Aims: Patients undergoing allogeneic hematopoietic stem cell transplantation (allo-HSCT) are highly susceptible to infectious complications due to immunosuppression and delayed immune reconstitution, with pneumonia remaining a major cause of early morbidity and mortality. Composite inflammation- and protein-based biomarkers, including the C-reactive protein-to-albumin ratio (CAR) and fibrinogen-to-albumin ratio (FAR), have prognostic value in oncology, but their role in predicting pneumonia and survival after allo-HSCT is unclear.Methods: This retrospective, single-center study included 203 adult allo-HSCT recipients treated between 2020 and 2023. Pre transplant inflammatory and protein-based biomarkers were assessed 7-14 days before transplantation, and Cox proportional hazards regression was used to identify independent predictors of one-year post-transplant pneumonia and mortality.Results: Pneumonia occurred in 17.7% of patients, and one-year mortality was 41.9%. Although lower albumin, hemoglobin, and platelet levels and higher procalcitonin (PCT) concentrations were associated with pneumonia in univariate analyses, no biomarker independently predicted post-transplant pneumonia after multivariate adjustment. In contrast, pre-transplant PCT (HR=1.020, p
Introduction & Objective: Graft-versus-host disease (GVHD) is a major complication of allogeneic hematopoietic stem cell transplantation (allo-HSCT), with limited treatment options for steroid-resistant cases. Ruxolitinib, a JAK1/2 inhibitor, has shown promise in treating steroid-resistant acute (aGVHD), chronic (cGVHD), and overlap GVHD (oGVHD), but real-world data remain limited. This study evaluated the real-world efficacy and safety of ruxolitinib in allo-HSCT patients with steroid-resistant GVHD. Materials & Methods: This retrospective, multicenter study included adult patients treated with ruxolitinib for Grade II or higher aGVHD or moderate-to-severe cGVHD at nine centers in Turkey (2017-2024). Clinical characteristics, treatment responses, and adverse events were recorded. Primary outcomes were overall response rate (ORR) and overall survival (OS). Results: Among 80 patients (mean age: 39.3 ± 13.3 years; 60 males), 39 had aGVHD, 68 cGVHD, and 15 oGVHD. The ORR was 72 of 80 patients (90.0%) (complete response: 37 of 80 [46.3%], partial response: 35 of 80 [43.8%]). The 1-year and 2-year OS rates were 91.3% and 82.5%. Severe cGVHD (p < 0.001) and lack of response to ruxolitinib (p = 0.018) were associated with reduced OS. Adverse events included infections in 40 of 80 patients (50.0%), cytopenias in 23 of 80 (28.7%), and cytomegalovirus reactivation in 20 of 80 (25.0%). Conclusion: In this retrospective multicenter cohort, ruxolitinib was associated with high response rates in steroid-refractory GVHD, while disease severity remained a key determinant of survival, and findings should be interpreted as exploratory.
Mesenchymal stem/stromal cells (MSCs) exhibit broad differentiation capability and strong immunoregulatory potential mediated through intercellular communication and the release of diverse paracrine mediators. They represent a promising but still investigational therapeutic approach for managing complications associated with allogeneic hematopoietic stem cell transplantation (allo-HSCT). This review provides an updated synthesis of MSC biology, their bidirectional interaction with immune cells, and their functional contribution to the hematopoietic niche. It also evaluates current clinical evidence regarding the therapeutic roles of MSCs and MSC-derived extracellular vesicles (EVs) in acute and chronic graft-versus-host disease (aGVHD/cGVHD), as well as in poor graft function. Mechanistic insights encompass macrophage polarization toward an anti-inflammatory phenotype, inhibition of dendritic cell maturation, enhancement of regulatory T-cell expansion, and modulation of cytokine signaling pathways. Within the bone marrow milieu, MSCs contribute to stromal restoration and angiogenic repair. Recent phase II/III trials in steroid-refractory (SR)-aGVHD have demonstrated overall response rates ranging from 48 to 71%. Efficacy appears particularly enhanced in pediatric patients and with early MSC administration. Across studies, MSC therapy shows a favorable safety profile; however, heterogeneity in response and inconsistent survival outcomes remain notable limitations. For poor graft function, limited prospective studies indicate hematopoietic recovery following third-party MSC infusions, and combination approaches such as co-administration with thrombopoietin receptor agonists are under investigation. MSC-derived EVs emulate many immunomodulatory effects of their parental cells with a potentially safer profile, though clinical validation remains in its infancy. MSC-oriented interventions hold substantial biological and therapeutic promise, offering a favorable safety margin; however, clinical translation is hindered by product variability, suboptimal engraftment and persistence, and inconsistent efficacy across studies. Future directions should emphasize standardized manufacturing and potency assays, biomarker-driven patient and timing selection, optimized conditioning and dosing strategies, and the systematic appraisal of EV-based or genetically modified MSC products through controlled trials.
