Smoldering myeloma in a patient with B12 deficiency: The importance of appropriate differential diagnosis of peripheral plasma cells

Smoldering myeloma (SMM) is an asymptomatic stage characterized by bone marrow plasma cells infiltration between 10-60% in absence of myeloma-defining events and organ damage. Until the revision of criteria of MM to require treatment, two main prognostic models, not overlapping each other, were proposed and used differently in Europe and in US. Novel manageable drugs, like lenalidomide and monoclonal antibodies, with high efficacy and limited toxicity, improvement in imaging and prognostication, challenge physicians to offer early treatment to high-risk SMM. Taking advantage from the debates offered by SOHO Italy, in this review we will update the evidence and consequent clinical practices in US and Europe to offer readers a uniform view of clinical approach at diagnosis, follow-up and supportive care in the SMM setting.

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Quantitative, Dynamic 18F-FDG PET/CT in Monitoring of Smoldering Myeloma: A Case Report

Diagnostics ◽

10.3390/diagnostics11040649 ◽

2021 ◽

Vol 11 (4) ◽

pp. 649

Author(s):

Christos Sachpekidis ◽

Matthias Türk ◽

Antonia Dimitrakopoulou-Strauss

Keyword(s):

Bone Marrow ◽

Plasma Cells ◽

Autologous Transplantation ◽

Standardized Uptake Value ◽

Focal Lesions ◽

Fdg Uptake ◽

Pet Ct ◽

18F Fdg ◽

Smoldering Myeloma

We report on a 52-year-old patient with an initial diagnosis of smoldering myeloma (SMM), who was monitored by means of dynamic and static positron emission tomography/computed tomography (PET/CT) with the radiotracer 1⁸F-fluorodeoxyglucose (18F-FDG). Baseline PET/CT revealed no pathological signs. Six months later, a transition to symptomatic, multiple myeloma (MM) was diagnosed. The transition was not accompanied by focal, hypermetabolic lesions on PET/CT. However, a diffusely increased 18F-FDG uptake in the bone marrow, accompanied by a marked increase of semi-quantitative (standardized uptake value, SUV) and quantitative, pharmacokinetic 18F-FDG parameters, was demonstrated. After successful treatment, including tandem autologous transplantation, the diffuse uptake in the bone marrow as well as the semi-quantitative and quantitative parameters showed a marked remission. This response was also confirmed by the clinical follow-up of the patient. These findings suggest that in MM a diffuse 18F-FDG uptake in the bone marrow may indeed reflect an actual bone marrow infiltration by plasma cells. Moreover, SUV values and kinetic parameters, not only from myeloma lesions but also from random bone marrow samples, may be used for MM monitoring. This could be particularly helpful in the follow-up of myeloma patients negative for 18F-FDG-avid focal lesions.

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Cutaneous Rosai-Dorfman Disease With Increased Number of Eosinophils: Coincidence or Histologic Variant?

Archives of Pathology & Laboratory Medicine ◽

10.5858/arpa.2010-0554-cr ◽

2011 ◽

Vol 135 (12) ◽

pp. 1597-1600 ◽

Cited By ~ 10

Author(s):

John J Cangelosi ◽

Victor G Prieto ◽

Doina Ivan

Keyword(s):

Differential Diagnosis ◽

Pituitary Gland ◽

Plasma Cells ◽

S100 Protein ◽

Clinical Context ◽

Cutaneous Involvement ◽

Eosinophilic Infiltrate ◽

First Case ◽

Rosai Dorfman Disease ◽

Skin Biopsies

Rosai-Dorfman disease (RDD) is characterized histologically by a dense histiocytic infiltrate with emperipolesis and associated lymphocytes, plasma cells, and neutrophils. Eosinophils are not commonly associated. We report a patient with initial thymus and pituitary gland involvement by RDD, who later developed papules on the groin and axilla. Skin biopsies showed admixed histiocytic infiltrates (lymphocytes, neutrophils, and plasma cells) without emperipolesis. A prominent eosinophilic infiltrate was also observed, a feature not, to our knowledge, previously reported. Immunohistochemistry revealed positivity for CD68 (most cells) and S100 protein (scattered cells) and was negative for anti-CD1a. The diagnosis of RDD was established in the clinical context after comparison with the thymic and pituitary lesions (similar histologic features, albeit with fewer eosinophils, and immunohistochemical profiles). We present the first case, to our knowledge, of multicentric RDD with cutaneous involvement and associated prominent eosinophilic infiltrate. Thus, RDD should be included in the differential diagnosis of mononuclear infiltrates containing eosinophils.

