Hyperviscosity syndrome (HVS) refers to the clinical sequelae of increased blood viscosity. government site. Although these treatments are helpful in the acute phase, they typically do not alter the prognosis of the disease process, which is the underlying etiology. Other health conditions can cause the same signs and symptoms as multiple myeloma. As MM cancer cells grow and proliferate within the bone marrow, it struggles to produce enough healthy blood cells. Thirty-nine NDMM patients with HVS were included. MM affects the nervous system in around 1% of people with the condition. The .gov means its official. official website and that any information you provide is encrypted We investigated a retrospective cohort study of NDMM with HVS between 2011-2021. Overall survival (OS) was calculated from the MM diagnosis until death or the last follow-up (whichever occurred first). 2011 Apr;82(4):224-30. doi: 10.1016/j.optm.2010.10.009. Retina. Haemostasis disorder with HVS in NDMM was confirmed in our case-controls analysis related to lower fibrinogen level and slightly elevated INR, both likely related to haemodilution (correlation with M-protein level). 2017 Jul. Waldenstrom macroglobulinemia. However, there is some evidence that various factors can make MM more likely, including: Doctors need to perform various tests to diagnose MM, including: According to research, there are several staging systems for MM, with modern staging systems measuring blood chemicals, such as: Thanks to new treatments and improved supportive care, survival rates for people with MM have significantly increased over the last few decades. People with MGUS have low levels of a blood protein called monoclonal protein, or M protein. Multiple myeloma. J Clin Apher (2019) 34:171354. government site. Article writing: P-ED, SH, BR, AT. Clinical Journal of Oncology Nursing, 8(5), 473-480. These diseases (eg, multiple myeloma, Waldenstrm macroglobulinemia, blood dyscrasias) should be definitively treated with the appropriate oncologic therapy, or the HVS will typically recur within a few weeks, requiring further pheresis. This site needs JavaScript to work properly. Optical coherence tomography images of the right (c) and left (d) eyes revealed near total absorbance of subretinal fluid in the right eye and a notable decrease in subretinal fluid (asterisk) in the left eye. Is the ketogenic diet right for autoimmune conditions? Seminars in Thrombosis and Hemostasis, 29(5), 535-542. Zarkovic, M., & Kwaan, H. C. (2003). Fluid substitution was albumin 5% alone for 60%, and albumin 5% + fresh frozen plasma (FFP) for 40% of the patients. Scientists do not know the exact cause of MM. PMC -, Sanders TE, Podos SM, Rosenbaum LJ. PRUZANSKl W. Serum viscosity and hyperviscosity syndrome in IgG multiple myeloma: Report on 10 patients and a review of the literature. The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. (2003). The diagnostic hypothesis was hyperviscosity syndrome associated with multiple myeloma reactivation. Hyperviscosity in IgG myeloma: detection and treatment in community medical facilities. Clin Lymphoma Myeloma Leuk. Emergency medical services personnel should be attentive to airway, breathing, and circulation (the ABCs) and provide symptomatic support. The diagnosis of hyperviscosity syndrome (HVS) is confirmed by measurement of elevated serum viscosity in a patient with characteristic clinical manifestations of HVS. Serum viscosity and hyperviscosity syndrome in IgG multiple myeloma. doi:10.1016/0277-5379(91)90020-E, 9. Caimi G, Carlisi M, Urso C, Lo Presti R, Hopps E. Clinical disorders responsible for plasma hyperviscosity and skin complications. Before symptoms? In comparison with NDMM control (n =76, median follow-up of 5 years, IQR 2.7-8), no difference was observed for paired criteria, cytogenetics, and percentage of auto-HSCT performance (Table3; Supplementary Table2). Monoclonal gammopathy is a family of disorders in which monoclonal gammopathy of unknown significance (MGUS) and smoldering myeloma are the asymptomatic variants whereas multiple myeloma