Spontaneous Regression Of Natural Killer Cell Lymphoma
Isobe, Y., Aritaka, N., Sasaki, M., Oshimi, K., & Sugimoto, K. (2009). spontaneous regression of natural killer cell lymphoma. Journal of clinical pathology, 62(7), 647–650. https://doi.org/10.1136/jcp.2008.062976
View Original Source →Abstract
Spontaneous tumour regression is extremely rare in aggressive lymphoma. A case of natural killer (NK) cell lymphoma with cutaneous manifestation showed an indolent clinical course, and the relapsed nodular lesion disappeared spontaneously without any treatment. Although only small number of T cells were present in the primary skin lesion, there was massive CD8-positive cytotoxic T cell infiltration in the relapsed lesion. This is believed to be the first report of an abscopal effect on NK cell lymphoma. Infiltration of cytotoxic T cells strongly suggests immunological attack against the lymphoma cells.
Case Details
Disease Location
Skin (eyelid) submandibular region
Personal Characteristics
65 -year-old female, japanese
Clinical Characteristics
Was referred due to itching, swelling and erythema of the right periocular skin for 2 years, physical exam showed red-bluish pigmentation, oedematous swelling and subcutaneous induration in the right lids and periocular region with no other findings wbc count was 7.6x10^9/l (71.5% neutrophils, 0.5% eosinophils, 5% monocytes and 22% lymphocytes), hemoglobin level was 14.3 g/dl; platelet count was 273x10^9/l. Serum lactate dehydrogenase and soluble forms of interleukin 2 receptor levels were 750 iu/l and 1040 u/ml respectively. Antibodies against hib and adult t cell leukemia virus were negative anti-ebv antibody test indicated previous ebv exposure (see article for further details) skin biopsy specimens demonstrated dense infiltration of atypical large polymorphic lymphocytes into the dermis and subcutaneous adipose tissue, accompanied by angiocentric and angiodestructive lesions immunohistochemistry showed the infiltrating cells positive for CD56, t cell intracellular antigen-1 and granzyme b; and negative for CD3 and 20 in situ hybridization of the ebv-encoded rnas confirmed that these atypical cells were infected with ebv. Some of these tumor cells expressed latent membrane protein 1. Flow cytometry of cell surface markers showed that the tumor cells were positive for CD2, 8, 38, 56, and negative for CD3, 4, 5, 7, 16, 19, 20, 25, 30, t cell receptor alpha-beta and t cell receptor gamma-delta southern blot analysis detected no rearrangement bands of tcr c-beta and j-gamma genes cytogenetic analysis showed normal karyotype in 20 cells examined. The diagnosis was cutaneous involvement of NK cell type of extranodal NK/t cell lymphoma, nasal type (NK-enkl) bone marrow exam was normal six courses of chop chemo plus 40 gy involved field irradiation completely eradicated the skin lesion. During treatment, transient facial odema was observed 9 months after the treatment and being symptom-free, right submandibular lymphadenopathy measuring 2.0x2.8cm appeared in april 2002 histological exam of the ln showed a lymphoma lesion containing large atypical lymphocytes with the same morphology and immunophenotype as those detected in the skin lesion; many tia-positive, granzyme-b positive CD3-positive cells, but few CD20 positive cells infiltrated into the lesion. Flow cytometric analysis showed infiltrating t cells were predominantly CD8-positive t cells and some t cells that were CD4-positive and CD25-negative. Southern blot analysis detected no rearrangement of bands of tcr c-beta and j-gamma genes and confirm clonal proliferation of ebv-infected cells. A relapsed NK cell lymphoma diagnosis was made. Discussion mentions that this case cannot be properly categories into any current class of NK cell lpd
Remission Characteristics
Chemo plus 40 gy eradicated the skin lesion the second tumor regressed without any therapy and completely disappeared within 2 months. Patient remains symptom free for more than 5 years
Treatment & Mechanisms
Proposed Remission Mechanisms
Radiotherapy might have induced the ctls of the patient to attack the lymphoma cells more efficiently than before the tumor cells may have been eradicated by host-cell-mediated immunity reacting to the tumor-associated antigens including ebv-encoded gene products
Clinical Treatment
Chop chemo plus 40 gy
Non-Clinical Treatment
None reported