Monoklonální gamapatie - úvodní slovo k supplementu

prof. MUDr. Zdeněk Adam, CSc.

Onkologie 2022: 16(Suppl.B): 4-6  

Plasma cell neoplasms, multiple myeloma, solitary plasmocytoma a plasma cell leukemia. Clinical symptoms, diagnostic criteria and therapy

prof. MUDr. Zdeněk Adam, CSc., prof. MUDr. Luděk Pour, Ph.D., prof. MUDr. Marta Krejčí, Ph.D., MUDr. Ing. David Zeman, Ph.D., doc. MUDr. Eva Vlčková, Ph.D., doc. MUDr. Milan Krtička, Ph.D., prof. MUDr. Pavel Šlampa, CSc., MUDr. Soňa Štěpánková, Ph.D., MUDr. Gabriela Romanová, MUDr. Zuzana Adamová, Ph.D., doc. MUDr. Jiří Vaníček, Ph.D., doc. MUDr. Zdeněk Řehák, Ph.D., MUDr. Renata Koukalová, Ph.D., doc. MUDr. Veronika Matušková, Ph.D., MUDr. Zdeněk Král, CSc., MUDr. Libor Červinek, Ph.D., MUDr. Viera Sandecká, Ph.D.

Onkologie 2022: 16(Suppl.B): 7-32 | DOI: 10.36290/xon.2022.054  

According the WHO classification of malignant hematologic diseases the group plasma cell neoplasms consists of multiple myeloma, solitary plasmocytoma and plasmocytic leukemia. Diagnosis of multiple myeloma requires ≥10% clonal bone marrow plasma cells or a biopsy proven plasmacytoma plus evidence of one or more multiple myeloma defining events (MDE) namely CRAB (hypercalcemia, renal failure, anemia, or lytic bone lesions) features felt related to the plasma cell disorder, bone marrow clonal plasmacytosis ≥60%, serum involved/uninvolved free light chain (FLC) ratio ≥100 (provided involved FLC is ≥100 mg/L), or >1 focal lesion...

Monoclonal gammopathy of undetermined signifikace (MGUS and monoclonal gammopathy of clinical significance (MGCS)

prof. MUDr. Zdeněk Adam, CSc., MUDr. Ing. David Zeman, Ph.D., prof. MUDr. Luděk Pour, Ph.D., prof. MUDr. Marta Krejčí, Ph.D., MUDr. Soňa Štěpánková, Ph.D., prof. MUDr. Vladimír Vašků, Ph.D., doc. MUDr. Eva Vlčková, Ph.D., doc. MUDr. Zdeněk Řehák, Ph.D., MUDr. Renata Koukalová, Ph.D., MUDr. Gabriela Romanová, MUDr. Zuzana Adamová, Ph.D., MUDr. Zdeněk Král, CSc., MUDr. Viera Sandecká, Ph.D.

Onkologie 2022: 16(Suppl.B): 33-56 | DOI: 10.36290/xon.2022.055  

Monoclonal gammopathy of undetermined significance (MGUS) is characterized by the presence of a monoclonal protein (M-protein) without evidence of multiple myeloma (MM), Waldenstrom‘s macroglobulinemia (WM), amyloidosis (AL), or a related plasma cell proliferative disorder. MGUS is found in approximately 3 % of persons > 70 years of age and in about 1 % of those > 50 years old. At Mayo Clinic from 1960 through 1994, the risk of progression was 1 % per year. This risk of progression continued even after  of a stable M-protein. The risk for developing MM, WM, or AL was increased 25-fold, 46-fold, and 8.4-fold,...

Monoclonal gammopathy of undetermined signifikace (MGUS and monoclonal gammopathy of clinical significance (MGCS)

prof. MUDr. Zdeněk Adam, CSc., MUDr. Ing. David Zeman, Ph.D., prof. MUDr. Luděk Pour, Ph.D., prof. MUDr. Marta Krejčí, Ph.D., MUDr. Soňa Štěpánková, Ph.D., prof. MUDr. Vladimír Vašků, Ph.D., doc. MUDr. Eva Vlčková, Ph.D., doc. MUDr. Zdeněk Řehák, Ph.D., MUDr. Renata Koukalová, Ph.D., MUDr. Gabriela Romanová, MUDr. Zuzana Adamová, Ph.D., MUDr. Zdeněk Král, CSc., MUDr. Viera Sandecká, Ph.D.

Onkologie 2022: 16(Suppl.B): 33-56  

Monoclonal gammopathy of undetermined significance (MGUS) is characterized by the presence of a monoclonal protein (M-protein) without evidence of multiple myeloma (MM), Waldenstrom‘s macroglobulinemia (WM), amyloidosis (AL), or a related plasma cell proliferative disorder. MGUS is found in approximately 3 % of persons > 70 years of age and in about 1 % of those > 50 years old. At Mayo Clinic from 1960 through 1994, the risk of progression was 1 % per year. This risk of progression continued even after  of a stable M-protein. The risk for developing MM, WM, or AL was increased 25-fold, 46-fold, and 8.4-fold,...

