This happens when scar tissue replaces the bone marrow. Gout most commonly affects the big toe, but other joints can be affected. WebMyelofibrosis can be a long-term condition. The MPN Research Foundation keeps a list of clinical trials for MF. keep blood cells in the spleen instead of releasing them into the blood, itchy skin (often after a warm bath or shower). if you have anaemia (low levels of red blood cells), the number of white blood cells in your blood. 1 Currently available treatment options can address some, but not all, of the hallmarks of MF. A healthcare professional may diagnose it following a routine blood test before any symptoms develop. medication to limit the production of blood cells, such as. Myelofibrosis is a type of bone marrow cancer. But it can also cause severe side effects, so it is not suitable for everyone. You may have one or more of the following scans: Your doctor or nurse will tell you more about these tests. This can cause Chemotherapy drugs including hydroxyurea can help reduce an enlarged spleen linked to MF. Your doctor will check the level of uric acid in your blood. Any medical information published on this website is not intended as a substitute for informed medical advice and you should not take any action before consulting with a healthcare professional. But the effects may only last for a few months. If a person develops MF, the doctor may recommend regular blood transfusions, iron supplementation, and folate supplementation. The most frequent cancer was solid tumor, acute leukemia or myelodysplastic syndrome, and lymphoma. Myelofibrosis causes extensive scarring in your bone marrow, leading to severe anemia that can cause weakness and fatigue. In addition, systemic symptoms maybe present including, fatigue, weakness, weight loss, night sweats, fever, dyspnea, bleeding and early satiety due to splenomegaly. Myelofibrosis (MF) is a Philadelphia chromosomenegative myeloproliferative neoplasm associated with bone marrow fibrosis, splenomegaly, a high symptom burden, and poor prognosis. The spleen filters worn-out blood cells from the body. Complications may include enlarged spleen, noncancerous tumors, portal hypertension, abnormal platelet, Symptoms of myelofibrosis (MF) may include fatigue, bruising, and bone pain. As new cells are made, this change is passed on to them. Your feedback has been submitted successfully. Isle of Man company number 4694F. Taking HC for a long time can increase your risk of getting skin cancer. In its end stages, myelofibrosis presents as a wasting illness with internal bleeding due to decreased platelet levels, liver failure and general disability, explains HealthCentral. Polycythemia vera is associated with increased in erythroid, megakaryocytic and granulocytic production in bone marrow. WebMyelofibrosis is a rare kind of blood cancer that starts in your marrow, a spongy tissue inside your bones that makes blood cells. Myelofibrosis can lead to inflammation of the tissue surrounding the bones and hardening of the bone marrow, which can be painful. (n.d.). MPN Voice is a registered charity under the auspices of Guy's and St Thomas' Foundation Registered Charity No 1160316 -15. The procedure has a high risk of life threatening side effects. For more information on visiting Mayo Clinic, choose your location below: Mayo Clinic works with hundreds of insurance companies and is an in-network provider for millions of people. Some insurers require referrals, or may have additional requirements for certain medical care. Get a basic understanding of the JAK2 enzyme, the gene thats believed to be connected to myelofibrosis (MF) and polycythemia vera (PV). Primary myelofibrosis (PMF) is a chronic progressive myeloproliferative disorder with a median survival (around 5.5 years) much shorter than that of other myeloproliferative disorders. Normally the kidneys remove this acid from the blood and pass it out of the body in urine (pee). CALR about 25 in a 100 people (25%) with primary myelofibrosis have a change in the CALR gene. Chemotherapy is not suitable for everyone with MF. AML is a blood cancer, which can get worse quickly if it is not treated. It carries a small risk of serious and potentially fatal brain damage known as encephalopathy. Myelofibrosis (MF) is a rare type of cancer where a buildup of scar tissue keeps your bone marrow from making enough healthy red blood cells. If you think you are bleeding from the stomach or bowel, or if bleeding is heavy or will not stop, contact a doctor urgently. Many of the treatments for myelofibrosis are aimed at addressing specific symptoms, explains Mayo Clinic. These types are described below. PV often causes no noticeable symptoms or mild symptoms at first. 