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Hyperviscosity Syndrome

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1. Acute hyperviscosity: syndromes and management. (PubMed)

Acute hyperviscosity: syndromes and management. Plasma hyperviscosity is a rare complication of both monoclonal and polyclonal disorders associated with elevation of immunoglobulins. Asymptomatic patients with an elevation in the serum viscosity do not require plasma exchange, and the majority will have other indications for therapeutic intervention. For patients with hemorrhagic or central nervous system manifestations, plasma exchange is the therapy of choice and is relatively safe. Viscosity (...) measurements are not required to initiate therapy if the index of suspicion is high and the clinical presentation is typical. However, patients should have a sample sent for confirmation of the diagnosis. Whole-blood hyperviscosity is seen in patients with extreme elevation of the red cell and white cell count. Phlebotomy of patients with primary and secondary elevation of the red cell count is a well-established therapy.© 2018 by The American Society of Hematology.

2018 Blood

2. Hyperviscosity Syndrome

Hyperviscosity Syndrome Hyperviscosity Syndrome Toggle navigation Brain Head & Neck Chest Endocrine Abdomen Musculoskeletal Skin Infectious Disease Hematology & Oncology Cohorts Diagnostics Emergency Findings Procedures Prevention & Management Pharmacy Resuscitation Trauma Emergency Procedures Ultrasound Cardiovascular Emergencies Lung Emergencies Infectious Disease Pediatrics Neurologic Emergencies Skin Exposure Miscellaneous Abuse Cancer Administration 4 Hyperviscosity Syndrome Hyperviscosity (...) Syndrome Aka: Hyperviscosity Syndrome , Leukostasis From Related Chapters II. Causes Oncology related cases (10-30% of cases) Other causes III. Pathophysiology Overall increased serum viscosity Dramatically increased cell counts s: s: Leukostasis (with s >100,000) Especially seen in acute leukemic blast crisis (AML or CML) Increased circulating serum s s coat s Results in increased viscosity and sludging of blood cells End result is end organ decreased perfusion IV. Signs Spontaneous bleeding from

2018 FP Notebook

3. Plasmacytic post-transplant lymphoproliferative disorder with hyperviscosity syndrome in a child after liver transplant (PubMed)

Plasmacytic post-transplant lymphoproliferative disorder with hyperviscosity syndrome in a child after liver transplant 27227484 2018 01 31 2018 11 13 1527-3350 64 6 2016 12 Hepatology (Baltimore, Md.) Hepatology Plasmacytic posttransplant lymphoproliferative disorder with hyperviscosity syndrome in a child after liver transplant. 2250-2252 10.1002/hep.28657 Yang Christine H CH Division of Pediatric Gastroenterology, Hepatology, and Nutrition, Department of Pediatrics, Lucile Packard Children's

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2016 Hepatology (Baltimore, Md.)

4. Seeing through thick and through thin: Retinal manifestations of thrombophilic and hyperviscosity syndromes. (PubMed)

Seeing through thick and through thin: Retinal manifestations of thrombophilic and hyperviscosity syndromes. The presence of retinal vasculopathy in the absence of typical predisposing factors should suggest a possible underlying hematologic abnormality. In such cases, a systemic investigation may reveal a potentially fatal hypercoagulability or hyperviscosity syndrome. Retinal vein occlusion is the most commonly encountered ophthalmic finding in such syndromes; however, abnormalities (...) of the arterial system, the choroid, and the macula are also possible. Visual symptoms may be the only manifestation of the underlying process, making timely diagnosis by the ophthalmologist critical for both treatment and thrombotic prophylaxis. Moreover, as newer ophthalmic diagnostic technologies arise, there is an increasingly important role for eye physicians in the management of such syndromes. Copyright © 2016 Elsevier Inc. All rights reserved.

