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. Other conditions associated with blood hyperviscosity include shock, rheumatic disease, and diabetes.
Presentation
Hyperviscosity syndrome patients present with three main symptoms [1]: neurologic symptoms, visual abnormalities, and bleeding tendencies. Neurological symptoms include vertigo, somnolence, ataxia, paresthesias and headaches. Bleeding may occur at various sites like gums, uterus, rectum or nasal mucosa or exaggerated cutaneous bleeding after minor injuries and bruising. Ophthalmic symptoms comprise decreased visual acuity, diplopia or vision loss [2].
Patients may also present with symptoms of heart failure, hypertension, metabolic syndrome or anorexia [3], deafness [4], nystagmus or dementia [5].
Symptoms may vary from patient to patient and even in the same patient at different moments in time, according to viscosity levels. Patients may also present with dyspnea, chest pain or high output heart failure. In children, establishing the clinical diagnosis is even more difficult, as they exhibit nonspecific symptoms like tachypnea, cyanosis, hypotonia, seizures, irritability, apnea, apathy, weak suckling, plethora and abdominal distension.
Entire Body System
- Congestive Heart Failure
Physical examination disclosed hepatosplenomegaly, congestive heart failure, and multiple sites of bleeding. [ncbi.nlm.nih.gov]
Chest radiography, for instance, can help diagnose pulmonary infection and congestive heart failure, while craniocerebral computer tomography can exclude other causes of seizures and altered sensorium. [symptoma.com]
Neurologic manifestations are frequent and varied, and may include the following: Vertigo Hearing loss Paresthesias Ataxia Headaches Seizures Somnolence progressing to stupor and coma Other manifestations may include heart failure, shortness of breath [emedicine.medscape.com]
Imaging studies should include a CT and/or MRI of the brain if the patient presents with significant neurological abnormalities and/or a chest x-ray if the patient presents with additional symptoms of congestive heart failure (CHF). [capriles-urgencias.com]
- Plethora
In children, establishing the clinical diagnosis is even more difficult, as they exhibit nonspecific symptoms like tachypnea, cyanosis, hypotonia, seizures, irritability, apnea, apathy, weak suckling, plethora and abdominal distension. [symptoma.com]
[…] infants IDMs Delayed cord clamping Transfusions, e.g., twin-twin, maternal -> fetal Trisomies, e.g., Down Syndrome, 13, & 18 Clinical signs of hyperviscosity: Lethargy Hypotonia Weak suck Difficult to arouse Irritable when aroused Tremulousness Seizures Plethora [uichildrens.org]
Symptoms and Signs Symptoms and signs of hyperviscosity syndrome are those of heart failure, thrombosis (cerebral and renal vessels), and CNS dysfunction, including tachypnea, respiratory distress, cyanosis, plethora, apnea, lethargy, irritability, hypotonia [merckmanuals.com]
- Epilepsy
This case of running and laughing fits was presumed to be one of the temporal lobe epilepsy induced by the hyperviscosity syndrome brought on by neonatal polycythemia. [ncbi.nlm.nih.gov]
Respiratoric
- Pleural Effusion
286 888 Pregnancy Trauma 898 Foreign Body Esophageal 902 Procedural Sedation 904 Pseudotumor Cerebri 910 Pulmonary Contusion 920 Purpura 926 Peritonsillar Abscess 928 Phalangeal Injuries Hand 934 Phenytoin Poisoning 940 Pityriasis Rosea 946 Red 948 Pleural [books.google.com]
Further imaging studies demonstrate pulmonary hypertension, alveolar infiltrates, pleural effusions, increased systemic vascular resistance, increased myocardial strain and hypoperfusion of both peripheral (gut, kidney) and central (brain, myocardium) [symptoma.com]
