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Biomedical subjects

Grzegorz Rymkiewicz

Publications and source records attributed to Grzegorz Rymkiewicz.

6 recordsLinked to original sources

Unusual IgD+/CD38-follicular lymphoma with leukemic presentation.

Follicular lymphoma (FL) is a low-grade lymphoma, with rare presentation of leukemic phase in peripheral blood at the diagnosis. We describe a 49-yr-old woman who developed leukemic phase of FL in a 3 mo period after histological diagnosis of peripheral lymph node. To confirm the final diagnosis, flow cytometry (FCM) of peripheral blood, nested PCR with bcl-2 rearrangement, and cytogenetic analysis of peripheral blood and bone marrow cells were done. Lymphoma cells were negative for CD38 and expressed monoclonal surface immunoglobulins with relatively strong and "bright" IgD and "dim" kappa-chain by FCM analysis. Although the patient presented with generalized lymphadenopathy, massive peripheral blood and bone marrow involvement, she achieved complete clinical response after first-line chemotherapy COP and rituximab. She is still in a good condition with follow up over 2 yr.

ADP-ribosyl Cyclase 1↗

Unusual cyclin D1 positive marginal zone lymphoma of mediastinum.

We report a case of 43-yr-old Caucasian female with an unusual, cyclin D1 positive marginal zone lymphoma (MZL) of mucosa-associated lymphoid tissue (MALT) type of the mediastinum. To date, only about 30 cases of this entity have been published. They occur mainly in Asian females with a history of coexisting autoimmune disease. To our knowledge, this is the first case of mediastinal MZL with cyclin D1 expression. In the span of 6 yr this patient's tumor recurred three times, was surgically treated, and initially diagnosed as paraganglioma. The diagnosis was based on histopathological examination only. Our final diagnosis of MZL was made by combined evaluation of histopathology (HP), immunohistochemistry (IH), flow cytometry (FCM), fluorescence in situ hybridization (FISH), and molecular biology studies. We found a positive cyclin D1 reaction by IH and cyclin D1 mRNA (CCND1) overexpression by reverse transcription polymerase chain reaction (RT-PCR). Very high cyclin D1 to beta-actin mRNA ratio in this case was comparable with the ratio, characteristic for mantle cell lymphoma (MCL). However, there was no translocation t(11;14) found by FISH and an immunophenotype by IH and FCM was consistent with MZL ruling out MCL diagnosis. In addition, our case differs from other, previously reported thymic MZL lymphoma cases by no autoimmune disease association, Caucasian origin, and the absence of the plasmacytic differentiation on both HP/IH.

Adult↗

Transformation in lymphomas--morphological, immunophenotypic and molecular features.

During the course of lymphoma, a clinically more aggressive process with different morphology may develop, referred to as lymphoma transformation. Clonal relationship and pathogenic mechanism of this process are widely debated. The aim of the study was to evaluate morphology, immunophenotype (including EBV status) and clonal relationship in nine cases of lymphoma transformation. Among the six patients with low grade B-cell lymphomas three transformed into high grade B-cell lymphomas (two into diffuse large B-cell lymphoma, one into Burkitt lymphoma) and three into Hodgkin lymphoma. Three other patients with Hodgkin lymphoma presented with transformation into diffuse large B-cell lymphoma in two patients and peripheral T-cell lymphoma in one patient. In all cases there was a sudden clinical change as well as change in morphology and phenotype. In five of the nine patients studied EBV-LMP1 was demonstrated by immunohistochemistry in large transformed lymphoma cells. In two cases molecular studies revealed a different pattern of immunoglobulin gene rearrangement in the large transformed cells as compared to the small cells of primary indolent lymphoma. Thus, they represented secondary, arising de novo neoplasm.

Adult↗

Frequent aberrations of chromosome 8 in aggressive B-cell non-Hodgkin lymphoma.

Translocations involving chromosome 8 are the most common aberrations in B-cell non-Hodgkin lymphoma (B-NHL). The presence of the typical t(8;14)(q24;q32) or its variants has been confirmed in all cases of Burkitt lymphoma (BL), in some cases of Burkitt-like lymphoma (BLL), and in diffuse large B-cell lymphoma (DLBCL). The alterations lead to deregulated expression of c-myc protein by a chromosomal translocation joining C-MYC gene with sequences from immunoglobulin (Ig) enhancers. The C-MYC gene rearrangement plays an essential role in leukemogenesis of BL and probably plays a part in other aggressive NHLs. The present study was undertaken to compare the cytogenetic features in cases of BL, BLL, and DLBCL. We detected chromosomal aberrations by G-banding and fluorescence in situ hybridization (FISH) painting in 10 cases of aggressive B-NHL and used FISH to visualize the C-MYC gene rearrangement. Chromosome 8 was most frequently involved in structural aberrations (8/10 cases), and 4 cases showed the typical t(8;14)(q24;q32). Only two of 5 patients suspected of having BL fulfilled all the criteria for this diagnosis; in the others, chromosome 8 was aberrant, but the absence of C-MYC rearrangement or the results of flow cytometry excluded the diagnosis of BL. All BLL cases showed C-MYC overexpression, but only one had a rearrangement of the C-MYC gene; the remaining cases showed other aberrations of chromosome 8. This study indicates that the mechanisms of C-MYC activation involved in BLL can be different from that for the BL.

Adolescent↗

Mantle cell lymphoma presenting with paraproteinemia.

Mantle cell lymphoma (MCL) is most frequently diagnosed by the routine histological (HP) and immunohistochemical (IH) examination. Herein we present a case of 47-yr-old female with general lymphadenopathy and leukemic blood picture. She was initially diagnosed with marginal zone lymphoma (MZL). This diagnosis was based not only on the HP/IH examination but also on the presence of IgM paraproteinemia. Flow cytometry (FCM) examination was helpful in making of the final diagnosis of MCL. Fluorescence in situ hybridization and cyclin D1 mRNA detection by RT-PCR additionally confirmed the MCL diagnosis. The IgM paraproteinemia is rare in MCL, although is a common feature of lymphoplasmacytoid lymphomas (LPL) and is considered a major characteristic of Waldenström's macroglobulinemia (WM).

DNA, Neoplasm↗

Significance of chromosomal markers in the diagnosis of mantle cell lymphoma (MCL).

According to the REAL/WHO classification, the diagnosis of mantle cell lymphoma (MCL) should be based on clinical, histopathological, immunological and cytogenetic or molecular data. This study is based on 13 cases, which were initially diagnosed as MCL with the use of conventional cytogenetic method and fluorescent in situ hybridization (FISH). MCL is associated with a specific cytogenetic aberration t(11;14)(q13;q32). The chromosomal analyses confirmed the MCL diagnosis in four cases. A neartetraploid cell line and two copies of t(11;14) were observed in three cases. These results correspond with a blastoid variant of MCL, accompanied by aggressive course and poor prognosis. The presence of karyotype with t(11;14) as the sole anomaly predicts an intermediate clinical outcome. Six patients had normal karyotypes, which is characteristic for the typical form of MCL, associated with a better prognosis. In this study we show that detection of chromosomal abnormalities is useful in diagnosis of MCL and has some prognostic significance.

Chromosomes, Human, Pair 11↗