Primary plasmacytoma of generalized lymph nodes: A long survivor

Maekawa, Takafumi; Murakami, Hirokazu; Tamura, Jun’ichi; Sawamura, Morio; Naruse, Takuji; Tsuchiya, Jun

doi:10.1002/ajh.2830430316

Cited by 9 publications

(5 citation statements)

References 11 publications

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“…EMP often runs an indolent clinical course with a tendency for local recurrence, and progresses to plasma cell myeloma in about 15% of patients. (13, 25) Only occasional cases of plasmacytoma expressing IgA (IgA plasmacytoma) have been reported in the literature,(3, 20, 23, 34) and the clinical and pathologic features of these tumors remain poorly defined. We identified an unusual index case of a young woman with multiply recurrent nodal IgA-expressing plasmacytomas first presenting at age 10.…”

Section: Introductionmentioning

confidence: 99%

Nodal and Extranodal Plasmacytomas Expressing Immunoglobulin A

Shao

Raffeld

et al. 2010

American Journal of Surgical Pathology

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Plasmacytomas expressing immunoglobulin A are rare and not well characterized. In this study, nine cases of IgA-positive plasmacytomas presenting in lymph node and three in extranodal sites were analyzed by morphology, immunohistochemistry, and PCR examination of immunoglobulin heavy and kappa light chain genes. Laboratory features were correlated with clinical findings. There were seven males and five females; age range was 10 to 66 years (median, 32 years). Six of the patients were younger than 30-years-old, five of whom had nodal disease. 67% (6/9) of the patients with nodal disease had evidence of immune system dysfunction, including human immunodeficiency virus (HIV) infection, T-cell deficiency, autoantibodies, arthritis, Sjögren's syndrome, and decreased B-cells. An IgA M-spike was detected in 6/11 cases, and the M-protein was nearly always less than 30 g/L. All patients had an indolent clinical course without progression to plasma cell myeloma. Histologically, IgA plasmacytomas showed an interfollicular or diffuse pattern of plasma cell infiltration. The plasma cells were generally of mature Marschalko type with little or mild pleomorphism and exclusive expression of monotypic IgA. There was an equal expression of kappa and lambda light chains (ratio 6:6). Clonality was demonstrated in 9 of 12 cases: by PCR in 7 cases, by cytogenetic analysis in 1 case, and by immunofixation in 1 case. Clonality did not correlate with pattern of lymph node infiltration. Our results suggest that IgA plasmacytomas may represent a distinct form of extramedullary plasmacytoma characterized by younger age at presentation, frequent lymph node involvement and low risk of progression to plasma cell myeloma.

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Section: Introductionmentioning

confidence: 99%

Nodal and Extranodal Plasmacytomas Expressing Immunoglobulin A

Shao

Raffeld

et al. 2010

American Journal of Surgical Pathology

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“…[16]48 MParatracheal, precarinal, subcarinal, aortopulmonaryIgG λM-Spike 2.1 g/dL, λ FLC 1613 mg/LNoneInvolved field radiation (50 Gy in 25 fractions)No responsePersistent disease following first-line radiationVTD-PACE, then auto SCT, then lenalidomide maintenanceIn CR 18 months following auto-SCTMatsushima et al . [17]56 FMandibular, cervical, axillary, para-aorticIgA KPresent, though not quantifiedNoneCyclophosphamide and prednisolone (10 courses)Complete response (CR)Relapse 6 years following initial CRMelphalan and prednisolone (10C)In CR 9 years following initial diagnosisGorodetskiy et al . [18]48 FDiffuse LAD, most prominent at supraclavicular, femoral nodesIgA KM-Spike 7.2 g/dLNoneCHOP for 9 cyclesComplete response (CR)Relapse at 3 months and again at 19 monthsInitial relapse treated with 1C CEVD, subsequent treated with 1C CHOEPIn CR 18 years following initial diagnosisMenke et al .…”

Section: Discussionmentioning

confidence: 99%

Primary extramedullary plasmacytoma with diffuse lymph node involvement: a case report and review of the literature

Naymagon

Abdul-Hay

2019

J Med Case Reports

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Background Primary plasmacytomas are localized proliferations of clonal plasma cells occurring in the absence of a systemic plasma cell dyscrasia such as multiple myeloma. Primary plasmacytomas most commonly manifest as solitary lesions of the bone or of the upper aerodigestive tract. Presentation in a lymph node is very uncommon and can often be initially mistaken for lymphoma. Because they are local phenomena, primary plasmacytomas are managed with local therapies such as radiation or, less commonly, excision. Multifocal presentations are rare and are often not amenable to local treatment modalities, thus requiring systemic therapies. Because of their rarity, standardized treatment guidelines are not established, and treatment paradigms borrow heavily from those employed in multiple myeloma. Multifocal presentation in lymph nodes is nearly unheard of with only seven such cases reported in the existing literature, only four of which were diffuse enough to require systemic therapy. Here we describe the most diffuse and widely distributed instance of primary lymph node plasmacytoma yet reported and present a description of its successful treatment with systemic therapy. Case presentation A 71-year-old Asian man presented with progressive fatigue in the setting of diffuse hypermetabolic lymphadenopathy throughout his chest, abdomen, and pelvis. A diagnosis of lymphoma was initially suspected; however, a lymph node biopsy was consistent with plasmacytoma. A bone marrow biopsy was unremarkable, and no monoclonal protein was identified, establishing a diagnosis of primary extramedullary plasmacytomas of the lymph nodes. He was treated with a myeloma-like regimen consisting of four cycles of bortezomib/dexamethasone followed by two cycles of thalidomide/prednisone with improvement in symptoms and near complete resolution of prior hypermetabolic lymphadenopathy. He remains in remission over 18 months following completion of therapy. Conclusion This case report and accompanying literature review highlight the exceedingly rare and easily misclassified entity of primary plasmacytoma of diffuse lymph nodes. Importantly, we demonstrate that this entity may be treated with, and demonstrate excellent response to, systemic therapies often employed in multiple myeloma.

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“…Plasmacytomas that occur in the absence of multiple myeloma rarely, if ever, present with FUOs. [8][9][10][11][12][13][14] Some nodal and extranodal plasmacytomas present clinically, and may express IgA as indolent lymphoproliferative disorders. Some indolent plasmacytomas are termed "smoldering multiple myelomas," and represent the evolution of a plasmacytoma into multiple myeloma.…”

Section: Discussionmentioning

confidence: 99%

“…2,3 Isolated plasmacytomas rarely cause FUOs. [9][10][11][12] Rare reports have involved isolated immunoglobulin A (IgA)-secreting plasmacytomas in various anatomical locations. 9,13 However, no reports have been published of IgA-secreting plasmacytomas presenting with FUOs.…”

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confidence: 99%