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The Non-Hodgkin’s Lymphomas and Plasma Cell Dyscrasias
Published in Harold R. Schumacher, William A. Rock, Sanford A. Stass, Handbook of Hematologic Pathology, 2019
Lynne V. Abruzzo, L. Jeffrey Medeiros
This category in the Revised European-American Classification includes low-grade lymphomas that were originally designed as low-grade B-cell lymphoma of the mucosa-associated lymphoid tissue (MALT) and monocytoid B-cell lymphoma. Both MALT-lymphoma and monocytoid B-cell lymphoma are thought to represent different clinical presentations of the same B-cell neoplasm. Splenic marginal zone lymphoma is closely related to these neoplasms. Monocytoid B-cell lymphoma, low-grade B-cell lymphoma of MALT, and splenic marginal zone lymphoma are believed to arise from normal marginal zone B-cells in the lymph node, their extranodal counterparts, or spleen, respectively. A feature shared by all of the marginal zone B-cell lymphomas is that they have distinctive histologic findings that are not easily or reproducibly classified in previously published classification schemes.
Diagnosis of Leukemia, Lymphoma, and Myeloma
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
Other indolent lymphomas, such as splenic marginal zone lymphoma, present almost exclusively with a large spleen. Variants include extranodal marginal zone lymphoma (previously known as maltoma) which presents with symptoms related to affected sites. For example, the gastric variety, which is often associated with infection by Helicobacter pylori, presents with abdominal discomfort, feeling bloated, and heartburn. Cutaneous lymphomas, such as mycosis fungoides, affect the skin and present with widespread psoriatic-like lesions which progress to plaque and tumor formation over long periods of time, sometimes several years, but in the later stages affect lymph nodes, other organs and blood (such as in Sézary syndrome) (Fig. 4.41).
Case 100: An Unsolved Mystery
Published in Layne Kerry, Janice Rymer, 100 Diagnostic Dilemmas in Clinical Medicine, 2017
The haematology team reviewed the patient and reported that the findings were possibly in keeping with splenic marginal zone lymphoma. A bone marrow biopsy found no evidence of marrow involvement by lymphoma, leishmaniasis or amyloidosis. Immunochemistry showed normal numbers of interstitial B and T lymphocytes and plasma cells.
Splenic marginal zone B-cell lymphoma associated with ruptured breast implants: case report and review of the literature
Published in Case Reports in Plastic Surgery and Hand Surgery, 2021
Mark G. Evans, Melissa A. Mueller, Frederik Chen, Larry S. Nichter
Splenic marginal zone B-cell lymphoma is a rare malignancy, accounting for less than 2% of all lymphoma cases [12]. It was first described in 1992 and is now considered a separate entity in the World Health Organization (WHO) classification [13]. The three types of marginal zone B-cell lymphomas are splenic marginal zone lymphoma (SMZL), nodal marginal zone lymphoma (NMZL), and extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma). The diagnosis of SMZL is made based on lymphocyte morphology, immunophenotype, cytogenetic abnormalities, bone marrow histology, and spleen histology if available. When microscopic examination of the spleen is not possible, clinical splenomegaly and typical morphologic and immunophenotypic blood and bone marrow findings are sufficient to make a diagnosis.
Deciphering the genotype and phenotype of hairy cell leukemia: clues for diagnosis and treatment
Published in Expert Review of Clinical Immunology, 2019
Margot C.E. Polderdijk, Michiel Heron, Saskia Kuipers, Ger T. Rijkers
Maitre et al. [74] described many mutations that have not been associated with HCL before or at least to a very limited extent. The KDM6A mutation is located on the X chromosome, and could perhaps explain the male predominance of HCL. The mutation results in loss of function of a lysine demethylase protein, which may sensitize tumor cells to EZH2 inhibitors. The KLF2 mutation appears in 10–16% of patients. This gene is involved in inhibition of the NFκB pathway, as well as B-cell homing to lymph nodes. The mutations found are either in the zinc finger domain or nuclear localization signal, and both lead to loss of function of the protein. The exact mechanisms are unknown, but it is consistent with the NFκB activation that is reported in HCL [76]. The mutation is found also in splenic marginal zone lymphoma. U2AF1 splicing factor mutations are reported by Durham et al. [27], but only in HCLv. Similarly, these researchers found CCND3 mutations in 13% of HCLv patients, which led to loss of the PEST domain and increased expression of CCND3. Elevated levels of the related CCND1 mRNA have been found also, but it is not clear what the implications for this are [77]. The product of CCND1, cyclin D1, is sometimes used as a diagnostic marker for HCL.
Diagnostic and therapeutic splenectomy for splenic lymphomas: analysis of the National Cancer Data Base
Published in Hematology, 2019
Jaleh Fallah, Adam J. Olszewski
Splenic lymphomas range from indolent diseases like splenic marginal zone lymphoma (SMZL) and follicular lymphoma (FL) to aggressive entities like diffuse large B cell lymphoma (DLBCL) or hepatosplenic T-cell lymphoma [1–3]. Historically, splenectomy was the main diagnostic and therapeutic intervention for splenic lymphomas, but the availability of sensitive diagnostic imaging, flow cytometry, and biomolecular analysis has largely obviated the need for an invasive surgical approach to diagnosis or staging in lymphomas [2,4–7]. Rare subtypes like the splenic diffuse red pulp B-cell lymphoma may still require splenectomy for unequivocal diagnosis [8]. The fact that many splenic lymphomas involve the blood and bone marrow, and that they can be effectively treated with immunochemotherapy has further decreased the need for therapeutic splenectomy [9–12].