Hello, I was diagnosed with MGUS last May. I was discovered to have cold agglutinin disease (CAD) after I gave a blood donation, and I was referred by my primary to a hematologist, who diagnosed me with autoimmune hemolytic anemia (AIHA) and MGUS.
I am IgG lambda and my M-spike as of that time was 1.3 g/dL (13 g/L), with the uninvolved immunoglobulins coming in low. My light chain ratio was 0.03 at that time and a bone marrow draw had me at 5-10%.
My AIHA is mild, with hemoglobin around 14 and RBC and hematocrit just into low. My platelets have been dropping over the past year and the last draw in December had them at 126. My spleen is mildly enlarged and I take folic acid and Centrum every day to help with blood production.
The hematologist told me I was high risk for progression at my September appointment. As I can tell so far, I have no genetic markers that would place me at high risk.
I would be interested to hear from anyone who has AIHA or CAD with MGUS and learn of their experience.
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Jer610 - Name: Jerry
- Who do you know with myeloma?: self
- When were you/they diagnosed?: 5/2014
- Age at diagnosis: 66
Re: MGUS with CAD and AIHA
Jerry,
Sorry, I can't help you with your question regarding CAD and AIHA, since I've never heard of these diseases. But do you know why your doc said you have a high risk for progressing? The Mayo Clinic uses the following three items for classifying the risk of progression for MGUS patients.
1. Non-IgG isotype
2. Serum M protein greater than 1.5 g/dL
3. Kappa / lambda or lambda/kappa ratio being skewed, or out of normal range.
For patients with 0 of these major risk factors, the absolute risk of progression at 20 years is 5%. For patients with 1, the risk is 21%. For those having 2 of these conditions, it's 37%, and for those meeting all three of those criteria, it's 58%.
From what you stated here, you only meet one of these criteria (abnormal FLC ratio). Did your M-spike also go over 1.5 g/dL?
I believe that there is currently no documented correlation between cytogenetics and the risk of progression of an MGUS patient.
Sorry, I can't help you with your question regarding CAD and AIHA, since I've never heard of these diseases. But do you know why your doc said you have a high risk for progressing? The Mayo Clinic uses the following three items for classifying the risk of progression for MGUS patients.
1. Non-IgG isotype
2. Serum M protein greater than 1.5 g/dL
3. Kappa / lambda or lambda/kappa ratio being skewed, or out of normal range.
For patients with 0 of these major risk factors, the absolute risk of progression at 20 years is 5%. For patients with 1, the risk is 21%. For those having 2 of these conditions, it's 37%, and for those meeting all three of those criteria, it's 58%.
From what you stated here, you only meet one of these criteria (abnormal FLC ratio). Did your M-spike also go over 1.5 g/dL?
I believe that there is currently no documented correlation between cytogenetics and the risk of progression of an MGUS patient.
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Multibilly - Name: Multibilly
- Who do you know with myeloma?: Me
- When were you/they diagnosed?: Smoldering, Nov, 2012
Re: MGUS with CAD and AIHA
Hello Multibilly, I've read many of your posts over the past months and greatly enjoy your input.
Your question about high risk is a good one. I think it stems from this: CAD and AIHA are rarities that usually arise after treatment for CLL and very unusually arise before or at the same time as a gammopathy. AIHA involves IgG attacking the red cells and sometimes platelets, as in my case. CAD involves IgM and, very less often, IGG. The gammopathy slowly crowds my bone marrow while my marrow is trying to compensate for the increased hemolysis. Somewhere along the line something has to give.
As I understand it, if my counts drop to levels that my physician deems needs treating, and it is due to AIHA, he will start me on usually prednisone. Chemo treatment could then be an event that accelerates the growth of the gammopathy.
If, on the other hand, the plasma cell proliferation affects blood cell production, the crash would be a CRAB event.
I'm not sure if I've got this all completely right, but, right now, I'm feeling good and don't have to see my doc until the end of June. I have a myeloma panel and CT scan already in the pipeline for the middle of June, and we'll see where we're at from there.
