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Biomedical subjects

P Bettelheim

Publications and source records attributed to P Bettelheim.

At least 91 records · Page 5Linked to original sources
PubMed1988

T cell receptor alpha mRNA transcription in T-lymphoblastic transformation of chronic myelocytic leukemia.

Analysis at the DNA and RNA level revealed a mature genetic marker profile in a case of T type blast crisis of chronic myelocytic leukemia. T cell receptor beta chain gene rearrangement as well as T cell receptor alpha mRNA transcription was demonstrated in blasts of the malignant clone. Corresponding findings were obtained from immunological phenotyping. Blasts were found to be CD7+, CD1+, CD3+, CD4+, CD8+, and TdT- and classified as common/mature thymocytes. The presence of the breakpoint cluster region gene on chromosome 22 excluded the possibility of a second neoplastic process.

Adult↗
PubMed1988

[T- and B-cell double lymphoma: immunologic characterization using monoclonal antibodies].

The coexistence of two non-Hodgkin lymphomas in one patient is extremely rare. We describe a patient who suffered for 17 years from chronic lymphocytic leukemia of B-cell origin with bone marrow and peripheral blood involvement until he developed clinically typical mycosis fungoides of the skin. The neoplastic lymphatic cells in the skin infiltrates were T-cells of the helper/inducer phenotype and thus differed morphologically and by immunological markers from the neoplastic cells in peripheral blood and bone marrow. The clonal B-cell expansion was controlled well over many years by cytotoxic drug therapy, the cutaneous T-cell lymphoma only with limited success by oral photochemotherapy owing to lack of compliance. The patient died 3 years after the diagnosis of MF from a parallel exacerbation of both diseases. Extensive immunohistochemical studies verified the nature of both lymphomas in vivo and at autopsy.

Aged↗
PubMed1987

[Oral anticoagulant therapy--renaissance of an old therapy?].

Although the antithrombotic potential of oral anticoagulants is undisputed, bleeding complications constitute a serious problem. One of the main causes for these complications has been a lack of standardization of the prothrombin time. The introduction of the International Normalized Ratio (INR) has led to a better standardization of prothrombin time. Thus, the same level of anticoagulation can be reached using different reagents and therefore over- and undercoagulation can be avoided. Furthermore, the benefit/risk ratio can be improved by adapting the intensity of anticoagulation to the indication. The following clinical conditions are established indications for treatment with oral anticoagulants: Prevention of cardiac emboli in acute anterior myocardial infarction with atrial thrombus, in patients with atrial fibrillation with or without mitral valve disease, in patients with prosthetic heart valves and in patients with dilated cardiomyopathy. Furthermore, oral anticoagulants should be given to patients after femoropopliteal bypass. A relatively mild oral anticoagulant treatment (INR 2-3) is sufficient to prevent recurrences of venous thrombosis and pulmonary emboli. The duration of treatment in patients with venous thromboembolism depends on some clinical features and the results of clotting tests which indicate an increased tendency to thrombosis.

Administration, Oral↗
PubMed1987

[Right heart failure as a clinical sign of pulmonary artery sarcoma].

A 43 year-old female patient was admitted on account of severe dyspnoea of several months' duration and thrombocytopenia. The clinical symptomatology was compatible with pulmonary embolism, but no source of embolization was found and heparin therapy did not lead to clinical improvement. Angiography of the pulmonary artery revealed multiple filling defects. Symptoms improved after treatment with cortisone and so a malignant process was assumed. The patient died from right ventricular failure before the diagnosis could be established. At autopsy a sarcoma of the pulmonary artery, obviously originating from the pulmonary trunk was found. Thrombocytopenia most probably resulted from consumption of platelets by a mechanism corresponding to the Kasabach-Merritt syndrome. The difficulties in the diagnosis of pulmonary artery sarcomas are discussed and the 69 previously published cases are reviewed.

Adult↗
PubMed1987

In vitro culture studies of granulocyte/macrophage and erythroid progenitor cells in lymphoproliferative disorders.

Granulocyte/macrophage progenitor cells (CFU-GM) and erythroid progenitor cells (BFU-E) have been assayed in peripheral blood (PB) and/or bone marrow (BM) from 12 patients with acute lymphocytic leukemia (ALL), 16 patients with chronic lymphocytic leukemia (CLL) and 31 patients with various forms of non-Hodgkin lymphoma (NHL) without BM involvement. Progenitor cell growth in PB and BM from the NHL patients did not differ statistically from controls (p greater than 0.1). CFU-GM and BFU-E per ml PB were markedly increased in ALL and CLL patients (p less than 0.001) while CFU-GM and BFU-E per plated BM cells from these patients were severely depressed (p less than 0.001). Lymphoblasts from one ALL patient failed to inhibit CFU-GM and BFU-E-derived colony growth from control PB mononuclear cells. The high levels of circulating progenitor cells in ALL and CLL patients clearly distinguish them from other cytopenic hematological malignancies, in which decreased progenitor cell levels have been demonstrated previously (acute myeloid leukemia, hairy cell leukemia). The cause of this finding and its pathophysiological implication still remains to be established.