Necrobiotic xanthogranuloma and monoclonal gammopathy

MUDr. Viera Sandecká, Ph.D.

Onkologie 2022: 16(Suppl.B): 57-60 | DOI: 10.36290/xon.2022.056  

Necrobiotic xanthogranuloma is a progressive and destructive disease associated with paraproteinemia, multiple organ involvement, and an increased risk of developing lymphoproliferative malignancy, such as multiple myeloma, chronic lymphocytic leukemia, or Hodgkin and non-Hodgkin lymphoma. The present article describes a complicated case of a female patient diagnosed with multiple myeloma and necrobiotic xanthogranuloma whose serious health complications were not typical for the diagnosis of multiple myeloma. A thorough diagnostic algorithm and multidisciplinary cooperation of various experts led to the definitive diagnosis. The article also includes...

Review of daratumumab efficacy in all monoclonal gammopathies and other diseases and our experience with daratumumab, lenalidomide and dexamethasone therapy in 74 patients

prof. MUDr. Zdeněk Adam, CSc., prof. MUDr. Luděk Pour, Ph.D., prof. MUDr. Marta Krejčí, Ph.D., MUDr. Ing. David Zeman, Ph.D., doc. MUDr. Zdeněk Řehák, Ph.D., MUDr. Renata Koukalová, Ph.D., MUDr. Viera Sandecká, Ph.D., MUDr. Martin Štork, Ph.D., MUDr. Zdeněk Král, CSc.

Onkologie 2022: 16(Suppl.B): 61-82 | DOI: 10.36290/xon.2022.057  

Multiple myeloma (MM) is a hematological malignancy characterized by renal insufficiency, bone lesions, anaemia, and hypercalcemia. In this modern era of medicine, even with the development of drugs like immunomodulatory drugs (IMiDs) and proteasome inhibitors (PI), the treatment of MM prevails as a challenge. However, even after the attainment of total remission, relapse of MM and disease progression is frequent. That is why there is an urgent requirement to develop novel monoclonal antibody drugs. The latest drugs for the treatment of relapsed and refractory MM (RRMM) approved by the Food and Drug Administration (FDA) is daratumumab. In this article,...

Waldenström´s macroglobulinemia, overview of clinical symptoms, and therapy and our experience with ibrutinib therapy

prof. MUDr. Zdeněk Adam, CSc., MUDr. Ing. David Zeman, Ph.D., prof. MUDr. Luděk Pour, Ph.D., prof. MUDr. Marta Krejčí, Ph.D., MUDr. Martin Krejčí, doc. MUDr. Zdeněk Řehák, Ph.D., MUDr. Renata Koukalová, Ph.D., MUDr. Viera Sandecká, Ph.D., MUDr. Martin Štork, Ph.D., MUDr. Gabriela Romanová, MUDr. Zuzana Adamová, Ph.D., MUDr. Zdeněk Král, CSc.

Onkologie 2022: 16(Suppl.B): 83-102 | DOI: 10.36290/xon.2022.058  

Waldenström macroglobulinemia (WM) is a lymphoplasmacytic lymphoma with immunoglobulin M (IgM) monoclonal protein. Clinical features include anemia, thrombocytopenia, hepatosplenomegaly, lymphadenopathy, in advanced disease hyperviscosity, kryoglobulinemia, B-symptoms and in rare cases the disease can infiltrate CNS (Big-Neel syndrom) or lungs. Presence of IgM monoclonal protein associated with clonal lymphoplasmacytic cells in bone marrow confirms the diagnosis. The L265P mutation in MYD88 is detectable in more than 90% of patients and is found in the majority of IgM MGUS patients. Not all patients who fulfill WM criteria require therapy; these patients...

Social and psychological effects on disease course

prof. MUDr. Zdeněk Adam, CSc., PhDr. Mgr. Jeroným Klimeš, Ph.D., doc. MUDr. Zdeněk Boleloucký, CSc.

Onkologie 2022: 16(Suppl.B): 103-110 | DOI: 10.36290/xon.2022.059  

The diagnosis of a malignant disease fundamentally changes the identity of the patient, turning a healthy person into a sick one. Numerous studies have aimed to analyse how an individual’s future destiny is influenced by whether they live alone or in a firmly established cohabitation relationship or in a marriage. Most analyses dealing with this issue concluded that living in a couple relationship was a positive prognostic factor. On the other hand, developing a malignant disease at a young age increases the risk of couple breakup. It is also stress that the patient is exposed to during treatment that has a negative impact on the course of the...