Plasma Cell Neoplasms
Wojciech Gorczyca in Atlas of Differential Diagnosis in Neoplastic Hematopathology, 2014
Malignant lymphomas with extensive plasmacytic differentiation may be indistinguishable morphologically from plasma cell neoplasms, especially when plasma cells predominate (e.g., after treatment that eliminated the B-cell component or in limited biopsy specimens that reflect plasma cell-rich areas). Lymphoplasmacytic lymphoma and other types of B-cell lymphomas, especially marginal zone lymphoma (MZL), can be differentiated from plasma cell neoplasms by demonstration of a clonal B-cell component with monotypic expression of the same immunoglobulin light chains. MZL differs by clinical presentation and lack of a significant serum M-protein. Occasional cases of MZL may be difficult to distinguish from extraosseous plasmacytoma. Lymphoplasmacytic lymphoma shows intranuclear inclusions and gradual transition from small lymphocytes into plasma cells. The latter may have a “flame cell” appearance often associated with the IgA isotype. Plasmacytoma is distinguished from myeloma by radiologicand clinical data. In occasional cases of Castleman’s disease, plasma cell type may display monotypic (IgA/lambda) plasma cells and requires differentiation from plasma cell tumors.
Historical Milestones
Tariq I. Mughal in Precision Haematological Cancer Medicine, 2018
In 1944, Jan Waldenström in Uppsala described two patients who presented the following symptoms: bleeding, lymphadenopathy, elevated erythrocyte sedimentation rate, high serum viscosity and increased lymphoid cells in the bone marrow. He speculated that the high serum viscosity was due to an abnormally large protein (a macroglobulin), later characterized as a monoclonal immunoglobulin of the M class (IgM), following the availability of electrophoresis of the serum proteins. The disease was then named Waldenström’s macroglobulinaemia. It was initially classified as a plasma cell neoplasm and is now classified as a lymphoplasmacytic lymphoma. Importantly he also conceptualized the notion of monoclonal versus polyclonal gammopathy, which later led to the recognition of monoclonal gammopathy of unknown significance (MGUS) and an association to develop MM; in contrast patients with polyclonal gammopathy (also known as ‘hypergammaglobulinemia’) often had a benign immune or inflammatory disorder. In 1956, Leonard Korngold and Rose Lipari, in New York noted that Bence–Jones proteins were related to abnormal serum proteins, and in their honour Bence–Jones proteins were designated as either kappa (κ, after Korngold) or lambda (λ, after Lipari). Meanwhile, the seminal work done by Robert Kyle in Rochester, Minnesota led to the description of MGUS and SMM and the estimation of the risk of progression to myeloma. In 1975, the Durie–Salmon Staging system, proposed by Brian Durie and Sydney Salmon in Tucson, Arizona based on myeloma burden, was introduced to assess prognosis and improve treatment approaches.
Different Types of Leukemias, Lymphomas, and Myelomas
Tariq I Mughal, John M Goldman, Sabena T Mughal in Understanding Leukemias, Lymphomas, and Myelomas, 2017
This is a variant of myeloma, first described by Jan Waldenström in Uppsala (Sweden) in 1961, in which a high concentration of immunoglobulin M (IgM) paraprotein is found. It is also known as lymphoplasmacytic lymphoma and is characterized by plasmacytoid lymphocytes, which uniquely have features of both lymphocytes and plasma cells (Fig. 4.44). The incidence is about 0.5 per 100,000 of adult population and the median age at diagnosis is about 65 years, with males being affected more often. Bone lesions are extremely rare and the main classic problems relate to plasma hyperviscosity. A prognostic staging system based on the level of IgM, hemoglobulin concentration, and the beta-2-microglobulin is currently being pursued (Table 10.1).
