These excess light chains are called bence jones proteins see annotated history section. Extreme hyperviscosity has caused blindness and con vulsions. Occurring in 10% 30% of patients with hyperviscosity syndrome, wm is an uncommon bcell proliferative disorder characterized by bone marrow in ltration and production of monoclonal immunoglobulin m. Hyperviscosity presents as headache, dizziness, vertigo, blurring of vision or shortness of breath in early stages. Hyperviscosity syndrome an overview sciencedirect topics. The reasons for elevated viscosity are increased protein content and large molecular size, abnormal polymerization, and abnormal shape of immunoglobulin molecules. This syndrome is caused by the accumulation of igm proteins large molecules in the blood. Pdf therapeutic apheresis in hyperleukocytosis and. M4a crunch time em blood n stuff individual mp3 files 199 mb zip crunch time em blood n stuff written summary 340 kb pdf. Promote circulation iv fluid, plasma expander mannitol 4. Single plasmapheresis with one plasma volume replacement about 3 l usually results in a dramatic improvement in patients with macrogobulinemia because of its predominant. Chemical warfare agent issues during the persian gulf war 2002, by persian gulf war illnesses task force and united states. Your veterinarian will perform a thorough physical exam on your pet, taking into account the background history of symptoms and possible incidents that might have led to this condition. Problems associated with hyperviscosity syndrome include.
Polycythemiahyperviscosity syndrome university of iowa. Individual mp3 files 187 mb zip crunch time fm hemeonc written summary 351 kb pdf. Nevertheless, this is the picture that can be produced by an increased. Infants with symptoms consistent with hyperviscosity should be tested immediately. Serum hyperviscosity may cause a variety of clinical manifestations including bleeding from mucosal. Clinical features include a predisposition to bleeding from mucosal surfaces, dilatation and segmentation of retinal and conjunctival veins, and central nervous. Serum igm level as predictor of symptomatic hyperviscosity in. Correction of hyperviscosity by apheresis northwestern scholars. Rheology and hyper viscosity syndrome vol1number 3. Viscosity is a property of a given liquid that describes the resistance to flow of one layer over anotherhow fluid a liquid is, how capable it is of flowing. Correction of hyperviscosity by apheresis northwestern. The buildup of the igm proteins thickens the blood eventually impairing blood flow. When it goes up to between 4 and 7 owing to the presence of abnormal serum proteins, the patient develops signs and symptoms resulting directly from the increased viscosity.
N2 therapeutic apheresis is an extracorporeal blood purification technique designed for the removal of either plasma plasmapheresis or cellular blood components cytapheresis. Symptoms of high blood viscosity include spontaneous bleeding from mucous membranes, visual disturbances due to retinopathy, and neurologic symptoms ranging from headache and vertigo to seizures and coma. The clinical manifestations of hyperviscosity syndrome respond, at least transiently, to plasma exchange pe. Patients with igm serum levels greater than 50 grams per liter gl are considered to be at an increased risk for hyperviscosity syndrome. Hyperviscosity definition and meaning collins english. Carfilzomib, rituximab, and dexamethasone card treatment offers a. Other manifestations include prolonged local bleeding at sites of minor table 1. Hyperviscosity syndrome in a hypercholesterolemic patient. Severe hypertriglyceridemia associated with hyperviscosity syndrome in an adult patient with acute pyelonephritis bahad. The hyperviscosity syndrome results from severe hypergammaglobulinemia. The role of chronic hyperviscosity in vascular disease.
Macroglobulinemic patients with the hyperviscosity syndrome and a relative serum viscosity greater than 4. Cerebral infarction in igg multiple myeloma with hyperviscosity. One type is due to serum hyperviscosity and is characterized by spontaneous bleeding with neurologic and ocular disorders. When the blood gets too thick it has trouble traveling through the smallest blood vessels. Serum igm level as predictor of symptomatic hyperviscosity. Lecturer of internal medicine faculty of medicine tanta university slideshare uses cookies to improve functionality and performance, and to provide you with relevant advertising. Capillary hematocrit 65% at 46 hours of life should be followed up immediately with a peripheral venous or arterial spun hematocrit. Several other signs and symptoms of the condition are related to the excess igm, which can thicken blood and impair circulation, causing a condition known as hyperviscosity syndrome. The increase in blood volume was attribut able to an increase in plasma volume. Hyperviscosity syndrome in multiple myeloma sciencedirect. Apr 11, 2010 hyperviscosity is a syndrome, not a final diagnosis. Full text get a printable copy pdf file of the complete article 1. Monoclonal hypergammaglobulinemia resulting in hyperviscosity syndrome is seen in multiple myeloma and waldenstroms macroglobulinemia. Blood hyperviscosity syndrome bhvs is a rare and serious complication of multiple myeloma mm.