is the malignant variant showing different signs and symptoms related to bone lesions, renal failure and anemia. Six patients (16%) had sequela or complications related to HVS: three (8%) early deaths and three (8%) ophthalmologic sequelae. Scientists believe, however, that cases of MM could almost double within the next 20 years. National Comprehensive Cancer Network. The .gov means its official. [QxMD MEDLINE Link]. It is most commonly observed in Waldenstrom macroglobulinemia (WM), affecting 10-30% of patients, and has been reported in 2-6% of patients with multiple myeloma ( 1 - 6 ). e20028 Background: Multiple myeloma (MM) is a disease predominantly of older adults with an average age of 69 at diagnosis. 3 (4):217-26. Lancet Oncol (2016) 17:e32846. A single-center study can reduce the bias of treatment heterogeneity over ten years for the case-control analysis. Conclusion: Although the association between systemic sclerosis and multiple myeloma is rare, it should be remembered in cases of . At 66 years of age, he presented with epistaxis, gait instability, and somnolence. This site needs JavaScript to work properly. Perez Rogers A, Estes M. Hyperviscosity Syndrome. Twenty-one patients (58%) received TPE in the apheresis center with centrifugation, 12 (33%) in the ICU with filtration, and 3 (9%) in both units. Written informed consent for participation was not required for this study in accordance with the national legislation and the institutional requirements. Multiple myeloma (MM) accounts for 10% of all hematologic malignancies. Kumar S, Paiva B, Anderson KC, Durie B, Landgren O, Moreau P, et al. 2021 Jan. [QxMD MEDLINE Link]. Copyright 2023 Oncology Nursing Society. [citation needed] The median time to the next treatment was 2.1 years (95%IC 1.43.4, Figure2B). Hyperviscosity syndrome (HVS) is a rare complication of newly diagnosed multiple myeloma (NDMM) related to high tumour burden. Hyperviscosity syndrome in plasma cell dyscrasias. In the absence of concurrent effective chemotherapy for MM, a rebound phenomenon was described as related to persistent M-protein production (10). 2013;48:e245. Epub 2017 Dec 14. Lancet (2019) 394:2938. Eur J Cancer Clin Oncol (1991) 27:14015. Revised international staging system for multiple myeloma: A report from international myeloma working group. No use, distribution or reproduction is permitted which does not comply with these terms. 1981 Nov-Dec;26(3):157-69. doi: 10.1016/0039-6257(81)90065-5. Clinicopathologic features in two cases of multiple myeloma. Next review due: 02 June 2024, numbness and weakness in the legs and feet, problems controlling your bladder and bowels. It can lead to bone, blood, kidney, and sometimes neurological problems. Experts understand the general processes driving MM. Multiple myeloma may not cause any signs or symptoms in its early stages. If plasma/cellular pheresis is not readily available and the patient is decompensating, one may try vigorous intravenous hydration coupled with a 2-3 unit phlebotomy in the interim as a temporizing measure. FOIA Asymptomatic patients with an elevation in the serum viscosity do not require plasma exchange, and the majority will have other indications for therapeutic intervention. [ 3, 4] Constitutional symptoms and cardiorespiratory symptoms may. FOIA Postgraduate Medical Journal, 83(978), 217-223. Signs and symptoms often appear once the tumour grows in the bone marrow or somewhere outside of the bone marrow. Br J Ophthalmol. (2007). [8]. Hyperviscosity syndrome in IgA multiple myeloma Hyperviscosity syndrome - Cancer Therapy Advisor Prevalence of corneal findings and their interrelation with hematological findings in monoclonal gammopathy. Ethical review and approval was not required for the study on human participants in accordance with the local legislation and institutional requirements. Hidden in the Eyes-Recurrence of Systemic Hemopathies Reportedly "In Remission": Six Cases and Review of Literature. 