Plasma Cell Leukemia: Definition, Presentation, and Treatment

Gundesen, Michael; Lund, Thomas; Moeller, Hanne E. H.; Abildgaard, Niels

doi:10.1007/s11912-019-0754-x

Cited by 77 publications

(80 citation statements)

References 64 publications

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“…The prevalence of T cells showed a gradual increase during progression, but this was not observed for the other subsets. In patients with PCL, we observed a unique profile with higher percentages of all analyzed subsets compared to sMM and MM, as circulating APCs in PCL are less dependent from the BM niche [20]. The variability of the BM microenvironmental composition among patients indicates the apparent heterogeneity of MM beyond the molecular level, thus importing an additional challenge for efficient patients' stratification.…”

Section: Composition Of the Bm Microenvironment At Different Stages Omentioning

confidence: 78%

Deep Phenotyping Reveals Distinct Immune Signatures Correlating with Prognostication, Treatment Responses, and MRD Status in Multiple Myeloma

Papadimitriou

Tsakirakis

Malandrakis

et al. 2020

Cancers

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Despite recent advances, Multiple Myeloma (MM) remains an incurable disease with apparent heterogeneity that may explain patients’ variable clinical outcomes. While the phenotypic, (epi)genetic, and molecular characteristics of myeloma cells have been thoroughly examined, there is limited information regarding the role of the bone marrow (BM) microenvironment in the natural history of the disease. In the present study, we performed deep phenotyping of 32 distinct immune cell subsets in a cohort of 94 MM patients to reveal unique immune profiles in both BM and peripheral blood (PB) that characterize distinct prognostic groups, responses to induction treatment, and minimal residual disease (MRD) status. Our data show that PB cells do not reflect the BM microenvironment and that the two sites should be studied independently. Adverse ISS stage and high-risk cytogenetics were correlated with distinct immune profiles; most importantly, BM signatures comprised decreased tumor-associated macrophages (TAMs) and erythroblasts, whereas the unique Treg signatures in PB could discriminate those patients achieving complete remission after VRd induction therapy. Moreover, MRD negative status was correlated with a more experienced CD4- and CD8-mediated immunity phenotype in both BM and PB, thus highlighting a critical role of by-stander cells linked to MRD biology.

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Section: Composition Of the Bm Microenvironment At Different Stages Omentioning

confidence: 78%

Deep Phenotyping Reveals Distinct Immune Signatures Correlating with Prognostication, Treatment Responses, and MRD Status in Multiple Myeloma

Papadimitriou

Tsakirakis

Malandrakis

et al. 2020

Cancers

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“…Only patients diagnosed with Plasma Cell Leukemia were excluded from the study. Plasma cell leukemia was defined at least having more than 20% clonal plasma cell in the peripheral blood 5 . First line treatment agents, International Staging System (ISS), demographic and clinical characteristics and survival periods were recorded from files.…”

Section: Methodsmentioning

confidence: 99%

Concepts of Double Hit and Triple Hit Disease in Multiple Myeloma, Entity and Prognostic Significance

Baysal

Demirci

Ümit

et al. 2020

Sci Rep

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Risk assessment in newly diagnosed multiple myeloma patients (NDMM) is the first and the most crucial determinant of treatment. With the utilization of fiSH analysis as a part of routine practice, high riskMultiple Myeloma (MM) is defined as having at least one of the mutations related with poor prognosis including; t(4;14) t(14;16), t(14;20), del 17p, p53 mutation, gain 1q and del 1p. M-Smart MM risk stratification guideline by Mayo Clinic has proposed a concept similar to high grade lymphomas. Having two of the high risk genetic abnormalities were defined as double hit MM and having any three as triple hit MM. Based on these definitions which may bring a much more clinically relatable understanding in MM prognosis, we aimed to assess our database regarding these two concepts and their probable significance in terms of outcome and prognosis. We retrospectively evaluated 159 newly diagnosed multiple myeloma patients and their clinical course. Among these patients; twenty-four patients have one high risk determinant and also seven and two patients were classified as double hit MM and triple hit MM respectively. Overall survival (OS) of the patients with double hit MM was 6 months, 32.0 months for patients with single high risk abnormality and 57.0 months for patients with no high risk abnormality. Univariate analysis showed that Double Hit and Triple Hit MM is a predictive of low OS. Hazard Ratio of patients with one high risk abnormality was 1.42, double-hit MM patients was 5.55, and triple-hit MM patients was 7.3. Despite the development of novel drugs and their effects of prolonging survival, the treatment has not been individualized. Understanding the biology of each patient as a unique process will be the success of the treatment. As it is known that some MM patients harbor high risk genetic abnormalities according to fiSH analysis, we can continue the argument that some patients bring an even higher risk and that can be defined as double or triple hit MM.

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“…Historically around 60-70% of reported cases have been primary PCL, although secondary PCL is increasing as survival for MM has improved and now there appears to be an even distribution of primary versus secondary PCL [3,4]. PCL is diagnosed using the Kyle's criteria which consist of having at least 20% circulating plasma cells and a total plasma cell count of at least 2 × 10 9 /L in the peripheral blood [2,4,5]. The most common laboratory findings include leukocytosis, anemia, and thrombocytopenia [3].…”

Section: Discussionmentioning

confidence: 99%