INTRODUCTION:Rituximab-cyclophosphamide-doxorubicin-vincristine-prednisolone (R-CHOP) is a standard first-line treatment in large B-cell lymphoma (LBCL). Dose intensity is frequently reduced because of toxicity or concerns of tolerability. The true impact of such treatment modifications on the response rates is difficult to assess in nonrandomized comparisons because of complex and reciprocal interactions between various determinants of both variables. To avoid these confounding biases, this study was designed to analyze subsequent outcomes after a complete response is achieved with frontline therapy, aiming to uncover an independent link between R-CHOP dose-intensity and long-term outcomes in LBCL. METHODS:Patients treated between 2012 and 2024 were included. Reduced dose-intensity was predefined as more than 20% dose reduction for any one of the drugs for two cycles or a cumulative delay of ≥21 days. The primary and secondary objectives were the duration of complete response and the cumulative incidence of relapse (CIR), respectively. RESULTS:The LBCL cohort consisted of 1369 consecutive cases. For this study, 953 were eligible, with 728 and 225 patients in the dose-intense and reduced-intensity groups, respectively. Duration of complete response was significantly longer for the dose-intense group with a 3-year estimate of 87.8% vs 68.8% (hazard ratio: 0.39, p < .0001). The estimated 3-year cumulative incidence of relapse was 10.3% vs 21.4% (hazard ratio: 0.51, p = .004), favoring the dose-intense group. Reduced dose intensity was an independent risk factor on multivariate analysis for both endpoints. CONCLUSIONS:Focusing on long-term outcomes after CR, this study demonstrates an increased risk of relapse when R-CHOP intensity is reduced, providing novel evidence that complements the association between reduced treatment intensity and inferior LBCL outcomes.
Background: Chronic lymphocytic leukemia (CLL) is a biologically heterogeneous disease characterized by variable clinical outcomes. The introduction of targeted therapies, particularly the BCL-2 inhibitor venetoclax, has significantly improved treatment outcomes in patients with relapsed/refractory (R/R) CLL. However, real-world data on the safety and effectiveness of venetoclax-based regimens remain limited. Methods: In this multicenter retrospective study, 147 adult patients with R/R CLL treated with venetoclax between April 2019 and August 2025 were analyzed. Venetoclax was administered as monotherapy or in combination with rituximab, obinutuzumab, or ibrutinib. Adverse events were graded according to CTCAE v4.0, and treatment responses were assessed based on IWCLL criteria. Survival outcomes, including overall survival (OS) and progression-free survival (PFS), were evaluated using Kaplan–Meier analysis. Results: The median age at venetoclax initiation was 64 years, and patients had received a median of two prior lines of therapy. Combination therapy was administered in 78.9% of patients. The overall response rate was 83.0%, including complete remission in 65.3% of patients. Grade ≥ 3 hematologic adverse events included neutropenia (18.4%), thrombocytopenia (14.3%), and anemia (7.5%). Tumor lysis syndrome (TLS) occurred in 28.6% of patients, predominantly during the dose ramp-up phase. At a median follow-up of 61 months, median OS and PFS were both 60 months. Bulky disease was associated with inferior survival outcomes. Conclusions: Venetoclax-based therapy is effective and well tolerated in patients with R/R CLL in a real-world setting. High response rates and durable survival outcomes were observed despite the inclusion of patients with high-risk clinical and biological features. These findings support the use of venetoclax as a key component of modern CLL treatment strategies and highlight the importance of real-world evidence in optimizing patient management.
