1/185. An expert system for the interpretation of flow cytometric immunophenotyping data.The development of high-grade non-Hodgkin's lymphomas in hiv-positive patients and patients with acquired immune deficiency syndrome (AIDS) is a well known phenomenon. The proper classification of these neoplasms often requires a multiparameter approach, including the interpretation of a large panel of immunologic markers analyzed by flow cytometry. The availability of individuals with the required expertise to properly interpret these marker studies is limited. For this reason, we have designed an expert system to automate the analysis of immunophenotyping panels in both hiv-related and non-hiv-related hematopoietic neoplasms. The expert system, which we call "Professor Fidelio", runs on IBM-compatible computers under Windows 3.0. The system is designed to accept any number of markers studied from a repertoire of 35 markers. Professor Fidelio functions on the basis of heuristic classification of defined diagnostic patterns. Nine specific patterns (Stem Cell, Myeloid and/or Monocytic, Erythroid, Megakaryocytic, Immature B-cell, Immature T-cell, Mature B-cell, Mature T-cell, and plasma cell) and one "non-specific" pattern have been agreed upon. Fidelio's knowledge base contains the definitions of each of these patterns and the heuristics for excluding patterns when an incomplete panel of markers is performed. The inference engine interprets the findings (including the age of the patient) and reports the patterns which are matched, the differential diagnosis, the suggested diagnosis from the list of differentials if the marker studies are specific, and recommendations for additional tests which may be valuable in establishing the diagnosis.(ABSTRACT TRUNCATED AT 250 WORDS)- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
2/185. Co-incidental presentation of IgA lambda multiple myeloma and pleural involvement with IgM kappa non-Hodgkin's lymphoma.Pleural effusions occur in approximately 6% of patients with myeloma. The aetiology is multifactorial and effusions due to pleural myelomatous involvement are rare, occurring in < 1% of cases. We report the case of a 68-year-old lady who presented with IgA myeloma and a concurrent pleural effusion due to a second IgM kappa producing B cell neoplasm. The former responded but the latter was resistant to standard myeloma therapy.- - - - - - - - - - ranking = 0.5keywords = neoplasm (Clic here for more details about this article) |
3/185. Cutaneous necrosis as a terminal paraneoplastic thromboembolic event in a patient with non-Hodgkin's lymphoma.Thrombotic complications in non-Hodgkin's lymphoma often originate in the large veins. We describe a patient with refractory advanced high-grade lymphoma who presented with the rare complication of extensive cutaneous necrosis due to thrombosis of dermal vessels; there was also a recent new peak of monoclonal IgM-kappa protein. Direct immunofluorescence demonstrated immune deposits with complement in the dermal vessel wall. Based on these observations and on published data, we suggest that these complexes were the trigger for the thrombotic events and that the monoclonal IgM acted as xenoreactive antibodies, initiating a cascade of events. The first step of this cascade was activation of the complement and the membrane attack complex, which caused secretion of IL-1 alpha by endothelial cells, followed by overexpression of tissue factor on the surface of the dermal vessel wall endothelium. Dermal vessel thrombosis was the final event in this cascade.- - - - - - - - - - ranking = 0.01264202640473keywords = complex (Clic here for more details about this article) |
4/185. Pulmonary intravascular lymphomatosis: presentation with dyspnea and air trapping.Intravascular lymphomatosis (IVL) is a rare lymphoid neoplasm that is typically of B-cell lineage and characterized by proliferation of malignant cells within small arterioles, capillaries, and venules. We report a patient with pulmonary IVL who presented clinically with progressive dyspnea, fever, and a dry cough. Pulmonary function tests revealed a marked decrease in diffusion capacity with airflow obstruction and severe air trapping. High-resolution CT (HRCT) of the chest with inspiratory and expiratory images revealed mosaic attenuation consistent with air trapping. Transbronchial biopsies revealed the diagnosis of IVL with capillary expansion in the alveolar and peribronchiolar interstitial tissue. IVL should be considered in the differential diagnosis of a patient with an interstitial lung disease, air trapping on pulmonary function tests, and mosaic attenuation on HRCT. Transbronchial biopsies may be the initial diagnostic procedure of choice.- - - - - - - - - - ranking = 0.5keywords = neoplasm (Clic here for more details about this article) |
