Fun facts about AA
Diagnostic Criteria / Differential diagnosis for AA
Treatment and response criteria for AA
Fun facts about MDS
More MDS fun facts
100
10-25 years of age and > 60 years of age
What is the biphasic age distribution for peak incidence of aplastic anemia
100
Granulocytes < 500/mm3 Platelets < 20,000/mm3 Reticulocytes < 1% in the presence of anemia
What is at least 2 out of 3 needed in peripheral blood to meet diagnostic criteria for severe aplastic anemia
100
Atgam 20mg/kg/dose IV daily x 8 days
What is the dose and frequency of Atgam used for severe AA?
100
Stable cytopenia (~ 6 months), dysplasia (> 10% in 1 or more of 3 major bone marrow lineages), blasts 5-19%
What is naming 3 of the diagnostic criteria for MDS? Also exclusion of other potential disorders for dysplasia/cytopenia, 1 of 3 decisive criteria (dysplasia, blasts or specific MDS karyotype del (5q) or (20q), +8 or -7/del(7q)
100
In the general population 5 per 100,000 people per year
What is the incidence of MDS? < 40 yo 0.2 per 100,000, > 70 is 26 per 100,000, > 80 is 48 per 100,000
200
Symptoms of anemia and skin or mucosal hemorrhage or visual disurbances.
What is the most common presentation of patients with aplastic anemia?
200
Bone Marrow hypoplastic with < 25% cellularity or moderately hypoplastic (25-50% ceullarity with < 30% of remaining cells being hematopoetic
What is Bone marrow biopsy criteria for diagnosing severe aplastic anemia?
200
5mg/kg po divided bid on days 1-180
What is the dose, frequency and duration of cyclosporine used for severe AA?
200
CMML-1, CMML-2, atypical CML, JMML
What is subtypes of the MDS/MPN (myeloproliferative neoplasms) type?
200
serum Epo levels < 500 mU/ml
What is in patients with MDS, what serum epo level is associated with a higher response rate in symptomatic patients? (lower epo levels and lower pretreatment RBC transfusion requirement)
300
Benzene, organophosphates, recreational drugs (i.e. MDMA, methamphetamine)
What is occupational or environmental exposures that are potential etiologic agents in aplastic anemia
300
Neutrophils < 200/mm3
What is how the diagnostic criteria differ for very severe AA from severe AA?
300
Return of a normal hematocrit, granulocyte count > 1500 and platelet count > 150,000
What is a considered a complete response after immunosuppressive treatment for severe AA?
300
Cases that present with features consistent with MDS and platelet counts > 450,000/m3
What is platelet count needed for classification of RARS-T?
300
Bone marrow aspiration with Prussian blue stain
What is needed in the initial evaluation for MDS to evaluate presence or absence of ringed sideroblasts (and presence of iron)? Ringed sideroblasts are erythroblasts with iron-loaded mitochondria visualized by Prussian blue staining. ringed sideroblasts found exclusively in pathologic conditions
400
Cotrimoxazone, phenytoin, carbamazepine, phenothiazines
What is drugs which have been reported as having a rare association with aplastic anemia?
400
Heritable bone marrow failure syndromes such as dyskeratosis congenital, Fanconi's anemia and Shwachman-Diamond syndrome
What is differential diagnosis to rule out acquired aplastic anemia?
400
Weekly taper of 5% of medication
What is the recommended taper schedule for cyclosporine after day 180 for severe AA?
400
chromosomal anomalies t (8:21) or inv 16
What is name 2 chromosomal anomalies where patients are considered to have AML regardless of blast count?
400
Scoring system to be used for initial prognostic and planning purposes.
What is IPSS (International Prognostic Scoring System)? WPSS permits dynamic estimation of prognosis at multiple time points during the course of MDS.
500
Fanconi's Anemia
What is the most common type of congenital aplastic anemia - Autosomal recessive in most cases, findings of short stature, café au lai spots and skeletal anomalies. Most commonly presents between 3-14 years, but occasionally presents in 30's
500
Macrocytosis
What is the MCV at presentation for most patients diagnosis with aplastic anemia?
500
Serum Ferritin > 100
What is when iron chelation therapy should be considered in AA?
500
Low, intermediate-1 (INT-1), intermediate-2 (int-2) and high
What is 4 distinctive risk groups in terms of survival and AML evolution according to the IPSS for MDS?
500
Marrow blast percentage, number of cytopenias and cytogenetic subgroup (good, intermediate, poor)
What are the significant independent variables for determining outcome for both survival and AML evolution?
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