Abstract
Diffuse large B-cell lymphoma (DLBCL) is a heterogeneous group of diseases of the lymphatic system, which is represented by de novo and secondary tumors resulting from the transformation of indolent lymphomas. In the absence of a long history of the disease at the stage of histological transformation (HT), it is difficult to distinguish between de novo and secondary diffuse large B-cell lymphoma. According to the data of a randomized study, we obtained clinical and laboratory data that are not typical for de novo diffuse large B-cell lymphoma. These include exclusive, predominant retroperitoneal localization, compression of the ureters/kidneys with or without the development of acute renal failure (ARF), unilateral lymphostasis of the leg due to compression of the inguinal, iliac lymph nodes by the conglomerate, intratumor in the central nervous system (CNS) at the onset/relapse/progression of the disease, discordant bone marrow involvement, blood involvement, paraprotein secretion.
Highlights
Diffuse large B-cell lymphoma (DLBCL) was isolated from the general group of lymphatic tumors by histological and immunotypic features—diffuse proliferation of large B-cells, which either can form a structure of diffuse growth or be diffusely scattered among nontumor cells of a specific organ [1, 2].In the past, lymphomas were classified solely for histological features
Five patients had preferential/exclusive retroperitoneal localization, and three of them had data of renal/ureteral compression—acute renal failure (ARF) and hydronephrosis. In two of these three cases, it combined with the presence of Mk paraprotein secretion, as well as with discordant lesion of the bone marrow. All this allows us to assume the transformation of indolent lymphomas into DLBCL
Neuroleukemia was diagnosed in one patient, no one initially had intratumor, and one patient had a relapse, and this patient was diagnosed with primary mediastinal large B-cell lymphoma (PMBL)
Summary
Diffuse large B-cell lymphoma (DLBCL) was isolated from the general group of lymphatic tumors by histological and immunotypic features—diffuse proliferation of large B-cells, which either can form a structure of diffuse growth or be diffusely scattered among nontumor cells of a specific organ [1, 2].In the past, lymphomas were classified solely for histological features. The advent of immunophenotyping of tumor cells changed the situation, but it turned out that some lymphoproliferative diseases (LPD), including DLBCL, do not have an unambiguous specific immunophenotype. Cytogenetic disorders made it possible to distinguish a number of diseases of the lymphatic system into separate nosological forms. These include c-MYC gene rearrangement and 11q aberration, characteristic of Burkitt’s lymphoma; rearrangement of gene BCL-2, Lymphoma characteristic of follicular lymphoma (FL); the CYCLIN D1 gene, characteristic of mantle cell lymphoma; t(2;5) (p23;p35); characteristic of anaplastic T-cell lymphoma, etc. The combination of rearrangement of the c-MYC gene with rearrangement of the BCL2 and/or BCL6 genes in the recent past made it possible to isolate a separate form of high-grade B-cell lymphoma from DLBCL [6–11]. The diagnosis was made after excluding all other large B-cell lymphomas
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