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Assessment of response to treatment
Published in Anju Sahdev, Sarah J. Vinnicombe, Husband & Reznek's Imaging in Oncology, 2020
Lawrence H Schwartz, Binsheng Zhao, Marius E Mayerhoefer
Based on the initial International Working Group (IWG) criteria for assessment of treatment response in lymphoma (which are also known as the Cheson criteria), the Lugano classification was published in 2014. Since the vast majority of lymphoma subtypes—including Hodgkin lymphoma, diffuse large B-cell lymphoma as the most common aggressive NHL, and follicular lymphoma as the most common indolent NHL—are FDG-avid, the Lugano classification recommends the use of 18FDG PET-CT for response assessment in lymphomas (23). For the few subtypes with variable FDG-avidity, which include mucosa-associated lymphoid tissue lymphoma (MALT) and other marginal zone lymphomas (MZL), small cell lymphocytic lymphoma/chronic lymphocytic leukaemia (SLL/CLL), and lymphoplasmacytic lymphoma (WaldenstrÖm's macroglobulinaemia), CECT is presently recommended, even though some studies have suggested that DWI could be better suited for this task, in particular for MALT lymphoma (48,49). The Lugano CT response criteria are more similar to the WHO criteria than to the RECIST criteria, because bidimensional, rather than unidimensional, measurements of up to six target lesions are used. Based on these bidimensional measurements, the sum of the products of diameters (SPD) is calculated, and a 50% cutoff is used to distinguish between partial response (≥50% SPD decrease, without new lesions), stable disease (<50% SPD decrease, without new lesions), and progression (increase ≥50% SPD, or new lesions); for CR, complete resolution of all extranodal lesions, and regression of nodes to ≤1.5 cm long axis diameter, is required.
Different Types of Leukemias, Lymphomas, and Myelomas
Published in Tariq I Mughal, John M Goldman, Sabena T Mughal, Understanding Leukemias, Lymphomas, and Myelomas, 2017
Tariq I Mughal, John M Goldman, Sabena T Mughal
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).
Multiple myeloma
Published in Pat Price, Karol Sikora, Treatment of Cancer, 2014
There have been a small number of reports of myeloma and MGUS developing in two or more members of one family, but it is unclear to what extent this reflects genetic or environmental factors.20 Epidemiological studies have shown familial clustering21 and a significantly increased incidence of MGUS, myeloma, chronic lymphocytic leukaemia and lymphoplasmacytic lymphoma in first degree relatives of individuals with MGUS reflecting the likely influence of shared genetic and environmental factors in the pathogenesis of these diseases.5,165 Conclusive evidence for a genetic variation predisposing to myeloma has now come from molecular epidemiological studies that have identified genetic loci associated with an increased risk of developing myeloma22 and further studies will explore the impact of genetic loci in disease pathogenesis and treatment.
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.
Strategies to overcome the diagnostic challenges of autoimmune hemolytic anemias
Published in Expert Review of Hematology, 2023
Wilma Barcellini, Bruno Fattizzo
As mentioned before, bone marrow compensation, as highlighted by reticulocytosis, is a major determinant of AIHA severity. The evaluation of endogenous erythropoietin levels is a useful test in patients with inadequate reticulocytosis and may prompt the administration of recombinant erythropoietin agents that may improve anemia in >70% of cases [56,57]. Bone marrow evaluation (morphology, cytometry, cytogenetics, and biopsy) has been recently advised in CAD at diagnosis and in wAIHA relapsed after or refractory to first-line therapy with steroids [4,25,58]. Along with whole body CT scan, it is pivotal to diagnose AIHA associated with lymphoproliferative diseases, as well as to myelodysplasia or bone marrow failure syndromes. Moreover, the type of lymphocyte infiltrate (T or B) may be helpful to choose a better target-therapy [59,60]. CAD deserves a specific mention, since a clonal, low-grade lymphoproliferation distinct from lymphoplasmacytic lymphoma and marginal zone lymphoma, is almost invariably present [25,49,52]. It has been currently included in the World Health Organization (WHO) classification of hematolymphoid tumors [61]. Molecular analysis may be useful, since the MYD88 L265P mutation, typical of lymphoplasmacytic lymphoma/Waldenström’s macroglobulinemia, is usually negative in CAD, whilst KMT2D and CARD11 mutations have been described [62,63]. Further molecular analysis and next-generation sequencing would help to confirm associated conditions in selected cases (congenital anemias, immunodeficiencies, myelo- and lympho-proliferative disorders) [64].
Chemokine receptor CXCR4: An important player affecting the molecular-targeted drugs commonly used in hematological malignancies
Published in Expert Review of Hematology, 2020
Liangliang Li, Ye Chai, ChongYang Wu, Li Zhao
This indolent B-cell malignancy is a rare subtype of lymphoplasmacytic lymphoma characterized by the presence of immunoglobulin M (IgM)-secreting lymphoma cells in the bone marrow, and most Waldenstrom’s macroglobulinemia (WM) patients (80%–95%) have myeloid differentiating factor 88 (MYD88) mutations [23]. Currently, there is no standard of care for WM. For most patients, rituximab-based chemoimmunotherapy regimens provide durable responses [24]. However, WM cells gradually appear refractory to rituximab. Furthermore, rituximab intolerance is being found in increasing proportions of WM patients [25,26]. Thus, new therapeutic choices are urgently needed. As a novel therapeutic option, the BTK inhibitor ibrutinib has been approved for the treatment of newly diagnosed and relapsed/refractory WM patients, and successful advances have been obtained. However, MYD88 and CXCR4 mutational status in WM patients affects the clinical response to ibrutinib [27].