A Case of Duodenal Relapse of Epstein-Barr Virus-positive, CD56-negative Extranodal NK/T-cell Lymphoma, Nasal Type / 대한소화기내시경학회지

Mature T-cell and natural killer-cell neoplasms account for 10 to 15% of all non-Hodgkin's lymphomas. Of the various subtypes of mature T-cell and NK-cell lymphomas, extranodal NK/T-cell lymphoma, nasal type (nasal type NK/T-L) is relatively more common among Asians including Koreans. Nasal type NK/T-L is an aggressive, Epstein-Barr virus-associated lymphoma with characteristic expression of NK-cell antigen CD56. In this report, we present an unusual case of EBV(+), CD56(-) NK/T-L of oropharynx which recurred in duodenum after the period of complete remission lasting for 10 years. A 58-year-old woman presented with 3 months history of abdominal pain. Gastroduodenoscopy showed the diffuse wall thickening with multiple ulcerations in bulb and proximal second portion of the duodenum. Pathological examination revealed the infiltration of atypical lymphocytes, which was positive for CD3, CD4, CD5, TIA-1, and EBV and was negative for CD15, CD20, and CD56, consistent with NK/T-L of mature T-cell origin. The past medical history included the presence of oropharyngeal mass 10 years earlier, which was diagnosed as polymorphic reticulosis. The mass resolved completely after the radiation therapy, and she remained free of the disease for 10 years. Upon review, the oropharyngeal biopsy showed an identical morphology and immunophenotype with duodenal lesion. In conclusion, we experienced an unusal case of NK/T-cell lymphoma, nasal type recured in the duodenum.

Epstein-Barr Viral RNA(EBERs) Expression in Conventional Malignant Lymphoma and Polymorphic Reticulosis of Upper Aerodigestive Tract

The author examined the immunophenotype and expression of Epstem-Barr virus RNA (EBERs) used in the situ hybridization technique in 20 cases of conventional malignant lymphoma and 28 cases of polymorphic reticulosis and malignant lymphoma with features of polymorphic reticulosis occured in the upper aerodigestive tract including the upper digestive tract(palatine tonsil), and upper respiratory tract(nasal cavity, paranasal sinus, nasopharynx). The results obtained were summarized as followings; 1. The favorable site of malignant lymphoma that occured in the upper aerodigestive tract was in the palatine tonsil(11 out of 20 cases, 55%), those of polymorphic reticulosis and malignant lymphoma with features of polymorphic reticulosis were nasal cavity and nasopharynx(19 out of 28 cases, 78%). 2. The immunophentype of malignant lymphoma that occured in the upper aerodigestive tract was mostly B-cell phenotype (15 out of 20 cases, 75%), and that of polymorphic reticulosis and malignant lymphoma with features of polymorphic reticulosis was predominantly T-cell phenotype(22 out of 28 cases, 79%). 3. The EBERs positivity of malignant lymphoma that occured in the upper aerodigestive tract was 25%(5 out of 20 cases), but that of polymorphic reticulosis and malignant lymphoma with features of polymorphic reticulosis was 57%(16 out of 28 cases). 4. The positive cases for EBERs revealed angiocentricity with necrosis(16 out of 21 cases, 76%), predominantly T-cell phenotype(19 out of 21 cases, 90%), and favorably involved the nasal cavity and nasopharynx(16 out of 21 cases, 76%). Based on the above results, it was concluded that polymorphic reticulosis and malignant lymphoma with features of polymorphic reticulosis that occurred in the upper respiratory tract was an EBV-positive angiocentric T-cell lymphoma favorably involving the nasal cavity and nasopharynx.

Polymorphic Reticulosis

Polymorphic reticulosis(PMR) is a unique clinicopathological entity of unknown etiology, which commonly present as an aggressive, necrotizing lesion of the upper respiratory tract. It is a separate nosologic entity from Wegener's granulomatosis and idiopathic mildine destructive disease. The origin of the cells composing polymorphic reticulosis has been controversial. We reviewed 15 cases of polymorphic reticulosis with respect to clinical and histologic bases, and immunohistochemical studies were done using UCHLI (CD45RO), as a T cell marker, CD-20 as a B cell marker and alpha- I -antichymotrypsin as a histiocytic marker. Almost all cases showed characteristic histologic and immunohistochemical features similar to those of peripheral T cell lymphoma. Thus, this study indidcated that polymorphic reticulosis is a type of malignant lymphoma of T cell lineage.

