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PubMed2001

Primary lymphoma of the conjunctiva--a rare manifestation of indolent non-Hodgkin's lymphoma.

Lymphoid tumours of the conjunctiva are extremely rare. Such tumours can appear as an isolated neoplasm or as part of a systemic disease. We describe the clinical and histopathologic findings of a patient with bilateral conjunctival lymphoma who presented with a 5-year history of chronic conjunctivitis. After clinical staging, including (1) thoracic and abdominal computerised tomography scans, (2) magnetic resonance tomography of the brain and orbita, (3) fibre-optic gastrointestinoscopy, (4) colonoscopy and (5) bone marrow biopsy,.no other foci of the lymphoma were found. The patient received radiation therapy and achieved complete remission. We conclude that conjunctival lymphoma should be included in the differential diagnoses of chronic conjunctivitis. Persisting signs and symptoms of conjunctivitis not responding to standard treatment should prompt biopsy.

Biopsy↗

PubMed1987

Radiation leukemia virus and X-irradiation induce in C57BL/6 mice two distinct T-cell neoplasms: a growth factor-dependent lymphoma and a growth factor-independent lymphoma.

Two different classes of neoplastic T cells were isolated from radiation leukemia virus (RadLV)-inoculated and from X-ray-treated C57BL/6 mice. One consisted of growth factor-dependent T-cell lymphoma (FD-TCL) lines which were established from the spleens and thymuses of treated mice within a day of lymphoma detection. FD-TCL cells were often eudiploid and could be grown in pure culture only at high concentrations, or on stromal feeder layers. Non-thymic, factor-dependent TCL cells produced interleukin-2 upon lectin stimulation, and were autostimulatory because they secreted growth factor(s) constitutively. Single cell cloning of FD-TCL cells in semisolid medium required the addition of exogenous conditioned medium. In vivo, FD-TCL cells that were injected intraperitoneally or intravenously homed to the spleen, proliferated in it and killed the injected mice. FD-TCL cells did not produce local tumors at the site of subcutaneous injection. The isolation and study of FD-TCL cells was facilitated by their cultivation on stromal hematopoietic monolayers in supplemented "lymphocyte medium", until an autostimulating, self-sustaining concentration of FD-TCL cells was obtained. FD-TCL cells could not be grown from lymphoid tissue of normal, control mice. In contrast, T-cell lymphoma (TCL) lines, which were established from virus-induced thymomas which had been kept in situ for 4-6 weeks after detection, consisted of factor-independent cells that possessed an aneuploid karyotype (in some cases trisomic for chromosome No. 15), and produced local tumors at the site of subcutaneous injection. These cells could be cloned in semisolid medium without addition of exogenous factor(s). The phenotypic markers of TCL cells differed from those of FD-TCL cells, suggesting heterogeneity in the stages of differentiation at which cells can give rise to growth factor-independent (TCL) and to growth factor-dependent (FD-TCL) lines.

Animals↗

PubMed1989

Characterization of cell markers in type B retroviral-induced thymic lymphomas--I. Surface antigen phenotype and karyotype in developing and primary lymphomas.

CFW/D mice injected neonatally with a type B retrovirus, (DMBA-LV), rapidly develop thymic lymphomas. The present study examined simultaneously the karyotype and the expression of surface antigens on thymocytes from DMBA-LV treated mice at 28, 35 and 42 days of age during the development of lymphomas, and of cells from primary lymphomas. DMBA-LV treatment resulted in disturbances in the proportions of thymic Lyt 1+2-, Lyt 1-2+ and Lyt 1+2+ subpopulations. As a group, virus-treated mice showed a significant decrease in the size of the Lyt 1+2- subpopulation in thymuses during tumor development. However, developing tumors and primary tumors showed individual patterns of alteration in the proportions of thymocyte subpopulations rather than consistent trends for any one thymocyte subpopulation to increase or decrease. Cells with the abnormal chromosome complement, trisomy 15, were present early in the development of tumors and became the predominant karyotype in fully developed tumors. A correlation between the appearance of trisomy 15 and a particular thymocyte surface antigen phenotype was not detected. The results suggest that retrovirus infection disrupts, but does not eliminate the ability of developing thymocytes to form phenotypically distinct subpopulations.

