Presentation
The duplicated product had not been observed at presentation in malignant disease, as far as we are aware; thus, the findings are discussed with regard to this case and other possible smoldering leukemias. [ncbi.nlm.nih.gov]
[…] consider Chronic Myelomonocytic Leukemia ) Lack of neutrophilia (if present consider BCR-ABL1+CML or atypical CML) Elevated blasts, but not to leukemic levels in either: Peripheral blood blasts 2-19%, based on 200 leukocyte differential, OR Bone marrow [surgpathcriteria.stanford.edu]
Blasts are commonly present. RAEB-1 is defined by 2-4% blasts in the peripheral blood. RAEB-2 is defined by 5-19% blasts in the peirpheral blood. [imagebank.hematology.org]
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
- Falling
Most people with RAEB will fall into the intermediate-1, intermediate-2 or high risk categories. The RAEB-1 type of MDS transforms to an acute leukaemia in approximately 25% of cases. [leukaemia.org.au]
+25 FRUIT FROM ALL SEASONS ( Winter, Fall, Spring, Summer). Dozens of fresh fruits for you to blast ( banana, apples, pineapple, orange, lemon, papaya, strawberry, watermelon ) and much more. Slice as much as you can, as quickly as you can. [play.google.com]
Myeloid neoplasms with more than 50% bone marrow erythroid cells in which blasts comprise over 20% of the non-erythroid cells, but less than 20% of all cells, are now classified as MDS, with the vast majority of these falling into the MDS-EB category. [emedicine.medscape.com]
Prognosis Median survival of RAEB falls in the 1-2 year range. The best outcome is usually observed in RAEB-1. Chromosomal abnormalities have independent prognostic significance and are to be included in risk assessment at diagnosis. [atlasgeneticsoncology.org]
[…] bone marrow less than 5% 5% to 10% 11% - 20% 21% - 30% 0 0.5 1.5 2.0 Cell DNA changes (cytogenics) Good Intermediate Poor 0 0.5 2.0 Low blood counts or cytopenias 0 or 1 cytopenia 2 or 3 cytopenias 0 0.5 Together, the scores tell which risk group you fall [aamds.org]
- Exposure to Benzene
Exposure to radiation such as in the presence of an atomic bomb or nuclear reactors. Exposure to benzene and chemicals used in the production of rubber and petroleum. [syndromespedia.com]
Better recognised correlations are the late consequences of cytotoxic chemotherapy and ionising radiation with both high and low concentration of exposure to benzene injurious via marrow damage. [cmej.org.za]
Acquired Senescence Mutagen exposure Genotoxic therapy Alkylators Topoisomerase II interactive agents ß-emitters (phosphorus-32) Autologous bone marrow transplantation Environmental or occupational exposure (e.g., benzene) Tobacco [slideshare.net]
[…] to benzene, pesticides, organic solvents, and other cytotoxic agents or environmental carcinogens. [archivesofpathology.org]
- Sepsis
Neutropenic bacterial sepsis, exacerbated by granulocyte dysfunction, responds poorly to standard therapy and remains a major cause of death in 20 - 40% of individuals. [cmej.org.za]
The cause of death was documented in 29 (48%) of the 60 patients who had died: 8 pneumonia, 7 cardiac failure, 4 cardiac arrest, 3 sepsis, 2 metastatic lung cancer and 1 each of metastatic breast cancer, myocardial infarction, stroke and lower gastrointestinal [nature.com]
- Ecchymosis
Symptoms and signs include increased bleeding episodes, bruising (ecchymosis), purpura (several hematoma) and petechial rash (reddish pinpoint rash on the skin) Neutropenia. [syndromespedia.com]
[…] potential risk to a fetus and to avoid pregnancy Adverse Reactions: Most common adverse reactions (>30%) by SC route are: nausea, anemia, thrombocytopenia, vomiting, pyrexia, leukopenia, diarrhea, injection site erythema, constipation, neutropenia and ecchymosis [unicare.com]
- Inflammation
Monocytes aid in response to inflammation What is the function of lymphocytes? The primary function of lymphocytes of the innate immune system is to deliver an immediate response to viral attack. [quizlet.com]
Liver, Gall & Pancreas
- Hepatomegaly
Symptoms and signs include increased risk of infection and sore throat Hepatomegaly or splenomegaly. This is due to rapid hemolysis of the blood component, which is filtered by the spleen and liver. Abnormal characteristics of blood cells. [syndromespedia.com]
