a few times i and a couple others have been questioned about posting here because b-pll is so rare. However i happened across something on the Leukemia and Lymphoma site.
If you go a third of the way down on the page under available funding they consider b-pll a subtype of cll.
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Interesting to read that B-PLL can also be considered to be a subtype of Mantle Cell Lymphoma (MCL) and that there's actually a spectrum of presentation from a subtype of CLL to a subtype of MCL: bloodjournal.org/content/12...
Then there's a T-PLL too! Makes you appreciate how it is possible to distinctly identify around 90 different Non Hodgkin's Lymphomas and how rare some of them must be!
B-PLL used to be lumped in with CLL, but it is different, so both B-PLL and Hairy cell got their own designation under WHO cancers in the mid 1970s.
B-PLL with t(11;14) translocation is now considered to be MCL.
Clinically B-PLL and CLL are quite different.. here is the most authoritative writing on management of it by noted expert Dr. Claire Dearden, 2015.
A landmark study in 1973 identified B-cell (B-PLL) and T-cell (T-PLL) subtypes of prolymphocytic leukemia. This rare aggressive subtype of leukemia had been recognized as distinct from chronic lymphocytic leukemia (CLL) by the clinical presentation with splenomegaly and high white blood cell count, the characteristic morphological appearance of the circulating lymphoid cells, and the poor clinical outcome
all i can say-since there is no specific targeted therapy drug for b-pll -i get treated as if i'm end stage cll. i have p17 deletion and p53 mutation. I don't know if my doctor did not expect imbruvica to work at all and thats why they had me at dana farber planning for stem cell transplant but at this time it seems to be working. Will it work just a short time-i actually don't know. There is practically no knowledge of it-just cll knowledge
my doctor says ventclexa will be next. the hcst i hope never but i will if i have to. However at my age it will be reduced intensity that keeps most of your own cells and adds another to combat your bad cells. I've posted before about how a true transplant and this different version actually work
What would be your RIC conditioning treatment? I was on idelalisib and rituxan, it worked very well for me.. and Dana-Farber has a long history with that treatment...
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