Bone Marrow↗
PubMed1987

Human blood basophils display a unique phenotype including activation linked membrane structures.

To evaluate the membrane marker profile of human basophils a panel of well-established monoclonal antibodies (MoAbs, n = 60) was used for a combined toluidine/immunofluorescence staining procedure. Myeloid-associated MoAbs (particularly MoAbs against the LFA-1 family (CD11, CDw18), MoAbs directed against lactosylceramide (CDw17), anti-glycoprotein (gp) 150 MoAbs MCS 2 and MY 7 (CDw13), anti-gp 67 MoAb MY 9, anti Fc gamma-receptor (mol wt 40 kd) MoAb CIKM5, anti-CR 1 MoAb E 11, and the antiglycolipid MoAb VIM-2) were reactive with basophils, indicating a close relationship to other mature myeloid cells. Under normal conditions, basophils surprisingly express at least three activation-linked structures not detectable on mature neutrophils, ie, the p45 structure defined by MoAbs OKT-10 and VIP-2b, the p24 structure identified by the CD9 MoAb BA-2, and the receptor for interleukin 2 (IL 2) recognized by three different MoAbs (anti-TAC, IL2RI, anti-IL 2). Moreover, under short-term culture conditions basophils both in mononuclear cell (MNC) suspension and as purified fractions display the HLA-DR and T4 antigens. The neutrophilic/eosinophilic structure 3-fucosyl-N-acetyllactosamine is expressed on basophils only after neuraminidase treatment. Basophils were not stained at all by CD 16 MoAbs directed against the Fc gamma-receptor (mol wt 50 to 70 kd) of neutrophils, by the MoAb 63D3 (CDw12) recognizing the monocyte/granulocyte-associated p 200 antigen, and by the CDw 14 antibodies (VIM-13, Mo 2) defining the monocyte-specific structure p 55. Enriched basophils freshly obtained from chronic granulocytic leukemia (CGL) patients yielded identical results in FACS analyses. In summary, these data indicate that basophils generate a unique combination of surface determinants and possibly represent an activated cell population.

Antibodies, Monoclonal↗
PubMed1987

T lymphocytes lack rearrangement of the bcr gene in Philadelphia chromosome-positive chronic myelocytic leukemia.

To study the possible involvement of T lymphocytes in Philadelphia chromosome (Ph)-positive chronic myelocytic leukemia (CML) we analyzed the arrangement of the bcr gene in T cell and non-T cell samples of 12 CML patients. Although all the patients showed bcr rearrangements in non-T cell fractions, T cell populations lacked respective gene recombinations. Moreover, by Southern blot analyses using T cell receptor beta chain sequences our data indicate polyclonality of T cell samples from 11 of 12 cases; in one patient a clonal T cell population could be identified. These results suggest that T lineages of most Ph-positive CML patients are not derived from pluripotent stem cells involved in leukemogenesis and thus confirm previous investigations based on cytogenetic or glucose-6-phosphate dehydrogenase analyses. The demonstration of polyclonal T cell populations may reflect persistence of stem cells committed to differentiate only into T cells.

Collodion↗
PubMed1986

A rapid and simple immunoperoxidase staining procedure for blood and bone marrow samples.

A fast and simple indirect immunoperoxidase staining technique is described, which can also be used for the characterization of cells with high endogenous peroxidase activity such as many haemopoietic cells. It is based on the combination of a newly developed glucose-oxidase plus glucose procedure for the inhibition of endogenous peroxidase activity with a standard 2 or 3 layer immunoperoxidase staining protocol. Glucose-oxidase plus glucose mixture completely inhibits endogenous peroxidase activity without having a detectable deleterious effect on any of the cellular antigens so far studied. In many instances this permits the use of only 1 layer of horseradish peroxidase (HRP)-labelled antibodies. The glucose-oxidase plus glucose mixture can also be added to cells together with the HRP-labelled antibody solution without losing its inhibitory effect for endogenous peroxidase activity and without leading to a visually detectable loss of the activity of the HRP conjugate. Consequently, the separate incubation step previously necessary for the inhibition of endogenous peroxidase activity becomes superfluous.

