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11 Cards in this Set
- Front
- Back
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Myelodysplasias affect which cells?
Clinical picture? Three major causes of myelodysplasia? |
pluripotential stem cell - ineffective hematopoiesis
pancytopenia with hyperplastic marrow alkylating agents in chemo radiation petrochemical exposure |
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What is RARS?
Which refractory anemia has the lowest risk of conversion to AML? What deficiency can cause RARS? What is RAEB? |
RARS - refractory anemia with ringed sideroblasts
RARS pyridoxine deficiency Refractory Anemia with Excess Blasts - between 5 and 20% myeloblasts in marrow |
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Which refractory anemia has the highest rate of conversion to AML?
Marrow myeloblasts between 5-30%, absolute monocyte count >1000 or >10% monocytoid cells in marrow: How do you treat myelodysplasia? |
RAEB-T (RAEB in Transformation)
CMML - Chronic Myelomonocytic Leukemia (NO PH gene) Supportive care, aggressive tx of infections, PRBC's when +sx, watch for iron overload |
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What are two Hematopoietic Growth Factors you can use in tx?
What is the only real opportunity for a cure in myelodysplasia? |
Neupogen, Leukine
BMT (patients are usually elderly, so not normally an option) |
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Polycythemia Vera is a chronic malignancy of what cells?
Clinical presentation of P-Vera? Lab features? |
Stem cells
Facial rubor, hyperviscosity (HA, dizziness, blurred vision), heaviness in arms/legs, PRURITUS with hot shower, bath increased H&H, nucleated RBC's, elevated LAP score, elevated WBC/platelets in 40-60% of patients |
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When do you see increased reticulin in P. Vera?
P. Vera has been linked to what syndrome involving occlusion of the hepatic vein? P. Vera is thought of as an end result for what disease? |
late in disease (spent phase)
Budd-Chiari acute leukemia |
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What are some secondary causes of elevated RBC counts?
Major criteria (A) for PVera diagnosis? Minor criteria (B)? |
hemoconcentration, pulmonary disease (COPD), EPO-producing tumors, hemoglobinopathy with high affinity hemoglobin, high altitude living
Elevated RBC mass, normal arterial SPO2 > 92%, splenomegaly thrombocytosis, high platelets, leukocytosis w/ no fever/infection, elevated LAP, B12 |
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Treatment for PVera?
Triad of myelofibrosis? (LEAP) What is myelofibrosis? |
lowered of RBC mass - scheduled phlebotomy; also hydroxyurea
Leukoerythroblastic anemia, Poikilocytosis, Splenomegaly myeloproliferative disorder characterized by marrow fibrosis and extrameduillary hematopoiesis |
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Pathogenesis of myelofibrosis?
Any treatments? Major COD in myelofibrosis? |
increased reticulin in marrow - due to PDGF, other cytokines in marrow
no therapy available for reversal of fibrosis; give blood cells PRN (transfusions, EPO, platelets) overwhelming infection |
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What is essential thrombocythemia?
Criteria for diagnosis? Clinical features? |
primary marrow disorder - increased platelets in peripheral blood, megakaryocytes in marrow
platelets >600,000, Hgb <13, no response to iron, no Ph chromosome, no fibrosis digital ischemia, erythromelalgia (burning pain w/ redness, increased skin temperature) - improves with ASA |
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Lab features of essential thrombocythemia?
What bleeding disease is associated with essential thrombocythemia? Treatments? |
elevated platelets, normal/elevated WBC, normal/decreased H&H, increased megakaryocytes in marrow, minimal reticulin
vonWillebrand's disease hydroxyurea, busulfan, chlorambucil, melphalan, plateletphoresis |
