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Hyperviscosity Syndrome

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.

Congestive Heart Failure
  • Physical examination disclosed hepatosplenomegaly, congestive heart failure, and multiple sites of bleeding.[ncbi.nlm.nih.gov]
  • 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]
  • 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]
  • heart failure with volume overload (rales, lower extremity edema, elevated jugular venous pressure, and hypoxia) Causes Increased serum viscosity usually results from increased circulating serum immunoglobulins and can be seen in Waldenström macroglobulinemia[emedicine.medscape.com]
  • Congestive heart failure is uncommon in rheumatoid HVS [20]. Only two cases of pulmonary arterial hypertension (PAH) leading to right heart failure have been reported [6,7].[oatext.com]
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]
Acrocyanosis
  • Type I cryoglobulinemia is frequently asymptomatic per se but patients may develop acrocyanosis, retinal hemorrhage, Raynaud's phenomenon, and arterial thrombosis.[orpha.net]
Petechiae
  • Petechiae: perdarahan kecil-kecil, sedikit (berupa titik-titik) dalam kulit, serosa, mukosa 7. Purpura: seperti petechiae tetapi lebih besar sedikit 8. Ecchymosis: hematoma subkutan diameter 1-2 cm, karena tindakan pengambilan darah. 9.[treesnasmart.blogspot.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]
  • 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]
  • Hyperviscosity may be manifested by oronasal bleeding, blurred vision, headaches, dizziness, nystagmus, deafness, diplopia, ataxia, paresthesias, or congestive heart failure.[mayomedicallaboratories.com]
  • 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]
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]
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]
Anuria
  • Ginjal: GFR menurun à iskemia tubular (oliguria) à nekrosis tubular à anuria à UREMIA 6. Adrenal: peningkatan produksi (aldosteron, glukokortikoid, katekolamin) 7. Traktus gastrointestinal: hemoragi (kolon), ulkus Curling 8.[treesnasmart.blogspot.com]
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]
  • 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]
  • The remaining patients had transient complications such as headache, short of breath and hot flush probably due to the circulatory disturbance.[ncbi.nlm.nih.gov]
  • Seite 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: 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[link.springer.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: 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[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]
  • These complications can include: stroke kidney failure decreased motor control loss of movement the death of intestinal tissue recurrent seizures Be sure to report any symptoms your baby is having to their doctor right away.[healthline.com]
  • Differential diagnoses will depend on the patient's presenting symptoms, but include coagulopathy, hemorrhagic or ischemic stroke, congestive heart failure, pulmonary edema and diabetic ketoacidosis.[clinicaladvisor.com]
  • 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, meningitis[umem.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: 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[link.springer.com]
  • She experienced progressive shortness of breath which culminated into persistent headaches, dizziness and bilateral chest pain.[oatext.com]
  • Hyperviscosity may be manifested by oronasal 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]
Paresthesia
  • 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]
  • Hyperviscosity syndrome is characterized by impaired tissue oxygen delivery with symptoms like headache, visual disturbances, loss of concentration, paresthesia, muscle weakness, and fatigue.[cardiophile.org]
  • 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]
  • […] discomfort (7) nausea or abdominal distress (8) feeling dizzy, unsteady, lightheaded, or faint (9) derealization (feelings of unreality) or depersonalization (being detached from oneself) (10) fear of losing control or going crazy (11) fear of dying (12) paresthesias[books.google.com]
  • Hyperviscosity may be manifested by oronasal bleeding, blurred vision, headaches, dizziness, nystagmus, deafness, diplopia, ataxia, paresthesias, or congestive heart failure.[mayomedicallaboratories.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.