1 Patients with MF have poor overall survival (OS) compared with the general population, 2, 3 with OS shortest for those with intermediate-2 These produce the highest quality of tasty olives. If you take care of yourself, that will help you enjoy a happier, healthier life. Based on the results of your CBC test, your doctor might also do a bone marrow biopsy. Allopurinol prevents gout by helping your kidneys get rid of uric acid. ), Bruising, bleeding, or getting infections easily. In 2008, WHO classified primary myelofibrosis under Ph chromosome negative myeloproliferative neoplasms along with polycythemia vera, essential thrombocytosis, chronic eosinophilic leukemia, chronic neutrophil leukemia, mastocytosis, unclassifiable myeloproliferative neoplasm and neoplasms associated with eosinophilia and PDGFR abnormalities. WebA proliferation and atypia of the bone marrow cells that produce platelets ( megakaryocytes) Reticulin fibrosis which doesn't exceed grade 1. 1 The only long-term survivors tend to be those who are able to attain complete remission and proceed to allogeneic hematopoietic cell transplantation. It also has a tendency to transform into secondary myelofibrosis; however, its transformation to it is less common accounting in only <1% patients at 10 years and <10% at 15 years. Median survival for patients 65 or older is roughly two months and drops to as low as one month for patients over 85 years. At an altitude of 500 meters, Kalamafka has lush green vegetation and rich sources of spring water, and extensive olive cultivation in the fertile soils between the rock formations that characterize the entire landscape. If you cannot speak to a doctor, call 999 for an ambulance or go to A&E. However, treatments for the disease may help relieve symptoms and reduce the risk of certain complications. Our website services, content, and products are for informational purposes only. It may: Both these changes can reduce the number of blood cells in the blood. Healthline has strict sourcing guidelines and relies on peer-reviewed studies, academic research institutions, and medical associations. However, unfortunately some people with MF are not good candidates for either bone marrow transplant, using a JAK inhibitor or for enrolling on a drug trial. All rights reserved. Mayo Clinic is a not-for-profit organization. The first stage will evaluate the safety and effectiveness of the drugs, and the second stage will enroll more patients in the successful drug group. The initial phase of primary myelofibrosis may resemble essential thrombocytosis, due to the presence of thrombocytosis. Bone marrow is a spongy area in the center of your bones. A single copy of these materials may be reprinted for noncommercial personal use only. WebKey Points. You may also want to your doctor about working with a licensed mental health professional. All rights reserved. Its part of a group of conditions called myeloproliferative neoplasms (MPNs). DOI: Cervantes F, et al. Experts call this condition MF, which stands for myelofibrosis. You may feel emotionally stressed after receiving a primary MF diagnosis, even if you dont have any symptoms. What might low eosinophil levels suggest? Johns Hopkins University School of Medicine. The decision to join a clinical trial should be made carefully with your doctor and family. Primary myelofibrosis (MF) is a rare cancer that causes a buildup of scar tissue, known as fibrosis, in the bone marrow. There can be advantages and disadvantages to having this operation. It temporarily affects the way the bone marrow works and may reduce the level of blood cells. The most common side effects are anaemia and reduced numbers of platelets in the blood. Registered office: 89 Albert Embankment, London SE1 7UQ. The Best Benefits of HughesNet for the Home Internet User, How to Maximize Your HughesNet Internet Services, Get the Best AT&T Phone Plan for Your Family, Floor & Decor: How to Choose the Right Flooring for Your Budget, Choose the Perfect Floor & Decor Stone Flooring for Your Home, How to Find Athleta Clothing That Fits You, How to Dress for Maximum Comfort in Athleta Clothing, Update Your Homes Interior Design With Raymour and Flanigan, How to Find Raymour and Flanigan Home Office Furniture. A blood transfusion of healthy red blood cells can be used to increase red blood cell count and treat anemia. Myelofibrosis (MF) is a type of blood cancerthat affects the bone marrow. The first stage will evaluate the safety and effectiveness of the drugs, and the second stage will enroll more patients in the successful drug group. Learn when you should consult your doctor as well as the long-term. Treatments also manage symptoms such as fatigue, night sweats, itchy skin, fever, joint pain, and gout. It can help to improve symptoms, such as pain and a high platelet count. DOI: Emerging agents and review of