Biomedical subjects

C A Hanson

Publications and source records attributed to C A Hanson.

At least 73 records · Page 4Linked to original sources

PubMed1991

Simultaneous lymphocyte predominant Hodgkin's disease and large-cell lymphoma.

Lymphocyte-predominant Hodgkin's disease (LPHD) is a subtype of Hodgkin's disease characterized by an indolent clinical course and by distinctive histological and immunological features. Coexistence of diffuse or nodular LPHD with large-cell non-Hodgkin's lymphoma (NHL) distant from the presenting site has rarely been reported. We studied three cases of simultaneous LPHD and large-cell NHL. Two cases involved men, aged 66 and 20 years, with neck and axillary masses, respectively. Biopsy of each mass revealed nodular LPHD. In one case the spleen contained areas of both LPHD and large-cell NHL, whereas only large-cell NHL was found in the spleen of the other patient. The patients are alive 49 months and 29 months after diagnosis. The third case was from a 4-year-old boy with a neck mass that revealed both diffuse LPHD and areas of large-cell NHL. Local recurrence prompted therapy, and the boy is in complete remission 31 months after diagnosis. Immunophenotyping in all three cases showed the Reed-Sternberg variant lymphocytic and histiocytic cells to be B-lymphocytes. The NHL cells in two cases were B-cells; in the child, the cells reacted only with leukocyte common antigen. Immunoglobulin heavy- and light-chain genes were rearranged in the NHL cells in the spleen of one case, and heavy-chain genes were rearranged in the lymph node of the child. It appears that when large-cell NHL and LPHD occur simultaneously, even when the large-cell NHL occurs at a site distant from the LPHD, the patient's clinical course is like the indolent course of LPHD rather than like the typically aggressive course of large-cell NHL. This clinical course, together with immunophenotyping and genotyping studies, suggests a developmental relationship between these two lymphomas when they occur simultaneously.

Adult↗

PubMed1991

Virus-associated hemophagocytic syndrome following bone marrow transplantation.

Multiple-organ infiltration by mature, benign erythrophagocytic histiocytes is the pathologic hallmark of the virus-associated hemophagocytic syndrome (VAHS). Although VAHS has been described in a number of clinical settings, it has been reported following bone marrow transplantation (BMT) only once previously. Our report identifies the clinical and laboratory features associated with VAHS and compares the immune defects described in VAHS with those known to occur following BMT.

Adenoviridae Infections↗

PubMed1991

Myoepitheliomas in inbred laboratory mice.

Myoepitheliomas are subcutaneous tumors that arise from myoepithelial cells of various exocrine glands. In a retrospective study of 142 tumors observed over a period of 3 years, myoepitheliomas occurred spontaneously in A/HeJ, A/J, BALB/cJ, BALB/cByJ, LLC.A/Ckc, and NOD/Lt inbred strains of mice. Tumors presented primarily in the subcutaneous tissues of the ventral neck (74% of the myoepitheliomas evaluated) but were observed in several other subcutaneous locations, including the head, perineum, and ventral abdomen. These areas were adjacent to salivary, mammary, clitoral, preputial, and Harderian glands. Forty myoepitheliomas were tested by the avidin-biotin complex technique with a panel of antisera specific for mouse keratins, intermediate filaments, and other cytoskeletal proteins to determine the cell type from which this neoplasm originated. Antibodies directed against the specific mouse keratins K5, K6, and K14, and a broadly cross-reactive cytokeratin antibody stained acinar and ductal myoepithelial cells in normal mammary, salivary, and Harderian glands, and neoplastic cells in all cases. Antisera directed against a smooth muscle actin (anti-alpha-sm-1) stained acinar myoepithelial cells of the glands and vascular smooth muscle but neither ductular myoepithelial cells nor tumor cells. This supports the notion that these tumors originate from extraglandular ductular myoepithelial cells. Southern blots, prepared from DNA extracted from nine myoepitheliomas, did not show restriction fragment length polymorphisms when mouse mammary tumor virus (MMTV) cDNA or Int-1 genomic DNA probes were used; this implies that a retrovirus is not the etiologic agent.

Age Factors↗

PubMed1991

Characterization of a new human multiple myeloma cell line, UMJF-2, which suppresses antibody production by B-lymphocytes in vitro.

