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Lymphoplasmacytic Lymphoma that is not WM?

Blood Cancers & Disorders | Last Active: May 4 12:25pm | Replies (38)

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Hi yes my wife has LPL IgG with Kappa Free Light Chains and MYD88 WT with systemic Neuropathy. She was diagnosed around 2 years ago because of the Neuropathy and it has been an absolute nightmare getting the Neuropthy linked to the LPL. She has seen the best in the UK and they said the symmetrical foot numbness was caused by a slight S1 left sided Radiculopathy. She finally has a new neurologist say that she may have sensory Neuropathy but not SFN as she has length dependant Neuropathy and this fits but the numb lips etc. don't fit as you can't have both length and non length dependant SFN, I have tried so hard to convince them this may not be actually true? and I think she actually has mixed fibre Neuropathy as there is reduced vibration senstivity in both large toes. So we thought we had got somewhere and now he says it is caused by Hypervigilance so we assume Function Neurological Disorder (FND) is what he is saying, but the symptoms just don't fit. So treatment is refused. I was hoping that she would start on Rituximab very soon then if the efficacy was not good enough to explore more intense treatment such as BR. The Neurologist said they don't want to start treatment in case they are wrong. This is a horrible illness and LPL IgG or other non IgM LPL are very poorly understood and basically ignored in the UK. I would be interest to know how others with non IgM LPL and Neuropathy presented? and how was it treated or found? she also has excess thirst nearly a gallon a day, weird transient ECG inverted T waves, and bowel issues all told not linked and skin lesions that come and go that maybe linked to her LPL but still not biopsied. Thanks Jon

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Replies to "Hi yes my wife has LPL IgG with Kappa Free Light Chains and MYD88 WT with..."

@jonyb
I can only imagine the frustration. My heart hurts for your wife, and you. It’s also difficult in the U.S. to find any studies and/or clinical trials for non IgM LPL.
I started with Rituximab by itself- the least toxic first line of treatment. I have not had any neuropathy pre/post treatment.
I wonder if high Kappa vs high Lambda light chain makes a difference in neuropathy? Or the MYD88 Wild mutation vs non Wild.
So many questions which are hopefully being addressed in the labs.
Keep advocating for your wife. Sending strength!