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ABO discrepancies in plasma cell myeloma (PCM) present unique challenges in blood typing tests and transfusion management. We present the case of a 51-year-old male with PCM who exhibited discrepancies between forward and reverse blood grouping. Further investigation revealed that the patient's blood type was a variant of blood group B. While type III discrepancies, typically characterized by elevated globulin levels causing false-positive reactions in both forward and reverse blood grouping, are common in multiple myeloma, our case differed due to the loss of B antigens secondary to the malignant condition. This caused a discrepancy in forward blood grouping. The rarity of ABO discrepancies in multiple myeloma underscores the importance of thorough evaluation. Awareness of potential antigen alterations in such patients is crucial to ensure safe transfusion practices.

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BACKGROUND: Plasma cell myeloma (PCM) is characterized by hypercalcemia, renal impairment, anemia, and bone destruction. While pleural effusion, ascites, abdominal pain, and bloody stool are common manifestations of lung disease or gastrointestinal disorders, they are rarely observed in patients with PCM. CASE SUMMARY: A 66-year-old woman presented with complaints of recurrent chest tightness, wheezing, and abdominal bloating accompanied by bloody stools. Computed tomography revealed pleural effusion and ascites. Pleural effusion tests showed inflammation, but the T-cell spot test and carcinoembryonic antigen were negative. Endoscopy showed colonic mucosal edema with ulcer formation and local intestinal lumen stenosis. Echocardiography revealed enlarged atria and reduced left ventricular systolic function. The diagnosis remained unclear. Further testing revealed elevated blood light chain lambda and urine immunoglobulin levels. Blood immunofixation electrophoresis was positive for immunoglobulin G lambda type. Smear cytology of the bone marrow showed a high proportion of plasma cells, accounting for about 4.5%. Histopathological examination of the bone marrow suggested PCM. Flow cytometry showed abnormal plasma cells with strong expression of CD38, CD138, cLambda, CD28, CD200, and CD117. Fluorescence in situ hybridization gene testing of the bone marrow suggested 1q21 gene amplification, but cytogenetic testing showed no clonal abnormalities. Colonic mucosa and bone marrow biopsy tissues were negative for Highman Congo red staining. The patient was finally diagnosed with PCM. CONCLUSION: A diagnosis of PCM should be considered in older patients with pleural effusion, ascites, and multi-organ injury.

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The presence of an oval-shaped lesion in the liver is mainly associated with either primary liver cancer or metastatic disease from another malignancy. However, we present the case of a 62-year-old patient diagnosed with plasma cell myeloma, which reveals that these kinds of lesions can also be found during the course of this disease. Rarity and non-specificity make this a very challenging diagnosis for radiologists. It involves a special alert from the doctors taking care of the patient. Biopsy may sometimes be necessary to make a correct diagnosis. It is significant to ensure that the correct treatment is implemented and that the patient is not exposed to the unnecessary diagnosis of another neoplastic disease.

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Salvage autologous hematopoietic cell transplantation (auto-HCT) may be used to treat relapse of plasma cell myeloma occurring after previous auto-HCT. When an insufficient number of hematopoietic stem cells have been stored from the initial harvest, remobilization is necessary. Here, we aimed to analyze the efficacy and safety of different doses of cytarabine (total 800 vs. 1600 vs. 2400 mg/m2) for remobilization. Sixty-five patients, 55% male, with a median age at remobilization 63 years, were included. Remobilization was performed with cytarabine_800 in 7, cytarabine_1600 in 36, and cytarabine_2400 in 22 patients. Plerixafor rescue was used in 25% of patients receiving cytarabine_1600 and 27% of those receiving cytarabine_2400. Patients administered cytarabine_800 were not rescued with plerixafor. Remobilization was successful in 80% of patients (57% cytarabine_800; 86% cytarabine_1600; 77% cytarabine_2400; p = 0.199). The yield of collected CD34+ cells did not differ between the different cytarabine doses (p = 0.495). Patients receiving cytarabine_2400 were at the highest risk of developing severe cytopenias, requiring blood product support, or having blood-stream infections. One patient died of septic shock after cytarabine_2400. In summary, remobilization with cytarabine is feasible in most patients. All doses of cytarabine allow for successful remobilization. Cytarabine_2400 is associated with higher toxicity; therefore, lower doses (800 or 1600 mg/m2) seem to be preferable.