9,10-Dimethyl-1,2-benzanthracene↗

PubMed1992

Localized extradural lymphoma: survival, relapse pattern and functional outcome. The Princess Margaret Hospital Lymphoma Group.

Between 1967 and 1988, 22 patients were treated at The Princess Margaret Hospital (PMH) for localized (stage IE) non-Hodgkin's lymphoma (NHL) presenting with spinal extradural compression. The median age of the patients was 55 years (range 18-76). Back pain (20 patients) and leg weakness (18 patients) were the commonest complaints at presentation. Seven patients (30%) were non-ambulatory (paraplegic or severely paretic) and four had imparied sphincter function. Diffuse histiocytic lymphoma (12 cases) was the commonest histology. All patients initially had laminectomy decompression and were referred to PMH post-operatively. One patient (with coincident metastatic carcinoma of the breast) was treated palliatively. The remaining 21 patients received radical post-operative treatment: radiation therapy (XRT) alone in 12 cases (25-45 Gy), radiation therapy plus systemic combination chemotherapy (combined modality therapy, CMT) in 9 cases. The overall actuarial survival for the radically treated patients was 55% at 10 years and there was a significant difference (p = 0.037) between those treated by XRT alone (33%) and those who received CMT (86%). Only one patient from each treatment group failed locally but the distant recurrence-free survival for the XRT group was 32% compared to 100% for the CMT group (p = 0.017). One patient developed primary central nervous system (CNS) relapse. The functional results of treatment were excellent: 19 of the 21 radically treated patients regained or retained normal ambulatory status and the remaining two patients had only minor disability. Decompressive surgery and radiotherapy for localized extradural lymphoma ensures a high rate of local control and functional recovery.(ABSTRACT TRUNCATED AT 250 WORDS)

Antineoplastic Combined Chemotherapy Protocols↗

PubMed1990

Chromosomal abnormalities in patients with non-cutaneous T-cell non-Hodgkin's lymphoma. The Nebraska Lymphoma Study Group.

In contrast to non-Hodgkin's lymphomas (NHL) with a B-cell phenotype, almost no data have been reported dealing with correlations between chromosomal abnormalities and characteristics of the disease in patients with T-cell NHL. In a retrospective analysis we studied all patients with a non-cutaneous T-cell NHL and chromosomal abnormalities that were evaluated at our institution; 20 patients could be identified. Numerical abnormalities involving chromosomes 3, 4, 5, 22 and X were observed most frequently. Structural abnormalities involved mainly the breakpoints 1q22-25, 6q23 and 11q13. There appeared to be an association between +7, breakpoints 2p23-24, 4p14-15, 8q21 and the presence of extranodal disease. All patients with +7 had a diffuse mixed histology. Patients with +2, +3, +11, +17, +18, +20 or breakpoint 1q22-25 had an immunoblastic lymphoma and patients with breakpoints 9q32-34 or 14q12 had a lymphoblastic lymphoma. No correlations were observed between chromosomal abnormalities and response to therapy, survival or phenotypic markers. Abnormalities involving the chromosomes containing the T-cell receptor genes and T-cell markers were infrequent. Several breakpoints were identified that correlate with already described oncogenes.

Adolescent↗

PubMed1992

Angiotropic lymphoma (intravascular large cell lymphoma) presenting with cauda equina syndrome.