Associated laboratory findings: Abnormal chest X-ray: cardiomegaly, increased vascularity, hyperaeration, alveolar infiltrates, pleural effusions. [uichildrens.org]
- Tachypnea
In children, establishing the clinical diagnosis is even more difficult, as they exhibit nonspecific symptoms like tachypnea, cyanosis, hypotonia, seizures, irritability, apnea, apathy, weak suckling, plethora and abdominal distension. [symptoma.com]
Delayed cord clamping Transfusions, e.g., twin-twin, maternal -> fetal Trisomies, e.g., Down Syndrome, 13, & 18 Clinical signs of hyperviscosity: Lethargy Hypotonia Weak suck Difficult to arouse Irritable when aroused Tremulousness Seizures Plethora Tachypnea [uichildrens.org]
Symptoms and Signs Symptoms and signs of hyperviscosity syndrome are those of heart failure, thrombosis (cerebral and renal vessels), and CNS dysfunction, including tachypnea, respiratory distress, cyanosis, plethora, apnea, lethargy, irritability, hypotonia [merckmanuals.com]
Her vital signs were significant for tachycardia (pulse 108), tachypnea (respiratory rate 22) on 3 liters/minute oxygen by nasal canula with oxygen saturation of 99%. On physical examination, she was in mild respiratory distress. [oatext.com]
Gastrointestinal
- Loss of Appetite
A 65-year-old woman with hypertension and diabetes mellitus was admitted due to loss of appetite, muscle weakness, breathlessness and discrete expectoration, without fever. [ncbi.nlm.nih.gov]
Loss of appetite: Some people with WM lose their appetite. Fever, sweats, weight loss: WM, like other lymphomas, can cause fevers (without an infection), drenching night sweats, and weight loss (without trying). These are called B symptoms. [cancer.org]
- Abdominal Distension
In children, establishing the clinical diagnosis is even more difficult, as they exhibit nonspecific symptoms like tachypnea, cyanosis, hypotonia, seizures, irritability, apnea, apathy, weak suckling, plethora and abdominal distension. [symptoma.com]
Jaw & Teeth
- Bleeding Gums
Abnormal bleeding: High levels of abnormal antibody can damage blood vessels, which can lead to problems like nosebleeds and bleeding gums. [cancer.org]
Patients usually present with non-specific symptoms such as epistaxis, bleeding gums, headaches, confusion, blurry vision, abdominal pain, fatigue, dyspnea, palmar erythema and stroke [2,5-15,17-19]. [oatext.com]
Cardiovascular
- Hypertension
Patients may also present with symptoms of heart failure, hypertension, metabolic syndrome or anorexia, deafness, nystagmus or dementia. [symptoma.com]
用 語 対 訳 hypertensive encephalopathy 高血圧性脳症 hypertensive episode 高血圧発作 hypertensive heart disease (HHD) 高血圧性心疾患 hypertensive intracerebral hemorrhage (ICH) 高血圧性脳内出血 hypertensive peripheral vascular disease 高血圧性末梢血管疾患 hypertensive pulmonary arteriopathy [j-circ.or.jp]
Hyperviscosity syndrome (HVS) is characterised by high serum viscosity and the involvement of multiple organs, commonly causing retinal haemorrhage, bleeding diathesis, pulmonary hypertension, congestive heart failure (CHF), neurologic deficits and death [ncbi.nlm.nih.gov]
[…] action=getRecordDetail&idt=7891452 Other title Hemorheological disturbances in essential hypertension (en) Author ZANNAD, F1 ; MEDEIROS, C; VOISIN, P; BRUNTZ, J. F; STOLTZ, F; GILGENKRANTZ, J. [pascal-francis.inist.fr]
- Acrocyanosis
Type I cryoglobulinemia is frequently asymptomatic per se but patients may develop acrocyanosis, retinal hemorrhage, Raynaud's phenomenon, and arterial thrombosis. [orpha.net]
Musculoskeletal
- Fracture
The present report describes a patient with IgA myeloma complicated by hyperviscosity syndrome and multiple bone fractures. [ncbi.nlm.nih.gov]
Methemoglobinemia 786 Hypercalcemia 790 Monoamine Oxidase Inhibitor Poisoning 792 Pancreatic Trauma 794 Multiple Myeloma 798 Parkinson Disease 802 Munchausen Syndrome 804 Myocardial Contusion 810 Neck Injury by StrangulationHanging 818 Penile Shaft Fracture [books.google.com]