Four cases of iga multiple myeloma with raised serum or plasma viscosity and clinical features of the hyperviscosity syndrome hvs are described. Plasmapheresis was effective in reducing blood viscosity and improving the clinical condition in all four patients. Hyperviscosity syndrome hvs is an oncologic emergency that classically. Symptoms of hyperviscosity syndrome typically do not develop unless the patients serum viscosity is especially elevated. Pulmonary hypertension secondary to hyperviscosity in a. Hvs can also result from increased cellular blood components typically white or red blood cells in hyperproliferative. May 14, 2018 hyperviscosity syndrome is a condition in which blood is unable to flow freely through the arteries. Blood hyperviscosity syndrome revealing multiple myeloma in. Diagnostic evaluation for chronic nasal disease cats with chronic rhinitis are frequently a diagnostic challenge. Dec 11, 2019 hyperviscosity syndrome hvs refers to the clinical sequelae of increased blood viscosity. Jan 06, 2017 hyperviscosity syndrome causes, diagnosis, treatment slideshare uses cookies to improve functionality and performance, and to provide you with relevant advertising. Effective treatment relies on the physical removal of the igm protein from the blood stream by a technique called plasma exchange or plasmapheresis. Polyclonal hyperviscosity syndrome in igg4related disease and associated conditions.
Indirect ophthalmoscopy with scleral depression along with retinal vessel diameter measurements are able to detect the earliest hyperviscosity syndromerelated complications and should be considered in the treatment. Therefore, patients need to be tested periodically for evidence of hyperviscosity syndrome progression. This condition is most common in infants and children and can affect their growth by reducing. Vision, blurred eye disorders merck manuals consumer.
Accumulation of excess igm in the bloodstream increases serum viscosity and may cause symptomatic hyperviscosity in up to 30% of patients. Pdf hyperviscosity syndrome in iga multiple myeloma. Unsurprisingly, these patients have a similar diagnosis and treatment. Hyperviscosity syndrome can develop in patients with plasma cell dyscrasias, particularly waldenstrom macroglobulinemia wm. Hyperviscosity syndrome free download as powerpoint presentation. Hyperviscosity may be manifested by nasal bleeding, blurred vision, headaches, dizziness, nystagmus, deafness, diplopia, ataxia, paresthesias, or congestive heart failure. In the patient with no clinical manifestations of the hyperviscosity syndrome cl, the relative serum viscosity was 3. Types 1 and 2 cryoglobulinemia type 2 cryoglobulins are also rheumatoid factors present in high titer are welldescribed causes of hyperviscosity as the gelling phenomenon that occurs in the peripheral circulation rapidly raises the viscosity of serum and is highly temperature dependent. High level of plasma proteins in the blood of dogs petmd. Disorders that simultaneously involve several organ systems can produce confusing clinical pictures, and the combination of oronasal bleeding, visual impairment, and altered sensorium is particularly striking in this regard. Fahey coined the clinical term hyperviscosity syndrome hvs in 1965. A common feature of both patients was the production of.
These results indicate that the concentration and molecular configuration of the myeloma protein are important determinants of the presence or absence of the hyperviscosity syndrome. Recognizing hyperviscosity syndrome in patients with. Severe hypertriglyceridemia associated with hyperviscosity. Hyperviscosity, however, can be relieved by adequate plasmapheresis, and recognition of the. We reported a 68yrold patient who developed monoplegia of the left leg as the first manifestation of multiple myeloma with hyperviscosity. Get a printable copy pdf file of the complete article 537k, or click on a page image below to browse page by page. Dec 05, 2014 hyperviscosity syndrome by mohamed abdelmotaal safa ass. Hyperviscosity syndrome is caused by elevated blood viscosity due to the liquid component plasma or serum, like in waldenstrom macroglobulinemia or multiple myeloma or to the cellular component, like in myeloproliferative diseases polycythemia or leukemia.
The presence of igm in the blood provides a tumor marker that can be followed during the course of treatment to gauge the involvement of disease in the bone. Untreated, longstanding hyperviscosity syndrome can cause lifethreatening complications. Links to pubmed are also available for selected references. Background viscosity is a property of a given liquid that describes the resistance to flow of one layer over anotherhow fluid a liquid is, how capable it is of flowing. Two cases of multiple myeloma with iga gammopathy and the syndrome of serum hyperviscosity are reported. Waldenstrom macroglobulinemia genetics home reference nih. Hyperviscosity syndrome is a group of symptoms triggered by an increase in the viscosity of the blood. Jul 03, 2016 hyperviscosity refers to any state in which there is increased viscosity of the blood. Hyperviscosity definition of hyperviscosity by medical. Iggkmultiple myeloma with hyperviscosity syndromeresponse. Clinical features include a predisposition to bleeding from mucosal surfaces, dilatation and segmentation of retinal and conjunctival veins, and central nervous system disturbances including headache, drowsiness, weakness and.