A 50-year-old man developed cauda equina syndrome of unknown etiology that was stable for 20 months. Two months prior to sudden death, he experienced new back pain, confusion, seizures, and multiple cranial nerve palsies. Neuropathologic examination revealed angiotropic lymphoma without parenchymal involvement or infarcts in the brain, spinal cord, and muscle. In addition, nerve roots in the cauda equina contained angiotropic lymphoma and infarcts of various ages. Angiotropic lymphoma should be considered as a cause of cauda equina syndrome and of disorders that affect the central and peripheral nervous systems concurrently.

Brain Neoplasms↗

PubMed2005

Patterned CpG methylation of silenced B cell gene promoters in classical Hodgkin lymphoma-derived and primary effusion lymphoma cell lines.

Hodgkin and Reed-Sternberg (HRS) cells of classical Hodgkin lymphoma (cHL) and primary effusion lymphoma (PEL) are derived from germinal center (GC) and post-GC B cells, respectively. Neither express many of the B cell genes or surface markers typically expressed by other GC-derived B cell lymphomas or normal B cells. This loss of B cell gene expression is not due to a lack of essential transcription factors, as studies have shown that the ectopic expression of missing transcription factors failed to reactivate endogenous target genes. These results implicate epigenetic mechanisms extinguishing B cell gene expression. Silenced endogenous B cell genes representing a surface receptor, B29 (Igbeta, CD79b), a signaling molecule, TCL1, and a transcription factor, Bob1 (OCA-B, OBF-1), were reactivated by 5-aza-2'-deoxycytidine, indicating that gene silencing in HRS and PEL cells is due to DNA methylation. Genomic bisulfite sequencing corroborated this prediction and revealed three distinct patterns of methylation for the silenced B29 and TCL1 promoters. These distinct patterns consisted of 5' promoter CpG methylation alone, 5' and 3' promoter CpG methylation sparing sites in the central cores, and complete CpG methylation throughout the promoter regions. The silenced Bob1 promoter showed one pattern of dense CpG methylation at essentially all sites. These consistent patterns predict that, although gene silencing in many HRS and PEL cells mimics appropriate gene silencing, in some cases of complete CpG methylation throughout entire promoters both the activation and targeting of methylation is abnormal.

B-Lymphocytes↗

PubMed2005

Multiple bilateral parotid sialoliths in a patient with mucosa-associated lymphoid tissue lymphoma (MALT lymphoma) of the salivary glands.

Multiple sialoliths in the major salivary glands is uncommon. Sjogren's syndrome (SS) is a chronic autoimmune disorder characterized by lymphocyte-mediated destruction of exocrine glands. There is an increased incidence of malignant lymphoma in patients with Sjogren's syndrome. We present a patient with MALT lymphoma of the salivary glands who also had multiple calculi in the parotid glands. Multiple sialoliths in the major salivary glands in the presence of MALT lymphoma has not previously been reported.

Antineoplastic Combined Chemotherapy Protocols↗

PubMed1988

Immunophenotypes of malignant lymphoma centroblastic-centrocytic and malignant lymphoma centrocytic: an immunohistologic study indicating a derivation from different stages of B cell differentiation.

Five cases of intermediate lymphocytic lymphoma (ILL), 13 cases of malignant lymphoma centrocytic (MLCC), and 27 cases of malignant lymphoma centroblastic centrocytic (MLCBCC) were studied morphologically and with the aid of a panel of monoclonal antibodies. The immunophenotypes of ILL and MLCC (IgM+/IgD+, MT1+, CALLA-) indicate a mantle zone or very early follicle center derivation. The immunophenotypes of MLCBCC (IgM+ or IgG/IgA+, MT1-, CALLA+) indicate a "true" follicle center derivation. The morphologic diversity of MLCBCC could not be related to specific immunophenotypes.

Adult↗

PubMed1999

Clinical significance of follicular lymphoma with monocytoid B cells. Non-Hodgkin's Lymphoma Classification Project.