10:815. Among MM patients, 36% experienced this complication at diagnosis in cohorts prior to the 21st century (7, 8). Diagnosis and treatment of multiple myeloma. The clinical triad of HVS includes mucosal bleeding (epistaxis, gum), visual abnormalities (blurring, papilledema, retinal bleeding, venule thrombosis), and neurological abnormalities (drowsiness, ataxia, cerebral bleeding) (4). Early detection may permit safer and effective treatment. Learn more. Nevertheless, only two patients (8%) were included in a clinical trial. -, Chin KJ, Kempin S, Milman T, Finger PT. Frontiers | Hyperviscosity Syndrome in Paraprotein Secreting Conditions Multiple myeloma may cause ocular pathology by direct infiltration or as extramedullary plasmacytomas resulting in displacement or compression of tissues, by causing hyperviscosity syndrome, and by immunoglobulin light chain deposition in ocular tissues. This site needs JavaScript to work properly. Can J Ophthalmol. Park, M. S., Kim, B. C., Kim, I. K., Lee, S. H., Choi, S. M., Kim, M. K., et al. Central retinal vein occlusions due to hyperviscosity syndrome. Prognosis of hyperviscosity syndrome in newly diagnosed multiple [3] Risk Factors Waldenstrom macroglobulinemia has an incidence of 3 per million people per year. (b), (a) Histopathology with H and E, 400 showed diffuse infiltration of plasmacytoid cells, Fundus photography of (a) right and (b) left eyes 3 weeks after chemotherapy, MeSH Please enable it to take advantage of the complete set of features! 2015 Feb. 9 (1):19-25. Some cohorts (18, 20) observed that IgG3 subclass was associated with HVS in IgG myeloma, which was not confirmed (7, 21). 2007 Oct-Dec;26(4):265-8. doi: 10.4314/wajm.v26i4.28324. 2017 Dec. 31 (6):927-940. HHS Vulnerability Disclosure, Help Hyperviscosity syndrome occurs when your blood thickens so much that it doesn't flow freely through your blood vessels. Hyperviscosity syndrome in multiple myeloma - PubMed Semin Thromb Hemost (2003) 29:46771. Thirty-nine NDMM patients with HVS were included. Ocular manifestations of multiple myeloma: three cases and a review of the literature. Information about multiple myeloma and ophthalmic manifestations was obtained from original journal articles, review articles, case reports and standard postgraduate textbooks. Genetic abnormalities and survival in multiple myeloma: the experience of the intergroupe francophone du mylome. Hu WL, Song JY, Li X, Pei XJ, Zhang JJ, Shen M, Tang R, Pan ZY, Huang ZX. We saw the efficacy of TPE associated with anti-plasma cell therapy with 86% of full recovery after one or two sessions and a good safety profile (no severe reaction during procedure). We also look at the causes of the condition, as well as diagnosis and treatment options. Although the patient was treated with chemotherapy and autologous stem cell transplantation soon after referral, he did not survive due to the aggressive course of the disease. 2020. National Comprehensive Cancer Network. https://profreg.medscape.com/px/getpracticeprofile.do?method=getProfessionalProfile&urlCache=aHR0cHM6Ly9lbWVkaWNpbmUubWVkc2NhcGUuY29tL2FydGljbGUvNzgwMjU4LXRyZWF0bWVudA==. Hyperviscosity Retinopathy and Immunogammopathy Maculopahy as New Onset of Multiple Myeloma. Intraocular manifestations of multiple myeloma. Can diet and exercise reverse prediabetes? doi:10.3816/CLML.2010.n.080, 12. Hyperviscosity syndrome with pulmonary involvement. (2019). This study is limited by the monocentric retrospective design. No exact diagnostic. Ann Intern Med (1972) 77:853. doi:10.7326/0003-4819-77-6-853, 8. Multiple myeloma. No major ischemic or bleeding was observed at diagnosis. Stone MJ, Bogen SA. Nevertheless, HVS in the modern therapeutic era for NDMM (median OS at 3.6 years) was associated with dismal prognosis compared to the control group from the same institution (median OS at 7.7 years, similar to other real life cohort (23)) with adjustment of cytogenetics and prognosis score. Statistical tests were performed using R software version 4.0.4 (https://www.r-project.org) and Prism version 9.2.0. This is a type of cancer that infects plasma cells. Unable to load your collection due to an error, Unable to load your delegates