Metachronous Marginal Zone Lymphoma Followed by a Peripheral T-Cell Lymphoma in the Same Patient. Report of Two Cases and Review of the Literature

Case Report

Austin Hematol. 2021; 6(1): 1031.

Metachronous Marginal Zone Lymphoma Followed by a Peripheral T-Cell Lymphoma in the Same Patient. Report of Two Cases and Review of the Literatures

Condom M1,4*, Climent F2, Varela M2, Gonzalez-Barca E1,3,4, Maluquer C1,4, Sureda A1,3,4 and Domingo-Domenech E1,4

1Department of Hematology, Catalan Institute of Oncology, Spain

2Department of Pathology, Bellvitge University Hospital, Spain

3Barcelona University, Spain

4Institut d’Investigació Biomèdica (IDIBELL), Spain

*Corresponding author: Maria Condom, Department of Hematology, Catalan Institute of Oncology, Hospital Duran i Reynals, Avinguda de la Gran via de l’Hospitalet, 199-203, 08908 L’Hospitalet de Llobregat, Barcelona, Spain

Received: January 12, 2021; Accepted: February 03, 2021;Published: February 10, 2021


Marginal Zone Lymphomas (MZL) and Peripheral T Cell Lymphomas (PTCL) are uncommon neoplasms and patients affected by both lymphomas in a lifetime have been rarely reported. Here we present two patients with an initial diagnosis of a MZL who further developed a PTCL. We also present the review of the literature of six other cases of MZL. Half of the cases reported are synchronous and the other half metachronous. Most MZLs reported are MALT subtype. There is no predominant subtype amongst PTCLs. Chronic exposure to proinflammatory cytokines, genetic predisposition or exposure to carcinogenic agents could be possible causes underlying this rare phenomenon.

Keywords: Marginal zone lymphoma; Peripheral t-cell lymphoma; Synchronous; Metachronous

Case 1

A 45-year-old man with no previous medical records presented with asthenia in May 2014. Laboratory tests revealed iron-deficiency anemia. Serologies and autoimmunity tests including celiac disease study where negative. Thoracic-abdominal CT scan showed an enlargement of the first portion of the duodenum and enlarged mesenteric lymph nodes of 2cm. Helicobacter Pilory (HP) was negative. The duodenum biopsy showed a prominent lymphoid infiltrate involving the lamina propia. Immunohistochemistry markers were positive for B-cell and negative for CD3, CD5, CD10, BCL6 and cyclin D1. Ki67 index was <5%. IGH showed a clonal rearrangement. Bone marrow was not infiltrated. The diagnosis of Mucosa Associated Lymphoma Tissue (MALT) lymphoma stage II1E, with duodenal involvement was made. The patient received 3 cycles of R-CHOP achieving a complete response. In March 2016, he relapsed with multiple enlarged abdominal lymph nodes. Duodenum biopsy showed a MZL. TCR came out polyclonal. He received 4 cycles of Rituximab and Fludarabine, achieving a partial remission. On January 2018, he presented with abdominal pain. PET-CT scan showed an increased metabolic activity of the colon, and enlarged mesenteric lymph nodes (SUV max 17). Unfortunately, the patient presented rapid disease progression and died before a diagnostic biopsy could be performed. Necropsy showed a mesenteric mass also affecting the small bowel and right colon wall. Microscopically, it showed a diffuse infiltration of the serosa and the intestinal wall by medium-large cells that were positive for CD3, CD4, CD5, CD7, BF1 and cytotoxic markers (TIA and Granzyme B), and negative for CD8. CD30 was extensively positive especially in the larger cells. Ki67 index was 90%. B-cell markers, ALK and EBERs were negative. Molecular studies showed a clonal rearrangement of the TCR beta. IGH rearrangement was polyclonal. Differential diagnosis included ALK negative anaplastic large cell lymphoma and monomorphic epitheliotropic intestinal T-cell lymphoma, but morphologic and immunophenotypic analysis did not meet diagnostic criteria. Finally, a diagnosis of PTCL-NOS was made.

Case 2

A 62-year-old woman with no previous medical records presented with a right ocular mass and enlarged supraclavicular lymph nodes in October 2003. Blood tests were within normal ranges. Thoracicabdominal CT scan showed no relevant findings. Lachrymal gland biopsy showed infiltration by MZL. Bone marrow was infiltrated. She was diagnosed with stage IVA MALT lymphoma and received Chlorambucil 2mg/day for a year with clinical resolution of both ocular mass and lymph nodes.

She remained uneventful until 2017, when she presented with a cervical enlarged lymph node. PET CT-scan showed supra- and infradiaphragmatic lymph nodes (SUVmax 12.5). Cervical lymph node biopsy was diagnostic of a PTCL, with T-follicular helper phenotype. EBERs was negative and TCR was clonal. B-cell markers and IGH rearrangement showed a small residual B-cell clonal population. She was started on CHOP chemotherapy, but after six cycles she presented with progressive disease. She was then treated with Bendamustine but had no response. She received salvage therapy with GEMOX, with progressive disease including multiple bones lesions. Unexpectedly, a biopsy of one of these lesions showed an aggressive B-cell lymphoma. Clonality studies showed that this lymphoma was related to the previous MALT lymphoma. The patient deceased shortly after the diagnosis.


MZL and PTCL are rare neoplasms. The coexistence of both in the same patient cannot be explained by pure randomness, since the probability of these tumors is very low in our population. According to local registries [1], Marginal Zone Lymphomas and T/NKcell lymphomas have an incidence of 2.59 and 2.01/100.000/year, respectively. In our area, the chances of developing both neoplasms within a 15 year-period of time are approximately 0.000012%. To our knowledge, only six cases of MZL and PTCL occurring in the same patient have been reported before in the literature [2-7]. The characteristics of these patients are summarized in (Table 1) (synchronous) and (Table 2) (metachronous).

Citation:Condom M, Climent F, Varela M, Gonzalez-Barca E, Maluquer C, Sureda A, et al. Metachronous Marginal Zone Lymphoma Followed by a Peripheral T-Cell Lymphoma in the Same Patient. Report of Two Cases and Review of the Literature. Austin Hematol. 2021; 6(1): 1031.