A new human plasma cell line, UMJF-2, has been derived from the bone marrow of a patient with multiple myeloma. Morphological studies disclosed large nucleoli, moderate numbers of mitochondria, and scant endoplasmic reticulum consistent with a plasmablastic morphology. The cells have immunologic characteristics of early plasma cells, including intense expression of cytoplasmic IgG-lambda and weaker, but discernible, expression of surface IgG-lambda. Cell surface antigens defined by the monoclonal antibodies OKT10 (CD38) and PCA-1, characteristic of mature plasma cells, and B1 (CD20), B4 (CD19), and I-2 (HLA-DR), characteristic of earlier stages of B-lymphocyte differentiation, are present on UMJF-2 cells. Cytogenetic studies reveal the presence of trisomy 12. UMJF-2 does not contain the Epstein-Barr virus by Southern blot analysis. Tissue culture media conditioned by these cells contains a soluble immunosuppressive factor, capable of inhibiting pokeweed mitogen induced IgM secretion by normal human B-lymphocytes. UMJF-2 provides a model for the study of the pathogenesis of polyclonal hypogammaglobulinemia in human multiple myeloma.

Aged↗

PubMed1991

Eosinophilic leukemia: a myeloproliferative disorder distinct from the hypereosinophilic syndrome.

Evidence to support the existence of eosinophilic leukemia (EL) as an autonomous eosinophilic proliferation analogous to other myeloproliferative disorders has been somewhat confusing. Partially obscuring the existence of EL as a distinct entity is the proposal that EL merely represents a clinically aggressive form of hypereosinophilic syndrome. This report details the clinical and pathologic findings in a case of EL. The presence of trisomy 8 and trisomy 21; morphologic, cytochemical, and ultrastructural findings of granular abnormalities and nuclear/cytoplasmic dysynchrony; and a clinical course similar to that of other myeloproliferative disorders support the existence of EL as a rare but distinct entity within the spectrum of myeloproliferative diseases.

Adult↗

PubMed1991

Chronic active Epstein-Barr virus infection and virus-associated hemophagocytic syndrome.

We describe two unusual cases of Epstein-Barr virus infection that were complicated by the virus-associated hemophagocytic syndrome, predominantly involving the spleen. Both patients were young adult men who presented with fever, pancytopenia, and hepatosplenomegaly. Both had prompt symptomatic and hematologic improvement following splenectomy. Severe constitutional symptoms recurred in one patient 1 month after splenectomy, and he died of septicemia 2 months later. In both cases, there was prominent hemophagocytosis in the splenic red pulp. Some hemophagocytosis was also noted in the liver from the fatal case. Unexpectedly, no hemophagocytosis was detected in the bone marrow biopsy specimens or marrow aspirates obtained from these patients. The DNA hybridization studies detected Epstein-Barr virus genomes in spleen samples from both patients, and both patients had atypical patterns of serologic response to the virus, suggesting that a defective immune response may lead to an unrestrained viral proliferation. We conclude that there is an association between chronic active Epstein-Barr virus infection and the hemophagocytic syndrome, but that the tissue distribution of the hemophagocytosis may be variable.

Adult↗

PubMed1991

True histiocytic lymphoma.

It is likely that a significant number of cases of THL will continue to be misdiagnosed unless appropriate cytochemical and immunologic markers are incorporated into the panel of studies performed at diagnosis. The clinical findings are nonspecific. With the possible exception of skin infiltration, the frequency of symptoms and signs appear to approximate those of intermediate grade lymphomas. Most commonly, it is the histopathological features that suggest the histiocytic nature of a malignant process, but these alone are not sufficient to secure the diagnosis. Thus, in the absence of a clearly defined lymphoid origin, it is the detection of specific histiocytic markers that establishes a lymphoma as THL. Only the recognition and careful study of additional cases will potentially enable clinical investigators to identify any unique characteristics and distinguish this disease from other lymphomas. For the present, it would appear that the treatment indicated for a patient with diffuse large cell lymphoma would also be appropriate for a comparable patient with THL. Although long-term disease-free survival in THL is possible, there are presently inadequate data to estimate curability.

Adult↗

PubMed1991

Concurrent eosinophilic fasciitis and cutaneous T-cell lymphoma. Eosinophilic fasciitis as a paraneoplastic syndrome of T-cell malignant neoplasms?

