Haematological diseases with monoclonal gammopathy – treatment

Monoclonal gammopathy (MG) is a wide spectrum of the lymphoplasma cell proliferative disorders manifested by the production of abnormal monoclonal protein, ranging in severity from the premalignant monoclonal gammopathy of undetermined significance (MGUS) and smoldering multiple myeloma (SMM) to the malignant: symptomatic multiple myeloma (MM), plasma cell leukemia (PCL) and Waldenström’s macroglobulinemia (WM). Treatment of MG depends on the character of the disease. MGUS and SMM require only observation, however regular follow‐ups are needed because of the risk of progression to symptomatic MM. Multiple myeloma treatment is based on systemic therapy (with novel agents, preferably triplets) and autologous hematopoietic stem cell transplantation in transplant‐eligible patients (operatively defined as being ≤65–70 years). PCL, as the most aggressive variant of the MG, requires immediate systemic therapy with proteasome inhibitors‐based regimens combined with classical chemotherapeutics including alkylating agents or anthracyclines, followed by transplantation (also allogeneic, in selected cases). In asymptomatic WM watchful waiting is an appropriate approach, while in symptomatic disease systemic therapy with rituximab in monotherapy or combined with bendamustine, an alkylating agent, a proteasome inhibitor, or ibrutinib is needed. In case of ophthalmological manifestation of MG standard management accompanied with local treatment (eg. keratoprosthesis) is reported as effective.