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PCAB (prednisone, cyclophosphamide, doxorubicin, carmustine) is a single-day regimen previously used for induction and now in relapsed/refractory multiple myeloma (RRMM). We retrospectively analysed the outcomes of 85 patients from five Australian centres. These included 30 patients (35.3%) who received PCAB with one additional agent (bortezomib most frequently). Median age of the patients was 65 years (37-80), with a median of four (1-8) prior lines of therapy. ORR was 37% (CR 4.9%). Median progression free survival and overall survival were 4.4 months (95% CI 3.5-6.7) and 7.4 months (95% CI 6.4-10.2), respectively. Extramedullary disease (EMD) was associated with shorter survival. Grade 3 or 4 cytopenia and febrile neutropenia occurred in 76.2% and 39.1%, respectively, with six (7.1%) treatment-related mortalities. Median inpatient stay was 3.3 days/28-day cycle (IQR 0.6-13), and for patients who died, a median of 20.2% of days alive were spent inpatient (IQR 6.4-39.1%). Three patients were successfully bridged to CAR T-cell therapy using PCAB, despite being penta-exposed and having EMD. PCAB may be considered as a useful salvage therapy amongst other polychemotherapy regimens in late relapse. Further studies is warranted to investigate and define its role as a bridging therapy to novel therapeutics.

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Multiple myeloma (MM) is an incurable malignant plasma cell neoplasm, representing the second most common hematopoietic cancer. As plasma cell neoplasms are clonal and often secrete a monoclonal protein (M-spike), laboratory diagnosis is usually straightforward, especially when ancillary studies such as immunohistochemistry, flow cytometry, and protein electrophoresis are available in addition to microscopic examination. Despite the repertoire of diagnostic tools, rare cases pose diagnostic dilemmas, especially when reagent antibodies do not react as expected, extent of disease is patchy, or when disease occurs in unique age groups. In this retrospective study, we report a series of challenging diagnostic cases, discussing aberrant findings and comparing them to more classic counterparts. Twelve cases collected during routine clinical sign-out were reanalyzed and include examples of MGUS, classic multiple myeloma, t(11; 14) rearranged myeloma, minimal residual disease, AA and AL amyloidosis, truncated light chain, non-secretory and non-producer myeloma, biphenotypic myeloma, oligoclonal expansion after bone marrow transplant, and plasma cell leukemia in a young adult. This cohort showcases the diversity of atypical presentations of plasma cell neoplasms, and we highlight standardized approaches to workup to avoid diagnostic pitfalls.

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Key Clinical Message: Plasma cell myeloma is a rare entity in the pediatric population. The peak incidence is in the seventh decade, with less than 2% of cases occurring in patients under the age of 40. It is worth noting that any destructive bony lesion in a child should be investigated. Abstract: Plasma cell myeloma (multiple myeloma) is the most common form of plasma cell neoplasm. It is a rare entity in young patients. The peak incidence is in the seventh decade, with less than 2% of cases occurring in patients under the age of 40. A male patient aged 9 years old with a progressive pain in lower back for about 1 month, aggravated by bending, associated with inability to stand upright, no any history of trauma. He complained about left pin-point chest pain, no any history of febrile illness. MRI showed a mass lesion of the L3 vertebra; CT scan revealed osteolytic lesions in the left T12, S2-sacral region, and left calvarium. Histology report of L3 lesion revealed cells with an eccentric nucleus, prominent Golgi apparatus and Flow cytometry revealed cells stained positive for CD 138 and CD56 and negative for CD45 expression. In situ hybridization identified k-light chain band restriction. Bone marrow evaluation was normal. A small serum monoclonal immunoglobin A spike of k-light chain type was noted. Other tests like complete blood count, lactate dehydrogenase levels, renal functional tests, and B2-microglobulin were normal. A diagnosis of plasma cell myeloma was made and the patient was started on emergent radiation to L3 lesion due to progressive neurological symptoms followed systemic therapy which resulted int reduction of L3 lesion. Plasma cell myeloma is extremely rare form of liquid tumor in the pediatric population, and it is important for any destructive bony lesion in a child to have appropriate work up.