This makes people with multiple myeloma more vulnerable to fractures (broken bones). Often fractures can occur with little or no trauma due to the weakening of the bone. [oncolink.org]
Malignant proliferation of plasma cells produces skeletal destruction, causing bone pain and pathologic fractures. (3) It accounts for 10% of all hematologic malignancies. [signavitae.com]
- Osteoporosis
At autopsy, almost all bones showed infiltration of multiple myeloma of IgA-kappa type and severe osteoporosis accompanied with proliferation of osteoclasts. [ncbi.nlm.nih.gov]
Multiple myeloma can cause osteoporosis, or generalized weakening of the bone, and weakness at the location of the "punched out" bony lesions. This makes people with multiple myeloma more vulnerable to fractures (broken bones). [oncolink.org]
- Severe Osteoporosis
At autopsy, almost all bones showed infiltration of multiple myeloma of IgA-kappa type and severe osteoporosis accompanied with proliferation of osteoclasts. [ncbi.nlm.nih.gov]
Eyes
- Diplopia
A 47-year-old man with hyperleukocytic chronic lymphocytic leukemia progressively developed retinal hemorrhages, headache, diplopia, dysequilibrium, slurred speech, nystagmus, ataxic gait, and hearing loss as his leukocyte count rose to a maximum of 968,000 [ncbi.nlm.nih.gov]
[…] https://doi.org/ 10.1007/3-540-29662-X_1304 Definition Often found in paraproteinemias most common Waldenström's macroglobulinemia, polycythemia, leukocytosis; symptoms include visual changes, headache, vertigo, nystagmus, dizziness, sudden deafness, diplopia [link.springer.com]
Ophthalmic symptoms comprise decreased visual acuity, diplopia or vision loss. Patients may also present with symptoms of heart failure, hypertension, metabolic syndrome or anorexia, deafness, nystagmus or dementia. [symptoma.com]
Hyperviscosity may be manifested by nasal bleeding, blurred vision, headaches, dizziness, nystagmus, deafness, diplopia, ataxia, paresthesias, or congestive heart failure. [mayomedicallaboratories.com]
Neurologic symptoms of hyperviscosity include blurring or loss of vision, headache, vertigo, sudden deafness, diplopia, ataxia, confusion, and disturbances of consciousness. (5) Clinically significant type I CG associated with multiple myeloma is sporadically [signavitae.com]
- Blurred Vision
She also showed symptoms and signs of hyperviscosity syndrome; hemorrhagic diathesis, blurred vision and episodes of transient ischemic attacks of the brain, and fractures of the bones by small powers of trauma. [ncbi.nlm.nih.gov]
Patients may present with any of the following: Spontaneous gum bleeding Epistaxis Rectal bleeding Menorrhagia Persistent bleeding after minor procedures Visual changes range from blurred vision to vision loss. [emedicine.medscape.com]
Hyperviscosity may be manifested by nasal bleeding, blurred vision, headaches, dizziness, nystagmus, deafness, diplopia, ataxia, paresthesias, or congestive heart failure. [mayomedicallaboratories.com]
Blurred vision, headache, vertigo, dizziness, nystagmus, deafness, and ataxia also occur in HVS. 1, 8, 12, 14 ⇓ ⇓ ⇓ – 18 Patients with severe HVS may have confusion, dementia, stroke, or coma. [bloodjournal.org]
Vision problems: Bleeding around the small blood vessels inside the eyes or poor circulation in these vessels caused by thickened blood might lead to blurred vision or blind spots. [cancer.org]
Ears
- Sudden Hearing Loss
Intravascular lymphomatosis presenting with sudden hearing loss. Journal of the Neurological Sciences, Vol. 232, Issue. 1-2, p. 105. CrossRef Google Scholar [cambridge.org]
Face, Head & Neck
- Epistaxis
We report a case of 71-year-old female with erosive rheumatoid arthritis with profoundly elevated rheumatoid factor (57,400 IU/ml; normal <35) who presented with findings consistent with HVS: profound weakness, headache, epistaxis and plasma viscosity [ncbi.nlm.nih.gov]
