essential thrombocytosis, a blood condition that occurs when bone marrow produces too In the series reported by Kotby, thrombomodulin was significantly low in CCHD patients compared to their age-matched controls. The PV Study Group found that the most common thromboembolic events, in descending order of frequency, were CVAs, myocardial infarction, peripheral arterial occlusion, pulmonary infarcts, and venous thromboses. The clinical spectrum of these events spans from a nonspecific and vague flulike illness and rash to potentially life-threatening pneumonitis and pancreatitis.4042 High level of suspicion with prompt oncology and targeted subspecialty consultation is necessary. On the other hand, some patients with decompensated erythrocytosis, iron deficiency states, those might have recurrent symptoms of hyperviscosity.[8]. An important and dreadful feature of hyperviscosity is overt thrombosis in organ systems, resulting in cerebrovascular accident and myocardial infarction. macroglobulinemia 2022 Oct 14;12(6):20220048. doi: 10.1098/rsfs.2022.0048. Cancerous plasma cells and transmitted securely. Oxidative stress and biochemical and genetic factors can furthermore contribute to this condition. 2021 Apr 20;12(4):605. doi: 10.3390/genes12040605. If prompt plasmapheresis cannot be obtained, a temporizing measure that can be performed emergently is intravenous phlebotomy. Perloff JK, Marelli AJ, Miner PD. 2007 Feb:11(1):87-95 [PubMed PMID: 17441400], Zarkovic M, Kwaan HC. Her symptoms improved following phlebotomy. Hematology/oncology clinics of North America. Malignant epidural spinal cord compression is managed in conjunction with neurosurgery, but it is classically treated using steroids and/or surgery and radiation therapy. During history taking, it is important to note any current or past hematological disorders as well as any family history of such conditions. 1. 2011 Jul 10:152(28):1110-9. doi: 10.1556/OH.2011.29155. We present a series of four patients with uncorrected cyanotic congenital heart disease who presented with a spectrum of features of hyperviscosity and discuss pathophysiology, clinical features, and management of hyperviscosity in detail. Obstruction can be benign or malignant. Malignant pericardial effusion may be treated with pericardiocentesis or a more permanent surgical intervention. The viscosity of blood can also be increased by deformity of the shape of red blood cells (RBCs) that causes RBC aggregation and reduced blood flow. J Pediatr 1977; 90:266. https://www.ncbi.nlm.nih.gov/books/NBK526081/. Hyperviscosity syndrome - Wikipedia Although treating the cancer is primary, recent studies acknowledge that aggressive treatment of the hyponatremia itself improves patient outcomes.16,17 Fluid restriction (limit to 500 to 1,000 mL per day) is an important component of SIADH management. Rosenthal A, Nathan DG, Marty AT, Button LN, Miettinen OS, Nadas AS. After the procedure, the patient should be asked to drink lots of fluids and avoid strenuous activities for one or two days. Close this message to accept cookies or find out how to manage your cookie settings. Hematologic malignancies, particularly acute leukemia, and high-grade lymphomas; solid tumors, Azotemia, hyperphosphatemia, hyperkalemia, hyperuricemia, acute renal failure, hypocalcemia, Multiple myeloma; breast cancer; squamous cell carcinoma of the head or neck, lung, kidney, or cervix, Progressive decline in mental function, weakness, anorexia, thirst, constipation, nausea, vomiting, decreased urine output, possible coma, Syndrome of inappropriate antidiuretic hormone, Hyponatremia, nausea, vomiting, constipation, muscle weakness, Single axillary/oral temperature 101.3F (38.5C) or sustained temperature 100.4F (38C) for one hour, and an absolute neutrophil count < 500 cells per mm, Oncology, infectious disease, palliative care, Waldenstrm macroglobulinemia (10% to 30%), leukemia, multiple myeloma, Spontaneous bleeding, shortness of breath, neurologic deficits (peripheral neuropathies), sausage-like hemorrhagic retinal veins, serum viscosity > 4 cP, Lung cancer, lymphoma, metastatic mediastinal tumors or lymph nodes, indwelling venous catheters, Facial edema, cough, dyspnea at rest, hoarseness, chest and shoulder pain, collateral venous circulation (chest wall), Oncology, cardiothoracic surgery, palliative care, Malignant epidural spinal cord compression, Breast cancer, multiple myeloma, lymphoma, lung and prostate cancers, New-onset back pain (worse when lying down), paraplegia (late presentation), Lung, esophageal, and breast cancers; lymphoma; leukemia; melanoma; infection; treatment complication; autoimmune reaction, Dyspnea, chest pain, or palpitations; pulsus paradoxus; Beck triad (muffled heart sounds, hypotension, increased jugular venous pressure), Pain, erythema, and swelling that progress to blanching, blistering, discoloration, and necrosis of the skin, Abdominal pain, nausea, vomiting, diarrhea, constipation, and dehydration; obstruction; bleeding; weight loss; dehydration, Oncology, gastroenterology, general surgery, infectious disease, radiology, Current radiation therapy (external, temporary internal, permanent internal, systemic), Dermatitis, cardiovascular disease, esophagitis, cystitis, sexual dysfunction, depression, Oncology, dermatology, cardiology, gastroenterology, urology/gynecology, Vague symptoms, such as flulike illness and rash, Calcium 7.0 mg per dL (1.75 mmol per L) or 25% decrease from baseline, Phosphorus 4.5 mg per dL (1.45 mmol per L) in adults, 6.5 mg per dL (2.10 mmol per L) in children, or 25% increase from baseline, Potassium 6.0 mEq per L (6.0 mmol per L) or 25% increase from baseline, Uric acid 8.0 mg per dL (476 mol per L) or 25% increase from baseline, Creatinine 1.5 times the upper limit of normal for age, External: focused on external beam therapy, External radiation source only, no radiation remains afterward, Temporary internal: radioactive material inserted via catheter and later withdrawn, Radiation source implanted for short term; patient is usually hospitalized until radiation source is removed, Children and pregnant women are usually not permitted to visit during hospitalization, Permanent internal: radioactive seed/pellet permanently implanted at the tumor site, Radiation source implanted for long term, and amount of radiation lessens over time; possibility of exposure to others, Contact with infants, pregnant women, puppies, and kittens often not recommended for days to months, depending on type, Radiation source is injected or swallowed and can leave bodily fluids radioactive for a short time, Caution against bodily fluid contact for infants, children, and pregnant women for a few days posttreatment. 2. Plasma hyperviscosity syndromes are treated by plasmapheresis (plasma exchange) to remove the paraproteins With further clinical improvement, he was discharged home on day seven. 2014 Jun;101(6):1599-603. doi: 10.1016/j.fertnstert.2014.02.045. Hyperviscosity syndrome - Cancer Therapy Advisor He improved on conservative treatment and was discharged after 5 days on oral iron therapy over and above his other medications. 2018 Nov:23(6):185-187. doi: 10.1097/NRL.0000000000000197. Hyperviscosity Syndrome Clinical Presentation: History, Physical, New-onset back pain in patients with cancer should be evaluated as epidural spinal cord compression until it is ruled out. Centrifuge and aliquot serum into plastic vial. This article updates a previous article on this topic by Higdon and Higdon.5. [1] However, this advantageous effect is offset by the increased serum viscosity that ultimately reduces blood flow and tissue perfusion. 2018 Jun 1:97(11):741-748 [PubMed PMID: 30215936], Tedeschi A, Conticello C, Rizzi R, Benevolo G, Laurenti L, Petrucci MT, Zaja F, Varettoni M. Diagnostic framing of IgM monoclonal gammopathy: Focus on Waldenstrm macroglobulinemia. 1Department of Pediatric Cardiology, Rabindranath Tagore International Institute of Cardiac Sciences, Kolkata, West Bengal, India, 2Department of Pediatric Cardiology, Pediatric Intensive Care, Rabindranath Tagore International Institute of Cardiac Sciences, Kolkata, West Bengal, India, 3Department of Pediatric Cardiology, Narayana Superspecialty Hospital, Howrah, West Bengal, India. Some patients are stable compensated erythrocytosis, adequate iron stores. Web. please confirm that you agree to abide by our usage policies. WebViscous fluids are thicker and travel more slowly. } Viscosity is formally defined as the measurement of the internal resistance of a fluid to