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Pulsatile Midline Solitary Plasmacytoma in the Frontal Head Region-A Case Report”

Bangladesh Journal of Neuroscience ◽

10.3329/bjn.v32i1.57412 ◽

2016 ◽

Vol 32 (1) ◽

pp. 39-42

Author(s):

Md Atikur Rahman ◽

Aklaque Hossain Khan ◽

Kanak Kanti Barua

Keyword(s):

Multiple Myeloma ◽

Differential Diagnosis ◽

Case Report ◽

Histological Examination ◽

Plasma Cells ◽

Extramedullary Plasmacytoma ◽

Solitary Plasmacytoma ◽

Rare Entity ◽

Head Region ◽

Malignant Plasma Cell

Primary craniocerebral plasmacytomas are uncommon and represent only 0.7 % of all plasmacytomas. In this case solitary plasmacytoma in the midline frontal head region of the skull and discuss the clinical features and prognosis of this tumor. Plasmacytoma can present as multiple myeloma, solitary plasmacytoma of the bone or extramedullary plasmacytoma. Solitary plasmacytoma is a rare entity that composes of malignant plasma cells and involves the bone to form only one or two lesions without evidence of disease dissemination. It accounts for only 4% of malignant plasma cell tumors. 50 years old male was suffering from plasmacytoma in the frontal head region in our case which is pulsatile. On images showed multiple differential diagnosis but after operation histological examination revealed plasmacytoma. Bangladesh Journal of Neuroscience 2016; Vol. 32 (1): 39-42

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IgG4-associated disease in differential diagnosis of inflammatory orbitopathy

Problems of Endocrinology ◽

10.14341/probl10223 ◽

2019 ◽

Vol 65 (5) ◽

pp. 367-372

Author(s):

Yaroslav O. Grusha ◽

Dilyara S. Ismailova ◽

Natalya Yu. Sviridenko ◽

Pavel I. Novikov ◽

Alla M. Kovrigina

Keyword(s):

Differential Diagnosis ◽

Gold Standard ◽

Granulomatosis With Polyangiitis ◽

Plasma Cells ◽

Eye Disease ◽

Systemic Diseases ◽

Long Period ◽

Inappropriate Treatment ◽

Systemic Inflammatory Disease ◽

Systemic Process

IgG4-associated disease (IgG4-RD) is a systemic inflammatory disease characterized by tumorlike sclerosing masses in different organs. Differential diagnosis in orbital IgG4-RD includes majority of conditions, such as thyroid eye disease (TED), sarcoidosis, granulomatosis with polyangiitis, idiopatic orbital inflammation, limphoproliferative diseases and others. A case of IgG4-RD with different organs involvement and complicated differential diagnosis is presented. This case demonstrates very uncommon manifestation of IgG4-RD, when orbital involvement was very similar with TED. Systemic process was not recognized during a long period of time and diagnosis of IgG4-RD was established only after biopsy of abnormally increased lacrimal gland. Differential diagnosis included other systemic diseases, first of all sarcoidosis, GPA, and lymphoma. Biopsy results were consistent with the gold standard of diagnosis, e. g. more than 40% of plasma cells were IgG4 positive. This case demonstrates the necessity of orbital biopsy before starting immunosuppression to avoid inappropriate treatment strategy.

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Extending the range of differential diagnosis of chronic traumatic encephalopathy of the boxer: Insights from a case report

Dementia & Neuropsychologia ◽

10.1590/1980-57642018dn12-010014 ◽

2018 ◽

Vol 12 (1) ◽

pp. 92-96 ◽

Cited By ~ 1

Author(s):

Leonardo Caixeta ◽

Iron Dangoni Filho ◽

Rafael Dias de Sousa ◽

Pedro Paulo Dias Soares ◽

Andreia Costa Rabelo Mendonça

Keyword(s):

