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B1 Anemia Stuff
Question | Answer |
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Explain the mnemonic CADET face Right. | CO2, Acid/Altitude, DPG, Exercise, Temperature all lower Hb O2 affinity causing a R shift. |
2,3-BPG __ the oxygen affinity of Hb by binding to the __ form of Hb (stabilizing the __ conformation). | DECREASES, DEOXY, TAUT |
HbF has __ affinity for O2 than adult Hb b/c it only __ binds 2,3-BPG. This is so the RBCs can __ O2 as they go through the placenta & not be influenced by small [change]. | MORE, LOOSELY, HANG ON TO |
Elevated 2,3-DPG levels in __ lower O2 affinity for Hb, permitting greater __ in capillaries of the tissues | TISSUES, UNLOADING OF O2 |
The __ affinity of Hb for O2 when bound to 2,3 DPG allows Hb to __ O2 efficiently at the __ found in the tissues. | REDUCED, RELEASE, PARTIAL PRESSURES |
W/o sufficient levels of 2,3 DPG Hb can act as an __. HbF binds __ to 2,3 DPG so that it can __ O2 until it gets to the fetus | O2 TRAP, ONLY WEAKLY, HANG ON TO |
Small amount of CO2 is transported in the blood as __. It binds to the __ chain of Hb & stabilizes the __ form which __ affinity for oxygen. | CARBAMATE, ALPHA, T, DECREASES |
CO binds to one of the __ and __ Hb affinity for O2 (__). This changes the shape of the __ so it looks more like __. | HEME GROUPS, INCREASES, (TRAPS IT), DISSOCIATION CURVE, MYOGLOBIN |
Heme-Heme Interactions are seen in the __ shape of the dissociation curve. Refers to the effects one heme group can have on another. Last O2 binds with about __ the affinity than the first. | SIGMOIDAL, 300x |
__ binding allows more O2 delivery in tissues. Very small changes in pO2 can greatly affect __. High pO2 in lungs facilitates __ of oxygen. Low pO2 in peripheral tissues facilitates __. These interaxns allow for O2 to load & unload at right time & place. | COOPERATIVE, SATURATION, LOADING |
O2 Dissoc. Curve: plot of __ (horizontal) vs __ (vertical). The __ shape shows that subunits __ in O2 binding. O2 binding at 1 subunit __ affinity at others. It's hard for the __ O2 molec to bind, but __ after that. R shift = __ O2sat. L shift = __ O2sat. | pO2, % SATURATION, SIGMOIDAL, COOPERATE, INCREASES, FIRST, EASIER, DECREASED, INCREASED |
Anything causing decreased __ can lead to Sickle Cell exacerbation, which means anything causing a __ shift. This includes: __, __, __, __, __ (Mnemonic: SCA exacerbation makes the pt DEBIL). | pO2, RIGHT, DEHYDRATION, EXCESSIVE EXERCISE, BLOOD LOSS, INFECTION, LOW O2 (or hypoventilation) |
Palliative care for SCA exacerbation: __ thru nasal cannula (“__”), replenish __ & __, & increase __. | O2, “HYPERVENTILATION”, FLUIDS, RBCs, HbF |
What effect does 2,3-DPG have on a SCA pt? | Decreases O2 affinity -> sickling. |
The Hb S mutation causes the substitution of a __ for a __ residue, which forms a __ on the __ that fits into a complementary site on the __ of another Hb molecule w/in the same RBC. | NONPOLAR VALINE, CHARGED GLUTAMATE, PROTRUSION, BETA-GLOBIN, ALPHA-CHAIN |
At low pO2, HbS __ inside RBCs, 1st forming a __, then subsequently assembling into a network of __ that __ & __ the cell, producing rigid misshapen __. | POLYMERIZES, GEL, FIBROUS POLYMERS, STIFFEN, DISTORT, RBCs |