flow but can simply be thought of as the thickness or stickiness of a fluid. British journal of haematology. Consequently, iron-deficient erythropoiesis leads to production of microcytic RBCs which are less deformable and paradoxically increase blood viscosity and impairs tissue oxygenation, thus leads to increased risk of stroke. While theseconditions are related to Of interest, hyperviscosity syndrome in COVID-19 may cause poor tissue perfusion, peripheral vascular resistance, and thrombosis ().In particular, low-shear areas are vulnerable to thrombosis due to a decrease in the dispersion of clotting factors and a reduction in the Neonatal hyperviscosity: randomized study of effect of partial plasma exchange The severity of clinical symptoms is directly related to the increased levels of serum viscosity, with progressively more severe symptoms occurring as the individual patients serum viscosity increases. Hyperviscocity: Treatment, Symptoms, and Causes Triglyceride glucose index is a significant predictor of severe The outcomes for patients with HVS depend on the primary tumor and the extent of spread. Diagnosis of HVS is made by determining an elevated serum viscosity which can be suggested by Rouleaux formation on peripheral smear or repeated malfunction of laboratory testing equipment. concluded that phlebotomy is not warranted to reduce an assumed risk of stroke because no such risk was demonstrated in their series of CCHD patients who were followed prospectively for 112 years. [1,7], Patients with CCHD and secondary erythrocytosis can be classified into two groups. Viscosity is formally defined as the measurement of the internal resistance of a fluid to flow but can simplybe thought of as the "thickness" or "stickiness" of a fluid. government site. National Library of Medicine For secondary erythrocytosis, however, there is no cut off for upper level of Hb. HVS is an oncological emergency, and timely treatment can prevent life-threatening complications such as thromboembolic events, myocardial infarction, and catastrophic ischemia that result in multiple organ failure. Normal Reports | Description of the problem Hyperviscosity syndrome (HVS) is a combination of clinical signs and symptoms related to increased blood viscosity. American family physician. This beneficial effect is offset by increased blood viscosity which impairs microvascular circulation and tissue perfusion leading to symptoms of hyperviscosity and microvascular thrombosis. Plasmapheresis can decrease serum viscosity by 20% to 30% and can be done on a daily basis until clinical resolution of symptoms. It is often associated with chemotherapy but can also occur after radiation and biologic therapies.6 The release of intracellular products (e.g., uric acid, phosphates, calcium, potassium) overwhelms the body's homeostasis.7 With increased use of ambulatory infusion centers, family physicians may encounter tumor lysis syndrome when reviewing standard postcancer treatment laboratory results or if a patient experiences known complications. Data Sources: A PubMed/MEDLINE search was completed using the key words neoplasms, tumor lysis syndrome, hypercalcemia of malignancy, syndrome of inappropriate antidiuretic hormone, febrile neutropenia, hyperviscosity syndrome, superior vena cava syndrome, epidural spinal cord compression, malignant pericardial effusion, emergency, drug therapy, therapy, chemotherapy, radiotherapy, surgery, diet therapy, biologic therapy, and immunologic therapy. Bethesda, MD 20894, Web Policies Gertz MA: Acute hyperviscosity: syndromes and management. Thrombomodulin (TM) which is a vascular endothelial cell surface receptor acts as an important mediator for thrombin-mediated activation of Protein C which governs the anticoagulant activity of endothelium. Epub 2018 Jul 23 [PubMed PMID: 30190019], Miyamoto Y, Hamasaki Y, Matsumoto A, Doi K, Noiri E, Nangaku M. Prediction of immunoglobulin M reduction via therapeutic dose of simple plasma exchange and double filtration plasmapheresis using membrane separation in patients with hyperviscosity syndrome caused by Waldenstrom macroglobulinemia. In short, hyperviscosity means that the blood becomes thick or gooey and may have difficulty flowing easily through your blood vessels. The degree to which laboratory markers of coagulation activation are elevated are substantially lower in secondary erythrocytosis than in PV, and there is no clear clinical evidence that secondary erythrocytosis poses an elevated thrombosis risk. Fibrinogen may play a causal role in ischemic stroke through several mechanisms. 