scholarly journals A Rare Case of Extramedullary Plasmacytosis

2021 ◽  
Vol 9 ◽  
pp. 232470962110406
Author(s):  
Kristen L. Farraj ◽  
Aboud Kaliounji ◽  
Deepthi Kagolanu ◽  
Nausheer Khan
Keyword(s):  
Cell Proliferation ◽  
Mucous Membrane ◽  
Plasma Cell ◽  
Rare Case ◽  
Plasma Cells ◽  
Gastric Polyp ◽  
Rare Form ◽  
Rare Presentation

Plasmacytosis is the abnormal proliferation of plasma cells in tissue, bone, blood, or exudates. Mucous membrane plasmacytosis is a rare form of plasmacytosis that is usually idiopathic in nature. A 68-year-old female underwent a diagnostic and therapeutic esophagogastroduodenoscopy, which revealed a friable antral erythematous gastric fold and a gastric polyp. Subsequent pathological testing was positive for diffuse polyclonal plasma cell proliferation, which was suggestive of a gastric plasmacytosis. In this article, we report a rare presentation of mucous membrane plasmacytosis.

scholarly journals Extensive erythema elevatum diutinum associated with IgA monoclonal gammopathy: a rare case report

2021 ◽  
Vol 7 (4) ◽  
pp. 593
Author(s):  
Manish Rijhwani ◽  
Divya Yadav ◽  
Manisha Nijhawan ◽  
Arvind Verma
Keyword(s):  
Monoclonal Gammopathy ◽  
Rare Case ◽  
Leukocytoclastic Vasculitis ◽  
Burning Sensation ◽  
Rare Form ◽  
Rare Presentation ◽  
Rare Case Report ◽  
Nodular Lesions ◽  
Erythema Elevatum Diutinum ◽  
Characteristic Features

<p class="abstract">Erythema elevatum diutinum (EED) is a rare form of leukocytoclastic vasculitis with an unclear pathogenesis. Almost 250 cases of EED have been reported in the literature, associated with several diseases and presented with various clinical features. Term 'diutinum' means chronic, describes one of the main characteristic features. The duration is very long, varying between one to more than 39 years. We report a rare presentation of EED in a 58-years-old man who presented with extensive and symmetrical, persistent, erythematous to violaceous   plaques over trunk and limbs and popular-nodular lesions present over pinna and dorsum of hands with burning sensation and itching over few lesions, in association with IgA monoclonal gammopathy.</p>

scholarly journals Extramedullary Plasmacytoma of the Tonsil

2011 ◽  
Vol 2011 ◽  
pp. 1-2 ◽  
Author(s):  
Kevin C. Huoh ◽  
Annemieke Van Zante ◽  
David W. Eisele
Keyword(s):  
Plasma Cell ◽  
Unusual Case ◽  
Plasma Cells ◽  
Tertiary Care ◽  
Neck Region ◽  
Extramedullary Plasmacytoma ◽  
University Hospital ◽  
Rare Form ◽  
Head And Neck Region ◽  
Radiologic Evaluation

Plasma cell tumors are a diverse group of neoplasms characterized by monoclonal proliferation of plasma cells. Extramedullary plasmacytoma (EMP) is a rare form of localized plasma cell tumor that arises most often in the head and neck region. We present an unusual case of EMP of the palatine tonsil from a tertiary care university hospital. We discuss the histopathologic and radiologic evaluation as well as treatment of EMP.

Multiple Myeloma Presenting as a Clivus and Brain Stem Plasmacytoma.

Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 5061-5061
Author(s):  
Arash Rezazadeh ◽  
Goetz H. Kloecker ◽  
Damian A. Laber
Keyword(s):  
Multiple Myeloma ◽  
Brain Stem ◽  
Plasma Cell ◽  
Plasma Cells ◽  
Complete Blood Count ◽  
Thoracic Vertebrae ◽  
Rare Presentation ◽  
Organ Systems ◽  
Brain Involvement ◽  
The Brain

Abstract Multiple myeloma is the most common hematological malignancy. MM can affect many organ systems; therefore, it may mimic different clinical syndromes at presentation. We report an extremely rare presentation of MM. CASE REPORT: A 62-year-old man presented with progressive cervical pain for 2 months and double vision for 2 weeks. Physical exam demonstrated a cranial nerve 6 palsy, nuchal rigidity and no other abnormalities. A magnetic resonance image (MRI) of the brain revealed a 5 cm enhancing mass in the clivus which extended into the brain stem, left internal auditory and carotid canal, nasopharynx, sphenoid and cavernous sinuses, without midline shift or hydrocephalus. MRI of the spine showed 2 small enhancing lesions in thoracic vertebrae 4 and 11. Computer tomography of the chest abdomen and pelvis demonstrated a lesion in the sternum and no other abnormalities. Technetium-99 bone scan showed uptake in the skull base mass only. Complete blood count and metabolic profile were normal except for an albumin of 3.2 g/dL. Serum protein electrophoresis with immunofixation revealed hypogammaglobulinemia without a monoclonal protein. 24 hour urine collection demonstrated monoclonal free kappa light chains. Transsphenoidal stereotactic biopsy of the mass showed an atypical plasma cell proliferation with multi-nucleation and mitotic activity, positive for kappa light chain. Bone marrow aspirate revealed 17% of the nucleated cell to be abnormal plasma cells, some with plasmablastic morphology. He improved rapidly with dexamethasone and was referred for radiotherapy. DISCUSSION: The most common neurologic complications of MM are spinal cord compression due to epidural plasmacytomas or vertebral fractures, and peripheral polyneuropathy due to the presence of antibodies directed against myelin structures or due to amyloid deposits. Brain involvement in MM is uncommon. Furthermore, neurologic symptoms due to plasmacytomas located either in the clivus and brain stem are extremely rare. A MEDLINE search from 1950 until July 2005 revealed only 9 cases of MM or plasmacytoma involving the brain stem. Since there is no published autopsy series on MM patients describing the percentage of patients with brain involvement at death, the denominator is difficult to assess. Clinically, extramedullary involvement from MM bears a poor prognosis, and has been described in cases of plasma cell leukemia. We speculate that the aggressive behavior of the MM in our patient might be consistent with the atypical phenotype of the plasma cells. CONCLUSION: The unusual presentation of this patient adds to the medical knowledge about the clinical spectrum of multiple myeloma.

scholarly journals Rare Presentation of Primary Extramedullary Plasmacytoma as Lip Lesion

2017 ◽  
Vol 2017 ◽  
pp. 1-5
Author(s):  
Mali Him ◽  
Maggie Meier ◽  
Vikas Mehta
Keyword(s):  
Multiple Myeloma ◽  
Cell Proliferation ◽  
Plasma Cell ◽  
Light Chain ◽  
Extramedullary Plasmacytoma ◽  
Solitary Plasmacytoma ◽  
Rare Presentation ◽  
Lower Lip ◽  
Light Chain Disease ◽  
Extramedullary Plasmacytomas

Malignant plasma cell proliferation can be presented as part of disseminated disease of multiple myeloma, as solitary plasmacytoma of bone, or in soft tissue as extramedullary plasmacytoma. Extramedullary plasmacytomas represented approximately 3% of all plasma cell proliferation. Approximately 80% of extramedullary plasmacytomas occur in the head and neck region while the other 4% occur in the skin and to a lesser extent in the lip. In this paper, we report a rare case of primary cutaneous plasmacytoma involving the lip in a 65-year-old male. The patient presented with a nonhealing lower lip sore for the past 3 years. Upon further workup, there was no evidence of multiple myeloma or light chain disease. The patient was treated with radiation therapy and his last follow-up revealed no evidence of multiple myeloma or light chain disease.

