Immunoblastic Sarcoma Following Waldenström’s Macroglobulinemia

Choi, Young Hwan; Yeh, G. T.; Reiner, Leopold; Spielvogel, Arthur R.

doi:10.1093/ajcp/71.1.121

Cited by 26 publications

(8 citation statements)

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“…Similarly, patients with LPL/WM may develop DLBCL, as has been described in isolated case reports and small series. [2][3][4][5][6][7][8][9][10] However, a large series of these cases has not been reported. Furthermore, risk factors for developing DLBCL in patients with LPL/WM are poorly understood.…”

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confidence: 99%

Diffuse Large B-Cell Lymphoma Occurring in Patients With Lymphoplasmacytic Lymphoma/Waldenström Macroglobulinemia

et al. 2003

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Of 92 patients with lymphoplasmacytic lymphoma/Waldenström macroglobulinemia (LPL/WM) treated at our institution, diffuse large B-cell lymphoma (DLBCL) also developed in 12 (13%). In 10 patients, DLBCL developed 12 to 128 months (median, 44 months) after the diagnosis of LPL/WM. Two patients had LPL/WM and DLBCL simultaneously. Clinicopathologic features at diagnosis of LPL/WM did not predict the risk of DLBCL. Onset of DLBCL was characterized by worsening constitutional symptoms, profound cytopenias, extramedullary disease, and organomegaly. Immunoglobulin light chain expression was identical in both LPL/WM and DLBCL. In situ hybridization for Epstein-Barr virus (EBV) in 8 cases of DLBCL was negative. Of 11 patients with clinical follow-up information available, 8 (73%) died within 10 months of diagnosis of DLBCL. DLBCL, most likely as a result of histologic transformation, occurs in a subset of patients with LPL/WM and is associated with aggressive clinical course and poor outcome. EBV is unlikely to be involved in transformation.

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confidence: 99%

Diffuse Large B-Cell Lymphoma Occurring in Patients With Lymphoplasmacytic Lymphoma/Waldenström Macroglobulinemia

et al. 2003

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“…Although we expected to recover a mu ÷ B cell from a Waldenstr6m's-like lesion, we did not expect to clone a restricted subset of malignant Ly-1 + Qa-1 + T cells. The repetitive B-Ly-1 + Qa-1 + T cell association may explain why patients with Waldenstr6m's macroglobulinemia may develop immunoblastic cell sarcomas following aggressive therapy (37,38). In fact, we would predict, in this latter circumstance, that T cell IBL sarcomas would express the T3 T4 phenotype.…”

Section: These Results Demonstrate That a Series Of Malignant Ly-1 ÷ mentioning

confidence: 98%

Regulation of B cell lymphomagenesis by a malignant Qal+ inducer T cell clone.

Reinisch¹,

Sing²,

Bacon³

et al. 1984

The Journal of Experimental Medicine

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Understanding of the immunobiology of leukemias and lymphomas has been considerably aided by two concomitant developments--the generation of a series of monoclonal antibodies that define specific surface antigens on tumor cells and the identification and characterization of sets of lymphocytes with highly specific immunologic functions.Immune regulation of chronic B cell lymphomagenesis has been particularly difficult to approach experimentally because few animal models exist. In the BCL 1 system (1-3), rapidly proliferating id + (idiotype) ~ B cells appear to depress the total number of both Ly T subsets in the spleen and LN as the tumor progresses (4). In the MOPC 315 myeloma model, id specific helper and suppressor T cells control the differentiation and maturation of MOPC 315 myeloma cells, both by cell contact and by factors secreted by both cell subsets (5-10). Both the BCL 1 and MOPC systems resemble the more rapidly proliferating B cell dyscrasias, rather than the chronic B cell lymphomas.To define the immunoregulatory cellular interactions that govern B cell lymphomagenesis, we have developed an experimental model of chronic B cell lymphoma induced by the retrovirus murine sarcoma-murine leukemia-Moloney (MSV-MuLV-M). The immunopathology of these B cell lymphomas is similar to Waldenstrom's macroglobulinemia in the human since the tumor-bearing C57B1/ 6 mice have elevated serum IgM, a high proportion of mu + B cells in the mesenteric lymph node and develop slowly proliferating plasmacytoid-lymphocytic lymphomas two years after injection with retrovirus. Repeated attempts by This research was supported by U.S. Public Health Services

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“…Although the appearance of 1L during the course of WM has been recognized in several reviews on WM (2, 3, 4, 6), well documented cases are rare. Table 1 summarizes the clinical and hematological characteristics of 14 cases reported in the literature (4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16) together with our own 2 cases. The length of the evolution of WM does not seem to influence the emergence of IL; in this sense, cases appearing from 3 months (2) to 16 yr (16) after the diagnosis of WM have been reported.…”

Section: Discussionmentioning

confidence: 99%

“…The most frequent clinical data that usually herald transformation to IL are a deterioration in the patient's performance status and rapid adenopathic growth; both of these occurred in our 2 patients. Although there are occasions when monoclonal IgM is maintained (3, 10,15), in most cases (S52,) it decreases during transformation ( 3 , 5, 6,7,8,9,11,12,14,16). Indeed, some authors have interpreted this decrease as a prelude to IL (2).…”

Section: Discussionmentioning

confidence: 99%