Eosinophilic fasciitis has been reported to precede hematologic malignant neoplasms such as myelomonocytic leukemia, lymphocytic leukemia, and Hodgkin's lymphoma. In this case study, eosinophilic fasciitis occurred concurrently with cutaneous T-cell lymphoma (mycosis fungoides). The clinical diagnosis of eosinophilic fasciitis was based on painful sclerodermatous lesions on the extremities and trunk without acrosclerosis. There was histologic confirmation with edema and lymphocytic inflammation in the superficial muscular fascia and dermis. Deposition of immune reactants was found in the fascia and dermis. In addition, peripheral eosinophilia and circulating immune complexes were detected. The diagnosis of cutaneous T-cell lymphoma (mycosis fungoides) was based on extensive erythematous cutaneous plaques, dermal and epidermal lymphocytic atypia, loss of pan-T-cell immunologic markers, and a cutaneous lesional T-cell receptor beta-chain rearrangement by Southern blot analysis. Eosinophilic fasciitis may occur as a paraneoplastic syndrome associated with hematologic malignant neoplasms, including mycosis fungoides. Cytokines or lymphokines released by activated immunocytes, either malignant leukocytes or normal leukocytes reacting to malignant cells, may be responsible for the eosinophilia and sclerosis seen in these associated hematologic malignant neoplasms.

Aged↗

PubMed1990

CD11c (LEU-M5) expression characterizes a B-cell chronic lymphoproliferative disorder with features of both chronic lymphocytic leukemia and hairy cell leukemia.

Chronic lymphocytic leukemia (CLL) and hairy cell leukemia (HCL) are two common chronic lymphoproliferative disorders, each having characteristic clinical, morphologic, and immunologic features. Phenotypically, CD5 reactivity in CLL and CD11c (Leu-M5) reactivity in HCL have characterized these two leukemias among B-cell disorders. In this study, we report 14 cases of a novel chronic lymphoproliferative disorder characterized by lymphocytosis and CD11c expression, but morphologically similar to CLL. The patients' ages ranged from 46 to 81 years (median 62). Eleven had palpable splenomegaly, five with markedly enlarged spleens; only one patient had generalized lymphadenopathy. The white blood cell count ranged from 5.2 to 131.0 x 10(9)/L (median 20.8). The morphologic diagnosis in all cases was CLL, with the cells usually having abundant cytoplasm. No morphologic features, of hairy cells were evident; tartrate-resistant acid phosphatase cytochemistry was negative in all cases. Bone marrow biopsies were available in 8 of 14. Four showed focal nodular infiltrates and two had diffuse infiltrates similar to CLL; two showed only minimal interstitial involvement. All cases expressed multiple B-cell markers, and 12 of 14 had monoclonal surface immunoglobulin. The leukemic cells of all cases strongly expressed CD11c, while CD5 was expressed in 7 of 14; only 1 of the 14 cases expressed the lymph node homing receptor, Leu-8. This unique group of leukemias appears to represent the malignant transformation of lymphocytes arising from a stage of lymphocyte differentiation between that found in typical cases of CLL and that of HCL. CD11c is known to have an important function in cellular adhesion and may be important in determining the pattern of lymphocyte tissue distribution found in this group of patients.

Acid Phosphatase↗

PubMed1990

T cell receptor beta-chain gene rearrangement without gamma-chain gene rearrangement in cutaneous T cell lymphoma: an unusual finding.

T cells from the blood and skin of a patient with cutaneous T cell lymphoma demonstrated rearrangement of the T cell receptor beta-chain gene in the absence of rearrangement of the gamma-chain gene. To our knowledge, this has not been previously reported. This finding was unexpected in light of prevailing concepts of T cell ontogeny. Potential explanations for it are discussed.

Aged↗

PubMed1990

Lymphocytic infiltrates of the skin in association with cyclosporine therapy.

Three patients, one of whom has been previously reported, had erythematous papules and nodules of the face and upper part of the chest during cyclosporine therapy for inflammatory skin diseases. Histologic examination and DNA analysis (performed in two cases) revealed benign dermal lymphocytic infiltrates. In two cases proliferation of only T cells occurred. In the third case, both T and B cell populations were expanded and there was vacuolar degeneration of the basal layer of the epidermis and IgG, IgM, and C3 deposition along the dermoepidermal junction. These findings may be the result of cyclosporine-induced immune dysregulation. The lesions resolved in all patients after therapy was stopped.

