http___mijelom.hr_sto-je-mm_opis-bolesti__2.xml
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Multiple Myeloma What is Multiple Myeloma? Multiple myeloma is a cancer of a type of white blood cell called plasma cells. Together with other white blood cells, plasma cells form part of our immune system which plays an important role in fighting infections. Plasma cells produce antibodies (immunoglobulins) which help body to combat infection and disease. When myeloma develops, there is uncontrolled proliferation of plasma cells resulting in a variety of symptoms. What Symptoms Do Myeloma Patients Experience ? In early stages, there may be no symptoms and discovery may be incidental eg abnormalities in blood tests are detected in course of screening investigations. Symptoms vary depending on the parts of body which are affected and how advanced disease is. These include - Bone pain - Repeated infections - Symptoms of anemia such as tiredness, weakness, feeling breathless more easily - Frequent infections - Increase bleeding and bruising - Vomiting, nausea - Severe back pain - Weakness or numbness in the legs These symptoms are not specific for myeloma and can be seen in other medical conditions. How do we test for Myeloma ? The aims of tests are to - Diagnose myeloma - Determine stage/extent of disease multiple myeloma Common tests used to evaluate multiple myeloma include blood test to: - Assess for levels of abnormal antibody proteins called M proteins from blood and also from urine samples - Assess kidney function - Assess for high calcium levels - Blood tests to assess other proteins like ‘albumin’ and ‘beta-2-microglobulin’, which are useful components in staging of the disease. - Bone marrow test - Imaging tests - X-rays of multiple regions of the body to look for any fracture or bone lesions caused by myeloma - Magnetic resonance imaging (MRI) to assess the spine and look for soft tissue tumors How is Myeloma Treated ? Not all patients require treatment immediately. The need for treatment depends on the extent/stage of disease and also presence of symptoms. In some patients the condition is “smouldering” ie myeloma is in a very early phase and patients are asymptomatic without evidence of complications associated with myeloma such as bone disease, kidney disease. Under such circumstances, therapy may not be immediately required but close followup for progression is required. Specific Myeloma Therapy The treatment chosen depends on various factors such as aggressiveness of myeloma, stage of disease and general health of patient. Each patient is different and treatment should be individualized. In some patients, the disease may be confined to a single lesion in bone or soft tissue with no evidence of disease elsewhere ie plasmacytoma. The treatment of choice in this case would be local radiotherapy with close followup to monitor for progression to myeloma. Treatment for myeloma may involve: - Chemotherapy to kill cancer cells - Corticosteroids which can slow the growth of myeloma - Immune modulating medications which target the growth of abnormal plasma cells in myeloma - Radiotherapy In addition, bone strengthening drugs (bisphosphonates) may also be given to reduce the chances of fractures Summary Multiple myeloma is a cancer involving plasma cells in the bone marrow. Therapy is complex with multiple drug options available for treatment as well as for supportive care. While myeloma is generally not cured with current available therapy, advances in understanding and knowledge of the disease as well as drug developments over past 30 years have resulted in improved survival.
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Multipli mijelom, poznat i kao mijelom, vrsta je karcinoma koštane srži koji se javlja u plazma stanicama, koje se normalno nalaze u koštanoj srži. Plazma stanice čine dio imunološkog sustava. Normalne plazma stanice proizvode anititjela (imunoglobuline) u borbi protiv infekcije. Kod multiplog mijeloma, nenormalne plazma stanice proizvode samo jednu vrstu antitijela, paraprotein ili M protein, koji nema korisnu funkciju. Multipli mijelom često se dijagnosticira i prati mjerenjem razine paraproteina. Koštana srž je „spužvasta“ tvar koja se nalazi u središtu većih kostiju u tijelu. Osim što sadržava plazma stanice, koštana srž središte je proizvodnje krvnih stanica (eritrocita, leukocita i trombocita). Kod multiplog mijeloma, oštećena je DNK plazma stanica, zbog čega stanica postane maligna ili kancerogena. Ove nenormalne plazma stanice zovu se mijelomske stanice. Za razliku od mnogih karcinoma, multipli mijelom ne postoji kao izraslina ili tumor, nego se mijelomske stanice dijele i šire unutar koštane srži. Multipli mijelom zahvaća više mjesta u tijelu gdje je koštana srž kod odraslih normalno aktivna, tj. unutrašnjost kostiju kralježnice, lubanje, zdjelice, rebara i područja oko ramena i kukova. Krajnji dijelovi udova kao što su šake, stopala i donji dijelovi ruku i nogu obično nisu zahvaćeni mijelomom i funkcija tih kritičnih područja obično je sasvim očuvana. Većina medicinskih problema vezanih uz multipli mijelom uzrokovana je nakupljanjem mijelomskih stanica u koštanoj srži i prisutnošću praraproteina u krvi ili mokraći. Česti problemi su bolovi u kostima, prijelomi, umor (uzrokovan anemijom), česte infekcije ili infekcije koje se ponavljaju (kao što su infekcije dišnog i mokraćnog sustava), oštećenje bubrega i hiperkalcemija (povišena razina kalcija u krvi). Kod nekih ljudi multipli mijelom razvije se nakon što im je dijagnosticirana monoklonska gamapatija neodređenog značenja (MGNZ), koju karakterizira povišena razina nenormalnog proteina (paraproteina) kao i kod multiplog mijeloma, ali bez drugih karakteristika multiplog mijeloma (manje od 10% plazma stanica u koštanoj srži i bez znakova oštećenja kostiju specifičnih za mijelom). Razina M proteina kod MGNZ-a je ispod 30g/L. Rizik od prijelaza MGNZ-a u aktivni multipli mijelom jako je nizak (samo 1% šanse u svakoj godini praćenja). U slučaju da je razina mijelomskih stanica u ukupnoj koštanoj srži viša od 10 – 30% i razina M proteina možda viša od 30g/L, stopa rasta može biti jako spora i bez pojave simptoma. To se naziva tinjajućim mijelomom. MGNZ, kao i tinjajući mijelom, mogu se jako sporo mijenjati tijekom godina i ne zahtijevaju aktivno liječenje. Vrlo je važno uspostaviti točnu dijagnozu i razlikovati MGNZ i tinjajući mijelom od aktivnog ili simptomatskog multiplog mijeloma koji zahtijeva liječenje. Kod vrlo malog broja oboljelih (1 – 4%) multipli mijelom može se razviti u plazmastaničnu leukemiju, koju karakteriziraju visoke razine plazma stanica u cirkulaciji u krvi2 i kraće preživljenje.