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86 Cards in this Set
- Front
- Back
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what hbs have the same amino acid substitution but at different places of the beta gene ? |
Hb C
Hb E Hb O |
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how do Hb C, Hb E, Hb O migrate on basic electrophoresis ?
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the same way
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how do Hb C, Hb E, Hb O migrate on acidic electrophoresis ?
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differently
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what amino acid is change in the Hb C,HbE, Hb O ?
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glutamic acid to lysine
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where is Hb C prevalent ?
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west Africa
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what position is the mutation of the Hb C ?
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sixth position of the beta chain
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HbC variant may only have homozygous states (t/F)
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F both homozygous and heterozygous
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HbCC tend to ... when dehydrated
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crystalize
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what cells are more vulnerable to intracellular crystallization ?
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older RBCs approaching 120 days
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why are older cells more vulnerable than younger ones for intracellular crystallization ?
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since they tend to lose water as they age
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cells containing Hb C are ...
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more rigid
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the increased rigidity of the RBC due Hb C cause the RBC to ...
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get destroyed in the spleen
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Hb CC would cause spleen to ...
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be enlarged and cause pain
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Hb CC cause a wide variety of clinical symptoms T/F
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F no symptoms maybe mild hemolytic anemia
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Hb C is one of the the more ... hemoglobinopathies
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benign
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the Hb value in the Hb CC is
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8-12 g/dl
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the retic level in the Hb CC is
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4-8%
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how does the MCV MCH and MCHC look like in Hb CC ?
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MCV and MCH are normal and MCHC is increased
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why is MCHC increased in Hb CC ?
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due to cellular dehydration
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how does the pb look in HbCC with respect to the anemia ?
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moderate normocytic normochromic anemia
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what cells are present in more than 90% of the pb HbCC ?
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target cells
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what cells are found in 5% of the pb in the HbCC ?
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spherocytes
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what type of crystals maybe seen in Hb CC especially after splenectomy ?
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hexagonal , rod shaped crystals , intracellular with round blunt ends
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lysine is a ... amino acid
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charged
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lysine usually precipitates T/F
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false it does not
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RbCs that contain HbC would undergo what?
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targeting
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what is the most percentage of Hbc in HbCC ?
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90%
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how does the Hb C immigrate ?
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with Hb A2 at alkaline pH
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HbF is ... in some instances of HbCC d
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increased to 7%
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Hb A is present in HbCC disease t/F
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F not at all
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what is the prognosis of HbCC ?
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patients are generally asymptomatic and do not require treatment
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HbAC is has symptoms ?
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no it is symptomless
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unlike HbS carrier state what percentage of cells is targeted ?
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30-40%
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in HbS trait what percentage is targeted ?
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none
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what percentage on electrophoresis is HbA in HbAC ?
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60-70%
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what percentage on electrophoresis is HbC in HbAC ?
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30-40%
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HbD variants travel on electrophoresis at the same rate as what hemoglobin ?
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hb S
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what is the most common HbD ?
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Hb D-punjab or Hb D-LA
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are HbDD and HbDA cases reported ?
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yes
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HbD may be found in combination with what 2 cases ?
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thalassemia or HbS
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in HbD what substitute glutamic acid ?
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glutamine
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at what position is glutamic acid substituted for glutamine in HbD ?
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121st position of the beta chain
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where is Hb-D punjab mostly found ?
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pakistan and north west india
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what is the frequency of HbD in pakistan and northwest india ?
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3%
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are there any clinical abnormalities associated with the heterozygous state of HbD ?
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no
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homozygous HbDD is often associated with ...
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splenomegaly
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what type of anemia is associated with HbDD ?
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mild hemolytic anemia
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RBC parameters are ... in HbDD
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decreased
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RBC indices are ... in HbDD
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normal
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what type of cells are frequently seen on the blood film of HbDD ?
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target cells
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what is the electrophoretic percentage of HbD in HbDD ?
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95%
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what is the percentage of HbF and HbA2 in HbDD ?
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normal
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HbD migrates at the same position like what Hb ?
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S
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how does the peripheral blood look like in a heterozygous case of HbD ?
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normal
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electrophoresis shows what percentage of HbD ?(heterozygous)
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30-40%
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what is the next step after electrophoresis to diagnose HbD ?
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sickling test
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if the sickling test is negative and the electrophoresis shows a bad at the S position then the case is ...
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HbD disease
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on acidic electrophoresis what agar is used ?
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citrate agar
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how does the HbD move on acidic agar ?
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towards the cathode along with HbA and A2
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how does the HbS move on the acidic agar ?
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towards the anode
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what is the most common beta chain hemoglobin variant in the world ?
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HbE
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where is HbE prevalent ?
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Cambodia
Laos Thailand Southeast asian decent |
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in HbE lysine subs glutamic acid at what position ?
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26
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HbEE and HbAE exists T/F
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T
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any of the HbEE or HbAE has clinical symptoms ?
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no maybe splenomegaly in the homozygous state
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where does HbE cause symptoms ?
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if there is another mutation for example for beta thal trait
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Hb-Ethalassemia trait is similar to what thal ?
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thal intermedia
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laboratory findings of HbE are similar to what Hb ?
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HbD
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HbEE causes what type of anemia ?
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mild microcytic normochromic/hyochromic hemolytic anemia
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red cell survival time is slightly ... inHb EE
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decreased
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why is the hemoglobin unstable ?
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due to weakening of bonds between monomers constituting the hemoglobin tetramer
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are target cells present in the HbEE ?
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yes
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HbE may show ...
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elevated rbc count + target cells
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in HbEA the red cell survival time is ...w/o anemia
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normal with no anemia
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HbE migrates closely to ... on basic media ?
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HbC HbA2
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HbE migrates ... than C and A2 on basic media
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slightly faster
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hbE migrates the same as what 2 hbs at acidic media ?
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A and A2
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HbE percentage in homozygous state ?
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92-98%
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HbF in HbEE is ...
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normal to slightly increased
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when the HbA2 is higher than 10% what should we expect and why ?
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one of the Hbs that migrate with HbA2 since HbA2 rarely exceeds 10% even when it is elevated
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HbA2 is elevated more than 10 % in a condition that is in combination with ..,
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beta thal (HbE/B 0)
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what is the clinical case similar to (HbE/ Beta 0)
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Beta thalassemia major
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Hb O is a beta chain variant that changes glutamic acid to lysine at what position ?
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121
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where was Hb O first identified ?
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in an arab boy
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the electrophoretic mobility of HbO arab is similar to ... at alkaline Ph
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HbC
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on citrate agar the Hbo migrates close to ...
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HbA
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