new diagnosed ITP

I am a 50 y.o. female that was otherwise healthy until 18 months ago when I had a sudden onset of petechia; was found to have a pancytopenia plt=7, WBC=2.3, Hgb=10.5; bone marrow biopsy showed MDS vs toxic effect;all other testing (lupus, viral, etc.-neg.);admitted for platelet transfusions (no other treatment); platelets improved but Hgb and WBC did not;in the next 2 weeks, plts up to 130 without any treatment other than the initial plt transfusions; Hgb continued to fall to 6 gms within 2 weeks (required packed RBC transfusion);then plts began to drop again; started on solumedrol 125 mg IV x1 and oral prednisone 60 mg; no improvement; admitted to the hospital; repeat bone marrow bx-was told "normal" although the comments report "suspicious for MDS, however the findings are nonspecific and possibility of malnutrition, drug effect and toxin insults could not be ruled out"; started on IV Solumedrol 500 mg daily x 3 with slight improvement in plts, then IVIG x 2 with continued plt improvement; finally Rituximab weekly x 4; plts, WBC and Hgb continued to improve (although WBC never returned to normal-always around 3; had a 2nd opinion at University of Michigan Hematology, who felt this was ITP; no problems until 3 weeks ago and began to have purpura and petechia again; plts=single digits and HGB down 3 gms in 2 weeks despite IV Solumedrol 1 gm, oral prednisone 60 mg,plt transfusions and 3 rituximab infusions; I am concerned that this may be something other than ITP; does the Hgb normally drop with ITP? looks like my WBC's have been low on and off in the past...feeling really overwhelmed

Pam:

It doesn't really sound like ITP. You have other cells involved here and ITP does not affect red or white cells. It sounds more like Evans Syndrome or possible MDS. Platelet transfusions generally do not help a person with ITP at all. The platelets last maybe a few hours at best.

It's probably time for a third opinion.

Thanks! I can't seem to get anyone to give me an answer as to why other cells are involved; I am now currently involved with a 3rd hematologist. I will keep looking!

Have they done a Coombs test? That would let you know if there were antibodies for red cell destruction.

Good luck. Please let me know when you find something out!

yes, my direct coombs was positive, also had elevated bilirubin and LDH when this first happened; been looking into Evan's Syndrome, I have alot of the criteria; thanks!

Sounds like that might be it. Not sure why there is so much difficulty diagnosing. Hopefully, you get a doctor who can make a definitive diagnosis soon!

I have Evans Syndrome. With me it was not hard to diagnose, but it is hard to treat. With me if my hemoglobin goes down my platelets go up, abnormally high.

The positive coombs test you had can be an indication you have hemolytic anemia. It is good you are getting another opinion, i dont know why they cant give you a diagnosis. It must be frustrating.

Saw the hematologist today; asked him about Evan's Syndrome; he said "maybe, but it doesn't matter b/c the treatments are the same"; little worrisome, I think. He did say that my direct coombs was negative this time; would prednisone have any effect (false negative) on coomb's? wants me to continue the prednisone for another month; have been on 60 mg of prednisone for 2 weeks and nothing has improved; I hate the side effects!

What is your red cell count? Haemoglobin is not a good measure of how many red cells you have and whether those are being killed off.

With white cell count it is more important which particular cells are low. Low neutrophils they will worry about far more than low lymphocytes. Low lymphocytes are common around here. Many with ITP and indeed any autoimmune disorder seem to have it.

Prednisone can affect test results, yes. Everyone hates the side effects and it's odd that he's keeping you on it if it's not doing much. Sometimes you have to advocate for yourself and suggest tapering.

pam_dayton@hotmail.com wrote: Saw the hematologist today; asked him about Evan's Syndrome; he said "maybe, but it doesn't matter b/c the treatments are the same"; little worrisome, I think. He did say that my direct coombs was negative this time; would prednisone have any effect (false negative) on coomb's? wants me to continue the prednisone for another month; have been on 60 mg of prednisone for 2 weeks and nothing has improved; I hate the side effects!

The doctor is right about that the treatments are the same. treatments for hemolytic anemia are the same as for ITP, (except for nplate and promacta). The prednisone would probably not affect the Coombs test result.

So on 60mg prednisone you are having no improvement in either platelets or hemoglobin after 2 weeks? Staying on for another month seems like alot. Not only are you having to deal with all the side effects but you are delaying getting to try some other treatment that might work.

RBC's are normal; when the pancytopenia started, my neutrophils were low; the neutrophils recovered eventually, but the WBC's never quite got back to normal

Guidelines say to try pred for 6 weeks before deciding it isn't working, so the doctor is following protocol.

Lastest labs; plt=5-no change with 3 weeks of prednisone and rituxiab x 3 doses;Hct/Hgb and RBC Count continue to fall slowly; peripheral spear shows spherocytes;elevated Retic Count, Normal LDH and Iron Studies. I did read somewhere that prednisone is recommended for 4-6 weeks (but thought I also read that if you have not repsonded within 7-10 days, it is unlikely that you are going to respond; hematologist did agree to decrease down to 40/day for now;