Prevention of postsplenectomy sepsis.
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Humans↗
Biomedical subjects
U Germing
Publications and source records attributed to U Germing.
[Aspects of internal medicine].
Chronic infectious diseases may run over many months and years, accompanied by only mild clinical signs and often without organ-specific symptoms. People with any kind of immuno deficiency are more frequently affected by chronic infections. History, physical examination, laboratory tests, microbiological tests and imaging methods may lead to an exact diagnosis. The most important differential diagnosis are autoimmune diseases, amyloidosis and malignant lymphoma.
Bacterial Infections↗
[Bone marrow involvement in hematologic neoplasms and solid tumors].
Bone marrow involvement is often observed in patients with hematological malignancies and solid tumors. Sensitive immunological and molecular biological methods allow the detection of isolated transformed cells in bone marrow samples. Like normal hematopoietic cells, tumor cells interact with bone marrow stroma through adhesion molecules. Since adhesion molecules play an essential role in the invasion and proliferation of malignant cells, new therapeutic approaches involving the inhibition of cell-cell and cell-matrix interactions are conceivable.
Bone Marrow↗
Pericardial effusion in chronic myelomonocytic leukemia (CMML): a case report and review of the literature.
Pericardial effusion is a rare but potentially life-threatening complication of chronic myelomonocytic leukemia. A case of a 70-year-old female with CMML is reported, who developed a severe pericardial effusion after an initially stable course of disease. She underwent pericardiocentesis and subsequent intracardial instillation of mitoxantrone with poor response. Subxiphoid pericardiotomy was successfully performed. Leukocytosis did not respond to hydroxyurea. Because of decreasing performance status of the patient systemic chemotherapy was not applied and the patient died within 2 weeks. A review of the literature shows that there is no effective treatment for pericardial effusion in CMML, and some common features may be noted.
Aged↗
Validation of the WHO proposals for a new classification of primary myelodysplastic syndromes: a retrospective analysis of 1600 patients.
In 1982, the French-American-British (FAB) cooperative group proposed a classification of myelodysplastic syndromes (MDS) based on morphological features in blood and bone marrow, namely on medullary and peripheral blast count, Auer rods, ring sideroblasts and the number of monocytes in the peripheral blood. This classification has been used for numerous studies regarding morphology, prognosis and treatment of MDS. Some details of this morphological classification remained unclear, and some patients were unclassifiable. A working group of the World Health Organization (WHO) recently proposed a new classification of MDS, based on a significant modification of the original FAB proposals. CMML and RAEB-T were removed from the MDS classification and RAEB was split into two groups with medullary blast counts below and above 10%. In addition, a group of patients with less than 5% medullary blasts but evidence of multilineage dysplasia was defined. MDS patients with 5q- as the sole chromosomal anomaly were also considered a separate group. The aim of the present study was to validate the new classification with respect to prognostic importance, and to correlate it with cytogenetic and hematological features in a large series of patients (n=1600) with a long-term follow up. We were able to confirm a significant difference in prognosis between RAEB I and RAEB II, as well as a difference between refractory anemia and multilineage dysplasia. Furthermore, patients with 5q- anomaly had a much better prognosis than other WHO subtypes, but this was only true for patients with a medullary blast count below 5%. In summary, the WHO classification appears to define morphological subgroups that are more homogeneous with respect to prognosis than the FAB subtypes.
Adolescent↗
Acute promyelocytic leukemia and pregnancy.
In acute promyelocytic leukemia (APL), the use of all-trans-retinoic acid (ATRA) as a differentiating agent induces complete remission in a high percentage of patients. In pregnancy, however, this drug bears the risk of severe teratogenicity to the child. We report the case of a 23-yr-old woman at 21 weeks' gestation suffering from APL. She was treated with ATRA (45 mg/m2) for 40 d and two courses of standard chemotherapy. The mother achieved complete remission within 22 d of treatment. Fetal development was normal, and a healthy premature girl was born in the 35th week of pregnancy. In a review of the literature we have identified 14 cases of APL in pregnancy treated with ATRA alone or in combination with chemotherapy. ATRA has been used as early as in the 3rd week of gestation and in no case have malformations or other teratogenic effects occurred. Side-effects, however, ranged from fetal cardiac arrhythmias to induction of labour. Although known to exhibit severe teratogenic effects during the first trimester of pregnancy, ATRA seems to be reasonably safe during the second and third trimesters in the treatment of APL. However, careful obstetric follow-up is mandatory regarding fetal cardiac complications.
