Multiple Myeloma (Part 1) - Update on Epidemiology, Diagnostic Criteria, Systemic Treatment and Prognosis / Mieloma múltiplo (Parte 1) - Atualização sobre epidemiología, critérios diagnósticos, tratamento sistêmico e prognóstico

Abstract Multiple myeloma (MM) is a hematological malignancy characterized by unregulated and clonal proliferation of plasma cells in the bone marrow; these cells produce and secrete an anomalous monoclonal immunoglobulin, or a fragment of this, called M protein. The clinical manifestations of MM result from the proliferation of these plasmocytes, the excessive production of monoclonal immunoglobulin and the suppression of normal humoral immunity, leading to hypercalcemia, bone destruction, renal failure, suppression of hematopoiesis and humoral immunity, increasing the risk for the development of infections. The increase in life expectancy of the world population led to a concomitant increase in the prevalence of MM, a pathology that usually affects the elderly population. The aim of this review is to update the reader on epidemiology, diagnostic criteria, differential diagnosis with other monoclonal gam-mopathies, systemic treatment and prognosis of MM.

Resumo O mieloma múltiplo (MM) constitui neoplasia maligna de origem hematológica caracterizada pela proliferação desregulada e clonal de plasmócitos na medula óssea; estas células produzem e secretam imunoglobulina monoclonal anômala, ou um fragmento desta, denominado proteína M. As manifestações clínicas do MM decorrem da proliferação destes plasmócitos, da produção excessiva de imunoglobulina monoclonal e da supressão da imunidade humoral normal, levando à hipercalcemia, destruição óssea, insuficiência renal, supressão da hematopoiese e da imunidade humoral,aumentandooriscoparaodesenvolvimento de infecções. O aumento na expectativa de vida da população mundial levou a concomitante incremento na prevalência do MM, patologia que habitualmente acomete a população idosa. O objetivo desta revisão é atualizar o leitor sobre a epidemiologia, critérios diagnósticos, diagnóstico diferencial com outras gamopatias monoclonais, tratamento sistêmico e prognóstico do MM.

Co-occurrence of myeloid neoplasm and plasma cell neoplasm

ABSTRACT Co-occurrence of myelodysplastic syndrome (MDS) and plasma cell neoplasm in patients with no history of chemo and/or radiotherapy is rarely reported. Herein, we report a case of a female in her seventieth decade of life who was referred to the hospital for pancytopenia. The patient was asymptomatic and was doing well overall. Serum protein electrophoresis was remarkable for a lambda-restricted monoclonal protein (IgG) estimated at 1.8g/dL. Immunoglobulin G serum level was also elevated, and serum Kappa/Lambda free light chain ratio was decreased. At that time, a bone marrow biopsy showed myelodysplastic syndrome with excess blasts-2 (MDS-EB2) and a monoclonal plasma cell proliferation. Some studies have shown that patients with plasma cell neoplasm could be associated with an increased risk of developing MDS compared to the general population. Based on reviewing the literature, to our knowledge, the pathological mechanism of the co-occurrence of both diseases is not yet clear.

Progress in smoldering multiple myeloma / 白血病·淋巴瘤

Smoldering multiple myeloma (SMM) is an asymptomatic clonal plasma cell disorder.There have been many advances in the diagnosis,prognosis and management of multiple myeloma at the 21th European Hematology Association Annual Meeting,especially SMM being discussed as a topic.These include a revised disease definition,new prognostic factors and new treatment options.

Monoclonal gammopathy of undetermined significance and smoldering multiple myeloma: biological insights and early treatment strategies / 白血病·淋巴瘤

Researches on monoclonal gammopathy of undetermined significance (MGUS) and smoldering muhiple myeloma (SMM) are at a low level although their incidence is high.This review discusses curent biological insights in MGUS/SMM and discusses how the integration of novel biological markers,molecular imaging,and clinical monitoring of MGUS/SMM could facilitate the development of early treatment strategies for high-risk SMM (early myeloma) patients in the future.

Genetic evolution and heterogeneity of multiple myeloma: a report from the 2013 EHA annual meeting / 白血病·淋巴瘤

Recent studies have shown that multiple myeloma (MM) is preceded by a premalignant state called monoclonal gammopathy of undetermined significance (MGUS) and smoldering multiple myeloma (SMM).Some of the genetic changes of MM are also present in MGUS and SMM,including chromosomal translocations,copy number changes,somatic mutations and so on.This article discusses the genetic heterogeneity.

Evolution, differential diagnosis and intervention strategies of monoclonal gammopathy of undetermined significances:reports in the 54 ASH annual meeting / 白血病·淋巴瘤

Monoclonal gammopathy of undetermined significances (MGUS) is a premalignant clonal disorder occurring in 4.2 ％ of adults ＞50 years old,and is associated with a 1％ risk every year of progression to multiple myeloma (MM),light-chain amyloidosis and related conditions.Differential diagnosis of asymptomatic and symptomatic monoclonal gammopathies is the determinant for starting therapy.Most patients with MGUS need frequent assessment and follow-up.A few patients with MGUS might progress into smoldering MM and symptomatic MM,or develop into light chain diseases such as AL amyloidosis and light chain deposition disease caused by M-protein,who should be followed with effective therapy.The above were in detail reviewed in the 54th American Society of Hematology (ASH) annual meetina.