Author Topic: Localized AL amyloidosis of small intestine only organ effected  (Read 10913 times)

johncoaster

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  • Reason for joining: patient al amyloidosis of GI track
  • Diagnosed: october 2013
Localized AL amyloidosis of small intestine only organ effected
« on: September 30, 2015, 01:46:15 pm »
I am searching for imformation regarding AL amyloid and Localized AL amyloid and  Localized AL Systemic.- I was diagnosed in oct 2013 with amyloidosis of small intestine, jejunum  area ,the dx was made after performing a Laparotomy  resection. Most of the jejunum is affected with subserosal bleeds,  haemorrhages, ulceration, acute inflammation with intermittent areas of thickening.through most of jejunum.-The Resected specimen was stained with congo red and showed a green birefringence in polarising light. Report said -Findings in immunohistochemistry favour AL Amyloidosis. - - -- Bone marrow biopsy stated  Mild marrow plasmacytois [3%] An immunoglobulin light chain restricted B-cell not detected. Less than 1%of total cells co- express CD38 and CD138 consistant with plasma cells. Plasma cells cannot be accurately quantified by flow cytometry as large numbers of cells are lost in processing. Anyway the SIg kappa = 3% SIg lambda =1%and lots of other numbers but i think these are the main numbers.--- Blood FLC [N Latex] were -Lambda =75mg/L [ref. range 8 to 27] HIGH -- Kappa =32mg/L[ref. range 7 to 22] HIGH  --K/L Ratio =o.43 [ref. range 0.31 to 1.56]NORMAL.---So this seems like the main markers used to determin my first diognosis of amyloidosis Oct 2013 -.now they had to do MASS SPECTOMOTRY which was done in May 2014 = unequivocally AL Amyloidosis. // I was started on mel/dex in August 2014 WITH FLC IN NORMAL RANGE PRIOR TO CHEMO.- Did 5 cycles 5 mths chemo.and realised after support group imformation Why am i on chemo with NORMAL FREE LIGHT CHAINS.-I stopped chemo after asking Dr same and getting this awnser [Thats the way it is, we have to move along with this appointment or i'll be here all night, i have other patients to see] Dec 2014  Dr then wrote out script for my chemo. This appointment took not even 10 mins and i had to travel 1000 klm to see him.-- Sought another Dr after that as he had me doing 18mths chemo.----New Dr Jan 2015 said chemo would have done me no good at all and thinks the amyloid is being produced locally in small bowel and no chemo required unless i was systemic.No signs of that and had a fat biopsy to check approx May 2015 test negitive.FLC Normal /////  I would like to know if AL amyloid light chain comes from plasma cells detected in your blood which is produced in bone marrow.---How can AL be produced in localized non systemic form of amyloidosis. Like they say i'me unequivaly AL but no free light chains eg [negitive bone marrow tests;] no systemic detected eg  [no other organ involved pluss negitive fat biopsy]  --- I noted this on my Oct 2013 FLC blood investigation and at the bottom it states  [PROTEIN COMMENT -normal  K/L FLC ratio./  increased FLC concentrations can occur not only when monoclonal light chains are present but also when immunoglobulin synthesis is elevated eg autoimmune diseases or immunoglobulin excretion is reduced eg renal impairment ]--- I realy don't know what type amyloid i have just doesn't make any sence to me  - AL - AL LOCALIZED -LOCALIZED. ///Isuffer with chronic sickness not nausa but flue type illness- fatuige no energy -chronic severe pain Left side small intestines-  shortness of breath is improving -shingle type rashes,itchy, sometimes painfull red lumps hands feet abdomen scalp pubic area, -left foot pain with pins needles and colder than the other -lately my eyes feel like grit in them and vision is hazy smokey.-chronic constipation- dizziness---Ime lucky my pain is under control but the feeling so sick all the time is exausting. Would be great to hear from anyone who can shed some light on this or has amyloidosis of small intestines to compair notes treatment ect.Sorry to make such a long post and go into such details THANKING YOU ALL   John
« Last Edit: October 03, 2015, 07:13:38 pm by Miriam Vered »