Acute respiratory failure secondary to hyperviscosity. Hyperviscosity of the blood has the potential to affect all the vital organs of the body. The most common cause of serum hyperviscosity is the presence of. The hyperviscosity syndrome commonly includes mucous membrane bleeding, retinopathy and loss of vision, and neurological disorders associated with elevated serum viscosity. Apheresis machine in action plasmapheresis consists of connecting the patient to an apheresis. Symptoms of high blood viscosity include spontaneous bleeding from mucous membranes, visual disturbances due to retinopathy, and neurologic symptoms ranging from headache and vertigo to. Increased serum viscosity usually results from increased circulating serum immunoglobulins and can be seen in such diseases as waldenstrom macroglobulinemia and multiple myeloma. Signs and symptoms of the hyperviscosity ocular hematologic neurologic cardiovascular. Viscosity is the property of fluids to resist flow. Screening for hyperviscosity syndrome benson viscometers. To the best of our knowledge, this is the second case reported in the literature treated. Hyperviscosity syndrome dr doha rasheedy assisstant professor geriatrics and gerontology department asu 2.
Hyperviscosity syndrome hvs refers to the clinical sequelae of increased blood viscosity. If you continue browsing the site, you agree to the use of cookies on this website. Prevention and treatment of hyperviscosity syndrome springerlink. Pdf blood hyperviscosity syndrome revealing multiple myeloma. Meanwhile, hyperviscosity caused by multiple myeloma might also have contributed to the stroke. It is the treatment of choice in hyperviscos ity syndrome, due to the presence of quantitatively or qualitatively abnormal plasma proteins such as paraproteins. Other conditions associated with blood hyperviscosity include shock, rheumatic disease, and diabetes. Testing for hyperviscosity syndrome involves measurements of serum andor plasma viscosity. Hyperviscosity syndrome is a common clinical manifestation of waldenstrom macroglobulinaemia wm, a malignant bcell lymphoma characterized by the excessive secretion of monoclonal igm owen et al, 2003. Hyperviscosity syndrome occurs in about 10 to 30 percent of wm patients. Definition viscosity refers to resistance to flow or stickiness, from the latin word viscum alba. Established hyperviscosity syndrome is a medical emergency that should be managed actively and promptly. Increased serum viscosity usually results from increased circulating serum immunoglobulins eg, macroglobulinaemia, multiple myeloma and can also result from increased cellular blood components eg, red or white blood cells in hyperproliferative states eg, leukaemias, polycythaemia and thrombocythaemia. The total serum protein concentration ranged from 14 to 19 g100 ml, of which 1217 g was myeloma.
Serum hyperviscosity has been identified in a variety of pathological conditions, including macroglobulinemia waldenstrom, certain polyclonal gammopathies, and, rarely, multiple myeloma. Hyperviscosity syndrome is a condition in which blood isnt able to flow freely through your arteries. Hyperviscosity occurs from pathologic changes of either cellular or protein. Plasma exchange in acute and chronic hyperviscosity syndrome iss. There may be blockages due to abundance of rbcs, proteins, or wbcs in the blood. Hyperviscosity syndrome multiple myeloma heart failure. Aug 31, 2005 minor complaints include dizziness, vertigo, headache, and impaired learning. Hyperviscosity syndrome the relative viscosity of serum is normally 141 8 times that of water. It can also occur with any abnormally shaped red blood cells, such as with sickle cell anemia. Measurement and clinical value of the plasma viscosity. Cerebral infarction is an uncommon complication in multiple myeloma with hyperviscosity. Hyperviscosity syndrome occurs in 510% of patients and is usually associated with an iga paraproteinemia, due to the tendency of the iga paraprotein to polymerize.
Elevated blood, plasma, or serum viscosity occurs in a number of hematologic disorders. Apr 06, 2018 hyperviscosity syndrome is a pathological condition in which the blood is not able to flow freely through the arteries. Individuals with symptomatic waldenstrom macroglobulinemia can experience general symptoms such as fever, night sweats, and weight loss. Centipoise cp is the standard measuring unit and normal serum viscosity is between 1. Occurring in 10%30% of patients with hyperviscosity syndrome, wm is an uncommon bcell proliferative disorder characterized by bone marrow infiltration and production of monoclonal immunoglobulin m. Conclusions retinal manifestations of hyperviscosity syndrome occur at lower serum igm and sv levels than previously reported. Full text full text is available as a scanned copy of the original print version. Blood hyperviscosity syndrome is a rare and serious complication of multiple myeloma.
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