Central Nervous System ◽

Differential Diagnosis ◽

Chronic Traumatic Encephalopathy ◽

Vitamin B12 Deficiency ◽

Corticobasal Degeneration ◽

Behavioral Symptoms ◽

Cranial Trauma ◽

B12 Deficiency ◽

The Central Nervous System ◽

Sports Activities

ABSTRACT Sports activities associated with repetitive cranial trauma have become a fad and are popular in gyms and even among children. It is important to consistently characterize the consequences of such sports activities in order to better advise society on the real risks to the central nervous system. We present the case of a former boxer reporting cognitive and behavioral symptoms that began six years after his retirement as a boxer, evolving progressively with parkinsonian and cerebellar features suggestive of probable chronic traumatic encephalopathy (CTE). Using our case as a paradigm, we extended the range of differential diagnosis of CTE, including corticobasal degeneration, multiple system atrophy, vitamin B12 deficiency, neurosyphilis, frontotemporal dementia and Alzheimer’s disease.

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PC3 - 216 Presentation of Intradural Extramedullary Plasmacytoma with Intratumoral Hemorrhage: Case Report and Review of Literature

Canadian Journal of Neurological Sciences / Journal Canadien des Sciences Neurologiques ◽

10.1017/cjn.2016.395 ◽

2016 ◽

Vol 43 (S4) ◽

pp. S22-S22

Author(s):

A. Ghare ◽

F. Haji ◽

K. MacDougall

Keyword(s):

Multiple Myeloma ◽

Differential Diagnosis ◽

Plasma Cells ◽

Extramedullary Plasmacytoma ◽

Left Frontal Lobe ◽

Axial Mass ◽

Dural Tail ◽

History Of ◽

Intradural Extramedullary ◽

Contrast Ct

Plasmacytomas are solitary tumours characterized by neoplastic proliferation of plasma cells and can be found isolated or in associated with multiple myeloma. Plasmacytomas uncommonly occur intracranially, and dural plasmacytomas without involvement of the calvarium are exceedingly rare. Reported cases indicate durally-based plasmacytomas mimick the appearance of meningioma, lymphoma or sarcoma of the dura. The authors report a case of a 77-year-old male with known multiple myeloma who presented with a 3-week history of confusion, speech impediment, and right sided weakness. A non-contrast CT scan revealed a dense extra-axial mass in the left frontal lobe with initial concerns of an extra-axial hemorrhage. A subsequent MRI demonstrated a contrast enhancing mass with a broad-based dural tail and no underlying calvarial lesion. Differential diagnosis included meningioma or intracranial plasmacytoma. The patient underwent surgical resection and was found to have intratumoural hemorrhage, with pathology confirming plasmacytoma. In the published literature, there are only 20 prior reports of dural plasmacytomas (with and without primary calvarial infiltration), of which only five previous cases reported associated intratumoural hemorrhage. Our case, along with this literature, suggests that new onset of focal neurologic deficits in patients with a history of multiple myeloma merits careful investigation, and that intracranial plasmacytoma should be considered on the differential diagnosis even when imaging reveals masses consistent with hemorrhage or meningioma.

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Plasmocytosis and Immunoparesis but Not Neutrophil to Lymphocyte Ratio Can Predict Time to Treatment in Smoldering Myeloma

Blood ◽

10.1182/blood.v126.23.5358.5358 ◽

2015 ◽

Vol 126 (23) ◽

pp. 5358-5358

Author(s):

Alessandra Romano ◽

Marzia L Consoli ◽

Marina Parisi ◽

Maide Cavalli ◽

Nunziatina L Parrinello ◽

...

Keyword(s):

