A new family of human endogenous retroviral sequences was recently discovered by way of its relationship to the simian sarcoma-associated virus (SSAV). One molecular clone, termed S71, contains sequences related to the genes coding for the group-specific antigens (gag) and polymerase (pol) proteins of SSAV. At the 3' end of this human retroviral element we have now found a 535-bp region which shows features characteristics of a retroviral long terminal repeat, including potential signal sequences essential for transcriptional control. By means of Southern blotting and in situ hybridization, the sequence was mapped to chromosome 18 band q21.

Animals↗

PubMed1988

[IgD paraproteinemia in immunocytoma].

The extremely rare immunocytoma with IgD-paraproteinemia was observed in a 65-year-old man with infiltrations of the throat and the sinuses. The diagnosis was made on the basis of several biopsy specimens and serum immunoelectrophoresis. In spite of intensive chemotherapy and radiotherapy a remission was not achieved. In the final stages, a transformation into an immunoblastic sarcoma accompanied by an acute plasma-cell leukemia appeared. The patient died 11 months after diagnosis from renal failure due to infiltrations of the kidneys. The poor prognosis and the poor response to radiotherapy may be associated with the IgD-paraproteinemia. Serum electrophoresis and immunoelectrophoresis proved to be sensitive parameters for monitoring tumor recurrence.

Aged↗

PubMed1988

Essential thrombocythemia. Clinical characteristics and course of 61 cases.

Sixty-one patients with essential thrombocythemia (ET) were followed from 1974 through 1987 at the Medizinische Poliklinik. Fifty-one patients (84%) presented with thromboembolic complications, and eight patients (13%) with hemorrhages. In seven patients (12%), a thrombocytosis was detected accidentally. Disturbances of the microcirculation (67%), mainly of the fingers and toes (53%), were the most frequent thromboembolic symptoms. The mean age of all patients was 58 years (male patients, 61 years; female patients, 56 years). The average platelet count at diagnosis was 897,000/microliter. The average maximal platelet count was 1.231 X 10(6)/microliter (range, 500,000/microliter to 4 X 10(6)/microliter). Seventy-two percent had a moderate leukocytosis (average, 12,400/microliter), 34% a splenomegaly, 29% a hepatomegaly. Signs of hypermetabolism were infrequent, lactate dehydrogenase (LDH) and uric acid elevations, if present, were moderate. Bleeding time and viscosity were normal in most patients. Spontaneous platelet aggregation was increased in 81% of patients (n = 40). Platelet aggregation studies with the aggregation inducing substances adenosine diphosphonate (ADP), platelet activating factor (PAF), thrombin, collagen, and adrenalin showed hypoaggregation in most patients. Adrenalin-induced aggregation distinguished best between ET-patients and reactive thrombocytosis showing hypoaggregation in all ET-patients tested (n = 16) and in none of 22 controls. Bone marrow studies were performed in 57 patients. The histologic studies (done in 49 patients) were consistent with a chronic myeloproliferative disorder in all cases. In 41 cases (84%) the picture of a megakaryocytic myelosis was found, in 12 of these a granulocyte-rich form of megakaryocytic myelosis. Cytologic studies only (eight patients) did not differentiate ET well from reactive thrombocytosis. Platelet aggregation studies and bone marrow histology may be of help in the diagnosis of difficult cases of thrombocytosis. The Philadelphia status was negative in all cases studied (14 patients). Fourteen patients died. The causes of death were thromboembolic complications in probably 11 and acute leukemia in two patients. The probability of 10-year survival is 64% after a mean follow-up time of approximately 5 years. It appears that considering the average age of ET patients at diagnosis, life expectancy is close to normal.

Bone Marrow↗

PubMed1988

Trimethoprim-sulfamethoxazole desensitization in AIDS.

Trimethoprim-sulfamethoxazole (TMS) desensitization was carried out in three patients with AIDS and Pneumocystis carinii pneumonia (PCP) in whom treatment with TMS had to be discontinued after 8 to 12 days due to an allergic reaction. Although the pneumonia was under control we decided for a desensitization to TMS because of the frequent reinfection and the high mortality rate particularly if treatment is incomplete. On the first day the patients took 0.4 mg/2 mg trimethoprim/sulfamethoxazole orally. The dose was increased during 9 successive days to 80 mg/400 mg trimethoprim/sulfamethoxazole. From the 10th to the 16th day 160 mg/800 mg trimethoprim/sulfamethoxazole was given daily and subsequently twice daily which is the recommended dose for prophylaxis of PCP. The desensitization was successful in two patients and a PCP prophylaxis was possible.

