Symptoms of hypercalcaemia can include: extreme thirst feeling sick stomach pain needing to urinate frequently Only 20% of individuals with MGUS progress to MM. FOIA Eventually, the kidneys may stop working properly. Eric Alexander Savitsky, MD is a member of the following medical societies: Society for Academic Emergency MedicineDisclosure: Nothing to disclose. Bethesda, MD 20894, Web Policies This is known as hyperviscosity and can cause problems such as: Kidney damage can occur in people with multiple myeloma. National Comprehensive Cancer Network. All patients with multiple myeloma should thus undergo thorough ophthalmic examination at the time of initial diagnosis and during follow-up. Red stars denote severe cases with neurological symptoms. [Myeloma serum hyperviscosity syndrome treated with plasmapheresis]. doi:10.3816/CLML.2010.n.080, 12. Overall survival (OS) was calculated from the MM diagnosis until death or the last follow-up (whichever occurred first). MM pathophysiology has a widespread impact on the body. No major ischemic or bleeding was observed at diagnosis. Plasmapheresis is usually well tolerated and safe. government site. It may happen because you have too many red blood cells, white blood cells or blood proteins. Hyperviscosity syndrome. Semin Thromb Hemost. . Scientists do not know the exact cause of MM. Can diet help improve depression symptoms? Front. JCO (1993) 11:15538. The Oncology Nursing Society (ONS) is a professional association that represents 100,000 nurses and is the professional home to more than 35,000 members. Plasma hyperviscosity is a rare complication of both monoclonal and polyclonal disorders associated with elevation of immunoglobulins. We did not find any NDMM with IgG3 subclass associated with HVS based on eight IgG myeloma tested (29% of the IgG MM). HVS presentation was heterogeneous, with asymptomatic, mild, and neurological forms in 23%, 59%, and 18% of cases, respectively. To avoid vital complications (thrombosis, gut, or cerebral bleeding), early management with therapeutic plasma exchange (TPE) for HVS is advised as well as management in the intensive care unit (ICU) for severe forms (neurological signs, bleeding, or thrombosis related to HVS) (9, 10). Ophthalmic manifestations include proptosis, diplopia, lid ecchymosis, xanthomatosis, conjunctival and corneal crystalline and non-crystalline deposits, scleritis, episcleritis, secondary glaucoma, ciliary body cysts, ciliochoroidal effusion, uveal plasmacytoma, hyperviscosity retinopathy, retinal vasculitis, detachment of sensory retina and retinal pigment epithelium, and neuro-ophthalmic manifestations. Barry E Brenner, MD, PhD, FACEP is a member of the following medical societies: Alpha Omega Alpha, American Academy of Emergency Medicine, American College of Chest Physicians, American College of Emergency Physicians, American College of Physicians, American Heart Association, American Thoracic Society, New York Academy of Medicine, New York Academy of Sciences, Society for Academic Emergency MedicineDisclosure: Nothing to disclose. The site is secure. Clipboard, Search History, and several other advanced features are temporarily unavailable. (2007). Right orbital edema masquerading a hematologic malignancy. M-protein levels correlated with INR (cor -0.57, p<0.001) and fibrinogen level (cor -0.35, p<0.001). NDMM patients with HVS had higher M-protein level at diagnosis (Figure3A), higher 2-microglobulin level, lower albumin level, higher plasma cell infiltration in bone marrow, higher ISS3 rate, higher INR and lower fibrinogen compared to NDMM controls (Figures3B, C). Hyperviscosity syndrome (HVS) is a rare complication of newly diagnosed multiple myeloma (NDMM) related to high tumour burden. 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. Hyperviscosity syndrome in plasma cell dyscrasias. Disclaimer. Palumbo A, Avet-Loiseau H, Oliva S, Lokhorst HM, Goldschmidt H, Rosinol L, et al. Clin Lymphoma Myeloma Leuk (2010) 10:4648. MNT is the registered trade mark of Healthline Media. doi:10.1002/jca.21529, 10. 2018 May;18(2):135-149. doi: 10.1007/s10238-017-0479-9. Methods: This . 1986 Summer-Fall;6(3):169-75. doi: 10.1097/00006982-198600630-00007. It can affect the bones, blood, kidneys, and brain. Therapeutic plasma exchanges were used in 92% of patients, which were effective and well tolerated. Riccardi A, Gobbi PG, Ucci G, Bertoloni D, Luoni R, Rutigliano L, et al. Bortezomib, a newly approved proteasome inhibitor for the treatment of multiple myeloma: Nursing implications. Fundus photography of the (a) right eye and (b) left eye showed dilation, (a) Chest X-ray showed left upper lung mass (asterisk) with soft-tissue density. Outcome and survival of myeloma patients diagnosed 20082015. International myeloma working group consensus criteria for response and minimal residual disease assessment in multiple myeloma. We investigated a retrospective cohort study of NDMM with HVS between 2011-2021. symptoms? (2003). 