Background/Objectives: Allogeneic hematopoietic stem cell transplantation is associated with increased cardiovascular risk driven by endothelial dysfunction, chronic inflammation, and treatment-related metabolic disturbances, including dyslipidemia. In the contemporary era of post-transplant cyclophosphamide-based prophylaxis, the prognostic significance of dyslipidemia-particularly as assessed by non-HDL cholesterol-remains unclear. In this study, we aimed to compare the engraftment days, graft-versus-host disease (GVHD) development, relapse, overall survival rates, and cardiovascular mortality in patients using myeloablative/reduced intensity conditioning regimens with post-transplant cyclophosphamide (PTCy) 50 mg/kg/day for 2 days in patients with acute leukemias. Methods: A total of 95 adult patients with acute leukemias were included in their first remission who underwent matched sibling donor transplantation with PTCy (50 mg/kg on days +3 and +4). Patients were stratified according to pre-transplant non-HDL-C levels (<160 mg/dL vs. ≥160 mg/dL). Matched related donors were selected for the patients. All patients received either myeloablative or reduced-intensity conditioning based on EBMT criteria, with fludarabine-based combinations including busulfan, treosulfan, or TBI, along with ATLG administered at a total dose of 15 mg/kg. Peripheral blood stem cells were used for all transplants, and GVHD prophylaxis consisted of cyclosporine. Results: Platelet (median 13 vs. 14 days) and neutrophil (median 14 vs. 15 days) engraftment times and veno-occlusive disease (VOD) rates were comparable across groups (all p > 0.05); cumulative incidences of grade II-IV aGVHD at +100 days, grade III-IV aGVHD at +100 days, and moderate-severe cGVHD at 1 year, relapse-free survival, and non-relapse mortality at 1 year were comparable in two cohorts (all p > 0.05). GVHD-free/relapse-free survival (GRFS) at 1 year was also comparable across groups (p = 0.15). Median GRFS was 150 (95% CI: 120-330) days and 270 (95% CI: 154-not reached) days, respectively [HR was 0.68 (0.40-1.15), p = 0.15; GRFS at 1 year was 66.6% vs. 52.0%, respectively]. The groups were also comparable in terms of overall survival (OS). Follow-up ranged from 0.5 to 108 months, and median follow-up was 60 months in two cohorts. Median OS was not reached in non-HDL-C < 160 (95% CI: 70 months-not reached) and 67 months in non-HDL-C ≥ 160 groups (95% CI: 13 months-not reached) (Log rank = 0.21). No cardiovascular death events occurred during the follow-up period. Conclusions: In this homogeneous matched sibling donor transplant cohort with extended follow-up and uniform administration of post-transplant cyclophosphamide, cyclosporine-based GVHD prophylaxis, and anti-thymocyte lymphoglobulin (ATLG), pre-existing dyslipidemia was not associated with an adverse impact on GRFS, NRM, PFS, CMV reactivation, OS or long-term cardiovascular mortality.
A 57 -year -old woman with relapsed Philadelphia chromosome -negative B -cell acute lymphoblastic leukemia developed diffuse non -blanching erythematous skin lesions following allogeneic hematopoietic stem cell transplant. The lesions appeared concomitantly with hepatic sinusoidal obstruction syndrome and persisted despite engraftment and supportive therapy. Skin biopsy excluded graft -versus -host disease. Upon initiation of defibrotide for moderate hepatic sinusoidal obstruction syndrome, both hepatic and cutaneous findings resolved completely. This case underscores the systemic endothelial -protective potential of defibrotide, extending beyond the hepatic microvasculature, and highlights cutaneous endotheliopathy as a possible manifestation of transplant -associated endothelial injury.
Background/Objectives: Real-world data on the therapeutic use of FLT3 inhibitors in Turkey remain limited. Therefore, we retrospectively evaluated outcomes from 13 academic centers nationwide, focusing on the multikinase inhibitor midostaurin in patients with newly diagnosed FLT3-mutated acute myeloid leukemia (AML). Methods: We collected comprehensive information regarding treatment efficacy, safety, and tolerability. Results: The overall response rate to intensive chemotherapy (3 + 7) plus midostaurin was 87.7%, with a complete remission rate of 84.2%, consistent with previously reported clinical trial results. Treatment discontinuation due to intolerance or toxicity was low (3.5%). One patient discontinued therapy because of septic shock during induction, and another due to a drug-drug interaction during consolidation. Median overall survival was 21.4 months. Allogeneic stem cell transplantation was performed in first remission in 52.6% of patients. Five patients (8.8%) were refractory to induction therapy, and relapse occurred in 21.1% (12 patients). Conclusions: These findings support the effectiveness and acceptable tolerability of midostaurin in routine clinical practice for FLT3-mutated AML.
