Hyperviscosity syndrome is a clinical symptom complex. In many cases, the syndrome is simply referred to by the abbreviation HVS. The cause of hyperviscosity syndrome is an increased concentration of so-called paraproteins in the plasma of the blood. As a result of the increased viscosity, the flowability of the blood decreases, which can lead to a variety of complications in the organism.
What is hyperviscosity syndrome?
The main feature of hyperviscosity syndrome is increased viscosity, or viscosity, of the blood. Basically, the viscosity of blood depends on the concentration of paraproteins dissolved in plasma. Their chemical and physical properties have a direct effect on the viscosity and thus the flowability of the blood. Hyperviscosity syndrome as a result of elevated paraproteins in plasma occurs in a number of malignant diseases. These include Waldenström’s disease and multiple myeloma. In addition, hyperviscosity syndrome also occurs in some benign diseases, such as rheumatoid arthritis, Felty’s syndrome and lupus erythematosus. Hyperviscosity syndrome occurs in nearly ten percent of cases of multiple myeloma and in up to 30 percent of all cases of Waldenström’s disease.
Causes
To understand the causes of hyperviscosity syndrome, some basics regarding blood viscosity are important. In principle, this is dependent on a number of different factors. The most influential of these are plasma viscosity, hematocrit, and red blood cell deformability. Deviations of one or more of these factors from the normal values lead to changes in blood viscosity. For example, plasma viscosity is increased in multiple myeloma. Typical for multiple myeloma is the detection of atypical blood proteins or paraproteins. Possible symptoms include spontaneous fractures, renal insufficiency in the presence of a plasmacytoma kidney, and hyperviscosity syndrome. This occurs frequently in the presence of cerebral circulatory disorders and neurological deficits. The hematocrit, for example, is increased in so-called exsiccosis and influences the blood viscosity. Exsiccosis refers to dehydration of the body. It occurs when fluid intake is too low compared to excretion. The deformability of red blood cells or erythrocytes is increased, for example, in the context of sickle cell anemia. This is a form of anemia due to sickle-shaped red blood cells. A special pathological hemoglobin causes the red blood cells to deform when oxygen saturation is low. As a result, severe circulatory disturbances occur in organs and the body tissues. A fatal course of sickle cell anemia is possible. If the blood viscosity is increased, in most cases circulatory disturbances occur in the so-called end-stream areas of the vascular system. This results in a reduced blood supply to tissues and organs, and the circulatory disturbances depend on the severity of the increase in viscosity.
Symptoms, complaints, and signs
Numerous different symptoms and complaints are possible in the setting of hyperviscosity syndrome, and they vary from patient to patient. They depend on the type of viscosity increase as well as the severity of the condition. Some organs, such as the heart, kidneys and brain, are very sensitive to circulatory disturbances. Functional restrictions of the corresponding organs are often the result. In early stages, therefore, respiratory distress, neurological deficits, kidney and heart failure frequently occur. Typical marks may also appear on the skin, the so-called livedo reticularis. As a result of the slowed blood flow, the risk of thrombosis and embolism increases. The likelihood of such complications increases, especially in bedridden patients. In general, many affected patients complain of a general feeling of weakness, loss of appetite, fatigue and shortness of breath. Anemia may develop due to bleeding from the mucosa and nose, as platelet function is impaired. Nosebleeds and bleeding of the oral mucosa occur as a result of impaired blood clotting. Also, bleeding time after injury is longer than usual. Typical symptoms of the central nervous system are dizziness and headaches, drowsiness up to coma, and epileptic seizures.Sensory disturbances are also possible. Sometimes those affected complain of visual disturbances. Hearing loss may occur as part of the hyperviscosity syndrome. Angina pectoris sometimes develops on the heart.
Diagnosis and course of the disease
Hyperviscosity syndrome is usually diagnosed during a blood test. First, the attending specialist discusses the individual medical history with the patient. Any symptoms that occur provide indications of the disease and its severity. In a so-called serum electrophoresis, the increased concentration of paraproteins can be detected. The blood viscosity is measured with a capillary viscometer and shows the increased values. A further indication of hyperviscosity syndrome can also be provided by complications during blood collection, such as clogged cannulae.
