Diffuse large B-cell lymphoma presenting in the leukemic phase

Abstract

Diffuse large B-cell lymphoma (DLBCL) is the most common non-Hodgkin lymphoma comprising a heterogeneous group of disorders with variable histological and clinical behavior. Although other lymphomas may present in the leukemic phase more frequently, this appearance is unusually observed among DLBCL cases. Diagnosing lymphoma is not always easy, and the patient's clinical status quite often may hamper invasive procedures for diagnosis pushing the clinician to look for alternatives to reach the nearest possible accurate diagnosis. The authors report the case of a middle-aged man who presented the history of malaise, weight loss, and low-grade fever. The peripheral blood count showed leukocytosis with the presence of blasts and thrombocytopenia. The cytological morphology and immunophenotyping of the peripheral blood and bone marrow aspirate, as well as the bone marrow biopsy accompanied by a thorough immunohistochemical analysis, rendered the diagnosis of DLBCL in the leukemic phase. The patient was prescribed R-CHOP with a favorable outcome. Intra-abdominal lymph node biopsy was avoided because of the patient's critical medical condition. The authors highlight this rare form of presentation of DLBCL as well as the combination of peripheral blood, bone marrow aspirate, and bone marrow biopsy for reaching the diagnosis in cases were a lymph node sample is unavailable for the diagnostic work-up.

Leukemic phase and CSF involvement of diffuse large B‐cell lymphoma with a complex karyotype including a TP53 deletion

“…Leukemic presentation of DLBCL is a rare entity that often presents with significant extranodal involvement . This patient with TP53 deletion and complex cytogenetics received R‐Hyper‐CVAD chemotherapy with twice‐weekly intrathecal (IT) therapy (alternating methotrexate and cytarabine) for two cycles.…”

Leukemic presentation of high-grade B cell lymphoma with MYC and BCL2 rearrangement—a series of two cases and review of literature

“…Sarkozy et al [8] have described 37 cases of follicular lymphoma with leukemic phase at diagnosis which was associated with poor prognosis. Similarly, authors have described cases of DLBCL [9,10], angioimmunoblastic T cell lymphoma [3] and anaplastic large cell lymphoma [11] with an unusual overt leukemic phase at diagnosis. Peripheral blood lymphocytosis at initial presentation is seldom described in high-grade B cell lymphoma with MYC and BCL2 rearrangement [12].…”

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.…”