Hyperviscosity Syndrome: Symptoms, Causes, Treatment

What are the symptoms of hyperviscosity syndrome?

Hyperviscosity syndrome is a rare but serious complication that can occur in people with Waldenström macroglobulinemia, a type of blood cancer. The symptoms of hyperviscosity syndrome can vary in severity and may include:

1. Vision changes: Blurred vision, double vision, or loss of vision due to increased blood viscosity (thickness) affecting blood flow to the eyes.
2. Headache: Severe, throbbing headaches due to increased blood pressure and blood vessel constriction.
3. Fatigue: Feeling extremely tired or weak due to reduced blood flow to the body’s tissues.
4. Confusion: Confusion, disorientation, or difficulty concentrating due to decreased blood flow to the brain.
5. Seizures: Seizures or convulsions can occur if the brain is deprived of oxygen due to reduced blood flow.
6. Dizziness or lightheadedness: Feeling dizzy or lightheaded due to decreased blood flow to the brain.
7. Shortness of breath: Difficulty breathing or feeling winded due to increased blood pressure and reduced oxygen delivery to the body’s tissues.
8. Palpitations: Irregular heartbeat or palpitations due to changes in blood viscosity and cardiac output.
9. Cerebral edema: Swelling of the brain tissue, which can lead to increased intracranial pressure and potentially life-threatening complications.
10. Stroke-like symptoms: Symptoms similar to those experienced during a stroke, such as weakness, numbness, or paralysis on one side of the body.

If you or someone you know is experiencing these symptoms, it is essential to seek immediate medical attention. Hyperviscosity syndrome is a medical emergency that requires prompt treatment to prevent serious complications and potentially life-threatening consequences.

What are the causes of hyperviscosity syndrome?

Hyperviscosity syndrome is a rare but serious complication that can occur in people with certain blood disorders, primarily Waldenström macroglobulinemia (WM), a type of non-Hodgkin lymphoma. The main cause of hyperviscosity syndrome is the presence of an abnormally high concentration of abnormal proteins, such as IgM antibodies, in the blood.

The specific causes of hyperviscosity syndrome are:

1. Waldenström macroglobulinemia (WM): WM is a type of non-Hodgkin lymphoma that produces excess amounts of IgM antibodies, which can increase blood viscosity.
2. Monoclonal gammopathy of undetermined significance (MGUS): MGUS is a condition characterized by the presence of an abnormal protein (monoclonal protein) in the blood, which can also increase blood viscosity.
3. Multiple myeloma: Multiple myeloma is a type of cancer that affects plasma cells, which produce antibodies. In some cases, it can lead to increased blood viscosity.
4. Polycythemia vera: Polycythemia vera is a rare blood disorder characterized by an overproduction of red blood cells, which can increase blood viscosity.
5. Dehydration: Severe dehydration can lead to increased blood viscosity due to the concentration of red blood cells.
6. Hypovolemia: Hypovolemia, or low blood volume, can also contribute to increased blood viscosity.
7. Cryoglobulinemia: Cryoglobulinemia is a rare condition characterized by the presence of abnormal proteins in the blood that are soluble only at low temperatures. These proteins can increase blood viscosity when the body temperature rises.

When these conditions are not properly managed, they can lead to an accumulation of abnormal proteins in the blood, resulting in hyperviscosity syndrome. This complication can be life-threatening if left untreated or if treatment is delayed.

How is the diagnosis of hyperviscosity syndrome made?

The diagnosis of hyperviscosity syndrome is typically made through a combination of physical examination, laboratory tests, and imaging studies. The following are the common diagnostic steps:

1. Physical examination: A thorough physical examination is performed to assess the patient’s overall condition, including blood pressure, pulse, and neurological function.
2. Complete Blood Count (CBC): A CBC is done to measure the concentration of red blood cells, white blood cells, and platelets in the blood. This test can help identify any abnormalities in blood cell counts.
3. Blood viscosity measurements: Blood viscosity is measured using a viscometer, which measures the flow of blood through a narrow tube. This test can help assess the degree of hyperviscosity.
4. Electrophoresis: Electrophoresis is a laboratory test that separates proteins in the blood based on their electrical charge and size. This test can help identify abnormal proteins, such as IgM antibodies, that may be contributing to hyperviscosity.
5. Imaging studies: Imaging studies like computed tomography (CT) or magnetic resonance imaging (MRI) scans may be performed to evaluate for signs of stroke, bleeding, or other complications related to hyperviscosity syndrome.
6. Serum protein electrophoresis: This test measures the concentration and type of proteins in the blood serum. It can help identify abnormal proteins, such as M-proteins, which are characteristic of Waldenström macroglobulinemia.
7. Plasma viscosity measurements: Plasma viscosity measurements are similar to blood viscosity measurements but are performed on the plasma component of the blood rather than whole blood.
8. Laser Doppler velocimetry: This non-invasive test uses laser light to measure blood flow in small vessels and can help assess blood viscosity and flow patterns.
9. Other tests: Additional tests may be ordered depending on the individual patient’s symptoms and medical history, such as:

a. Coagulation studies: To evaluate bleeding or clotting disorders.

b. Renal function tests: To assess kidney function.

c. Liver function tests: To evaluate liver function.

d. Electrocardiogram (ECG): To assess heart function.

e. Neuroimaging: To evaluate for signs of brain damage or stroke.

A comprehensive diagnostic approach is necessary to confirm the diagnosis of hyperviscosity syndrome and rule out other conditions that may present with similar symptoms.

What is the treatment for hyperviscosity syndrome?

The treatment for hyperviscosity syndrome depends on the underlying cause and severity of the condition. The primary goal of treatment is to reduce blood viscosity, alleviate symptoms, and prevent complications. Here are some common treatment options:

1. Plasmapheresis: Plasmapheresis is a process that removes the abnormal proteins from the blood, reducing blood viscosity. This is often performed as an outpatient procedure.
2. Chemotherapy: Chemotherapy may be used to reduce the production of abnormal proteins and slow the progression of the underlying disease.
3. Corticosteroids: Corticosteroids, such as prednisone, can help reduce inflammation and swelling in the brain and other tissues.
4. Vasopressors: Vasopressors, such as dopamine or dobutamine, may be used to increase blood pressure and improve blood flow to vital organs.
5. Diuretics: Diuretics, such as furosemide, can help remove excess fluids and reduce blood volume.
6. Blood transfusions: Blood transfusions may be necessary to replace lost blood cells or correct anemia.
7. Plasma exchange with immunoadsorption: This involves exchanging the patient’s plasma with a substitute plasma that has been treated to remove the abnormal proteins.
8. Immunotherapy: Immunotherapy, such as rituximab, may be used to target and destroy abnormal B cells that produce the abnormal proteins.
9. Supportive care: Supportive care measures, such as pain management, anti-seizure medications, and respiratory therapy, may be necessary to manage symptoms and prevent complications.

In severe cases of hyperviscosity syndrome, hospitalization may be necessary to provide close monitoring and intensive care. Treatment is usually initiated in an intensive care unit (ICU) setting.

It’s essential to work closely with a healthcare team, including hematologists, oncologists, and other specialists, to develop a personalized treatment plan tailored to the individual’s needs.

Remember that prompt recognition and treatment of hyperviscosity syndrome are crucial to preventing serious complications and improving outcomes.