Complications
Hyperviscosity syndrome causes numerous symptoms and complications in the body. The organs and regions in the body that are supplied with blood are particularly affected. Respiratory distress may occur, leading to a panic attack in many patients. Furthermore, there are also symptoms of the heart, so that in the worst case the patient can also die from heart failure. The kidneys can also be affected by insufficiency, making the patient dependent on dialysis or a donor kidney. The patient’s quality of life and life expectancy are reduced by hyperviscosity syndrome. The affected person suffers from a general feeling of illness and feels weak. There is fatigue and loss of appetite. Furthermore, dizziness and nausea also occur, and it is not uncommon for the affected person to faint. Sensitivity of the body is also reduced and loss of vision or hearing may occur. In the worst case, the patient falls into a coma. Since hyperviscosity syndrome is not a disease in its own right, treatment is usually causal. Acute emergencies can be resolved with the help of medications. Complications usually depend on the underlying disease of hyperviscosity syndrome.
When should you see a doctor?
People who suffer from circulatory problems should always see a doctor. If there is an increase in cold limbs, numbness of the skin, sensory disturbances or a feeling of pressure on the vessels, a visit to the doctor is necessary to clarify the symptoms. If there are disturbances in digestive activity, abnormalities when going to the toilet or pain in the upper body, a doctor should be consulted. If breathing restrictions, shortness of breath or anxiety occur, the affected person needs help and support. An irregularity of the heart rhythm, changes in blood pressure or dizziness must be examined and treated. If there is a general feeling of illness, malaise, unsteadiness of gait or decreased drive, a visit to the doctor is recommended. If everyday tasks can no longer be performed as usual or if the usual level of performance drops, a doctor should be consulted. If skin changes, discoloration, or skin blemishes appear, these should be presented to a physician. In case of involuntary itching or open wounds, good medical care is necessary. There is a risk of further diseases, as pathogens can enter the organism. In case of internal weakness, tiredness and fatigue, a doctor should be consulted. If the complaints persist over a longer period of time, this is considered unusual and in need of treatment. Sleep disturbances, decreased muscle strength or irregularities in muscle activity should be investigated and treated.
Treatment and therapy
Treatment of hyperviscosity syndrome always depends on the cause. In acute cases, it is necessary to dilute the blood by infusions. Further treatment of viscosity symptoms is usually symptomatic, such as by plasma exchange. A cell separator separates the plasma from the cellular components. However, plasma exchange is recommended only in emergencies, such as epileptic seizures, coma or heart failure. To cure hyperviscosity syndrome, the underlying disease must be treated. The prognosis of the disease also depends on this.
Prevention
There are no concrete measures to prevent hyperviscosity syndrome. This makes it all the more important to consult a specialist at the first signs of the disease.Regular blood tests also help detect the disease early.
Follow-up
There are no specific preventive or aftercare measures for hyperviscosity syndrome. Therefore, regular medical checkups are extremely important. They should help relieve symptoms. This simultaneously reduces the risk of heart problems. Medical treatment is essential, as there is no self-help in follow-up care. Only medical therapy can prevent momentous problems that may cause the patient’s death. The earlier the diagnosis and treatment, the more likely a positive outcome. To prevent an outbreak of the syndrome, those at risk can avoid threatening stressful situations. Otherwise, there is a risk that they will become unconscious. In that case, those present must immediately call an emergency physician and place the patient in the recovery position. Involving family members is an important point in this context, because it allows them to help in an emergency. The disease can trigger a loss of appetite, whereupon those affected often lose weight and suffer from deficiency symptoms. A consistent and regular diet with balanced meals stabilizes health and counteracts excessive weight loss. The relevant recommendations of the doctor or a fixed meal plan will help.
What you can do yourself
Unfortunately, in most cases with hyperviscosity syndrome, the patient has no options for self-help. For this reason, the syndrome must always be treated by a physician. This can prevent serious complications, which in the worst case can lead to the death of the patient. Above all, early diagnosis and treatment has a very positive effect on the further course of the disease. If the patient loses consciousness due to the syndrome and faints, an emergency physician must be notified. Until the emergency physician arrives, it is important to ensure a stable lateral position and stable breathing. Furthermore, the affected person should avoid stressful situations. Since hyperviscosity syndrome can also lead to a loss of appetite, the affected person should pay attention to a regular and, above all, healthy diet. This can prevent deficiency symptoms and weight loss. In the case of surgical procedures, the patient should inform the attending physician about the condition in order to avoid severe bleeding and associated complications. Regular examinations and check-ups with the doctor can also alleviate the symptoms of the syndrome and prevent possible heart problems. The syndrome itself cannot be prevented.