Your question about high risk is a good one. I think it stems from this: CAD and AIHA are rarities that usually arise after treatment for CLL and very unusually arise before or at the same time as a gammopathy. AIHA involves IgG attacking the red cells and sometimes platelets, as in my case. CAD involves IgM and, very less often, IGG. The gammopathy slowly crowds my bone marrow while my marrow is trying to compensate for the increased hemolysis. Somewhere along the line something has to give.
As I understand it, if my counts drop to levels that my physician deems needs treating, and it is due to AIHA, he will start me on usually prednisone. Chemo treatment could then be an event that accelerates the growth of the gammopathy.
If, on the other hand, the plasma cell proliferation affects blood cell production, the crash would be a CRAB event.
I'm not sure if I've got this all completely right, but, right now, I'm feeling good and don't have to see my doc until the end of June. I have a myeloma panel and CT scan already in the pipeline for the middle of June, and we'll see where we're at from there.
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Jer610 - Name: Jerry
- Who do you know with myeloma?: self
- When were you/they diagnosed?: 5/2014
- Age at diagnosis: 66
Re: MGUS with CAD and AIHA
Wow, that is a nasty brew of several things all going on at the same time. I am impressed that your doctors could diagnose all three of these things as separate, but concurrent afflictions. Hope you find some fellow patients with similar conditions that can share their experiences.
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Multibilly - Name: Multibilly
- Who do you know with myeloma?: Me
- When were you/they diagnosed?: Smoldering, Nov, 2012
Re: MGUS with CAD and AIHA
Jerry - I would have to agree with Multibilly: That's a whole lot!
I would be interested to see if anyone else here in the forums is familiar with your condition(s).
Just the same, let's hope for the best. Keep well-monitored and check anything out that seems concerning.
I would be interested to see if anyone else here in the forums is familiar with your condition(s).
Just the same, let's hope for the best. Keep well-monitored and check anything out that seems concerning.
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Toni - Name: Toni
- Who do you know with myeloma?: self - MGUS
- When were you/they diagnosed?: April 2014
- Age at diagnosis: 51
Re: MGUS with CAD and AIHA
As the other posts have stated, it is rare to have MGUS associated with cold agglutinin-associated AIHA (autoimmune hemolytic anemia). However, it is not without precedent.
More typically, however, cold agglutinin AIHA is associated with other indolent B cell malignancies (lymphoplasmacytic lymphoma / Waldenström macroglobulinemia [LPL/WM], marginal zone lymphoma, and CLL, as stated). However, there are case reports describing AIHA in multiple myeloma patients, stating up to 10% of multiple myeloma patients with significant anemia (hemoglobin < 6) may have AIHA. And that, in these cases, the AIHA was related to the involved paraprotein (M-protein).
With you normal hemoglobin, it seems a bit odd that you were diagnosed with cold agglutinin AIHA. I would suggest that the labs be repeated and, if not confirmed, it introduces the potential of false positive results secondary to the paraprotein. But keep an eye out for signs and symptoms of CAD, such as something called livedo reticularis (web-like rash on legs) and acrocyanosis (blue-white discoloration of fingers or toes). Right now there does not appear to be anything to worry about.
With the MGUS, continue to monitor semi-annually and consider annual bone surveys. There is a 1% annual progression-to-MM rate. And, as stated above (at least based on what we see), your risk is not overwhelmingly high (low in fact). However, you still must watch.
Best of luck and keep us updated.
More typically, however, cold agglutinin AIHA is associated with other indolent B cell malignancies (lymphoplasmacytic lymphoma / Waldenström macroglobulinemia [LPL/WM], marginal zone lymphoma, and CLL, as stated). However, there are case reports describing AIHA in multiple myeloma patients, stating up to 10% of multiple myeloma patients with significant anemia (hemoglobin < 6) may have AIHA. And that, in these cases, the AIHA was related to the involved paraprotein (M-protein).
With you normal hemoglobin, it seems a bit odd that you were diagnosed with cold agglutinin AIHA. I would suggest that the labs be repeated and, if not confirmed, it introduces the potential of false positive results secondary to the paraprotein. But keep an eye out for signs and symptoms of CAD, such as something called livedo reticularis (web-like rash on legs) and acrocyanosis (blue-white discoloration of fingers or toes). Right now there does not appear to be anything to worry about.