2005 Jan-Feb;219(1):43-8. doi: 10.1159/000081782. Preston FE, Cooke KB, Foster ME, Winfiel DA, Lee D. Myelomatosis and the hyperviscosity syndrome. Censoring distributions as a measure of follow-up in survival analysis. (2003). Limited data is available on the diagnostic details and outcomes of MM patients 50 years old, and particularly how these trends have changed over the past two decades. Some people with multiple myeloma have bruising and unusual bleeding (haemorrhage), such as frequent nosebleeds, bleeding gums and heavy periods. Multimodal therapy (TPE + steroid + anti-plasma cell treatment) efficacy was confirmed for all patients from both clinical (resolution of symptoms in hours after TPE) and biological perspectives (Supplementary Figure1B). This site needs JavaScript to work properly. 8600 Rockville Pike Behl D, Hendrickson AW, Moynihan TJ. [Full Text]. Early deaths (in the first two months) occurred in 21% of older patients (>65 years). Korn EL. 866-257-4ONS (866-257-4667). Hyperviscosity in plasma cell dyscrasias. Thomas EL, Olk RJ, Markman M, et al. 2011;364:104660. The pathogenesis of MM is complex with high heterogeneity, and the development of the disease is a multistep process. [QxMD MEDLINE Link]. Multiple myeloma with plasmacytoma of the clivus bone presenting with multiple cranial nerve III, IV, and VI palsy: A diagnostic dilemma. Caimi G, Carlisi M, Urso C, Lo Presti R, Hopps E. Clinical disorders responsible for plasma hyperviscosity and skin complications. After 1980, only a Mexican series of five patients was presented (19). Ophthalmic manifestations of multiple myeloma. The https:// ensures that you are connecting to the Multiple myeloma (MM) is a hematologic cancer characterized by the accumulation of clonal and malignant plasma cells in the bone marrow that produce monoclonal abnormal immunoglobulins ("paraprotein") [].Cases with CRVO-mimicking retinopathy in MM are usually associated with hyperviscosity state that is a collection of clinical findings that may reflect increased serum . (DF) Overall survival based on presence of plasma cells in blood by cytology analysis (D), score R-ISS (low=R-ISS1 and R-ISS2; high=R-ISS3) (E) and cytogenetic risk (high risk= presence of t(4;14) or del(17p) by FISH; standard risk=others) (F). Investigating the power of music for dementia. The definitive treatment varies according to the diagnosis but often involves chemotherapeutic agents, such as alkylating agents or nucleoside analogs, which should be addressed with the consulting hematologist/oncologist to prevent further deterioration and possible recurrent episodes. 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. 119(10):2205-8. With the current treatment revolution in MM, there are no recent data about the impact of HVS on MM prognosis. Perez Rogers A, Estes M. Hyperviscosity Syndrome. The .gov means its official. [12], Because bleeding is the most common sign of HVS, urgent plasmapheresis using a cell separator should be carried out for patients experiencing visual symptoms, to reduce the likelihood of blindness from retinal hemorrhages/retinal detachment. Kumar S, Paiva B, Anderson KC, Durie B, Landgren O, Moreau P, et al. Rev Med Interne. HHS Vulnerability Disclosure, Help The treatment of HVS was based mainly on TPE for 36 patients (92%), but three patients only received steroids. Further inquiries can be directed to the corresponding author. In a context of HVS suspicion or for asymptomatic patients with a high protein concentration, the diagnosis can be confirmed by ophthalmologic examination. doi:10.1016/S0140-6736(19)31240-1, 13. Avet-Loiseau H, Attal M, Moreau P, Charbonnel C, Garban F, Hulin C, et al. 13 (2):238-40. NDMM patients with HVS need to be included in clinical trials and biological bank to confirm our results. Group TIMW. J Clin Oncol (2015) 33:28639. Before 2014, HVS was a criterion for initiating treatment (3). Cancerous plasma cells push out the healthy cells that work to protect you from infection. -, Ziaei M, Elgohary MA, Bremner FD. People with multiple myeloma are particularly vulnerable to infection because the condition interferes with the immune system, the body's natural defence against infection and illness. In a multivariate analysis of pooled HVS and controls, HVS (HR 2.7; 95%CI 1.45.4, p=0.004) and high-risk cytogenetics (HR 2.8; 95%CI 1.45.7, p=0.005) were the only two independent adverse factors for OS (Figure3E). Am J Hematol (2018) 93:138493. 67:102-106. 1973 Feb 17;1(7799):359-60. Lancet Oncology, 4(11), 679-685. Palumbo A, Anderson K. Multiple myeloma. Cerebral infarction in IgG multiple myeloma with hyperviscosity. This accounts for 1-2% of hematological cancers. 