Hematology Flashcards
Abciximab, epifibatide, tirofiban
Inhibits GpIIb/IIIa directly
Ristocetin (function)
Activates vWF to bind GpIb.
Failure of aggregantion: vWF D and Bernard- Soulier synd
FFP and prothrombin complex concentrate
Reverse action of warfarin immediately. Can be given with Vit K in cases of severe bleeding
Factor Shortest half life
F (7)
Factor longest Half-life
F (2)
Antithrombin (MOA)
Inhibits thrombin (IIa)
Factors: 7a, 9a,10a,11a,12a
Heparin
Enhances the activity of antithrombin
Principal target of antithrombin
Thrombin (IIa) and factor Xa
tPA
Thrombolytic
Factor V Leiden mutation
Factor V resistant to inhibition by activated protein C
Recurrent pregnancy loss
Iron deficiency
Low iron
High TIBC
Low Ferritin
High RDW
Low RI
Spoon nails (Koilonychia)
Plummer Vinson Synd
Iron deficiency anemia
Esophageal webs
Dysphagia
alpha-thlassemia
a-globin gene deletion on chromosome 16.
Can be cis or trans
A thalasemia
Hemoglobin H disease (- -/ - a) ; excess B globin forms B4
Moderate to severe microcytic hypochromic anemia
A thalasemia
Hemoglobin Barts disease (- -/ - -) ; NO a globin. Excess gamma globin form
Hydrops fetalis; incompatible with life
B THALASEMMIA
Point mutation in splice sites and promoter sequences on chromosome 11. High prevalence in Mediterranean descent.
B thalassemia minor (heterozygote)
B underproduced
Usually asymptomatic
Dx high HbA2 (> 3.5%)
B thalassemia major (homozygote)
B chain absent
Target cells
Anisopoikilocytosis
Marrow expansion (crew cut on skull)
Chipmunk facies
Hepatosplenomegaly
High risk of parvo B19
High HbF and HbA2
Lead poisoning (enzymes inhibition)
Inhibits ferrochelatase and ALA dehydratase
Lead poisoning symptoms
Lead lines on gingivae (burton) and metaphyses
Encephalopathy
Erythrocytes basophilic stippling
Abdominal colic
Sideroblastic anemia
Drops (wrist and foot droop)
Lead poisoning tx
Chelation (succimer)
EDTA
Dimercaprol
Sideroblastic Anemia causes:
X-linked defect ALA synthase
Alcohol
Lead poisoning
Vitamin B6 deficiency
Copper deficiency
Drugs (isoniazid, linezolid)
Sideroblastic anemia findings
High iron
Normal or low TIBC
High ferritin
Ringed sideroblast
Basophilic stippling of RBC
Sideroblastic anemia Tx:
B6 pyridoxine (cofactor of ALA synthase)
Megaloblastic anemia causes:
Vitamin B12 defi
Folate defi
Meds:
- hydroxyurea
- phenytoin
- methotrexate
- sulfa drugs
High homocysteine, NORMAL methylmalonic acid
Folate deficiency
High homocysteine, high methylmalonic acid
Vitamin B12 deficiency
Orotic aciduria cause:
Defect in UMP synthase
Inability to convert OROTIC acid to UMP
Orotic aciduria symptoms
Present in children as:
Failure to thrive
Developmental delay
Megaloblastic anemia refractory to folate and B12
Orotic acid in urine
Hemolysis lead to:
Increased LDH
Low haptoglobin
Reticulocytes
Unconjugated bilirubin
Pigmented gallstones
Urobilinogen in urine
Sickle cell anemia
Point mutation in B-globin gene
Glutamic acid ——> valine
Sickle cell anemia (AR) : electrophoresis
Low HbA
High HbF
High High HbS