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Aim This study aims to investigate demographic data, survival rates, and the relationship of these rates with surgery in a large case series including multiple myeloma (MM) patients. Method MM cases were analyzed retrospectively using the latest version of the SEER database published in April 2020. This version covers January 1975 to December 2017. Patients were classified according to gender, age, and race/ethnicity. Tumors were classified according to their localization, grade, year of diagnosis, and follow-up results. Results There were 60,239 patients diagnosed with Plasma Cell Myeloma. While 670 patients (1.2%) were operated on, 43,976 patients (76.7%) did not indicate operation, and 12,670 patients (22.1%) could not be operated on despite the recommendation. The mean survival was 62 months in those without an indication for surgery, and 42 months in patients with an indication but could not be operated on, and the difference was significant (p = 0.001). The mean survival was 58 months in the operated patients, and 42 months in the patients who could not be operated on despite the indication, and the difference was significant (p = 0.001). There was no difference between those who did not indicate surgery and those who were operated on with an indication (p = 0.243). Conclusion In multiple myeloma, the best prognosis is in the group of patients who received medical treatments without any indication for operation, while an indication for operation indicates a worse prognosis. A worse prognosis should be expected in patients who do not accept the operation or who cannot be operated on compared to the operated patients (AU)

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Solitary extramedullary plasmacytoma (SEP) is a collection of plasma cells in soft tissue tumors characterized by monoclonal plasma cells without systemic symptoms or evidence of bone disease. We present a case of SEP in a 49-year-old African American patient who presented with a slowly enlarging eyelid mass and underwent an excisional biopsy with ophthalmology before the diagnosis was confirmed by pathology in the absence of systemic symptoms or bone disease. Our review found only six confirmed cases of SEP of the eyelid described in the literature. In such cases, treatment is typically surgical excision or radiotherapy. Our patient was treated with radiation after the excision was incomplete. This case report adds another rare case of SEP of the eyelid to the literature.

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AIM: This study aims to investigate demographic data, survival rates, and the relationship of these rates with surgery in a large case series including multiple myeloma (MM) patients. METHOD: MM cases were analyzed retrospectively using the latest version of the SEER database published in April 2020. This version covers January 1975 to December 2017. Patients were classified according to gender, age, and race/ethnicity. Tumors were classified according to their localization, grade, year of diagnosis, and follow-up results. RESULTS: There were 60,239 patients diagnosed with Plasma Cell Myeloma. While 670 patients (1.2%) were operated on, 43,976 patients (76.7%) did not indicate operation, and 12,670 patients (22.1%) could not be operated on despite the recommendation. The mean survival was 62 months in those without an indication for surgery, and 42 months in patients with an indication but could not be operated on, and the difference was significant (p = 0.001). The mean survival was 58 months in the operated patients, and 42 months in the patients who could not be operated on despite the indication, and the difference was significant (p = 0.001). There was no difference between those who did not indicate surgery and those who were operated on with an indication (p = 0.243). CONCLUSION: In multiple myeloma, the best prognosis is in the group of patients who received medical treatments without any indication for operation, while an indication for operation indicates a worse prognosis. A worse prognosis should be expected in patients who do not accept the operation or who cannot be operated on compared to the operated patients.

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Although reports of familial clustering of hematologic malignancies have appeared for decades, the cause(s) of this uncommon occurrence is still not completely understood. Most modern investigations, however, support a genetic rather than an environmental exposure as a cause of this observation. Most pedigrees of families with familial hematologic malignancies demonstrate age of onset anticipation, with the disease diagnosed at an earlier age in successive generations. The cause of this phenomenon is clear in some familial neurologic disorders (trinucleotide repeat expansion) but not at all clear in familial hematologic malignancies. In preparation for molecular studies of familial clustering of hematologic malignancies, we have collected pedigrees on 738 families and have previously demonstrated anticipation in those with familial plasma cell myeloma, chronic lymphocytic leukemia, Hodgkin lymphoma or non-Hodgkin lymphoma (NHL). Here we present data on 36 families with both plasma cell myeloma and NHL in their pedigrees and demonstrate strong evidence for anticipation in these families. We encourage all health care personnel to ask patients multiple times about family medical history and carefully take note of family histories from individuals with uncommon illnesses and to refer families with clustering of such illnesses for further investigation.

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The review presents data from the literature on the role of Tumor necrosis factor-α (TNF-α) and ionizing radiation (IR) in the pathogenesis and treatment of plasma cell myeloma (PCM). There was analyzed disturbance of regulation of functioning of this cytokine, which affects the interaction of the immune system with substrate plasma cells under the influence of negative external factors, including ionizing radiation IR. Modern directions of therapy of this disease using the latest technologies are presented, in particular CAR T-cell therapy, which will allow to optimize in the future treatment of this disease and, thus, improve the quality and life expectancy of PCM patients.

