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Biomedical subjects

C Aul

Publications and source records attributed to C Aul.

At least 145 records · Page 8Linked to original sources

[Atypical joint symptoms as initial symptoms of varicella infection in chronic myeloid leukemia].

A 25-year-old man, known to have chronic myeloid leukaemia for four years, acutely developed a fever of 39.5 degrees and severe pain in the shoulder and hip joints. There was no evidence of joint disease. Treatment with indomethacin briefly improved the symptoms, but within 72 hours the patient developed a fulminant illness with high fever and clinical as well as biochemical signs of a severe consumption coagulopathy. Herpetiform efflorescences appeared over the head and trunk shortly before death. At autopsy there was histological and immunohistochemical evidence of a varicella infection with hepatitis, oesophageal involvement and severe internal organ bleedings. In immune-compromised patients with atypical prodromal symptoms a varicella infection must be considered so that causal treatment with acyclovir and hyperimmune-globulin can be begun in time.

Adult↗

Septicemia due to Streptococcus mitis in neutropenic patients with acute leukemia.

Eight neutropenic patients with acute lymphocytic or nonlymphocytic leukemia had septicemia due to different strains of Streptococcus mitis (St. mitis), a microorganism not commonly recognized as a special pathogen in leukemic patients. Four of the patients had been treated with high-dose cytosine arabinoside as part of the cytostatic regimen, six had a central venous line and four patients had oral lesions prior to the infection. Selective gut decontamination consisted of co-trimoxazole/colistin in five patients and quinolones in three patients. The first three patients died, either due to interstitial pneumonia with the adult respiratory distress syndrome (ARDS), or due to infection-triggered disseminated intravascular coagulation despite prompt empiric antibiotic therapy including vancomycin. The other patients improved after empiric supplementation of penicillin G (30 Mega/day) to the antibiotic regimen. Beginning ARDS in two of these patients dramatically responded to high-dose steroids. We conclude that St. mitis is a major pathogen in neutropenic leukemic patients. Infection appears to occur independently of acute leukemic cell type, regimen of selective gut decontamination, venous access, visible oral lesions or treatment with high-dose cytosine arabinoside. The clinical course of our patients raises questions about the value of commonly recommended empiric antibiotic regimens, which were clearly ineffective to control infections with St. mitis in this patient group. Our data indicate that immediate antibiotic therapy with penicillin G is indicated and may be life-saving for suspected St. mitis infections in neutropenic leukemic patients.

Adult↗

Infection prophylaxis in neutropenic patients with acute leukaemia--a randomized, comparative study with ofloxacin, ciprofloxacin and co-trimoxazole/colistin.

Preliminary results are presented of an ongoing, prospective, randomized, study comparing ofloxacin, ciprofloxacin and co-trimoxazole/colistin for the prevention of infection in patients with acute leukaemia. The results for 59 patients (median age 47 years, range 21-72) included 88 episodes of neutropenia, each associated with a course of cytotoxic therapy. The main factor measured was the time elapsed from the beginning of neutropenia (neutrophils less than 500/microliter) until the first infectious febrile episode. The median time for the period was 12 days (range 1-56) for the cotrimoxazole/colistin group, 15 days (range 1-38) for the ofloxacin group and 20 days (range 1-36) for the ciprofloxacin group (differences not significant). Microbiologically proven major infections occurred in 10/27 treatment courses with co-trimoxazole/colistin 7/31 courses with ofloxacin and 7/30 courses with ciprofloxacin (P not significant). These were mostly due to Gram-positive cocci. There were no Gram-negative infections in the quinolone groups compared with one major Pseudomonas aeruginosa infection in the co-trimoxazole/colistin group. No Pneumocystis carinii infections were encountered. Adverse reactions associated with co-trimoxazole/colistin required discontinuation of medication in 11/27 treatment courses because of compliance problems, skin reactions or gastrointestinal intolerance. There were significantly fewer discontinuations in the ofloxacin (n = 2) and in the ciprofloxacin groups (n = 3). Major side effects of the quinolones included persistent icterus in one patient receiving ofloxacin and psychiatric symptoms in one patient receiving ciprofloxacin. It is concluded from these data that there were no statistically significant differences between the three treatment groups in respect of the prevention of infection.(ABSTRACT TRUNCATED AT 250 WORDS)

Bacterial Infections↗

Two types of acquired idiopathic sideroblastic anaemia (AISA)

