Monocytoid B-cell lymphoma of the prostate.

A case of a primary malignant non-Hodgkin's lymphoma of the prostate, with the histological and immunohistochemical features of monocytoid B-cell lymphoma, is presented. The tumor histology was identical to that described in the forms of node-based monocytoid B-cell lymphoma being composed of a dense, monomorphous lymphoid infiltrate with ovoid nuclei and rather abundant, pale cytoplasm. Phenotypic analysis revealed high expression of B markers 4KB5 and L26, and negativity for T-associated antigens. This unusual localization broadens the spectrum of extra-nodal sites of monocytoid B-cell lymphoma.     

Primary malignant lymphoma of the urinary bladder. A case report

A patient with primary malignant lymphoma of the urinary bladder is presented. Grossly, the bladder showed multiple submucosal masses. Histologically and immunohistochemically, diffuse B-cell lymphoma of the medium-sized cell type was revealed. On the basis of clinicopathological features, the present case resembled previously recorded cases of bladder lymphoma. The pathogenesis of this primary bladder lymphoma was presumably associated with follicular or chronic cystitis. Primary lymphoma of the bladder is a condition that is very rarely included in series of extranodal lymphomas, and there is a curious sex difference in its occurrence rates between Japan and Western countries. Primary lymphoma of the bladder may be considered a lymphoma that originates fro mucosa-associated lymphoid tissue.     

Diffuse large B-cell lymphoma with distinctive patterns of splenic and bone marrow involvement: clinicopathologic features of two cases.

Two unusual cases of large B-cell lymphoma with predominant splenic and bone marrow (BM) involvement and similar clinical and histopathologic features are described. Both patients presented with nonspecific constitutional symptoms, unexplained cytopenias, and splenomegaly. Splenectomy revealed diffuse red pulp involvement by large B-cell lymphoma. The perisplenic lymph nodes were also involved diffusely with effacement of normal nodal architecture, excluding a diagnosis of intravascular large B-cell lymphoma. BM biopsies revealed striking erythroid hyperplasia without overt morphologic evidence of involvement by lymphoma. Immunoperoxidase staining of the marrow biopsies with antibodies to CD20 and erythroid-associated antigens revealed involvement by large B-cell lymphoma morphologically resembling the early pronormoblasts. In both cases there was prominent, but not exclusive, intravascular/intrasinusoidal lymphomatous marrow infiltration. These cases represent an unusual variant of large B-cell lymphoma with distinctive patterns of splenic and BM involvement. Furthermore, they underscore the difficulties in identifying intrasinusoidal marrow infiltration by lymphoma in H&E-stained biopsy slides and demonstrate that this pattern of marrow infiltration may be seen in cases of large B-cell lymphoma distinct from the intravascular variant.     

One patient, two lymphomas. Simultaneous primary gastric marginal zone lymphoma and primary duodenal follicular lymphoma

We report the case of a 73-year-old man who presented with a 2- to 3-month history of epigastric discomfort and guaiac-positive stool. An upper gastrointestinal endoscopy revealed a diffuse erythematous nodular mucosa and submucosal thickening in the stomach. Diffuse mucosal nodularity was also found in the second portion of the duodenum. A complete workup with histologic, immunohistochemical, and molecular studies revealed 2 distinct, apparently unrelated lymphomas, namely, a gastric marginal zone B-cell lymphoma (mucosa-associated lymphoid tissue type) in a background of Helicobacter pylori gastritis and a grade 1/3 duodenal follicular lymphoma. The patient was then treated with an H pylori eradication regimen. No therapy was given for his duodenal follicular lymphoma because his symptoms were thought to be due to the gastric disease and because the duodenal lesion was small. A 6-month follow-up with upper gastrointestinal endoscopy revealed only focal biopsy scarring in the stomach and an apparently normal duodenum. The follow-up biopsies revealed significant regression of his mucosa-associated lymphoid tissue lymphoma, but persistence of his duodenal follicular lymphoma. The combination of these 2 lymphomas in the same patient and the different clinical responses to antibiotic treatment make this case unique.     

T-cell lymphoma and the virus-associated hemophagocytic syndrome.

A 20-month-old girl had a disorder that by both clinical and histologic criteria resembled the virus-associated hemophagocytic syndrome in the setting of Epstein-Barr virus infection. Subsequent investigation revealed histologic evidence of disseminated T-cell lymphoma. DNA hybridization studies displayed a monoclonal T-cell receptor beta chain rearrangement, in the absence of clonal immunoglobulin gene rearrangement, and a single band in the analysis for the fused termini of the Epstein-Barr virus genome. These results suggest the presence of a monoclonal population of T lymphocytes infected with Epstein-Barr virus. The diagnosis of lymphoma was confirmed at autopsy. The authors discuss the association of Epstein-Barr virus infection with the development of T-cell lymphoma and propose that the previous reports of virus-associated hemophagocytic syndrome include cases of unrecognized T-cell lymphoma.     

