About werlhof disease
What is werlhof disease?
Idiopathic thrombocytopenic purpura (ITP) is a not infrequent autoimmune bleeding disorder characterized by the abnormally low levels of blood cells called platelets, creating a condition known as thrombocytopenia. Platelets are specialized blood cells that help prevent and stop bleeding by inducing clotting. In many ITP cases, there are no readily apparent causes or underlying disease (idiopathic), but frequently there are associated collagen vascular diseases or underlying neoplasms, most frequently lymphoid. The cells of the immune system, lymphocytes, produce anti-platelet antibodies that attach to the platelets. The presence of antibodies on platelets leads to their destruction in the spleen. The disorder is characterized by abnormal bleeding into the skin resulting in bruising, which is what the term purpura means. Bleeding from mucous membranes also occurs, and may subsequently result in low levels of circulating red blood cells (anemia).
ITP presents as a brief, self-limiting form of the disorder (acute ITP) or a longer-term form (chronic ITP). Acute ITP accounts for about 50% of cases, and chronic ITP accounts for the remainder. Eighty percent (80%) of the children with ITP have the acute form while the chronic form affects mostly adults. The acute form usually resolves without treatment (spontaneously) within three to six months. When thrombocytopenia lasts for more than six to 12 months, ITP is classified as the chronic form. Onset of acute ITP is often rapid, while the onset of the chronic form may be gradual.
What are the symptoms for werlhof disease?
Small red bumps on your skin
One of the most common symptoms of ITP is a skin condition called petechiae. These are small red bumps on your skin caused by bleeding from underneath.
Petechiae can look like a red rash at first, but the bumps are slightly raised, scattered, and the size of pinpoints. They can also have a purplish tinge.
If you notice petechiae or any unusual rash, see a healthcare professional for diagnosis and treatment.
Bruises are the result of injuries that cause capillaries to break just underneath the surface of the skin. Blood comes out of these broken capillaries, forming a pool. This creates -and-blue bruises.
Bruises become yellow and fade over time as the blood reabsorbs into the body. They’re not usually cause for concern.
There can be many reasons for bruising, including:
- thinning of the skin, like from aging or another cause
- herbal supplements
- sun damage
However, you might notice you’re bruising a lot more easily, especially on your arms and legs. You might even wake up with bruises, or get new bruises without having been hurt.
Sometimes bruising easily is a symptom of ITP. Low levels of blood platelets can make it more difficult for your blood to clot, causing the skin to bruise even after a minimal injury.
Bruises from ITP are called purpura. They’re usually purple. They can even appear inside your mouth.
Bleeding from your gums
When we think of the word “skin,” the gums may not come to mind at first. But ITP can affect this delicate skin too.
According to the National Heart, Lung, and Blood Institute, gum bleeding from ITP may occur during dental work, like a routine teeth cleaning or other dental procedures.
In severe cases, your gums could bleed from brushing and flossing.
You may attribute this bleeding to gingivitis (gum inflammation) instead of an ITP symptom. At your cleanings, your dentist may also notice bruises inside your mouth.
Nosebleeds (epistaxis) occur when the delicate skin inside your nose bleeds. Nosebleeds can be relatively common and have a variety of causes, including:
- sinus infections
- blood-thinning medications
If you find yourself having many weekly nosebleeds despite being well and keeping your nose moist, see a healthcare professional to determine the cause and get treatment.
Low platelet counts from ITP can affect your menstrual cycle, making periods heavier than usual.
While heavy periods might seem more like a nuisance than anything else, they can also lead to complications, such as anemia.
An occasional heavy menstrual cycle might not be cause for concern. But if you begin experiencing a heavy period every month, see a healthcare professional.
Blood in your urine or stools
The low platelet count associated with ITP can also cause bleeding in your urine or stools. At first, you might mistake urine in the blood as a sign of infection. However, bladder infections often accompany other symptoms, such as:
- lower back or flank (kidney) pain
- frequent urination
- abdominal pain
Blood in the stool is never normal. If you see blood in your urine or stools, follow up with a healthcare professional. It could be a sign of ITP.
