Plasmacytoma Research Articles

Drug Expenditure, Price, and Utilization in US Medicaid: A Trend Analysis for New Multiple Myeloma Medications from 2016 to 2022.

Multiple myeloma (MM) is the most common plasma cell tumor type. In late 2015, the FDA approved three new medications for MM. These medications were ixazomib, daratumumab, and elotuzumab. However, their utilization, reimbursement, and price in the Medicaid program have not been analyzed before. A retrospective drug utilization study using the national Medicaid pharmacy claims data from 2016 to 2022 in the US. The primary metrics of analysis were utilization (number of prescriptions), reimbursement (total spending), and price (reimbursement per prescription). The overall Medicaid utilization of MM medications increased from 1671 prescriptions in 2016 to 34,583 prescriptions in 2022 (1970% increase). Moreover, the overall Medicaid reimbursement for the new MM medications increased from USD 9,250,000 in 2016 to over USD 214,449,000 in 2022 (2218% increase). Daratumumab had much higher utilization, reimbursement, and market shares than its competitors. Ixazomib was the most expensive medication compared to daratumumab and elotuzumab. The results of this study demonstrate that CMS utilization and spending on MM medications have significantly grown since 2016. Daratumumab has by far the highest utilization, spending, and market share. The utilization of and spending on specific pharmaceuticals are clearly impacted by policy and clinical guideline recommendations.

PB2575: HIGH INCIDENCE OF RESPIRATORY TRACT INFECTIONS FOLLOWING COVID-19 WAVES IN PATIENTS WITH PLASMA CELL DYSCRASIAS

PB2575: HIGH INCIDENCE OF RESPIRATORY TRACT INFECTIONS FOLLOWING COVID-19 WAVES IN PATIENTS WITH PLASMA CELL DYSCRASIAS

MGUS'ta azalmış GADD45A gen ifadesi seviyesi

Purpose: Monoclonal gammopathy of undetermined significance (MGUS) is a plasma cell dyscrasia. It is known that MGUS has an increased risk of progression to multiple myeloma (MM), and also prepares the ground for diseases such as Waldenstrom macroglobulinemia (WM), non-Hodgkin lymphoma, and chronic lymphocytic leukemia (CLL). Our study aimed to evaluate whether some important p53 pathway genes differ in terms of expression in MGUS and healthy individuals.
 Materials and methods: For the study, bone marrow was collected from 8 healthy individuals and 8 individuals diagnosed with MGUS, and RNA samples were isolated. Expression levels of various genes involved in the p53 pathway were compared using the RT2-profiler PCR array. β-actin housekeeping gene expression level was used for normalization. The Pearson Correlation analysis and Receiver Operating Characteristic (ROC) analysis were conducted.
 Results: Among the genes whose expression levels were examined in this study, it was determined that the expression level of only the GADD45A gene decreased significantly in MGUS compared to the control group (p=0.027). Pearson correlation data showed that GADD45A gene expression was highly correlated with 12 of the other genes (APAF1, CDK4, PCNA, BAX, CDKN2A, CASP9, CHECK2, MDM2, RB1, P53, BCL2, CHEK1) examined in the p53 pathway (r>0.7). In addition, according to the ROC analysis, GADD45A is detected to have a strong discrimination power between MGUS and healthy individuals (AUC=0.797 and p=0.015).
 Conclusion: The decreased expression of the GADD45A gene in the MGUS group compared to the control group may be useful as a new biomarker to detect the risk of progression from MGUS to MM.

POEMS Syndrome-Clinical Picture and Management. Current Knowledge.

POEMS syndrome is a rare form of plasma cell dyscrasia. Difficulties arise already at the stage of making the diagnosis (complex and heterogeneous clinical picture) and continue during the course of treatment (lack of guidelines for therapy, data coming mainly from reports and short series of patients). In this article we review the current state of knowledge on POEMS syndrome diagnostics, clinical characteristics, prognosis, reported treatment outcomes and the emergence of the new therapeutic strategies.