5/185. Cutaneous waldenstrom macroglobulinemia in transformation.waldenstrom macroglobulinemia is a low-grade B-cell lymphoproliferative disorder of the elderly with characteristic monoclonal IgM-producing neoplastic infiltrates of the bone marrow, lymph node, and spleen. Cutaneous manifestations are usually nonspecific such as purpura, ulcers, and urticarial lesions. These lesions are caused by hyperviscosity of the blood, immune complex-mediated vascular damage, paraprotein deposition, and amyloid deposition. Specific skin lesions occur rarely and generally consist of translucent, flesh-colored papules composed of monoclonal IgM deposits. Rarely, there may be violaceous lesions composed of low-grade lymphoplasmacytic infiltrates characteristic of waldenstrom macroglobulinemia. Both cutaneous manifestations of the disease, as well as disease transformation to high-grade, large cell lymphoma are rare. We report two very unusual cases of waldenstrom macroglobulinemia with documented skin disease that demonstrated transformation to high-grade lymphoma. Both patients were elderly men with long-standing waldenstrom macroglobulinemia involving the bone marrow, who subsequently developed skin involvement by the disease. waldenstrom macroglobulinemia can rarely manifest as cutaneous disease, sometimes as a high-grade transformation of low-grade waldenstrom macroglobulinemia elsewhere. Distinction of cases of transformed waldenstrom macroglobulinemia from de novo cutaneous large cell lymphoma may be important, because the two entities are likely biologically different.- - - - - - - - - - ranking = 0.0063210132023652keywords = complex (Clic here for more details about this article) |
6/185. Reactive angioendotheliomatosis or intravascular histiocytosis? An immunohistochemical and ultrastructural study in two cases of intravascular histiocytic cell proliferation.Two elderly women with complex medical histories presented with erythematous patches, in one case involving the face and forearms, and in the other both elbows. Punch biopsies from both patients revealed intravascular proliferations of medium-sized and large cells with luminal occlusion typical of angioendotheliomatosis. Immunostaining did not show either lymphocytic or endothelial cell antigens but was consistent with a histiocytic differentiation of the intravascular cells in both cases, and was further substantiated by ultrastructural examination in one case. One patient received a course of cyclophosphamide therapy over 15 days. skin lesions faded but did not disappear. The patient died 10 months later from cardiac and renal failure, which was most probably unrelated to the skin lesions. In the other case, lesions diminished but did not entirely resolve with treatment with low doses of oral prednisone. Angioendotheliomatosis can be divided into a malignant variant, which is an angiotropic lymphoma mostly of B-cell phenotype, and a benign, reactive variant, which is characterized by a proliferation of cells expressing endothelial cell markers. Only one case of angioendotheliomatosis with cells of histiocytic differentiation has been published previously under the name of intravascular histiocytosis. Our cases are very similar to the latter. The question arises as to whether intravascular histiocytic cell proliferation is a neoplastic proliferation of histiocytes or an early stage of classic reactive angioendotheliomatosis representing the residual cells associated with organization of microthrombi, which will be later followed by endothelial cell proliferation.- - - - - - - - - - ranking = 0.0063210132023652keywords = complex (Clic here for more details about this article) |