Adult↗
Two types of acquired idiopathic sideroblastic anaemia (AISA): a time-tested distinction.
In 1982, acquired idiopathic sideroblastic anaemia (AISA) was included by the French-American-British (FAB) Co-operative Group in their classification of myelodysplastic syndromes (MDS). However, the malignant potentiality of AISA has always been a matter of debate. In different series, median survival and rates of transformation into acute myeloid leukaemia (AML) varied extensively. On cytomorphological grounds, AISA can be divided into pure (dyserythropoietic) sideroblastic anaemia (PSA), in which dysplasia is confined to erythropoietic cells, and a true myelodysplastic form (RARS), which is characterized by additional dysplastic features of granulopoiesis and/or megakaryopoiesis. In a previous study, based on retrospective analysis of 94 patients with AISA, we found that both types of sideroblastic anaemia differed considerably in terms of survival and risk of AML transformation. Almost identical results have now been obtained through a prospective study of 232 new patients with AISA. The difference in survival between PSA and RARS remained significant over the whole period of follow-up (survival after 3 years being 77% vs. 56%; P = 0.003), and the incidence of AML did not increase with time in the PSA group, even in the long term. This prospective study strongly supported our conclusion that cytomorphological distinction between PSA and RARS provides valuable prognostic information.
Adult↗
Long-term remission after intensive chemotherapy in advanced myelodysplastic syndromes is generally associated with restoration of polyclonal haemopoiesis.
The clonality of peripheral blood cells was assessed in eight female patients with myelodysplastic syndrome (MDS) by means of the human androgen receptor gene-based assay (HUMARA). The patients were in complete remission for a median follow-up time of 83 months after intensive chemotherapy. X-chromosome inactivation patterns (XCIPs) indicated polyclonal haemopoiesis in five patients. Two patients had skewed lyonization (i.e. unbalanced XCIPs in both granulocytes and T cells) and one patient presented monoclonal granulocytes together with polyclonal T cells. We conclude that long-term remission in MDS following intensive chemotherapy is usually associated with polyclonal haemopoiesis.
Adult↗
[Clinical course, morphology and prognosis of chronic myelomonocytic leukemia].
BACKGROUND: The FAB group proposed to distinguish 2 subgroups of chronic myelomonocytic leukemia (CMML): Depending on the total leukocyte count a myelodysplastic type (< 13,000/microliter) was separated from a myeloproliferative type (> 13,000/microliter). Prognostic factors are not well-established until now. PATIENTS AND METHODS: Based on retrospective analyses of patients with CMML diagnosed at our institution, we compared the presenting clinical and hematological features of both disorders and examined the natural course of the disease and prognostic factors. RESULTS: Out of 225 patients with CMML there were 115 patients with myelodysplastic type (MDS-CMML) and 110 patients with myeloproliferative type (MPD-CMML). Median age of patients at diagnosis and sex ratio were not different. Splenomegaly and hepatomegaly were more common in MPD-CMML. With regard to laboratory findings, patients with MPD-CMML presented with significantly higher LDH values. Except for WBC, peripheral blood counts were not different. Median percentage of bone marrow blasts was 8% in both disorders. Signs of bone marrow dysplasia were comparable in both disorders. Cumulative survival rates were similar in both disorders. Five years after diagnosis, actuarial survival for patients with MPD-CMML was 24%, as compared to 15% for patients with MDS-CMML. The probability of transformation to AML was higher in MDS-CMML (29% vs 18% after 5 years). Elevated LDH values, low hemoglobin values and male sex were independent risk factors for the entire group and for the MDS-CMML group. Using the Düsseldorf score, we could define risk groups within MDS-CMML with a median survial of 12 vs 40 months (p = 0.00005). Prognostic factors could not define risk groups within the MPD-CMML group. CONCLUSIONS: In summary, these data suggest that MDS-CMML and MPD-CMML are clinically distinguishing conditions, but the separation provides little prognostic information. The Düsseldorf score can be used to provide risk stratification in CMML.
