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Please advise regarding storage pool defect.

This Premium Q&A, reviewed and published, features a real conversation between an iCliniq user and a physician.

Patient's Query

Hi doctor,

My son has storage pool defect. Now, his doctor suspects Crohn's disease on him. All cut tests will result in horrible bleeding time. Should hematology be involved? Should the doctors use coagulant as a precaution? Your further advice would be helpful. Thank you.

Answered by Dr. Prakash. H. M.

Hi,

Welcome to icliniq.com.

Based on your query, my opinion is as follows:

  1. Yes, patients with storage pool deficiency (platelet function disorder) will have increased and horrible bleeding time. We are looking at one of the important bleeding disorder here.
  2. Unless they have decided to take a biopsy to confirm Crohn's, nothing much should be necessary. Deeper biopsy can cause excessive bleeding.
  3. Only, if there is any bleeding or if they intend to take a biopsy, antifibrinolytic agents such as Epsilon-aminocaproic Acid (EACA) and Tranexamic Acid will be given. Local sealants like gelfoam most of the times will be adequate. If necessary, platelet transfusions will be the next option.
  4. Yes, if the treating doctor does not have knowledge of storage pool defects, hematologist needs to be involved.
  5. If they intend to take a biopsy then precautionary transfusion of platelets or antifibrinolytic agents may be necessary. If they are just planning to look and take a biopsy later then no precaution is necessary.
  6. Make sure that the doctor has knowledge about his condition of storage pool defect before the procedure. If the doctor knows it then based on the procedure involved, hematology will be necessary for supportive therapy.

Treatment plan

If planning for biopsy then platelet for transfusions to be kept ready along with antifibrinolytics.

Patient's Query

Hi doctor,

Your helpful advice possibly saved my son from a huge bleed. The doctor did not have the knowledge of storage pool defect and just before placing my son to sleep we talked about biopsy. I had explained to him about your advice and she stopped the biopsy. She made an appointment with the upper GI surgeon for the procedure. I appreciate your valuable advice. You saved my son, Thank you.

Answered by Dr. Prakash. H. M.

Hi,

Welcome back to icliniq.com.

You are welcome. Good to know it could be of help. You are taking very good care of him. Do keep the doctors updated about his condition, if not, they can unknowingly cause damage.

Answered byDr. Prakash. H. M.

Medically reviewed byiCliniq medical review team

Published At April 30, 2016
Reviewed AtJune 6, 2023

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

Dr. Prakash. H. M.
Dr. Prakash. H. M.

Hematology

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Please advise regarding storage pool defect.

Read answers about:

bleeding disordertranexamic acidcrohn's diseaseantifibrinolyticsepsilon-aminocaproic acid (eaca)platelet transfusionstorage pool deficiency

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