On cytological bone marrow examination we distinguished between pure sideroblastic anaemia (PSA), which is confined to dyserythropoiesis, and refractory anaemia with ring sideroblasts (RARS), which is characterized by additional dysplastic features of granulopoiesis and/or megakaryopoiesis. In a follow-up study of 94 patients with AISA diagnosed according to FAB criteria for myelodysplastic syndromes we found a striking difference in the risk of leukaemic transformation between PSA and RARS (5 year cumulative rate 1.9% v. 48%). Overall survival was much better in PSA than in RARS (5 year cumulative chance 69% v. 19%). Infections and haemorrhages were frequent causes of death in RARS but not in PSA. Bone marrow culture studies (CFU-GM) were performed on 10 consecutive patients with PSA and RARS, respectively. RARS patients showed grossly impaired colony growth, typical of the myelodysplastic syndromes. Patients with PSA had persisting colony formation, even if moderately decreased in frequency, with numbers of CFU-GM being inversely correlated with the degree of erythroid hyperplasia in the bone marrow. We conclude that on cytomorphological grounds AISA can be divided into pure (dyserythropoietic) sideroblastic anaemia (PSA) and a true myelodysplastic form (RARS), with both types differing considerably in terms of survival, risk of leukaemic transformation and findings on bone marrow culture (CFU-GM).

Acute Disease↗

The role of low-dose cytosine arabinoside and aggressive chemotherapy in advanced myelodysplastic syndromes.

The efficacy of low-dose cytosine arabinoside (Ara-C) and aggressive chemotherapy was assessed in 67 patients with advanced myelodysplastic syndromes (MDS). In most cases, treatment was started because of worsening peripheral cytopenia, increase in bone marrow blasts, or transition of MDS to acute myeloid leukemia (AML). Of 51 patients (age range, 18-82 years) receiving low-dose Ara-C by subcutaneous bolus injection (10 mg/m2 every 12 hours) or continuous intravenous infusion (20 mg/m2/day), nine (18%) entered complete remission (CR) and four (8%) had a partial response (PR). Duration of CR varied from 4 to 25+ months. Overall survival of patients treated with Ara-C was not superior to that of a historical control receiving supportive care only. Hematologic toxicity of low-dose Ara-C was considerable, with 12 patients (24%) dying of hemorrhage or infection during the initial treatment course. Sixteen patients (age range, 17-65 years) who presented with a Karnofsky score of more than 80% were chosen for aggressive chemotherapy using standard AML protocols. In this group, nine CR and two PR were obtained. Early death from pneumonia occurred in two patients, and three patients had refractory disease. The factors most strongly associated with successful remission induction were (1) presence of Auer rods in granulocyte precursors, and (2) a comparatively low medullary blast count (less than 30%) at the start of treatment. Median duration of bone marrow aplasia for patients entering CR was 21 days (range, 6-51). Prolonged remissions (22+, 27+, and 29 months, respectively) could be achieved in three of four patients receiving consolidation and maintenance chemotherapy after induction of CR. From these data we conclude that aggressive chemotherapy should not generally be considered contraindicated in advanced MDS. In patients with a good Karnofsky score, this form of treatment may be more advantageous than the currently favored low-dose Ara-C, which is also myelotoxic, but induces remissions in only a minority of patients.

Adolescent↗

Age related randomized comparison of sequentially applied high-dose versus intermediate dose cytosine arabinoside in combination with mitoxantrone (S-HAM) in the treatment of relapsed and refractory acute myeloid leukemia: study design and preliminary results.

In a prospective randomized trial, the pending question was addressed whether Cytosine arabinoside (Ara-C) should be applied at high or intermediate dose to patients with relapsed or refractory acute myeloid leukemia. Based upon the previously established regimen of the sequential application of Ara-C and Mitoxantrone (S-HAM) patients below 60 years of age were randomized to receive Ara-C at either 3.0 g/m2 vs 1.0 g/m2 per dose while older patients were randomly assigned to either 1.0 g/m2 or 0.5 g/m2 Ara-C. At the present early stage 51 patients have entered the study and 37 are currently evaluable for response and toxicity. Complete remissions were achieved in 14 of 28 patients below 60 years of age and in 3 of 8 older cases. Predominant side effects consisted of nausea and vomiting, diarrhea and stomatitis. Further recruitment of patients and longer follow-up is required for the assessment of the various treatment arms.

Age Factors↗

[Alcohol-induced disorders of the hematopoietic system].

