CLL or SMZL differentiation or relation

[lukidude]

Hello, I am new to the site. My relative was diagnosed with CLL about 3 years ago and told they would 'watch and wait' until the wbc began to rise significantly, but has now been diagnosed with SMZL by a new hospital. But currently treatments so far not working (rituxmab or ibrutinib). Whilst I have every faith in the 2nd hospital with the new diagnosis, and did not have faith in the previous one that left my relative without treatment perhaps too long, as now found in bone marrow to be at stage 4, I am puzzled by these 2 diagnoses and how they may or may not be related and whether the treatments are similar or different, or whether someone could actually have both at the same time. Whilst I doubt very much that the 2nd hospital has a wrong diagnosis (as marked outstanding) as the drugs are not working and whilst I think the same drugs can be used for both conditions, I wondered how these 2 conditions differ and how the diagnosis differentiates between the 2 or why it is that both conditions are treated with similar drugs?

Also, if someone has an unusual blood type, as these are blood related conditions, can this impact upon the success of treatments?

What are the genetic markers that may affect treatment? Are these tested automatically or do they have to be asked for?

What other drugs may be available in the UK - I can only see these on NICE guidelines for this condition? I note however that the newest drug idelasibib is not available through UK NHS for SMZL, but only CLL. Why would this be?

How do these conditions relate or differ?

[Judyanne]

Hi, The different lymphomas are diagnosed by the cd markers in flow cytometry. I was diagnosed with Sll. However, a biopsy revealed most likely extranodal marginal zone. New diagnosis is Sll and/or enmzl. However, 2nd opinion also added smzl. The marginal zone lymphomas are not as common as CLL and therefore not as many trials and drug approvals as CLL. I have bright cd 20 in one clone and have had a significant response to obinutuzumab, the next generation monoclonal antibody to rituxan. I would ask what they are basing the diagnosis of smzl on. Based on what I read, Ibrutinib is only 50% effective in extranodal marginal zone. It may be the same for smzl. I would look at the studies.

[Judyanne]

Hi, There is a 2018 ASH abstract on smzl characteristics. You can find this by going on the IELSG (International extranodal Lymphoma Study Group) website.

[lukidude in reply to Judyanne]

Thanks Judyanne, will take a look. I guess from that it is possible to get more than one condition at the same time. How high, in the scheme of things is 128 to the power of whatever? Could that be a sign of it becoming aggressive?

[lukidude]

Thanks for that tip, I will change the setting

[SuzeJc]

Hi, how is your relative doing? Did the access other treatments in the UK? I didn't know ibruitinib was available for SMZL in the UK except on trial. I'm also in the UK and having concerns about the difference in drug approvals between CLL and SMZL