Renal Önemi Olan Monoklonal Gammopatiler
Özet
Renal Önemi Olan Monoklonal Gammopatiler (MGRS), malignite kriterlerini karşılamayan plazma veya B-hücre klonlarının ürettiği monoklonal proteinlerin böbrek hasarına yol açtığı, prognozu olumsuz etkileyen heterojen bir hastalık grubudur. MGRS, direkt ya da indirekt mekanizmalarla glomerüler ve tübüler hasara neden olarak proteinüri, hematüri ve ilerleyici böbrek yetmezliği gibi klinik tablolarla kendini gösterir. Hastalığın tanısı, serum/idrar elektroforezi ve immünfiksasyon yöntemlerinin yanı sıra, lezyonların yapısal özelliklerini (organize veya organize olmayan birikimler) ortaya koyan böbrek biyopsisi ile konur. Tedavi yaklaşımında temel amaç, böbrek fonksiyonlarını korumak amacıyla patolojik klona yönelik kemoterapi ajanlarının (proteazom inhibitörleri, monoklonal antikorlar vb.) kullanılmasıdır. Son dönem böbrek yetmezliği gelişen ve böbrek nakli planlanan hastalarda, nüks oranının yüksek olması sebebiyle nakil öncesinde tam hematolojik remisyonun sağlanması kritik öneme sahiptir. Sonuç olarak, MGRS yönetiminde hematolog ve nefrologların multidisipliner işbirliği ve farkındalığın artırılması başarı şansını doğrudan etkilemektedir.
Monoclonal Gammopathy of Renal Significance (MGRS) represents a heterogeneous group of disorders where monoclonal proteins, produced by small B-cell or plasma cell clones that do not meet malignancy criteria, cause significant kidney damage. MGRS leads to glomerular and tubular lesions through direct or indirect mechanisms, presenting clinically with proteinuria, hematuria, and progressive renal insufficiency. Diagnosis relies on serum/urine electrophoresis, immunofixation, and renal biopsy, which identifies structural traits of the deposits (organized or non-organized). The primary therapeutic goal is preserving renal function by targeting the underlying pathologic clone with chemotherapy agents such as proteasome inhibitors and monoclonal antibodies. In patients with end-stage renal disease who are candidates for kidney transplantation, achieving complete hematological remission prior to the transplant is vital due to the high recurrence rate. Consequently, early recognition and a multidisciplinary collaboration between hematologists and nephrologists are crucial for improving patient outcomes in MGRS management.
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