The devolution of a mature plasma cell dyscrasia into a fatal plasmablastic lymphoma

Introduction: Plasmablastic lymphoma is a rare, aggressive, non-Hodgkin’s lymphoma with an untreated prognosis as poor as three months. There exists scant literature describing transformation of plasmablastic lymphoma from a more benign dyscrasia, the mature plasmacytoma. This case report describes the transformation of plasmablastic lymphoma from a mature plasma cell neoplasm/plasma cell myeloma in an atypical combination of patient characteristics. Case Report: A 66-year-old man presented with acute onset right lower extremity pain and rapidly progressive mobility loss. He was found to have a lytic lesion in the lateral right iliac wing. Biopsy revealed the lesion to be plasmablastic lymphoma with Epstein–Barr virus (EBV) positivity by in situ hybridization with a Ki-67 proliferation index >99%, and strongly staining CD138 and MUM-1. CD20 and PAX-5 were negative. A bone marrow biopsy from the right iliac crest showed mature plasma cells without evidence of plasmablastic lymphoma cytology found in the initial specimen. These specimens showed CD138 positivity with 15–20% plasma cells with Kappa positive clonality by in situ hybridization, and diffusely Epstein–Barr virus negative by in situ hybridization. Further plasma cell fluorescence in situ hybridization study showed a clone with a TP53 deletion and an immunoglobulin heavy chain gene rearrangement that did not translocate to one of the common plasma cell dyscrasia translocation partners (FGFR3, CCND1, MAF, or MAFB). Additionally, a near-tetraploid subclone was observed in approximately 60% of nuclei. Also, there was gain of BCL2 gene or chromosome 18/18q, gain of BCL6 gene or chromosome 3/3q and MYC amplification. There was no MYC and BCL2 and/or BCL6 rearrangements. Our patient was neither HIV-positive nor immunocompromised, rather Epstein–Barr virus positive with a quantitative polymerase chain reaction level greater than 67,000. He was started on Daratumumab combined with etoposide, vincristine, doxorubicin, cyclophosphamide, and prednisone. Conclusion: This case exhibits a unique presentation of plasmablastic lymphoma in terms of disease presentation, unique risk factors, including HIV-negativity and male-assigned sex, and the creativity of treatment utilized.

Expression of β2 microglobulin on malignant multiple myeloma plasma cells

Background. β2‑microglobulin is a low molecular weight glycoprotein that is part of the major histocompatibility complex. The concentration of β2‑microglobulin in the blood serum of patients increases with various oncohematological pathologies, including multiple myeloma. One of the methods for detecting membrane β2‑microglobulin is flow cytometry. This article discusses the expression of β2‑microglobulin and key markers on the membrane of malignant cells in patients with multiple myeloma at the onset of the disease.Aim. To study the expression of β2‑microglobulin and markers CD45, CD56 and CD19 on the membrane of tumor plasma cells in the primary diagnosis of multiple myeloma.Materials and methods. The study of β2‑microglobulin and CD45, CD56 and CD19 was carried out in 37 patients with multiple myeloma by 8‑color flow cytometry using a panel of monoclonal antibodies for the diagnosis of plasma cell tumors (EuroFlow, 2012).Results. The expression of β2‑microglobulin in combination with markers CD45, CD56 and CD19 on tumor plasma cells was assessed, illustrations of the expression of these markers are presented. Out of 37 patients with multiple myeloma, hyperexpression of β2‑microglobulin was noted in 23 (62.2 %), in other cases there were normal or reduced levels of this protein in other cases.Conclusion. Tumor plasma cells overexpress β2‑microglobulin in a significant percentage of cases (62.2 %). This allows further assessment of the clinical significance of such aberrant expression and its relationship with serum levels of β2‑microglobulin.

IL6Myc mouse is an immunocompetent model for the development of aggressive multiple myeloma.

Multiple Myeloma (MM) is a plasma cell neoplasm originating in the bone marrow and is the second most common blood cancer in the United States. One challenge in understanding the pathogenesis of MM and improving treatment is a lack of immunocompetent mouse models. We previously developed the IL6Myc mouse that generates plasmacytomas at 100% penetrance that phenotypically resemble aggressive MM. Using comprehensive genomic analysis, we found that the IL6Myc tumors resemble aggressive MM by RNA and protein expression. We also found that IL6Myc tumors accumulated fusions and missense mutations in genes that overlap significantly with human myeloma, indicating that the mouse is good model for studying disease etiology. Lastly, we derived cell lines from IL6Myc tumors that express cell surface markers typical of MM and readily engraft into mice, home to the bone marrow, and induce osteolytic disease. The cell lines may be useful in developing immunotherapies directed against BAFF-R and TACI, though not BCMA, and may also be a good model for studying dexamethasone resistance. These data indicate that the IL6Myc model is useful for studying development of aggressive MM and for developing new treatments against such forms of the disease.