2018:2018():8629429. doi: 10.1155/2018/8629429. Marked ST segment depression in anterior, lateral and inferior leads, ECG from same patient as in Figure 1. [14], Patients with CCHD with very high HCT and symptoms of hyperviscosity should initially get volume replacement and correction for iron deficiency anemia. WebIn many cases, there is no obvious predisposing cause. [1][2][3][4], Any pathologic elevation of the cellular components (erythrocytes, leukocytes, or platelets) or acellular components (protein) of blood can cause hyperviscosity. Short-term management is directed at symptom control, whereas long-term management is directed at controlling the underlying hematologic condition. Viscosity is measured in the unit of centipoise (cp). Copyright 2023 American Academy of Family Physicians. Surgical treatment was not considered in view of poor ventricular function. Epistaxis Rectal bleeding Menorrhagia Persistent bleeding after minor procedures Visual changes range from blurred vision to vision loss. Content may require purchase if you do not have access. A complete hemogram showed elevated serum Hb (19.6 g/dl), HCT (62.4%), and total red cell count (7.84 million/l). Find out more about saving to your Kindle. (Instruction manual: BV200 Automated Clinical Viscometer User Manual. Despite the iron deficiency, she underwent limited phlebotomy with volume replacement by fresh frozen plasma. Signs of febrile neutropenia include a single axillary/oral temperature of 101.3F (38.5C) or higher or a sustained temperature of 100.4F (38C) or higher for one hour, and an absolute neutrophil count (ANC) less than 500 cells per mm3 or an expected decrease of ANC to less than 500 cells per mm3 in the next 48 hours. Feature Flags: { As a library, NLM provides access to scientific literature. Web1. Epub 2016 Jul 5 [PubMed PMID: 27378193], Jeevasankar M, Agarwal R, Chawla D, Paul VK, Deorari AK. About 25% of HVS cases secondary to myelomas are caused by IgA, followed by IgG myelomas at less than 5% of cases. Indian journal of pediatrics. Epub [PubMed PMID: 21712172]. Unraveling the Balance between Genes, Microbes, Lifestyle and the Environment to Improve Healthy Reproduction. In some animals, the gammopathy may initially be monoclonal because of the predominance of a single immunoglobulin class (usually IgG). 1993:(): [PubMed PMID: 27831681], Castillo JJ, Garcia-Sanz R, Hatjiharissi E, Kyle RA, Leleu X, McMaster M, Merlini G, Minnema MC, Morra E, Owen RG, Poulain S, Stone MJ, Tam C, Varettoni M, Dimopoulos MA, Treon SP, Kastritis E. Recommendations for the diagnosis and initial evaluation of patients with Waldenstrm Macroglobulinaemia: A Task Force from the 8th International Workshop on Waldenstrm Macroglobulinaemia. on the Manage Your Content and Devices page of your Amazon account. This material may not otherwise be downloaded, copied, printed, stored, transmitted or reproduced in any medium, whether now known or later invented, except as authorized in writing by the AAFP. "corePageComponentGetUserInfoFromSharedSession": true, The associated defects in hemostasis seen with PV have not been demonstrated in patients with secondary erythrocytosis. http://www.bimjonline.com/Imageoftheweek/Image28.05.2012/Figure%201.jpg, https://m.youtube.com/watch?feature=youtu.be&v=jTsjCZ9QxW8. Benson Viscometers; Ver. [1] A large series by Perloff et al. Although the frequency of tumor lysis syndrome is increasing in those with solid tumors, it is most common with hematologic malignancies, particularly acute leukemia and high-grade lymphomas. Samples at ambient temperature are aspirated by the sample probe, which is heated to 37 degrees C. Viscosity results are calculated by the instrument at both 25 degrees C and 37 degrees C temperature points. Author disclosure: No relevant financial affiliations. Blood hyperviscosity plays a role in the pathogenesis of several cardiovascular diseases, including hypertension, atherosclerosis,and the metabolic