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OBJECTIVE: identify the nature of anti-inflammatory and pro-inflammatory cytokine regulation in different periods of plasma cell myeloma (PCM) natural history with evaluation of its role as a prognostic criterion for the disease course in the Chornobyl NPP (ChNPP) accident survivors. MATERIALS AND METHODS: Levels of pro-inflammatory (IL-6, TNF-α) and anti-inflammatory (IL-10) cytokines both with their relationship were studied in the stage I-II and stage III PCM patients (n = 74) in different periods of the disease natural history i.e. remission/stabilization and progression. Study groups included the ChNPP accident survivors (n = 35) and non-irradiated subjects (n = 39). Immunoenzymatic method was applied using the Vector-Best CJSC commercial kits. RESULTS: There was a unidirectional increase in the levels of IL-6, TNF-α, and IL-10 in irradiated persons, and an elevation of IL-6 and TNF-α concentration but with a decreased level of IL-10 in non-irradiated subjects compared to control at the time of PCM diagnosis. Period of the disease remission/stabilization in PCM stage I-II patients featured a decrease in IL-6 concentration regardless of the exposure to ionizing radiation, while TNF-α content remained at the level of the control group. There was a significant increase in IL-6 concentration in both study groups during the disease relapse, while TNF-α level remained unchanged compared to stabilization phase of the disease. According to the obtained data a certain contribution of radiation exposure to the PCM pathogenesis as a possible predictor of the exacerbated disease course cannon be excluded. CONCLUSION: Determining the serum level of pro-inflammatory and anti-inflammatory cytokines (IL-6, TNF-α and IL-10 respectively) provides advancement in assessment of the PCM course and predict the effectiveness of administration of therapy protocols.

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Multiple myeloma (MM) is a multifactorial genetic disorder caused by interactive effects of environmental and genetic factors. The proper locus of the TP53 gene (17p13.1) and its protein is essential in genomic stability. The most common variant of the TP53 gene-p.P72R (rs1042522)-shows functional variation. The aim of our study was a complex analysis of the TP53 p.P72R variant and TP53 gene expression in relation to chromosomal changes of the TP53 gene locus, as well as MM risk and outcome. Genomic DNA from 129 newly diagnosed MM patients was analyzed by methods of automated DNA sequencing (for TP53 variant analysis) and cIg-FISH (for chromosomal aberrations analysis). RNA was used in real-time PCR to determine the TP53 expression. In MM patients, the TP53 variant was not in Hardy-Weinberg equilibrium. The RR genotype was associated with lower MM risk (OR = 0.44, p = 0.004). A higher number of plasma cells was found in patients with RR genotype in comparison to those with PP + PR genotypes (36.74% vs. 28.30%, p = 0.02). A higher expression of the TP53 gene was observed in PP + PR genotypes vs. RR homozygote (p < 0.001), in smokers vs. non-smokers (p = 0.02). A positive Pearson's correlation was found between the TP53 expression level and the number of plasma cells (r = 0.26, p = 0.04). The presence of chromosome 17 aberrations with or without TP53 locus did not affect the MM risk and outcome. Similar results were observed in the case of TP53 gene expression and the p.P72R variant.

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Objective: To review biochemical parameters, clinical characteristics, demographics, radiological and histopathological findings, treatment modalities and outcomes used to examine patients with coexisting multiple myeloma and prostate adencocarcinoma. METHODS: The systematic review comprised search on PubMed, Google Scholar, Science Direct and the Directory of Open Access Journal databases for case reports published till June 1, 2022. The search was done in line with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines using appropriate key words. Case reports included were those dealing exclusively with human subjects, were published in the English language and had free, full-text, public access. Quality assessment was done using Joanna Briggs Institute's Critical Appraisal Checklist for Case Reports. Data was extracted and the case reports were evaluated for demographic, diagnostic and treatment parameters. RESULTS: Of the 515 studies initially identified, 5(0.97%) were analysed; all males with mean age 68.6±10.78 years. The most common symptom reported at presentation was low back pain 3(60%), Osteolytic lesions were seen in 4(80%) patients on imaging with elevated prostate surface antigen levels. Anaemia was found in 3(60%) patients and 2(40%) had thrombocytopenia. Conclusion: Multiple myeloma and prostate adenocarcinoma can coexist although it is rare. Awareness regarding the possible coexistence of the two prominent cancer types may further help clinicians during their practice in considering multiple myeloma as a differential diagnosis when encountered with patients having osteolytic bony lesions along with elevated levels of prostate-specific antigen. PROSPERO Registration Number: CRD42022334906.