Acquired Immunodeficiency Syndrome↗

PubMed1988

[Clinical characterization of essential thrombocythemia in comparison with other myeloproliferative diseases and reactive thrombocytoses].

60 patients with essential thrombocythemia (ET) have been retrospectively and prospectively followed from 1974 through 1987. The presenting signs and symptoms and the course of the disease were analyzed and compared to 10 patients with persisting reactive thrombocytosis selected from 6,000 patients with reactive thrombocytosis and to 50 patients with other myeloproliferative diseases. 54 ET-patients presented with complications, 46 with thrombembolic, 3 with hemorrhagic problems and 5 with thrombembolic and hemorrhagic problems. In 6 patients ET was detected accidentally. Disturbances of the microcirculation, mainly of the fingers and the toes, were the most frequent symptom. The average maximal platelet count was 1,207,000/microliter. The average platelet count at diagnosis was 880,000/microliter. 16 patients had an elevation of the serum creatinine at diagnosis, which deteriorated during the course of the disease. Bone marrow examinations were performed in 56 patients, histology in 48 patients, cytology in 29 patients. In contrast to the clinical diagnosis the histological diagnosis was in 4 cases each polycythemia vera and myeloproliferative syndrome without further specification. 12 patients died thus far. The causes of death were thrombembolic complications in 9, acute leukemia in 2 patients, in 1 patient the cause of death is not known. 10 years after diagnosis 61% of the patients are still alive. It appears that ET is a more important risk factor for the disturbances of the micro- and macrocirculation than has been recognized until now. ET is, if thrombembolic complications are avoided, a disease with a relatively benign course.

Aged↗

PubMed1988

[Thrombocyte function in essential thrombocythemia and reactive thrombocytosis].

Blood of 16 patients with essential thrombocythemia (ET), 9 patients with reactive thrombocytosis (RT) and 13 healthy persons was used for platelet aggregation studies. When the aggregation was induced with adenosine diphosphate (0.01 microM), collagen (0.1 micrograms/ml) or platelet activating factor (PAF 0.5 microM) the plasma of the patients with ET showed significantly decreased aggregation (35%-44% of the value for the control groups). Independent of inhibitors of platelet aggregation, thrombin (0.05 U/ml) caused similar aggregation in healthy controls and patients with ET; patients with RT showed an increase aggregation. Adrenalin-induced aggregation discriminated best between patients with ET and controls. Adrenalin in concentrations ranging from 0.01 micrograms/ml to 100 micrograms/ml caused comparable dose-related amounts of aggregation in healthy controls and patients with RT. Over the whole concentration range, patients with ET showed significantly decreased aggregation (28%-34% of the value for the control groups). This difference proved to be independent of the influence of inhibitors of platelet aggregation. Though concentrations of alpha1-acid glycoprotein never reached inhibitory levels in the plasma of patients with ET (n = 12) they were significantly higher compared with those in normal plasma (n = 12). Fibrinogen concentrations in plasma of ET-patients (n = 12) were in the normal range. Cellular adenosine 3'-5'-cyclic monophosphate concentrations in ET (n = 10) are comparable with normal values (n = 5). The significance of the results for diagnosis and better pathophysiological understanding of ET is discussed.

Blood Platelets↗

PubMed1988

Randomized study on the treatment of chronic myeloid leukemia (CML) in chronic phase with busulfan versus hydroxyurea versus interferon-alpha.

For palliative therapy during the chronic phase of CML busulfan has proved to be the drug of choice. During the past years hydroxyurea and also interferon-alpha have gained increasing significance since they might prolong the duration of the chronic phase. In a multicenter study it is being determined, whether the use of hydroxyurea or of interferon-alpha instead of busulfan prolongs the duration of the chronic phase of Philadelphia positive CML. Additional goals are the examination of whether the types of disease evolution and the terminal phases differ between the treatment groups, and the prospective recognition of prognostic criteria for the duration of the chronic phase of CML. By December 31, 1987, 326 CML-patients had been randomized, 150 for busulfan, 150 for hydroxyurea and 26 for interferon-alpha. The average age is 50 years. 59 patients reached the end of the chronic phase, 55 died. The mean observation time of all patients is 1.34 years. At present no significant difference in survival is recognizable between the busulfan and hydroxyurea groups. Fewer adverse effects have been observed in the hydroxyurea group. Philadelphia chromosome negative patients show a higher average age and tend to have lower white blood cell and platelet counts. The number of patients having received interferon-alpha is still too small to allow evaluation. This report intends to document organization and progress of this study which to our knowledge is, at present, the largest ongoing prospective multicenter study on the therapy of CML.

