Spontaneous Remission In A Patient With Follicular Lymphoma Carrying T Cell-rich Neoplastic Follicles And A New Complex Variant Translocation Of T(14;18): T(5;14;18)(p15;q32;q21.3)
Kato, M., Uemura, Y., Sato, K., Tsuruoka, Y., Yokoi, S., Nishio, Y., … Miura, I. (2015). spontaneous remission in a patient with follicular lymphoma carrying T cell-rich neoplastic follicles and a new complex variant translocation of t(14;18): t(5;14;18)(p15;q32;q21.3). Leukemia & Lymphoma, 56(7), 2187–2189. https://doi.org/10.3109/10428194.2014.987767
View Original Source →Abstract
Follicular lymphoma (FL) is a neoplasm of mature B cells that has a nodular cell growth pattern. Eighty percent of patients with FL have t(14;18)(q32;q21.3), the standard type of 18q21.3/BCL2 trans...
Case Details
Disease Location
Follicular lymphoma lymph nodes thoracic vertebra right submandibular, left supraorbital
Personal Characteristics
60 -year-old female
Clinical Characteristics
Presented with left cervical ln swelling in september 2006 CT showed generalized adenopathy hb level was 12.3g/dl, platelet count was 290x10^3/ul, wbc count was 5.4x10^3/ul. Serum level of ldh was 146iu/l and soluble interleukin-2 receptor was elevated to 847iu/l pathological diagnosis of the cervical ln was fl grade 2 neoplastic follicles consist of reactive t cells (CD3+, 5+, and pax5-) and tumor cells (CD5-, 10+, 20+, bcl-2+, CD23-, pax5+, and sox-11-) (at this point in the article there is a lot of jargon and i'm not sure what is relevant, e.g. Mib-1 index, karyotypic interpretation by g-banding) pathological diagnosis was fl and the break point of 12q21 was a locus of the bcl2 gene. Fish was applied to the fixed cells: fusion signals resulting from juxtaposition of the 12q32/igh and the 18q21.3/bcl2 were detected in 71% of interphase cells, indicating the presence of t(14;18)(q32;q21.3). Karyotype was reinterpreted as t(5;14;18)(p15;q32;q21) clinical stage was iiia and fl international prognostic index indicated intermediate risk lymph nodes regressed and serum levels of ldh remained within the normal range, she was followed up without treatment 10 months later she experience progressive malaise and swelling of the lower extremities and developed paralysis of both lower extremities on admission, the liver and spleen were not palpated and the superficial ln were not felt either. Hb level was 13.1g/dl, platelet count 240x10^3/ul, wbc count 6.2x10^3/ul, serum ldh level was 186iu/l, serum sil-2r level 547iu/l even though lymph nodes regressed, MRI revealed an extradural tumor at the fifth thoracic vertebra the extradural tumor was excised and was positive for CD10, 20, bcl2, bcl6 and surface immunoglobulin IGG-kappa) determined by both flow cytometry and ihc pathological diagnosis was consistently fl grade 2 but this time reactive t cells were not increased in neoplastic follicles. G-banding analysis was 46,xx[20}, fish showed t(14;18) fusion signals in 73.8% of interphase cells r-chop was initiated and there was a complete response 20 months later, she demonstrated a right submandibular ln of 2cm and left supraorbital tumor of 1.5cm in diameter, and no other lymphadenopathy via CT histology of the right submandibular ln showed nodules separated by fibrous bands and was still diagnosed as fl grade 2, t cells and CD21 + fdc was decreased, chromosomal analysis revealed 47,xx,t(5;18)(p15;q21.3),add(10)(qq22),+ 18[1]/46,xx[2] but fish showed t(14;18) fusion signals in 77.6% of interphase cells
Remission Characteristics
After r-chop and her levels of ldh and sil-2r returned to normal CT confirmed a complete response the supraorbital tumor dissapeared spontaneously within 2 months and she has been followed up for 36 months without any signs of relapse
Treatment & Mechanisms
Proposed Remission Mechanisms
The sr of the supraorbital tumor may be partly explained by the increase of CD8+ t cells in neoplastic follicles
Clinical Treatment
Excisional biopsy of extradural tumor 6 courses of r-chop (rituximab, cyclophophamide, doxorubicin, vincristine, prenisone) chemo