Although follicular lymphoma (FL) is very common in the Western world, very little information is available regarding the frequency and significance of monocytoid B cells (MBC) in FL. We recently completed a clinicopathologic study of 1,378 cases of non-Hodgkin's lymphoma. In this study, a research data sheet was designed to conduct research on several types of lymphomas, one part of which was evaluating the presence of intrafollicular clear cells and extrafollicular MBC in 326 cases diagnosed as FL by one of the pathologists (B.N.N.). For each case diagnosed as FL, the presence of intrafollicular clear cells or extrafollicular MBC was scored as pure FL (no intrafollicular clear cells or extrafollicular MBC), FL with intrafollicular clear cells, FL with less than 5% MBC, and FL with greater than 5% MBC. Of 326 cases classified as FL, 252 (77%) had no intrafollicular clear cells or extrafollicular MBC and therefore were called pure FL. In 36 cases (11%), intrafollicular clear cells were seen, but no extrafollicular MBC. There were no clinical differences between such cases and the 252 cases of pure FL. In eight cases of FL (2%), MBC clusters were rare (<5%). In contrast, 30 cases of FL (9%) had a prominent (>5%) proliferation of extrafollicular MBC; these 30 cases had a significantly shorter failure-free survival (P = .001) and overall survival (P = .04) than the 252 cases of pure FL. The shorter survival of these 30 cases appeared to be independent of the international prognostic index (IPI), stage, and treatment. The FFS of this group remained shorter than that of cases with pure FL when the analysis was restricted to patients treated with Adriamycin-containing regimens and either a favorable (0 to 3) IPI score (P = .001) or advanced stage (III/IV) disease (P = .015). In conclusion, FL with a prominent (>5%) MBC component constitutes a substantial proportion (9%) of FL and has distinctive morphology, and these patients have a significantly shorter survival than those with pure FL.

B-Lymphocytes↗

PubMed2001

Primary effusion lymphoma: a liquid phase lymphoma of fluid-filled body cavities.

Primary effusion lymphoma (PEL) is a B-cell neoplasm characterized by infection of the tumor clone by human herpesvirus type-8/Kaposi's sarcoma-associated herpesvirus (HHV-8/KSHV) and by liquid growth in fluid-filled body spaces. During its entire clinical course, the lymphoma tends to remain localized to the serous body cavities with no formation of solid tumor masses. The epidemiology of PEL points to a close link with underlying immunodeficiency of the host, as most cases develop in individuals severely immunocompromised because of preexisting acquired immunodeficiency syndrome. The histogenesis and pathogenesis of PEL have been clarified to a sizeable extent by intensive investigations performed since the disease recognition in 1995. PEL is composed of postgerminal center B cells, which bridge immunoblastic and anaplastic features and typically display a non-B, non-T phenotype consistent with late stages of B-cell differentiation. HHV-8/KSHV is thought to play a major role in PEL pathogenesis via expression of several viral latent genes, which have the potential to affect B-cell growth. Other factors involved in PEL pathogenesis include deregulation of cytokine and growth factor autocrine loops, molecular alterations of the tumor DNA, cell cycle abnormalities, stimulation and selection by antigen, and infection by Epstein-Barr virus, which occurs in 70% of PEL cases. In the years since the disease discovery, the distinctiveness of the biological and clinicopathological features of PEL has prompted its recognition as an independent lymphoma category by the World Health Organization classification system of hematologic neoplasms.

Diagnosis, Differential↗

PubMed1991

The lymphomas: Hodgkin's disease and non-Hodgkin's lymphomas.

This review presents an overview of a heterogeneous group of disorders that fall under the general heading of lymphomas. Many specific subtypes and presentations exist; their presentations, treatments, and prognoses vary greatly. Patients with lymphomas are generally treated with external beam radiation therapy, chemotherapy, or a combination of these two modalities. The community pharmacist may play an important role in management of patients by recognizing and alleviating the distressing side effects of treatment. Unfortunately, prevention and early detection play little role in the management of lymphomas. The improvement in survival rates of patients with all but indolent NHL is a direct result of advances in chemotherapy.

