ch. 10 hematology
Terms
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- microspherocytes
-
-small spherical RBCs
-high volume-to-surface ratio
-less deformable
-trapped & destroyed in spleen -
-growth failure
-splenomegaly
-chronic transfusions - hereditary spherocytosis
- hereditary spherocytosis: Tx
-
1.folic acid supplementation
2.splenectomy (after age 6) - when to do splenectomy in hereditary spherocytosis pts?
- after age 6 (due to sepsis risk w/encapsulated bugs)
- hereditary spherocytosis: dx confirmation
- (+) osmotic fragility test
- sickling phenomenon: exacerbated by...
-
1.hypoxia
2.acidosis
3.temperature changes
4.dehydration - T/F: sickling shortens RBC survival time --> chronic hemolytic anemia
- true
- sickle cell disease presents clinically at age x
- 4 months (HbF decreases, HbS increases)
- common manifestations of anemia in sickle cell pts
-
1.pallor
2.jaundice
3.splenomegaly
4.systolic ejection murmur
5.delayed sexual development & growth - T/F: splenic sequestration can lead to hypovolemic shock
- true
- autoinfarction of spleen
-
1.microvascular obstruction
2.infarction
3.fiborsis - sickle cell disease pts w/fever - must be wary of...?
- invasive bacterial infection
- prophylaxis for sickle cell disease pts
- penicillin starting at age 4 months until age 5
- sickle cell disease: required vaccinations
-
1.HIB (2,4,6, 12-15 mos)
2.Prevnar (2,4,6, 12-15 mos)
3.PPV (age 2, 4-6 yrs) - sickle cell pt w/ams + seizure + focal paralysis. dx?
- stroke (large cerebral vessel occlusion)
- priapism in sickle cell pts occurs at ages x
- age 6-20
- sickle cell pts' hearts
- enlarged hypertrophic --> CHF
- sickle cell pts' GI
- microvascular obstruction of intestines -> ileus -> rebound tenderness
- purpose of hydroxyurea in sickle cell pts
- 1.reduce # & severity of vaso-occlusive crises
-
dactylitis:
what is it?
what age group? -
-hand-foot syndrome
-symmetrical, painful swelling of dorsal surface of hands & feet
-etiology:avascular necrosis of metacarpal & metatarsal bones
-age group: 4-6 mos - painful crises in sickle cell: Tx
-
NSAIDS
narcotics - X made in liver, dependent on vitamin K
-
1.coagulation factors 2,7,9,10
2.antithrombotic factors C,S - causes of vitamin K deficiency
-
1.malabsorption (CF)
2.abx
3.meds (coumadin, phenobarbital, phenytoin) - most common disorder from vitamin K deficiency
- hemorrhagic disease of the newborn
- prophylaxis for hemorrhagic disease of the newborn
- vitamin K 1mg IM
- generalized ecchymosis + GI hemorrhage + bleeding fr/circumcision site & umbilical stump
-
hemorrhagic disease of newborn
(vitamin K deficiency) - hemorrhagic disease of newborn: Tx
-
1.FFP
2.prothrombin complex concentrate - hemorrhagic disease of newborn: confused w/...
-
1.liver disease
2.DIC - physiologic anemia of infancy: occurs at age x
-
2-3 mos (full-term)
6 wks (premie) - T/F: ESR elevated in anemia of chronic disease
- true
- hypochromic microcytic anemias: most common causes
-
1.iron deficiency
2.thalassemias
3.anemia of chronic disease - enhances nonheme iron absorption
- ascorbic acid (vit. C)
- adjusted reticulocyte count (ARC)
- measured hct / expected hct x retic count
- ARC < 2
- ineffective erythropoiesis
- ARC > 2
- effective erythropoiesis (i.e. hemolysis, chronic blood loss)
- pica: seen in...
-
1.lead poisoning
2.iron deficiency anemia - iron deficiency in premies seen as early as age x
- 3 months
- baby w/severe anemia (hb < 3): symptoms
-
1.CHF
2.tachy
3.S3
4.cardiomegaly
5.hepatomegaly - iron deficiency anemia most common at ages x
- 6 mos - 2 years
- mild/moderate anemia: tx
- 3-6 mg/kg/day elemental iron
- iron deficiency tx results expected in...
- 1 month
- most common RBC enzyme defect
- G6PD deficiency
- Heinz bodies = ?
- globin precipitant seen in G6PD deficiency
- G6PD: etiologies
-
1.sulfonamides
2.nitrofurantoin
3.primaquine
4.dimercaprol
5.fava beans
6.infection - protects RBCs from oxidative stress
- reduced glutathione
- G6PD deficiency results in...
- decreased NADPH
- G6PD: Tx
-
1.avoidance
2.supportive
3.IVF
4.pRBCs transfusion
5.urine alkalinization -
bite/blister cells:
seen in...
occur b/c... - G6PD deficiency: macrophages take bite out of cells' Heinz bodies