16 yr old son has ITP/Hemolytic Anemia nd now Hypocellularity

[joshmom]

My son was diagnosed with hemolytic anemia 8 years ago-treated with steroids and disappeared until 4 months ago.
This time his red blood cells were not being destroyed so we were seeing hematologist every other week for blood tests.
3 weeks ago-I rushed him to hospital as he was bleeding from his nose and bruises started appearing before my eyes. His platelet count was down to 1,000.
In hospital for a week and had bone marrow biopsy-preliminary results were fine.
Just got back final report and it showed "hypocellularity" according to my son's hematologist. He told me that my son would have to have another bone marrow biopsy in 6 months ( I guess when his steroid treatments are over)
The hematologist would not go further into detail with me on this as he knows I get online and research everything (I think this irritates him :-( )
My question is does anyone on here have hemolytic anemia Itp and hypocellularity?? What exactly does this "hypocellularity" mean?
Thank you,
Debi (Josh's concerned mom)

[dkimmel]

Was your son ever tested for PNH? It is an extremely rare form of hemolytic anemia often missed or mistaken for something else. Are your sons white cells, red cells, & platelets low?

[joshmom]

He was tested positive for Autoimmune hemolytic anemia a few months ago, but his red blood cells strangely were not being destroyed-all his blood counts were mostly normal with the exception of the coombs test being positive for AIHA. 3 weeks ago he was diagnosed with ITP when his platelet count dropped to 1,000.
Looking at his blood reports for the past few months does show his white blood count to be lower than normal but nothing too drastic. His platelet count is back up to 288,000 after being on steroids for 3 weeks.
With all this though, he now has this "hypocellularity" and I am just not sure if it is because of the other 2 disorders or else if maybe he has something else primary that is causing the ITP and AIHA???

[joshmom]

Dkimmel--I forgot to ask is there a specific test for the PNH?
Not sure if he has been tested specifically for that but wouldn't it show up in the coombs test for hemolytic anemia?
Thank you :-)

[dkimmel]

Sorry my post was so brief earlier, I hadn't had enough coffee yet. Let me start by saying how sorry I am to hear about your son. I can't imagine how you must feel. This has really got to be tough on a teenager. But fortunately you are seeking out info. Don't let your doctor make you feel bad about it either
I don't know very much about ITP or AIHA. Hypocellular marrow can be associated with aplastic anemia, and PNH is a very rare hemolytic anemia. AA and PNH are very closely related. Since your son has hypocellular marrow and a hemolytic anemia, you might want to check into it, even if just to rule it out. There is a simple and specific test for PNH. It is called flow cytometry where they test for CD59 and sometimes CD55. Not very many labs do this test. Your doctor will probably have to send it somewhere. But having the correct diagnosis is paramount for recieving beneficial treatment. Again, if only to rule things out and make you more confident in his current diagnosis. Also I can't stress enough the importance of seeing a specialist in whatever disease your son has. Local hematologist just don't have the experience in these rare diseases. Please keep us posted.

[joshmom]

Thank you so much DK!! I too feel it is important to know exactly what is causing all these disorders in my son. I am getting frustrated that is why I turned to these boards. My son's hematologist wont give me details anymore because I question EVERYTHING. You seem to have great knowledge-do you have PNH or AA?? Who would you recommend me to take my son to? we live on the west coast (Vegas)-we have family in Bham alabama --I have been told that the children's hospital there is one of the best.
Debi :-))

[joshmom]

Dk----Just like you I haven't had my coffee fill yet...I forgot to ask why the doc would want to wait 6 months to re-do the bone marrow biopsy-is it because my son is still on steroids?

[dkimmel]

I don't know why your doc would want to wait 6 mos. You should ask him. So what if you question and investigate, you're entitled to. You should, no doctor knows everything. It is a science. Have you ever had a second opinion?
I live on the east coast so I cannot recommend any particular doctor or facility to you. Also I was an adult when dx with AA 10 years ago. Currently in remission, but recently dx with PNH. (yeh, both) I am not familiar with pediatric doctors. You should do a seperate post on this site asking for specialist info around your area. You may want to consider traveling though. Even just for a second opinion, then correspond with the specialist and have treament near home.

[Ruth Cuadra]

Hi, Debi.

You should take a look at our list or map of treatment centers for places near where you are. Stanford, City of Hope, and UCLA in California or the Mayo Clinic in Phoenix are possible choices.

There was a recent thread about cellularity here.

DKimmel has given you the right advice: a second opinion is always important and your son's doctor should not discourage you from learning. It sounds like you do understand a lot of the details, but s/he should be able to answer whatever questions you have.

Regards,
Ruth Cuadra

[joshmom]

Thank you Ruth and DK!

Ruth,
We are from Birmingham Alabama originally and some of my relatives are living there along with my son's father. I saw on your list that UAB was one of the hospitals mentioned and I wondered if you heard of any good specialists located there. This would be worth the trip back home.

Thank you again for your input
Debi

[Ruth Cuadra]

I don't know anyone in particular at UAB, but you could contact the Aplastic Anemia & MDS International Foundation (toll free 800/747-2820) to ask about specialists in Alabama.

Ruth

[joshmom]

Hi DKimmel

I had the doctor's office fax me the bone marrow results.
Looks like they did a Flow Cytometry Immunophenotyping.
Not exactly sure how to read that part but the final diagnosis of the whole bone marrow biopsy is:
Leukopenia with left shift in the myeloid series
Thrombocytopenia
Hypocellular bone marrow with trilineage hematopiesis and adequate to mildly increased megakararyocytes
Decreased marrow storage iron
It states that there is no definitive evidence of infectious process present and no evidence of malignancy but clinical correlation is needed

[dkimmel]

I'm sorry to say that I will not be very helpful in helping you understand much of that. Hopefully some others with better knowledge will share. I think that the flow cytometry immunophenotyping would be checking for cytogenic abnormalities. If you were wondering if that was a test for PNH, it is not. I believe the dereased marrow storage iron is consistent with having a hemolytic anemia. Leukopenia means decreased white count, thrombocytopenia means decreased platelets. I'm sure you already know that, though. Maybe push your doc for a better explanation... easier said than done, I know.