Primary breast lymphoma.

We report the case of a 20-year-old female with lymphoma of the breast. Mammography showed an asymmetric pattern of confluent densities without any discrete mass. Sonography revealed diffuse heterogenous echoic mass intermingled with low-and medium level echoes. We present the clinical, radiographic and histologic features of primary breast lymphoma with a brief review of the literatures.     

Expression of perforin in nasal lymphoma. Additional evidence of its natural killer cell derivation.

Eight patients with nasal lymphoma in whom fresh-frozen tissues were available were studied to elucidate the nature of the lymphoma cells. Two cases were diagnosed as diffuse, large cell lymphoma, and the remaining six cases as diffuse, mixed cell types. Immunohistochemical studies revealed that all of the cases were positive for perforin, which is a specific marker for cytotoxic T or natural killer (NK) cells. As all of the cases were CD8 negative, the perforin-positive finding further confirmed the concept that nasal lymphoma is a distinct neoplastic entity derived from NK or NK-related cells. Light microscopic immunohistochemical studies revealed that these nasal lymphoma cases could be classified into Leu19(CD56)+Leu4(CD3)+ (two cases) and Leu19(CD56)+Leu4(CD3)- (six cases) types according to the phenotypes of the proliferating cells. However, simultaneous staining for perforin and Leu4 (CD3) using immunoelectron microscopy on the Leu19+Leu4+ cases showed that the perforin-positive cells were different from the Leu4-positive cells. This finding suggests that the Leu4-positive cells are not neoplastic NK cells but reactive T cells. Six cases were positive for EBER-1 by in situ hybridization analysis. This finding reconfirms the previous studies that Epstein-Barr virus plays a significant role in the pathogenesis of nasal lymphoma.     

Continuous lymphoblastoid suspension cultures from cells of haematopoietic organs of baboons with malignant lymphoma. Report III: Immunological studies.

Lymphoblastoid cultures established from haematopoietic organs of baboons with lymphoma belong to the B-cell type. Cytoplasmic antigen is revealed in 2--6% of suspension culture cells established from lymphomatous baboons by indirect immunofluorescence reaction using serum from human Burkitt's lymphoma patients with antibody titer to capsid antigen of Epstein-Barr virus 1: 320. There is a correlation between the number of antigen-containing cells in these cultures and the number of cells in which herpes virus is revealed electron microscopically. Sera of baboons with lymphoma contain high titers of antibodies to Epstein-Barr (EBV) virus. Specific differences between antigens being produced in cultures by HBV (herpes virus of baboon) and EBV (Epstein-Barr virus) are minimal, that indicates their immunologic similarity.     

Detection of HTLV-I proviral sequences in CD30-positive large cell cutaneous T-cell lymphomas.

To investigate the possibility that cutaneous T-cell lymphomas of large cell type may be associated with human T-cell leukemia/lymphoma virus type I infection in nonendemic regions, tissue samples from six cases of large cell cutaneous T-cell lymphoma and four cases of small cell cutaneous T-cell lymphoma were screened for the presence of integrated proviral human T-cell leukemia/lymphoma virus type I DNA. Combined use of Southern blot hybridization and enzymatic DNA amplification revealed human T-cell leukemia/lymphoma virus type I-specific sequences in all cases of large cell cutaneous T-cell lymphoma and in none of the cases of small cell cutaneous T-cell lymphoma. These results suggest that in nonendemic areas, a significant proportion of large cell cutaneous T-cell lymphoma cases are associated with human T-cell leukemia/lymphoma virus type I.     

Chromosomes and causation of human cancer and leukemia. XLI. Cytogenetic experience with non-hodgkin,non-burkitt lymphomas

Twelve cases with non-Hodgkin, non-Burkitt lymphoma were studied cytogenetically, primarily with material obtained through bone marrow aspiration. An evaluation of the use of bone marrow material for karyotypic studies in lymphoma revealed it to be a rather unfruitful approach in that successful cytogenetic findings were obtained in less than 10% of all newly diagnosed cases of lymphoma observed during a period of several years. It would appear that the utilization of lymph node material or material obtained from tissues frequently and extensively involved by lymphoma (e.g., spleen) yield significantly more successful results than bone marrow material. The cytogenetic findings encountered were similar to those recently summarized for lymphoma and included, among others, three cases with 14q+, two cases with 6q?, two cases with iso-8q, one case with an iso-6p chromosome. Of interest was the demonstration of karyotypic changes compatible with those in lymphoma in two cases where the presence of a complicating acute myeloblastic leukemia or other acute leukemia was suspected. In addition, definitely abnormal cells compatible with those seen in lymphoma were found in the marrow of a patient in whom cytologic examination failed to reveal their presence. From these findings it is apparent that cytogenetic findings can be used as important diagnostic aids in some patients with lymphoma with complicated and/or uncertain histologic and clinical manifestations.     

