MDS-RARS to MDS-RAEB2

[Shriya]

8 months back my father-in-law hemoglobin count was low around 10 or 12. He was given some Iron injection for a period of six months but there was no progress. Instead the count went down to 8. Hence we took Bone Arrow Biopsy and Aspiration tests. The report on 21 Aug suggested MDS-RARS, with 67% of ring sideroblasts. On Aug 28, he had a blood transfusion with 2 units of platelets. Later we were referred to a oncologist where he had to undergo the test again. The report on Aug 29th suggest MDS-RAEB2, with 10%. Can anyone help me with clarifications on following:

1) What is the difference between blasts and ring sideroblasts?
2) In a span of 10 days how did 67% of ring sideroblast get converted to 10% blast. Can this happen or there could be something wrong with the reports.
3) He has been a diabetics patient for last 10 years. He has been taking proper diets and always shown normal sugar levels. The Karyotyping is showing normal and He has been already prescribed to take 1 capsule of 5mg lenalidomide daily for 21 days. He sometimes feel tired other than this he has never had any other complaints. So I am worried if I should go with medicine or not.

Thanks in advance.

[Chirley]

Has he had a copper level taken?

I was diagnosed MDS RARS which progressed to MDS. RAEB 1 in four months. Then they did a copper level found out I had low copper. I had copper replacement and the MDS went away.

[Birgitta-A]

Hi Shriya,
You could look at this site of this forum about MDS. http://www.marrowforums.org/mds.html. MDS is very complicated so it will take months before you will understand what is happening to your father-in-law.

1 Blast cells are immature blood cells and ringed sideroblasts are immature red blood cells containimg ring-shaped iron deposits.

2 One of the bone marrow biopsies/aspirations is not representative for his bone marrow – probably the first one.

3 Good that his karyotype is normal ! If your father-in-law has MDS-RAEB2 the drug most doctors try first is Vidaza. Revlimid (lenalidomide) is best for patients with the 5q chromosome aberration. Revlimid can help patients with normal chromosomes too – I am taking that drug and have other aberrations than 5q and hope it will help me.

As Chirley wrote it is very important to control copper level because copper deficit will give the same symtoms as MDS.

Then you have to follow his hemoglobin, white blood cells and platelets. Write your own report with all info.
Kind regards
Birgitta-A
74 yo, dx MDS Interm-1 2006, supportive therapy with transfusions, iron chelating drugs, drugs for low white blood cells until 2010. Positive results with Thalidomide + Prednisone during almost 3 years. Now trying Revlimid + prednisone.

[gramous]

hallo Shriya,

I'm so sorry that your father is on the mds boat.

Ring sideroblast are not the same than blast.
Ring sideroblast are immature red blood cells with iron deposit... these blood cell are inefficient because the not good utilisation of iron but don't indicate a evolution of the desease to leukemia.
Blast are immature cells at the predifferenciation level (before the differenciation between red blood cells, white blood cells and platelets).
The blasts increasing (more than 5% in bone marrow aspiration) are a sign of desease progression and indicate that a more agressive treatment than supportative care must be initiated.... like vidazza.
RARS is a sub entity of mds which is usually in low risk category...I think that the probability of evolution to leukemia is the lowest of mds subtype... So I can't believe that evolution can happened so far, I think that the first bonemarrow biopsy was non representative..... Moreover, the fact that his platelets are low and the hemoglobine also makes me think that he was not RARS but eventually RMCD (dysplasia with sideroblast of more than one line cell, here platelets and red blood cell). In RARS type, the doctor test also the jak2 mutation.
Mark also that you can have simultanious ring sideroblast (caracteristic of RARS of RCMD) and blast.
The other point that I don't understand : why is the first choice treatment lenalidomide because this is usually used for the patient with 5q chromosomal delation... Vidazza is usually the first treatment choice for high risk mds... When vidazza doesn't work, you can use, I think, revlimid of dacogen (can anyone confirme this?).

Check the copper and zink level, here it is automaticcely check when you're sideroblastic type because a depletion in copper can cause the same symptoms.

I hope that can help and I'm sorry for my bad English.

Take care of your father, moral and support are important in this desease.

friendly regards,