Whipple disease is a rare, chronic multisystem disease associated with the recently characterized organism Tropheryma whippelii. Extraintestinal manifestation involving the central nervous system, heart, and joints occasionally occurs. Involvement of the abdominal lymph nodes, especially the mesenteric and periaortic nodes, is not uncommon. However, peripheral lymphadenopathy as the sole clinical manifestation of Whipple disease is rare. We describe 2 patients with Whipple disease whose initial manifestation was lymphadenopathy. Lymph nodes from both patients showed infiltration of the sinuses by macrophages containing periodic acid–Schiff–positive, diastase-resistant, sickle-like structures. Electron microscopic evaluation confirmed the presence of rodlike organisms. DNA from each sample was amplified by the polymerase chain reaction using a specific set of oligonucleotide primers developed against the 16S ribosomal RNA coding sequence of T whippelii. The histopathologic features and differential diagnosis of lipogranulomatous lymphadenopathy secondary to Whipple disease, as well as use of molecular-based assays, are discussed.
Clinical, morphological, and ultrastructural studies are presented of three cases which had been diagnosed from lymph node biopsies as reticulum cell sarcoma and which later developed a leukemic phase. One patient had a diffuse type of reticulum cell sarcoma, the second a nodular type, and the third a mixed nodular and diffuse histologic pattern. The second patient developed a leukemic phase much later than the other two patients and had a longer period of survival. The three patients survived for 25 days, 10 weeks, and 15 months after diagnosis. The neoplastic cells are characterized morphologically in the lymph nodes, in the bone marrow aspirates, and in the peripheral blood by light microscopy and in two cases by electron microscopy also. There appears to be a spectrum of cell types in the leukemic reticulum cell sarcoma ranging from primitive reticulum cells or histiocytes to more differentiated monocytoid cells. No evidence for differentiation toward granulocytic elements was seen morphologically or cyto-chemically. The leukemic phase of reticulum cell sarcoma appears to be closely related to or identical to histiocytic or monocytic leukemia (Schilling type) and should be differentiated from hairy cell leukemia (leukemic reticuloendo-theliosis). Necropsy findings from three cases are reviewed.
Previous reports have suggested that nodular lymphocyte predominance Hodgkin's disease (NLPHD) is a germinal center-derived B-cell lymphoma that is distinct from other types of Hodgkin's Disease. A relationship between NLPHD and simultaneous or subsequent development of large-cell (LC) non-Hodgkin's lymphoma (NHL) has been established. Both Reed-Sternberg cell variants in NLPHD and NHL cells in these cases express B-cell-associated antigens, and in some cases the B-cell lineage of the NHL has been confirmed by immunoglobulin gene rearrangement studies. The B-cell phenotype and the indolent course of both lymphomas suggest histologic progression of NLPHD to B-cell NHL, rather than a de novo LCNHL unrelated to Hodgkin's Disease. We report a unique case of T-large-cell lymphoma (TLCL) following successful chemotherapy of NLPHD. A 54-year-old male was treated with seven cycles of mechlorethamine, vincristine, procarbazine, prednisone chemotherapy for NLPHD and 4 years later developed recurrent adenopathy. Lymph node biopsy showed a diffuse LCNHL. Frozen section immunotyping and gene rearrangement studies confirmed the diagnosis of TLCL. To our knowledge, this case represents only the second report of TLCL associated with NLPHD and is of significance in that: (1) it demonstrates that T-cell neoplasia can occur in the setting of NLPHD; (2) this case does not appear to represent histologic progression of NLPHD and most likely represents de novo disease that may be secondary to chemotherapy; and (3) the clinical course may differ from the favorable prognosis seen in NLPHD associated with B-cell NHL.
Abstract Inbred Palmerston North (PN) mice are a newly reconized model of systemic lupus erythematosus.In this study PN mice with established autoimmune disease were treated until death with cyclophosphamide (8 mg/kg/day) or hydrocortisone (10 mg/kg/day). These doses had previously been found to prevent or suppress disease in another lupus model, the NZB/NZW mouse. In the PN strain, autoantibodies, severity of glomerulo‐nephritis, and longevity were not influenced by treatment. Furthermore, the incidence of neoplasms was not increased in PN mice receiving prolonged therapy with immunosuppressive drugs. Unlike NZB/NZW mice, PN mice were resistant to the effects of cyclophomide and hydrocortisone.
A direct correlation between the percentage of cells in S phase of the cell cycle and the clinical behavior of lymphocytic lymphomas of low, intermediate, and high grade malignancy has been described. The histopathologist has used the mitotic rate and other morphologic criteria such as size of cells and nuclear characteristics as predictors/indicators of the aggressiveness of a tumor. We compared the S phase values of 22 cases of poorly differentiated lymphocytic lymphoma (PDL) of the B cell type, using flow cytometric measurement of DNA content, to morphologic features and mitotic rate (MR). The 22 cases were divided into 3 histologic groups: nodular PDL composed of small, cleaved lymphocytes (11 cases); follicular mantle zone lymphoma and those of intermediate differentiation (6 cases); and "blastic" PDL (5 cases). In Group 1 there was excellent correlation of MR, percentage of cells in S phase, and proportion of large cells (transformed lymphocytes) per high power field (HPF). In Group 2, this correlation was not found between MR and percentage of cells in S phase in five of the six cases. The high S phase in this group did correlate with the large proportion of large cells found primarily in pseudofollicular proliferation centers and in remnants of true follicular centers. These cells may have a prolonged S phase and thus fewer mitoses were seen. In Group 3, although both MR and S phases were high, a direct correlation between them as noted in the Group 1 cases was not seen, but an excellent correlation of the high S phase and the number of blasts was present. The fact that three of the five patients in this group died rapidly (within less than 2 years of presentation) and the two survivors were experiencing rapid progression of disease, supports the concept that this group represents a clearly different, more aggressive subclass of PDL.