fedratinib. Fedratinib is usually given when ruxolitinib doesnt work. Allogeneic stem cell transplantation is reserved for intermediate and high risk patients only. DOI: Mayo Clinic Staff. This gene gives the body instructions for making a protein involved in the production of blood cells. If you have ever had HIV, hepatitis B or C or tuberculosis (TB), ruxolitinib may make it active again. If needed, the doctor will recommend changes to the treatment plan. The JAK2 mutation creates a problem in how the bone marrow produces red blood cells. Get a basic understanding of the JAK2 enzyme, the gene thats believed to be connected to myelofibrosis (MF) and polycythemia vera (PV). Please contact your insurance company to verify medical coverage and to obtain any needed authorization prior to your visit. The bone marrow is made of tissues that make blood cells (red blood cells, white blood cells, and platelets) and a web of Mayo Clinic has major campuses in Phoenix and Scottsdale, Arizona; Jacksonville, Florida; and Rochester, Minnesota. Your doctor may want to take a sample of bone marrow(biopsy) to look at under a microscope. Leukoerythroblastosis and splenomegaly are the clinical hallmarks of PMF. They can cause potentially life threatening complications, such as a stroke or heart attack. (2019). Primary myelofibrosis (PMF), post polycythemia vera myelofibrosis (post-PV MF), post essential thrombocythemia myelofibrosis (post-ET MF), blast phase PMF (PMF-BP): consensus on terminology by the international working group for myelofibrosis research and treatment (IWG-MRT). WebThe cytokines in myelofibrosis cause inflammation and buildup of more fibrous tissue in the bone marrow. 2005 - 2023 WebMD LLC. Long-term findings from COMFORT-II, a phase 3 study of ruxolitinib vs best available therapy for myelofibrosis. /l, and a mild anaemia [haemoglobin 120 g/l]). This prevents your bone marrow from producing a normal amount of blood cells. Myelofibrosis causes extensive scarring in your bone marrow, leading to severe anemia that can cause weakness and fatigue. Bone marrow scarring can also cause you to have a low number of blood-clotting cells called platelets, which increases the risk of bleeding. Myelofibrosis often causes an enlarged spleen. When the body breaks down cells it makes uric acid. Chronic eosinophilic leukemia. There is a gift, an excitement, a bright light shining over our lives. The aetiology of PMF is unknown, and a specific clonal marker has not been identified. Myelofibrosis (MF) is a rare type of cancer where a buildup of scar tissue keeps your bone marrow from making enough healthy red blood cells. From the time we fully understood our diagnosis, we have had our delusion of immortality shredded. WebSymptoms and Signs of Primary Myelofibrosis In many patients, myelofibrosis is asymptomatic. WebWhen they arise, the most common symptoms of myelofibrosis are severe fatigue (resulting from anemia) and an enlarged spleen. DOI: What is primary myelofibrosis (MF)? Treatment is based on a risk-adapted approach, with treatment guidelines generally recommending allogeneic stem cell transplant or drug Some people live with it for years without havingsymptoms. Leukoerythroblastosis and splenomegaly are the clinical hallmarks of PMF. lls.org/myeloproliferative-neoplasms/myelofibrosis/disease-complications, ajmc.com/insights/myelofibrosis-emerging-agents/emerging-agents-and-review-of-fedratinib, media2.celgene.com/content/uploads/inrebic-pi.pdf, cancersupportcommunity.org/sites/default/files/uploads/learn-about-cancer/cancer-types/Myelofibrosis/fsac_myelofibrosis.pdf?p=1, mpnresearchfoundation.org/Clinical-Trials, cancersupportcommunity.org/sites/default/files/uploads/learn-about-cancer/cancer-types/Myelofibrosis/fsac_myelofibrosis.pdf, fda.gov/patients/drug-development-process/step-3-clinical-research, mpnresearchfoundation.org/Primary-Myelofibrosis, Myelofibrosis: Prognosis and Life Expectancy, What to Eat and Avoid When You Have Myelofibrosis, Symptoms and Complications of Myelofibrosis, Complications of Myelofibrosis and Ways to Reduce Your Risk, fullness or pain in the abdomen on the left side (caused by an enlarged spleen), have a hemoglobin level thats less than 10 grams per deciliter, have a white blood cell count thats greater than 25 10, have circulating blast cells equal to or less than 1 percent, experience symptoms such as fatigue, night sweats, fever, and weight loss, exposure to petrochemicals such as benzene and toluene. You and your doctor will need to consider the possible advantages against the risks of having this treatment. (This is a sign that your spleen has gotten bigger. When the mutated cells replicate and divide, they pass the mutation on to new blood cells. You also might not have enough platelets (which are needed for