Characteristics and outcomes of COVID-19 in patients with plasma cell dyscrasias during the first Omicron wave in Beijing since December 2022: a retrospective study at a National Clinical Research Center for Hematologic Disease.

The wave of coronavirus disease 2019 (COVID-19) with Omicron variant reached its first peak in Beijing, China in December 2022. We delineated characteristics and factors associated with adverse outcome of patients with plasma cell dyscrasias (PCDs) and COVID-19 during the first month of the wave. A total of 104 patients with a median age of 65 years were included in the study, with multiple myeloma (74%, n=77) and primary Immunoglobulin light chain amyloidosis (16.3%, n=17) being the two most common disease. Overall, severe or critical COVID-19 was developed in 18 (17.3%) patients, with a total all-cause mortality rate of 4.8% (n=5). The vaccination coverage was 41% and 48.1%, before and during the upsurge of Omicron, respectively, calling for the improvement of vaccination in PCD patients. Multivariable analysis revealed that age was the only independent risk factors (OR=1.14, 95% CI: 1.06-1.26, p = 0.002) associated with the development of severe or critical disease. Among patients with severe or critical group, low levels of albumin (HR=18.29; 95% CI: 1.82-183.44, p = 0.013) and high levels of lactic dehydrogenase (LDH) (HR=0.08; 95% CI: 0.01-0.65, p = 0.018) were associated with longer time to negative conversion of COVID-19.

Zinc Oxide Nanoparticles Trigger Autophagy in the Human Multiple Myeloma Cell Line RPMI8226: an In Vitro Study.

Multiple myeloma (MM) is a malignant clonal proliferative plasma cell tumor. Zinc oxide nanoparticles (ZnO NPs) are used for antibacterial and antitumor applications in the biomedical field. This study investigated the autophagy-induced effects of ZnO NPs on the MM cell line RPMI8226 and the underlying mechanism. After RPMI8226 cells were exposed to various concentrations of ZnO NPs, the cell survival rate, morphological changes, lactate dehydrogenase (LDH) levels, cell cycle arrest, and autophagic vacuoles were monitored. Moreover, we investigated the expression of Beclin 1 (Becn1), autophagy-related gene 5 (Atg5), and Atg12 at the mRNA and protein levels, as well as the level of light chain 3 (LC3). The results showed that ZnO NPs could effectively inhibit the proliferation and promote the death of RPMI8226 cells in vitro in a dose- and time-dependent manner. ZnO NPs increased LDH levels, enhanced monodansylcadaverine (MDC) fluorescence intensity, and induced cell cycle arrest at the G2/M phases in RPMI8226 cells. Moreover, ZnO NPs significantly increased the expression of Becn1, Atg5, and Atg12 at the mRNA and protein levels and stimulated the production of LC3. We further validated the results using the autophagy inhibitor 3-methyladenine (3‑MA). Overall, we observed that ZnO NPs can trigger autophagy signaling in RPMI8226 cells, which may be a potential therapeutic approach for MM.

Solitary Extramedullary Plasmacytoma of Nasal Cavity.

Solitary extramedullary plasmacytoma (SEP) of the nasal cavity is a rare neoplastic condition characterized by the localized proliferation of abnormal plasma cells. We present a case of SEP involving the nasal cavity in a 40-year-old male patient who presented with nasal obstruction and recurrent epistaxis. The diagnosis was confirmed through clinical evaluation, imaging studies, and histopathological examination of excised specimen. The patient underwent trans-nasal endoscopic excision of nasal mass without any adjuvant therapy, which resulted in successful local control. This case report highlights the clinical presentation, diagnostic approach, treatment modalities, and favourable prognosis associated with solitary extramedullary plasmacytoma of the nasal cavity.

Pathological significance of plasma cell infiltration in diagnosing lymph node diseases