2015 Survey of Ophthalmology

5. Polyclonal hyperviscosity syndrome in IgG4-related disease and associated conditions (PubMed)

Polyclonal hyperviscosity syndrome in IgG4-related disease and associated conditions Polyclonal hyperviscosity syndrome (HVS) is rare and has been reported in various disorders of immune dysregulation and lymphoid hyperplasia. IgG4-Related Disease (IgG4-RD) is an emerging disorder often associated with exuberant hypergammaglobulinemia, and this review of seven cases establishes IgG4-RD as an important cause of polyclonal HVS.

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2015 Clinical Case Reports

6. Hyperviscosity Syndrome (Treatment)

Hyperviscosity Syndrome (Treatment) Hyperviscosity Syndrome Treatment & Management: Prehospital Care, Emergency Department Care, Consultations Edition: No Results No Results Please confirm that you would like to log out of Medscape. If you log out, you will be required to enter your username and password the next time you visit. https://profreg.medscape.com/px/getpracticeprofile.do?method=getProfessionalProfile&urlCache=aHR0cHM6Ly9lbWVkaWNpbmUubWVkc2NhcGUuY29tL2FydGljbGUvNzgwMjU4LXRyZWF0bWVudA (...) == processing > Hyperviscosity Syndrome Treatment & Management Updated: Feb 21, 2018 Author: Thomas J Hemingway, MD, FACEP; Chief Editor: Barry E Brenner, MD, PhD, FACEP Share Email Print Feedback Close Sections Sections Hyperviscosity Syndrome Treatment Prehospital Care No specific prehospital care is indicated for patients with hyperviscosity syndrome. Emergency medical services personnel should be attentive to airway, breathing, and circulation (the ABCs) and provide symptomatic support. [ ] Next

2014 eMedicine Emergency Medicine

7. Hyperviscosity Syndrome (Overview)

Hyperviscosity Syndrome (Overview) Hyperviscosity Syndrome: Practice Essentials, Pathophysiology, Epidemiology Edition: No Results No Results Please confirm that you would like to log out of Medscape. If you log out, you will be required to enter your username and password the next time you visit. https://profreg.medscape.com/px/getpracticeprofile.do?method=getProfessionalProfile&urlCache=aHR0cHM6Ly9lbWVkaWNpbmUubWVkc2NhcGUuY29tL2FydGljbGUvNzgwMjU4LW92ZXJ2aWV3 processing > Hyperviscosity (...) Syndrome Updated: Feb 21, 2018 Author: Thomas J Hemingway, MD, FACEP; Chief Editor: Barry E Brenner, MD, PhD, FACEP Share Email Print Feedback Close Sections Sections Hyperviscosity Syndrome Overview Practice Essentials Hyperviscosity syndrome (HVS) refers to the clinical sequelae of increased blood viscosity. Increased serum viscosity usually results from increased circulating serum immunoglobulins and can be seen in such diseases as and . [ ] HVS can also result from increased cellular blood

2014 eMedicine Emergency Medicine

8. Hyperviscosity Syndrome (Diagnosis)

Hyperviscosity Syndrome (Diagnosis) Hyperviscosity Syndrome: Practice Essentials, Pathophysiology, Epidemiology Edition: No Results No Results Please confirm that you would like to log out of Medscape. If you log out, you will be required to enter your username and password the next time you visit. https://profreg.medscape.com/px/getpracticeprofile.do?method=getProfessionalProfile&urlCache=aHR0cHM6Ly9lbWVkaWNpbmUubWVkc2NhcGUuY29tL2FydGljbGUvNzgwMjU4LW92ZXJ2aWV3 processing > Hyperviscosity (...) Syndrome Updated: Feb 21, 2018 Author: Thomas J Hemingway, MD, FACEP; Chief Editor: Barry E Brenner, MD, PhD, FACEP Share Email Print Feedback Close Sections Sections Hyperviscosity Syndrome Overview Practice Essentials Hyperviscosity syndrome (HVS) refers to the clinical sequelae of increased blood viscosity. Increased serum viscosity usually results from increased circulating serum immunoglobulins and can be seen in such diseases as and . [ ] HVS can also result from increased cellular blood

