A Case of Splenic Marginal Zone Lymphoma with Mismatched Morphology and Phenotype, Karyotype and Clinical Course

Case Report

Ann Hematol Oncol. 2015;2(1): 1016.

A Case of Splenic Marginal Zone Lymphoma with Mismatched Morphology and Phenotype, Karyotype and Clinical Course

Sorigue M1*, Juncà J2, Gassiot S1, Navarro JT2, Mate JL3 and Millà F2

1Department of Hematology, Hospital Gerans Trias i Pujol, Spain

2IJC, Hospital Germans Trias i Pujol, Spain

3Department of Pathology, Hospital Germans Trias i Pujol, Spain

*Corresponding author: Sorigue M, Department of Hematology. Hospital Germans Trias i Pujol, Ctra. Canyet s/n 08916 Badalona, Spain

Received: November 06, 2014; Accepted: January 05, 2015; Published: January 07, 2015


Background: Splenic marginal zone lymphoma is a rare chronic lymphoproliferative neoplasm with a very indolent clinical course and a noncharacteristic phenotype and karyotype. Peripheral blood morphology can be the first clue to the diagnosis.

Methods: Here, we report a case of a patient with splenic marginal zone lymphoma, alive 20 years after initial diagnosis, with very atypical and immature lymphocytes in peripheral blood smear since then.

Results: Peripheral blood phenotype and karyotype and pathological analysis of splenectomy sample, as well as the clinical evolution were compatible with splenic marginal zone lymphoma. Conversely, peripheral blood morphology was, from the start, atypical and suggestive of a high-grade lymphoproliferative disorder.

Conclusion: Integrating all clinical and laboratory data is essential to make an appropriate diagnosis and guide the therapeutic options offered to patients.

Keywords: Splenic marginal zone lymphoma; Atypical morphology; Indolent lymphoma


MZL: Marginal Zone Lymphoma; MALT: Mucosa-Associated Lymphoid Tissue; SMZL: Splenic Marginal Zone Lymphoma

Case Presentation

A 65 year-old woman was sent for hematologic evaluation in 1994 because of the incidental finding of peripheral blood lymphocytosis. She was asymptomatic. On physical examination splenomegaly, without hepatomegaly or any enlarged lymph nodes, was palpable. The peripheral blood smear revealed large-sized cells, with a wide nucleus occupying most of the cell space, with immature and reticulated chromatin, and one or more conspicuous nucleoli. In some cells the nucleus adopted an irregular form. The cytoplasm was basophilic without any granularity (Figure 1). However, phenotypical exam of the lymphocytes was compatible with SMZL and peripheral blood cytogenetic analysis revealed a complex karyotype, including a trisomy of chromosome 3 and an imbalanced traslocation involving chromosome 7q36. Methacrylate-embedded bone marrow showed a nodular and paratrabecular pattern of infiltration by small, mature and monomorphic lymphocytes. The diagnosis of SMZL was made.