syndrome. [ 9] Viscosity increases logarithmically at the lowest shear rates and this effect is magnified at higher hematocrit levels. Increased viscosity, especially of the blood. Seminars in thrombosis and hemostasis. Disclaimer. Serious cardiovascular events contribute to the increased mortality rate among patients with PV. Abnormal Reports, SI Normal Reports | Careers. Neurologic Hypoxemia associated with cyanotic CHD does not result in panmyelosis but an increase in only the red cell mass. Rheumatic conditions such as seropositive rheumatoid arthritis, systemic lupus erythematosus, and Sjogren syndrome compromise polyclonal causes of HVS as well as Castleman disease and HIV infection.[5][6][7][8]. Causes Journal of ophthalmology. Other signs and symptoms may include cough, dyspnea at rest, hoarseness, chest and shoulder pain, and swelling or discoloration of the neck or upper extremities.2 Often, collateral venous circulation causes distension of the superficial veins in the chest wall.3,24 Although superior vena cava syndrome is a clinical diagnosis (Figure 125 ), contrast-enhanced computed tomography of the chest is most commonly used to confirm clinical suspicion.3,24, Management can include radiation, steroids, and chemotherapy to reduce a malignant obstruction. The association of elevated hematocrit and fibrinogen levels with increased stroke risk has led to continued interest in hemorheologic factors and their role in the development of vascular disease and acute stroke. 2018 Oct 11:132(15):1627. doi: 10.1182/blood-2018-07-863787. Underlying disorders to consider in patients with hyperviscosity syndrome include the following: Multiple myeloma. Definitions.net. [9], Phlebotomy causes a fall in red cell mass and serum viscosity. Therapy should be based on the severity of signs and symptoms rather than the calculated degree ofviscosity. WebMedical Encyclopedia Polycythemia - newborn Polycythemia - newborn Polycythemia can occur when there are too many red blood cells (RBCs) in an infant's blood. Elevated blood viscosity is the result of either red blood cell shape deformity or a pathological increase in serum proteins, red blood cells (RBC), white blood cells (WBC), or platelets. [9], Perloff et al. Dysproteinemias Causing Vascular Purpura 2017 Jun 6;162:125-134. doi: 10.1016/j.jprot.2016.08.018. The neurologist. Palliative care is increasingly appropriate as a part of the team approach for treating patients with cancer. Seminal hyperviscosity is not associated with semenogelin degradation or sperm deoxyribonucleic acid damage: a prospective study of infertile couples. For patients with compensated secondary erythrocytosis, phlebotomy is not indicated. The mainstays of treatment include supportive therapy, plasma exchange or plasmapheresis, and chemotherapy. On admission, she was found to have polycythemia (HCT 72%) and red cell indices suggestive of iron deficiency state. Hyperviscosity syndrome (HVS) is a pathological condition in which blood is thicker than normal and as a result flow is decreased. Collection Container/Tube: Red top (serum gel/SST are not acceptable). Inpatient management is indicated to address the precipitating malignancy and includes hematology and oncology consultation.3, Superior vena cava syndrome is caused by gradual compression of the superior vena cava where it enters the right atrium, leading to edema and retrograde flow.24 Lung cancer is the most common malignant cause, although it can also be caused by lymphoma, metastatic mediastinal tumors or lymph nodes, and indwelling catheters. A 28-year-old male, known case of ventricular septal defect (VSD) and aortopulmonary window with Eisenmenger syndrome, presented with sudden onset precordial pain radiating to the left shoulder and arm, associated with shortness of breath and profuse sweating. A hematology/oncology consultant should administer this, and it is strongly recommended to consult with this expert as soon as HVS is identified. Keep specimen at 37 degrees C (eg, 37 degrees C Thermopak, heat block) until after centrifugation. Salimipour H, Mehdizadeh S, Nemati R, Pourbehi MR, Pourbehi GR, Assadi M. Unusual neurologic manifestations of a patient with cyanotic congenital heart disease after phlebotomy. Summarize the treatment options