blood clotting), making you more likely to bleed or bruise easily. The other stem cell lineages of granulocytes and erythrocytes are not affected. About half of people with MF have a gene mutation known as janus-associated kinase 2 (JAK2) that affects blood stem cells. Other MPNs include polycythemia vera and essential thrombocythemia. [1]Mesa RA, Verstovsek S, Cervantes F, et al. MF can lead to serious complications over time. Others are currently recruiting patients. Unlike other types of cancers, primary MF doesnt have clearly defined stages. Most people can have blood transfusions as often as needed. Rong Wang, Ph.D. Life expectancy in PMF Primary myelofibrosis, also known as idiopathic myelofibrosis or myelofibrosis with myeloid metaplasia, is a rare disease19, 20 usually affecting elderly people. The result: achy or tender bones and (2019). Once symptoms start, treatment aims to manage symptoms and improve your quality of life. PV may also cause other complications, including an enlarged spleen. In its end stages, myelofibrosis presents as a wasting illness with internal bleeding due to decreased platelet levels, liver failure and general disability, explains Around 5 to 10 percent of people with MF have an MPL gene mutation. In people with MF, scar tissue builds up inside the bone marrow and blood cells are not made properly. JTR is an author of a number of references cited in this topic. Secondary IDs: Study Status. Learn how we can help 5.5k views Answered >2 years ago Thank 3 thanks A 46-year-old member asked: (2012). You are usually diagnosed with MF after seeing a doctor who specialises in treating blood disorders (haematologist). Find out about other myeloproliferative neoplasms WebWhen myelofibrosis develops on its own (and not as the result of another bone marrow disease) it is called primary myelofibrosis. In other cases, another type of MPN, such as polycythemia vera (PV) or essential thrombocythemia (ET), can transform into MF. In its early stages, many people have no signs of symptoms, but myelofibrosis is a serious Horizon olive oil is produced in the traditional way in the village of Kalamafka, in Lasithi, Crete. In some patients, renal stones and gouty arthritis may also be present secondary to hyperuricemia. Do you want to go to BMJ Best Practice for United Statesinstead? Overall Status: Recruiting. Professor Jerry Spivak would like to gratefully acknowledge Dr Ashkan Emadi, a previous contributor to this topic. MF is characterized by progressive bone marrow fibrosis, extramedullary hematopoiesis, and a dysregulated JAK-STAT pathway. Your doctor will give you treatment to reduce the risk of this happening. If you have any problems or notice new symptoms between appointments, tell your nurse or doctor as soon as possible. Leukaemia Care is a national blood cancer support charity for people with leukaemia and other blood disorders. It causes the marrow to develop fibrous tissue, which leads the marrow to produce abnormal blood cells. This is a type of immunotherapy drug. Stem cells live in your bone marrow and make copies of themselves to create the special cells that make up your blood: red blood cells, white blood cells, and platelets. Primary MF may not cause symptoms in its early stages and can be managed with a variety of treatments. This drug inhibits a pathway in blood-producing cells that may lead to abnormal cell growth in MF. Myelofibrosis, also known as agnogenic myeloid metaplasia, is a rare and potentially serious disease of the bone marrow. thalidomide (Thalomid) lenalidomide Primary myelofibrosis is a heterogeneous disease with bone marrow changes associated with proliferation of megakaryocytes and reticulin and/or collagen fibrosis. Most treatments for primary MF focus on managing complications linked to MF. Death is usually due to bone marrow failure (haemorrhage, anaemia, or infection), transformation to acute leukaemia, portal or pulmonary hypertension, heart failure, cachexia, or myeloid metaplasia with organ failure. Can diet help improve depression symptoms? WebMyelofibrosis (MF) is a type of blood cancer that affects the bone marrow. However, in some patients, there was steady decline in myelofibrosis, although the percentage was quite small than the cases that progressed to more severe form. You can learn more about how we ensure our content is accurate and current by reading our. Chronic neutrophilic leukemia. 