Monoclonal antibodies in multiple myeloma – breakthrough in the therapy

Myeloma multiplex (MM) is one of the most common haematological malignancies. In recent years, due to new drugs introduction, overall survival of the patients has improved very much. Nevertheless new active ingredients are being searched. There is growing attention to monoclonal antibodies. Over 20 molecules are being investigated, but none of them has already been approved in MM. Work on elotuzumab, daratumumab and in bone disease on denosumab is the most advanced. In this article we describe the current state of knowledge

Terapia pomalidomidem u pacjenta z 17-letnim wywiadem szpiczaka plazmocytowego

The article is dedicated to pomalidomide efficacy in heavily pretreated patient with a long term history of plasma cell myeloma. We provide the data on effectiveness and safety in relapsed and refractory myeloma.W artykule opisano efekty leczenia pomalidomidem (POM) u pacjenta z wieloletnim wywiadem szpiczaka plazmocytowego, po wielu liniach chemioterapii. Przytoczono dane z piśmiennictwa na temat skuteczności i bezpieczeństwa POM u pacjentów z oporną i nawrotową postacią tej choroby

Nowotwór nerki u pacjentów ze szpiczakiem plazmocytowym — opis dwóch przypadków i przegląd piśmiennictwa

Multiple myeloma (MM) is characterized by malignant spreading of monoclonal plasma cells in bone marrow. Renal cell carcinoma (RCC) is a result of neoplastic proliferation of epithelial cells in nephron proximal convoluted tubule and forms 95% of malignant kidney’s neoplasms. There are some reports about coexistence of MM and RCC but it is a rare phenomenon. We describe two cases of patients in which during the therapy of MM, RCC was detected accidentally. Radical nephrectomy in early stage of RCC allowed complete recovery and did not interrupt the continuation of MM’s therapy. Additionally, similar cases from literature are discussed.Szpiczak plazmocytowy (PCM) charakteryzuje się ekspansją nowotworowych komórek plazma­tycznych w szpiku. Rak nerkowokomórkowy (RCC) jest rezultatem złośliwej proliferacji komórek nabłonkowych kanalików proksymalnych nefronu i stanowi 95% złośliwych nowotworów nerki. Współistnienie PCM i RCC to zjawisko bardzo rzadkie, istnieją jedynie pojedyncze doniesienia na ten temat. W pracy przedstawiono opis dwóch chorych, u których w trakcie terapii PCM wykryto przypadkowo RCC. Radykalna nefrektomia (we wczesnej fazie choroby) umożliwiła całkowite wyleczenie RCC i kontynuację terapii PCM. Ponadto w artykule omówiono podobne przypadki opisane w literaturze

Zajęcie ośrodkowego układu nerwowego w przebiegu szpiczaka plazmocytowego – opis przypadku i przegląd literatury

In multiple myeloma extramedullary involvement occurs in 13% of patients. Central nervous system (CNS) involvement is extremely rare and comprises 1% of cases. Review of literature shows only limited data on this subject and consists mostly of few case reports. Scarce knowledge about CNS involvement in the course of multiple myeloma makes its diagnosis and treatment very difficult and challenging. The authors describe a clinical case which illustrates the problem from a clinician point of view. Furthermore, we analyzed the available data from literature and summarized the current state of knowledge on diagnosis, potential prognostic factors, treatment and prognosis in CNS myeloma involvement

POEMS Syndrome: Real World Experience in Diagnosis and Systemic Therapy - 108 Patients Multicenter Analysis

