Hyperviscosity syndrome is a group of symptoms triggered by an increase in the

viscosity The viscosity of a fluid is a measure of its resistance to deformation at a given rate. For liquids, it corresponds to the informal concept of "thickness": for example, syrup has a higher viscosity than water. Viscosity quantifies the int ...

of the

blood Blood is a body fluid in the circulatory system of humans and other vertebrates that delivers necessary substances such as nutrients and oxygen to the cells, and transports metabolic waste products away from those same cells. Blood in the cir ...

. Symptoms of high

blood viscosity Hemorheology, also spelled haemorheology (from Greek ‘αἷμα, ''haima'' ' blood' and rheology, from Greek ῥέω ''rhéō'', 'flow' and -λoγία, ''-logia'' 'study of'), or blood rheology, is the study of flow properties of blood and its ...

include spontaneous bleeding from

mucous membrane A mucous membrane or mucosa is a membrane that lines various cavities in the body of an organism and covers the surface of internal organs. It consists of one or more layers of epithelial cells overlying a layer of loose connective tissue. It i ...

s, visual disturbances due to

retinopathy Retinopathy is any damage to the retina of the eyes, which may cause vision impairment. Retinopathy often refers to retinal vascular disease, or damage to the retina caused by abnormal blood flow. Age-related macular degeneration is technically inc ...

, and neurologic symptoms ranging from

headache Headache is the symptom of pain in the face, head, or neck. It can occur as a migraine, tension-type headache, or cluster headache. There is an increased risk of depression in those with severe headaches. Headaches can occur as a result ...

and

vertigo Vertigo is a condition where a person has the sensation of movement or of surrounding objects moving when they are not. Often it feels like a spinning or swaying movement. This may be associated with nausea, vomiting, sweating, or difficulties w ...

seizures An epileptic seizure, informally known as a seizure, is a period of symptoms due to abnormally excessive or synchronous neuronal activity in the brain. Outward effects vary from uncontrolled shaking movements involving much of the body with lo ...

and

coma A coma is a deep state of prolonged unconsciousness in which a person cannot be awakened, fails to respond normally to painful stimuli, light, or sound, lacks a normal wake-sleep cycle and does not initiate voluntary actions. Coma patients exhi ...

. Hyperviscosity occurs from pathologic changes of either cellular or protein fractions of the blood such as is found in

polycythemia Polycythemia (also known as polycythaemia) is a laboratory finding in which the hematocrit (the volume percentage of red blood cells in the blood) and/or hemoglobin concentration are increased in the blood. Polycythemia is sometimes called e ...

multiple myeloma Multiple myeloma (MM), also known as plasma cell myeloma and simply myeloma, is a cancer of plasma cells, a type of white blood cell that normally produces antibodies. Often, no symptoms are noticed initially. As it progresses, bone pain, ...

(particularly IgA and IgG3),

leukemia Leukemia ( also spelled leukaemia and pronounced ) is a group of blood cancers that usually begin in the bone marrow and result in high numbers of abnormal blood cells. These blood cells are not fully developed and are called ''blasts'' or ...

monoclonal gammopathies Monoclonal gammopathy, also known as paraproteinemia, is the presence of excessive amounts of myeloma protein or monoclonal gamma globulin in the blood. It is usually due to an underlying immunoproliferative disorder or hematologic neoplasms, esp ...

such as

Waldenström macroglobulinemia Waldenström macroglobulinemia () is a type of cancer affecting two types of B cells: lymphoplasmacytoid cells and plasma cells. Both cell types are white blood cells. It is characterized by having high levels of a circulating antibody, immunogl ...

sickle cell anemia Sickle cell disease (SCD) is a group of blood disorders typically inherited from a person's parents. The most common type is known as sickle cell anaemia. It results in an abnormality in the oxygen-carrying protein haemoglobin found in red bl ...

, and

sepsis Sepsis, formerly known as septicemia (septicaemia in British English) or blood poisoning, is a life-threatening condition that arises when the body's response to infection causes injury to its own tissues and organs. This initial stage is follo ...

. Types of hyperviscosity syndromes vary by pathology; including

serum Serum may refer to: * Serum (blood), plasma from which the clotting proteins have been removed **Antiserum, blood serum with specific antibodies for passive immunity * Serous fluid, any clear bodily fluid *Truth serum, a drug that is likely to mak ...

hyperviscosity, which may cause neurologic or ocular disorders; polycythemic hyperviscosity, which results in reduced blood flow or capillary

perfusion Perfusion is the passage of fluid through the circulatory system or lymphatic system to an organ or a tissue, usually referring to the delivery of blood to a capillary bed in tissue. Perfusion is measured as the rate at which blood is deliver ...

and increased organ congestion; and syndromes of hyperviscosity, caused by reduced deformability of

red blood cells Red blood cells (RBCs), also referred to as red cells, red blood corpuscles (in humans or other animals not having nucleus in red blood cells), haematids, erythroid cells or erythrocytes (from Greek ''erythros'' for "red" and ''kytos'' for "hol ...

, often evident in sickle cell anemia.

Cause

High cell counts are seen in conditions such as

(raised red blood cells) or

(more

white blood cell White blood cells, also called leukocytes or leucocytes, are the cells of the immune system that are involved in protecting the body against both infectious disease and foreign invaders. All white blood cells are produced and derived from mult ...

s, especially in acute leukemic blast crises). May occur with a white blood cell count greater than 100,000/mm³ (100×10⁹/L).

Diagnosis

Although elevated whole blood viscosity is a better measure of hyperviscosity and more common and clinically important, serum viscosity and plasma viscosity are more frequently measured. Normal plasma viscosity is between 1.4 and 1.8 centipoise while symptoms from hyperviscosity typically occur greater than 4 centipoise (about 4 times more viscous than water) and require emergency treatment. Patients will also have evidence of their underlying disorder. Those with myeloma will typically display a rouleaux formation on a peripheral smear and a large

globulin The globulins are a family of globular proteins that have higher molecular weights than albumins and are insoluble in pure water but dissolve in dilute salt solutions. Some globulins are produced in the liver, while others are made by the immune ...

gap, indicative of a significant paraprotein load. Although it used to be the case that test results would take a few days to return, the development of a new generation of automated clinical viscometers means that results can now be obtained within minutes, allowing accurate diagnosis and more targeted therapy. If hyperviscosity is confirmed, treatment can commence early on in the diagnosis. Fundoscopic examination reveals dilation of retinal veins and flame shaped retinal hemorrhages.

Treatment

Plasmapheresis may be used to decrease viscosity in the case of myeloma, whereas leukapheresis or

phlebotomy Phlebotomy is the process of making a puncture in a vein, usually in the arm, with a cannula for the purpose of drawing blood. The procedure itself is known as a venipuncture, which is also used for intravenous therapy. A person who performs ...

may be employed in a leukemic or polycythemic crisis, respectively.

Blood transfusion Blood transfusion is the process of transferring blood products into a person's circulation intravenously. Transfusions are used for various medical conditions to replace lost components of the blood. Early transfusions used whole blood, but mo ...

s should be used with caution as they can increase serum viscosity. Hydration is a temporizing measure to employ while preparing pheresis. Even after treatment, the condition will recur unless the underlying disorder is treated.Serum viscosity and electrophoresis are recommended before and after plasmapheresis in order to correlate viscosity and M-spike with patient symptoms. This correlation may be useful for anticipating the need for repeat plasmapheresis.

References

External links

* - "Hyperviscosity - newborn" {{DEFAULTSORT:Hyperviscosity Syndrome Blood disorders Syndromes

Cause

Diagnosis

Treatment

See also

References

External links