Lymphomas of the salivary glands: a systematic review
Published in Acta Oto-Laryngologica, 2023
Ahmed Ehsan Al-Khafaf, Fahd Al-Shahrestani, Yusuf Baysal, Lise Mette Rahbek Gjerdrum, Steffen Heegaard, Lars Møller Pedersen, Preben Homøe
Of the 169 included studies, 43% (n = 74) were cohorts (level of evidence 4) contributing to 1579 patient cases and 56% (n = 95) were case reports (level of evidence 2b). The included studies contained 1640 patient cases for data extraction. The most frequent lymphoma subtypes were common B-cell lymphomas: EMZL (n = 1298) (See Figure 2C & 2D), diffuse large B-cell lymphomas (DLBCL) (n = 119), follicular lymphoma (FL) (n = 82) and mantle cell lymphomas (MCL) (n = 10). The rarer subtypes of B-cell lymphomas were grouped together as ‘Other BCL’ (n = 68), which included lymphoplasmacytic lymphoma (LPL), small lymphocytic lymphoma (SLL), Burkitt’s lymphoma (BL), and other B-cell lymphomas not otherwise specified. Additionally, 30 Hodgkin lymphomas (HL) and 21 patients with T-cell lymphoma (TCL) including anaplastic large T-cell lymphoma (ALCL) (n = 3) were included.
Discontinuation of immunoglobulin replacement therapy in patients with secondary antibody deficiency
Published in Expert Review of Clinical Immunology, 2020
Vishesh Patel, Juthaporn Cowan
An example of unnecessarily prolonging IGRT was provided by a clinician at The Ottawa Hospital, and has not been published. A 72-year-old man presented with significant hypogammaglobulinemia at the time of diagnosis with lymphoplasmacytic lymphoma. Due to a chronic illness involving weight loss and diarrhea in addition to hypogammaglobulinemia, he was initiated on IGRT. There was a striking clinical response marked by decreased diarrhea, weight gain, and recovery of good health. The dosage of IGRT was titrated to remission of diarrhea and fatigue. Trough serum IgG levels before IVIG doses were about 10 g/L. Treatment of the lymphoma using chemotherapy was also initiated around the same time and led to a complete clinical remission of his disease. During several years of IGRT, discussions considering a trial stoppage had come up; however, the patient was disinclined to discontinue treatment given his excellent tolerance of IVIG and his association of the impressive convalescence with IGRT. A year later, while on IGRT, he developed a severe pulmonary embolism and survived on oral anticoagulation. IGRT was discontinued, and quantitative immunoglobulin levels remained in the mid-normal range thereafter, without relapse of fatigue, diarrhea, or weight loss. He had received the recommended adult vaccinations for immunocompromised individuals. He did well without an occurrence of severe infection in the following year. Although IGRT is likely not the only cause of this serious adverse event, this case highlights how unnecessary treatment could potentially be a contributing factor.
Herpes Simplex Virus 2 Meningoencephalitis-Associated Bilateral Optic Neuritis and Radiculitis
Published in Neuro-Ophthalmology, 2020
William L. Conte, Faten El Ammar, Asadolah Movahedan, Hassan A. Shah, Jeffrey Nichols, Adil Javed
A 57-year-old white female with a history of IgM lymphoplasmacytic lymphoma in remission presented with 4 weeks of worsening malaise, mild bilateral blurred vision, confusion, and headaches. Her neurological exam was unremarkable for any motor or sensory impairments. MRI of the brain showed nonspecific white matter lesions. MRI of the orbits had significant motion artefact and within these confines, no overt optic nerve lesions or enhancement were found. A lumbar puncture was performed which revealed opening pressure of 24.5 cm H20 (although she was in a flexed position), WBC 142 (86% lymphocytes), protein 163 mg/dL, and glucose 32 mg/dL. Cytology and flow cytometry were negative for malignant cells. PCR was positive for HSV-2. Other infectious workup was negative. She was prescribed acyclovir 10 mg/kg every 8-h and acetazolamide 500 mg twice a day.
Related Knowledge Centers
- B Cell
- Immunoglobulin M
- Lymphoproliferative Disorders
- Precancerous Condition
- Protein Isoform
- White Blood Cell
- Cancer
- Multiple Myeloma
- NON-Hodgkin Lymphoma
- Plasma Cell Dyscrasias