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INTRODUCTION: The majority of patients with advanced neoplasms have religious/spiritual needs, and for most of them religion and/or spirituality is important. The psychology of religion and spirituality is one the forms of support offered to patients with advanced cancer. R/S are factors which impact health-related quality of life (HRQoL). The aim of this paper was to assess the influence of R/S on the HRQoL of patients diagnosed with MM. MATERIALS AND METHODS: The patients filled out anonymous questionnaires about R/S and the HRQoL scale. The clinical data were collected from medical records. RESULTS: The study sample consisted of 83 patients with MM (51.8% women), with a mean age of 64.9 years. The leading denomination among the respondents was Catholic (N = 83, 100%): 36% described themselves as deep believers (N = 30), 53% as believers (N = 44), and 11% as nonpracticing believers (N = 9). Most patients were receiving ongoing treatment (59.8%), while 40.2% were in remission from the disease. Patients in remission declared a significantly higher interest in R/S issues than patients in active treatment and had a higher rate of intrapsychic R/S struggles dominated by anxiety and guilt. A moderate negative correlation between interest in R/S issues and unfavorable assessment of physical functioning and role functioning was observed. Anger towards God positively correlated with a negative assessment of emotional functioning. CONCLUSIONS: The findings highlight the importance of R/S for the HRQoL of MM patients and show that their QoL depends on the types of R/S coping used.

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Amyloidosis is a term describing the extracellular deposit of fibrils composed of subunits of several different normal serum proteins in various tissues. Amyloid light chain (AL) amyloidosis contains fibrils that are composed of fragments of monoclonal light chains. Many different disorders and conditions can lead to spontaneous splenic rupture, including AL amyloidosis. We present a case of a 64-year-old woman with spontaneous splenic rupture and hemorrhage. A final diagnosis of systemic amyloidosis secondary to plasma cell myeloma was made with infiltrative cardiomyopathy and possible diastolic congestive heart failure exacerbation. We also provide a narrative review of all documented cases of splenic rupture associated with amyloidosis from the year 2000 until January 2023, along with the main clinical findings and management strategies.

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BACKGROUND: Mutations in the RAS-MAPK pathway, such as KRAS, NRAS, and BRAF, are known as high-risk factors associated with poor prognosis in patients with various cancers, but studies in myeloma have yielded mixed results. METHODS: We describe the clinicopathologic, cytogenetic, molecular features, and outcomes of 68 patients with RAS/BRAF-mutated myeloma, and compare with 79 patients without any mutations. RESULTS: We show that KRAS, NRAS, and BRAF were mutated in 16%, 11%, and 5% of cases, respectively. RAS/BRAF-mutated patients had lower hemoglobin and platelet counts, higher levels of serum lactate dehydrogenase and calcium, higher percentage of bone marrow plasma cells, and more advanced R-ISS stage. RAS/BRAF mutations were associated with complex karyotype and gain/amplification of CKS1B. The median overall survival and progression-free survival were significantly shorter for RAS/BRAF-mutated patients (69.0 vs. 220.7 months, p = 0.0023 and 46.0 vs. 60.6 months, p = 0.0311, respectively). Univariate analysis revealed that KRAS mutation, NRAS mutation, lower hemoglobin, elevated lactate dehydrogenase, higher R-ISS stage, complex karyotype, gain/amplification of CKS1B, monosomy 13/RB1 deletion and lack of autologous stem cell transplantation were associated with poorer prognosis. Multivariate analysis showed that KRAS mutation, lower hemoglobin level, higher level of serum calcium, higher ISS stage, and lack of autologous stem cell transplantation predict inferior outcome. CONCLUSIONS: RAS/BRAF mutations occur in 30%-40% of myeloma cases and are associated with higher tumor burden, higher R-ISS stage, complex karyotype, and shorter overall survival and progression-free survival. These findings support testing for RAS/BRAF mutations in myeloma patients and underscore the potential therapeutic benefits of RAS/BRAF inhibitors.

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