A 26‐year‐old female presented with an episode of severe mucus membrane bleeding. Investigations revealed prolonged prothrombin time (PT), and partial thromboplastin time (PTT), normal thrombin time (TT) and reptilase time, thrombocytopenia, a positive test for lupus anticoagulant (LA), as well as anti‐cardiolipin antibodies (ACL). A toxicology screen for toxic drugs and coumadin was negative. Coagulation factor assays revealed low levels for factor II and XII. Low level inhibitor to factor II was demonstrated. Patient had a negative VDRL test and positive anti‐nuclear antibodies (ANA). The diagnosis of acquired hypoprothrombinaemia secondary to circulating inhibitor induced by LA was made, and then the patient was started on prednisone, which led to cessation of the bleeding and normalization of PT and PTT, as well as an increase of factor II and factor XII levels. A few months later, the patient developed arthralgia and alopecia, and antibodies against double‐stranded DNA were detected, and the diagnosis of systemic lupus erythematosis (SLE) was confirmed. The patient continued to have mild prolongation of PT and PTT while on a low dose of prednisone, but she had no bleeding symptoms. A computed tomography scan of the brain was carried out for unexplained central nervous system (CNS) symptoms, and it revealed mild hydrocephalus, which was thought to be part of the CNS manifestations of SLE. It was concluded that patients with SLE may present with haemostatic defects that are a result of either platelet‐related causes (quantitative or qualitative) or coagulation factor deficiency secondary to circulating inhibitor, or both, in the absence of other features of SLE which may appear later.
BACKGROUNDIt has been reported that point mutations of the ras gene occur frequently in acute myeloid leukemia (AML) and myelodysplastic syndrome (MDS). However, the prognostic significance of ras gene mutations in patients with these disorders has been a controversial issue. Although abnormalities in the neurofibromatosis 1 (NF1) gene, which is a gene involved in the ras pathway, have been observed frequently in patients with juvenile chronic myelogenous leukemia, the role of these abnormalities in adult patients with AML or MDS is not clear.METHODSIn this study, bone marrow specimens that were obtained from previously untreated patients with AML and MDS were examined for ras mutations, and the levels of NF1 protein expression were measured.RESULTSRas point mutations were detected in 16 of 83 patients with AML (19%) and in 21 of 90 patients with MDS (23%). Fourteen of 28 patients with chronic myelomonocytic leukemia (50%) had ras gene mutations. Decreased bone marrow levels of NF1 protein (< 70% of the median level detected in control bone marrow specimens) were observed in 20 of 66 patients with AML (30.3%) and in 8 of 29 patients with MDS (27.6%); none of the patients with ras gene mutations had decreased bone marrow levels of NF1 protein. The presence of a mutant ras gene was associated with a shorter complete remission duration (CRD) in patients with MDS (P = 0.05) but had no effect on survival in patients with AML or MDS. Patients with AML who had decreased NF1 protein levels had a slightly longer CRD compared with patients who had normal NF1 levels (P = 0.07). However, the NF1 level had no significant impact on survival among patients with AML or MDS. When patients with low NF1 levels were grouped with patients who had ras mutations, the patients who had AML had a significantly longer CRD compared with the patients who had MDS (P = 0.02).CONCLUSIONSThe current results suggest that a reduction in NF1 protein expression and the presence of ras point mutations may complement each other and that they both play a complex role in the biology of AML and MDS. Cancer 2003;97:441–9. © 2003 American Cancer Society.DOI 10.1002/cncr.11036
Extramedullary myeloid cell tumour (EMMT) localised to the mediastinum is a rare manifestation of acute myeloid leukaemia, forming less than 4% of all cases of EMMT. In contrast to other types of EMMT, cytogenetic characteristics of this rare entity are relatively unknown. This report describes a patient with EMMT who had evidence of superior vena cava syndrome and normal peripheral blood counts at diagnosis. The results from an initial biopsy specimen were consistent with a diagnosis of mediastinal large B cell lymphoma. A diagnosis of acute myeloid leukaemia was made three months after initial diagnosis by bone marrow examination. Review of the initial biopsy specimen showed strong positivity for myeloperoxidase, revealing that the patient had been initially misdiagnosed as having large B cell lymphoma. Cytogenetic studies revealed a near triploid and near tetraploid karyotype with structural abnormalities in 12 and three metaphases, respectively. Review of the literature showed that a near tetraploid or triploid karyotype is found in most of the reported cases of mediastinal EMMT. Thus, the presence of a near triploid/tetraploid karyotype and mediastinal EMMT may represent a specific subset of EMMT. The biological relevance of this observation is discussed.