Adult↗
Congenital asplenia detected in a 60 year old patient with septicemia.
A 60 year-old female who had never been seriously ill, was brought to the emergency ward after she had been found somnolent at home. She developed renal failure, meningitis, respiratory distress and disseminated intravascular coagulation. Overwhelming septicemia was evident, and streptococcus pneumoniae was isolated from blood and cerebrospinal fluid. Surprisingly, peripheral blood smears showed numerous Howell-Jolly-bodies, indicating severe impairment of splenic function. On abdominal ultrasound, CT-scan, and scintigraphy no spleen could be detected. There was no history of abdominal surgery. Apparently, congenital asplenia, which was not noticed until the age of 60, was responsible for the patient's life-threatening septicemia. We suggest that, in cases of severe septicemia, the examination of a blood smear is useful to detect functional (or congenital) asplenia.
Bacteremia↗
[Role of aggressive treatment strategies for myelodysplastic syndromes].
Myelodysplastic syndromes (MDS) constitute a heterogenous group of acquired bone marrow disorders characterized by ineffective hematopoiesis, cellular dysfunction and an increased risk of transformation into acute myeloid leukemia (AML). The percentage of medullary blast cells and the karyotype at diagnosis are the most important predictors of survival. Patients with more than 10% blast cells or an unfavourable karyotype (chromosome 7 abnormalities or complex aberrations) usually survive less than 12 months. This review article focuses on the roles of intensive AML-type chemotherapy, autologous stem cell transplantation and allogeneic bone marrow transplantation in the management of patients with advanced MDS. Recent studies suggest that, with appropriate selection of patients, intensive chemotherapy produces high rates of complete remission. Chances of entering remission are particularly high in patients with a good Karnofsky score, bone marrow blast count < 30% and normal karyotype. When compared with acute myeloid leukemia, results of autologous bone marrow transplantation in MDS are disappointing. A major disadvantage of this approach is the delayed recovery of hematopoiesis. Autologous peripheral blood progenitor cell transplantation overcomes this difficulty and is currently explored as consolidation therapy after successful remission induction with polychemotherapy in an intergroup study of the EORTC and EBMT. Allogeneic bone marrow transplantation remains the treatment of choice for younger MDS patients, offering a good chance of cure if the transplantation is performed at an early stage of disease or if the patient receives the transplant in complete remission after conventional chemotherapy.
Humans↗
Pseudomonas aeruginosa orbital phlegmon in a patient treated for myelodysplastic syndrome with concomitant Sjögren's syndrome.
Pseudomonas aeruginosa orbital infections have been described very rarely in patients with neutropenia after chemotherapy. We report the case of a woman with the unusual association of Sjögren's disease and myelodysplasia, who suffered from a Pseudomonas aeruginosa orbital phlegmon after chemotherapy for her myelodysplastic syndrome. Partial intestinal antibiotic decontamination with ciprofloxacine did not prevent the infection. She was treated successfully with intravenous ceftazidime, netilmicin and granulocyte-colony stimulating factor (G-CSF). The normalization of the granulocyte count seems to play a crucial role for recovery. We present the clinical and radiological findings, discuss the therapy and review the literature concerning ocular infections due to Pseudomonas. Other infections due to this germ in immunocompromised hosts are briefly reviewed.
Aged↗
Factors influencing G-CSF-mediated mobilization of hematopoietic progenitor cells during steady-state hematopoiesis in patients with malignant lymphoma and multiple myeloma.
