Continue supportive care or begin Vidaza?

[S001]

My dad has been on Erythropoeitin injections since August and he is transfused with 2 units of blood every 2-3 weeks, when his hemoglobin level dips to 7 or so. After transfusion, his hemoglobin count hovers around 9.

His platelets and WBC counts however, are normal (with platelets lying anywhere between 60K - 150K and WBC count around 14).

According to doctors, the disease hasn't progressed and it is at the stage it was in, at diagnosis. They are recommending 6 cycles of Vidaza, but they aren't very conclusive with their suggestion as well.

Apart from the low hemoglobin counts, my father is fortunately not facing any other medical condition, though he has faced tiny episodes of infection causing an enlarged lymph node below the ear (under the jawline) and fever, with mild cough.

We'r confused if we should try Vidaza or continue with supportive care. Dad doesn't like getting tired towards the end of the 2nd or 3rd week and then getting pricked for it. But we'r also hesitant about trying Vidaza because of possible side effects and/or no response.

Any suggestions/pointers?

[bebop]

what is his dr recommending? is he considering bmt? 59 is fairly young so he might just get the good results. good luck with whatever he decides

[launch]

I concur... So very young at only 59yrs.... Continued meds as supportive care gives time for modern medicine to find the "Cure" or better meds to manage the condition/disease... Never give up!

[S001]

Originally Posted by bebop

what is his dr recommending? is he considering bmt? 59 is fairly young so he might just get the good results. good luck with whatever he decides

On the last visit to the doc, he mentioned that my dad made a good BMT candidate, since he doesn't face any other medical issues. But we haven't pressed for it till now. Another appointment with the doc today, so will be discussing options for my dad. Lets see what we decide.

And thank you.

[S001]

Originally Posted by launch

I concur... So very young at only 59yrs.... Continued meds as supportive care gives time for modern medicine to find the "Cure" or better meds to manage the condition/disease... Never give up!

Never give up indeed. Prayers for everybody dealing with this too.

[S001]

The doctors today said that we should try 2 cycles of Vidaza, because the blood transfusion need seems to increasing. For one, don't people have to try Vidaza for 4 cycles before deciding if it is working or not?

Also, they don't want to do another BMB (there was one done at diagnosis in May and then in June - both of which showed the same results), but aren't we supposed to get a BMB done to know if the disease is progressing or not?

[Bob Macfarlane]

Was your dad ever in the military? He is pretty young to have MDS.

[S001]

Originally Posted by Bob Macfarlane

Was your dad ever in the military? He is pretty young to have MDS.

No, wasn't. Doctors mentioned that it's rare for my dad to have MDS in the first place because he is Asian. Young too. :S

[Zoe's Life]

S,

I was diagnosed at 47. Since then I have "met" many people younger than I am who are diagnosed. In fact, in Germany a study was conducted which included 232 patients under 50. The same study referred to other studies which had 151 patients under 50 and another study with 205 patients under 60. I don't think it is as uncommon as people think.

I am concerned that your dad is still on erythropoetin if it isn't working (I think transfusions every 2-3 weeks isn't working, unless he was receiving them more frequently before that). I can't cite them right now (maybe someone else can), but I think there have been problems with continuing erythropoetin when it isn't working. Honestly, I don't even recall the problems at the moment.

Do you know what, if any, chromosomes are affected? A lot of people here have had great success with Vidaza. Personally, I would try it if I were getting frequent transfusions, although my next drug will likely be Revlimid.

Zoe

[Greg H]

It's really great that you are looking out for your Dad.

Like Zoe, I am very curious about the chromosomes, as well as whether he has a PNH clone, hypocellular marrow, and/or is HLA-DR15 positive. Also the blast count. Do you know his IPSS score?

Younger folks with MDS sometimes respond well to immunosuppressive therapy [IST] using ATG and Cyclosporine, and all of the things I mentioned have shown up in one study or another as possible markers that indicate a good probability of response to IST.

Birgitta is more up to date on the Vidaza studies than I am, but the initial work that got it approved for use in the US was on higher-risk patients (i.e., lots of blasts, bad or multiple chromosomal abnormalities, multiple cytopenias). Despite his frequent need for transfusions, I'm wondering if he's high risk or not?

Can you give us a little more info from his BMB reports?

Take Care!

Greg

[S001]

Originally Posted by Zoe's Life

S,

I was diagnosed at 47. Since then I have "met" many people younger than I am who are diagnosed. In fact, in Germany a study was conducted which included 232 patients under 50. The same study referred to other studies which had 151 patients under 50 and another study with 205 patients under 60. I don't think it is as uncommon as people think.

I am concerned that your dad is still on erythropoetin if it isn't working (I think transfusions every 2-3 weeks isn't working, unless he was receiving them more frequently before that). I can't cite them right now (maybe someone else can), but I think there have been problems with continuing erythropoetin when it isn't working. Honestly, I don't even recall the problems at the moment.

