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14 Cards in this Set
- Front
- Back
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what reticulocyte values do you see in aplastic anemia?
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- <1% --> low reticulocytes dictate that this is a problem with production
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where do you perform a bone marrow biopsy/aspirate in an adult?
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- usually PSIS, in obese person can use sternum
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what is the normal percentage of blasts in an aspirate? myeloid:erythroid ratio?
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- blasts: 0-5%
- myeloid:erythroid ratio: 2-4:1 |
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what does the bone marrow biopsy look like in aplastic anemia?
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- not enough cells, way too much fat (>90%)
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what is fanconi anemia? how does it present? what is the pathophys?
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- aplastic anemia: AR, usually FANCA genetics
- presents in kids, abnormal thumbs, cytopenia - cannot respond to dsDNA damage --> end up with weird cross like structures on karyotype --> damages accumulate over time |
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what is shwachman-diamond? how does it present? what is the pathophys?
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- aplastic anemia: AR, SBDS gene mutation (unlike FA all children have this defect)
- exocrine pancreas deficiency, skeletal abnormalities, BM failure - SBDS regulates rRNA metabolism --> damage stem cells b/c do not have rRNA to help make Hb |
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what is dyskeratosis? how does it present? what is the pathophys?
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- aplastic anemia: X-linked, defect in telomerase function
- ectodermal dysplasia, BM failure, cancer predisoposition - telomeres abnormally short b/c of genetic defects --> need stem cell transplant |
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why is chloramphenicol not used in US anymore?
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- used for coughs, but can cause aplastic anemia
- can still get it in other countries |
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how does parvovirus B19 cause aplastic anemia?
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- hurts red cell precursors in bone marrow
- see it in ppl with hemolytic anemia b/c they're dependent in erythropoesis |
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what is paroxysmal nocturnal hemoglobinuria (PNH)? how does it present? what is the pathophys?
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- acquired aplastic anemia: defect in PIG-A gene, cannot anchor CD55 & CD59
- dark urine at night - CD55 &CD59 limit complement activation --> without these cells are susceptible to lysis often at night |
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what are the classifications of aplastic anemia?
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- non-severe: transfusion-dependent for RBCs, not platelets
- severe: hypoplastic BM + 2 of the following = platelets <20,000, corrected reticulocyte count <1%, neutrophil <500 - very severe: same as severe but neutrophils <200 |
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what are two ways you treat aplastic anemia?
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- immune suppression or stem cell replacement by trransplantation
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what is the immune suppression mechanism of treatment for aplastic anemia?
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- anti-thrombocyte globulin (from antibodies generated in animals against T cells)
- cyclosporine A (inhibits calcinurin in T cells) - corticosteroids - mainstay in older pts who are not candidates for transplant |
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what kind of stem cell transplant do you want to give to aplastic anemia?
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- allogenic transplant b/c don't want to do auto b/c they have defective BM cells
- need to avoid alloimmunization by not givind them RBC transfusions |
