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Clinical Case Reports Mar 2023Cardiac bradyarrhythmia and conduction disorder may be rare, but recurrent adverse events caused by bortezomib. Here we report a case with POEMS syndrome presenting...
Cardiac bradyarrhythmia and conduction disorder may be rare, but recurrent adverse events caused by bortezomib. Here we report a case with POEMS syndrome presenting severe heart block after bortezomib plus dexamethasone therapy. After permanent pacemaker implantation, bortezomib was restarted and maintained, resulting in sustained complete response for POEMS syndrome.
PubMed: 36873076
DOI: 10.1002/ccr3.7004 -
Zhongguo Yi Xue Ke Xue Yuan Xue Bao.... Feb 2023POEMS syndrome is a rare disease caused by monoclonal plasma cell proliferative disorder.The typical signs include peripheral neuropathy,organ enlargement,endocrine...
POEMS syndrome is a rare disease caused by monoclonal plasma cell proliferative disorder.The typical signs include peripheral neuropathy,organ enlargement,endocrine disease,M proteinemia,and skin changes.In clinical practice,the atypical,complex,and changeable clinical manifestations of this syndrome can easily lead to misdiagnosis and missed diagnosis.A case of POEMS syndrome with peripheral edema and ascites as the main manifestations is reported in this paper.
Topics: Humans; Ascites; POEMS Syndrome; Edema; Skin
PubMed: 36861172
DOI: 10.3881/j.issn.1000-503X.14697 -
Oncology Letters Mar 2023Due to its unique clinical, immunological and molecular genetic characteristics, biclonal lymphoplasmacytic lymphoma/Waldenström macroglobulinemia (LPL/WM) with...
Due to its unique clinical, immunological and molecular genetic characteristics, biclonal lymphoplasmacytic lymphoma/Waldenström macroglobulinemia (LPL/WM) with polyneuropathy, organomegaly, endocrinopathy, monoclonal protein and skin changes (POEMS) syndrome is extremely rare in clinical practice, and there is no standard treatment for patients afflicted with this condition. In the present case report, a rare case of double LPL/WM with POEMS syndrome is described. The patient, a 65-year-old male, exhibited significant renal impairment and polylymphadenopathy. The patient was treated with rituximab and his symptoms were resolved following two courses of treatment. A review of the literature was performed, comparing the present case with previous cases. It is hoped that this case report will enable clinicians to gain a better understanding of this disease.
PubMed: 36817054
DOI: 10.3892/ol.2023.13683 -
Journal of Community Hospital Internal... 2022Polyneuropathy, Organomegaly, Endocrinopathy, M-protein, Skin changes (POEMS) syndrome is a rare disorder with multiple presentations and a constellation of symptoms. We...
Polyneuropathy, Organomegaly, Endocrinopathy, M-protein, Skin changes (POEMS) syndrome is a rare disorder with multiple presentations and a constellation of symptoms. We present a 62 year-old female who presented to the Emergency Department for acute dyspnea. Chest Xray showed sclerotic lesions in the ribs and thoracic spine. Further imaging studies with computed tomography (CT) and positron emission tomography (PET) scans were suggestive of a benign process. Improvement was seen with supportive management. A few months later, patient developed neurological symptoms with reduced exercise tolerance. Mixed demyelinating and axonal polyneuropathy was diagnosed by electromyography. Further work up with bone marrow biopsy and immunochemistry testing revealed lambda and kappa plasma cell disorder, with elevated vascular endothelial growth factor (VEGF). Patient was diagnosed with POEMS and initiated on chemotherapy. POEMS syndrome is commonly missed due to its rarity and varied clinical presentations. VEGF plays a crucial role in the diagnosis. Management requires a multidisciplinary approach.
PubMed: 36816171
DOI: 10.55729/2000-9666.1121 -
European Journal of Ophthalmology Sep 2023Plasma cell dyscrasias are a wide range of severe monoclonal gammopathies caused by pre-malignant or malignant plasma cells that over-secrete an abnormal monoclonal... (Review)
Review
Plasma cell dyscrasias are a wide range of severe monoclonal gammopathies caused by pre-malignant or malignant plasma cells that over-secrete an abnormal monoclonal antibody. These disorders are associated with various systemic findings, including ophthalmological disorders. A search of PubMed, EMBASE, Scopus and Cochrane databases was performed in March 2021 to examine evidence pertaining to ocular complications in patients diagnosed with plasma cell dyscrasias. This review outlines the ocular complications associated with smoldering multiple myeloma and monoclonal gammopathy of undetermined significance, plasmacytomas, multiple myeloma, Waldenström's macroglobulinemia, systemic amyloidosis, Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal gammopathy and Skin changes (POEMS) syndrome, and cryoglobulinemia. Although, the pathological mechanisms are not completely elucidated yet, wide-ranging ocular presentations have been identified over the years, evolving both the anterior and posterior segments of the eye. Moreover, the presenting symptoms also help in early diagnosis in asymptomatic patients. Therefore, it is imperative for the treating ophthalmologist and oncologist to maintain a high clinical suspicion for identifying the ophthalmological signs and diagnosing the underlying disease, preventing its progression through efficacious treatment strategies.
