Myelofibrosis on a background of essential thrombocytosis

[Kashnav]

Hi,

I am 65 year old male. I was diagnosed by "Essential thrombocytosis" 4 years back and was prescribed hydroxyurea (also known as hydroxycarbamide) for reduction of the platelet count to the normal range. I'm also diabetic from past 20 years.

Frequency of hydroxyurea usage - one tablet on one day and two tablet on next consecutive day.

Been experiencing low platelet, Heamoglobin and high Leukocytes of late.

Latest CBC report shows below numbers -

Hemoglobin : 6.8 g/dl
RBC : 1.85 million/cummm
Total Leucocytes Count : 10110 cells/cumm
Platelet Count : 56220 cells/cumm

As per suggestion from oncologists done a Bone Marrow aspiration and trephine biopsy below is impression "Myelofibrosis on a background of essential thrombocytosis"

Detailed report is below

Report - Peripheral blood smear shows normocytic normochromic red cells with round macrocytes. There is a prominent leucoerythroblastic reaction. Thrombocytopenia + Bone marrow aspiration yielded a 'dry tap' hence cannot be commented upon. Biopsy shows adequate number of bony trabeculae. Intervening marrow shows large areas of fibrosis. Section is negative for granulomas.

Questions -

Does above imply i have developed Myelofibrosis ?

Are there any tests that need to be done to diagnose the condition or extent of involvement?

What are treatment options available?

Can it be treated with tablets or require chemotherapy / radiation?

Please suggest, your advice is appreciated.

Thanks,
Kashnav

[DanL]

Kashnav,

One seemingly logical step would be to get tested for v617f jak2 protein. presence of this protein, which can be checked from a blood draw, would increase the likelihood of response to one of the new clinical trial drugs know as JAK inhibitors. There are several good reports on about 3 different JAK1/2 inhibitors.

another question though, has your doctor commented on an increased spleen size? splenamegaly along with marrow fibrosis is usually necessary for a diagnoses of Primary Myelofibrosis.