Malignant lymphoma of the gastrointestinal tract and mesentery. A clinico-pathologic study of the significance of histologic classification. NHL Study Group of the Comprehensive Cancer Center West

A series of 92 malignant lymphomas of the gastrointestinal tract and mesentery obtained from a population-based registry was studied to assess whether the newly defined concept of mucosa-associated lymphoma has clinical relevance. The cases were grouped according to localization; gastric, intestinal, and mesenteric lymphoma. All cases were reviewed histologically, graded according to the Working Formulation, and reclassified according to the Kiel classification, which was modified to include the categories low- and high-grade mucosa-associated lymphoma. Clinical data, as well as staging and follow-up data, were related to both the original diagnosis and the diagnosis after reclassification. The results showed that the distribution of the types of lymphoma is related to site: centroblastic lymphoma was predominant in the stomach, lymphoblastic in the bowel, and follicular centroblastic-centrocytic in the mesentery. Gastrointestinal lymphoma was disseminated in approximately 50% of the patients at presentation. Survival analysis revealed that classification according to the original Kiel classification and grading according to the Working Formulation provided important prognostic information, whereas introduction of mucosa-associated lymphoma as an entity did not. It was concluded that modification of current classifications to include a separate category for mucosa-associated lymphoma is not useful.     

Low-grade primary B-cell lymphoma of the lung. An immunohistochemical, molecular, and cytogenetic study of a single case

A case of primary low-grade pulmonary lymphoma is described. The histologic features conformed to those laid down by Saltzstein for a diagnosis of "pseudolymphoma." However, the immunocytochemical and molecular investigations confirmed the tumor to be a low-grade B-cell lymphoma of mucosa-associated lymphoid tissue (MALT). Cytogenetic studies revealed an abnormal karyotype with a translocation t(1;14). This is the first reported case of an abnormal karyotype in a case of a lymphoma of MALT.     

Malignant lymphoma with primary presentation in the spleen. A study of 20 patients

We describe 20 adult patients with malignant lymphoma with primary presentation in the spleen. The most common presenting symptoms were fever, malaise, and weight loss. Physical examination revealed prominent splenomegaly without palpable lymphadenopathy. Small lymphocytic lymphoma was the most frequent histologic type (11/20), followed by large cell lymphoma and mixed cell lymphoma (3/20 each). Bone marrow involvement was found in ten of 17 patients. At laparotomy, lymph node involvement, usually retroperitoneal, was found in six of 13 patients. There was liver involvement in seven of 15 patients. Follow-up has been relatively short, with an average of 24 months (range, one to 48 months). Four patients died as a result of progressive disease, one died of sepsis after splenectomy, and one died two years after diagnosis of a stroke. The prognosis in primary splenic lymphoma appears to be similar to that in nodal lymphoma.     

Biclonality of composite B- and T-cell lymphomas. A case report

Most composite lymphomas which are composed morphologically of two different tumor cell types are considered to represent different morphological expressions of a single clone. However, in recent years, composite B- and T-cell lymphomas and biclonality of B-cell lymphoma have been reported. We experienced a case of composite lymphoma which initially developed as cutaneous lymphoma composed of lymphoplasmacytes associated with large clear cells. It was confirmed that the tumor cells of these two systems were biclonal on the basis of surface markers and DNA rearrangements, i.e. B cells of the IgG kappa type, showing IgH and kappa chain DNA rearrangement, and T-cells with CD4 surface marker, showing rearrangement of the T-cell receptor beta chain gene. This case showed a predominant B-cell pattern at the initial stage, and terminated in T-cell lymphoma, as revealed at autopsy. Therefore we considered this case to be a unique composite lymphoma showing biclonality of both B- and T-cell systems, providing a number of suggestions for future study of malignant lymphoma.     

Intravascular large cell lymphoma: diagnosis on renal biopsy.

The case is reported of a woman aged 60 yrs who presented with systemic symptoms and who was found to have proteinuria of 3.5 g per day. A renal biopsy revealed numerous neoplastic cells filling many of the glomerular capillary lumina. Immunoperoxidase stains revealed that the phenotype of the malignant cells was LCA+, L26+, MB2+, UCHL1-, CD43-, CAM5.2- and S100-, indicating that they were of lymphoid origin and B-cell lineage. The diagnosis of intravascular large cell lymphoma was therefore made. Remission was induced by chemotherapy with CAVP (cyclophosphamide, adriamycin, vincristine and prednisone). A subsequent relapse was treated with cyclophosphamide, VP16 and prednisone, and again remission occurred. This is the first case known to the authors in which the diagnosis of intravascular large cell lymphoma was made on renal biopsy. We confirm the experience of others that chemotherapy with regimens utilized in other varieties of large cell lymphoma may also be appropriate for this unusual neoplasm.     