2003 Oct;29(5):467-71. doi: 10.1055/s-2003-44554. Hyperviscosity Syndrome in Patients With Multiple Myeloma | ONS Home Oncology Nursing Forum Number 3 / May 2008 Clinical Challenges Article Hyperviscosity Syndrome in Patients With Multiple Myeloma Ellen C. Mullen Noel Mendez adverse event management ONF 2008, 35 (3), 350-352. e20028 Background: Multiple myeloma (MM) is a disease predominantly of older adults with an average age of 69 at diagnosis. PMC Figure3 Comparison to NDMM controls. J Clin Apher (2019) 34:171354. Clinical practice guidelines in oncology: Multiple myeloma v.3.0. Rajkumar SV, Kyle RA HVS in NDMM patients is a rare but life-threatening complication associated with high lethality in older patients and be a potential dismal prognosis factor in the modern treatment era. (2000). Finally, controls were not matched for cytogenetic and prognosis scores. Only one patient had increased protein level after one month of therapy related to a refractory status at the front-line treatment without HVS recurrence. Front Oncol. Timely detection of hyperviscosity syndrome and initiation of plasma exchange to remove paraproteins can significantly alter the clinical course and be potentially lifesaving. Serum hyperviscosity leading to vascular disturbances plays a major role in the microvascular changes of the retina causing vascular dilation. Sloop G, Holsworth RE Jr, Weidman JJ, St Cyr JA. An official website of the United States government. The condition belongs to a spectrum of disorders affecting the growth of long-lived plasma cells. [QxMD MEDLINE Link]. These cells can overwhelm the kidneys in people with MM, causing blockages, inflammation, and cell damage. Among the 46 cases of MM with suspected HVS referred at Saint Louis Hospital, Paris, France, between 20112021, 39 (85%) patients with HVS confirmation were included (Figure1). 2015 Feb. 9 (1):19-25. 2009 Aug. 27(3):459-76. Arch Ophthalmol. No thrombosis or major bleeding was observed. This is because the cancer cells in your bone marrow can stop blood-clotting cells called platelets from being made. Unlike Waldenstrm macroglobulinemia, hyperviscosity syndrome is a rare occurrence in multiple myeloma. Lancet (2019) 394:2938. The site is secure. The patient underwent chemotherapy and despite initial laboratory improvement, 19 digits required amputation. Ultimately, the underlying dysproteinemia or blood cell dyscrasia needs to be addressed, as plasmapheresis and similar therapies do not alter the underlying disease process. Before developing active MM, individuals pass through two earlier asymptomatic disease stages called monoclonal gammopathy of undetermined significance (MGUS) and smoldering multiple myeloma (SMM). THE SIGNS and symptoms of the hyper- viscosity syndrome include bleeding from mucous membranes, retinopathy with dis- tended veins and hemorrhages, neurologic dis- orders and congestive heart failure. Eighteen patients (47%) died during the follow-up, including three early deaths (one septic shock, one pneumonia and one liver failure) two months after the diagnosis in older patients (>65 years), thirteen deaths with active MM and two deaths related to transplant-related toxicity (one post-auto-hematopoietic stem cell transplantation [HSCT] and one post-allo-HSCT) (Supplementary Table2). Plasma cells are white blood cells, and MM can affect their production and growth. The median age for MM patients with HVS at diagnosis was 59 years (IQR 5168, Table1). All patients except one had at least one CRAB criterion. doi:10.1111/j.1365-2141.1978.tb01077.x, 19. Hyperviscosity syndrome is described in a patient with multiple myeloma and IgG1(K) myeloma protein. However, there is evidence that genetic mutations in tumor suppressor cells could play a role. PT (prothrombin time) with no dilution, factor II, V, VII, X (dilution of 1/10) were measured using Neoplastin CI+ (Stago) on a STAR Analyser. Multiple myeloma (MM) is a plasma cellderived neoplasm characterized primarily by the abnormal production of monoclonal immunoglobulin (M-protein) found in serum or urine and resulting in various complications (1). These are calcium elevation, renal insufficiency, anemia, Hypercalcemia is a common complication of the blood cancer multiple myeloma. Unauthorized use of these marks is strictly prohibited. Multiple myeloma (MM) is a hematologic malignancy resulting from the uncontrolled proliferation of neoplastic plasma cells and the excessive production of monoclonal immunoglobulins, both of which may lead to hyperviscosity retinopathy. Unable to load your collection due to an error, Unable to load your delegates due to an error. Clinical findings improved with vigorous plasmapheresis; reduction in the myeloma protein concentration and the blood viscosity paralleled this improvement. Abeykoon JP, Zanwar S, Ansell SM, Winters J, Gertz MA, King RL, et al. MeSH terms Antineoplastic Agents / therapeutic use Blood Proteins / metabolism Blood Viscosity* . Boudin L, Romeo E, Mavrovi E, Tsitsi Nding P, Blade JS, de Jaureguiberry JP, et al. Hyperviscosity as a complication in a variety of disorders. A high level of calcium in the blood (hypercalcaemia) can develop in people with multiple myeloma because too much calcium is released from affected bones into the bloodstream. It's an emergency and needs quick investigation and treatment. 