due to an error. Serum hyperviscosity leading to vascular disturbances plays a major role in the microvascular changes of the retina causing vascular dilation. Federal government websites often end in .gov or .mil. [Myeloma serum hyperviscosity syndrome treated with plasmapheresis]. [16]. Most of the patients received bortezomib and high-dose steroids (95%) associated with an immunomodulatory drug (43%) or alkylating agents (42%). Therapeutic Apheresis, 6(1), 45-52. Jeffrey L Arnold, MD, FACEP is a member of the following medical societies: American Academy of Emergency Medicine, American College of PhysiciansDisclosure: Nothing to disclose. Some people with multiple myeloma have bruising and unusual bleeding (haemorrhage), such as frequent nosebleeds, bleeding gums and heavy periods. Ocular findings may be the first manifestations of the disease. for: Medscape. Sloop G, Holsworth RE Jr, Weidman JJ, St Cyr JA. It may happen because you have too many red blood cells, white blood cells or blood proteins. Clin Case Rep. 2015 Apr. Clipboard, Search History, and several other advanced features are temporarily unavailable. (2002). (2007). Hyperviscosity syndrome (HVS) refers to the clinical sequelae of increased blood viscosity. Guidelines on the use of therapeutic apheresis in clinical practice evidence-based approach from the writing committee of the American society for apheresis: The eighth special issue. Timely detection of hyperviscosity syndrome and initiation of plasma exchange to remove paraproteins can significantly alter the clinical course and be potentially lifesaving. No rebound effect was observed at one month post TPE with a median protein level decrease of 66% (IQR 6073, Supplementary Figure1C). and transmitted securely. Journal of Emergency Medicine, 18(1), 23-26. Dumas G, Merceron S, Zafrani L, Canet E, Lemiale V, Kouatchet A, et al. (b) Diffuse infiltration of plasmacytoid cells over the left upper lung (H and E, 400). MeSH Bilateral central retinal vein occlusion; Hyperviscosity retinopathy; Multiple myeloma. Kundu, S., Dey, A., & Sengupta, A. J Clin Med. One patient did not receive MM treatment except TPE and steroids related to multiple septic shocks and died one month after the HVS diagnosis. ONS is committed to promoting excellence in oncology nursing and the transformation of cancer care. Armillas-Canseco F, Hernndez-Mata C, Gmez-Ruiz I, Aguayo A, Martnez-Baos D. Hyperviscosity syndrome: A 30-year experience in a tertiary referral center in Mexico city. Bethesda, MD 20894, Web Policies On the other hand, MM plasma cells produce mostly monomeric IgG or dimeric IgA M-protein, leading to fewer blood viscosity increased than pentameric IgM. Keywords: Most cohorts (including 5 patients) of MM with HVS were published in the 1970s when no efficient treatment was available for MM, which explained the higher occurrence of symptomatic HVS (56%) (7, 18). Moderate coagulation abnormalities were observed in NDMM with HVS, with slightly elevated INR (median 1.4), along with slightly decreased levels of factor II, V, VII and X. Fibrinogen levels in NDMM with HVS was normal in median (2.2 g/L), but eight patients (21%) had fibrinogen below 2 g/L. Skeletal processes Skeletal complications are. However, like all cancers, MM develops when genetic mutations cause particular cells to grow uncontrollably. Radovi M, Duri D, Trajkovi B, Radojici B, Pekic B, Tomasevi R, Duji A. Ann Intern Med. High-risk cytogenetics was described as the presence of translocations t (4, 14) or del(17p) by fluorescence in situ hybridization (FISH) according to the consensus from the period of 20112021 (15, 16). Methods: This . The PubMed wordmark and PubMed logo are registered trademarks of the U.S. Department of Health and Human Services (HHS). Oncologic emergencies. Beyond the CRAB symptoms: A study of presenting clinical manifestations of multiple myeloma. Hyperviscosity Syndrome Clinical Presentation - Medscape correlation of measured myeloma cell mass with presenting clinical features, response to treatment, and survival. One TPE can be sufficient