Expression of interleukin-6 in polymorphic reticulosis: immunohistochemical study of 5 cases

Peripheral T cell lymphoma encompasses lymphomas with a variety of histologic appearances and clinical patterns. Recently, it has been suggested that almost all of the histologic features described under the name of polymorphic reticulosis(PR), lethal midline granuloma, and midline malignant reticulosis can be included in those generally described for malignant lymphomas of peripheral T cell origin(PTCL). There have been few studies of pathogenesis or tissue damage mechanism in PR patients. The need for a precise mechanism for tissue damage has important therapeutic implications. Using immunohistochemical methods with polyclonal anti IL-6 antibody, the authors describe 5 cases of PR with clinically and pathologically typical PR demonstrating a high expression of IL-6. According to classification, 2 cases of grade 1 PR showed the highest expressions, and 2 cases of grade 2 PR with atypical lymphoid cells showed moderate activity, but one case progressed into frank lymphoma(grade 3) and lost IL-6 expression. This strongly implies that some cases of PR have a different mechanism of tissue damage from frank PTCL, despite the one disease spectrum. Further studies on more cases may help clarify the pathogenesis.

EBV in situ hybridization study for non-Hodgkin's lymphomas

Epstein-Barr virus(EBV) has been implicated in the pathogenesis of B-lymphoproliferative disorders, T-cell lymphomas and Hodgkin's disease. In this report, we performed an in situ hybridization study on EBV genome in 10 cases of nasal non-Hodgkin's lymphoma(NHL), 20 cases of Waldeyer's ring(WR) NHL, and 20 cases of nodal NHLs to document EBV association with lymphomas in Koreans. For immunophenotyping, monoclonal antibodies for CD 20, MB 2, CD 45Ro & CD 43 were used. For in situ hybridization study, EBV DNA probe for Bam HI 'V' fragment and EBV RNA probe for EBER and BHLF were used. Twenty two cases(44%) of malignant lymphomas were positive for EBV genome. Generally, T-cell lymphomas showed a higher positive rate(61%) than B-cell lymphomas(24%). Among T-cell lymphomas, nasal lymphomas showed a higher positive rate(80%) than WR(50%) or nodal lymphomas(50%). Of 22 EBV genome positive cases, 10 cases were positive for EBER, 10 cases for BHLF, and 2 cases for both EBER and BHLF. The histologic types by Working Formulation(WF) were not correlated with EBV genome positive rate, whereas lymphomas showing the histologic spectrum of polymorphic reticulosis(PR) showed a higher positive rate(65%) than lymphomas without PR-like features(40%). These results indicate that nasal T-cell lymphomas with the histologic spectrum of PR are strongly associated with EBV and that the anatomic site may be an important factor in this association.

Immunohistochemical Study of Upper Respiratory Tract Malignant Lymphomas with Features of Polymorphic Reticulosis

Malignant lymphomas of the upper respiratory tract are relatively common in the Orient including Korea. They have a marked clinicopathologic and immunophenotypic diversity. We retrospectively reviewed 20 cases of malignant lymphomas with features of polymorphic reticulosis in the upper respiratory tract. Histopathologic findings consisted of polymorphism of lymphoid cells in 17 cases(85%), angiocentricity in 14 cases(70%), coagulation necrosis in 12 cases(60%), and epitheliotropism in 8 cases(40%). By Working Formulation, 14 cases(70%) were classified as diffuse large cell type, 4 cases(20%) as diffuse mixed small cleaved and large cell type, one case as immunoblastic type, and one case as anaplastic large cell type. On immunostaining, one showed B-cell phenotype and 19 cases showed T-cell phenotype. The B-cell type was histologically diffuse large cell type. From these results, we concluded that polymorphic reticulosis is a type of peripheral T-cell lymphoma with angiocentricity.

Radiation Therapy Result of Polymorphic Reticulosis / 대한치료방사선과학회지

During the period from January, 1975, to June, 1989, one hundred patients with histopathologically proven polymorphic reticulosis in the upper respiratory tract were treated with radiation therapy and the analysis of treatment results was undertaken. One hundred patients (69 males, 31 females) with a mean age of 46 years (range 12-79 years) were presented. Nasal cavity was the most frequent site of involvement(56%), and 44 cases had multifocal sites of involvement. The incidence of cervical lymph node metastasis at initial diagnosis was 24%. Staging was determined by Ann-Arbor classification, retrospectively. The number of patients of stage IE, IIE, IIIE and IVE were 35, 60, 1, and 4, respectively. The overall 5 year actuarial survival rates were 38.4%. The difference in 5 year survival rates between patients with stage IE and IIE, with solitary and multiple, with CR and PR after irradiation were significant statistically. For the analysis of failure patterns, failure sites include the following: local failure alone(30/55=54.6%), systemic failure alone(9/55=16.4%), both local and systemic failure(16/55=29.0%). Retrograde slide review was available in 29 cases of PMR with respect to histopathologic bases, and immunohistochemical studies were performed using MTI and DACO-UCHL-1 as T-cell markers, MB2 as a B-cell marker and alpha-1-antichymotrypsin as a histiocytic markers. All that 29 cases showed characteristic histologic features similar to those of peripheral T-cell lymphoma and showed positive reactio to the T-cell marker. These findings suggest strongly that quite a significant portion of PMR may be in fact T-cell lymphoma.