Antineoplastic Combined Chemotherapy Protocols↗

PubMed1981

Lymphoma-associated papulosis: lymphomatoid papulosis associated with lymphoma.

Lymphomatoid papulosis is a condition characterized by the continuing appearance of self-healing inflammatory papulonodules which histologically show atypical cells simulating a malignant process. We describe two patients who had features typical of lymphomatoid papulosis in whom a lymphoreticular malignancy developed. The first patient developed Hodgkin's disease 30 years after the onset of lesions that resembled lymphomatoid papulosis, and the second patient developed a lymphocytic lymphoma 11 months after the appearance of the self-healing papules. While most patients with lymphomatoid papulosis have a benign protracted course, "lymphoma-associated papulosis" represents the malignant end of this spectrum, in which lymphomatoid papulosis is associated with a lymphoma. All patients with lymphomatoid papulosis require close, long-term follow-up.

Biopsy↗

PubMed1980

The otolaryngologic manifestations of malignant lymphoma with a high content of epithelioid histiocytes (Lennert's lymphoma).

Malignant lymphoma with a high content of epithelioid histiocytes is a rare non-Hodgkin lymphoma first described by Lennert and Mestdagh (Lennert's lymphoma) in 1968. Distinguishing characteristics include frequent involvement of the nasopharynx, Waldeyer's ring, and cervical lymph nodes. Of the four patients included in this report, three had Waldeyer's ring involvement and four had cervical lymphadenopathy. Our experience and that of others demonstrate the unpredictable and variable course of the disease, as well as the inconsistent response to various therapeutic modalities; especially interesting is the remission obtained in one patient treated with corticosteroids alone, in contrast to the others who followed a rapid downhill course to death in spite of (or as a complication of) chemotherapy and radiation. The role of the otolaryngologist in the diagnosis and management of the disease is emphasized.

Adult↗

PubMed1990

Genotypic and immunophenotypic analysis of anaplastic large cell lymphoma (Ki-1 lymphoma).

Genotypic and immunological analysis was performed in 10 patients with anaplastic large cell lymphoma (Ki-1 lymphoma), 4 were male and 6 female. Immunophenotypically, 9 of these patients expressed some T cell markers; CD 2 in 9, CD 4 in 9, CD 3 in 6, CD 8 in 4, and UCHL-1 in 8. For B cell markers, 4 patients expressed B 1 and one expressed L 26; these patients also expressed T cell markers. In genotypic analysis, 9 of 10 cases displayed some incidence of T cell receptor gene (TCR) rearrangement or deletion, which was found in 3 patients for C beta 1, 7 for C beta 2, 8 for J gamma, 5 for C gamma (Hind III), 5 for C gamma (EcoRI), 3 for J delta 1 (Hind III), 3 for J delta 1 (BamHI), 3 for J delta 2, and 2 for C delta. One patient with rearrangement of TCR also showed rearrangement of immunoglobulin heavy chain. Another patient exhibited rearrangement of both TCR and immunoglobulin chain. These results indicate a common T cell lineage for this type of lymphoma.

Adolescent↗

PubMed1991

Report of a primary lymphoma of the conjunctiva. A lymphoma of MALT origin?

A case of primary conjunctival lymphoma is reported herein. A 47-year-old woman presented with an indolent salmon-coloured tumour of the lower epibulbar conjunctiva. Histologically, we found a lymphoid infiltrate in the epithelium and subepithelium consisting of two components. One component was characterized by centrocytic-like cells and the other by lymphocytic, plasmacytoid and plasma cells. An immunohistological study demonstrated a light chain restricted immunoglobulin expression of the tumour cells as well as some residual polytypic follicles exhibiting reticular dendritic cells. Tumour cell proliferation was associated with a T cell reactive population. A comprehensive investigation (in particular an orbital scan) did not demonstrate any other localization of this lymphoma. Such a lesion raised the question of a possible MALT origin, by analog with MALT-lymphomas of the gastrointestinal tract described by Isaacson and colleagues.