Rising Plasma Cell Proliferation By Ki67/CD138 Ratio at Relapse Is a Marker of High Risk Disease in Multiple Myeloma

Blood ◽  
2015 ◽  
Vol 126 (23) ◽  
pp. 2991-2991
Author(s):  
Peter A. Forsberg ◽  
Tomer M Mark ◽  
Sujitha Yadlapati ◽  
Adriana C Rossi ◽  
Roger N Pearse ◽  
...  
Keyword(s):  
Multiple Myeloma ◽  
Bone Marrow ◽  
Cell Proliferation ◽  
Overall Survival ◽  
High Risk ◽  
Plasma Cell ◽  
Plasma Cells ◽  
Initial Therapy ◽  
First Line ◽  
High Risk Disease

Abstract Background: Assessment of malignant plasma cell cycling via plasma cell labeling index (PCLI) has been a validated prognostic tool in multiple myeloma (MM) for years but utilization remains limited. We recently developed a novel immunohistochemical (IHC) co-staining technique for CD138 and Ki67 expression to quantify plasma cells in active cycling. Previously presented results from newly diagnosed patients demonstrate that having an elevated ratio of plasma cells in active cycle by co-expression of CD138 and Ki67 (>5%) is associated with aggressive disease and poor outcomes including shorter overall survival (OS). The expansion of subclones with higher proliferative capacity following initial therapy may be an indicator of a higher risk relapse event and indicate poor prognosis. Here we assess MM patients (pts) with Ki67/CD138 co-staining on bone marrow samples both at diagnosis and relapse to assess the impact of changes in cell cycling ratio on outcomes with subsequent therapy and overall clinical course. Methods: A retrospective cohort study of pts with treated symptomatic MM was performed by interrogation of the clinical database at the Weill Cornell Medical College / New York Presbyterian Hospital (WCMC/NYPH). For inclusion in the analysis, pts must have had bone marrow evaluation with double-staining for Ki67 and CD138 by immunohistochemistry both at diagnosis and relapse. Pts must have completed their first line and relapse treatments at WCMC/NYPH. The Ki67% was calculated as the ratio of plasma cells expressing CD138 that were also found to express Ki67. Treatment outcomes were stratified and compared based on alterations in Ki67% between diagnosis and relapse. Results: We identified 37 pts with bone marrow sampling that was evaluated for CD138 and Ki67 co-expression both at diagnosis and at the time of relapse. These pts had undergone a median of 2 lines of prior treatment at the time of relapse bone marrow biopsy (range 1-7). 19 pts were identified to have a rising Ki67% between diagnosis and relapse defined at a 5% or greater increase, the other 18 pts had stable or decreased Ki67%. Pts with a rising Ki67% at relapse had a shorter OS with a median of 72 months vs not reached (p=0.0069), Figure 1. Pts who had rising Ki67% at relapse had shorter progression free survival (PFS) on first line treatment with a median of 25 vs 47 months (p=0.036), Figure 2. Additionally pts with rising Ki67% had a trend towards shorter PFS with the treatment they received after relapse with median of 12.5 vs 3.5 months (p=0.09). Relapse regimens were most commonly carfilzomib (n=9), pomalidomide (5) or ixazomib (4) based. 37% of pts (7/19) with rising Ki67% achieved PR or better on relapsed treatment vs 67% (12/18) with stable Ki67%. Discussion: The presence of clonal evolution and selection of higher risk clones under therapeutic pressure in multiple myeloma is a key feature of disease progression. The ability to improve risk stratification at the time of relapse may help guide clinical decision making to best suit individual patient needs. We have identified rising plasma cell proliferation through quantification of Ki67/CD138 co-expression at relapse to be a useful marker of high risk disease evolution. This appears to help identify the emergence of higher risk clones which are ultimately responsible for treatment resistant disease. Patients with rising Ki67% were more likely than patients with stable Ki67% to have early relapses to initial therapy, were less likely to achieve responses to relapse regimens or to maintain their response and had shorter overall survival. Further evaluation is needed to identify if different approaches to patients with increasing proliferation may improve outcomes in these patients. Figure 1. Figure 1. Figure 2. Figure 2. Disclosures Mark: Calgene: Membership on an entity's Board of Directors or advisory committees, Research Funding, Speakers Bureau. Rossi:Calgene: Speakers Bureau. Pearse:Celegen: Consultancy. Pekle:Celgene: Speakers Bureau; Takeda: Speakers Bureau. Perry:Celgene: Speakers Bureau; Takeda: Speakers Bureau. Coleman:Celgene: Speakers Bureau; Takeda: Speakers Bureau. Niesvizky:Celgene: Consultancy, Speakers Bureau.