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]
  • It is the treatment of choice in hyperviscosity syndrome, due to the presence of quantitatively or qualitatively abnormal plasma proteins such as paraproteins.[ncbi.nlm.nih.gov]
  • Treatment includes hydration with diuresis, plasmapheresis, and control of the underlying disease. The current treatment for WM is chemotherapy (i.e., alkylating agents and nucleoside analogs) and the monoclonal antibody rituximab.[ncbi.nlm.nih.gov]
  • We conclude that plasma viscosity is a major determinant of capillary blood flow and that plasmapheresis is an efficient treatment of abnormal microcirculation caused by increased plasma viscosity.[ncbi.nlm.nih.gov]
  • Effects of combined myeloma treatment and prognostic value of serum IL-2 level and absolute number of CD3 determination are discussed.[ncbi.nlm.nih.gov]

Prognosis

  • 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 macroglobulinaemia is a predictive model for characterisation 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]
  • 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]
  • Etiology The pathogenetic processes in simple cryoglobulinemia generally appear to be related to those of the underlying lymphoproliferative diseases.[orpha.net]
  • DEHIDRASI PRIMER • Terjadi akibat pemasukan cairan yg sedikit/kehilangan cairan tanpa elektrolit. • Olahraga yg berat disertai keluarnya keringat yg banyak • Etiologi: 1. Gangguan menelan 2.[treesnasmart.blogspot.com]
  • Bukti menunjukkan bahawa etiologi panmyelosis adalah proliferasi neoplastik yang tidak dikawal. Asal transformasi sel stem masih tidak diketahui. Gambar 1.[lovedoc.org]

Epidemiology

  • Little epidemiologic information is available on hyperviscosity symdrome.[emedicine.medscape.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]
  • Summary Epidemiology The prevalence is unknown.[orpha.net]
  • Epidemiology Hyperviscosity syndrome secondary to monoclonal gammopathies The most common cause of hyperviscosity syndrome due to increased paraproteins is Waldenstrom's macroglobulinemia. HVS has been reported in 10-30% of these patients.[clinicaladvisor.com]
  • Mieloma memiliki insiden yang tinggi pada kelompok etnik Afro-Carribean dibandingkan Kaukasian tapi itu hanya sedikit dari epidemiologi khusus.[docslide.com.br]
Sex distribution
Age distribution

Pathophysiology

  • 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]
  • The proper understanding of the causes, pathophysiology, diagnosis and management of the plasma hyperviscosity syndrome is based on good knowledge of malignant paraproteinaemias, properties of immunoglobulins, rheology of blood in the microcirculation[ncbi.nlm.nih.gov]
  • (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]
  • Pathophysiology Viscosity is a property of liquid and is described as the resistance that a liquid exhibits to the flow of one layer over another. As serum proteins or cellular components increase, the blood becomes more viscous.[emedicine.medscape.com]
  • Useful For Suggests clinical disorders or settings where the test may be helpful Detection of increased viscosity Monitoring patients with hyperviscosity syndrome Clinical Information Discusses physiology, pathophysiology, and general clinical aspects[mayomedicallaboratories.com]

Prevention

  • 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]
  • Long-term management is directed at control of the underlying disease to prevent production of the monoclonal protein.[scholars.northwestern.edu]
  • 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]
  • Long-term management is directed at control of the underlying disease to prevent production of the monoclonal protein . A consult to a hematologist/oncologist is required. [3, 4] References / Further Reading Bekelman J, Jackson N, Donehower R.[emdocs.net]

References

Article

  1. Adams BD, Baker R, Lopez JA, Spencer S. Myeloproliferative disorders and the hyperviscosity syndrome. Emerg Med Clin North Am. 2009;27(3):459-76.
  2. Rajagopal R, Apte RS. Seeing through thick and through thin: Retinal manifestations of thrombophilic and hyperviscosity syndromes. Surv Ophthalmol. 2016;61(2):236-47.
  3. 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.
  4. Mehta J, Singhal S. Hyperviscosity syndrome in plasma cell dyscrasias. Semin Thromb Hemost 2003;29:467-471.
  5. Stone MJ. Waldenström's macroglobulinemia: hyperviscosity syndrome and cryoglobulinemia. Clin Lymphoma Myeloma 2009;9(1):97-99.
  6. Ramos-Casals M, Stone JH, Cid MC, Bosch X. The cryoglobulinaemias. Lancet 2012;379(9813):348-360
  7. Della Rossa A, Tavoni A, Bombardieri S. Hyperviscosity syndrome in cryoglobulinemia: clinical aspects and therapeutic considerations. Semin Thromb Haemost 2003;9(5):473-477.
  8. Stone MJ, Pascual V. Pathophysiology of Waldenström's macroglobulinemia. Haematologica 2010;95:359-364.

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Last updated: 2018-06-21 23:48