for hyperviscosity syndrome. When fluid has low viscosity, it travels quickly and without much difficulty. This test is not useful for patients with small concentrations of monoclonal proteins. HVS exists as a clinical triad of neurological symptoms, mucosal bleeding, and visual disturbances. Cryoglobulinemia can also lead to small-vessel vasculitis, which can cause purpura; hyperviscosity and direct vascular trauma can contribute. Plasma hyperviscosity is a rare complication of both monoclonal and polyclonal disorders associated with elevation of immunoglobulins. Black VD, Lubchenco LO, Koops BL, et al. Failure to follow specimen handling instructions may cause false-low results. "corePageComponentUseShareaholicInsteadOfAddThis": true, Rare causes include immobilization, medications, and parathyroid carcinoma.12,13, Symptoms of hypercalcemia include progressive decline in mental function, weakness, anorexia, thirst, constipation, nausea, vomiting, decreased urine output, and coma. The .gov means its official. It can be caused either by a deformity of the shape of red blood cells or by any pathological elevation of cellular or acellular components of blood, particularly, immunoglobulins [1,3]. 1993 Jan-Feb;30(1):63-8. doi: 10.3109/01485019308988370. [1,4], Once dehydration and iron deficiency are ruled out as precipitating causes of hyperviscosity symptoms, phlebotomy may be safely performed with concomitant volume replacement. National Library of Medicine On admission, the right lower limb was markedly cold and edematous with bluish discoloration, and the right pedal pulse was not palpable. Febrile neutropenia is one of the most common complications related to cancer treatment, particularly chemotherapy. Although viscosities greater than 1.5 centipoises (cP) are abnormal, hyperviscosity is rarely present unless the viscosity is greater than 3 cP. Yes, mental confusion can be a sign of MM. ST changes improve after conservative treatment. Then enter the name part Polycythaemia and the hyperviscosity syndrome in newborn infants. 19952023 Mayo Foundation for Medical Education and Research. On admission, serum troponin I was elevated at 37312 ng/L (reference limit <19 ng/L). More permanent surgical solutions for management of malignant pericardial effusions, such as pericardial windows and indwelling pericardial catheters, are associated with improved patient outcomes compared with percutaneous pericardiocentesis alone. Epub [PubMed PMID: 30309878], Espinosa-Barberi G, Galvn Gonzlez FJ, Miranda Fernndez S, Viera Pelez D, Medina Rivero F, Marrero Saavedra D. Vasoproliferative retinopathy secondary to Waldenstrm's disease. In the setting of severe acute renal insufficiency, early hemodialysis can be key, necessitating nephrology referral.6, Hypercalcemia occurs in 10% to 30% of patients with cancer and is characterized by a serum calcium level of more than 10.5 mg per dL (2.63 mmol per L).11 It is most often associated with multiple myeloma and breast cancer, but is also common in squamous cell carcinomas of the head or neck, lung, kidney, or cervix.12 The causes of hypercalcemia fall into three general categories: humoral, bone invasion, and rare causes.11 Humoral causes, such as production of parathyroid hormonerelated protein and increased vitamin D3, are most common (80% of cases). The National Cancer Institute estimates that 14.5 million persons in the United States have cancer, and that number could reach 19 million by 2024.1 Family physicians should be familiar with the most prevalent oncologic emergencies because stabilization is often necessary, in addition to referrals for managing the underlying malignancy and initiating palliative measures.2 Some oncologic emergencies are insidious and take months to develop, whereas others manifest over hours, causing devastating outcomes such as paralysis and death.3 In many patients, cancer is not diagnosed until a related condition emerges. A patient-focused approach that includes education; cancer-specific monitoring; and team-based care, including palliative care, with continuous communication is recommended.4 Most oncologic emergencies can be categorized as metabolic, hematologic, structural, or treatment related (Table 15 ).
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