scholarly journals Acquired Hemophilia A in IgG4-Related Disease: Case Report, Immunopathogenic Study, and Review of the Literature

2020 ◽  
Vol 11 ◽  
Author(s):  
Sébastien Sanges ◽  
Emmanuelle Jeanpierre ◽  
Benjamin Lopez ◽  
Jules Russick ◽  
Sandrine Delignat ◽  
...  
Keyword(s):  
Cell Proliferation ◽  
Plasma Cell ◽  
Plasma Cells ◽  
Hemophilia A ◽  
Acquired Hemophilia A ◽  
Acquired Hemophilia ◽  
Autoantibody Production ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Disease Case

We report the observation of a 75-year-old patient referred for cervical lymphadenopathies. A pre-lymphadenectomy blood work revealed an asymptomatic elevation of aPTT with low factor VIII (FVIII) levels and high anti-FVIII antibodies titers, consistent with acquired hemophilia A (AHA). Histological work-up of a cervical lymphadenopathy revealed benign follicular hyperplasia with IgG4+ lymphoplasmacytic infiltration; and serum IgG4 levels were markedly elevated, compatible with IgG4-related disease (IgG4-RD). He was successfully treated with a 9-month course of prednisone, secondarily associated with rituximab when an AHA relapse occurred. As this patient presented with an unusual association of rare diseases, we wondered whether there was a link between the two conditions. Our first hypothesis was that the anti-FVIII autoantibodies could be directly produced by the proliferating IgG4+ plasma cells as a result of broken tolerance to autologous FVIII. To test this assumption, we determined the anti-FVIII IgG subclasses in our patient and in a control group of 11 AHA patients without IgG4-RD. The FVIII inhibitor was mostly IgG4, with an anti-FVIII IgG4/IgG1 ratio of 42 at diagnosis and 268 at relapse in our patient; similar values were observed in non-IgG4-RD AHA patients. As a second hypothesis, we considered whether the anti-FVIII activity could be the result of a non-specific autoantibody production due to polyclonal IgG4+ plasma cell proliferation. To test this hypothesis, we measured the anti-FVIII IgG4/total IgG4 ratio in our patient, as well as in several control groups: 11 AHA patients without IgG4-RD, 8 IgG4-RD patients without AHA, and 11 healthy controls. We found that the median [min-max] ratio was higher in AHA-only controls (2.4 10-2 [5.7 10-4-1.79 10-1]), an oligoclonal setting in which only anti-FVIII plasma cells proliferate, than in IgG4-RD-only controls (3.0 10-5 [2.0 10-5-6.0 10-5]), a polyclonal setting in which all IgG4+ plasma cells proliferate equally. Our patient had intermediate ratio values (2.7 10-3 at diagnosis and 1.0 10-3 at relapse), which could plead for a combination of both mechanisms. Although no definitive conclusion can be drawn, we hypothesized that the anti-FVIII autoantibody production in our IgG4-RD AHA patient could be the result of both broken tolerance to FVIII and bystander polyclonal IgG4+ plasma cell proliferation.

scholarly journals The Early Diagnostic Dilemma in Angioimmunoblastic T Cell Lymphoma with Excessive Plasma Cells Proliferation