Clinical features often include anaemia, hepatomegaly, splenomegaly, lymphadenopathy and rash. 62. Criteria for diagnosis of JMML 63. [slideshare.net]
Skin
- Petechiae
(effective October 01, 2015) D46.21 Refractory anemia with excess of blasts 1 (effective October 01, 2015) D46.22 Refractory anemia with excess of blasts 2 (effective October 01, 2015) Signs and Symptoms Anemia Easy bruising or bleeding Neutropenia Petechiae [seer.cancer.gov]
Clinically: fatigue and bleeding bruising hematuria and petechia (M4) What are the lab findings, clinical symptoms and the predominant cell is in each category. [quizlet.com]
Another sign of a low platelet count is petechiae; these are small red or purple spots under the skin that are caused by bleeding. Infections are due to both low white blood cell counts as well as dysfunction of the white blood cells. [oncolink.org]
• Petechiae on the lower extremities or on the buccal mucosa (if severe thrombocytopenia is present). • Splenomegaly may be present, especially in patients with chronic myelomonocytic leukemia (CMML). 27. [slideshare.net]
Most common adverse reactions by IV route also included petechiae, rigors, weakness and hypokalemia. [unicare.com]
- Erythema
Background: Myelodysplastic syndrome (MDS) is a group of bone marrow disorders associated with dyplasia of myeloid elements that may have cutaneous manifestations including infections, vasculitis, Sweet's syndrome, pyoderma gangrenosum, erythema elevatum [thedoctorsdoctor.com]
[…] females with reproductive potential of the potential risk to a fetus and to avoid pregnancy Adverse Reactions: Most common adverse reactions (>30%) by SC route are: nausea, anemia, thrombocytopenia, vomiting, pyrexia, leukopenia, diarrhea, injection site erythema [unicare.com]
- Urticaria
[…] granulocyte–macrophage colony-forming units (CFU-GM) and that these cells are replaced by polyclonal T cells that do not suppress CFU-GM after ATG treatment.31 The current study is comparable with previous studies in terms of toxicity.26,29 Fever and urticaria [nature.com]
- Purpura
Symptoms and signs include increased bleeding episodes, bruising (ecchymosis), purpura (several hematoma) and petechial rash (reddish pinpoint rash on the skin) Neutropenia. [syndromespedia.com]
Neurologic
- Confusion
[…] by 5-20% myeloblasts in the marrow (RAEB-T) Refractory anemia with excess blasts in transformation characterized by 21-30% myeloblasts in the marrow (>30% blasts is defined as acute myeloid leukemia) (CMML)Chronic myelomonocytic leukemia, not to be confused [quizlet.com]
The blasts in MDS are often small and can be confused with lymphocytes in thickly spread or poorly stained smears. [emedicine.medscape.com]
- Asthenia
The patient’s presenting symptoms were severe odynophagia, muscle pain, asthenia, fever with chills and diaphoresis, and a tender submandibular growth. Upon physical examination, a bilateral submandibular mass was found. [omicsonline.org]
Workup
Serum
- Neutrophilia
The percentage of blasts is scored as follows: Related Criteria Classification of Neutropenia Classification of Neutrophilia Serum Levels That Differentiate Anemia of Chronic Disease from Iron-Deficiency Anemia Diagnostic Criteria for Disseminated Intravascular [medicalcriteria.com]
Neutrophilia- ( bacterial infection, metabolic intoxication,drug intoxication, tissue necrosis) Eosinophilia- (allergies, skin disease, parasites), Basophilia- (Chronic mylogenous leukemia, polycythemia), Monocytes- (chronic infections, convalescence [quizlet.com]
Differential Diagnosis ) Increased blasts make these less likely If chemotherapy or radiation therapy related, should be reported as " therapy-related myeloid neoplasm " 3 /μL monocytes (if present consider Chronic Myelomonocytic Leukemia ) Lack of neutrophilia [surgpathcriteria.stanford.edu]
- Decreased Platelet Count
Some cases of MDS are difficult to classify, as in cases with neutropenia (decreased circulating neutrophils) and thrombocytopenia (decreased platelet count). The incidence of MDS increases with age. [syndromespedia.com]
Laboratory
- Leukocytosis