Objective: To investigate the value of plasma cells for diagnosing lymph node diseases. Methods: Common lymphadenopathy (except plasma cell neoplasms) diagnosed from September 2012 to August 2022 were selected from the pathological records of Changhai Hospital, Shanghai, China. Morphological and immunohistochemical features were analyzed to examine the infiltration pattern, clonality, and IgG and IgG4 expression of plasma cells in these lymphadenopathies, and to summarize the differential diagnoses of plasma cell infiltration in common lymphadenopathies. Results: A total of 236 cases of lymphadenopathies with various degrees of plasma cell infiltration were included in the study. There were 58 cases of Castleman's disease, 55 cases of IgG4-related lymphadenopathy, 14 cases of syphilitic lymphadenitis, 2 cases of rheumatoid lymphadenitis, 18 cases of Rosai-Dorfman disease, 23 cases of Kimura's disease, 13 cases of dermal lymphadenitis and 53 cases of angioimmunoblastic T-cell lymphoma (AITL). The main features of these lymphadenopathies were lymph node enlargement with various degrees of plasm cell infiltration. A panel of immunohistochemical antibodies were used to examine the distribution of plasma cells and the expression of IgG and IgG4. The presence of lymph node architecture could help determine benign and malignant lesions. The preliminary classification of these lymphadenopathies was based on the infiltration features of plasma cells. The evaluation of IgG and IgG4 as a routine means could exclude the lymph nodes involvement of IgG4-related dieases (IgG4-RD), and whether it was accompanied by autoimmune diseases or multiple-organ diseases, which were of critical evidence for the differential diagnosis. For common lesions of lymphadenopathies, such as Castleman's disease, Kimura's disease, Rosai-Dorfman's disease and dermal lymphadenitis, the expression ratio of IgG4/IgG (>40%) as detected using immunhistochemistry and serum IgG4 levels should be considered as a standard for the possibility of IgG4-RD. The differential diagnosis of multicentric Castleman's diseases and IgG4-RD should be also considered. Conclusions: Infiltration of plasma cells and IgG4-positive plasma cells may be detected in some types of lymphadenopathies and lymphomas in clinicopathological daily practice, but not all of them are related to IgG4-RD. It should be emphasized that the characteristics of plasma cell infiltration and the ratio of IgG4/IgG (>40%) should be considered for further differential diagnosis and avoiding misclassification of lymphadenopathies.

Features of diagnostics and treatment of various plasmacytoma options

Aim. Demonstration of clinical cases of plasmacytoma, both solitary and one of the manifestations of multiple myeloma. Materials and methods. A brief literature review on the diagnosis of bone and extramedullary plasmacytoma and clinical observations of various tumor options from the personal practice of the authors is presented. Results. Two clinical observations of the primary diagnosis of solitary plasmacytoma are presented. In one case, there was a bone tumor, in the other − an extramedullary tumor. In both observations, a generalization of the process was subsequently noted. Three clinical cases of primary diagnosis of multiple myeloma accompanied by the development of plasmacytoma are also presented. In two cases, there was a lesion of the bone marrow, in one – a multiple-focal form of the disease. In two situations, a non-secreting variant of multiple myeloma was diagnosed. The features of diagnosis and treatment were analyzed. Conclusion. In the diagnosis of focal forms of plasmacytoma (without lesions to the bone marrow and secretion), the leading role belongs to histological and immunohistochemical studies.

Plasma Cell Neoplasm with Clear Cell Morphology—A Diagnostic Dilemma

Plasma Cell Neoplasm with Clear Cell Morphology—A Diagnostic Dilemma

Anaplastic Lymphoma Kinase Positive Large B-Cell Lymphoma Mimicking as Plasma Cell Neoplasm: A Rare Case Report

Anaplastic lymphoma kinase positive (ALK+ve) large B-cell lymphoma (LBCL) is a rare, aggressive subtype of diffuse LBCL with unique morphological characteristics and unusual immunophenotypic features that have substantial overlap with other hematologic and nonhematologic neoplasms. Here, we present a 48-year-old male who presented with progressive swelling on both sides of the neck and left eye followed by protruding mass in the left nose over a span of 6 months. Based on the biopsy findings of the left nasal mass, a plasma cell neoplasm was initially diagnosed; later on the diagnosis was modified as ALK + ve LBCL based on the aggressive clinical course and biopsy findings of cervical lymph node. The patient was started on chemotherapy and is tolerating well.

Trends in risk for therapy-related myelodysplastic syndrome/acute myeloid leukemia after initial chemo/immunotherapy for common and rare lymphoid neoplasms, 2000-2018.