POEMS syndrome, a rare plasma cell disorder, is challenging both in the diagnostic and therapeutic management. We present real word retrospective analysis of 108 cases analyzing clinical features and therapeutic modes. We compare our results with the available literature. This is the first description with such wide use of proteasome inhibitors in first line treatment. POEMS (Polyneuropathy, organomegaly, endocrinopathy, M-protein, skin changes) syndrome is a rare and challenging plasma cell disorder, both in the diagnostic and therapeutic management of the disease. Currently, the literature on POEMS is sparse with most evidence being case reports and small case studies. We present a retrospective real world experience of 108 patients with POEMS. We analyzed the clinical features and therapeutic interventions. Regarding clinical features, our findings demonstrated that skin lesions, thrombocythemia and polycythemia were present less frequently than reported previously. Regarding clinical interventions, this is one of the largest analyses of front line treatment in POEMS and the first one to include frequent utilization of proteasome inhibitors (37%). Bortezomib monotherapy was the most effective therapy achieving complete remission/very good partial remissions (CR/VGPR) in 69% of patients. Thirty percent of patients proceeded to planned autologous stem cell transplant (ASCT) as part of the front-line treatment resulting in statistically superior progression-free (PFS) and overall survival (OS) compared to non-ASCT treated patients (P= .003). In multivariate analysis, anemia, thrombocytopenia, and as age over 60 were associated with a negative impact on patient outcomes

Outcome of Second Primary Malignancies Developing in Multiple Myeloma Patients

Background: There is an increased risk of second primary malignancies (SMPs) in patients with multiple myeloma (MM). This multinational 'real-world' retrospective study analyzed the characteristics and outcomes of MM patients that developed SPMs.Results: 165 patients were analyzed: 62.4% males; 8.5% with a prior cancer; 113 with solid SPMs, mainly =stage 2; and 52 with hematological SPM (hemato-SPM), mainly MDS/AML. Patients with hemato-SPM were younger (p = 0.05) and more frequently had a prior AutoHCT (p = 0.012). The time to SPM was shorter in the older (>65 years) and more heavily pretreated patients. One hundred patients were actively treated at the time of SPM detection. Treatment was discontinued in 52, substituted with another anti-MM therapy in 15, and continued in 33 patients. Treatment discontinuation was predominant in the patients diagnosed with hemato-SPM (76%). The median OS following SPM detection was 8.5 months, and the main cause of death was SPM. A poor ECOG status predicted a shorter OS (PS 3 vs. 0, HR = 5.74, 2.32-14.21, p < 0.001), whereas a normal hemoglobin level (HR = 0.43, 0.19-0.95, p = 0.037) predicted longer OS.Conclusions: With the continuing improvement in OS, a higher proportion of MM patients might develop SPM. The OS following SPM diagnosis is poor; hence, frequent surveillance and early detection are imperative to improve outcomes

Results of Polish Adult Leukemia Study Group (PALG) project assessing TP53 mutations with next-generation sequencing technology in relapsed and refractory chronic lymphocytic leukemia patients — an 18-month update

Indtroduction and methods: In chronic lymphocytic leukemia (CLL), molecular and cytogenetic diagnostics are crucial for the determination of accurate prognosis and treatment choice. Among different genetic aberrations, del(17p13) or TP53 mutations constitute high-risk factors, and early identification of such defects is a high priority for CLL patients. While cytogenetic diagnostics is well-established and accessible for the majority of CLL patients in Poland, molecular diagnostics of TP53 mutations is performed only in a few ERIC-certified centers (eight as of September 2020), and only two of these employ next-generation sequencing (NGS) for routine analysis of TP53 status in CLL patients. Here we report the interim results of a project assessing TP53 mutations with NGS technology in relapsed or refractory CLL patients with confirmed negative del(17p13) status. 249 patients from 32 clinical centers were included in the study. Results: NGS analysis revealed TP53 mutations in 42/249 (17%) patients, half of whom (21/249, 8.5%) had subclonal mutations (VAF ≤10%). These results are in line with published data in relapsed/refractory CLL patients. Conclusions: The results of the project demonstrated the feasibility and accuracy of NGS testing in CLL patients despite several initial logistical and technical obstacles. Our study also proved that, with appropriate funding, CLL patients from any hematological center in Poland can have access to state-of-the-art molecular diagnostic

Unusual clinical manifestations of multiple myeloma

Multiple myeloma accounting for 10% of malignancies of haematopoietic system is a heterogenous disease. In the era of individual approach to the patient, we are more and more interested in distinct features and forms of this malignancy. In the article, we described rare MM manifestations with reference to location (central nervous system and skin), M-protein produced by the tumor cells (biclonal and immunoglobulin M), early onset of the disease (young versus old) and pregnancy in MM patients