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Biomedical subjects

P G Scheurlen

Publications and source records attributed to P G Scheurlen.

At least 19 recordsLinked to original sources

Immunoglobulin and T cell receptor gene rearrangements in lymphoproliferative disorders.

Thirty-one samples representing Hodgkin's and non-Hodgkin's lymphomas, angioimmunoblastic lymphadenopathy (AILD), and benign follicular hyperplasia in HIV infections were examined for rearrangements of the immunoglobulin (Ig) and T cell receptor (TcR) beta-chain gene loci. In 11 of 12 non-Hodgkin's lymphomas (classified as Burkitt lymphoma (2), centrocytic lymphoma (1), centrocytic-centroblastic lymphoma (5), centroblastic lymphoma (3], only rearranged Ig genes could be detected. The exceptional case was an unclassified high-grade lymphoma, which represented a rearrangement of the TcR beta-chain. We also examined DNA from lymphoid neoplasms in which the lineage of the malignant cell was still controversial. Rearrangement of the TcR could exclusively be demonstrated in all 3 cases of AILD. One Ig gene rearrangement and 4 TcR beta-chain rearrangements were found in 13 samples of Hodgkin's lymphomas (11 lymph nodes, 1 pleura effusion and 1 bone biopsy with proven infiltration). Examination of 3 cases of benign follicular hyperplasia in HIV infection represented one Ig rearrangement.

DNA, Neoplasm

Effects of human alpha-interferon on granulocyte-macrophage progenitor cells (CFU-GM) in vitro.

Two preparations of human interferon (IFN)-alpha were assessed for their influence on granulocyte-macrophage progenitor cells (CFU-GM) in vitro. Both highly purified human IFN-alpha Ly and recombinant IFN-alpha 2a suppressed CFU-GM colony formation in a dose-dependent manner using low-density bone-marrow target cells. Suppression of CFU-GM colony formation was accompanied by an increase in clusters. However, depletion of monocytes, T lymphocytes and B lymphocytes from low-density bone-marrow cells resulted in insensitivity of progenitor cells to IFN-alpha. These results demonstrate that the effects of human IFN-alpha on myeloid progenitor cells (CFU-GM) are mediated by accessory cells within the bone marrow.

Cell Division

Lymphocytic plasmocytoid lymphoma with a three-banded gammopathy: reactivity of one of these paraproteins with cytomegalovirus.

We report the case of a 70-year-old woman suffering from small lymphocytic, plasmocytoid lymphoma with abdominal lymphomas and infiltration of the lung and the bone marrow. A three-banded, IgG lambda, IgM lambda and IgA lambda-paraproteinemia was determined using immunofixation. Because of the patient's high antibody titre against cytomegalovirus (CMV), the possible reactivity of these paraproteins with CMV was studied. The immunoglobulins were transferred to nitrocellulose sheets by a contact diffusion blotting system. CMV was applied to these sheets and the IgG lambda-paraprotein was shown to bind CMV. The reactivity of only one of the paraproteins with CMV suggests an oligoclonal origin of this gammopathy. In addition to the malignant disease an abnormal immune response to a CMV infection could be the cause of this three-banded gammopathy.

Aged

[Transitory oligoclonal paraproteinemia with virus infection and malignant lymphatic disease].

Oligoclonal paraproteinaemia occurred in two patients with malignant lymphocytic disease (highly malignant non-Hodgkin lymphoma, B-cell type of acute lymphocytic leukaemia), in one case during a cytomegalovirus infection and in the other during an infectious mononucleosis. At that time both patients were in complete remission. Paraproteinaemia in the first case disappeared within a year where the transformation from an initially four-banded paraproteinaemia (2 IgM-lambda and 2 IgG-lambda) into a three-banded paraproteinaemia (IgG-lambda) could be observed. In the second patient the concentration of the paraprotein decreased considerably. Because both patients were no longer under cytostatic treatment after manifestation of the paraproteinaemia, and were in complete remission during the whole of the observation period (4 years and 1 year), a direct relationship with the primary disease is unlikely. The transitory paraproteinaemia appears to be rather the result of an acquired defect of the immune system.

Agammaglobulinemia

[IgD plasmocytoma: clinical and differential diagnostic features (author's transl)].

A 64-year-old man died of IgD plasmocytoma only two months after onset of first symptoms. The course of the disease was characterized by marked renal insufficiency, hypercalcaemia with cardiac arrhythmias and bone destruction, in addition to raised phosphate concentration and alkaline phosphatase activity. The electrophoresis diagram was unremarkable and had no M-peak. But immunoelectrophoretic analysis of serum demonstrated a IgD paraprotein; in addition there was a monoclonal light chain, type lambda. At post-mortem the diagnosis of plasmocytoma was made; in addition there was renal amyloidosis.

Arrhythmias, Cardiac

Acquired isolated factor X deficiency associated with systemic amyloidosis. Case report and review of literature.

The demonstration of acquired isolated factor X deficiency and of a biclonal homogeneous lambda and kappa light polypeptide chain proteinuria in a patient of 49 years having an obscure hepatomegaly gave clinical evidence for the rate, amyloidosis-associated factor X deficiency, which could be proven by postmortem examination. The mechanisms by which amyloid may affect factor X levels in this case may be preferentially binding to the amyloid fibrils of lambda light chain type.

Amyloidosis

[Contribution to the aetiology of "reversible hepatic dysfunction" (Stauffer's syndrome) associated with renal tumours].

Biochemical changes, especially isolated rise in alkaline phosphatase and increased thromboplastin time, which have been described as "reversible hepatic dysfunction" (Stauffer's syndrome) were found in a 47-year-old patient with hypernephroma and hepatomegaly without liver metastases at post-mortem examination. The alkaline phosphatase could not be distinguished from the placental isoenzyme (Regan's enzyme). Increased thromboplastin time was due to circulating fibrinogen degradation products.

Adenocarcinoma

Phytohaemagglutinin-induced lymphocyte transformation and histologic findings in patients with chronic aggressive hepatitis before and during immunosuppressive therapy.

In vitro lymphocyte transformation (14C-thymidine incorporation rate following stimulation by PHA) was studied in 19 patients with histologically proven chronic aggressive hepatitis. In the majority of the cases measurements were performed prior to and during a combined (azathioprine/corticosteroid) immunosuppressive therapy. In comparison to normal controls, the majority of the patients showed a significantly reduced lymphocyte transformation before the treatment started. During the immunosuppressive therapy there was a normalization of PHA-responsiveness in most of the patients, or an improvement, at least. Bioptic control seems to be the most reliable parameter to prove a therapeutic effect, since the histologic criteria for a decrease of the inflammatory activity were the last to occur. After transient cessation of the therapy, in 4 out of 6 cases a decrease of lymphocytic PHA-responsiveness was developed again, paralleled by histologic activation of the process where biopsy could be performed. This observation favours a long-term therapy for at least several years.

Adult