With the MGUS, continue to monitor semi-annually and consider annual bone surveys. There is a 1% annual progression-to-MM rate. And, as stated above (at least based on what we see), your risk is not overwhelmingly high (low in fact). However, you still must watch.
Best of luck and keep us updated.
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Dr. Ken Shain - Name: Ken Shain, M.D., Ph.D.
Beacon Medical Advisor
Re: MGUS with CAD and AIHA
Thank you for replying Dr. Shain.
Our local blood bank contacted me after donating platelets saying that my platelet count was too low for a good donation. Several days after that, a physician from the blood bank called me and said that my whole blood was thickening at lab temperatures and said that I had CAD. He sent me a letter and I took it to my primary, who sent me to a hematologist who diagnosed AIHA and MGUS from his labs. I don't believe that my hematologist did a screening for CAD once he arrived at AIHA.
All said, I've felt none the worse for it all, feel very healthy, and I'll take that every time.
Also, thank you Multibilly and Toni for your input.
Our local blood bank contacted me after donating platelets saying that my platelet count was too low for a good donation. Several days after that, a physician from the blood bank called me and said that my whole blood was thickening at lab temperatures and said that I had CAD. He sent me a letter and I took it to my primary, who sent me to a hematologist who diagnosed AIHA and MGUS from his labs. I don't believe that my hematologist did a screening for CAD once he arrived at AIHA.
All said, I've felt none the worse for it all, feel very healthy, and I'll take that every time.
Also, thank you Multibilly and Toni for your input.
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Jer610 - Name: Jerry
- Who do you know with myeloma?: self
- When were you/they diagnosed?: 5/2014
- Age at diagnosis: 66
Re: MGUS with CAD and AIHA
I was wondering if I could get some input concerning my labs. As stated previously, I was diagnosed with MGUS in May of 2014, I initially had two myeloma panels done about 7 weeks apart and a CT scan. In June 2015 I had my first myeloma panel and CT scan in a year. These are my results from all three panels:
Apr 30, 2014 Jun 17, 2014 Jun 9, 2015
IgA 44 40 36 mg/dL
IgG 1740 1557 1817
IgM 43 41 39
M-spike 1.3 1.3 1.3 g/dL
Kappa 4.2 6.12 7.07 mg/l
Lamba 198.88 186.08 216.67
KL ratio 0.02 0.03 0.03
Do these labs suggest hypogammaglobulinemia? If so, does my diagnosis of AHIA together with being IgG anti-platelet positive with splenomegaly suggest a diagnosis of common variable immune disease?
As mentioned previously, my anemia has been mild and my labs going back four years are very consistent, although there has been a gradual increase in reticulocyte count (4.6%) and my bilirubin is just into high for the past six months. My latest CT scan noted emphysema pattern in my centrilobular lung and no masses of neck, chest, or pelvis.
The reason I am asking these questions is because I have thought of my MGUS in terms of pre- multiple myeloma. I did not think of asking my hematologist about this in June and it only occurred to me after reading several other threads on this site concerning suppressed immunoglobulins.
Apr 30, 2014 Jun 17, 2014 Jun 9, 2015
IgA 44 40 36 mg/dL
IgG 1740 1557 1817
IgM 43 41 39
M-spike 1.3 1.3 1.3 g/dL
Kappa 4.2 6.12 7.07 mg/l
Lamba 198.88 186.08 216.67
KL ratio 0.02 0.03 0.03
Do these labs suggest hypogammaglobulinemia? If so, does my diagnosis of AHIA together with being IgG anti-platelet positive with splenomegaly suggest a diagnosis of common variable immune disease?
As mentioned previously, my anemia has been mild and my labs going back four years are very consistent, although there has been a gradual increase in reticulocyte count (4.6%) and my bilirubin is just into high for the past six months. My latest CT scan noted emphysema pattern in my centrilobular lung and no masses of neck, chest, or pelvis.
The reason I am asking these questions is because I have thought of my MGUS in terms of pre- multiple myeloma. I did not think of asking my hematologist about this in June and it only occurred to me after reading several other threads on this site concerning suppressed immunoglobulins.
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Jer610 - Name: Jerry
- Who do you know with myeloma?: self
- When were you/they diagnosed?: 5/2014
- Age at diagnosis: 66
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