Busulfan↗

PubMed1988

[Chemotherapy of chronic myelocytic leukemia. Status of the German Multicenter Study on busulfan versus hydroxyurea versus alpha interferon, October 1897].

For palliative therapy during the chronic phase of CML busulfan has proved to be the drug of choice. During the past years hydroxyurea and also interferon-alpha have gained increasing significance since they might prolong the duration of the chronic phase. In a multicenter study it is being determined, whether the use of hydroxyurea or of interferon-alpha instead of busulfan prolongs the duration of the chronic phase of Philadelphia-positive CML. Additional goals are the examination, whether the types of disease evolution and the terminal phases differ between the treatment groups, and the prospective recognition of prognostic criteria for the duration of the chronic phase of CML. By November 26, 1987, 321 CML-patients had been randomized, 147 for busulfan, 149 for hydroxyurea and 25 for interferon-alpha. The average age is about 50 years. 59 patients reached the end of the chronic phase, 55 died. The mean observation time of all patients is 1.34 years. At present no significant difference in survival is recognizable between the busulfan and hydroxyurea groups. Fewer adverse effects have been observed in the hydroxyurea group. Philadelphia chromosome-negative patients show a higher average age and tend to have lower white blood cell and platelet counts. The number of patients having received interferon-alpha is still too small to allow evaluation. This report intends to document organization and progress of this study which to our knowledge is, at present, the largest ongoing prospective multicenter study on the therapy of CML.

Busulfan↗

PubMed1988

Human endogenous retroviruses.

One of the unique features of retroviruses is their ability to integrate their genetic information in the genomes of their host cells, including the germ line, and to persist there as so-called proviruses. Proviruses which are contained in the germ line of a given species and are inherited from generation to generation like cellular genes are called endogenous retroviruses (for review see 1). Although the function or bioiological role of endogenous retroviruses still remains to be elucidated, they have been detected in almost all vertebrate species examined. The most relevant properties of endogenous, genetically transmitted retroviruses are summarized in Table 1. Endogenous retroviruses persist in cellular DNA, are transmitted through the germ line, and possess a transposon-like structure (2) which enables them to integrate at any position of the cellular genome. Endogenous retroviruses can be activated by certain chemicals such as mutagens/carcinogens or mitogens, by radiation, and by other mechanisms such as DNA-viruses or physiological processes (e.g. aging) to express antigens or to form infectious virus particles. Their biological relevance is unknown but may include involvement in physiological processes such as protection against superinfection by related retroviruses, similar to observations made with exogenous retroviruses in some animal model systems.

Blotting, Southern↗

PubMed1988

[Early manifestations of infection with HIV. Stages and early symptoms].

Explore the source record for details and available documents.

AIDS-Related Complex↗

PubMed1988

[Epidemiology of HIV infection].

Explore the source record for details and available documents.

Acquired Immunodeficiency Syndrome↗

PubMed1987

[Bronchoscopic detection of pneumocystis carinii pneumonia].

Explore the source record for details and available documents.

Acquired Immunodeficiency Syndrome↗

PubMed1987

[24-year survival in chronic myeloid leukemia].

A 55-year-old woman was diagnosed as having chronic myeloid leukemia in 1961. After 9 years with stable white blood cells, progression of the disease was noted (white cells up to 180 X 10(9)/l; splenomegaly to the umbilicus). Busulfan was given over 4 weeks resulting in a remission of 13 years duration. Progression was observed again in 1983, 22 years after the initial diagnosis of chronic myeloid leukemia. Therapy with hydroxyurea resulted in another remission of shorter duration. Two chromosome studies showed the Philadelphia chromosome in 100% of metaphases without additional aberrations. In 1985 blast crisis developed. The cause of death as determined at autopsy was an undiagnosed miliary tuberculosis. The presented case is of special interest since (1) it is a report of the longest surviving patient with chronic myeloid leukemia, and (2) in contrast to other cases with long survival, this patient did not show chromosomal mosaicism or any additional chromosomal aberrations.

Aged↗