Alcohol has a variety of pathologic effects on hematopoiesis. It directly damages erythroid precursors, thereby contributing to macrocytosis and the anemic state of chronic alcoholics. Megaloblastic anemia in chronic alcoholism results from a combination of nutritional deficiency and the effect of ethanol as a folate antagonist. Experimental studies suggest that alcohol may disturb hepatic folate metabolism. Ethanol induces sideroblastic anemia, perhaps by direct interference with heme synthesis. Further, chronic ingestion of alcohol can lead to various types of hemolytic anemia caused by alterations in the erythrocyte membrane lipids which occur in association with alcoholic liver disease. Alcohol directly suppresses platelet formation and decreases the platelet life span. These two mechanisms contribute to thrombocytopenia which is a common complication to chronic alcoholism. Since ethanol also interferes with platelet function, prolongation of bleeding the time is common at all stages of alcoholism. Chronic ingestion of alcohol is associated with a diminished marrow granulocyte reserve and may lead to neutrocytopenia. Ethanol-induced dysfunction of granulocytes, monocyte-macrophages and T-lymphocytes undoubtedly contributes to the predisposition to infection observed in alcoholics. In contrast to alcohol-induced changes in liver, heart and central nervous system, hematopoietic disorders are reversible after alcohol withdrawal.

Alcoholism↗

Acute myelofibrosis in megakaryoblastic leukemia with translocation between chromosomes 8 and 14.

In a 45-year-old woman with severe normochromic anemia (Hb 2.8 g%) an extensive myelofibrosis and infiltration of the bone marrow with small blasts was observed histologically. Cytochemical examination of the blasts showed a negative peroxidase and a strongly positive alpha-NE reaction. PAS reaction was slightly granular positive in the cytoplasmic protuberances of the blasts and in the platelets. Marker analysis yielded no evidence of lymphatic origin of the blasts. In flow-cytometric studies of 230,000 cells a homogeneous 2c blast population could be identified. Cytogenetic analysis revealed an abnormal pseudo-diploid karyotype characterized by 2 acrocentric marker chromosomes caused by a translocation of chromosomes 8 and 14, as usually seen in Burkitt type lymphoma. Finally the reaction product of platelet-specific peroxidase could be demonstrated in the perinuclear cisternae of the endoplasmic reticulum by electron microscopy. Highly elevated beta-thromboglobulin and platelet factor 4 plasma levels were also measured. Following an ineffective treatment with daunoblastine and ARA-C, the patient died of pseudomonas aeruginosa septicemia after having received high-dose ARA-C treatment.

Biopsy, Needle↗

[X-ray image and differential diagnosis of pulmonary changes in Hodgkin's disease].

Of 291 patients with histologically confirmed lymphogranulomatosis, 45 (15.5%) with lung involvement were assessed. 22 (49%) of the patients with affected lung had the nodular sclerosing type of Hodgkin's disease. Radiologically, 15 patients showed a focal solitary manifestation of lymphogranulomatosis, whereas in 19 patients multiple, plane or circumscript areas of shadow were seen in one or both lobes of the lung. 11 further patients had unilateral or bilateral pleural effusions. Nodular (35%) and pneumonic (22%) opacities are the most frequent x-ray manifestations of pulmonary lymphogranulomatosis. The variable appearance of the lesions makes it difficult to differentiate against pulmonary infections, especially inflammatory complications, the incidence of which may be higher with these patients. In view of the far-reaching consequences, therefore, the nature of the pulmonary lesions should be clarified by biopsy in case of doubt, as early as possible (transbronchial biopsy and, if necessary, open lung biopsy).

Adult↗

[Thrombotic thrombocytopenic purpura].

Thrombotic thrombocytopenic purpura (TTP) is an uncommon disorder which usually occurs in young adults. It is characterized by a pentad of clinical findings: fever, neurological abnormalities, renal dysfunction, microangiopathic hemolytic anemia and thrombocytopenia. The histological hallmark is the presence of platelet thrombi occluding the microcirculation of multiple organs. The etiology and pathogenesis of disseminated platelet aggregation are uncertain and obviously not uniform in individual patients. Experimental findings suggest that microthrombi may result from intravascular platelet activation or form secondarily at sites of vessel wall damage. The differential diagnosis of TTP includes the hemolytic uremic syndrome in which the microangiopathic changes are exclusively found in the kidneys. When untreated, TTP invariably runs a progressive and fatal course. In recent years, prognosis has been improved by new forms of therapy such as plasmapheresis or infusions of fresh frozen plasma which may lead to recovery in about 80% of patients.

Blood Coagulation Factors↗