Objective: Acute graft versus host disease (GVHD) occurs in 20-80 % of patients undergoing allogeneic hematopoietic stem cell transplantation (allo-HSCT). Of these patients, 40 % will be resistant to steroids, which is the standard first-line approach. There is no standard second line treatment approach for patients with steroid refractory acute GVHD (SR-aGVHD). Alpha-1 antitrypsin is a protease inhibitor and has anti-inflammatory and immune regulatory properties. Here we report the outcomes and safety data of 17 patients treated with alpha-1 antitrypsin for SR-aGVHD. Material and methods: Patients who received at least 2 lines of alpha-1 antitrypsin treatment for SR-aGVHD at five transplant centers in T & uuml;rkiye were included in this retrospective study. Results: The median number of alpha-1 antitrypsin treatment line patients received was 4 (range, 2-5). The median time between alpha-1 antitrypsin administration and response was 65 days (range, 10-138 days). Overall response rate was 70.6 %. When the first- and second-month response rates were compared according to GVHD organ involvement, we found that the response rates were similar in skin, liver and gastrointestinal system involvement (p = 0.281 and p = 0.305, respectively). No grade 3-4 anemia, thrombocytopenia or neutropenia was observed after alpha-1 antitrypsin treatment. Two patients had cytomegalovirus infection and 1 patient had pneumonia. At a median follow-up of 7 months, overall survival was 70.6 % and median overall survival was not reached. Conclusion: In conclusion, alpha-1 antitrypsin is an effective and safe treatment option in patients with SRaGVHD, with response rates of up to 70 % in patients with skin, liver and gastrointestinal system involvement. Larger studies are needed to establish a standard second and subsequent treatment approach in patients with SR-aGVHD.
BACKGROUND:Allogeneic hematopoietic stem cell transplantation is a curative therapy for malignant and non-malignant hematologic disorders. Conditioning regimens, such as those based on busulfan, are utilized to promote engraftment. Busulfan-based regimens are commonly employed but exhibit significant toxicity, leading to interest in alternate drugs such as treosulfan, which may offer a superior safety profile while preserving efficacy. METHODS:This retrospective study examined 240 adult patients diagnosed with acute myeloid leukemia or acute lymphoblastic leukemia who received allogeneic hematopoietic stem cell transplantation at nine transplantation centers in Türkiye from 2010 to 2024. Patients underwent either a busulfan-based or treosulfan-based conditioning regimen. RESULTS:Engraftment was effective in both groups, with neutrophil engraftment happening earlier in the busulfan group (13 days vs. 15 days, p = 0.001). The incidence of veno-occlusive disease was significantly higher in the busulfan group (10.1 % vs. 0.9 %, p = 0.002). Cytomegalovirus infections were more prevalent in the busulfan group (51.2 % vs. 26.1 %, p < 0.001). Acute graft versus host disease occurred more frequently in the busulfan group (27.9 % vs. 18.9 %, p = 0.128); nonetheless, this difference did not reach statistical significance. No significant differences in relapse rates were detected through the groups. The overall survival was not reached in the busulfan group, while the treosulfan group demonstrated an overall survival of 40 months. CONCLUSION:Treosulfan-based conditioning regimens demonstrate similar efficacy to busulfan-based regimens while presenting a more acceptable toxicity profile. Treosulfan-based regimens also show a lower occurrence of veno-occlusive disease and cytomegalovirus infections.
Background: Multiple myeloma (MM) is a heterogeneous plasma cell malignancy with non-specific symptoms and disease heterogeneity at clinical and biological levels. This non-specific set of symptoms, including bone pain, anemia, renal failure, hypercalcemia, and neuropathy, can mislead diagnosis as chronic or benign conditions, resulting in a delay in diagnosis. Timely identification is paramount to prevent organ damage and reduce morbidity. Methods: In this review, we present an overview of recent literature concerning the factors leading to the delayed diagnosis of MM and the impact of delayed diagnosis. This includes factors relevant to physicians and systems, diagnostic processes, primary healthcare services, and laboratory and imaging data access and interpretation. Other emerging technologies to diagnose NCIs include AI-based decision support systems and biomarker-focused strategies. Findings: Delayed diagnosis can lead to presentation at advanced disease stages associated with life-threatening complications and shorter progression-free survival. Patients are often seen by many physicians before they are referred to hematology. Understanding of clinical red flags for MM in primary care is inadequate. Our findings indicate that limited access to diagnostic tests, inconsistent follow-up of MGUS/SMM patients, and a lack of interdepartmental coordination delay the diagnostic process. Conclusions: Multimodal tools for early diagnosis of MM. Educational campaigns to raise awareness of the disease, algorithms dedicated to routine care and novel technologies, including AI and big data analytics, and new biomarkers may serve this purpose, as well as genomic approaches to the premalignant MGUS stage.
