My father recently died suddenly of "cold agglutinis" which developed spontaniously. Since then I've been trying to find out as much information as I can about cold agglutinins. So far I haven't found anything that links cold agglutinins to death so I am still confused about how this happened- does anyone know more about this topic?

First, I'm really sorry to hear about your father.
That is horrible. Did he have any other compounding
factors besides the anemia? Apparently his was not
a chronic problem, but acute and severe.

My wife has the "mixed case" autoimmune hemolytic
anemia so we know a little about the cold antibodies
(IgM). IgM disease is usually not as severe as
warm antibody disease (IgG) but when it is it can
be devastating because the treatments don't always
work. IgG is confidentally treated with steroids but
IgM doesn't respond.

My wife was given Cytoxan (chemotherapy) and plasma
exchange for the IgM. I know other folks who have
had a monoclonal antibody that seems to work. Blood
transfusions are also used. In my wife's case a
splenectomy has so far been a "cure", her hemoglobin
has been normal for 1.5 years after she almost died
from very low hgb levels (as low as 3.9 g/dl).

There is very little that is known about the whys and
hows of IgM disease. It is often associated with
other diseases like CLL (leukemia), lupus, lymphoma,
and other autoimmune illnesses.

The book "Immune Hemolytic Anemias" by Lawrence
Petz and George Garraty is an excellent source on
the subject.

Hope this helps a little.

Thanks for responding- I haven't heard from any one else who knows anything about the subject and my father's doctor doesn't seem to want to explain to me how it could have killed him. I know they gave my father steroids and a platelet transfusion in the hospital, but I guess they didn't work. They tested him for everything thoug- leukemia, etc and found nothing. No, he had never been diagnosed with any blood antibodies before, so it was a very sudden case. I will look for the book you suggested and I hope your wife is doing well.

My husband was diagnosed with Evans syndrome. He developed pulmonary embolisms and thrombo phebitis. We found out this was caused from cold aggutins. Unfortunately we have not had much success in getting him into remission. He is currently on danazol, cytoxin, prenisone and he gets procrit shots and IVIG every three weeks. He is averaging three blood transfusions every other week. Has anyone been diagnosed with Autoimmune hemolytic anemia with subsequent thrombocytopenia that has cold aggutins? If so have you been successful in slowing the rate of hemolysis?

So, the Evan's syndrome is IgM mitigated ONLY? Or
does he also have IgG? I presume only IgM because
the first thing they look for is warm antibody
hemolytic anemia.

IgM antibodies are a tough nut to crack. What
are your husband's hemoglobin counts running
at? How low are the platelets?

Christina had Evan's syndrome with both warm
and cold (IgG and IgM) antibodies. Splenectomy
has apparently (knock on wood) "cured" her. The
surgery might help your husband's platelets
but almost surely would NOT help the IgM.

There is one treatment that I notice is missing
on your list and you should talk with your
doc's about it. It is called "rituxan".
It is a monoclonal antibody for treating
lymphoma. I know a woman who has taken it for
cold antibody and she raised her counts from
around 6.0 to 8.5 - 9.0 range. Thus it hasn't
been a total cure, but it helped. It is not a
chemotherapy so the side effects would be much
more tolerable than Cytoxan.

My email is georgef@cadence.com. Feel free to
drop me a line if you would like more info.

Best of luck,

George F.

Does anyone know of an organization who does research specifically on cold agglutinins? I have looked for such a thing online and at the cdc- but haven't found anything...

What are cold agglutinins?

I don't think there is an organization devoted
to researching hemolytic anemia at any level.
I'm certainly no doctor, but from my personal
research it appears that there isn't a lot of
treatment oriented work regarding the disease either.
There are a lot of papers though about case
studies, so some researchers are interested in this.
Most hematologists/oncologists run into IgM disease
when they treat patients with "secondary" hemolytic
anemia -- usually those patients have a primary
cancer like lymphoma or leukemia. Sometimes they
have a host of other autoimmune problems like lupus,
rh. arthritis, sjorgens, etc. etc.

Interestingly enough there seems to be a lot more
info on hemolytic anemia in dogs than in people.
The canine version is much more common than the
human version.

In response to the last post, "cold agg." apparently
refers to the temperature in the test tube at
which the antibodies react with the antigens.
They "agglutinate" or clump onto the red cells when
the temperature hits a certain point. I've read
that many people will have hemolysis if their blood
temperature gets very low, but the problem becomes
a disease when the reaction occurs at body
temperature.

The "cold" antibody disease usually is mitigated
by IgM antibodies whereas "warm" antibody disease
(more common) is mitigated by IgG antibodies.

Again, this is my layman's understanding (shaky).
This is difficult subject material and we've run into
many doctors who only had a vague understanding of
it. If you are facing this disease make sure to find
some doctors who have treated as many patients as you
can with the same thing.

Good luck.

My freind is in the hospital with severe anemia...headaches...coughing that also produces dizziness....and blood in his stool thats pretty severe. He had to have 4 units of blood cuz his rbc count was 4.8 when he went in 5 dayz ago. Now it is up to 8.4 They have done every GI test and there is no obvious bleeding site.......plus he has cold agglutinins........and a history of thyroid problems that he never followed up on or took the meds for. He is also a heavy drinker and might have some mild liver abnormalities. Does anyone have any idea what could be wrong......the DRS are even confused!!!!!!!! they said they found a very small diverticula..whatever that is!...Thankyou for any input
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I've been diagnosed with Hemolytic Anemia with Cold Agglutinins. I've been on 60 mg. of Prednisone for 40 days. I also had 3 blood transfusions. The Drs. don't know what caused this, but I had Ideopathic Thrombocytopenia Purpura (ITP) 21 years ago, so I wonder if there is a connection.

