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Refractory Anemia with Excess Blasts

RAEB-2


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]
  • 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]
  • 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]
Fatigue
  • 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]
  • 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]
  • Symptoms include fatigue, anemia, bleeding, and bruising. The mainstay of treatment is supportive care.[ugr.es]
  • Common symptoms may include: fatigue, shortness of breath, easy bleeding and bruising, frequent and recurring infection. The majority of patients are 60 or older however, MDS can be seen in younger populations.[nebraskamed.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 shown[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]
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]
Pneumonia
  • After 17 months of a stable disease state, the patient developed pneumonia, progression of cytopenia, and reduced cellularity and blast mass in the bone marrow. He was given G-CSF to overcome the pneumonia.[ncbi.nlm.nih.gov]
  • This can cause serious problems, such as pneumonia and other lung problems. aspiration listen (AS-pih-RAY-shun) Removal of fluid or tissue through a needle.[cancer.gov]
Diarrhea
  • […] transfusions, median 3 (0–12) 4 (1–19) 0.18 Documented infections 12 (24%) 9 (24%) 0.86 FUO 33 (67%) 26 (68%) 0.90 2 WHO extrahematological toxicity 6 a (12%) 5 a (13%) 0.84 Median days of hospitalization 26 (12–44) 25 (6–65) 0.21 a Gastrointestinal: grade 3 diarrhea[doi.org]
  • Symptoms include weight loss, loss of appetite, nausea and vomiting, diarrhea, muscle weakness, fatigue, low blood sugar, low blood pressure, and patchy or dark skin.[cancer.gov]
Palpitations
  • 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, dyspnea, palpitations[basicmedicalkey.com]
Hepatosplenomegaly
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]
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]
  • Certain types of Aspergillus may cause disease, especially in people who have suppressed immune systems. aspirate Aspirate (pronounced AS-pih-rit) refers to fluid, tissue, or other substance that is withdrawn from a body cavity, cyst, or tumor.[cancer.gov]
Headache
  • 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, dyspnea, palpitations, headache[basicmedicalkey.com]
  • It can cause headaches, nausea, vomiting, dizziness, confusion, loss of consciousness, and other health problems. acetyl group listen (A-seh-til groop) A small molecule made of two carbon, three hydrogen, and one oxygen atoms.[cancer.gov]

Workup

Normocytic Anemia
  • Diagnosis Comment: There is leukocytosis and bicytopenia (normocytic anemia and thrombocytopenia). Dysplasia is present in all 3 hematopoietic cell lines and blasts at 5%. In addition, reticulin staining is at grade 2.[gpec.ubc.ca]

Treatment

  • Analyses of EFS From Start of Treatment Treatment regimen was also predictive of outcome.[bloodjournal.org]
  • Finally, main drawbacks from single-center studies are either the small sample size or intrastudy treatment variations.[doi.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]
  • Censoring at allogeneic hematopoietic stem cell transplantation, the OS difference between the treatment groups increased, particularly among patients aged 60-74 years (HR 0.48, 95 % CI 0.26-0.89).[ncbi.nlm.nih.gov]

Prognosis

  • 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]
  • Abstract Prednimustine, an ester of chlorambucil and prednisolone, was evaluated for efficacy and toxicity in a selected group of leukemia patients with a poor prognosis.[ncbi.nlm.nih.gov]
  • (Type 2 RAEB), the prognosis is around 10 months survival Proper care should be taken to treat and control complications from MDS.[dovemed.com]

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]

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. Last Update September 28, 2016. Accessed February 1, 2017.[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]
Sex distribution
Age distribution

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

  • 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]
  • Aloxi listen (uh-LOK-see) A drug used to prevent nausea and vomiting caused by chemotherapy. It is also used to prevent nausea and vomiting after surgery.[cancer.gov]
  • 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]
  • Bone Marrow Transplantation in Treating Patients With Leukemia, Myelodysplasia, or Lymphoblastic Lymphoma Completed NCT00003187 Phase 2, Phase 3 cyclophosphamide;cyclosporine;cytarabine;methotrexate;methylprednisolone 14 Treatment of Bone Marrow to Prevent[malacards.org]

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