Historically, survivors of common lymphoid neoplasms (LNs) had increased risks for therapy-related myelodysplastic syndrome/acute myeloid leukemia (tMDS/AML). Despite major treatment advances in the treatment of LNs over the last two decades, a comprehensive evaluation of tMDS/AML trends following both common and rare LNs treated in this contemporary period is lacking. In US cancer registries during 2000-2018, we identified 1496 tMDS/AML cases among 186,503 adults who were treated with initial chemo/immunotherapy for first primary LN and survived ≥1 year. We quantified tMDS/AML standardized incidence ratios (SIRs), excess absolute risks (EARs, per 10,000 person-years), and cumulative incidence. The highest tMDS/AML risks occurred after precursor leukemia/lymphoma (SIR=39, EAR=30), Burkitt leukemia/lymphoma (SIR=20, EAR=24), peripheral T-cell lymphoma (SIR=12, EAR=23), chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL; SIR=9.0, EAR=27), and mantle cell lymphoma (SIR=8.5, EAR=25). Elevated risks (SIRs=4.2-6.9, EARs=4.9-15) also were observed after all other LN subtypes except hairy cell leukemia and mycosis fungoides/Sézary syndrome. Among patients treated more recently, tMDS/AML risks were significantly higher after CLL/SLL (SIR2000-2005=4.8, SIR2012-2017=10, Ptrend=0.0043), significantly lower after Hodgkin (SIR2000-2005=15, SIR2012-2017=6.3, Ptrend=0.024) and marginal zone (SIR2000-2005=7.5, SIR2012-2017=2.3, Ptrend=0.015) lymphomas, and non-significantly lower after mantle cell lymphoma (SIR2000-2005=10, SIR2012-2017=3.2, Ptrend=0.054), lymphoplasmacytic lymphoma/Waldenström macroglobulinemia (SIR2000-2005=6.9, SIR2012-2017=1.0, Ptrend=0.067), and plasma cell neoplasms (SIR2000-2005=5.4, SIR2012-2017=3.1, Ptrend=0.051). EAR and cumulative incidence trends generally were similar to SIR trends. Median survival after tMDS/AML was 8.0 months (interquartile range, 3.0-22.0). Although tMDS/AML risks are significantly elevated after initial chemo/immunotherapy for most LNs, patients treated more recently have lower tMDS/AML risks, except after CLL/SLL. Though rare, the poor prognosis following tMDS/AML emphasizes the importance of continued efforts to reduce treatment-associated toxicity. This research was supported in part by the Intramural Research Program of the National Cancer Institute, National Institutes of Health. LMM, GMD, REC, and CBS verified the data, and all authors had access to the data and made the decision to submit for publication.

Extramedullary plasmacytoma presenting as a rapidly growing lateral neck mass: A case report

Introduction: We detail the rare clinical presentation of an extramedullary plasmacytoma as a rapidly growing lateral neck mass. The pathology and its management are discussed. A rapidly growing extramedullary plasmacytoma infiltrating the cervical spine. Case Report: A 53-year-old male presented to our emergency room with a one-month history of worsening left-sided neck pain and an enlarging lateral neck mass. Evaluation with cervical imaging and laboratory analysis were performed. The patient underwent a soft tissue biopsy with subsequent spinal cord decompression and an occipital-cervical fusion. Pathology revealed the diagnosis of plasmacytoma. The patient was referred to oncology and radiation therapy for adjuvant treatment. Conclusion: This case represents the unusual presentation of a rapidly growing extramedullary plasmacytoma to the soft tissues of the neck with subsequent erosion of the base of the skull and upper cervical vertebral spine. It was treated effectively with posterior cervical decompression, arthrodesis, and adjuvant radiation and chemotherapy.

BI18 The role of an acute on-call dermatology service in a haemato-oncology unit using pioneering treatments: a retrospective analysis of 134 dermatology consultations in a tertiary hospital