Sudden death due to lymphomatous infiltration of the cardiac conduction system.

We present the case of a 62-year-old white female with a history of previously treated angioimmunoblastic T-cell lymphoma, who died suddenly 1 day after presentation for presumed tumor recurrence. Postmortem gross examination revealed a 4-cm cardiac mass that was adherent to the free wall of the right atrium. Histologic examination showed extensive infiltration of the cardiac conduction system by lymphoma, which likely lead to fatal arrhythmia. Histological examination revealed systemic dissemination by an Epstein-Barr virus (EBV)-positive large B-cell lymphoma. Careful examination of the cardiac conduction system on serial sections was crucial in documenting the cause of death due to lymphomatous infiltration. As in this patient, lymphomatous infiltration of the heart may produce sudden cardiac death without obvious premortem signs of cardiac compromise. This case illustrates the utility of examination of the cardiac conduction system in necroscopy studies of sudden death.     

OPD4-positive T-cell lymphoma of the liver in systemic lupus erythematosus.

Primary malignant lymphoma of the liver occupying the right lobe, 14 x 9 x 7 cm in size, developed in a 30-year-old man with a 4-year history of autoimmune hemolytic anemia. The diagnosis of systemic lupus erythematosus (SLE) accompanying thrombocytopenia had been made clinically 10 months earlier. The liver biopsy specimen revealed diffuse proliferation of large lymphoma cells expressing the activated helper/inducer T-cell phenotype (LCA+, UCHL1+, OPD4+, LN3+, MT1-, L26-, MB1-, Leu M1-, Ki-1-, KP1-). The lymphoma was successfully treated by chemotherapy and irradiation. Intractable thrombocytopenia provoked fatal esophageal hemorrhage. At autopsy, no lymphomatous lesion was identified, and the hepatic right lobe contained an encapsulated necrotic lesion without any viable tumor cells. The bone marrow revealed marked hyperplasia of erythroid and megakaryocytic series. Extramedullary hematopoiesis was demonstrated in the liver, spleen and lymph nodes. This is the second case of primary hepatic T-cell lymphoma associated with SLE.     

Nasal T-cell lymphoma associated with hemophagocytic syndrome. Immunohistochemical and genotypic studies.

A 53-year-old man with nasal T-cell lymphoma exhibited hemophagocytic syndrome as a terminal event. Immunohistochemical studies revealed that neoplastic cells were derived from T cells. Genotypic analysis of DNA samples that were obtained from the frozen tissue specimens demonstrated clonal rearrangements of the T-cell receptor beta-chain genes. No rearrangement was observed in the immunoglobulin heavy-chain gene. To our knowledge, only three cases of nasal T-cell lymphoma with hemophagocytic syndrome have been reported before the present case. All of these cases occurred in Oriental patients. The present report suggests the beneficial effect of high-dose glucocorticoid therapy on the prolongation of survival compared with that of the other three cases.     

Bone marrow involvement by non-Hodgkin's lymphom.

We retrospectively studied 156 patients with non-Hodgkin''s; ymphoma (NHL) in order to evaluate the incidence of bone marrow (BM) involvement at the time of diagnosis. The incidence of marrow involvement in NHL was 35% (55 patients). The most common histologic type of lymphoma was diffuse large cell lymphoma with 30% of the cases having bone marrow involvement. The highest incidence of marrow involvement was seen in immunoblastic lymphoma & the lowest incidence in diffuse mixed cell lymphoma. Bone marrow lymphoma was present in 50% of low-, 34% of intermediate-, and 55% of high-grade lymphomas. B-cell vs T-cell lymphoma in cases of marrow involvement was 56% to 42%. The most frequent pattern of marrow involvement was interstitial & diffuse (56% & 31%). The paratrabecular pattern was rare (4%). Most lymphomas (42%) extensively involved marrow space greater than 76% of the total marrow space. Discordant histology between lymph node and BM was seen in 10 cases (18%). Biopsy was the best method compared to smear & clot section (sensitivity 82% vs 72% vs 69%). In conclusion, our study revealed that the most common histologic type of marrow lymphoma was diffuse large cell type with frequent interstitial and diffuse pattern and extensive involvement of the marrow space. No predilection for a trabecular pattern was found. This result was in contrast to that in the Western literature.