to resolve symptoms of HVS (10). doi: 10.1371/journal.pone.0276048. Hyperviscosity syndrome (HVS) is a rare complication of newly diagnosed multiple myeloma (NDMM) related to high tumour burden. Blood (2015) 126:4741. doi:10.1182/blood.V126.23.4741.4741, 20. Elevated plasma immunoglobulin levels in multiple myeloma can lead to hyperviscosity syndrome (HVS). Symptoms of hyperviscosity usually appear when the normal serum viscosity of 1.4 to 1.8 cp reaches 4 to 5 cp, corresponding to a serum immunoglobulin M (IgM) level of at least 3 g/dL, an IgG level of 4 g/dL, and an IgA . Clinical features and prognosis of multiple myeloma and orbital extramedullary disease: Seven cases report and review of literature. Hyperviscosity syndrome is described in a patient with multiple myeloma and IgG1(K) myeloma protein. MM causes bone symptoms, such as pain, fractures, and collapsed vertebrae, which may lead to a curved spine and spinal cord compression. Patient data were obtained in conformity with the Declaration of Helsinki. Rajkumar SV, Dimopoulos MA, Palumbo A, Blade J, Merlini G, Mateos M-V, et al. Outcome and survival of myeloma patients diagnosed 20082015. That is why an individual may require multiple blood tests, imaging tests, and biopsies for doctors to diagnose MM and determine a suitable treatment plan. Barry E Brenner, MD, PhD, FACEP Program Director, Emergency Medicine, Einstein Medical Center Montgomery To outline the ophthalmic manifestations of multiple myeloma. Three thromboses occurred for three patients during the front-line treatment by bortezomib + cyclophosphamide + dexamethasone (VCD): one acute myocardial ischemia and two deep venous thrombosis. Data analysis and interpretation: P-ED, SH, BR, VS, BA, AT. Epub 2010 Dec 28. Moreover, because MM is complex, an individual may require various treatments to improve their outlook. We mitigate this effect with the multivariate analysis which confirmed the dismal prognosis of HVS. Durie BG, Salmon SE. Investigating the power of music for dementia. Related to the unfavourable prognosis of HVS, including these patients in clinical trials seems necessary. Hyperviscosity syndrome (HVS) is a combination of clinical signs and symptoms related to increased blood viscosity. Figure3 Comparison to NDMM controls. The Supplementary Material for this article can be found online at: https://www.frontiersin.org/articles/10.3389/fimmu.2022.1069360/full#supplementary-material, 1. Hemingway, T. J., & Savitsky, E. J. Hyperviscosity syndrome in plasma cell dyscrasias - PubMed Journal of Korean Medical Science, 20(4), 699-701. However, there is evidence that genetic mutations in tumor suppressor cells could play a role. Timely detection of hyperviscosity syndrome and initiation of plasma exchange to remove paraproteins can significantly alter the clinical course and be potentially lifesaving. Hyperviscosity syndrome - Wikipedia Concerning clinical form, the presentation of HVS was heterogenous (Figure2A; Table2): 23% were clinical asymptomatic, 59% had mild clinical signs (43% epistaxis, 33% blurry vision, and 23% headache) and 18% had a severe form with neurological symptoms. Optical coherence tomography images of the right (c) and left (d) macula revealed marked subretinal fluid (asterisk) and intraretinal fluids (arrows), (a) Chest X-ray showed left upper lung mass (asterisk) with soft-tissue density. Conception and design: P-ED, SH, AT. Hyperviscosity syndrome. Clin Exp Optom. This accounts for 1-2% of hematological cancers. Objective: To outline the ophthalmic manifestations of multiple myeloma. (b) Computed tomography of the chest noted a 6.5 cm left upper lung mass (asterisk) with rib destruction, (a) Histopathology with H and E, 400 showed diffuse infiltration of plasmacytoid cells in the bone marrow. Drew, M. J. HVS presentation was heterogeneous, with asymptomatic, mild, and neurological forms in 23%, 59%, and 18% of cases, respectively. A clinical staging system for multiple myeloma. Ophthalmic manifestations of multiple myeloma - PubMed
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