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

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

prof. MUDr. Zdeněk Adam, CSc.¹, prof. MUDr. Luděk Pour, Ph.D.¹, prof. MUDr. Marta Krejčí, Ph.D.¹, MUDr. Ing. David Zeman, Ph.D.², doc. MUDr. Eva Vlčková, Ph.D.³, doc. MUDr. Milan Krtička, Ph.D.⁴, prof. MUDr. Pavel Šlampa, CSc.⁵, MUDr. Soňa Štěpánková, Ph.D.⁶, MUDr. Gabriela Romanová⁷, MUDr. Zuzana Adamová, Ph.D.⁸, doc. MUDr. Jiří Vaníček, Ph.D.⁹, doc. MUDr. Zdeněk Řehák, Ph.D.¹⁰, MUDr. Renata Koukalová, Ph.D.¹⁰, doc. MUDr. Veronika Matušková, Ph.D.¹¹, MUDr. Zdeněk Král, CSc.¹, MUDr. Libor Červinek, Ph.D.¹, MUDr. Viera Sandecká, Ph.D.¹

¹ Interní hematologická a onkologická klinika, LF MU a FN Brno

² Ústav laboratorní medicíny - Oddělení klinické biochemie, LF MU a FN Brno

³ Neurologická klinika, LF MU a FN Brno

⁴ Klinika úrazové chirurgie, LF MU a FN Brno

⁵ Klinika radiační onkologie - LF MU a Masarykův onkologický ústav, Brno

⁶ Centrum kardiovaskulární a transplantační chirurgie, FN u svaté Anny, Brno

⁷ Oddělení klinické hematologie - specializovaná ambulance pro koagulační poruchy, LF MU a FN Brno

⁸ Chirurgické oddělení, Nemocnice Frýdek Místek a Chirurgické oddělení Vsetínské nemocnice

⁹ Radiologická klinika, LF MU a FN u svaté Anny, Brno

¹⁰ Oddělení nukleární medicíny, Masarykův onkologický ústav, Brno

¹¹ Oční klinika, LF MU a FN Brno

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 on magnetic resonance imaging (MRI). The presence of del(17p), t(4;14), t(14;16), t(14;20), gain 1q, or p53 mutation is considered high-risk multiple myeloma. Presence of any two high risk factors is considered double-hit myeloma; three or more high risk factors is triple-hit myeloma. 

Solitary plasmacytoma is an infrequent form of plasma cell dyscrasia that presents as a single mass of monoclonal plasma cells, located either extramedullary or intraosseous. In some patients, a bone marrow aspiration can detect a low monoclonal plasma cell infiltration which indicates a high risk of early progression to an overt myeloma disease. Before treatment initiation, whole body positron emission tomography-computed tomography or magnetic resonance imaging should be performed to exclude the presence of additional malignant lesions. For decades, treatment has been based on radiation, but studies exploring the potential benefit of systemic therapies for high-risk patients are urgently needed, becouse in solitary plasmocytomas of diameter more the 5 cm is the response to radioterapie often incomplete.

Primary plasma cell leukemia (PCL) has a consistently ominous prognosis, even after progress in the last decades. PCL deserves a prompt identification to start the most effective treatment for this ultra-high-risk disease. The aim of this position paper is to revisit the diagnosis of PCL according to the presence of circulating plasma cells in patients otherwise meeting diagnostic criteria of multiple myeloma. The presence of ≥5% circulating plasma cells in patients with MM had a similar adverse prognostic impact as the previously defined PCL by more the 20% ciculating plasma cells. Therefore PCL is defined by International Myeloma Working Group by the presence of 5% or more circulating plasma cells in peripheral blood smears in patients otherwise diagnosed with symptomatic multiple myeloma. 

Diagnostic procedures and critarias are discusse in this paper and even therapy of this diesease overviewed.

Keywords: multiple myeloma, solitary plasmocytoma, plasma cell leukemia, daratumumab, bone marrow transplantation.

Accepted: October 4, 2022; Published: October 14, 2022  Show citation