A rare case of de novo CD5+ diffuse large B-cell lymphoma in leukemic phase and positive for CD13

Abstract

We report a case of de novo diffuse large B-cell lymphoma (DLBCL) in leukemic phase, positive for both CD5 and CD13. Morphologic evaluation, flow cytometric immunophenotyping, karyotyping and polymerase chain reaction studies were performed. Neoplastic lymphocytes appeared as blast-like cells, positive for CD19, CD20, CD5, CD13, CD79a, HLADR, and with restriction for surface immunoglobulin K light chains. Rearrangement of IgH gene, BCL2/IgH translocation and complex karyotype were found. The patient was treated with RCOMP regimen and achieved complete remission. However, only one month after the first restaging of disease, the patient presented with symptoms attributable to central nervous system involvement and her clinical conditions worsened rapidly. While both CD5 expression and leukemic presentation are uncommon findings in DLBCL, positivity for CD13 is very rare. The outcome of our patient shows the poor prognosis of CD5+ DLBCL with leukemic presentation. The possible role of CD13 coexpression is discussed.

A Clinical Triad with Fatal Implications: Recrudescent Diffuse Large B-cell Non-Hodgkin Lymphoma Presenting in the Leukemic Phase with an Elevated Serum Lactic Acid Level and Dysregulation of the TP53 Tumor Suppressor Gene – A Case Report and Literature Review

“…Using a Medline search with the key words “diffuse large B-cell lymphoma AND leukemic/leukemic phase” and “diffuse large B-cell lymphoma AND leukemic/leukemic presentation”, we identified a total of 16 case reports and 2 case series of de novo or relapsed DLBCL presenting in the leukemic phase that were published in English ( Table 1 ). 7 - 24 In one case series describing the clinical course of 40 individuals with leukemic presentations of de novo DLBCL, 32 (80%) patients died with a median follow-up of 18 months. 23 The median follow-up for the 8 surviving patients was 34.5 months.…”

“…In addition to lymphoma involvement in the bone marrow and peripheral blood, many of these patients also manifested with other sites of extra-nodal disease, including spleen, lung, liver, bone, cerebral spinal fluid, bowel, kidney, pancreas, adrenal gland, and testes. 9 , 17 , 19 , 22 , 24 The diversity of organ involvement may be explained by the high tumor burden associated with circulating malignant cells and the differential expressions of adhesion molecules on cell surfaces. 25 The imbalance of adhesion molecule expression in tumor cells leading to peripheral blood involvement is well described in chronic lymphocytic leukemia and intravascular B-cell lymphoma.…”

CD5+ Diffuse Large B-Cell Lymphoma With Leukemic Transformation: A Rare Case With Central Nervous System Involvement, Treated With R-CHOP and Intrathecal Methotrexate/Cytarabine

“…The literature is also unclear as to the optimal treatment regimens for the combined presentation of CD5+ DLBCL with leukemic transformation. An increasing number of case reports separately detail both DLBCL with leukemic transformation [7][8][9][10][11] and CD5 positivity [4,12], but mentions of dual presentation have been limited, as have case series and larger studies [5][6]. The authors of these reports, where treatment is discussed, ultimately favor an anthracycline-based treatment regimen, often paired with rituximab, though R-hyper-CVAD with alternating IT MTX/cytarabine [13] has also been reported.…”

“…The CD5 positivity is of particular concern, as it has been posited to support B-cell survival and decrease feedback mechanisms that can lead to cell death [ 3 ]. It is also more likely to present in an advanced clinical stage after CNS involvement and carries a poorer prognosis due to a less favorable response to chemotherapy [ 4 ].…”

Leukemic High Grade B Cell Lymphoma is Associated With MYC Translocation, Double Hit/Triple Hit Status, Transformation, and CNS Disease Risk: The Mayo Clinic Experience

“…Other reports of L-DLBCL are confined primarily to case reports. [18][19][20][21][22] These reports focus on LI at the time of original DLBCL diagnosis, and not during relapse. Only one report seems to have documented L-DLBCL at relapse.…”