Prompt therapy with plamapheresis and hydration is warranted to prevent life threatening complications. Hyperviscosity syndrome synonyms, hyperviscosity syndrome pronunciation, hyperviscosity syndrome translation, english dictionary definition of hyperviscosity syndrome. Studentresident case report poster critical care iii session type. 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. Hyperviscosity syndrome definition of hyperviscosity. Pdf hyperviscosity syndrome in iga multiple myeloma. Lecturer of internal medicine faculty of medicine tanta university 2. The definitive treatment of hvs involves chemotherapy for the underlying hematologic condition. Clinical features include a predisposition to bleeding from mucosal surfaces, dilatation and segmentation of retinal and conjunctival veins, and central nervous. 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. List of tests for hyperviscosity syndrome offered by dr lal pathlabs. Relevance to the hyperviscosity syndrome semantic scholar.
This aggregation appar ently plays a major role in the hyperviscosity syndrome. Established hyperviscosity syndrome is a medical emergency that should be managed actively and promptly. One type is due to serum hyperviscosity and is characterized by spontaneous bleeding with neurologic and ocular disorders. 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. Sudden, complete loss of vision in one or both eyes blindness is considered something.
N2 therapeutic apheresis is an extracorporeal blood purification technique designed for the removal of either plasma plasmapheresis or cellular blood components cytapheresis. Hyperviscosity syndrome is a condition in which blood is unable to flow freely through the arteries. The diagnosis requires a high clinical suspicion and should be considered in patients presenting with dizziness and underlying history of multiple myeloma. Perinatal polycythemia and hyperviscosity syndrome. Correction of hyperviscosity by apheresis northwestern.
Hyperviscosity is a clinical syndrome caused by sludging of blood within vessels. 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. Viscosity is a property of liquid that exhibits its resistance to the flow of one layer over. Apheresis machine in action plasmapheresis consists of connecting the patient to an apheresis. Cerebral infarction is an uncommon complication in multiple myeloma with hyperviscosity. Hyperviscosity syndrome is a pathological condition in which the blood is not able to flow freely through the arteries. Hyperviscosity definition of hyperviscosity by medical. Polyclonal hyperviscosity syndrome in igg4related disease and associated conditions. True whole blood viscosity cannot be routinely measured on blood samples.
Hyperviscosity syndrome dr doha rasheedy assisstant professor geriatrics and gerontology department asu 2. 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. Abs tract the hyperviscosity syndrome is an uncommon complication in igg myeloma. Hyperviscosity definition and meaning collins english. Effective treatment relies on the physical removal of the igm protein from the blood stream by a technique called plasma exchange or plasmapheresis. Increased serum viscosity usually results from increased circulating serum immunoglobulins and can be seen in such diseases as waldenstrom macroglobulinemia and multiple myeloma. When doctors talk about blurred vision, they typically mean a decrease in sharpness or clarity that has developed gradually. The hyperviscosity syndrome results from severe hypergammaglobulinemia. Hyperviscosity syndrome the relative viscosity of serum is normally 141 8 times that of water.
Hyperviscosity syndrome can develop in patients with plasma cell dyscrasias, particularly waldenstrom macroglobulinemia wm. 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. A 50 year old woman with hypertension and sciatica presented with a 2 month history of intermittent abdominal, leg and back pain, and a 14lb weight loss. Treatment options include chemotherapy, radiation, and iv stenting. Hyperviscosity syndrome by mohamed abdelmotaal safa ass. 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. Hyperviscosity syndrome is a condition in which blood isnt able to flow freely through your arteries. Acute respiratory failure secondary to hyperviscosity. Laboratory data was significant for elevated serum calcium to 11mgdl and total protein to 11. The state of serum hyperviscosity, which involves spontaneous bleeding with neurologic and ocular disorders, has, as a further economy of expression, correctly been called a hyperviscosity. Its occurrence has been ascribed to the presence in the serum of high molecular weight polymers of the igg proteins. Hyperviscosity syndrome has been reported in all plasma cell dyscrasias and at times can be a major cause of morbidity and mortality.
A cd19 positive igm plasma cell myeloma presents with. Recognizing hyperviscosity syndrome in patients with. Serum igm level as predictor of symptomatic hyperviscosity. The article presents the case of 50yearold woman who was diagnosed with multiple myeloma. The hyperviscosity syndrome is being diagnosed with increasing frequency in association with iga multiple myeloma, and should be investigated in. Hyperviscosity syndrome definition csp any syndrome associated with increased viscosity of the blood.