Patients may present with any of the following: Spontaneous gum bleeding Epistaxis Rectal bleeding Menorrhagia Persistent bleeding after minor procedures Visual changes range from blurred vision to vision loss. [emedicine.medscape.com]
Center) Edited by Alex Koyfman MD (@EMHighAK) and Stephen Alerhand MD (@SAlerhand) Scenario #1 A 72 year-old male recently diagnosed with multiple myeloma presents to your Emergency Department complaining of abrupt onset of ataxia, blurry vision, and epistaxis [emdocs.net]
Patients usually present with non-specific symptoms such as epistaxis, bleeding gums, headaches, confusion, blurry vision, abdominal pain, fatigue, dyspnea, palmar erythema and stroke [2,5-15,17-19]. [oatext.com]
Neurologic
- Headache
A 47-year-old man with hyperleukocytic chronic lymphocytic leukemia progressively developed retinal hemorrhages, headache, diplopia, dysequilibrium, slurred speech, nystagmus, ataxic gait, and hearing loss as his leukocyte count rose to a maximum of 968,000 [ncbi.nlm.nih.gov]
الصفحة 324 - Headache attacks lasting 4-72 hours (untreated or unsuccessfully treated). C. Headache has at least two of the following characteristics: 1. Unilateral location. 2. Pulsating quality. 3. [books.google.com]
Reference work entry DOI: https://doi.org/ 10.1007/3-540-29662-X_1304 Definition Often found in paraproteinemias most common Waldenström's macroglobulinemia, polycythemia, leukocytosis; symptoms include visual changes, headache, vertigo, nystagmus, dizziness [link.springer.com]
- Seizure
Chest radiography, for instance, can help diagnose pulmonary infection and congestive heart failure, while craniocerebral computer tomography can exclude other causes of seizures and altered sensorium. [symptoma.com]
The symptoms associated with this condition include headaches, seizures, and a reddish tone to the skin. If your infant is unusually sleepy or doesn’t want to feed normally, this is an indication that something is wrong. [healthline.com]
Symptoms may include: Extreme sleepiness Feeding problems Seizures There may be signs of breathing problems, kidney failure, low blood sugar, or newborn jaundice. [medlineplus.gov]
The main symptoms and signs of neonatal polycythemia are nonspecific and include ruddy complexion, feeding difficulties, lethargy, hypoglycemia, hyperbilirubinemia, cyanosis, respiratory distress, and seizures. [merckmanuals.com]
+56 2 3543746; Fax: +56 2 6641329; E-mail: [email protected] Abstract: A 60-year women was admitted to ICU because seizures, poor peripheral perfusion and acute renal failure. [content.iospress.com]
- Vertigo
Vertigo as the First Sign of Chronic Myeloid Leukemia: A Case Report and Literature Review. Case Reports in Otolaryngology, Vol. 2013, Issue., p. 1. [cambridge.org]
Reference work entry DOI: https://doi.org/ 10.1007/3-540-29662-X_1304 Definition Often found in paraproteinemias most common Waldenström's macroglobulinemia, polycythemia, leukocytosis; symptoms include visual changes, headache, vertigo, nystagmus, dizziness [link.springer.com]
Neurological symptoms include vertigo, somnolence, ataxia, paresthesias and headaches. Bleeding may occur at various sites like gums, uterus, rectum or nasal mucosa or exaggerated cutaneous bleeding after minor injuries and bruising. [symptoma.com]
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. [en.wikipedia.org]
Streptococcal Disease 1156 Tuberculosis 1158 Tympanic Membrane Perforation 1164 Suicide Risk Evaluation 1166 Gastritis 1168 Syncope 1172 Ovarian CystTorsion 788 1195 Ventilator Management 1198 Ventricular Peritoneal Shunts 1202 Urinary Tract Fistula 1174 Vertigo [books.google.com]
- Stroke
