How Raynaud's Phenomenon and Cold Agglutinin Disease are Related
- Cold agglutinin disease (CAD) is a rare type of autoimmune disorder. With autoimmune conditions, a person's body is attacked by their own immune system.
- Raynaud’s phenomenon, on the other hand, can be a secondary condition to an autoimmune disorder like CAD.
- Similarly, symptoms of both conditions can be triggered by cold weather, hence the “cold” in cold agglutinin disease.
Similarly, symptoms of both conditions can be triggered by cold weather, hence the “cold” in cold agglutinin disease.
Learning about CAD
Read MoreRelated: Cold Agglutinin Disease (CAD): The Basics
To expand upon this condition’s relation to the “cold,” when the affected person is exposed to cold temperatures, certain proteins that normally attack bacteria (known as IgM antibodies) instead attach themselves to red blood cells and bind them together into clumps (known as agglutination), and these cells are eventually prematurely destroyed. This can lead to anemia (low red blood cells) and other issues. These symptoms may get worse due to lower temperatures.
"The treatment depends on what's causing the disease," Dr. Choi tells SurvivorNet.
Dr. Choi went on to explain more about CAD and some typical symptoms to look out for.
"Symptoms of CAD are more apparent and severe when you're exposed to cold temperature," he said. "For example, during winter time, or when you are touching something cold, it can trigger the disease to act up. So, when your anemia becomes severe, your hemoglobin goes down. Then you can have symptoms of anemia."
These symptoms may include:
- Fatigue
- Shortness of breath
- Dizziness
People with CAD may also experience symptoms associated with autoimmune disease, such as:
- Finger tips turning blue/which due to lack of oxygen
- Malaise (feeling unwell in general)
To determine how to go about treatment for CAD, first your doctor will need to figure out if an underlying conditioninfection, autoimmune disease, or blood canceris causing it. When the disease is caused by lymphoma, cancer treatment will be used to get CAD under control.
"Sometimes you can have a general unwell feeling, mostly because your oxygen level in your body is low," Dr. Choi adds.
If your doctor believes that you may have CAD, they will perform a series of blood tests to help with a diagnosis before beginning their investigation into whether there is an underlying condition causing the CAD.
The treatment options for this disorder depend on if there is an underlying condition and what that condition is. In some cases, treatment may not be necessary.
Dr. Roy Smith, a hematologist/oncologist at the University of Pittsburgh, tells SurvivorNet that in some instances, simply avoiding the cold may be all the treatment someone needs. "Often, it's a matter of just telling a person to wear gloves or wear a coat, keep their house at a higher temperature, or move to another place … something along those lines."
"The first step is to determine whether they actually need to be treated or not, says Dr. Smith.
When cold agglutinin disease is caused by a blood cancer like lymphoma, your doctor may suggest chemotherapy or a targeted therapy to get the underlying condition under control.
"You want to treat the underlying condition that's driving CAD, so the standard-of-care for that condition is a chemotherapy or therapy to target those lymphoma cells. When CAD is acting up significantly, then you want to treat the [disease] itself," Dr. Choi said.
"The first line treatment is something called Rituximab," he explained. The drug "specifically targets lymphocytes, which is a type of blood cells."
Dr. Jun Choi explains treatment options for CAD.
Dr. Choi explained that most patients respond relatively well to Rituximab, which is a type of drug called a monoclonal antibody. The drug may also be used after a relapse.
"In the minority of patients who do not respond to this standard therapy … there are many other treatment options that are being developed in clinical trials," Dr. Choi said.
Understanding Raynaud’s Phenomenon
Raynaud's phenomenon is a condition that causes decreased blood flow to the fingers, according to John Hopkins Medicine. In less common cases, there can also be a decreased blood flow to the toes, ears, nose, nipples, or knees. Spasms of the blood vessels in those areas cause this “phenomenon” to occur. Just like with many other conditions, the spasms can be triggered in response to stress or, as we mentioned, cold weather.
Raynaud's can be present on its own, which is most common, or it could be a secondary condition of another autoimmune disease, or connective tissue disease, which is a disorder involving the tissue that supports our organs and other parts of the body.
Raynaud's, in its primary form, often begins between ages 15 and 25, and doesn’t wreak as much havoc as secondary Raynaud's.
Factors that can increase your risk of developing the condition, along with having cold agglutinin disease are:
- A connective tissue or autoimmune disease
- Chemical exposure
- Cigarette smoking
- Injury or trauma
- Repetitive actions, such as typing or use of tools that vibrate like a jack hammer
- Side effects from certain medicines
Symptoms of this condition can vary from person to person. Common symptoms include:
- Fingers that turn pale or white then blue when under stress or exposed to cold
- Hands that may become swollen and painful when warmed and/or turning red when warmed
- Sores on the finger pads in more severe cases
- Gangrene in the fingers that causes infection or needs amputation, which is rare
Managing Symptoms of Raynaud’s Disease
Unfortunately, there is no cure for Raynaud’s, but symptoms can be managed by following the below:
- Avoiding exposure to cold
- Keeping warm with gloves, socks, scarf, and a hat
- Stopping smoking
- Wearing finger guards over fingers with sores
- Avoiding trauma or vibrations to the hand (such as with vibrating tools)
- Taking blood pressure medicines during the winter months to help reduce constriction of the blood vessels
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