Miriam Vered

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Re: Localized AL amyloidosis of small intestine only organ effected
« Reply #1 on: October 03, 2015, 07:14:05 pm »
Hello John,
Welcome to the forum. I’m very sorry to hear about your illness and the difficult time you have been through. You can ask your doctors to consult with the NAC doctors about your case. The contact details are available at: https://www.ucl.ac.uk/amyloidosis/nac
 
As a general answer to your question:
A focus of free light chain producing cells can in theory occur anywhere including potentially the small intestine. This could cause localised amyloidosis of the small intestine. This is seen more commonly in other parts of the body such as the uro-genital tract and the respiratory tract, but it could also occur in the small intestine. If free light chain concentrations in the blood and urine are normal and there is no paraprotein in the blood or urine and there is no evidence of amyloid deposits in any other organs then systemic chemotherapy would not be the appropriate treatment.
 
I hope that helps. 
 

johncoaster

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  • Reason for joining: patient al amyloidosis of GI track
  • Diagnosed: october 2013
Re: Localized AL amyloidosis of small intestine only organ effected
« Reply #2 on: October 04, 2015, 05:46:19 am »
Thank you Miriam, and thank you for excepting me to group. Also big thanks to Pat Pinchin for healping me. Miriam if you have any articles on localised could you please post, there just doesn;t seem to be much out there concerning my case and to ask the people in the know eg specialists one has to wait untill there next appointment .in some cases that could be 6 mths. I was told at one time not long ago that there are cases of patients with AL that FLC levels normal and do not show up in bloods ect, as you stated and this makes it hard to treat with chemo as they dont have any markers to go on eg are FLC levels falling or not.This accours in approx 3%of patients. I would like to know as much as i can to try understand what is what and maybe avoid misstakes eg [chemo for non systemic ALamyloidosis.]  Can misstakes be made with typing eg AL or AA ,one has to ask ones selfthese questions,-.Do my symptoms fit my condition and if not why.- I seen another patient on a group with chrones causing his symptoms one has to be on the ball as,-[ far to often doctors seem to put it all down to the main disease and thats that full stop.]  - Miriam can you tell me the difference between AL localized and just localized or are these the same thing. All the best to everyone, Cheers  John.

Miriam Vered

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Re: Localized AL amyloidosis of small intestine only organ effected
« Reply #3 on: October 06, 2015, 11:56:20 am »

Quote
Miriam if you have any articles on localised could you please post


We have a patient information sheet on localised amyloidosis - available here.
A recent paper published by the NAC doctors, available here, summarised results for 606 patients treated at the NAC for localised amyloidosis over a period of 30 years.  These results were very reassuring as only one of these patients progressed to systemic AL amyloidosis and there were no deaths from progressive amyloidosis.


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Can misstakes be made with typing eg AL or AA


The NAC doctors and laboratory staff can review biopsy material from other centres to check that amyloid has been typed correctly. You can ask your doctors to contact the NAC doctors to enquire about this possibility.


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Do my symptoms fit my condition and if not why


The NAC doctors will be happy to discuss your case with a doctor who has seen you and who has access to your medical records. I'm sorry to keep repeating myself but that is really the best advice I can give on this forum.


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can you tell me the difference between AL localized and just localized or are these the same thing


Localised amyloidosis means amyloid deposits just affecting one organ. Localised amyloid is usually of AL type, but may sometimes be of a different type.



Localised AL amyloidosis is caused by a local focus of plasma cells producing amyloidogenic light chains that produce amyloid deposits just in one place.


The patient information sheet discusses this in more detail.


I hope this helps.


johncoaster

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  • Reason for joining: patient al amyloidosis of GI track
  • Diagnosed: october 2013
Re: Localized AL amyloidosis of small intestine only organ effected
« Reply #4 on: October 07, 2015, 06:36:12 am »
Thank you once again Miriam, I am not that great with words, you organized my post in a manner where it is so easy to read.and your awnsers were straight to the point. Fantastic. Thank you for the article on localized ,it was of a great help to me and hopefully to others.   john.

