Hyperviscosity syndrome is a blood condition that can cause severe complications such as respiratory failure, pulmonary edema, and congestive heart failure. It is most commonly caused by Waldenström macroglobulinemia but is associated with a variety of conditions including myeloproliferative disorders in which there is overproduction of blood cells.
Definition & Facts
Viscosity by definition is the stickiness of the blood; that is, its resistance to flow. It is a measure to determine the actual ability of the blood to flow through the body's blood vessels. The blood of those affected by hyperviscosity syndrome is too thick or sticky and does not flow freely through the body's arteries.
Blockages occur in the vessels and are a result of a large amount of either red blood cells or white blood cells or proteins that are found in the blood. This blood condition is most often found in infants and young children but can continue into adulthood.
Symptoms & Complaints
Vertigo, headaches, seizures, blurred vision, and hearing loss are also symptoms of the condition. The visual abnormalities are often seen due to dilation of the actual retinal veins. A few neurological symptoms include confusion and cognitive impairment. When severe or left undiagnosed, hyperviscosity syndrome can lead to heart failure, kidney failure, paralysis, necrosis of intestinal tissue, or coma.
There are many causes of hyperviscosity. Gestational diabetes is a causal factor. Certain birth defects and the incorrect clamping of the umbilical cord after birth may also cause hyperviscosity syndrome. When twins share blood between them in the uterus, a condition called twin-to-twin transfusion syndrome, there is a higher chance of the babies developing the blood syndrome as well.
Other causes of hyperviscosity syndrome include leukemia, cancer of the blood, and essential thrombocytosis which occurs when the bone's marrow creates too many blood platelets. Other medical conditions such as sickle-cell anemia and sepsis can lead to the blood condition as well.
Diagnosis & Tests
If a medical professional suspects hyperviscosity syndrome, the first test ordered is a blood test. This will help them determine the amount of red and white blood cells in the bloodstream. Further tests are necessary to properly diagnose the syndrome and may include an analysis of urine, blood sugar test, creatinine test and blood gas test. The urinalysis determines the amount of glucose, blood and protein existing in the urine. A blood sugar test does just what the name implies, and checks the sugar levels of the blood. A creatinine test will measure the quantity of blood in the kidneys.
Finally, a blood gas test will demonstrate the level of oxygen found in the blood. During the test, symptoms may be uncovered such as kidney failure or breathing problems and will help the doctor make a positive diagnosis.
Symptoms will be assessed during the diagnostic process as well a patient's medical history and family history of disease. A child who is under-developing in growth or skills can be a red flag of the disease to a doctor. This can occur due to the reduced blood flow to the child's vital organs including the kidneys and brain.
Treatment & Therapy
The severity of the blood disorder will determine the course of treatment needing to be taken. Mild cases may only require monitoring for possible complications further down the road. Those suffering from more severe cases may need a very common medical procedure, a blood transfusion. A matching donor for the patient's blood type would be found through a tested family member or a blood bank, and then the transfusion performed. The replacement of blood is performed in hopes that it will decrease the amount of red blood cells in the bloodstream of the person affected.
Plasmapheresis may be ordered by a doctor as treatment as well. In general terms, this is when the blood is taken out of the body and treated, then it is returned to the patient. Treatments of the blood can include separating the blood's components and only returning some of them to the patient or exchanging only some of the components of the blood with those of a donor. This treatment is used for a variety of diseases and conditions, but it is not often used in the United States.
When the condition if found in babies, an increase in fluids is commonly recommended by doctors. This boost of fluids will help reduce the blood's thickness and increase flow in the body's arteries and vessels. If the infant does not respond well to the extra feedings and fluid intake, intravenous fluids may need to be given. With those patient's whose hyperviscosity syndrome is caused by an underlying condition, that particular condition will need to be treated first.
Prevention & Prophylaxis
Babies affected by the disease have a good chance of recovery when the case is mild and treatment is received and tolerated. More severe cases or those not responding to treatments will lead to further complications later in life and can range from stroke to recurrent seizures.