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 ). In similar fashion leukapheresis, plateletpheresis, and phlebotomy also decrease the serum viscosity by decreasing the existing cellular component in excess. Before doi:10.1016/S1470-2045(21)00466-6, 14. Time to next treatment was calculated as the time from the start of front-line therapy to an event (start of a new line of treatment, death, or last follow-up; whichever occurred first). Inclusion criteria were patients aged 18 years with NDMM and HVS confirmation based on abnormalities related to HVS at ophthalmologic examination (performed for clinic symptoms of HVS or at the discretion of the physician for patients who had high M-protein) or severe clinical forms (neurological symptoms, bleeding, or thrombosis related to HVS). Eur J Cancer Clin Oncol (1991) 27:14015. [QxMD MEDLINE Link]. [16]. In the case of MM, these cells are long-lived plasma cells, which can stay in the bone marrow for long periods. Retrieved September 6, 2007, from. Optometry. What are MGUS, smoldering multiple myeloma, and active myeloma? eCollection 2022. Furthermore, the use of antibodies against CD38 has completely modified the standard of care in the front-line of MM and need to be tested in HVS NDMM (only two patients in our cohort received it) (12, 13). Bethesda, MD 20894, Web Policies Multiple myeloma definitions (diagnosis, treatment criteria, response, relapse) were based on the recommendations of the International Myeloma Working Group (IMWG) (1, 14). eCollection 2019 Jan-Dec. Ferreira Santos da Cruz N, Milhomens Filho JAP, Ferraro DMN, Polizelli MU, de Moraes Ambrogini NSB. Introduction. Kundu, S., Dey, A., & Sengupta, A. Copyright 2023 Oncology Nursing Society. Most of the patients received triplet association with a backbone of bortezomib and high dose steroids (95%) associated with an immunomodulatory drug like thalidomide (21%), lenalidomide (34%), or alkylating agents like cyclophosphamide (37%) or melphalan (5%) (Supplementary Table1). Authors Somsri Wiwanitkit 1 , Viroj Wiwanitkit 2 Affiliations 1 Private Academic Consultant, Damipur, India. This can lead to conditions such as: Around 50% of people with MM have kidney problems when their MM produces symptoms. On one hand, the principal cause of HVS is pentameric IgM in the context of Waldenstrm macroglobulinemia (WM). Emergency medical services personnel should be attentive to airway, breathing, and circulation (the ABCs) and provide symptomatic support. 21. doi:10.1016/S1470-2045(14)70442-5, PubMed Abstract | CrossRef Full Text | Google Scholar, 2. Please confirm that you would like to log out of Medscape. Thirty-nine NDMM patients with HVS were included. The session was well tolerated for 58 of 59 TPE sessions. 8600 Rockville Pike Weaver A, Rubinstein S, Cornell RF. for: Medscape. 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. Most of these variables are direct or indirect measures of the tumour burden, the basis of the historic prognosis classification of Durie & Salmon (2). 2022 Mar 21;58(3):456. doi: 10.3390/medicina58030456. Zarkovic, M., & Kwaan, H. C. (2003). The remaining authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. Time to next treatment (B) and overall survival (C) of NDMM with HVS. Written informed consent for participation was not required for this study in accordance with the national legislation and the institutional requirements. Multiple myeloma (MM) is the second-ranking malignancy in hematological tumors. The site is secure. MM can interfere with an important, lifelong process called bone remodeling. sharing sensitive information, make sure youre on a federal [Bing-Neel syndrome: Report of 4 cases and literature review.]. Plasmapheresis can also reverse HVS-induced retinal changes promptly, including reducing retinal venous diameter and increased venous blood viscosity. An official website of the United States government. Eur J Intern Med. Conclusion: Although the association between systemic sclerosis and multiple myeloma is rare, it should be remembered in cases of . The estimate of the incidence of HVS was calculated from the EMMY registry, listing the lines of treatment initiated for MM every year over a period of three months since 2017 in our center. Most patients with MM will present with anemia, bone pain, and elevated creatinine levels.1 Although very rare in patients with MM, symptoms of hyperviscosity can occur, including mucosal bleeding, loss of vision, and neurologic abnormalities.2 Therapeutic apheresis has been the standard of care for treating hyperviscosity . Nevertheless, only two patients (8%) were included in a clinical trial. Around 60% of people who have MM develop a bone fracture at some point. Bethesda, MD 20894, Web Policies 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.
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hyperviscosity in multiple myeloma