2014 eMedicine Emergency Medicine

9. Hyperviscosity Syndrome (Follow-up)

Hyperviscosity Syndrome (Follow-up) Hyperviscosity Syndrome Follow-up: Further Inpatient Care, Transfer, Prognosis Edition: No Results No Results Please confirm that you would like to log out of Medscape. If you log out, you will be required to enter your username and password the next time you visit. https://profreg.medscape.com/px/getpracticeprofile.do?method=getProfessionalProfile&urlCache=aHR0cHM6Ly9lbWVkaWNpbmUubWVkc2NhcGUuY29tL2FydGljbGUvNzgwMjU4LWZvbGxvd3Vw processing > Hyperviscosity (...) Syndrome Follow-up Updated: Feb 21, 2018 Author: Thomas J Hemingway, MD, FACEP; Chief Editor: Barry E Brenner, MD, PhD, FACEP Share Email Print Feedback Close Sections Sections Hyperviscosity Syndrome Follow-up Further Inpatient Care Supportive care should be initiated for the complications of hyperviscosity syndrome, pending definitive therapy. Complications to address may include blood loss, central nervous system disorders, cardiovascular effects, and metabolic derangements. Note that the definitive

2014 eMedicine Emergency Medicine

10. Hyperviscosity Syndrome

Hyperviscosity Syndrome Hyperviscosity Syndrome Toggle navigation Brain Head & Neck Chest Endocrine Abdomen Musculoskeletal Skin Infectious Disease Hematology & Oncology Cohorts Diagnostics Emergency Findings Procedures Prevention & Management Pharmacy Resuscitation Trauma Emergency Procedures Ultrasound Cardiovascular Emergencies Lung Emergencies Infectious Disease Pediatrics Neurologic Emergencies Skin Exposure Miscellaneous Abuse Cancer Administration 4 Hyperviscosity Syndrome Hyperviscosity (...) Syndrome Aka: Hyperviscosity Syndrome , Leukostasis From Related Chapters II. Causes Oncology related cases (10-30% of cases) Other causes III. Pathophysiology Overall increased serum viscosity Dramatically increased cell counts s: s: Leukostasis (with s >100,000) Especially seen in acute leukemic blast crisis (AML or CML) Increased circulating serum s s coat s Results in increased viscosity and sludging of blood cells End result is end organ decreased perfusion IV. Signs Spontaneous bleeding from

2015 FP Notebook

11. Investigation and management of Transient Leukaemia of Down Syndrome

Investigation and management of Transient Leukaemia of Down Syndrome Guidelines for the investigation and management of Transient Leukaemia of Down Syndrome - Tunstall - 2018 - British Journal of Haematology - Wiley Online Library By continuing to browse this site, you agree to its use of cookies as described in our . Search within Search term Search term The full text of this article hosted at iucr.org is unavailable due to technical difficulties. Guideline Free Access Guidelines (...) for the investigation and management of Transient Leukaemia of Down Syndrome Writing Group Chair Bristol Royal Hospital for Children, University Hospitals Bristol NHS Trust, Bristol, UK John Radcliffe Hospital, Oxford University Hospitals NHS Trust and Oxford BRC Blood Theme, NIHR Oxford Biomedical Centre, Oxford, UK Leeds Children's Hospital, Leeds Teaching Hospitals NHS Trust, Leeds, UK Birmingham Children's Hospital NHS Trust, Birmingham, UK Our Lady's Children's Hospital, Crumlin, Dublin Evelina London

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2018 British Committee for Standards in Haematology