Mhelenx

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  • Localised paranasal amyloidosis, systemic AL type
  • Reason for joining: Localised paranasal AL Amyloidosis, patient
  • Diagnosed: 2005
Re: Localized AL amyloidosis of small intestine only organ effected
« Reply #5 on: October 20, 2015, 12:53:39 am »
Hi John, I also have Localised AL Amyloidosis but in the Paranasal Sinuses. My bone marrow, fat biopsy, Echo and many other scans and tests were negative to systemic involvement. My recent Lambda=2.6mg/L [ref. range 5.7 to 26.3] --Kappa=6.7mg/L[ref. range 3.3 to 19.4]  --FLC Ratio =2.58 [ref. range 0.26 to 1.26] RAISED. I am due to start Chemotherapy with Velcade and Dexamethasone to help reduce the inflammatory/cellular infiltrate although speculative before sinus surgery. I too remain puzzled as to why I have systemic type Amyloidosis in a localised presentation. Although I have Asthma and IBS, but as far as I am aware they are not caused by my AMY. I live in chronic pain head, neck and facial pains which is still not under control. Good luck, I hope you find some answers soon.
MhelenX✨

johncoaster

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  • Posts: 15
  • Reason for joining: patient al amyloidosis of GI track
  • Diagnosed: october 2013
Re: Localized AL amyloidosis of small intestine only organ effected
« Reply #6 on: October 22, 2015, 03:06:04 am »
Hi Mhelenx, thank you for reply,it seems strange if we are localized, why treat with chemo, i can understand the steriod to reduce inflamation.I am so sorry to hear the pain you are going through.Maybe the steriods might releive such pain with reduction of inflamation. Three weeks ago i asked my local DR to try prednislone steriod to see if it would reduce pain in my GI,  it has helped. -.Are you on any treatment for IBS and pain releif for head neck and face. I watched a video on myloma tv that dear Pat posted and it mentioned about coat hanger pain in the back of neck i found this vidio very helpfull.-- I noticed your raised FLC ratio i think this is the important one they use for monitoring.ect.Maybe they want that in normal range hence chemo.All the best keep in touch,-  john.

Mhelenx

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  • Posts: 24
  • Localised paranasal amyloidosis, systemic AL type
  • Reason for joining: Localised paranasal AL Amyloidosis, patient
  • Diagnosed: 2005
Re: Localized AL amyloidosis of small intestine only organ effected
« Reply #7 on: October 22, 2015, 07:49:12 pm »
Hi John, I think Chemo is the last straw option to try and reach the amyloid producing cells and DEX for the inflammation. I have a lot of antibiotics per year with steroids, this gives me a couple of weeks temporary relief then instead of improving, my condition is worsening. The only pain relief I take is soluble paracetamol and cyclizine for nausea  as it works quicker and more effectively than the tablets. I tried codeine phosphate but stopped as I get nausea and vomitting and it doesn't ease the symptoms. I am still waiting for a referral to a pain clinic as I am struggling with the pain. I also have a low tolerance to medication, possible because I have gastric reflux disease so it is trial and error to find something I can take without adverse side effects. I treat my IBS with food by monitoring what I eat from experience and use Imodium as a backup. My raised FLC ratio puzzles me as my Kappa and Lambda readings are normal. I am researching FLC ratio to try and understand exactly what it all means. All the best to you too John and stay in touch.
MhelenX✨

Miriam Vered

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Re: Localized AL amyloidosis of small intestine only organ effected
« Reply #8 on: October 23, 2015, 05:10:34 am »
Hi Helenx,


A good explanation of FLC ratios was posted on the forum by Joca within the discussion of FLC ratios here (about half way down the page). She also linked to a YouTube video that you may find helpful.


Mhelenx

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  • Posts: 24
  • Localised paranasal amyloidosis, systemic AL type
  • Reason for joining: Localised paranasal AL Amyloidosis, patient
  • Diagnosed: 2005
Re: Localized AL amyloidosis of small intestine only organ effected
« Reply #9 on: October 23, 2015, 08:22:16 am »
Thanks for the link Miriam with the FLC ratio explanation. I guess I can now deduce that the reason my FLC ratio is raised is because I have abnormal plasma cells secreting kappa chains.
MhelenX✨