A Case of Synchronous Bone Marrow Chronic Myelomonocytic Leukemia (CMML) and Nodal Marginal Zone Lymphoma (NMZL)

Abstract

Patient: Male, 67Final Diagnosis: Nodal marginal zone lymphoma and chronic myelomonocyticSymptoms: Cervical lymphadenopathy and leukocytosisMedication: —Clinical Procedure: —Specialty: OncologyObjective:Rare co-existance of disease or pathologyBackground:Leukemias and lymphomas can arise from myeloid or lymphoid stem cells. Combined myeloid leukemia and non-Hodgkin’s lymphoma (NHL), either synchronous or metachronous, rarely occur in the same patient. This report is of a 67-year-old man with a synchronous diagnosis of both bone marrow chronic myelomonocytic leukemia (CMML) and nodal marginal zone lymphoma (NMZL), which a peripheral low-grade B-cell NHL.Case Report:A 67-year-old Caucasian man, who was a long-term cigarette smoker, presented with a five-year history of leukocytosis and cervical lymphadenopathy. He had no symptoms of night sweats, fever, or weight loss. Review of his medical records showed a progressively increasing leukocytosis with a peak of 58×109/L. Computed tomography (CT) imaging of the chest and abdomen showed lymphadenopathy, including enlarged cervical, axillary, mediastinal, and retroperitoneal lymph nodes. Bone marrow biopsy and histology showed CMML. Lymph node biopsy and histology showed NMZL. The patient was treated for NMZL with weekly intravenous rituximab infusions. Although his CMML was stable, the patient requested an evaluation for treatment with hematopoietic allogeneic stem cell transplantation (ASCT). At the time of this report, the patient remains asymptomatic.Conclusions:The synchronous occurrence of bone marrow CMML and NMZL in a single patient is rare and may be attributed to a genetic mutation common to both. There are no current treatment guidelines for this group of patients, and treatment strategies should be individualized to provide an optimum outcome or symptomatic improvement.

Proposed diagnostic criteria for classical chronic myelomonocytic leukemia (CMML), CMML variants and pre-CMML conditions

“…In a small group of patients with CMML, a co-existing lymphoproliferative neoplasm is diagnosed, such as a lymphocytic leukemia, non-Hodgkin lymphoma or multiple myeloma. 51-60 In most patients, the lymphoid neoplasm is detected first, and CMML is considered to develop as treatment-induced, secondary, leukemia. 51,57 In other patients, CMML is diagnosed first and later a lymphoid neoplasm is detected during follow-up.…”

The first description of a singular case of synchronous chronic myelomonocytic leukemia and diffuse large b‐cell lymphoma

“…The synchronous diagnosis of CMML and lymphomas is a quite rare event. Anecdotal cases of association with angioimmunoblastic T‐cell lymphoma, plasma cell disorders, and marginal B‐cell cell lymphoma have been described 12 . This is the first clinical description of association of CMML with DLBCL.…”

“…Anecdotal cases of association with angioimmunoblastic T-cell lymphoma, plasma cell disorders, and marginal B-cell cell lymphoma have been described. 12 This is the first clinical description of association of CMML with DLBCL. About 40% of CMML patients carry mutations in the chromatin remodeling gene ASXL1, 13 which confers inferior clinical outcome.…”

Occurrence of a Clonal T-Cell Population in a Case of Chronic Myelomonocytic Leukemia

“…Very rarely, patients with CMML have a co-existing lympho-proliferative disorder (LPD). 5 - 10 In most cases, the lymphoid neoplasm is diagnosed first, and the CMML is considered to be a secondary therapy-induced form of leukemia. 11 - 15 We report herein a unique case of de-novo CMML, with an underlying clonal T-cell population and describe its clinical presentation and laboratory findings.…”