Multipl myelom tanılı hastalarda tedavi yanıtlarının ve prognostik özelliklerin ekstramedüller tutulum varlığı ile ilişkisinin değerlendirilmesi
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Tarih
2024
Yazarlar
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Cilt Başlığı
Yayıncı
Necmettin Erbakan Üniversitesi, Tıp Fakültesi
Erişim Hakkı
info:eu-repo/semantics/openAccess
Özet
Multipl myelom (MM), immunoglobülin üreten plazma hücrelerinin neoplastik proliferasyonu ve bu hücrelerin monoklonal immunoglobülin üretimi ile karakterize hematolojik bir malignitedir. MM tüm kanserlerin yaklaşık %1-2 ‘sini oluşturur. Hematolojik maligniteler arasında lenfomalardan sonra ikinci sırada yer alır ve hematolojik malignitelerin ortalama %10’unu oluşturur.
Kemik iliği dışında klonal plazma hücrelerinin varlığı ekstramedüller myelom (EMM) olarak tanımlanır. EMM; kemik ilişkili plazmositom (paraskletal tutulum) ve ekstramedüller hastalık olarak 2 ana alt grupta sınıflandırılmaktadır. Kemik ilişkili plazmositomlar, kortikal kemiğin hasarı sonrası aksiyel kemikte (kosta, vertebra, kafatası, sternum ve pelvis) ortaya çıkan tümoral kitlelerdir. Ekstramedüller hastalık hematojen yayılıma sekonder gelişen, kemik iliğinden uzak bir anatomik alanda (çoğunlukla karaciğer, deri, santral sinir sistemi, plevra, böbrekler, lenf nodları ve pankreas) görülen plazma hücre infiltrasyonu veya yumuşak doku tümörleri olarak tanımlanır. PET/BT, MR gibi sensitif görüntüleme yöntemlerinin kullanımının artmasıyla daha yüksek oranlarda EMM saptanmaya başlanmıştır. MM hastalarında tanı anında EMM %6-8 iken, hastalık seyrinde bu oranın %10-30’a ulaştığı gösterilmiştir. Literatür taramalarında EMM’nin diğer risk ve prognostik faktörlerden bağımsız olarak kötü prognoz ile ilişkili olduğu belirtildiği için EMM varlığı ortaya konması gereken bir konu haline gelmiştir.
Ülkemizde EMM üzerine yapılan çalışma sayısı kısıtlı olup, yaptığımız bu çalışma ile MM tanılı hastalarda EMM’li hastaların demografik, laboratuvar, klinik özelliklerinin değerlendirilmesi ve EMM varlığının prognoz üzerindeki etkilerini değerlendirerek literatüre katkı sağlamak amaçlanmıştır.
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Çalışmaya, Meram Tıp Fakültesi Hastanesi İç Hastalıkları Anabilim Dalı Hematoloji Kliniğinde 2015- 2023 yılları arasında MM tanısı konulan 111 hasta dahil edilmiştir. Çalışma retrospektif yapılmış olup yaşı 18 üstünde olan hastalar, tanı anında PET/BT görüntülemesi olan hastalar ve tanı anında laboratuvar değerlerine ulaşılabilen MM tanılı hastalar çalışmaya dahil edilmiştir.
Hastaların, hastane otomasyon sistemindeki ve klinik izlem dosyalarındaki tüm verileri geriye dönük incelenmiştir. Hastalara ait kaynaklardan tanı konulma anındaki yaş (yıl), cinsiyet, MM tipi, ekstramedüller myelom varlığı (paraskletal tutulum yeri, ekstramedüller hastalık), genetik veriler, kemik iliği ve PET/BT bulguları, ilk sıra tedavi ve tedavi yanıtı, otolog kemik iliği transplantasyonu, evreleme ve prognoz (nüks, sağkalım, son durum) kaydedilmiştir. Toplanan veriler doğrultusunda EMM varlığının hastalık üzerindeki etkileri değerlendirilmiştir.
Çalışmaya katılan hastaların laboratuvar incelemelerinden tam kan sayımı, sedimentasyon, kreatinin, kalsiyum, serum LDH düzeyi, total protein, serum albümin düzeyi, CRP, serum Ig G-A-M düzeyi, serbest kappa ve lambda hafif zincir düzeyleri ve oranı, beta-2 mikroglobülin sonuçları kayıt altına alınmıştır.