We retrospectively analyzed factors influencing PBPC mobilization during steady-state hematopoiesis in 52 patients with malignant lymphoma (n=35) or multiple myeloma (n=17) who received 77 cycles of G-CSF (12.5-50 microg G-CSF/kg/day). For 15 of these patients, the first mobilization cycle (12.5 microg G-CSF/kg/day) was followed by a second course with an increased dose of G-CSF (25 or 50 microg/kg/day). Leukapheresis was started on day 4, about 2 h after s.c. G-CSF administration, and repeated on 2-5 consecutive days. CD34+ cells were determined by flow cytometry in each apheresis product and in the peripheral blood prior to G-CSF administration, beginning on day 4. Colony assays were performed on cryopreserved samples prior to autografting. In the 15 patients receiving two mobilization cycles the higher G-CSF dose was associated with higher levels of CD34+ cells, a higher mean yield of CD34+ cells per apheresis (p<0.05), and a higher percentage of successful (>2x10(6) CD34+ cells/kg) collections (p=0.058). Patients with limited previous cytotoxic therapy (n=19, up to six cycles of a standard regimen such as CHOP and/or less than 20% marrow irradiation) who received a daily dose of 12.5 microg G-CSF/kg had higher levels of circulating CD34+ cells, a higher mean yield of CD34+ cells per apheresis (p<0.05), and a higher percentage of successful collections (p<0.05) compared with patients previously treated with more intensive radiochemotherapy (n=15). Ten of 20 patients (50%) who failed during the first cycle were successful during subsequent cycles with escalated doses of G-CSF. Trough levels of circulating CD34+ cells on day 4 were predictive for success or failure to achieve >2x10(6) CD34+ cells/kg, especially in heavily pretreated patients. In conclusion, a daily dose of 12.5 microg G-CSF/kg seems sufficient to mobilize PBPC during steady-state hematopoiesis in the majority of patients who have received limited previous radiochemotherapy. Higher doses of G-CSF, up to 50 microg/kg/day, mobilize more PBPC and should be considered for patients previously treated with intensive radiochemotherapy or those failing to mobilize sufficient numbers of CD34+ cells with lower doses of G-CSF.
Adult↗
[Pneumococcal septicemia in functional asplenia: first manifestation of systemic autoimmune disease?].
A 35 year old female was admitted into the hospital because of rapid onset fever and chills. Streptococcus pneumoniae could be isolated from blood as the responsible pathogen for septicemia. Necroses of fingers and feet occurred. The clinical signs of an overwhelming post-splenectomy infection (OPSI) were evident. High-dose penicillin was administered and the patient recovered. Howell-Jolly-bodies were seen in peripheral blood smears. A spleen within normal size could be demonstrated in CT and sonography. Angiologic findings showed intact splenic arteries and a normal vein, whereas the small splenic vessels were rare. MRT of the spleen using Endorem (Fe), showed only a minimal uptake of the RES. In a scintigram of spleen and liver using 320 MBq Tc-99m nanokoll, the spleen was not visible. Thus, functional asplenia was demonstrated by Howell-Jolly-Bodies and by image methods. An increased antinuclear antibody level and a Sm-antibody lead to the diagnosis of undifferentiated connective tissue disease. As far as we know this is the first case that functional asplenia was the first symptom of a systemic autoimmune disease.
Adult↗
Pleural carcinosis confirmed by adjuvant cytological methods: a case report.
We describe a case of a 43-yr-old woman presenting a progressive pleural effusion. The patient was known to have an acute myeloid leukemia in complete remission after allogenic bone marrow transplantation. Suspicion of pleural carcinosis was raised cytologically and confirmed by immunocytochemistry, DNA-cytometry, and atomic force microscopy. We emphasize the use of these additional methods for the distinction of adenocarcinoma cells in effusions from reactive mesothelial cells.
Acute Disease↗
Increasing incidence of myelodysplastic syndromes: real or fictitious?
Over the past 10-20 years, there has been a growing interest in the myelodysplastic syndromes (MDS). Due to difficulties of diagnosis, classification and case recording, the epidemiological features of MDS are still poorly defined. Recently, a number of cancer registries have published data on the regional occurrence of MDS, suggesting that MDS are much more common than previously thought. The crude incidence of MDS in these studies was 3.5-12.6 per 100,000 population per year. In people over the age of 70 years, incidence rates varied between 15 and 50 per 100,000 per year. Contrary to the assumption of most hematologists, cancer surveys usually failed to demonstrate a rising incidence of MDS. In those studies showing a significant increase in MDS, the rising number of cases was probably due to increased physician awareness and extended use of invasive diagnostic procedures in elderly people. Differences in incidence figures between regional studies may be attributed to several causes, including regional variations in disease incidence, small and ill-defined reference populations, bias due to patient referral patterns, varying intensity of diagnostic procedures and different observation periods. Because of the paucity of clinical symptoms and insignificance of morphological bone marrow changes particularly in early stage MDS, the currently available incidence figures are likely to underestimate the true incidence of MDS. Large-scale epidemiological studies are required for obtaining truly representative statistics on the incidence and prevalence of the MDS. In industrialized countries, a dramatic increase in these disorders can be expected over the next few decades due to the 'greying' of the population.
