Presentation
Although the patient presented with highly complex chromosomal abnormalities, his initial clinical presentation was that of typical refractory anemia with excess blasts. Shortly after diagnosis (7 months), the patient developed acute leukemia. [ncbi.nlm.nih.gov]
The number of megakaryocytes is also diminished and there are prominent micro-megakaryocytes present throughout. Iron stores are present and no ringed sideroblasts are seen. The bone marrow is >90% cellular. [gpec.ubc.ca]
[…] simulators (Increased blasts make these less likely) Other myelodysplastic disorders Refractory Anemia with Excess Blasts Acute Myelogenous Leukemia Blasts Blasts frequently ≥20% in peripheral blood and/or bone marrow AML defining translocations not present [surgpathcriteria.stanford.edu]
Anderson presentation. The presentation to treatment interval was greater than 2 months in 1%, 3%, and 4% of the AML, RAEB-t, and RAEB patients, respectively. [bloodjournal.org]
Entire Body System
- Fatigue
Symptoms include fatigue, anemia, bleeding, and bruising. The mainstay of treatment is supportive care. [ugr.es]
The most frequently encountered toxicities were cytopenias, fatigue, hyperglycemia, hypokalemia, hypophosphatemia, and QT prolongation. Dose-limiting toxicities (DLT) were cardiac arrhythmia-atrial fibrillation and QT prolongation. [ncbi.nlm.nih.gov]
Epidemiology Incidence 35-40% of MDS 15-20% RAEB-1 20% RAEB-2 Associated reticulin fibrosis seen in ˜ 10% of MDS; most are RAEB-F (fibrosis) Age Older individuals, primarily > 50 years of age Presentation Often seek medical attention for symptoms of anemia Fatigue [basicmedicalkey.com]
Symptoms are referable to the specific cell line most affected and may include fatigue, weakness, pallor (secondary to anemia), increased infections and fever (secondary to neutropenia), and increased bleeding and bruising (secondary to thrombocytopenia [merckmanuals.com]
- Fever of Unknown Origin
Overall, induction deaths were due to infectious episodes occurring during the aplastic phase ( n = 14) and cerebral hemorrhage ( n = 2); the incidence of fever of unknown origin as well as of documented infection was similar between the two groups, as [doi.org]
- Candidiasis
Intravenous fluconazole was used for proven candidiasis infection, while amphotericin B was given for aspergillosis or other invasive fungal infections when suspected (fever persisting for more than 7 days while on treatment with broad-spectrum antibiotics [doi.org]
- Gangrene
Reasons for not autografting the six mobilizing patients were early relapse ( n = 2), which occurred in both cases after 2 months from CR achievement, refusal ( n = 1), infection ( n = 2) and severe uncontrolled diabetes resulting in ischemic left foot gangrene [doi.org]
Musculoskeletal
- Short Arm
The 5q- associated with RAEB and other preleukemic syndromes was present in 100% of the cells; however, 60% of the cells had a highly unusual derivative chromosome involving the short arm of chromosome 1 and the long arm of chromosome 5, i.e., t(1;5) [ncbi.nlm.nih.gov]
Psychiatrical
- Suggestibility
It is suggested that imbalance in immunoregulatory T cells, which has a crucial role in the control of hemopoiesis, may be a primary event in the pathogenesis of myelodysplastic syndrome which eventually develops into an overt leukemia. [mdanderson.influuent.utsystem.edu]
This result suggests that t(6;9) represents one of the pathways from MDS to leukemia in patients with ANLL. [ncbi.nlm.nih.gov]
- Withdrawn
Myelomonocytic Leukemia de Novo Myelodysplastic Syndromes Previously Treated Myelodysplastic Syndrome Refractory Anemia Any 17 and under 18 - 34 35 - 50 51 - 69 70+ Available Active, not recruiting Not yet recruiting Recruiting Completed Terminated Suspended Withdrawn [patientwing-dev.vitaltrax.net]
Neurologic
- Headache
20% RAEB-1 20% RAEB-2 Associated reticulin fibrosis seen in ˜ 10% of MDS; most are RAEB-F (fibrosis) Age Older individuals, primarily > 50 years of age Presentation Often seek medical attention for symptoms of anemia Fatigue, dyspnea, palpitations, headache [basicmedicalkey.com]
Workup
Serum
- Auer Rods Present
rod present in blast, 6 - 8% myeloblasts, neutrophils show nuclear hypolobulation RAEB-2: 47 year old woman with 12% myeloblasts and occasional Auer rods: Normocellular biopsy Normal erythroid precursors but shift to immature granulocytes Same patient [pathologyoutlines.com]
Treatment
Analyses of EFS From Start of Treatment Treatment regimen was also predictive of outcome. [bloodjournal.org]