Topics: Humans; Paraproteinemias; Eye; Eye Diseases; Treatment Outcome
PubMed: 36760117
DOI: 10.1177/11206721231155974 -
BMC Cancer Feb 2023B cell maturation antigen (BCMA) targeted immunotherapies have demonstrated remarkable clinical efficacy in multiple myeloma (MM). Here, we evaluated the BCMA expression...
BACKGROUND
B cell maturation antigen (BCMA) targeted immunotherapies have demonstrated remarkable clinical efficacy in multiple myeloma (MM). Here, we evaluated the BCMA expression in MM and other plasma cell dyscrasias (PCDs), hoping to provide a potential treatment strategy for the relapsed/refractory PCDs besides MM.
METHODS
From January 2018 to August 2021, 377 patients with PCDs were enrolled in this study, including 334 MM, 21 systemic light chain amyloidosis (AL), 5 POEMS syndrome, 14 monoclonal gammopathy of undetermined significance (MGUS), and three monoclonal gammopathy of renal significance (MGRS). The membrane-bound BCMA expression measured by multiparameter flow cytometry was defined by BCMA positivity rate and the mean fluorescence intensity (MFI).
RESULTS
The patients with MM had a median BCMA positive rate of 88.55% (range, 0.2% - 99.9%) and median BCMA MFI of 1281 (range, 109 - 48586). While the median BCMA positive rate in other PCDs was 55.8% (6.2% -98.9%), and the median BCMA MFI was 553 (182- 5930). BCMA expression level was negatively associated with hemoglobin concentration in multivariate analysis in terms of BCMA positive rate and MFI.
CONCLUSIONS
In conclusion, BCMA has the potential to be a therapeutic target for other PCDs besides MM.
Topics: Humans; Multiple Myeloma; B-Cell Maturation Antigen; Paraproteinemias; Immunotherapy; Lymphoma, B-Cell; Immunotherapy, Adoptive
PubMed: 36750969
DOI: 10.1186/s12885-023-10591-1 -
Medicine Jan 2023The coexistence of the extranidal marginal zone lymphoma (MZL) of mucosa-associated lymphoid tissue (MALT) and multiple myeloma (MM) is an exceedingly rare situation.... (Review)
Review
RATIONALE
The coexistence of the extranidal marginal zone lymphoma (MZL) of mucosa-associated lymphoid tissue (MALT) and multiple myeloma (MM) is an exceedingly rare situation. The rare situation precludes any evidence-based guidelines for MZL or MM.
PATIENT CONCERNS AND DIAGNOSES
We presented a unique case of the coexistence of primary mediastinal MALT lymphoma and MM like polyneuropathy, organomegaly, endocrinopathy, M-protein, skin syndrome.
INTERVENTIONS AND OUTCOMES
The patient was first diagnosed with polyneuropathy, organomegaly, endocrinopathy, M-protein, skin syndrome in the department of neurology, then MM in the department of hematology, and the mediastinal MALT simultaneously coexisting with MM was found by biopsy in the department of thoracic surgery. The patient received combination therapy with rituximab and bortezomib followed by lenalidomide maintenance. To understand MZL lymphoma with plasmacytic differentiation better, we analyzed cases of MZL lymphomas with plasma cell neoplasms. Most of these cases were MZL lymphomas with light chain-restricted plasmacytic differentiation. The lymphomas relapsed with plasma cell neoplasms or transformed into plasma cell neoplasms after anti-lymphoma therapy.
LESSONS
The case demonstrated clinical complexity and the importance of the detailed assessment. The case and literature review demonstrated the value of detecting light chain-restricted plasmacytic differentiation for the treatment of MZL lymphoma with rituximab plus lenalidomide or bortezomib.
