseas where he has been admitted three times for blood transfusion. He was admitted for the first time before seven months, the second time before six months, and recently for the third time during the last month when he was transfused with eight packs of blood. He also vomited blood as he did almost one year back during his latest hospital admission due to four varicose veins in the esophagus. A Fibroscan and liver biopsy were done during the last two months which revealed that the spleen is enlarged. He has consulted an oncologist, a hematologist, and a liver surgeon. Lately, the surgeon has told that they have detected a problem in the liver but they are not certain whether they will do a spleen ablation, as they have not found the cause of the disease yet. The liver cirrhosis and sickle cell hypothesis were set aside as the doctors did not find any conclusive elements to validate those. The laboratory tests he has undergone are myelogram, histopathology, electrophoresis, Fibroscan, CT scan abdomen, hepatitis, chest X-ray, endoscopy, blood chemistry, liver biopsy, liver ultrasound with Doppler in the last two months, chest radiology, and bone marrow. Earlier he was taking Cotipred and his current medications are Folic acid and Propranolol 10 mg. I have attached the reports of all his laboratory tests and the latest abstracts released by the hospital where he is currently undergoing treatment.

Kindly help.

Hello,

Welcome to icliniq.com.

Thank you for your query.

After going through all the data, the primary disease is the liver parenchymal disease which might be autoimmune. For further evaluation, get an ENA profile test done. The spleen is enlarged and engulfs blood cells, this is also secondary to liver disease. It is called hypersplenism. I suggest you follow a few instructions mentioned below.

  1. If you need repeated blood transfusions, remove the spleen surgically after appropriate vaccines according to the local guidelines.
  2. Build up his vitamin B12, folic acid, pyridoxine, vitamin D, and iron levels to support hematopoiesis.
  3. Give a therapeutic trial of prednisolone 1.5 mg/ kg/ day, tapering dose after a fortnight for liver disease.
  4. Get back when you get the ENA profile results.

I hope this has helped you.

Thank you.

Patient's Query

Hi Doctor, Thanks so much for taking the time to review all the attachments. Pardon me, but I’d to ask how sure we are of the diagnosis you just gave? Clearly the child has seen sooo many doctors and no one could find out? Lastly I’d to ask what we should expect if ENA profile test is positive what would be next steps if negative what would be the next steps? Because the child’s life is already at risk at this point, are there any specific doctors would you recommend us to take the next steps with? Currently Doctors in the Philippines are saying they won’t do a spleen ablation because the problem is not the spleen. They also said the child does not have a liver cirrhosis as they thought, but have some liver problem… there are planning a surgery, but we are not so certain on what the exact procedure would be… we are scared to an extent… By the way we recently did ELLA test for the child, while waiting for ENA profile test results, atttached is the ELLA test if this can help somehow… Thanks so much!
hi. welcome back. nothing can be 100% sure. ANA test is negative. this is by ELIA method. currently, method of choice for ANA test is by immune fluorescent method. there's no cirrhosis of liver but there's is parenchymal disease which is causing backflow of blood from spleen. spleen is trapping and destroying blood. ENA profile might indicate antibodies which are damaging liver. we can give immune suppression therapy for this. the proper doctor would be a hematologist to treat this disease.

Same symptoms don't mean you have the same problem. Consult a doctor now!

Dr. Mubashir Razzaq Khan
Dr. Mubashir Razzaq Khan

Hematology

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