Primary Radiation Therapy of Polymorhic Reticulosis / 대한치료방사선과학회지

From 1979 to 1987, 33 patients with polymorphic retrculosis (PMR) limited in the upper airway were treated with primary radiation therapy and the analysis of treatment results was undertaken. Systemic symptoms such as fever, night sweats, and weight loss were noted in 48%. The nasal cavity was most frequently involved (85%), although involvement of PNS (33%) and palate (30%) was not uncommon. The 5 and 10 year actuarial survival rates were 47% and 40%, respectively. The difference in NSD between patients with in-field failure and those without it was significant statistically. Also, field size was significantly smaller in patients with marginal failure than those without it. During the follow-up period, systemic failure was found in 5 patients (diffuse histiocytic lymphoma in 4, histiocytic medullary retriculosis in 1). In this study, we can suggested that total radiation dose of at least 4500 cGy with generous treatment volume should be delivered to achieve better local control and that the development of an effective systemic chemotherapeutic regimen is required to improve the survival after systemic relapse.

Immunopathologic Study and Clinical Correlation of Non-Hodgkin's Lymphoma

In Korea, the proportion of B-cell and T-cell lymphomas has not yet been clearly estimated. In the present study, we analysed clinicopathologic and immunophenotypic findings in 60 cases of non-Hodgkin's lymphomas(NHL) and in 17 cases of polymorphic reticulosis. Immunohistochemical studies for monoclonal antibodies, such as MB2, UCHL-1, Kappa/Lambda light chains, and IgG/IgM/IgD heavy chains were performed on formaldehyde-fixed paraffin embedded tissue sections. Among 60 cases of NHL, 28 cases(46.7%) expressed B-cell markers, 29 cases(48.3%) T-cell markers, and 3 cases(5.0%) lacked detectable markers, whereas 16 of 17 cases(94.2%) of polymorphic reticulosis reacted with T-cell markers. The mean survival of the 16 cases of T-cell lymphoma group was shorter than that of the lymphoma group positive for B-cell markers.

Histopathological and immunohistochemical studies of polymorphic reticulosis

This study reviewed 65 cases of polymorphic reticulosis (PR) with respect to clinical and histopathologic bases, and immunohistochemical studies were done using MT1 and UCHL as T-cell markers, MB2 as a B-cell marker and alpha-1-antichymotrypsin as a histiocytic marker. The results obtained were as follows: 1. The male to female ratio was 2.4:1 and the mean age of patients was 44.5 years. The sites involved primarily were the nasal cavity, tonsil and pharynx and about one-fourth of the total cases showed extensive involvement of two anatomical sites at initial presentation. 2. Almost all cases showed characteristic histologic features similar to those of peripheral T-cell lymphoma and showed positive reaction to the T-cell marker. The above immunohistochemical findings suggest strongly that quite a significant portion of PR is in fact T-cell lymphoma.

Polymorphic reticulosis with colonic lesion: a case report

A 38-year-old man was admitted with a high fever, sore throat, and right upper quadrant pain. Nine months before his admission, he had undergone right hemicolectomy under the impression of intestinal lymphoma. But there had been no evidence of lymphoma on microscopic examination. Under the postoperative diagnosis of inflammatory bowel disease, corticosteroid therapy was tried without response. On the follow-up colonoscopic examination, an ovoid ulcer, with convergence of the surrounding mucosal folds at the descending colon and an irregularly shaped ulcer at the ileocolic anastomotic site, were found. The colonoscopic diagnosis was Behcet's colitis. After pathologic slides of biopsy and surgical specimens obtained from the palatine tonsil and colon were reviewed, the diagnosis of polymorphic reticulosis was made. The patient received anticancer chemotherapy, including cyclophophamide and glucocorticosteroid. To date, colonic involvement of polymorphic reticulosis has not been reported. Because of the similarity of the colonoscopic findings to those of Behcet's colitis, polymorphic reticulosis should be included in the differential diagnosis of inflammatory bowel disease. We assume that this is the first case of polymorphic reticulosis involving the colon with characteristic colonoscopic findings.