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35 Cards in this Set
- Front
- Back
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if a person inherits 2 different abnormal genes from both parents then he is called a ... |
double heterozygous
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inheritance of 1beta s and 1 beta c results in
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hb sc disease
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what does Hb Sc patients have
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mild hemolytic anemia with moderate enlargement of the spleen
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what may people with sc disease develop that is similar to the s disease complication ?
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vaso-occulsive blocking but with less severity
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what cells reflect the HbC defect ?
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target
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what cells reflect the HbS defect ?
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sickle
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Hbsc cells have a special look characteristic which is ?
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finger like projections that usually protrude outside the cell
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HbS - alpha is ... common and is clinically ...
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usually
insignificant |
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HbS-Beta zero there is ... production of HbA
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no
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the HbS- beta plus has 2 types what are they?
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clinically severe or asymptomatic
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how is the sickle cells in patients with HbS - beta zero
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irreversibly sickled
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HbS -beta zero has moe chances of ... |
vas occlusive problems and more severe anemia
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it is Easy to distinguish between sickle cell disease and the HbS beta zero T/F
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F difficult
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will HbS-beta zero have more sickling or less than a HbSS ?
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less since he has half the genes of S and with no production of HbA there is HbF to cover for it hence making the case milder
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will HbS-beta zero be more thalassemic than a homozygous thal ?
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less due to less alpha chains
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what is the most common sickling disease variant in the mediterranean pop ?
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HbS-beta
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in severe HbS-beta zero what is the Hb lab level ?
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5-10 g/dl
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what is the reticulocyte count in the HbS-beta zero ?
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10-20%
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how does the RBCs look in HbS-beta zero ?
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microcytic
hypochromic marke anisocytosis poikilocytosis basophilic stippling target cells sickle cells |
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Mcv Mch and Mchc are ... in HbS-Beta zero
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decreased
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what 2 cells are seen in the pb of the hbs - beta zero ? |
target and sickle cells
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when we have a hypo chromic microcytic RBC can it be a pure sickle cell ?
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no because SCD has usually normocytic normochromic
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whats the difference between HbSS and HbS-beta zero when it comes to HbA2
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in HBSS the A2 is not elevated while in the heterozygous case it is
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measurement of what globin chains in the reticulocytes might be a helpful diagnostic tool ?
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alpha and beta
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HbS-beta zero has a B/alpha ratio of
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0.5/1
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HbSS has a B/a ratio of
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1/1
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what is the marked anisocytosis reflected by ?
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high rdw
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Hb-S-B plus have a less or more severe changes ?
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less
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S levels in s-beta plus are
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55-75%
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A levels in S-Beta plus are
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15-30%
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A2 levels and F are ... in S-beta plus
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characteristically elevated
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what HbD when combined with S would cause a clinical abnormality ?
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HbD- La
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what HbD when combined with S would cause a clinical abnormality ? |
sickling crisis and moderate hemolytic anemia
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what is the definitive test for separation and verificatio of the HbS and D ? |
citrate agar electrophoresis |
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HbE - Beta have an anemia that vary in ... |
severity (mild to severe) |