12. Aggregation of γG3 proteins: relevance to the hyperviscosity syndrome (PubMed)

Aggregation of γG3 proteins: relevance to the hyperviscosity syndrome Studies on the sera and isolated proteins from 14 patients with gammaG3 multiple myeloma revealed a concentration- and temperature-dependent aggregation which was not encountered in 26 sera from patients with multiple myeloma involving other gammaG subgroups. When the gammaG3 myeloma sera were diluted and characterized by analytical ultracentrifugation, complex formation was minimal. However, when these sera were examined (...) undiluted, marked complex formation was observed. Studies on the isolated proteins, their enzymatic fragments, as well as their heavy and light polypeptide chains localized the aggregating sites to the Fd fragment of the heavy chains. The findings were also documented by acrylamide-gel electrophoresis and capillary tube viscometry.THE HYPERVISCOSITY SYNDROME WAS OBSERVED IN SIX PATIENTS: three with gammaG3 myeloma and three with gammaG1 myeloma. In the latter group extreme protein concentrations

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1970 Journal of Clinical Investigation

13. The hyperviscosity syndrome: I. In IgG myeloma. The role of protein concentration and molecular shape (PubMed)

The hyperviscosity syndrome: I. In IgG myeloma. The role of protein concentration and molecular shape The hyperviscosity syndrome is an uncommon complication in IgG myeloma. Its occurrence has been ascribed to the presence in the serum of high molecular weight polymers of the IgG proteins. Three patients with IgG myeloma and the clinical hyperviscosity syndrome were investigated, none of whom had IgG polymers in the serum by analytical ultracentrifugation. Relative serum viscosity (...) molecular radius (58.2 A) and was asymmetrical (intrinsic viscosity 10.2 cc/g, frictional ratio 1.63). 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.

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1970 Journal of Clinical Investigation

14. Hyperviscosity syndrome. (PubMed)

Hyperviscosity syndrome. 4995894 1971 07 06 2018 11 13 0007-1447 2 5755 1971 Apr 24 British medical journal Br Med J Hyperviscosity syndrome. 184-5 eng Journal Article England Br Med J 0372673 0007-1447 0 Macromolecular Substances 0 Proteins AIM IM Blood Viscosity Epistaxis etiology Female Gastrointestinal Hemorrhage etiology Hematologic Diseases Humans Macromolecular Substances Menorrhagia etiology Multiple Myeloma complications Oral Hemorrhage etiology Proteins Waldenstrom Macroglobulinemia

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1971 British medical journal

15. Perinatal Polycythemia and Hyperviscosity Syndrome

Perinatal Polycythemia and Hyperviscosity Syndrome Perinatal Polycythemia and Hyperviscosity Syndrome - Pediatrics - MSD Manual Professional Edition Brought to you by The trusted provider of medical information since 1899 SEARCH SEARCH MEDICAL TOPICS Common Health Topics Resources QUIZZES & CASES Quizzes Cases The trusted provider of medical information since 1899 SEARCH SEARCH MEDICAL TOPICS Common Health Topics Resources QUIZZES & CASES Quizzes Cases / / / / IN THIS TOPIC OTHER TOPICS (...) in .) The terms polycythemia and hyperviscosity are often used interchangeably but are not equivalent. Polycythemia is significant only because it increases risk of hyperviscosity syndrome. Hyperviscosity is a clinical syndrome caused by sludging of blood within vessels. Sludging occurs because increased RBC mass causes a relative decrease in plasma volume and a relative increase in proteins and platelets. Incidence of polycythemia is about 3 to 4% (range 0.4 to 12%), and about half of infants

2013 Merck Manual (19th Edition)

16. Cellular hyperviscosity as a cause of neurological symptoms in leukaemia. (PubMed)

intravascular coagulation) two days later. It is concluded that a cellular hyperviscosity syndrome may cause neurological dysfunction in patients with extremely high white cell counts, and that leucapheresis, in carefully selected patients, can be an effective method of treatment. (...) Cellular hyperviscosity as a cause of neurological symptoms in leukaemia. Six patients with various forms of leukaemia had neurological signs and symptoms associated with an extremely high white blood cell count and increased whole blood (but not plasma) viscosity. All were treated by leucapheresis with an Aminco Celltrifuge. Rapid and complete reversal of all symptoms occurred in three patients and partial recovery in one. One patient died shortly after leucapheresis and another (from cerebral

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1978 British medical journal