Adult↗
Problems in the classification of CMML--dysplastic versus proliferative type.
The FAB group proposed to distinguish two subgroups of chronic myelomonocytic leukemia (CMML). Depending on the total leukocyte count, a myelodysplastic type (MDS-CMML) (< or = 13,000 microl(-1)) was separated from a myeloproliferative type (MPD-CMML) (> 13,000 microl(-1)). Based on retrospective analyses of 158 patients with CMML, we compared the presenting clinical and hematological features of both disorders and examined whether the refined classification is important in terms of prognosis. There were 81 patients with MDS-CMML and 77 patients with MPD-CMML. Median age of patients at diagnosis (70 versus 72 years) was not different. The sex ratio showed a preponderance of males in the MPD group (m:f; 2.1:1). Splenomegaly was more common in MPD-CMML (54 versus 30%; P = 0.002). With regard to laboratory findings, patients with MPD-CMML presented with significantly higher LDH values (medians 295 versus 231 U ml(-1); P = 0.008) and higher serum deoxythymidine kinase levels (medians 150 versus 41 U microl(-1); P = 0.0025). Except for white blood cell count (WBC), peripheral blood counts were not different. Median percentage of bone marrow blasts was 9% and cumulative survival rates were similar in both disorders. Two years after diagnosis, actuarial survival for patients with MPD-CMML was 33%, as compared to 50% for patients with MDS-CMML (P = 0.31). The probability of transformation to AML was higher in MDS-CMML (32 versus 17% after 5 years), but this difference also did not reach statistical significance. The survival of patients with MDS-CMML was similar to that of other MDS patients (RAEB) who had corresponding medullary blast counts. Using the Düsseldorf-score, we could define two risk groups within MDS-CMML with a median survial of 12 versus 40 months (P = 0.001). None of the known scoring systems could define risk groups within the MPD-CMML group. In summary, these data suggest that MDS-CMML and MPD-CMML are clinically distinguishing conditions, but the separation provides little prognostic information. Further studies are needed to clarify whether response to therapy is different in MDS-CMML and MPD-CMML.
Adult↗
Patient-controlled versus staff-controlled analgesia with pethidine after allogeneic bone marrow transplantation.
Patients treated by allogeneic bone marrow transplantation (aBMT) suffer prolonged oropharyngeal mucositis pain. The aim of this study was to prospectively compare patient-controlled analgesia (PCA) with an established regimen of staff-controlled analgesia using pethidine (meperidine). Twenty patients undergoing aBMT for haematologic neoplasias or malignant lymphomas randomly received pethidine intravenously either continuously plus supplemental bolus doses on request through the transplant unit staff or by PCA. Pain intensity was assessed by patient self report using a visual analogue scale (VAS) and daily pethidine intake was documented. In addition, the pethidine consumption of 20 aBMT-patients receiving staff-controlled analgesia prior to initiation of the study, but not reporting pain, was compared retrospectively with that of patients receiving the same analgesia regimen under study conditions. PCA significantly diminished both pethidine consumption and pain intensity compared with staff-controlled analgesia. The maximum pethidine intake was 440.1 +/- 111.8 mg/24 h in the patient-controlled and 640.9 +/- 128.9 mg/24 h in the staff-controlled analgesia group (mean +/- 95% CI). Mean pain scores remained under 50% but reached 70% in the staff-controlled analgesia group. Pethidine dosage by staff-controlled analgesia increased under study conditions, suggesting that mere pain-assessment and a 'competing' analgesic method motivated the BMT-unit staff to administer higher pethidine doses. This observation is discussed as a possible Hawthorne effect. Previous studies using morphine demonstrated that PCA diminishes opioid requirement compared to continuous or staff-controlled application in bone marrow recipients. In contrast to these studies, PCA additionally improved pain relief in the present investigation.
Adult↗