Çalışmamızda literatüre benzer olarak EMM’ye sahip hastalarda erkek cinsiyetin üstünlüğü ve yaş ortalamasının 61,76 ± 11,98 ile literatüre benzerlik gösterdiği gözlenmiştir. Literatürdeki birçok yayına kıyasla çalışmamızda EMM sıklığı daha yüksek saptanmıştır. EMM insidansının yüksek olmasını ön planda merkezimizdeki PET/BT yönteminin yaygın olarak kullanılmasına ve çalışmamıza tanı anında PET/BT’si olan hastaların dahil edilmesinden dolayı kaynaklandığını düşünmekteyiz. Çalışmamızda ve literatürde benzer olarak tanı anında EMM tutulumu olarak sıklıkla paraskletal tutulum gerçekleştiği gözlemlenmiştir. Genel olarak laboratuvar parametrelerinde istatistiki olarak anlamlı farklılıklar görülmese de literatüre benzer olarak EMM olan grupta daha yüksek LDH seviyesi, beta-2 mikroglobülin düzeyi ile daha düşük anemi, platelet ve serum albümin seviyeleri gözlemlenmiştir. Literatüre benzer olarak EMM olan hastalarda IgG alt tipi gözlenmiş olup literatürden farklı olarak lambda hafif zincir sıklığı anlamlı olarak daha fazla saptanmıştır. EMM olan hastalarda literatüre benzer olarak PET/BT incelemede daha fazla litik lezyon ve metabolik aktivasyon gözlemlenmiştir. Son olarak EMM’ye sahip hastalarda EMM olmayan hastalara göre daha yüksek evreye sahip olma, daha fazla ve daha hızlı nüks gelişme, ortalama ve progresyonsuz sağkalımda anlamlı azalma gibi prognostik özelliklerin ön planda olduğu; tanı anındaki ekstramedüller hastalık grubunda da progresyonsuz ve ortalama sağkalımda anlamlı azalma gözlemlenmiştir.
Multiple myeloma (MM) is a hematological malignancy characterized by neoplastic proliferation of immunoglobulin-producing plasma cells and the production of monoclonal immunoglobulin by these cells. MM accounts for approximately 1-2% of all cancers. It ranks second among hematological malignancies after lymphomas and accounts for an average of 10% of hematological malignancies. The presence of clonal plasma cells outside the bone marrow is defined as extramedullary myeloma (EMM). EMM; It is classified into 2 main subgroups: bone-related plasmacytoma (parascletal involvement) and extramedullary disease. Bone-related plasmacytomas are tumoral masses that occur in the axial bone (costa, vertebra, skull, sternum and pelvis) after damage to the cortical bone. Extramedullary disease is defined as plasma cell infiltration or soft tissue tumors that develop secondary to hematogenous spread and occur in an anatomical area distant from the bone marrow (mostly liver, skin, central nervous system, pleura, kidneys, lymph nodes and pancreas). With the increasing use of sensitive imaging methods such as PET/CT and MRI, higher rates of EMM have begun to be detected. While EMM is 6-8% at the time of diagnosis in MM patients, it has been shown that this rate reaches 10-30% during the course of the disease. Since literature reviews indicate that EMM is associated with poor prognosis, independent of other risk and prognostic factors, the presence of EMM has become an issue that needs to be revealed. The number of studies on EMM in our country is limited. With this study, we aimed to contribute to the literature by evaluating the demographic, laboratory and clinical characteristics of patients with EMM and the effects of the presence of EMM on prognosis in patients diagnosed with MM. 111 patients diagnosed with MM between 2015 and 2023 in the Hematology Clinic of the Internal Medicine Department of Meram Medical Faculty Hospital were included in the study. The study was conducted retrospectively. Patients over 18 years of age, patients who had PET/CT imaging at the time of diagnosis, and patients diagnosed with MM whose laboratory values were available at the time of diagnosis were included in the study. All data of the patients in the hospital automation system and clinical follow-up files were examined retrospectively. From patient sources, age (years) at diagnosis, gender, MM type, presence of extramedullary myeloma (parascletal involvement, extramedullary disease), protein electrophoresis and genetic data, bone marrow and PET/CT findings, first-line treatment and treatment response, autologous bone marrow transplantation, staging and prognosis (recurrence, survival, outcome) were recorded. In line with the collected data, the effects of the presence of EMM on the disease were evaluated. Laboratory examinations of the patients participating in the study included complete blood count, sedimentation, creatinine, calcium, serum LDH level, total protein, serum albumin level, CRP, serum Ig G-A-M level, free kappa and lambda light chain levels and ratio, beta-2 microglobulin results were recorded. In our study, similar to the literature, it was observed that the male gender was predominant in patients with EMM and the average age was similar to the literature with 61.76 ± 11.98 years. Compared to many publications in the literature, the frequency of EMM was found to be higher in our study. We think that the high incidence of EMM is primarily due to the widespread use of the PET/CT method in our center and the inclusion of patients with PET/CT at the time of diagnosis in our study. Similarly, in our study and in the literature, it was observed that parascletal involvement frequently occurs as EMM involvement at the time of diagnosis. Although there were no statistically significant differences in laboratory parameters in general, similar to the literature, higher LDH levels, beta-2 microglobulin levels and lower anemia, platelet and serum albumin levels were observed in the EMM group. Similar to the literature, IgG subtype was observed in patients with EMM, and unlike the literature, the frequency of lambda light chain was found to be significantly higher. Similar to the literature, more lytic lesions and metabolic activation were observed in PET/CT examination in patients with EMM. Finally, prognostic features such as having a higher stage, developing more and faster recurrences, and a significant decrease in mean and progression-free survival are at the forefront in patients with EMM compared to patients with normal MM diagnosis; A significant decrease in progression-free and median survival was also observed in the extramedullary disease group at diagnosis.
