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Non-Hodgkin Lymphoma
Published in Pat Price, Karol Sikora, Treatment of Cancer, 2020
Piers Blombery, David C. Linch
Peripheral T-cell lymphoma accounts for approximately 5%–10% of cases of NHL in Western countries. They are a heterogenous group of lymphoproliferative disorders which are biologically diverse. This is reflected in their different clinical presentation as well as their prognosis and response to therapy. PTCL can be broadly separated into those that have primarily nodal, extra-nodal, and leukemic presentations. The nodal PTCLs can be further divided into four main subtypes: angioimmunoblastic T-cell lymphoma (AITL), anaplastic large cell lymphoma (ALCL), ATLL, and PTCL-NOS. Although this classification is largely based on clinical, morphological, and immunophenotypic features, GEP studies have validated this classification by demonstrating distinct patterns of gene expression in each group.82 Furthermore, GEP data have helped clarify the presence of various subtypes within the PTCL-NOS category (which is largely a diagnosis of exclusion), including a “cytotoxic” subtype with an apparently inferior outcome.82 Aggressive extra-nodal PTCLs include such entities as hepatosplenic T-cell lymphoma (HSTL), extra-nodal NK/T-cell lymphoma (nasal type), and intestinal T-cell lymphomas.
Gene Rearrangement in Leukemias and Lymphomas
Published in John T. Kemshead, Pediatric Tumors: Immunological and Molecular Markers, 2020
The isolation and characterization of the immunoglobulin and the T cell receptor recombinant DNA probes has been of value in many clinical problems studied by this methodology. The ability to discriminate between reactive and malignant lymphoid populations, the identification of the evolution of subclones of neoplastic clones, and the facility to determine whether one tumor has evolved into another are all of benefit to the clinician.23,31–33,94 However, the interpretation of data generated by this technology must be performed in the context of all the clinical, histological, and immunological information that is available. Sensitive assays are prone to artifacts and the neoplastic process itself may lead to malfunctions in the somatic recombination of Ig and TCR genes. If these factors are taken into consideration, the analysis of the genetic configurations of the Ig and TCR genes has a role to play in the diagnosis of lymphoproliferative disorders.
Lymphoma
Published in Anju Sahdev, Sarah J. Vinnicombe, Husband & Reznek's Imaging in Oncology, 2020
Sarah J Vinnicombe, Rodney J Hicks
The WHO classification recognizes four broad groupings associated with an increased incidence of lymphoma and lymphoproliferative disorders (1): Primary immunodeficiency syndromesInfection with HIVPost-transplant lymphoproliferative disordersIatrogenic immunodeficiency-associated lymphoproliferative disorders (e.g. from methotrexate)
Complement-directed therapy for cold agglutinin disease: sutimlimab
Published in Expert Review of Hematology, 2023
Cold agglutinin disease (CAD) is a rare autoimmune hemolytic anemia (AIHA) with autoantibodies produced by a distinct low-grade clonal B-cell lymphoid proliferation in the absence of underlying disease [1–4]. Lymphoproliferative disorders are a group of heterogeneous diseases characterized by unregulated and excessive production of lymphocytes (i.e. B-cells, T-cells, or natural killer cells), which may be caused by iatrogenic or acquired genetic mutations [5]. CAD falls within the category of small B-cell lymphoid neoplasms, which is the preferred terminology for low-grade B-cell lymphomas as they may not be indolent. However, CAD does not fulfill the criteria of a B-cell lymphoma and is instead described as a B-cell lymphoid proliferation. CAD is distinct from lymphoplasmacytic lymphoma and unlike lymphoplasmacytic lymphoma or marginal zone lymphoma it does not transform into large cell lymphoma (Table 1) [1,2,6,7]. CAD is distinguishable from cold agglutinin syndrome (CAS), which is characterized by the presence of cold agglutinin antibodies secondary to underlying conditions such as malignancy or infection [8]. Primary CAD accounts for about 15–25% of AIHAs and has a prevalence of 16/1,000,000 in Europe [3,9–11].
The great masquerade: A rapidly growing pulmonary nodule
Published in Canadian Journal of Respiratory, Critical Care, and Sleep Medicine, 2022
Monica Mullin, Arjun Mehta, Stephanus Van Eeden, Janice Leung
This case highlights the limitations of small tissue samples in the diagnosis of lymphoproliferative disorders. The final diagnosis was a rare entity that is commonly misdiagnosed.4 DLBCL-spindle cell variant is a very rare diagnosis and not commonly on the differential for tumors with spindle cells, given only approximately 20 cases have been reported in the literature.5 The spindle cell variant of DLBCL is not a specific subtype accepted by the current World Health Organisation (WHO) classification of non-Hodgkin’s lymphoma. Rather, it represents an unusual morphological variant of DLBCL. This may explain the under-reporting of this morphological variant in the pathology literature. Lymphoma can also have a variety of presentations in the lungs, but DLBCL is rarely diagnosed from pulmonary nodules6–8 and in a case series with cavitating pulmonary nodules including 10 patients, it required multiple biopsies to obtain the correct diagnosis.9 The most common presentation of DLBCL-spindle cell variant includes extra nodal involvement, which is often the spleen.5 Lung metastases from DLBCL spindle cell variant have not been reported and to our knowledge, it has not been diagnosed via lung nodule biopsy.
A rare case of idiopathic multicentric Castleman disease in a patient with long-standing systemic autoimmunity
Published in Scandinavian Journal of Rheumatology, 2022
M Saleh, M Hallbeck, C Sjöwall
This woman, with a 25 year history of Sjögren-like lupus, developed abdominal lymphadenopathy, irresponsive to anti-rheumatic therapy, combined with constitutional symptoms, hypergammaglobulinaemia, and severe cytopenia. Extensive investigation was necessary to exclude malignancy (particularly lymphoma), but the case illustrates that non-malignant lymphoproliferative disorders should also be considered. The diagnosis was facilitated by radiology, demonstrating multiple locations with lymphadenopathy combined with typical histopathology (major criteria). Additional clinical and laboratory findings (minor criteria) were also met to satisfy the diagnostic international consensus criteria for iMCD (8–10). In line with the international, evidence-based consensus treatment guidelines for iMCD, IL-6 inhibition was initiated (10). The patient has received a first infusion of tocilizumab without side-effects, and additional infusions are planned for every fourth week. The plan is further to evaluate the efficacy of tocilizumab with a new PET scan within 6–12 months.