Adult↗

PubMed1990

Richter's syndrome presenting as primary central nervous system lymphoma. Transformation of an identical clone.

The development of a central nervous system (CNS) large cell lymphoma in a patient simultaneously diagnosed with chronic lymphocytic leukemia (CLL) is reported. Although differences in phenotypic expression were demonstrated in study of the peripheral blood and CNS disease, identical immunoglobulin gene rearrangements were identified, providing evidence for evolution of two morphologically distinct neoplasms from the same clone. Beyond histologic transformation, acquisition of an aneuploid cell population in the CNS tumor was demonstrated by analysis of DNA content. Isolated parenchymal involvement of the CNS by large cell transformation of CLL has not been previously described; its relationship to CNS lymphoma and Richter's syndrome are reviewed.

Aged↗

PubMed1990

Kappa light chain gene rearrangement in T-cell acute lymphoblastic leukemia.

Immunoglobulin and T-cell receptor gene rearrangement assays are sensitive methods of detecting clonality in lymphoproliferative disorders. The lack of lineage specificity of immunoglobulin heavy chain and T-cell receptor beta and gamma gene probes in acute leukemia is well established. However, immunoglobulin light chain gene rearrangement traditionally has been considered a highly specific indicator of a clonal B-lineage process. The authors describe a case of T-cell acute lymphoblastic leukemia in which Southern blot hybridization studies showed rearrangement of the T-cell receptor beta chain gene. Unexpectedly, the immunoglobulin kappa light chain gene also was rearranged; no immunoglobulin heavy chain gene rearrangement was seen. Kappa rearrangement was confirmed with the use of three restriction endonucleases. No rearrangements were seen from normal skin tissue, making a restriction enzyme site polymorphism highly unlikely. Northern blot studies showed a normal-size, T-cell receptor beta chain gene transcript; no immunoglobulin RNA was identified. These results describe the first reported case of kappa light chain gene rearrangement in a T-cell acute lymphoblastic leukemia. The findings emphasize the necessity of interpreting molecular hybridization studies in conjunction with routine morphology and immunophenotyping studies.

Aged↗

PubMed1990

Immunophenotypic aberrancy in adult acute lymphoblastic leukemia.

Some recent reports indicate a high frequency of immunophenotypic aberrancy in acute lymphoblastic leukemia (ALL). To investigate this issue with regard to adult ALL, the authors reviewed the immunophenotyping data from 39 cases analyzed in their clinical laboratory. Flow cytometric analysis of peripheral blood and/or bone marrow was performed with the use of a panel of 21 B-cell, T-cell, and myeloid monoclonal antibodies (MoAbs). The surface antigen profiles of the leukemic cells were compared with those of normal bone marrow and thymic counterparts, as defined by current models. Twenty-six cases were B-precursor ALL, 8 were T-ALL, and 3 were B-ALL (Burkitt's leukemia). Only two cases coexpressed lymphoid and myeloid antigens. In contrast, intralineage aberrancy was quite common. Immunophenotypes from 13 of 26 B-precursor ALL cases deviated from normal B-lineage marrow cells as defined by a recent classification. The T-ALL cases were also immunophenotypically heterogeneous. This high incidence of aberrant antigen expression in adult ALL suggests that leukemogenesis also involves aberrant differentiation rather than purely a process of maturational arrest.

Adolescent↗

PubMed1990

Acute lymphoblastic leukemia with eosinophilia.

Acute lymphoblastic leukemia with eosinophilia is a rare but distinctive clinical entity. The eosinophilia in these patients can present before, concomitantly, or after the diagnosis of leukemia. Patients with this syndrome often suffer from the cardiovascular complications of severe eosinophilia, suffering excess morbidity and mortality as a result of their eosinophilia. Treatment of the eosinophilia in this syndrome consists of administration of induction chemotherapy, followed by prednisone and hydroxyurea if required for persistent eosinophilia. Eosinophilia often resolves with remission of leukemia, only to return at the time of relapse in a high percentage of cases. Patients with this syndrome characteristically have cytogenetic abnormalities involving the long arms of chromosomes 5 and 14. These cytogenetic abnormalities are not commonly seen in acute lymphoblastic leukemia and suggest that this syndrome may have a distinct pathophysiology and etiology. The affected region on chromosome 5 contains genes that control hematopoiesis, including eosinophilopoiesis. Further investigations into these cytogenetic abnormalities may provide insight into the etiology of the leukemia and eosinophilia characteristic of this syndrome.