1304 Definition Often found in paraproteinemias most common Waldenström's macroglobulinemia, polycythemia, leukocytosis; symptoms include visual changes, headache, vertigo, nystagmus, dizziness, sudden deafness, diplopia, ataxia, confusion, dementia, stroke [link.springer.com]
This can also enhance the risk of stroke instead of reducing the risk. [cardiophile.org]
You will only pick up this diagnosis by thinking about it, so multiple myeloma + altered mental status = hyperviscosity syndrome Treatment is with IVF and plasmapheresis (heme onc consult) And don't forget common stuff, like stroke, subdural hematomas [umem.org]
Complications may include: Death of intestinal tissue (necrotizing enterocolitis) Decreased fine motor control Kidney failure Seizures Strokes Neonatal polycythemia; Hyperviscosity - newborn Diab Y, Luchtman-Jones L. [medlineplus.gov]
This can cause problems such as poor circulation to the brain, which can lead to symptoms like those from a stroke. [cancer.org]
- Dizziness
[…] minutes: (1) Palpitations, pounding heart, or accelerated heart rate (2) Sweating (3) Trembling or shaking (4) Sensations of shortness of breath or smothering (5) Feeling of choking (6) Chest pain or discomfort (7) Nausea or abdominal distress (8) Feeling dizzy [books.google.com]
Reference work entry DOI: https://doi.org/ 10.1007/3-540-29662-X_1304 Definition Often found in paraproteinemias most common Waldenström's macroglobulinemia, polycythemia, leukocytosis; symptoms include visual changes, headache, vertigo, nystagmus, dizziness [link.springer.com]
Hyperviscosity may be manifested by nasal bleeding, blurred vision, headaches, dizziness, nystagmus, deafness, diplopia, ataxia, paresthesias, or congestive heart failure. [mayomedicallaboratories.com]
Circulation system symptoms: In hyperviscosity syndrome, the thickened blood causes poor brain circulation, leading to problems like headache, confusion, and dizziness. [cancer.org]
She experienced progressive shortness of breath which culminated into persistent headaches, dizziness and bilateral chest pain. [oatext.com]
Workup
Serum viscosity is the laboratory test that establishes the diagnosis of hyperviscosity syndrome. However, viscosity can be dependent on the sample temperature if cryoglobulins are present [6] [7]. Furthermore, if macroglobulinemia is the cause of this pathology, then it can also lead to rouleaux formation, visible on blood smears [5] and platelet dysfunction.
Complete blood cell count may show high white blood cell numbers in leukemia and leukostasis. Patients are usually anemic, as a consequence of the underlying disease, but red blood cell number can also be high in polycythemia vera.
A bone marrow biopsy may diagnose the cause of the hyperviscosity syndrome: leukemia, myeloproliferative disorder, plasma cell dyscrasia.
Other informative tests include serum and urine protein electrophoresis (a monoclonal spike confirms the presence of a gammopathy), metabolic and electrolyte panels (hypercalcemia and hyponatremia are frequent), prothrombin time, activated partial thromboplastin time and international normalized ratio. Significant proteinuria also suggests a gammopathy like multiple myeloma or Waldenstrom's macroglobulinemia.
In presence of an infection cultures should be obtained and imaging studies, as appropriate, should be performed. Chest radiography, for instance, can help diagnose pulmonary infection and congestive heart failure, while craniocerebral computer tomography can exclude other causes of seizures and altered sensorium.
Ophthalmologic examination is important in establishing retinal vein engorgement [8], hemorrhages, exudates, and papilledema. These findings should be distinguished from those caused by hypertension, that may coexist in the same patient. Central vein occlusion may occur in late stages.