Antibodies, Monoclonal↗
PubMed1986

Acute leukemia with mediastinal mass, lymphadenopathy, and monocytic precursor cells.

Cell marker analysis with monoclonal antibodies (MoAb) as well as differentiation studies with the chemical inducer TPA were used to identify a population of apparently lymphoid cells as monocytic precursor cells in a patient with acute leukemia. The initial manifestation of the disease with mediastinal mass and lymphadenopathy was followed by the appearance of small lymphocyte-like blast cells in bone marrow (BM) and peripheral blood (PB). Although a lymph node biopsy revealed an infiltration with monoblasts, the leukemic cells in BM and PB were not classifiable. However, when the patient relapsed after chemotherapy with large monoblasts and again with morphologically lymphoid blast cells, the latter could be classified by treatment with TPA. After incubation with the inducer (12-48 hr) the cells became positive with the MoAB VIM-D5, showed a strong reaction with alpha-naphthyl-acetate-esterase and developed macrophage like morphology, as well as phagocytic properties. During the terminal phase of the disease, small, lymphocyte-like blast cells predominated. These cells could be classified by a panel of MoABs. They expressed myeloid determinants (VIM-D5, VIM-2, MY 9, VIM-12, VIM-13) but showed no reactivity with MoABs specific for lymphocytic cells.

Adult↗
PubMed1986

Myeloid progenitor cells in the peripheral blood of patients with hairy cell leukemia and other "leukemic" lymphoproliferative disorders.

We assayed granulocyte-macrophage committed progenitor cells (CFU-GM), erythroid committed progenitor cells (BFU-E) and pluripotent hemopoietic progenitor cells (CFU-MIX) in the peripheral blood of patients with hairy cell leukemia (HCL), acute lymphocytic leukemia (ALL) and chronic lymphocytic leukemia (CLL). In 8 HCL patients retaining their spleens, the number of circulating CFU-GM, BFU-E and CFU-MIX were under the lower limits of normal controls in 6, 6 and 5 cases, respectively, and were in the lower normal ranges in the remaining cases. Six splenectomized HCL patients had generally more circulating progenitor cells than their nonsplenectomized counterparts. In the peripheral blood of 2 patients with ALL and 3 patients with CLL, progenitor cells of all types were markedly increased compared to their respective values in the blood of control subjects. Hairy cells from 2 HCL patients failed to inhibit CFU-GM, BFU-E and CFU-MIX derived colony growth from control peripheral blood mononuclear cells. In 3 HCL patients previously low circulating progenitor cells did not rise 5-7 months after RC-alpha 2-IFN treatment despite normalization of peripheral blood counts. Our results suggest that a reduction of the committed and pluripotent progenitor cell compartment might be at least in part responsible for the pancytopenia in the majority of patients with HCL.

Adult↗
PubMed1986

Thromboembolism in patients with prosthetic heart valves. An adequately controlled intense anticoagulant therapy and its influence on the occurrence of thromboembolism in relation to valve type.

This study was designed to evaluate the efficacy of carefully controlled treatment with oral anticoagulants in patients with different mechanical heart valve prostheses. One hundred eighty-one patients with various types of prosthetic valves (mitral 89, aortic 87, combined 5) received oral anticoagulation aiming at Thrombotest (TT) values between 5% and 12%. Median follow-up was 46 months; 80.8% of all TT determinations were below 12%. The thromboembolic rate was 0.25%/year in patients with aortic valve replacement (AVR) and 4.87%/year in patients with mitral valve replacement (MVR). There was a strikingly lower incidence of thromboembolism with newer types of valves (Björk-Shiley convex-concave) in the mitral position under exactly the same intensity and stability of anticoagulant treatment. Clinically overt valve occlusion could be almost completely prevented (0.12%/year) in prostheses at both sites. Severe hemorrhage occurred at a rate of 1.71%/year and fatal bleeding at a rate of 0.37%/year. Our results indicate that carefully controlled anticoagulation is effective in the reduction of thromboembolic complications at a reasonable risk of bleeding.

4-Hydroxycoumarins↗
PubMed1986

CSF cytology of a patient with conversion of an acute lymphatic leukemia to an acute eosinophilic leukemia.

The course of leukemic disease in a male adolescent with meningeal leukemia is described. The bone marrow aspirates showed a conversion from an acute lymphatic leukemia to an eosinophilic leukemia. Four weeks after the peripheral shift of phenotype two different cell clones were detected in one CSF smear. While under ultrahigh dose araC therapy the patient died 3 months after conversion. Possible explanation for the shift of phenotype and the peculiar leptomeningeal infiltration are discussed.

Adolescent↗
PubMed1986

B-immunoblastic lymphoma arising in angioimmunoblastic lymphadenopathy.

