Pleural Extramedullary Hematopoiesis

[disneydad]

My mom was diaganosed with MDS intermediate risk a little over two years ago. She has been through all the regular treatments with no success. She was getting transfusions of PRBC about every two weeks. Her platelets had dropped to about 30 and her WBC was up to the 120's. She is on Hydroxyurea. About a month ago, my moms, marrow biopsy showed mylodysplasia. Her spleen was enlarged to about 17 cm. Now she is in the hospital. At first they thought she had pneumonia, then they realized she had blood in the chest cavity. laproscopic surgery removed the clots but the surgeon could not identify a source of the bleed. The chest tubes continued draining blood for the next 4 days and the bleeding has finally appeared to at least slow and possibly stop.

The problem is that the biopsy from surgery showed signs of Extramedullary Hematopoiesis (ie that the pleura is manufacturing blood cells now). There do not appear to be any discreet nodules. This seems a very rare course for MDS. Unfortunately, I feel like we have reached the point that we just need to keep her comfortable. I would at least like to prevent any more bleeding. It is very painful. Does anyone here have any ideas?

[DanL]

with the extramedullary hematopoesis, have they tested your mom for presence of jak 1 and jak 2?also, have they tested for any other markers that might indicate myelofibrosis? there are a handful of new drugs that seem to have a positive impact on quality of life.

[disneydad]

I don't know about the specific markers or presence of jak but I know that the myelofibrosis was confirmed on marrow biopsy. We are still waiting for final results from the pleural biopsies. What drugs are you referring to?

[Birgitta-A]

Hi Disneydad,
You know Myelofibrosis is a special disease and then it is a symptom, that is common in many diseases like MDS. These patients have fibrosis in the bone marrow.

Patients with the disease Myelofibrosis often have enlarged spleen like your mother, they can have extramedullary hematopoesis (blood is manufactured outside the bone marrow), low HB, low platelets and increased WBCs. Your mother has very high WBCs. Do you know anything about the blast cells (immature cells) in the bone marrow?

I have never read anything about extramedullary hematopoesis in the pleura - the spleen is a very common place for that.

She has mylodysplasia that is a symptom indicating MDS.

Perhaps your mother has both Myelofibrosis and MDS?

The first drug for Myelofibrosis has now been approved. It is a JAK2 inhibitor called Ruxolitinib. It can help both JAK2 positive and negative patients. The drug can decrease the spleen and constitutional symptoms like fever, fatigue and so on but it can cause suppression of the bone marrow and I don't think that drug could help your mother.
http://www.medscape.com/viewarticle/753682
Kind regards
Birgitta-A
72 yo, dx MDS initially 2006. Dx then changed to Myelofibrosis with very severe fibrosis. After one year dx changed back to MDS Interm-1. Good results with Thalidomide + Prednisone.

[Birgitta-A]

Sorry! The link to info about the new drug was a little complicated. Here is another one:
http://www.jakafi.com/
Kind regards
Birgitta-A

[disneydad]

Thank You that is very helpful information. Ruxolitinib was reccomended just before she went into the hospital. We are still waiting on insurance information. It may be prohivitively expensive. We are also concerned that it may not be safe to risk the myelosuppression due to her low platelets. I guess that is why I feel like we are reaching the end of the road. I will continue to investigate the Ruxolitinib though.

[Birgitta-A]

Hi Disneydad,
Many patients with Myelofibrosis are not very happy about Ruxolitinib because there are other JAK2 inhibitors in trials that can improve counts for example CYT387.

Have your mother's bleeding stopped - otherwise she could perhaps get platelet transfusions. In emergency situations the best possible supportive therapy is important.
Kind regards
Birgitta-A