The clinician should keep in mind the fact that different methods of blood viscosity measurement may lead to different results. Hyperviscosity symptoms usually occur when the serum viscosity reaches 4-5 cp (normal range: 1.4-1.8 cp).
In children and newborns, hyperviscosity is more often due to polycythemia, therefore arterial or venous hematocrit should be measured. These patients often have associated hypoglycemia, hypocalcemia, thrombocytopenia, and hyperbilirubinemia due to increased red blood cell destruction. Further imaging studies demonstrate pulmonary hypertension, alveolar infiltrates, pleural effusions, increased systemic vascular resistance, increased myocardial strain and hypoperfusion of both peripheral (gut, kidney) and central (brain, myocardium) territories.
Serum
- Hyperglobulinemia
Hyperglobulinemia of multiple myeloma (MM) plays a role in hyperviscosity syndrome (HVS). [ncbi.nlm.nih.gov]
Incipient myelomatosis or "essential" hyperglobulinemia with fibrinogenopenia: A new syndrome? Acta Med Scand. 1944;! 17:216-247. 2. Bloch KJ, Maki DG. Hyperviscosity syndromes associated with immunoglobulin abnormality. [healio.com]
- Hyperglobulinemia
Hyperglobulinemia of multiple myeloma (MM) plays a role in hyperviscosity syndrome (HVS). [ncbi.nlm.nih.gov]
Incipient myelomatosis or "essential" hyperglobulinemia with fibrinogenopenia: A new syndrome? Acta Med Scand. 1944;! 17:216-247. 2. Bloch KJ, Maki DG. Hyperviscosity syndromes associated with immunoglobulin abnormality. [healio.com]
Pleura
- Pleural Effusion
286 888 Pregnancy Trauma 898 Foreign Body Esophageal 902 Procedural Sedation 904 Pseudotumor Cerebri 910 Pulmonary Contusion 920 Purpura 926 Peritonsillar Abscess 928 Phalangeal Injuries Hand 934 Phenytoin Poisoning 940 Pityriasis Rosea 946 Red 948 Pleural [books.google.com]
Further imaging studies demonstrate pulmonary hypertension, alveolar infiltrates, pleural effusions, increased systemic vascular resistance, increased myocardial strain and hypoperfusion of both peripheral (gut, kidney) and central (brain, myocardium) [symptoma.com]
Associated laboratory findings: Abnormal chest X-ray: cardiomegaly, increased vascularity, hyperaeration, alveolar infiltrates, pleural effusions. [uichildrens.org]
Treatment
The ocular findings were reversible after plasmapheresis and steroid treatment. Rheumatoid hyperviscosity syndrome can involve both retinal and choroidal circulation. The prominent microvasculopathy is reversible after appropriate treatment. [ncbi.nlm.nih.gov]
Definitive treatment of HVS is treatment of the underlying disorder (eg, chemotherapy). If the underlying disease process is left untreated, the hyperviscosity will recur. [emedicine.com]
Prognosis
Prognosis The overall prognosis for any patient will depend on the underlying condition and severity of any complications of hyperviscosity. [patient.info]
Prognosis Prognosis depends on the underlying cause of the hyperviscosity. For example, hyperviscosity secondary to an acute leukemia or multiple myeloma has a worse prognosis than that due to polycythemia vera or essential thrombocythemia. [clinicaladvisor.com]
Tujuan penelitian ini untuk mengetahui luaran (outcome) dan prognosis LLA dengan leukosit ≥50000/μL. Metode. Penelitian rancang bangun kohort retrospektif dilakukan di Instalasi Kesehatan Anak RSUP DR. [saripediatri.org]
Treatment and prognosis The International Prognostic Scoring System for Waldenström's macroglobulinemia is a predictive model for characterization of long-term outcome. [radiopaedia.org]
Clinical course, treatment, and prognosis of type I cryoglobulinemia largely depend on the underlying disorder. Patients with a benign monoclonal gammopathy of undetermined significance are generally asymptomatic or present with a mild disorder. [orpha.net]
