?WHY?- low platelets STILL > 1 yr post BMT

[MGirl]

I posted here last year, several months after our then 4 year old girl's 10/10 MUD BMT for SAA (in the setting of dyskeratosis congenita)/ no GVHD....

....she has done pretty OK by now(dancing class, gymnastics), other than a few large blips needing an ICU stay ...however--her platelets are still so LOW!!

A little history again: She did pick up adenovirus early in transplant as it was "going around" the transplant rooms, and her initial cell counts on day +10 dropped back to 0 for another 3-4 weeks after this happened. She received cidovofir to help get rid of it (itself, a potential suppressant).The highest they got to were 25 around the 1 year mark, and last week they dropped back down to half that again. She hasn't needed any transfusions for about 5 months (cut-off is platelet level of 10--she is typically at about 12-20); chimerisms have been 100% repetitively since her transplant. Her last BM biopsy report 6 months ago said "transplant engraftment failure" after no platelet precursors were seen, although our specialist said to "ignore that" since she had good red and white cell precursors and it was just the single cell line that was misbehaving. He didn't have any other advice than to "just wait....this can happen...and platelets are the most sensitive". We are waiting for another biopsy to be done shortly, although I suspect it will just say the same thing. We were under the impression from the hematologist that once even some platelet precursors settled in and started working, they would 'seed' themselves further. Since her counts are steady-state in the mid teens, we wonder what is happening to them then, preventing an increase in #s.

I asked for anti-platelet antibody tests to be done a while ago--they were negative. She did have a long history of getting mismatched platelet transfusions without our knowledge prior to BMT as they apparently didn't have adequate supplies of her rarer blood type (they didn't forecast her needing a BMT). I'm not sure if this test clears that concern of antibody development with mismatched platelets. She is CMV neg, neg for all other viral screens. She did have a massive issue with hemorrhagic cystitis from BK virus that she picked up during BMT, which was SO severe and painful and lasted nearly 7 months--but it magically cleared itself up one week (I assume with better immunity acquisition as her white counts improved) and that was about 4 months ago.

Our visit to the clinic this past week was the first time they admitted that something may not be right! FINALLY! ...but they still have no idea what the problem is, and have admitted it indirectly. They just want another BM biopsy to see what's happening again....then what???
What is going on?
What are we all missing?

She has had on/off colds through the winter, but that can't account for this year long lack of platelets. Her other counts are OK--hemoglobin roughly 100, WBC anywhere from 4-6. She has pretty good energy. She just never really had much of any response with platelet counts.

However, without platelets, we are extremely worried about the future....any childhood slip and fall, accident, or misadventure in the playground could be disastrous...we can't get on an airplane to go home (still.........), and she still has her big central line ( now for 1.5 years) in her chest due to having to monitor her counts so much still and avoid "pokes".

ANY suggestions would be very helpful!!!!
Any one with any where a similar situation .....we would love to hear what happened.
THANKS SO MUCH!!!!!!!!

[Neil Cuadra]

MGirl,

I'm glad to hear that your daughter has been able to resume normal childhood activities but sorry about her continuing low platelets. I don't have an explanation to suggest so I hope you'll tell us what you learn and what the doctors say about it at her next appointment.

Counts have mysterious ways of staying low or rising without warning, even more than a year after transplant, and we don't always know why. Mysterious or not, I'm sure you don't want to go much longer seeing her platelet count remain so low.

By the way, did your daughter's hair grow back fully? You were worried about that the last time we heard from you.

[MGirl]

Thanks for the note--yes, platelets remain a mystery. We are becoming experts in the field of thrombocytopenia post transplant! And yes....slowly but surely, her hair started to slowly grow back by about the 8-9 month mark after BMT (thanks for remembering!),...and with curly hair now. She is a trooper, and a cute one at that....!