7/185. Nasal and nasal-type T/NK-cell lymphoma with cutaneous involvement.Natural killer (NK) cells are a third lymphocyte lineage, in addition to B- and T-cells, that mediate cytotoxicity without prior sensitization. NK cells also have phenotypic and genotypic characteristics; they express the NK-related antigen CD56 and T-cell markers such as CD2 and CD3 epsilon, but their T-cell receptor (TCR) locus is not rearranged. Non-Hodgkin's lymphomas are divided into B- and T-cell neoplasms and NK-cell lymphomas. We describe 2 Japanese patients with nasal and nasal-type T/NK-cell lymphoma in which the skin, nasal/nasopharyngeal region, bone marrow, and lymph node were the sites of involvement. The clinical and histopathologic findings were recorded. In addition, immunophenotyping, TCR gene rearrangement, and the existence of Epstein-Barr virus (EBV) dna by polymerase chain reaction amplification were determined. Clinically, the cutaneous eruptions were purplish, hard, multiple nodules. Histologically, angiocentric proliferation of small-to medium-sized, pleomorphic, lymphoid cells were observed. They revealed hand-mirror-shaped lymphocytes with azurophilic granules with the use of Giemsa staining by touch smear. These lymphocytes were found to be positive to immunophenotyping for CD2 (Leu5b), CD3 epsilon (DAKO), CD4 (Leu3a), and CD56 (Leu 19). No clonal rearrangement of TCR-beta, -gamma, and -delta genes and immunoglobulin gene markers were found, and no positive results of identification of EBV dna were shown. The patients underwent cyclophosphamide, doxorubicin, vincristine, and prednisone chemotherapy with complete remission; however, both had recurrence of disease. Because NK-cell lymphomas express some T-cell markers, they may be mistakenly diagnosed as peripheral T-cell lymphomas if they are not investigated for the NK-cell-specific marker, CD56. Therefore the importance of immunophenotypic investigations of CD56 should be stressed. Also, the importance of clinical investigation of nasal/nasopharyngeal lymphomas should be stressed when NK-cell lymphoma is diagnosed involving the skin, because NK-cell lymphomas are often associated with the nasal and nasopharyngeal region.- - - - - - - - - - ranking = 0.5keywords = neoplasm (Clic here for more details about this article) |
8/185. Primary bilateral adrenal lymphoma.Primary adrenal lymphoma is a rare primary neoplastic disease of the adrenal glands, with up to 65 cases reported in the literature over the past 40 years. The increasing use and sophistication of medical diagnostic imaging has allowed this disease to be diagnosed more frequently premortem, presenting more opportunity for treatment. The true incidence of these neoplasms is not known, nor is it clear why historical autopsy series have not reported this neoplasm more frequently, and why its existence has been documented more recently with advanced diagnostic imaging. This review has presented our new case, and reviewed 5 more in the literature, bringing the total number of cases to over 70. Accumulation of more cases and the experience treating these cases is needed to develop a better picture of diagnostic procedures and treatment regimens that have maximum efficacy.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
9/185. Primary non-Hodgkin's lymphoma of the head of the pancreas: a case report and review of literature.Among malignant neoplasms of the pancreas, lymphomas have a very low incidence. Nevertheless, the dramatic difference in prognosis and treatment between pancreatic carcinoma and lymphoma stresses the importance of a correct diagnosis, especially because most lymphomas of the biliopancreatic region are low-grade B-cell type. The case observed by us shares clinical and pathological features with the few previously reported in literature, and focuses clinicians' attention on the diagnosis of this unusual neoplasm whose clinical behaviour is characterized by obstructive jaundice and overlaps with that of epithelial tumours.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
10/185. Hodgkin's disease and B cell lymphoproliferation in rheumatoid arthritis patients treated with methotrexate: a kinetic study of lymph node changes.We describe 2 patients with seropositive rheumatoid arthritis treated with methotrexate (MTX) who developed Hodgkin's disease (HD) and non-Hodgkin's lymphoma. Followup allowed a lymph node biopsy at 4 different time points in 1 patient and at 2 in the other. In the first patient, the steps included a long history of benign follicle hyperplasia, a polymorphic diffuse B cell lymphoproliferation, and finally HD unassociated with Epstein-Barr virus (EBV). In the second patient, a polymorphic diffuse lymphoproliferation was followed by a monomorphic large B cell lymphoproliferation associated with EBV. The cytogenetic analysis showed a monoclonal proliferation associated with the same chromosomal abnormalities found in 1 of the clones observed in the initial biopsy. These 2 cases illustrate the complexity of the role of MTX in the outbreak of such manifestations.- - - - - - - - - - ranking = 0.0063210132023652keywords = complex (Clic here for more details about this article) |
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