Myeloproliferative disorder-Marked leukocytosis 50 to > than 300x 10^9/L w/ an increase in mature and immature cells (shift left) of the granulocytic series. Thrombocytosis is common. [quizlet.com]
The laboratory tests revealed normocytic anemia with a hemoglobin concentration of 6.0 g/dl, as well as thrombocytopenia (61×109/l) and leukocytosis (12×109/l) with circulating blasts. [spandidos-publications.com]
In MPN, the presence of JAK2V617F has been associated with older age at diagnosis, higher hemoglobin level, leukocytosis and lower platelet count.21 Obviously, the small sample sizes in the del(5q)-related studies do not allow one to make statistically [nature.com]
Treatment
[…] service, product or treatment in your circumstances. [leukaemia.org.au]
Analyses of EFS From Start of Treatment Treatment regimen was also predictive of outcome. [bloodjournal.org]
Our data suggest that the propriety of administering AML Rx to patients with RAEB or RAEB-t who have poor prognosis without treatment is identical to the propriety of treating AML in this fashion. [ncbi.nlm.nih.gov]
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]
Yi JH, Huh J, Kim HJ et al (2013) Genome-wide single-nucleotide polymorphism array-based karyotyping in myelodysplastic syndrome and chronic myelomonocytic leukemia and its impact on treatment outcomes following decitabine treatment. [link.springer.com]
Prognosis
Our data suggest that the propriety of administering AML Rx to patients with RAEB or RAEB-t who have poor prognosis without treatment is identical to the propriety of treating AML in this fashion. [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]
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]
Prognosis The prognosis of MDS depends on the type. Refractory anemia and RARS have the best prognosis among possible MDS types. Patients with these types of MDS may live up to one more decade, even without transplantation. [syndromespedia.com]
Etiology
(Etiology) Refractory Anemia with Excess Blasts is caused by genetic mutations. [dovemed.com]
•Etiology: Possible chronic antigenic stimulation Abnormal Differentiation •Cell Cycle Maturation arrest. •Altered Proliferation. •Transcription Factors alterations •Impaired maturation •Cytopenias • Progression to leukemia 11. [slideshare.net]
Some patients will respond to immunosuppression (ATG) and/or biological response modifiers (lenalidomide) suggesting an immune-mediated etiology in some individuals. [atlasgeneticsoncology.org]
PATHOBIOLOGY The etiology and pathogenesis of MDSs remain poorly characterized. [archivesofpathology.org]
Epidemiology
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]
View Article : Google Scholar : PubMed/NCBI 5 Sekeres MA: The epidemiology of myelodysplastic syndromes. Hematol Oncol Clin North Am. 24:287–294. 2010. [spandidos-publications.com]
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
Bone marrow fibrosis: pathophysiology and clinical significance of increased bone marrow stromal fibres. Br J Haematol 2007;139:351–62. Web of Science Google Scholar 5. Farolino DL, Rustagi PK, Currie MS, Doeblin TD, Logue GL. [degruyter.com]
The pathophysiology of acquired aplastic anemia. N Engl J Med 1997; 1336: 1365–1372. 31 Molldrem JJ, Jiang YZ, Stetler-Stevenson M, Mavroudis D, Hensel N, Barrett AJ. [nature.com]
Myelodysplastic Syndromes: Review of Pathophysiology and Current Novel Treatment Approaches. Current Cancer Drug Targets 2007, 7:541-558 [slideshare.net]
The frequency of FLT3 mutations among hematologic malignancies suggests their significance in the pathophysiology of hematopoietic progenitor cells. [archivesofpathology.org]
Changes in DNA methylation in neoplasia: pathophysiology and therapeutic implications. Ann Intern Med. 2001; 134 (7): 573-86 http://dx.doi.org/10.7326/0003-4819-134-7-200104030-00011 PMid:11281740 Silverman LR. [mjhid.org]
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]
Rapidly find the answers you need with separate sections on diseases and disorders, differential diagnosis, clinical algorithms, laboratory results, and clinical preventive services, plus an at-a-glance format that uses cross-references, outlines, bullets [books.google.com]
Antibiotics can prevent a simple infection becoming life-threatening. [leukaemia.org.au]