We present a female patient with a B-immunoblastic lymphoma of the IgM-lambda type arising in angioimmunoblastic lymphadenopathy. An increased ratio of helper/inducer to suppressor/cytotoxic lymphocytes in the lymph node could have triggered the proliferation of B cells. Evolution of IgM cryoglobulinemia was paralleled by malignant transformation in the lymph node. A short-term in vitro chemosensitivity test could predict response to COP combination chemotherapy suggesting that in vitro chemosensitivity testing can be useful for the therapeutic management of angioimmunoblastic lymphadenopathy.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1986

[Clinical value of various classifications in adults with AML].

111 adults with acute myeloid leukemia were classified according to the morphological, immunological and cytogenetic features of their leukemic cells. A comparison was made between the clinical parameters on prognosis of these subgroups. Hemoglobin was the only parameter among all six FAB-subgroups to show a statistical significant difference. Other differences found applied to a few subgroups only. Again, immunologically or cytogenetically characterized subgroups did not show significant differences in clinical parameters or prognosis. Patients with pseudodiploid or hyperdiploid karyotype had a lower remission rate than the remainder. However, a cluster analysis in which all three classifications together with the clinical parameters were considered was best able to characterize groups with different prognosis.

Adolescent↗
PubMed1986

[Lineage infidelity of leukemia cells].

We phenotyped 548 blast populations from patients with acute de novo leukemia with a panel of 27 monoclonal antibodies and an antiserum against the enzyme terminal deoxynucleotidyl-transferase (TdT). In 56 cases (10,2%) we found a marker profile consisting of both myeloid and lymphoid characteristics (biphenotypic) leukemia = interlineage infidelity). In 49 out of the 56 cases the blast populations were clearly related to the myeloid (n = 41) or the lymphatic (n = 8) system. In those blast populations only one characteristic of the other differentiation could be detected. However, in 7 cases more than one myeloid as well as lymphatic characteristics were found on the same blast cells. In 4 cases (0.7%) the immunological phenotyping indicated the presence of two separate blast populations. The occurrence of a stem cell leukemia, an abnormal derepression of a genome, a defect of the membrane synthesis and the existence of a normal counterpart are taken into consideration as a cause of an "atypical" marker profile.

Acute Disease↗
PubMed1986

Acquired thrombasthenia due to GPIIb/IIIa-specific platelet autoantibodies.

An otherwise healthy woman developed a hemorrhagic diathesis with fluctuating clinical symptoms and laboratory findings, but without thrombocytopenia, over 8 years. In periods of bad clinical condition, a platelet defect, characteristic of thrombasthenia, was found. In contrast to classic thrombasthenia, electrophoresis of the patient's platelet membranes revealed normal amounts of glycoproteins IIb alpha, IIb beta, and IIIa in the normal positions. Monoclonal antibodies, specific for GPIIIa and GPIIb/IIIa, respectively, bound normally to the P1A1-positive platelets from the patient. Although no antibody and no platelet function inhibitor were evident in the autologous plasma, an IgG1 antibody that was bound to the patient's platelets and was directed against GPIIb/IIIa could be demonstrated. After elution from the patient's platelets, this antibody immunoprecipitated GPIIb (both subunits), IIIa, and a 200-kilodalton (kd) band (probably undissociated GPIIb/IIIa complex) from solubilized normal platelets, but did not react with thrombasthenic platelets. Adding the eluate from the patient's platelets to normal platelet-rich plasma immediately caused concentration-dependent inhibition of adenosine diphosphate (ADP)-induced and collagen-induced aggregation and also strong inhibition of ADP-stimulated fibrinogen binding. Because it was very unlikely from the patient's medical history that the antibody was caused by alloimmunization, the hemorrhagic diathesis must be interpreted as acquired thrombasthenia due to an anti-GPIIb/IIIa autoantibody.

Adult↗
PubMed1985

[Specific chromosome aberrations in 3 patients with Burkitt-like leukemia (Fab: L3)].

Karyotype analysis using the Tri-staining-technique (Chromomycin A3/Distamycin A/DAPI) and subsequent DAPI/AMD staining (Schweizer, 1981) was performed on the bone marrow and peripheral blood of three patients with "Burkitt-like" acute lymphatic leukaemia (FAB: L3). In two patients we found the specific translocation t(8;22). One patient displayed in addition a structural abnormality of chromosome 9, visualized with C-banding using the DA/DAPI stain, and in two patients we found specific abnormalities of chromosome 1. Several patterns of the abnormal chromosome 1 revealed an intercalated DA/DAPI positive C-band in the elongated long arm.

Adolescent↗