Plasma Cells ◽

Complete Blood Count ◽

Conflicts Of Interest ◽

Univariate Analysis ◽

Bone Lesion ◽

Progression Free Survival ◽

Absolute Lymphocyte Count ◽

Bone Lesions ◽

Time To Treatment ◽

Smoldering Myeloma

Abstract Objectives: We recently identified the ratio between absolute neutrophils count and absolute lymphocyte count, NLR ≥2, as a predictor of progression free survival (PFS) and overall survival (OS) in patients younger than 65 years with symptomatic Multiple Myeloma (MM). We retrospectively examined the NLR in a cohort of 140 smoldering Myeloma (sMM) defined accordingly to the updated IMWG 2014 guidelines accessed our Center between June 2006 and June 2014. Methods: NLR was calculated using data obtained from the complete blood count (CBC) at diagnosis and subsequently correlated with time to treatment (TTT) for symptomatic MM. All patients underwent bone marrow evaluation to estimate plasma cells infiltration (BMPC), Magnetic Resonance Imaging (MRI) to detect bone lesions, serum free-lite chain evaluation (sFLC) starting from January 2012 when they became evaluable in our center. Results: The mean NLR was 2.0 ± 0.1, lower than the value previously found for MM (2.7 ± 0.2, p= 0.005). Higher NLR was independent of BMPC amount, cytogenetics and sFLC. Using NLR ≥2 as predictive biomarker we could not predict TTT. In univariate analysis only BMPC ≥ 30% (p<0.0001), immunoparesis (suppression of one or more uninvolved immunoglobulins, p=0.017) and presence of at least one bone lesion at MRI (p<0.0001) could predict TTT. In multivariate analysis, these three parameters were independent (p<0.0001). Since new guidelines consider BMPC≥ 60% and positive MRI as myeloma-defining events, we proposed a simplified score model based on BMPC ≥ 30% (1 point) and immunoparesis (1 point). We identified patients with score 0 (20%) as low-risk (TTT at 60 months 0%), with score 1 (61%) as intermediate risk (TTT at 60 months 61%) and with score 2 as high-risk (TTT at 60 months 22%, p<0.0001). Conclusion: We confirmed BMPC and immunoparesis as strong predictors of outcome in sMM. Disclosures No relevant conflicts of interest to declare.

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Role of immune-related conditions in smoldering myeloma and MGUS.

Journal of Clinical Oncology ◽

10.1200/jco.2012.30.15_suppl.8104 ◽

2012 ◽

Vol 30 (15_suppl) ◽

pp. 8104-8104 ◽

Cited By ~ 2

Author(s):

Mary Kwok ◽

Neha Korde ◽

Elisabet E. Manasanch ◽

Manisha Bhutani ◽

Irina Maric ◽

...

Keyword(s):

Multiple Myeloma ◽

Bone Marrow ◽

High Risk ◽

Mayo Clinic ◽

Plasma Cells ◽

Risk Model ◽

Expression Patterns ◽

Autoimmune Disorder ◽

Epidemiological Studies ◽

Smoldering Myeloma

8104 Background: Recent guidelines emphasize tailored follow-up and the need for clinical trials for high-risk smoldering myeloma (SMM). Emerging evidence from epidemiological studies suggests that immune-related conditions play a role in the causation of myeloma precursor disease (SMM and monoclonal gammopathy of undetermined significance; MGUS) and are of clinical importance for the risk of developing multiple myeloma. The aim of our study is to assess whether there is an altered biology in SMM/MGUS patients with preceding immune-related conditions. Methods: From our ongoing prospective SMM/MGUS natural history study, we evaluated 56 SMM and 60 MGUS patients. Information on autoimmunity was identified at baseline. All patients underwent extensive clinical and molecular characterization. At baseline, all patients underwent bone marrow biopsy evaluation using immunohistochemistry and multi-color flow cytometry of plasma cells. We assessed expression patterns of adverse plasma cell markers (CD56 and CD117), and applied risk models based on serum immune markers and bone marrow findings. Results: Among enrolled SMM and MGUS patients, 7 (12%) and 9 (15%) had a preceding autoimmune disorder. We found SMM patients with (vs. without) a preceding autoimmune disorder to have a substantially lower rate of CD56 (28% vs. 61%) and CD117 (28% vs. 61%) expressing plasma cells. When we compared the same markers in MGUS patients, CD56 and CD117 expression patterns were similar among patients with vs. without preceding autoimmunity (10% vs. 17%, and 50% vs. 48%). Using the Mayo Clinic risk model, none of the SMM patients with a preceding autoimmune disorder had high-risk features; in contrast, 3/41 (7%) of those without a preceding autoimmune disorder were high-risk SMM. Using the Mayo Clinic risk model, none of the MGUS patients were high-risk independent of autoimmune status. Conclusions: Our prospective clinical study found SMM patients with preceding immune-related conditions to have less adverse biology, supportive of epidemiological studies suggesting the risk of developing multiple myeloma is substantially lower in these patients.

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