2021 ◽  
Vol 2021 ◽  
pp. 1-8
Author(s):  
Chunyan Wang ◽  
Xia Mao ◽  
Songya Liu ◽  
Cheng He ◽  
Ying Wang ◽  
...  
Keyword(s):  
Cell Proliferation ◽  
T Cell ◽  
Plasma Cell ◽  
Plasma Cells ◽  
Cell Lymphoma ◽  
Clinical Manifestations ◽  
Underlying Disease ◽  
T Cell Lymphoma ◽  
Diagnostic Dilemma ◽  
Angioimmunoblastic T Cell Lymphoma

Background. Angioimmunoblastic T cell lymphoma (AITL) is an aggressive Epstein–Barr virus-associated T cell lymphoma. Clinical syndromes of AITL are not confined to fever and lymphadenopathy, and patients may initially present with polyclonal plasma cell proliferation, which may obscure the underlying disease of AITL, delaying diagnosis. Case Presentation. Here, we report two AITL patients with excessive plasma cell proliferation in the bone marrow, peripheral blood, and ascites even mimicking plasma cell leukemia. Both of them had poor endings. Conclusions. Our report emphasizes the complexity of the clinical manifestations of AITL, which aims to increase the alertness of physicians and improve the rate of early diagnosis. Integrated diagnostic approaches such as histopathology, flow cytometry, cytogenetics, and molecular biology are essential for accurate diagnosis and precise therapy.

A rare case of plasmacytoma of mandible revealing plasma cell myeloma

2021 ◽  
Vol 2 (5) ◽  
Author(s):  
Hari Ram ◽  
◽  
Sneha Gupta ◽  
Praveen Kumar Singh ◽  
Shivani Sharma ◽  
...  
Keyword(s):  
Multiple Myeloma ◽  
Plasma Cell ◽  
Rare Case ◽  
Plasma Cells ◽  
Solitary Plasmacytoma ◽  
Bence Jones Protein ◽  
Jaw Bones ◽  
Plasma Cell Neoplasm ◽  
Cell Neoplasm ◽  
Single Bone

Multiple myeloma (MM) is a malignant proliferation of plasma cells with multiple foci. Plasmacytoma is a solitary plasma cell neoplasm involving a single bone. The most commonly involved bone is vertebra. Jaw bones are rarely involved as a first bone as they have lesser hematopoietic marrow. A solitary plasmacytoma may progress to multiple myeloma within few months to year. We present a case of a swelling of mandible that on further investigations confirmed the diagnosis of multiple myeloma. We have discussed the course of treatment given and its prognosis. Keywords: multiple myeloma; plasmacytoma of jaw; bence jones Protein; abnormal plasma cells; CD138.

Cancer-Testis (CT) Antigen Expression In AL Amyloidosis

Blood ◽  
2010 ◽  
Vol 116 (21) ◽  
pp. 4055-4055
Author(s):  
Michael A. Rosenzweig ◽  
Heather Landau ◽  
Achim A Jungbluth ◽  
Nicole Hanson ◽  
Denise Frosina ◽  
...  
Keyword(s):  
Multiple Myeloma ◽  
Cell Proliferation ◽  
Plasma Cell ◽  
Research Funding ◽  
Plasma Cells ◽  
Organ Involvement ◽  
Al Amyloidosis ◽  
Ct Antigen ◽  
Ct Antigens ◽  
Cancer Testis