Etiology
Hyperviscosity syndrome seems similar to central retinal vein occlusion and may be associated with systemic conditions such as diabetes and atherosclerosis; however, alternative etiologies should be considered in young otherwise healthy individuals. [ncbi.nlm.nih.gov]
Etiology The pathogenetic processes in simple cryoglobulinemia generally appear to be related to those of the underlying lymphoproliferative diseases. [orpha.net]
Cryoglobulinemia: study of etiologic factors and clinical and immunologic features in 443 patients from a single center. Medicine (Baltimore) 2001; 80:252. Kulger N, Sirvente J, Rigau V, Guillot B. [signavitae.com]
Epidemiology
Little epidemiologic information is available on hyperviscosity symdrome. [emedicine.com]
[…] 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. [ 1 ] Epidemiology [patient.info]
Epidemiology. Investigative techniques. Etiology Discipline Cardiology. [pascal-francis.inist.fr]
Pathophysiology
(Review of pathophysiology and effect of cytoreduction on prognosis in acute leukemia.) Porcu, P, Cripe, LD, Ng, EW. “Hyperleukocytic leukemias and leukostasis: a review of pathophysiology, clinical presentation and management”. [clinicaladvisor.com]
This review provides an overview of the pathophysiology of leukostasis and its management with leukapheresis. The use of TPE in the management of hyperviscosity syndrome is also discussed. [ncbi.nlm.nih.gov]
A basic knowledge of the principles of rheology is important in the understanding of its pathophysiology, especially the relationship between viscosity and flow conditions. [doi.org]
Prevention
Long-term management is directed at control of the underlying disease to prevent production of the monoclonal protein. [scholars.northwestern.edu]
In most cases, plasmapheresis is used in combination with corticosteroids and immunosuppressive drugs to prevent production of abnormal proteins or to treat the underlying disease. [ncbi.nlm.nih.gov]
Partial plasma exchange transfusion(XT) has been recommended as the treatment of choice in neonatal hyperviscosity although there remains no statistical proof that it prevents long-term CNS or other sequelae. [nature.com]
Medicine Seminars in thrombosis and hemostasis 2003 Symptomatic hyperviscosity is much more common in Waldenström's macroglobulinemia (10 to 30%) than it is in myeloma (2 to 6%) and long-term management is directed at control of the underlying disease to prevent [semanticscholar.org]
References
- Adams BD, Baker R, Lopez JA, Spencer S. Myeloproliferative disorders and the hyperviscosity syndrome. Emerg Med Clin North Am. 2009;27(3):459-76.
- Rajagopal R, Apte RS. Seeing through thick and through thin: Retinal manifestations of thrombophilic and hyperviscosity syndromes. Surv Ophthalmol. 2016;61(2):236-47.
- Sloop G, Holsworth RE Jr, Weidman JJ, St Cyr JA. The role of chronic hyperviscosity in vascular disease. Ther Adv Cardiovasc Dis. 2015;9(1):19-25.
- Mehta J, Singhal S. Hyperviscosity syndrome in plasma cell dyscrasias. Semin Thromb Hemost 2003;29:467-471.
- Stone MJ. Waldenström's macroglobulinemia: hyperviscosity syndrome and cryoglobulinemia. Clin Lymphoma Myeloma 2009;9(1):97-99.
- Ramos-Casals M, Stone JH, Cid MC, Bosch X. The cryoglobulinaemias. Lancet 2012;379(9813):348-360
- Della Rossa A, Tavoni A, Bombardieri S. Hyperviscosity syndrome in cryoglobulinemia: clinical aspects and therapeutic considerations. Semin Thromb Haemost 2003;9(5):473-477.
- Stone MJ, Pascual V. Pathophysiology of Waldenström's macroglobulinemia. Haematologica 2010;95:359-364.