BACKGROUNDTotal laboratory automation (TLA) is a relatively new way of improving the management of high volume clinical laboratories. TLA may reduce staff, reduce operating costs, decrease testing time and provide enhanced process control.OBJECTIVESEstablish a cost efficient TLA that is less labor intensive, improves productivity and reduces turnaround time (TAT).DESIGNImplementation of TLA for random glucose and troponin-T as sentinel tests to compare change in TAT.SETTINGTertiary hospital with high volume of laboratory tests.METHODSRoutine patient samples for random glucose and troponin-T were used to capture TAT. Information on staff grades and schedules before and after implementing the TLA, and cost of contracts to deliver the service were collected.MAIN OUTCOME MEASURESTAT, cost efficiency, and reduction in labor.RESULTSThe consolidation of contracts resulted in a reduction of 28.8 million SAR in direct costs. Staffing cost was reduced by 1.14 million SAR with less senior staff required; there were reductions in staff at both senior and junior level. The overall TAT for all tests was reduced by 32% in 2016 (after TLA implementation) compared to 2012 (before TLA implementation). The median TAT for random glucose tests was reduced by 21% (to 55.7 minutes in 2016 from 70.1 minutes in 2012). Evidence of test optimization by exploring the impact of stat tests, auto-dilutions and reruns on the overall TAT of the TLA is shown by comparing troponin T TATs after reclassifying stat tests (in 2016) to routine (in 2017). At the 75th percentile, there was a 27% reduction in TAT when comparing August 2016 to March 2017 with a 19% reduction in median TAT.CONCLUSIONBy moving from stat to routine assays, the TAT was reduced, which is counter-intuitive. The use of stat assays slowed down the performance of the TLA. A careful review of the mix of assays should be conducted to maximize performance and to ensure that the system delivers what is required.LIMITATIONSRoom for improvement by systematically analyzing and reviewing the impact of making minor changes that could have significant impact on TAT.
We find that thrombosis, venous or arterial, and obstetric complications are the most frequent clinical findings in our patients with circulating LAC. Incidental APS is not an uncommon finding in patients screened for APS. There is a clear association between the presence of LAC and an abnormal aPTT, which is much less obvious with the PT.
4092 Background: The adhesion molecule CD11b, which associates with the beta2-integrin to form the Mac-1 complex, is expressed in monocytic leukemias as well as other myeloid leukemias. Its expression on the lekemic cells has been reported to correlate with more aggressive course in adult patients with AML. The clinical relevance of CD11b expression in pediatric AML is not known. More importantly, the clinical relevance of CD11b expression on pediatric AML when these patients are treated with allogeneic hematopoietic stem cell transplant (HSCT). We investigated whether HSCT can modify the expected adverse effects of CD11b expression in pediatric AML patients. Methods: Immunophenotype data of 62 pediatric patients with AML, all treated with allogeneic HSCT, was reviewed. The expression of the CD11b on the blast population as determined by flow cytometry was correlated with clinical data and outcome. The median age of patients was 8 years (range: 8 months–14 years) and included 47 (76%) patients transplanted in their first complete remission (CR1). Fourty six (74%) of all patients had intermediate-risk cytogenetics and the remaining (26%) had adverse-risk cytogenetic abnormalities. Results: Twenty five (40%) of all studied patients expressed CD11b on the surface of the blasts at diagnosis. Patients with CD11b expression had significantly shorter overall survival (P=0.038) with median survival of approximately 11 months while the median survival in the CD11b negative patients has not been reached. Similarly, CD11 positive patients had significantly shorter event free survival (EFS) (P=0.03). When we considered only patients treated with HSCT in CR1, the OS and EFS for the CD11b positive patients were both significantly shorter (P=0.04 and P=0.05, respectively). Multivariate analysis including CD11b expression and cytogenetic risk identified CD11b expression as an independent prognostic factor for survival and EFS, while cytogenetic-risk stratification became no longer a predictor. Conclusion: Our data suggests that the expression of CD11b in pediatric AML is a significant independent poor prognostic factor and HSCT does not change this trend. Most of the CD11b positive patients die in their first year after HSCT and the management of these patients should be modified to address the biological basis of this subset of AML that expresses CD11b adhesion molecule. Disclosures: No relevant conflicts of interest to declare.
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