Multiple myeloma (MM) is a hematological malignancy characterized by neoplastic proliferation of immunoglobulin-producing plasma cells and the production of monoclonal immunoglobulin by these cells. MM accounts for approximately 1-2% of all cancers. It ranks second among hematological malignancies after lymphomas and accounts for an average of 10% of hematological malignancies. The presence of clonal plasma cells outside the bone marrow is defined as extramedullary myeloma (EMM). EMM; It is classified into 2 main subgroups: bone-related plasmacytoma (parascletal involvement) and extramedullary disease. Bone-related plasmacytomas are tumoral masses that occur in the axial bone (costa, vertebra, skull, sternum and pelvis) after damage to the cortical bone. Extramedullary disease is defined as plasma cell infiltration or soft tissue tumors that develop secondary to hematogenous spread and occur in an anatomical area distant from the bone marrow (mostly liver, skin, central nervous system, pleura, kidneys, lymph nodes and pancreas). With the increasing use of sensitive imaging methods such as PET/CT and MRI, higher rates of EMM have begun to be detected. While EMM is 6-8% at the time of diagnosis in MM patients, it has been shown that this rate reaches 10-30% during the course of the disease. Since literature reviews indicate that EMM is associated with poor prognosis, independent of other risk and prognostic factors, the presence of EMM has become an issue that needs to be revealed. The number of studies on EMM in our country is limited. With this study, we aimed to contribute to the literature by evaluating the demographic, laboratory and clinical characteristics of patients with EMM and the effects of the presence of EMM on prognosis in patients diagnosed with MM. 111 patients diagnosed with MM between 2015 and 2023 in the Hematology Clinic of the Internal Medicine Department of Meram Medical Faculty Hospital were included in the study. The study was conducted retrospectively. Patients over 18 years of age, patients who had PET/CT imaging at the time of diagnosis, and patients diagnosed with MM whose laboratory values were available at the time of diagnosis were included in the study. All data of the patients in the hospital automation system and clinical follow-up files were examined retrospectively. From patient sources, age (years) at diagnosis, gender, MM type, presence of extramedullary myeloma (parascletal involvement, extramedullary disease), protein electrophoresis and genetic data, bone marrow and PET/CT findings, first-line treatment and treatment response, autologous bone marrow transplantation, staging and prognosis (recurrence, survival, outcome) were recorded. In line with the collected data, the effects of the presence of EMM on the disease were evaluated. Laboratory examinations of the patients participating in the study included complete blood count, sedimentation, creatinine, calcium, serum LDH level, total protein, serum albumin level, CRP, serum Ig G-A-M level, free kappa and lambda light chain levels and ratio, beta-2 microglobulin results were recorded. In our study, similar to the literature, it was observed that the male gender was predominant in patients with EMM and the average age was similar to the literature with 61.76 ± 11.98 years. Compared to many publications in the literature, the frequency of EMM was found to be higher in our study. We think that the high incidence of EMM is primarily due to the widespread use of the PET/CT method in our center and the inclusion of patients with PET/CT at the time of diagnosis in our study. Similarly, in our study and in the literature, it was observed that parascletal involvement frequently occurs as EMM involvement at the time of diagnosis. Although there were no statistically significant differences in laboratory parameters in general, similar to the literature, higher LDH levels, beta-2 microglobulin levels and lower anemia, platelet and serum albumin levels were observed in the EMM group. Similar to the literature, IgG subtype was observed in patients with EMM, and unlike the literature, the frequency of lambda light chain was found to be significantly higher. Similar to the literature, more lytic lesions and metabolic activation were observed in PET/CT examination in patients with EMM. Finally, prognostic features such as having a higher stage, developing more and faster recurrences, and a significant decrease in mean and progression-free survival are at the forefront in patients with EMM compared to patients with normal MM diagnosis; A significant decrease in progression-free and median survival was also observed in the extramedullary disease group at diagnosis.
Açıklama
Anahtar Kelimeler
Multipl Myelom, Multiple Myeloma, Ekstramedüller Myelom, Ekstramedüller Hastalık, Paraskletal Tutulum, Parascletal Involvement, Extramedullary Myeloma, Extramedullary Disease
Kaynak
WoS Q Değeri
Scopus Q Değeri
Cilt
Sayı
Künye
Öz, S. N. O. (2024). Multipl myelom tanılı hastalarda tedavi yanıtlarının ve prognostik özelliklerin ekstramedüller tutulum varlığı ile ilişkisinin değerlendirilmesi. (Yayınlanmamış tıpta uzmanlık tezi) Necmettin Erbakan Üniversitesi, Tıp Fakültesi Dahili Tıp Bilimleri Bölümü İç Hastalıkları Anabilim Dalı, Konya.