ITP can also cause small lumps underneath the skin. These are called hematomas. They’re made of areas of clotted blood that pooled in the tissue deep underneath your skin.
A hematoma is a type of deep bruise. It typically only occurs with a moderate injury.
See a healthcare professional for any bumps on the skin or bumps within deeper tissues.
Red blood cells transport oxygen throughout your body, which helps your tissues and organs function properly. When you bleed, your body takes time to replace the blood.
In cases of moderate bleeding, this can lead to Anemia due to fewer-than-needed red blood cells. You might feel overly fatigued as a result.
Signs of excessive Fatigue include:
- needing more sleep than usual
- requiring daytime naps
- feeling tired during the day despite getting a good night’s sleep
- overall lack of energy for everyday activities
If you have any signs or symptoms of anemia, see a healthcare professional for diagnosis and treatment.
Low mental energy
Excessive Fatigue can affect more than your physical energy. It can affect your mental energy, too.
This may result in difficulties in school, work, or your social life. It can also lead to Depression and Anxiety when not treated.
What are the causes for werlhof disease?
Immune thrombocytopenia usually happens when your immune system mistakenly attacks and destroys platelets, which are cell fragments that help blood clot. In adults, this may be triggered by infection with HIV, hepatitis or H. pylori — the type of bacteria that causes stomach ulcers. In most children with ITP, the disorder follows a viral illness, such as the mumps or the flu.
What are the treatments for werlhof disease?
People with mild immune thrombocytopenia may need nothing more than regular monitoring and platelet checks. Children usually improve without treatment. Most adults with ITP will eventually need treatment, as the condition often becomes severe or long term (chronic).
Treatment may include a number of approaches, such as medications to boost your platelet count or surgery to remove your spleen (splenectomy). Talk with your doctor about the risks and benefits of your treatment options. Some people find that the side effects of treatment are more burdensome than the effects of the disease itself.
Your doctor will talk with you about over-the-counter medications or supplements you take and whether you need to stop using any that might inhibit platelet function. Examples include aspirin, ibuprofen (Advil, Motrin IB, others) and ginkgo biloba.
If your condition is severe or persists despite initial drug treatment, your doctor may suggest surgery to remove your spleen. This quickly eliminates the main source of platelet destruction in your body and improves your platelet count, though it doesn't work for everyone. Living without a spleen permanently increases your susceptibility to infection.
Although rare, severe bleeding can occur with ITP. Emergency care usually includes transfusions of platelet concentrates. Steroids and immune globulin may also be given through a tube in a vein.
What are the risk factors for werlhof disease?
ITP is more common among young women. The risk appears to be higher in people who also have diseases such as rheumatoid arthritis, lupus and antiphospholipid syndrome.
Is there a cure/medications for werlhof disease?
Medications to treat ITP may include:
- Steroids. Your doctor will likely start you on an oral corticosteroid, such as prednisone. Once your platelet count is back to a safe level, you can gradually discontinue taking the drug under the direction of your doctor. Long-term use of these medications isn't recommended because they can increase your risk of infections, high blood sugar and osteoporosis.
- Immune globulin. If corticosteroids don't help, your doctor may give you an injection of immune globulin. This drug may also be used if you have critical bleeding or need to quickly increase your blood count before surgery. The effect usually wears off in a couple of weeks.
- Drugs that boost platelet production. Medications such as romiplostim (Nplate) and eltrombopag (Promacta) help your bone marrow produce more platelets. These types of drugs can increase your risk of blood clots.
- Other drugs. Rituximab (Rituxan, Truxima) helps increase your platelet count by reducing the immune system response that's damaging your platelets. But this drug also can reduce the effectiveness of vaccinations, which may be needed if you later choose surgery to remove your spleen.