Abstract Abstract 4055 Cancer-testis (CT) antigens are a family of proteins normally expressed in immune privileged sites such as testicular germ cells and placenta, but are overexpressed in various malignant tumors (Scanlan et al. Immunological Rev. 2002). CT antigens are therefore useful markers of malignancy as well as potential targets for antigen specific cancer immunotherapy. In multiple myeloma, CT 7, CT10 and MAGE-A are homogenously expressed in up to 75% of cases, and their expression increases with disease stage and cell proliferation (Jungbluth et al. Blood, 2005). In addition, CT-7 and MAGE-A3 play a role in plasma cell proliferation and chemosensitivity (Atanackovic et al. Haematologica 2010). Immunogenicity of CT antigens is evidenced by spontaneous humoral responses against CT antigens in patients with multiple myeloma (Cohen et al. ASH abstract 2008). In addition, anti-CT antigen immune responses of donor derived T and B cells have been reported following allogeneic stem cell transplantation, suggesting CT antigens may serve as a natural target for a graft-versus myeloma effect (Atanackovic et al. Blood 2007). Systemic light-chain (AL) amyloidosis is a plasma cell dyscrasia related to multiple myeloma characterized by small numbers of non-proliferating, clonogenic plasma cells producing pathologic light chains. In this study, we investigated the expression of several CT antigens in patients with AL amyloidosis to identify potential targets for immunotherapy and determine their prognostic significance. Methods: Fifteen cases of AL amyloidosis were studied employing standard IHC techniques on paraffin-embedded archival tissues. Presence of plasma cells was verified by CD138 immunostain. The following monoclonal antibodies (to the following CT Antigens) were used: mAb MA454 (MAGE-A1), 6C1 (several MAGE-A antigens), 57B (MAGE-A4), E978 (NY-ESO-1), CT7-33(CT7), CT10#5 (CT10), #26 (GAGE). Immunopositivity was graded based on the amount of IHC-positive plasma cells. Results: All 15 patients had a confirmed diagnosis of AL amyloidosis with an average plasma cell burden of 12.7% of the cells in the marrow. Eighty-seven percent (13/15) had lambda disease and 13% (2/15) kappa disease. Organ involvement included kidney (n=7)), heart (n=8), peripheral nervous system (n=2), and GI/liver (n=2). Five patients (33%) had multi-organ involvement. All patients were treated uniformly with risk-adapted melphalan as their initial therapy. CT7 was present in 9/15 (60%) while CT10 was demonstrated in only 1/15 AL amyloid cases. Plasma cells did not stain with any other anti-CT mAb. There were no significant differences with regard to organ involvement, response to treatment or prognosis and CT antigen positivity in this small sample set. Discussion: This is the first study identifying CT7 as the prevalent CT antigen in plasma cells of patients with AL amyloidosis. The almost exclusive presence of CT7 in AL amyloidosis may have clinical significance. Further studies are planned on additional samples to confirm the prevalence of CT7 expression in AL amyloidosis, determine its immunogenicity and further investigate prognostic implications. Additional studies are needed to determine the biology of CT antigens in AL amyloidosis and their value as a potential target for immunotherapy. Disclosures: Comenzo: Millenium Pharmaceuticals: Membership on an entity's Board of Directors or advisory committees; Elan Pharmaceuticals: Consultancy; Genzyme: Research Funding; Celgene: Research Funding; Ortho: Research Funding.

scholarly journals The Development of a Myeloma-Like Condition in Mink with Aleutian Disease

Blood ◽  
1965 ◽  
Vol 25 (5) ◽  
pp. 736-742 ◽  
Author(s):  
DAVID D. PORTER ◽  
FRANK J. DIXON ◽  
AUSTIN E. LARSEN
Keyword(s):  
Cell Proliferation ◽  
Serum Protein ◽  
Plasma Cell ◽  
Plasma Cells ◽  
Viral Disease ◽  
Cell System ◽  
Protein Electrophoresis ◽  
Viral Etiology ◽  
Aleutian Disease Of Mink ◽  
Bence Jones Proteinuria

Abstract Aleutian disease of mink, apparently of viral etiology, is characterized by an extreme diffuse proliferation of plasma cells, and a marked hypergammaglobulinemia, which is electrophoretically heterogeneous. In some mink with this disease serially followed by serum protein electrophoresis, a transition to a homogeneous myeloma-like hypergammaglobulinemia, together with the appearance of Bence Jones proteinuria, was observed late in their course. Thus, this viral disease shows plasma cell proliferation beginning as a multiclonal hyperplasia which may terminate as a monoclonal, possibly neoplastic overgrowth of the plasma cell system.

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