Do you know what, if any, chromosomes are affected? A lot of people here have had great success with Vidaza. Personally, I would try it if I were getting frequent transfusions, although my next drug will likely be Revlimid.

Zoe

Yes, MDS isn't as uncommon for younger people as its made out to be. And we've discontinued EPO injections for dad now. Glad you pointed it out though.

As for the chromosomes affected, this is what the cytogenetics (FISH) stated -

"nuc ish(ABL1,BCR)x2[198/200]
i.e. there was no BCR-ABL fusion in 99% of the nuclei analysed

nuc ish(EGR1, D5S23, D5S721)x1[183/200]
i.e. there was both EGR1 and D5S23, D5S721 deletion in 91% of the nuclei. FISH pattern suggestive of monosomy 5.

nuch ish(D721x2),(D7S486x1)[170/200]
i.e. there was 7q deletion in 85% of nuclei analysed. In myelodysplastic syndrome, IPSS cytogenetic score is therefore equal to 1 (high risk IPSS)"

So that means monosomy 5 and 7q deletion. In another report there was a third one - trisomy 8 in 70% interphase cells.

[S001]

Originally Posted by Greg H

It's really great that you are looking out for your Dad.

Like Zoe, I am very curious about the chromosomes, as well as whether he has a PNH clone, hypocellular marrow, and/or is HLA-DR15 positive. Also the blast count. Do you know his IPSS score?

Younger folks with MDS sometimes respond well to immunosuppressive therapy [IST] using ATG and Cyclosporine, and all of the things I mentioned have shown up in one study or another as possible markers that indicate a good probability of response to IST.

Birgitta is more up to date on the Vidaza studies than I am, but the initial work that got it approved for use in the US was on higher-risk patients (i.e., lots of blasts, bad or multiple chromosomal abnormalities, multiple cytopenias). Despite his frequent need for transfusions, I'm wondering if he's high risk or not?

Can you give us a little more info from his BMB reports?

Take Care!

Greg

Apologize for my elementary knowledge of reading BMB's and BMA's. Which is why I have posted the reports below -

"Test: Histopathology Examination
Specimen type: Bone marrow trephine

Microscopy : Sections show a linear core of bone marrow which is hypercellular. The myeloid and erythroid ratio is markedly increased. There're sheets of eosinophils and their precursors. The erythroid series is markedly decreased. The megakaryocytes show dysplastic features with micro-megakaryocytes, monolabated megakaryocytes with occasional dysplastic ones.
There is no evidence of increase in blasts or ALIP.
There is a mild increase in reticulin fibres (grade 1).
Diagnosis: Bone marrow biopsy- Hyper cellular marrow with an increase in eosinophils (see description) Over all features are of a low grade myelodysplastic syndrome."

------------------------------------

"Peripheral Blood picture report -The red cells appear mildly hypochromic. Show oval macrocytes, tear drop shaped cells and contracted cells. The white cells show leucocytosis with an eosinophilia, neutrophil left shift with some blast cells and dysplastic changes in the neutrophils such as hypogranularity and hypoloburity. A cell count on 200 cells revelaed 2% myeloblasts (No Auer rods detected), 16.5% monocytes(promonocytes-4% added to this count), 10% eosinophilis, 23.5% lymphocytes. The platelets are reduced and hypogranular.

Comment:The features are of a myeloproliferative disorder and of a myelodysplasia.

Bone Marrow aspirate report - Indicate for bone marrow examination - To investigate leucocytosis with dysplastic changes
Aspirate - Difficult from the RPIC
Fragments - One or two, densely hypercellular
Cell trails - Normocellular for age
M:E Ratio 10:1
Erythropoiesis - Markedly depressed. Normoblastic with some megaloblastic changes. Show mild dyserythropoiesis and some cells show poor haemoglobinisation.
Granulopoiesis: Normocellular with giant metamyelocytes, show dysplastic changes and a marked eosinophilia. The myeloblast consist of 3% nucleated marrow cells.
Megakaryopoiesis: Appear normal in number and show small round forms, monolobalar forms and separate nuclei in some cells
Plasma cells: Appear normal
Lymphocytes: Appear normal
Other cells: None
Iron stores: Increased. Show ringed > 15% sideroblasts (As erythropoiesis is depressed only about 55 cells and 33 cells counted on separate counts and gives>15%. A total of 100 cells was not counted in one differential count for ringed sideroblasts)
Diff:

-----------------
NRBC: 8.7%
Blasts: 3.4%
Promyelocytes: 05%
Myelocytes: 8.7%
Metamyelocytes: 1.2%
Neutrophils: 23.7%
Monocytes: 9%
Eosinophilis: 23.7%
Basophilis: 00%
Lymphocytes: 12.5%
Plasma cells: 00%
Other cells: 00%

Comment: The features are compatible with a myelodysplastic syndrome - refractory anemia with excess blasts type 1. Surface marker study - Scanty blasts gated show myeloid phenotype. (Blasts are positive for CD34, CD117, CD33 and CD13). "