Chromosome Aberrations↗

PubMed1990

Epstein-Barr virus--determined clonality in posttransplant lymphoproliferative disease.

Analysis of the genomic termini of Epstein-Barr virus can provide valuable insight into the cofactor role of EBV in the development of B cell lymphomas and lymphoproliferative disease. We report EBV genomic findings in pathologic specimens from 10 patients who developed lymphoma or lymphoproliferative disease after renal or bone marrow transplantation. Endonuclease restriction patterns of EBV genomic termini are highly variable in size in both the episomal and linear configuration. This variability in fragment size permits direct assessment of tissue clonality in EBV-infected material. Hybridization with terminus-specific probes also reveals configuration of viral genome (circular and latent vs. linear and replicative). Nine of 10 patients had tumors with mono- or biclonal episomal markers, and 4 of 10 had evidence of linear or replicative virus. Analyses of virally determined markers were compared to immunoglobulin gene rearrangement studies, histologic immunophenotyping, cytogenetics, and clinical outcome. These 10 cases represent a spectrum of lymphoproliferative disorders ranging from benign polyclonal to malignant monoclonal disease. The molecular data lend credence to two important aspects of viral pathogenesis: (1) the finding of a homogeneous episomal population in the monoclonal tumors suggests that EBV infection is an early event in tumorigenesis that occurs before clonal expansion; and (2) therapeutic efficacy of acyclovir has been shown only in presence of polyclonal disease but may impact on intermediate stages where linear replicative virus can be found. Finally, the various assessments of tumor clonality were compared, and although heterogeneity was seen among patients and among diagnostic methods, analyses at the molecular level using virus and immunoglobulin gene specific probes were concurrent and provide the more sensitive means for detection of clonality.

Adolescent↗

PubMed1990

Genotypic analysis of T-cell clones derived from cutaneous T-cell lymphoma lesions demonstrates selective growth of tumor-infiltrating lymphocytes.

The nature of T cells contained within cutaneous lesions of cutaneous T-cell lymphoma (CTCL) has not been studied at the clonal level. T cells extracted from skin lesions of two CTCL patients were cloned by limiting dilution and propagated in interleukin-2 (IL-2) containing medium with periodic lectin stimulation. Twelve T-cell clones were derived from each patient. In both cases, genotypic analysis of the T-cell clones revealed that these clones had T-cell receptor (TCR) beta- and gamma-chain gene rearrangements distinct from the predominant, presumably malignant, clone present in the skin, lymph nodes, or blood. This suggests that they were derived from presumably reactive (non-malignant) T cells. Furthermore, these clones had gene rearrangements different from each other, indicating their multiple clonal origins. The failure to propagate in vitro the CTCL T-cell clone suggests that CTCL cells may have growth requirements different from normal T cells. Thus, conventional T-cell culturing methods using IL-2 and lectins as mitogen may selectively propagate the presumably reactive T cells contained within the skin lesions. The ability to selectively grow these reactive lesional T cells (so-called tumor infiltrating lymphocytes) raises the possibility that these cells could be used in adoptive immunotherapy.

Cell Division↗

PubMed1990

Ascites: its effect upon static inflation of the respiratory system.

This report presents a simple clinical method to quantify the pressure generated within ascites fluid and thus to examine the effect of that fluid upon lung volume. The intra-abdominal hydrostatic pressure (Pih) given in cm H2O is the height of the meniscus of a column of water above the anterior abdominal wall measured with the patient supine. Pih thus may be thought of as a pressure in excess of the height of the anterior abdominal wall. In 23 study subjects, Pih measured 7.0 +/- 4.8 cm H2O; Pih correlated inversely with all static volumes. Furthermore, abdominal compliance (measured as the ratio of 100 ml ascites fluid removed divided by the difference between Pih before and after fluid removal) correlated directly with functional residual capacity. We conclude that the effect of ascites upon respiratory function varies among patients; the variation may be explained in part by Pih.

Abdominal Muscles↗