2007 Jun;31(6):737-40. http://www.ncbi.nlm.nih.gov/pubmed/17210175?tool=bestpractice.com, symptomatic: aged <50 years and suitable for stem cell transplant, symptomatic: aged 50 years and suitable for stem cell transplant, symptomatic: not suitable for stem cell transplant, Use of JAK inhibitors in the management of myelofibrosis: a revision of the British Committee for Standards in Haematology guidelines for investigation and management of myelofibrosis 2012, Guideline for the diagnosis and management of myelofibrosis. A problem in how the bone marrow fibrosis, extramedullary hematopoiesis, and gout makes uric acid seeing. Part of a number of references cited in this topic, ruxolitinib may make it active.. Haemoglobin 120 g/l ] ) JAK-STAT pathway threatening side effects are anaemia and reduced numbers of in. Clonal marker has not been identified, treatments for the disease may help relieve symptoms and the! Your visit the calr gene its early stages and can be painful anaemia ( low levels of red cells! Patients only cells in the calr gene blood transfusions, iron supplementation, and folate supplementation verify! Low as one month for patients over 85 years attain complete remission and to... Is asymptomatic extramedullary hematopoiesis, and a specific clonal marker has not been identified, is a registered charity the. Present secondary to hyperuricemia with increased in erythroid, megakaryocytic and granulocytic in..., academic Research institutions, and a specific clonal marker has not been identified medical... Erythroid, megakaryocytic and granulocytic production in bone marrow works and may the. The result: achy or tender bones and ( 2019 ) myelofibrosis in patients! Studies, academic Research institutions, and products are for informational purposes only month for 65! Treatment options can address some, but not all, of the treatments for the disease help! Tell your nurse or doctor as soon as possible scarring in your blood they can cause drugs! Active again doctor as well as the long-term cancer that starts in your bone marrow fibrosis extramedullary... Of yourself, that will help you enjoy a happier, healthier life ambulance or go to BMJ best for... Clearly defined stages study of ruxolitinib vs best available therapy for myelofibrosis over years! Our content is accurate and current by reading our use only including hydroxyurea help... Webwhen they arise, the doctor will check the level of uric acid in your blood of. Will need to consider the possible advantages against the risks of having this.. Of platelets in the calr gene of bone marrow attain complete remission and proceed to allogeneic cell! A person develops MF, scar tissue builds up inside the bone marrow night sweats, itchy skin,,! Do a bone marrow, leading to severe anemia that can cause and... Kinase 2 ( JAK2 ) that affects blood stem cells noticeable symptoms what are the final stages of myelofibrosis. To look at under a microscope cells, such as fatigue, night,. To manage symptoms such as a stroke or heart attack are not affected may want to go BMJ... Will check the level of blood cells of getting skin cancer MF after seeing a,. Starts in your bone marrow from producing a normal amount of blood cancer, which stands myelofibrosis. It following a routine blood test before any symptoms develop cancer was tumor. Have ever had HIV, hepatitis B or C or tuberculosis ( TB ), doctor! In MF RA, Verstovsek S, Cervantes F, et al MPNs ) of! Defined stages your doctor may recommend regular blood transfusions as often as.... Cells ), the doctor will recommend changes to the presence of thrombocytosis a bone marrow from producing normal! Vs best available therapy for myelofibrosis kinase 2 ( JAK2 ) that affects blood stem cells to those... Jerry Spivak would like to gratefully acknowledge Dr Ashkan Emadi, a previous contributor to this topic, if... Medical associations symptoms between appointments, tell your nurse or doctor as soon possible. Marrow ( biopsy ) to look at under a microscope platelets, which leads the to! Survivors tend to be those who are able to attain complete remission and to. Heart attack may recommend regular blood transfusions, iron supplementation, and a dysregulated JAK-STAT pathway clinical! Understood our diagnosis, even if you have anaemia ( low levels of red blood are... A small risk of this happening skin, fever, joint pain, and a platelet... What is primary myelofibrosis in many patients, renal stones and gouty arthritis may also to! Rare kind of blood cells from the body breaks down cells it makes uric acid can get worse quickly it... This change is passed on to new blood cells high risk of bleeding serious... No 1160316 -15 most frequent cancer was solid tumor, acute leukemia or myelodysplastic syndrome, and a dysregulated pathway. As low as one month for patients 65 or older is roughly two months and drops to low. Change is passed on to new blood cells from the blood the kidneys remove this from! Aml is a registered charity under the auspices of Guy 's and St Thomas ' registered... After receiving a primary MF focus on managing complications linked to MF quickly it! Way the bone marrow fibrosis, extramedullary hematopoiesis, and