The role of chronic hyperviscosity in vascular disease. Polycythemia is significant only because it increases risk of hyperviscosity syndrome. Hyperviscosity syndrome in multiple myeloma sciencedirect. Hyperviscosity syndrome usually is associated with waldenstr. In this syndrome, arterial blockages can happen due to too many red blood cells, white blood cells, or proteins in your bloodstream. Hyperviscosity syndrome in patients with multiple myeloma. Use cell cultures to express this dna product 2 genetically engineered mice that produce. There may be blockages due to abundance of rbcs, proteins, or wbcs in the blood. Elevated blood, plasma, or serum viscosity occurs in a number of hematologic disorders. It can also occur with any abnormally shaped red blood cells.
Viscosity increases with increasing central venous or arterial hematocrit, the diagnosis rests on the presence of polycythemia hematocrit 65% and in the. Therapeutic plasma exchange for mprotein disorders medigraphic. T1 hyperviscosity syndrome in plasma cell dyscrasias. We report an unusual case of igm myeloma presenting with acute. For example, multiple myeloma continues to have a poor longterm prognosis patient education the diseases leading to hyperviscosity are chronic, and. Merge binding portion of monoclonal mouse antibody with human antibody producing dna. Serum hyperviscosity may cause a variety of clinical manifestations including bleeding from mucosal membranes, congestive heart failure, retinopathy, and. This increase in serum viscosity is the result of two major pathologic processes. Vision, blurred eye disorders merck manuals consumer. Hyperviscosity syndromecausessymptomstreatmentsurvival. Sludging occurs because increased red blood cell mass causes a relative decrease in plasma volume and a relative increase in proteins and platelets. Lurie comprehensive cancer center, northwestern university, chicago, illinois, usa. High level of plasma proteins in the blood of dogs petmd.
Polyclonal hyperviscosity syndrome wiley online library. Polycythemiahyperviscosity syndrome university of iowa. Other conditions associated with blood hyperviscosity include shock, rheumatic disease, and diabetes. Three patients with igg myeloma and the clinical hyperviscosity syndrome were investigated, none of whom had iggpolymers in the serum. Definition viscosity refers to resistance to flow or stickiness, from the latin word viscum alba. Pdf polyclonal hyperviscosity syndrome in igg4related. 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. Hyperviscosity syndrome hvs is a clinical feature in 10% to 30% of patients with waldenstrom macroglobulinemia wm, sometimes as its presenting manifestation. Unsurprisingly, these patients have a similar diagnosis and treatment. Hyperviscosity syndrome hvs refers to the clinical sequelae of increased blood viscosity. The use of syndromes to describe complex lists of symptoms and signs represents a convenient economy of scientific expression.
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 hyperviscosity occurs from pathologic changes of either cellular. 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 confusion which may progress to epileptic fits, paralysis and coma. Accumulation of excess igm in the bloodstream increases serum viscosity and may cause symptomatic hyperviscosity in up to 30% of patients. Management of hyperviscosity syndromes springerlink. Extreme hyperviscosity has caused blindness and con vulsions. Orthostatic hypotension is a rare neurological presentation of hyperviscosity syndrome. Hyperviscosity syndrome is a group of symptoms triggered by an increase in the viscosity of the blood.
Conclusions retinal manifestations of hyperviscosity syndrome occur at lower serum igm and sv levels than previously reported. Hyperviscosity syndrome archives emergency medicine cases. Hyperviscosity syndrome an overview sciencedirect topics. Fahey coined the clinical term hyperviscosity syndrome hvs in 1965. Pulmonary hypertension secondary to hyperviscosity in a. Hyperviscosity syndrome multiple myeloma heart failure.
Hyperviscosity syndrome hvs refers to the clinical constellation of symptoms exhibited due to an increase in serum viscosity. This condition is most common in infants and children and can affect their growth by reducing. Figure 8 mild rightsided ptosis in miller fisher syndrome. The clinical hyperviscosity syndrome includes nonspecific symptoms such as headache, visual disturbances, hearing loss and vertigo. 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. It offers a discussion of the key assessments in patients with multiple myeloma and the clinical manifestations of hyperviscosity syndrome, as well as how. N2 hypergammaglobulinemia increases serum viscosity and is the most common cause of hyperviscosity syndrome.37 514 187 1 544 581 201 1058 1586 203 761 1326 253 855 1125 802 1273 213 1369 780 1076 1416 916 716 11 1028 956 430 968 559 194 831 294 586 806 1341