S Food and Drug Administration (FDA) on May 2, 2006 for the treatment of patients with myelodysplastic syndromes (MDS) including previously treated and untreated, de novo and secondary MDS of all French-American-British (FAB) subtypes (refractory anemia [medical-dictionary.thefreedictionary.com]
Finally, main drawbacks from single-center studies are either the small sample size or intrastudy treatment variations. [doi.org]
ATTR), ALN-AT3 for the treatment of hemophilia, ALN-PCS for the treatment of severe hypercholesterolemia, ALN-HPN for the treatment of refractory anemia, and ALN-TMP for the treatment of hemoglobinopathies. [freethesaurus.com]
Prognosis
(Type 2 RAEB), the prognosis is around 10 months survival Proper care should be taken to treat and control complications from MDS. [dovemed.com]
We analyzed these subtypes of RAEB-T in terms of hematological characteristics, karyotype anomalies, and prognosis. [ncbi.nlm.nih.gov]
Patients with other abnormalities were considered as better prognosis if they did not have an antecedent hematologic disorder (AHD) and as worse prognosis if they had an AHD. [bloodjournal.org]
Etiology
Certain conditions have both an underlying etiology and multiple body system manifestations due to the underlying etiology. [icd10coded.com]
[…] increased blasts or Auer rods 2 categories based on prognosis RAEB-1 2-4% blasts in peripheral blood or 5-9% blasts in bone marrow RAEB-2 5-19% blasts in peripheral blood or 10-19% blasts in bone marrow Auer rods, if present, advance MDS case to RAEB-2 ETIOLOGY [basicmedicalkey.com]
(Etiology) Refractory Anemia with Excess Blasts is caused by genetic mutations. [dovemed.com]
[…] neoplasms; absolute monocytosis (> 1000/mcL [> 1/L]) in blood; significant increase in marrow monocyte precursors Chronic neutrophilic leukemia: Characterized by neutrophilia and absence of the Philadelphia chromosome and the BCR-ABL1 fusion gene The etiology [merckmanuals.com]
Epidemiology
[…] blasts in bone marrow RAEB-2 5-19% blasts in peripheral blood or 10-19% blasts in bone marrow Auer rods, if present, advance MDS case to RAEB-2 ETIOLOGY/PATHOGENESIS Hematopoietic Stem Cell Disorder Clonal hematopoietic stem cell disorder CLINICAL ISSUES Epidemiology [basicmedicalkey.com]
Epidemiology There are few data on the epidemiology of RAEB, which may account for 20-30% of all MDS cases. MDS is predominantly diagnosed in the elderly population. [atlasgeneticsoncology.org]
Thrombocytopenia Weakness or feeling tired Diagnostic Exams FISH (flourescence in situ hybridzation) Flow cytometry Immunocytochemistry Immunophenotyping Peripheral blood smear Progression and Transformation ~25% of RAEB-1 and 33% of RAEB-2 transform to AML Epidemiology [seer.cancer.gov]
Age-related incidence and other epidemiological aspects of myelodysplastic syndromes. Br J Haematol. 1992;82:358-67. INTERNET Besa EC, Woermann UJ. Myelodysplastic Syndrome. Medscape. [rarediseases.org]
Epidemiology and Clinical Features Epidemiology As with other types of myelodysplastic syndromes (MDS), MDS with excess blasts-1 (MDS-EB1) and MDS-EB2 tend to occur in older adults with a male predominance. [emedicine.medscape.com]
Pathophysiology
When patients have neutropenia or thrombocytopenia without excess blasts in the marrow, it is possible that the pathophysiology could be T-cell mediated in patients with MDS that do not have excessive blasts. [businessandleadership.com]
It will nevertheless, be necessary to compare our results to other MDS subgroups to determine whether the decrease of the RBP4 plasma level is related to MDS pathophysiology specific for RAEB-1, or if it is a result of inflammation, as the RBP4 is a negative [link.springer.com]
Prevention
Prevention Although there is a big genetic predisposition to MDS, some risk factors can be avoided to help prevent the disease. These include: Avoiding too much radiation exposure. [syndromespedia.com]
Collaborative overview of randomised trials of antiplatelet therapy, I: Prevention of death, myocardial infarction, and stroke by prolonged antiplatelet therapy in various categories of patients. [books.google.com]
Read now World Cancer Day 2019 This year we are joining the mission by collating some of the high impact research published in Oxford University Press journals which highlight prevention and risk factors of different cancers. [annonc.oxfordjournals.org]
Antibiotics can prevent a simple infection becoming life-threatening. [leukaemia.org.au]