Primary spleen extranodal NK/T cell lymphoma, nasal type, with bone marrow involvement and CD30 positive expression: a case report and literature review

Abstract

AimsPrimay spleen NK/T cell lymphoma is very rare. We report a case of 39-years-old male of primary splenic NK/T cell lymphoma with bone marrow involvement and CD30 positive expression.Case descriptionThe patient had high fever for 2 months, and CT scan revealed a diffuse splenomegaly without hepatomegaly. The diagnosis was established by splenectomy specimen and bone marrow biopsy. Normal spleen structure was destroyed by the diffusely infiltrated neoplastic cells, and one of the splenic hilar lymph nodes was involved. The lymphomatous cells were mainly medium-sized, mixed with small and large cells with pleomorphic nuclei and conspicuous nucleoli. Angiocentric growth pattern was present, with mitotic figures and apoptotic bodies easily being found. These neoplastic cells demonstrated a typical immunophenotype of CD2, CD3ε, CD7, CD4, CD56, TIA-1, Granzyme B, CD30 positive, and CD5, CD8, CD20, CD79a negative. The Epstein-Barr virus encoded RNAs (EBERs) genomes were also found in tumor cells by in situ hybridization, while no clonal rearrangement of the T cell receptor-γ genes (TCRG) was found. Biopsy of bone marrow revealed scattered atypical cells presented with a predominantly intrasinusoidal distribution. A diagnosis as primary spleen NK/T cell lymphoma, nasal type (ENKTL) with CD30 expression and bone marrow involvement was finally made. The patient received chemotherapy and was still alive 6 months after splenectomy.Clinical significancePrimary spleen ENKTL is very rare, it should be made with the combination of clinical feature, PET-CT image, and pathological characteristics, and should be distinguished from other lymphomas or leukemia involved in spleen.Virtual SlidesThe virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/13000_2014_169

Malignant effusions from extranodal NK/T‐cell lymphomas are frequently of anaplastic morphology with azurophilic granules and of T‐cell lineage

“…This expression rate is higher than the 30% in the WHO book of ENKTL . In de novo ENKTL, large cell morphology usually correlates with CD30 expression; and in some studies, a poorer prognosis . In this current study, the lymphoma cells of effusion ENKTL are usually large and anaplastic, which is distinct from de novo ENKTL.…”

“…10 Radiologic studies showed no evidence of lymphadenop- In de novo ENKTL, large cell morphology usually correlates with CD30 expression; and in some studies, a poorer prognosis. [35][36][37] In this current study, the lymphoma cells of effusion ENKTL are usually large and anaplastic, which is distinct from de novo ENKTL. We recommend that CD30 immunostaining be performed for all effusion ENKTL cases; and target therapy with brentuximab vedotin, an anti-CD30 antibody-drug conjugate, might be considered a therapeutic option for those with CD30-expressing tumors.…”

Splenic diffuse large B-cell lymphoma. Case report

“…Pure extranodal presentation are infrequent (9% of cases). Follicular lymphomas that compromise the spleen can exhibit one of two histologic patterns 9,10,12 .…”

Aggressive natural killer (NK)‐cell leukaemia and extranodal NK/T‐cell lymphoma are two distinct diseases that differ in their clinical presentation and cytogenetic findings

“…As ENKTCL can progress to systemic disease and involve bone marrow, it might have considerable morphological and immunophenotypic similarities to ANKCL . It has been debated whether ANKCL is a real entity or simply represents a leukaemic phase of ENKTCL …”

“…1 As ENKTCL can progress to systemic disease and involve bone marrow, [2][3][4][5] it might have considerable morphological and immunophenotypic similarities to ANKCL. 11,12 It has been debated whether ANKCL is a real entity or simply represents a leukaemic phase of ENKTCL. 13,14 In order to clarify this, we studied cases of bone marrow NK/T-cell neoplasms by classifying them into groups without extramedullary tumour and with additional extramedullary tumour(s), and compared their clinicopathological features.…”