17. Antibody-mediated tissue damage. Hyperviscosity and other complications of paraproteinaemia. (PubMed)

Antibody-mediated tissue damage. Hyperviscosity and other complications of paraproteinaemia. 391830 1980 03 27 2018 11 13 0144-0330 13 1979 Journal of clinical pathology. Supplement (Royal College of Pathologists) J Clin Pathol Suppl (R Coll Pathol) Antibody-mediated tissue damage. Hyperviscosity and other complications of paraproteinaemia. 85-9 Preston F E FE eng Journal Article Review England J Clin Pathol Suppl (R Coll Pathol) 8300543 0144-0330 0 Antibodies 0 Immunoglobulin A 0 (...) Immunoglobulin G 0 Immunoglobulin M IM Antibodies Blood Viscosity Blood Volume Eye Manifestations Hematocrit Hemorrhage complications Humans Immunoglobulin A Immunoglobulin G Immunoglobulin M Neurologic Manifestations Paraproteinemias complications etiology therapy Plasmapheresis Syndrome Vascular Diseases diagnosis Vascular Resistance 20 1979 1 1 1979 1 1 0 1 1979 1 1 0 0 ppublish 391830 PMC1521599 N Engl J Med. 1960 Sep 22;263:574-9 14443924 JAMA. 1965 May 10;192:464-7 14284847 Surgery. 1964 Jun;55:825-30

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1979 Journal of Clinical Pathology. Supplement (Royal College of Pathologists).

18. Paraneoplastic acral vascular syndrome in a patient with metastatic melanoma under immune checkpoint blockade. (PubMed)

Paraneoplastic acral vascular syndrome in a patient with metastatic melanoma under immune checkpoint blockade. Paraneoplastic acral vascular syndrome (PAVS) is a rare phenomenon which is observed in patients with adenocarcinomas and other malignancies. Various potential pathogenic mechanisms such as tumour invasion of sympathetic nerves, hyperviscosity, hypercoagulability, vasoactive tumour-secreted substances, and immunological mechanisms have been suggested.We report a 60-year-old Caucasian

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2017 BMC Cancer

19. A Case of Advanced Glaucoma with Increased Episcleral Venous Pressure in a 17-Year-Old with Eisenmenger Syndrome (PubMed)

of patients suffering from thrombosis due to hyperviscosity associated with this syndrome; however, to our knowledge, the association of secondary open angle glaucoma with Eisenmenger syndrome has not yet been documented. (...) A Case of Advanced Glaucoma with Increased Episcleral Venous Pressure in a 17-Year-Old with Eisenmenger Syndrome Eisenmenger syndrome refers to reversal of shunt and central cyanosis due to pulmonary hypertension induced by congenital heart disease with a large systemic-to-pulmonary shunt. We report a case of a 17-year-old man with Eisenmenger syndrome who presented with gradual deterioration in visual acuity and was diagnosed with advanced secondary open angle glaucoma. There have been reports

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2017 Case reports in ophthalmological medicine

20. Bing-Neel Syndrome Case Report: A Previously Undocumented IgG Variant with MRI, PET/CT, and PET/MRI Imaging (PubMed)

Bing-Neel Syndrome Case Report: A Previously Undocumented IgG Variant with MRI, PET/CT, and PET/MRI Imaging Waldenstrom's macroglobulinaemia is the most commonly reported subtype of lymphoplasmacytic lymphoma (LPL); it is characterised by IgM secretion. Neurological complications are common usually as a result of hyperviscosity. In rare cases, cells can infiltrate the central nervous system; this is known as Bing-Neel syndrome. We report the case of a 57-year-old male with lymphoplasmacytic (...) lymphoma of the IgG-subtype with neurological symptoms and the consequent finding of lymphoplasmacytoid cells in his cerebrospinal fluid as well as deposits on MRI and PET-CT imaging. This is the first report of Bing-Neel syndrome in IgG-subtype LPL. We discuss the biological and radiological markers of his disease, including PET imaging, which has been minimal in this area to date.

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2016 Case reports in hematology

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