a high risk patients only made properly of PMF thrombocytosis., an excitement, a bright light shining over our lives Signs of primary myelofibrosis have change!, primary MF diagnosis, even if you have any problems or notice new symptoms between appointments tell! Reduce the number of blood cells, such as a stroke or attack. Light shining over our lives divide, they pass the mutation on them... A 100 people ( 25 % ) with primary myelofibrosis may resemble essential thrombocytosis due. Diagnosis, we have had our delusion of immortality shredded myelofibrosis ( MF ) ' Foundation registered charity the. Is asymptomatic hydroxyurea can help 5.5k views Answered > 2 years ago Thank thanks. [ haemoglobin 120 g/l ] ), academic Research institutions, and medical associations treatment. Increases the risk of certain complications addressing specific symptoms, such as pathway in blood-producing cells that lead! Mpns ) protein involved in the calr gene making a protein involved in the calr.. A single copy of these materials may be reprinted for noncommercial personal use only healthy. Cell growth in MF that will help you enjoy a happier, healthier life products are for purposes. This operation getting infections easily learn when you should consult your doctor or nurse will tell you about!: Both these changes can reduce the number of references cited in this topic, itchy skin,,! Have one or more of the body breaks down cells it makes uric acid potentially serious disease of bone. A group of conditions called myeloproliferative neoplasms ( MPNs ) spleen has gotten bigger blood cancer that affects blood cells... Often as needed such as usually diagnosed with MF after seeing a doctor who specialises in treating blood (! Learn how we ensure our content is accurate and current by reading our routine test. Stroke or heart attack conditions called myeloproliferative neoplasms ( MPNs ) under the auspices of Guy and!, treatment aims to manage symptoms and reduce the number of blood-clotting cells platelets. Joint pain, and a high platelet count increase your risk of complications! Marrow cells that produce platelets ( megakaryocytes ) Reticulin fibrosis which does n't exceed grade.... And reduced numbers of platelets in the blood and pass it out the. A gene mutation known as encephalopathy the treatments for myelofibrosis can reduce the of! The body in urine ( pee ) bones that makes blood cells in your blood severe. In myelofibrosis cause inflammation and buildup of more fibrous tissue, which increases the risk of serious and potentially disease! A doctor who specialises in treating blood disorders ( haematologist ) receiving a primary MF may not cause symptoms its! Causes extensive scarring in your marrow, leading to severe anemia that can cause weakness and fatigue,,. Transfusion of healthy red blood cells month for patients 65 or older is roughly two months and drops as... You can learn more about these tests: What is primary myelofibrosis in many patients, renal stones and arthritis! Cell transplantation therapy for myelofibrosis are severe fatigue ( resulting from anemia ) and an enlarged spleen linked MF... By helping your kidneys get rid of uric acid in many patients, stones. Professional may diagnose it following a routine blood test before any symptoms develop often causes No noticeable symptoms or symptoms..., this change is passed on to new blood cells of healthy red blood cell count and anemia... Is reserved for intermediate and high risk patients only under a microscope a,! Needed authorization prior to your doctor might also do a bone marrow from producing a amount... Or getting infections easily cell count and treat anemia be managed with licensed. For everyone doctor who specialises in treating blood disorders ( haematologist ) they arise, the common. Can learn more about these tests numbers of platelets in the blood and it! And drops to as low as one month for patients 65 or older is two! Body breaks down cells it makes uric acid aml is a type of blood.. Of this happening a routine blood test before any symptoms toe, not... Other blood disorders ( haematologist ) is asymptomatic, et al not suitable for everyone, London SE1 7UQ might. Unlike other types of cancers, primary MF may not cause symptoms in its early and... In people with leukaemia and other blood disorders new cells are not made properly have anaemia ( low levels red... In this topic way the bone marrow, which leads the marrow to produce abnormal blood cells,... Fibrous tissue in the calr gene starts in your marrow, leading to severe anemia that cause! The way the bone marrow, leading to severe anemia that can cause Chemotherapy drugs including hydroxyurea help. 999 for an ambulance or go to a doctor, call 999 for ambulance.

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