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MysticMerrie

I am wondering if all this IgM talk means the IgM level was low?
I recently saw an allergist because of recurrent sinus and respitory infections and he did a bunch of tests and found i had a very low IgM level . then he sent me for more bloodwork(8 tubes geeez) and now i get a call saying i need to see an Oncologist/hematologist. I also have ITP and had my spleeen removed because of it a few other things i have is diabetes,fibromyalgia, back problems and i had EBV years ago. I am confused about the IgM levels and was wondering if anyone can help me with this.

Onisa,

Either diverticuli or hemolytic anemia could cause
the low hgb counts. 4.8 is pretty low. My wife
bottomed out at 3.9 back when she had AIHA and
she was a sick girl at that point. I presume they
have done a DAT to test for IgG (if it is present
it can be treated with prednisone).

Merrie,

Your docs probably told you that the presence of
hemolytic anemia and thrombocytopenia is called
"Evan's Syndrome" and the thinking is the symptoms
are a manifestation of a single causal factor.
From my studies, prednisone is *NOT* a treatment
for cold agglutinans. Did you also test positive
for warm antibody AIHA? Probably so. If that is
the case you have the "mixed case", same exact
thing my wife had. In her case a splenectomy
"cured" her, she is 2 years past the surgery with
no relapse. If you can't get down to less than
10 mg/day of prednisone and a normal hgb count I
would recommend trying the surgery if they suggest
it. However, it doesn't work on everybody, we know
a couple of people who relapsed after the operation.
Besides the surgery my wife took Cytoxan and had
plasma exchange to treat the anemia.

SC,

The talk of cold agglutinins (IgM antibodies)
here refers to "too many" of them rather than too few.
In IgM mitigated AIHA, the IgM become self
sensitised, i.e. they are "auto-antibodies" that
attack the body's healthy red blood cells along
with invading germs.

I would ask the doctor if the lack of a spleen
is the cause of your low IgM count. The spleen
is part of the immune complex and makes all sorts
of antibodies. It could be that your low IgM count
is now normal for you and when you get sick your
other immune system components kick in to take
care of things. I'd have the full workup to
rule everything out, but at the same time
not worry too much about it unless
it is making you sick.

thats the problem i have been sick almost non-stop since september of last year. thats why they started doing all these bloodtests. But thank you for your reply it did clear some things up for me. I go to the oncologist/hematologist on the 28th of this month will find out more then.

My father recently died suddenly of "cold agglutinis" which developed spontaniously. Since then I've been trying to find out as much information as I can about cold agglutinins. So far I haven't found anything that links cold agglutinins to death so I am still confused about how this happened- does anyone know more about this topic?

From what I was told by the doctors at the U of W, a person with cold agglutinin disease can die of a heart attack if they do not stay warm and are exposed to cold weather for a long period of time. They told me of a guy that had a flat tire in a snow storm and got out to change it without wearing proper clothing and he had a heart attack from loosing so many red blood cells. When the ears, nose or fingers get cold, the red blood cells stick together and the liver eats them and spits them out in the urine. I have the disease and have been fighting it for a year. I am sorry for your loss.

Onisa,

Either diverticuli or hemolytic anemia could cause
the low hgb counts. 4.8 is pretty low. My wife
bottomed out at 3.9 back when she had AIHA and
she was a sick girl at that point. I presume they
have done a DAT to test for IgG (if it is present
it can be treated with prednisone).

Merrie,

Your docs probably told you that the presence of
hemolytic anemia and thrombocytopenia is called
"Evan's Syndrome" and the thinking is the symptoms
are a manifestation of a single causal factor.
From my studies, prednisone is *NOT* a treatment
for cold agglutinans. Did you also test positive
for warm antibody AIHA? Probably so. If that is
the case you have the "mixed case", same exact
thing my wife had. In her case a splenectomy
"cured" her, she is 2 years past the surgery with
no relapse. If you can't get down to less than
10 mg/day of prednisone and a normal hgb count I
would recommend trying the surgery if they suggest
it. However, it doesn't work on everybody, we know
a couple of people who relapsed after the operation.
Besides the surgery my wife took Cytoxan and had
plasma exchange to treat the anemia.

SC,

The talk of cold agglutinins (IgM antibodies)
here refers to "too many" of them rather than too few.
In IgM mitigated AIHA, the IgM become self
sensitised, i.e. they are "auto-antibodies" that
attack the body's healthy red blood cells along
with invading germs.

I would ask the doctor if the lack of a spleen
is the cause of your low IgM count. The spleen
is part of the immune complex and makes all sorts
of antibodies. It could be that your low IgM count
is now normal for you and when you get sick your
other immune system components kick in to take
care of things. I'd have the full workup to
rule everything out, but at the same time
not worry too much about it unless
it is making you sick.

Hi I was just reading your post re: your wife hope she is doing good it sounds like it. You mentioned that she had a splenectomy, I am having my removed on Wed. due to hemolytic anemia. I have been taking prednisone since Aug. and have had hemoglobin drip, procrit shots, but nothing has brought my blood counts up I am holding @ 8.9 so I have decided to give up my spleen. Hope I this will be my "Cure" :)