In this study, we aimed to compare the engraftment days, graft-versus-host disease (GVHD) development, relapse and overall survival rates in patients using myeloablative/reduced intensity conditioning regimens with posttransplant cyclophosphamide (PTCy) 25 mg/kg x2 with Anti-T lymphocyte Globulin (ATLG) (n = 29) and PTCy 50 mg/kg x2 doses (n = 41) in patients with acute leukemias. Matched related, matched unrelated, 1 mismatched unrelated, and haploidentical donors were selected for the patients. Platelet (median 11 vs 17 days) and neutrophil (median 14 vs 15 days) engraftment times were shorter in ATLG+ PTCy25 treated patients (both p < 0.05); veno-occlusive disease rates, graft failure and poor graft functions were similar between the two approaches (all p > 0.05); cumulative incidences of grade II-IV aGVHD at +100 days, grade III-IV aGVHD at +100 days, and grade II-IV cGVHD at 1-year were comparable between ATLG+PTCy25 and PTCy50 groups (all p > 0.05). Cumulative incidences of relapse and non-relapse mortality at 1-year were similar in two cohorts (both p > 0.05). PTCy50 was associated with a statistically significant benefit in terms of GVHD-free/relapse-free survival (GRFS) at 1-year (p = 0.03). Median GRFS was 115 (95% CI: 42-214) days and 248 (95% CI: 151-not reached) days, respectively [HR was 0.51 (0.28-0.95), p = 0.03; GRFS at 1-year was 20.7% vs 44.3%, respectively]. However, the groups were comparable in terms of PFS and OS. Median PFS was 332 days (95% CI: 182 days-not reached) for ATLG+PTCy25 group. It was not reached (95% CI: 210 days-not reached) for the patients who received PTCy50. Median OS was not reached in either ATLG+PTCy25 (95% CI: 191 days-not reached) or PTCy50 groups (Log rank = 0.42). Our study showed that lowering PTCy dose with ATLG seems to accelerate platelet and neutrophil engraftment rates; confers similar survival and relapse rates, similar acute and chronic GVHD frequency despite increased GRFS at 1-year.
Objective: Vitamin D deficiency is common in adult patients undergoing allogenic hematopoietic stem cell transplantation (allo-HSCT). Since vitamin D is an important regulatory factor for the immune system, vitamin D deficiency may have effects on antitumor activity, relapse rates, graft versus host disease (GVHD) occurrence and infection rates in allo-HSCT. We aimed to investigate the effects of vitamin D levels on the outcome of allo-HSCT. Material and methods: This study included 211 patients who underwent allo-HSCT at seven transplant centers in T & uuml;rkiye. The impact of pretransplant vitamin D level on overall survival (OS), relapse rate, GVHD occurrence and engraftment times was analyzed retrospectively Results: Pretransplant vitamin D levels were not related to the neutrophil engraftment day (p: 0.887), relapse rate (p: 0.433) and GVHD occurrence (p: 0.391). At a median follow-up of 14 months, OS was 84.8 % and median OS was not reached. Univariate Cox Regression analysis showed that higher levels of vitamin D (>12 ng/mL) affected the survival rates (p = 0.029) (HR: 0.392: 95 % CI: 10.170-0.907). Conclusion: In our study, pretransplant vitamin D levels were not related to GVHD occurrence, relapse rate and engraftment times. However, we found that higher levels of pretransplant vitamin D levels (threshold is 12 ng/ mL) were associated with increased survival. Further studies with a larger population are necessary to reveal the role of vitamin D in patients undergoing allo-HSCT.