Abstract Our dermatology department receives the largest number of internal on-call referrals from the haematology–oncology department. Patients with haematological malignancies are particularly vulnerable to an array of dermatological conditions due to immunosuppression and multiagent exposure, which can impact cancer treatment and result in significant morbidity and even mortality. Our primary objective was to quantify and analyse the dermatological conditions encountered. Our secondary objective was to identify potential associations of dermatological conditions with haematological malignancy subtype and anticancer therapies. We conducted a retrospective single-centre review of acute haematology–oncology referrals to an on-call dermatology service made between August 2020 and November 2022. Consultations were retrospectively identified from the dermatology on-call referral log. In total, 134 patients were identified. Underlying haematological malignancies were categorized into lymphoid neoplasms (45%), myeloid neoplasms (29%), plasma cell dyscrasias (13%), Hodgkin lymphoma (6%) and other (7%), with acute myeloid leukaemia (AML; 20%) being the most frequently seen distinct haematological malignancy. Cutaneous adverse drug eruptions (23%), infections (13%), folliculitis/acneiform eruptions (10%) and eczematous dermatoses (7%) accounted for the most common diagnoses, followed by benign skin lesions (6%), cutaneous lymphoma (6%), leukaemia cutis (5%) and cutaneous vasculitis (4%). Notably, cutaneous drug reactions frequently occurred in patients with myeloid neoplasms (32%) and, out of all malignancies, most frequently in multiple myeloma (26%). Folliculitis and acneiform eruptions occurred predominantly in patients with myeloid lineage malignancy (i.e. AML and myelodysplastic syndrome; 73%), although it is unclear whether they were triggered by the underlying malignancy or treatment received. Fifteen patients (11.2%) undergoing novel chimeric antigen receptor (CAR) T-cell therapy were referred to our on-call dermatology service. In conclusion, dermatology plays an integral consultative service to patients from haematology–oncology. Skin biopsy is diagnostically useful and has been crucial in the early detection of lymphoma and leukaemia recurrence. However, with the recent development of new CAR T-cell therapies and other novel agents, new adverse dermatological complications are surfacing, and this is likely to increase the demand for dermatology input.

Targeting transcriptional kinase of CDK7 halts proliferation of multiple myeloma cells by modulating the function of canonical NF-kB pathway and cell cycle regulatory proteins

Multiple myeloma (MM) is an incurable plasma cell neoplasm. Despite several effective frontline therapeutic regimens, including Bortezomib (BTZ), relapse is almost inevitable; therefore, better therapeutic modalities to improve the outcomes are needed. Cyclin-dependent kinases (CDKs) are an essential constituent of the cellular transcriptional machinery and tumors including MM are critically dependent on transcription to maintain their oncogenic state. In the present study, we explored the efficacy of THZ1, a covalent CDK7 inhibitor in MM treatment using Bortezomib resistant (H929BTZR) cells and zebrafish xenografts. THZ1 showed anti-myeloma activity in the models of MM but had no effect on healthy CD34+ cells. THZ1 suppresses phosphorylation of carboxy-terminal domain of RNA polymerase II and downregulates the transcription of BCL2 family of proteins both in H929BTZS and H929BTZR cells leading to G1/S arrest and apoptosis. THZ1 mediates inhibition of bone marrow stromal cells-induced proliferation and activation of NF-kB signaling. The data derived from zebrafish xenografts of MM demonstrate that THZ1 combined with BTZ synergistically reduces tumor growth in zebrafish embryos. Collectively, our results reveal that THZ1 alone as well as in combination with BTZ has effective anti-myeloma activity.

Primary Cranio-Orbital Presentation of Multiple Myeloma.

A 49-year-old man presented with left upper eyelid ptosis noted after minor head trauma. Clinical examination revealed a soft, mildly tender mass of the superior orbit with left hypoglobus and relative proptosis. He had moderate limitation in supraduction, without visual or pupillary deficit. MRI imaging showed a superior extraconal cystic mass (Fig. 1C, D), and CT demonstrated erosion of the superior orbital roof and invasion of the frontal sinus (Fig. 1A, B).FIG. 1Excisional biopsy was performed via a bifrontal craniotomy with a frontal bone flap, exposing a well-encapsulated vascular lesion eroding through the orbital roof and frontal sinus (Fig. 2A). The mass was dissected from the surrounding dura, sinus mucosa, and periorbita, and removed in toto (Fig. 2B). The frontal sinus was obliterated and a split-thickness calvarial bone graft was used to reconstruct the orbital rim and roof, secured with mini plate fixation (Fig. 2C).FIG. 2Pathological analysis revealed plasma cell neoplasm (Fig. 2D) with clonal kappa-light proliferation (Fig. 2E), consistent with an extramedullary plasmacytoma. The full-body PET scan revealed multiple lytic lesions of the clavicle, rib, and vertebrae, conferring a diagnosis of multiple myeloma. He underwent an autologous stem cell transplant and started maintenance Bortezomib therapy with a good serologic response. Multiple myeloma accounts for 10% of hematologic malignancies and presents as a malignant plasma cell proliferation leading to bone marrow destruction. There are few reports of multiple myeloma initially presenting in the orbit. Solitary plasmacytoma without systemic disease may be treated with radiation therapy. Systemic disease mandates oncologic evaluation and consideration of chemotherapy or transplant.

POEMS syndrome misdiagnosed as CIDP: A case report

POEMS syndrome misdiagnosed as CIDP: A case report