Topics: Humans; Lymphoma, B-Cell, Marginal Zone; Rituximab; Multiple Myeloma; Lenalidomide; POEMS Syndrome; Bortezomib; Thymus Neoplasms
PubMed: 36705370
DOI: 10.1097/MD.0000000000032801 -
World Journal of Clinical Cases Jan 2023Polyneuropathy organomegaly endocrinopathy M-protein and skin changes (POEMS) syndrome is a rare paraneoplastic syndrome caused by a potential plasma cell tumor. The...
BACKGROUND
Polyneuropathy organomegaly endocrinopathy M-protein and skin changes (POEMS) syndrome is a rare paraneoplastic syndrome caused by a potential plasma cell tumor. The clinical manifestations of POEMS syndrome are diverse. Due to the insidious onset and lack of specific early-stage manifestations, POEMS syndrome is easily misdiagnosed or never diagnosed, leading to delayed treatment. Neurological symptoms are usually the first clinical manifestation, while ascites is a rare symptom in patients with POEMS syndrome.
CASE SUMMARY
A female patient presented with unexplained ascites as an initial symptom, which is a rare early-stage manifestation of the condition. After 1 year, the patient gradually developed progressive renal impairment, anemia, polyserosal effusion, edema, swollen lymph nodes on the neck, armpits, and groin, and decreased muscle strength of the lower extremities. The patient was eventually diagnosed with POEMS syndrome after multidisciplinary team discussion. Treatment comprised bortezomib + dexamethasone, continuous renal replacement therapy, chest and abdominal closed drainage, transfusions of erythrocytes and platelets, and other symptomatic and supportive treatments. The patient's condition initially improved after treatment. However, then her symptoms worsened, and she succumbed to the illness and died.
CONCLUSION
Ascites is a potential early manifestation of POEMS syndrome, and this diagnosis should be considered for patients with unexplained ascites. Furthermore, multidisciplinary team discussion is helpful in diagnosing POEMS syndrome.
PubMed: 36687199
DOI: 10.12998/wjcc.v11.i1.135 -
World Journal of Clinical Cases Dec 2022Solitary plasmacytoma and unicentric Castleman disease (UCD) are rare lymphoproliferative disorders characterized by monoclonal plasma cells and a single set of locally...
BACKGROUND
Solitary plasmacytoma and unicentric Castleman disease (UCD) are rare lymphoproliferative disorders characterized by monoclonal plasma cells and a single set of locally enlarged lymph nodes, respectively.
CASE SUMMARY
A 48-year-old Han Chinese man presented to our department with a neck mass and progressive foreign body sensation in his throat. F-FDG positron emission tomography revealed focally increased radioactivity centered around the hyoid, and computed tomography (CT) revealed osteolytic lesions. Histopathology revealed Castleman-like features and CD138/CD38-positive mature plasma cells. Systemic work-up ruled out the possibility of POEMS syndrome, lymphoma, and multiple myeloma, leading to a final diagnosis of solitary hyoid plasmacytoma with UCD. The patient underwent partial hyoid resection and selective neck dissection, followed by intensity-modulated radiotherapy. 99mTc-MDP single-photon emission computed tomography/CT reevaluation showed neither local recurrence nor distant bone metastasis at the 40-mo follow-up.
CONCLUSION
The diagnostic process and differential diagnosis of this rare case provided valuable educational information to clinicians.
PubMed: 36683640
DOI: 10.12998/wjcc.v10.i36.13364 -
The Journal of International Medical... Jan 2023POEMS syndrome is a rare, serious, multisystem disorder and its diagnosis is frequently missed due to its varied clinical presentation. We report here, a 69-year-old...
POEMS syndrome is a rare, serious, multisystem disorder and its diagnosis is frequently missed due to its varied clinical presentation. We report here, a 69-year-old woman with initial complaints of distended abdomen, who was misdiagnosed with tuberculosis but failed anti-tuberculosis treatment. Further examinations showed peripheral neuropathy, monoclonal plasma cell disease, sclerotic bone lesions, an elevated serum vascular endothelial growth factor (VEGF) concentration, lymph node hyperplasia, endocrine abnormalities, and skin hyperpigmentation. A diagnosis of POEMS syndrome was made and the patient responded to lenalidomide-based chemotherapy.
Topics: Humans; Aged; POEMS Syndrome; Vascular Endothelial Growth Factor A; Diagnosis, Differential; Abdomen
PubMed: 36624979
DOI: 10.1177/03000605221148410