Comparative first-line trials in large B-cell lymphoma (LBCL) have mostly failed over the last decade. Failures were commonly attributed to overestimation of the progression risk for the control arms, bringing the precision of the international prognostic index (IPI) into scrutiny. Simplified molecular IPI (smIPI), introduced at American Society of Hematology 2023, was developed to address the shortcomings of IPI. This study investigated smIPI and its potential implications as trial eligibility criteria among a multicentre LBCL cohort of 1439 patients. Patients diagnosed between 2012 and 2024 were included. Data were collected from institutional archives. The primary end-point was risk stratification for progression-free survival (PFS). Kaplan-Meier and Cox regression methods were used for survival analyses. The smIPI reclassified 38.2% and 5.3% of patients to higher and lower risk groups from IPI, respectively. Patients reclassified to higher risk groups by smIPI had an increased risk of progression than the rest of the group (hazard ratio = 1.34, p = 0.003). High-risk groups classified by IPI and smIPI had similar outcomes (3-year PFS 54.9% vs. 56.3%); however, the high-risk group size was expanded by 35.2% when defined by smIPI. The smIPI is easily applicable and more sensitive than IPI in identifying patients under high risk of progression who are ideal candidates to participate in clinical research.
BACKGROUND:We conducted a multi-center retrospective non-randomized study to clarify roles of 2 and 3 gr/m2 doses of Cyclophosphamide (Cy) for stem cell mobilization in Multiple Myeloma. MATERIAL AND METHODS:This study analyzed 169 adult patients who received Cy-based mobilization regimen at doses 2 and 3 gr/m2 between 2018 and 2024. All patients included in this study were 18 years old and older. Patients divided into two groups; group 1 represents 2 gr/m2 treated patients, and group 2 represents 3 gr/m2 dosing. These two groups then compared in terms of CD34+ cell number collected by apheresis, apheresis days, peak peripheral blood CD34+ value and rates of mobilization failures. RESULTS:Higher doses of Cy yielded more hematopoietic stem cells compared to the lower dose Cy [10.00 (3.00-28.90) vs 9.01 (5.00-14.90), p = 0.002]; while mobilization failures and apheresis days were comparable across groups (p = 0.112 and p = 0.648, respectively). Apheresis days required for adequate stem cell harvest among groups was found statistically similar (p = 0.648). CONCLUSION:This study indicated the superior efficacy of 3 gr/m2 dose of Cy in terms of stem cell yield. However, mobilization failure and prolonged apheresis sessions were not issues with lower doses.
What is this summary about? This summary describes the results of a clinical study that compared two different doses of a treatment, called inotuzumab ozogamicin (inotuzumab for short), for acute lymphoblastic leukemia (ALL for short). This summary describes the results for people aged 18 years and older who took part in the study. Why was this study done? People who took part in the study had a higher risk of developing a side effect called sinusoidal obstruction syndrome (SOS for short), also known as veno-occlusive disease (VOD for short), which is a rare condition where some of the small blood vessels in the liver become blocked. Researchers wanted to find out if receiving a lower dose than the recommended dose of inotuzumab reduced the likelihood of people developing SOS after a stem cell transplant. Researchers wanted to find out if a lower dose of inotuzumab would also impact the efficacy (how well inotuzumab works to treat ALL) in people with ALL and what other side effects occurred.
Refractory/relapsed classical Hodgkin’s lymphoma (R/RcHL) poses significant treatment challenges, with limited success rates in achieving long-term remission. Brentuximab Vedotin (BV), an anti-CD30 monoclonal antibody-drug conjugate, has emerged as a promising therapeutic option. This study aims to evaluate the efficacy and safety of BV monotherapy in R/RcHL patients, particularly regarding survival outcomes and adverse events. This multi-center retrospective study included 82 R/RcHL patients aged 18 and over, treated with BV monotherapy across 14 institutions in Turkey from June 2012 to June 2020. Data on demographics, clinical characteristics, treatment response, adverse events, and overall survival (OS) rates were collected and analyzed. Primary outcomes were overall treatment response rate and OS, while the secondary outcome focused on the adverse event profile of BV treatment. Among the patients (56.1
Solitary plasmacytoma refers to a neoplastic, clonal proliferation of plasma cells forming a single mass. They are divided based on their origin site; solitary bone plasmacytomas originate from the bones, and extramedullary plasmacytomas represent extraosseous tumors. These are rare tumors but carry a risk of transforming to multiple myeloma; thus, optimal management and meticulous follow-up are needed. Their rarity poses a major challenge in conducting large-scale clinical trials, leaving important gaps in evidence regarding best practices. Newer imaging techniques have improved the quality of staging, management decisions, and outcomes. Radiation still has a significant role in treatment algorithms, and adjuvant chemotherapy is gaining more importance; trials are underway in this area. Follow-up should contain biochemical tests as the proposed response definition criteria. We aimed to review the key studies and guidelines in this paper.