Antibodies, Monoclonal↗

PubMed2000

Tumor bulk as a prognostic factor for the management of localized aggressive non-Hodgkin's lymphoma: a survey of the Japan Lymphoma Radiation Therapy Group.

PURPOSE: To identify the prognostic factors that specifically predict survival rates of patients with localized aggressive non-Hodgkin's lymphoma (NHL). METHODS AND MATERIALS: The survey was carried out at 25 radiation oncology institutions in Japan in 1998. The 5-year event-free (EFS) and overall survival rates (OAS) were calculated, and univariate and multivariate analyses were done to identify which of the following factors, namely, gender, age, performance status (PS), serum lactate dehydrogenase (LDH) level, Stage (I vs. II), tumor bulk (maximum diameter), and treatment, were significant from the viewpoint of prognosis. RESULTS: A total of 1141 patients with Stage I and II NHL were treated by the Japanese Lymphoma Radiation Therapy Group between 1988 and 1992. Of them, 787 patients, who were treated using definitive radiotherapy with or without chemotherapy for intermediate- and high-grade lymphomas in working formulation, constituted the core of this study. Primary tumors arose mainly from extranodal organs (71%) in the head and neck (Waldeyer's ring: 36% and sinonasal cavities: 9%). The factors associated with poorer prognosis were age over 60 years old (p < 0. 0001), radiation therapy alone (p < 0.0001), PS = 2-4 (p = 0.0011), (sex male, p = 0.0078), a bulky tumor more than 6 cm in maximum diameter (p = 0.0088), elevated LDH (p = 0.0117), and stage II (p = 0.0642). A median dose of 42 Gy was delivered mainly to the involved fields. Short-course chemotherapy was provided in 549 (70%) patients. The 5-year OAS and EFS rates for all patients were 71% and 67%, respectively. According to the stage-modified International Prognostic Index, the 5-year EFS of the patients with risk factors from 0 to 1 was 76%, 61% for patients with two risk factors, and 26% for patients with three or more risk factors. CONCLUSION: Extranodal presentation, especially Waldeyer's ring and sinonasal cavities, is encountered more frequently in Japan than in Western countries. Tumor bulk is an important prognostic factor in patients with localized aggressive extranodal NHL. Short course chemotherapy followed by radiation therapy was associated with prolonged survival in patients with localized aggressive NHLs of extranodal origin and 0-1 risk factor.

Adult↗

PubMed1997

Localized diffuse large-cell lymphoma possibly coated with anti-tumor autoantibody: kappa lambda-dual-positive lymphoma.

We report a case of diffuse large-cell lymphoma which pursued a clinically indolent course while remaining untreated. The tumor cells expressed surface IgG, CD10, CD19 and CD20, but not surface IgM, IgD, IgA, CD5, CD16, CD32 and CD64. In addition, these cells appeared to coexpress kappa- and lambda-light chains on their surface. JH and J lambda genes were monoclonally rearranged, but not the J kappa gene. The present lymphoma was found to have arisen from follicle center cells expressing IgG lambda, while the surface kappa-light chain seemed to be extrinsic. Furthermore, the extrinsic immunoglobulin bearing the kappa-light chain may have belonged to IgG because no surface immunoglobulins other than IgG were detected on the cell surface. Then, the extrinsic IgG may have combined with certain molecules on the tumor-cell surface through its Fab portion because the tumor cells lacked all three receptors for the IgG-Fe portion. Fc gamma RI (CD64). Fc gamma RII (CD32) and Fc gamma RIII (CD16). From these findings and the patient's history of never having received a blood transfusion, we concluded that the present case might be of a B-cell lymphoma possibly coated with an IgG-type autoantibody which appeared to have anti-idiotypic activity.

Adult↗