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Ocular adnexal marginal zone B-cell lymphoma (OAMZL) of the mucosa-associated lymphoid tissue is a distinct subtype of B-cell lymphoma. OAMZL occasionally occurs on both sides with a varied sequence in the time course. However, few case reports have described clonal analysis of bilateral OAMZ. Here we present a case of biclonal OAMZL, that developed bilaterally at a 2-year interval. A 38-year-old woman was diagnosed with OAMZL in the right lower eyelid conjunctiva and received local radiation therapy, resulting in the disappearance of the tumor. Two years later, she developed another tumor in the left lower eyelid and was diagnosed with relapse of OAMZL. She was re-treated successfully with radiation therapy. Analysis of immunoglobulin (Ig) gene rearrangement in the bilateral tumor samples showed different clonotypic VDJ recombination within the Ig heavy chain gene and different patterns of rearrangement of the Ig light chain genes. The results indicated that independent B-cell clones causing the specific subtype of lymphoma had generated in both eyes. The biclonal nature of the lymphoma that developed sequentially in the same anatomic site in this case suggests that underlying inherent or environmental factors may lead to ongoing emergence of new tumor clones., (© 2024. Japanese Society of Hematology.)

We report a patient with hairy cell leukemia Japanese variant (HCL-Jv) that developed after radiotherapy for orbital adnexal MALT lymphoma. A 78-year-old man was diagnosed as having MALT lymphoma in the left conjunctiva in December 2003. The patient was treated by local radiotherapy and the tumor disappeared. Thereafter, he gradually developed leukocytosis and mild splenomegaly. In May 2009, the leukocyte count was 34,300 with 80% lymphoid cells. A diagnosis of HCL-Jv was made since the lymphoid cells showed a hairy morphology with round nuclei and indistinct nucleoli. These cells expressed CD11c, CD19, CD20, CD103 and showed weak reaction for tartrate-resistant acid phosphatase (TRAP). Bone marrow was infiltrated by atypical cells with an intrasinusoidal pattern. No treatment was needed as the patient was asymptomatic without anemia, thrombocytopenia or lymphadenopathy. Results of the immunoglobulin light chain expression and the heavy chain rearrangement in the tumor cells indicated that the two mature B-lymphoid neoplasms, MALT lymphoma and HCL-Jv, in this patient were derived from independent clones. This appears to be the first reported case of HCL-Jv associated with other lymphoid tumor. Further analysis is needed to clarify the risk of secondary malignancy in HCL-Jv.

Primary rectal MALT lymphoma is rare comprising less than 1% of MALT lymphomas. A 26-year-old man was referred to our hospital because of constipation and abdominal fullness. Colonoscopy revealed multiple submucosal tumors in rectum. Histopathological examination showed dense proliferation of small lymphoid cells, but lymphoepithelial lesions were not observed. The cells were CD5(-), CD10(-), CD20(+) and cyclinD1(-). The patient was diagnosed as having MALT lymphoma. The patient was negative for API2-MALT1 gene, and radiotherapy was performed and CR was achieved. With the accumulation of cases, establishment of a treatment strategy for primary rectal MALT lymphoma is expected in the future.

Only a few cases of primary lymphoma of the common bile duct have been reported up to now. Here, we report a mucosa-associated lymphoid tissue (MALT)-type lymphoproliferative lesion of the common bile duct with some considerations about MALT lymphoma and benign lymphoproliferation. The patient, a 71-year-old woman, was admitted to our hospital because of progressive epigastric pain. Abdominal ultrasonography, magnetic resonance cholangiopancreatography, endoscopic retrograde cholangiopancreatography, and abdominal computed tomography showed a non-specific finding of stenosis of the common bile duct due to thickening of the wall. Angiography provided little diagnostic information. Bile duct carcinoma was diagnosed and pylorus-preserving pancreaticoduodenectomy was performed. Histological examination revealed that the common bile duct was occupied by full-thickness proliferation of lymphoplasma cells with many reactive lymph follicles. The histological features were somewhat consistent with those of MALT lymphoma. However, we could not determine the clonal proliferation of lymphoplasma cells in either immunohistochemical immunoglobulin light chain restriction of the plasma cells or in polymerase chain reaction based immunoglobulin heavy chain gene rearrangement. The present case may be categorized as a borderline lesion close to benign lymphoproliferation. To the best of our knowledge, there have been no reports of cases similar to the present case.