Sickled cells frequently __ the flow of blood in __ by sticking to __ & the __ – this initiates __, bringing in even more __. This interruption in the supply of O¬2 leads to __ in tissue, causing __, & eventually death (__) of cells in the vicinity. | BLOCK, NARROW CAPILLARIES, THEMSELVES, ENDOTHELIUM, INFLAMMATORY RESPONSE, CELLS, LOCALIZED ANOXIA, PAIN, INFARCTION |
__ is the gold standard for increasing HbF. Other things that help but haven’t been widely studied inc: __, __, __, __. | HYDROXYUREA, EPO, NO, FOLATE, BUTYRATE |
The study of the deformation & flow of matter. | RHEOLOGY |
Hydroxyurea 1st used to __. As a myelosuppressive agent, it's possible that __ improve blood rheology & => reduced __. Also possible that reduced __, improved __, or __ may help. These hypotheses came w/ discovery that correlation btn __ & __ kinda poor. | INCREASE HbF, REDUCED LEUKOCYTE CTS, PRO-INFLAMM CYTOKINES, RBC-ENDOTHELIAL INTERAXNS, RBC RHEOLOGY, NO RELEASE, HbF LEVELS, CLINICAL EFFECT |
SCA exacerbation TX: adequate __, __, aggressive __ if infexn present, inhaled __ (vasodilator) & __ if high risk of vaso-occlusion. Intermittent __ w/ __ reduce risk of stroke. Weigh benefits w/ complications like __ (__), __, & __. | HYDRATION, ANALGESICS, ANTIBIOTIC THERAPY, NO, TRANSFUSIONS, TRANSFUSIONS, PACKED RBCs, HEMOSIDEROSIS (IRON OVERLOAD), BLOOD-BORNE INFEXNS, IMMUNOLOGIC COMPLICATIONS |
Hydroxyurea __frequency of painful crises & reduces __. __HbF -> __ sickling (interferes w/ __). It might also preferentially enhance gene transcription of the __. | DECREASES, MORTALITY, INCREASED, DECREASED, POLYMERIZATION, GAMMA CHAINS |
Butyrate increases __ & efficiency of translation of __. It may lead to __ in formation of HbF. | HbF, GAMMA-GLOBIN mRNA, POST-TRANSLATIONAL PREFERENCE |
Recombinant form of Human EPO __. Combine w/ __ for best results. Mg2+ Pidolate & Clotrimazole treatment for SCA exacerbation is in development. It’s proposed effect is to prevent __ & __ loss from RBCs → prevent RBC __. | INCREASES HbF, HYDROXYUREA, K+, Cl-, DEHYDRATION |
In anemia the spleen may be enlarged d/t __ b/c they have a __. In CML it could mean that the spleen has __ some __ b/c BM is pumping out too many __. | SEQUESTERED RBCs, SHORT LIFESPAN, TAKEN OVER, RBC FORMATION, RBC PRECURSORS |
The spleen functions to maintain __ of RBCs in the __ by removal of __ & __ RBCs. It also removes Ab-coated __ & Ab-coated __ from circulation. Ab synthesis occurs in the __. __ can result from an increase in these normal functions. | QUALITY, RED PULP, SENESCENT, DEFECTIVE, BACTERIA, BLOOD CELLS, WHITE PULP, SPLENOMEGALY |
Iron Deficiency Anemia: normal __, __cytic, __chromic – __serum iron, __TIBC, __ferritin. Hemolytic Anemia: __ serum bilirubin, __ reticulocytes (pumping out more to make up for __). Blood loss: __cytic, __chromic | |
__ deficiency. | SHAPES, MICRO, HYPO, LOW, HIGH, LOW, HIGH, HIGH, HEMOLYSIS, MICRO, HYPO, IRON |
RBCs of different sizes. | ANISOCYTOSIS |
RBCs of different shapes. | POIKILOCYTOSIS |
As a group __ are the most common single gene disorder in humans. They’re caused by imbalance in __ synthesis | THALASSEMIAS, GLOBIN CHAIN |
Normally, α- & ß-globin chain synth is __ so ea has a __ to form __ (__). In thalassemias, synthesis of either __ or __ is defective. | COORDINATED, PARTNER, α2ß2 (HbA), ALPHA, BETA CHAIN |
αo- or ßo-thalassemia | No globin of that type produced. |
α+- or ß+-thalassemia | Reduced production |
In α-Thalassemia there are __ α-globin genes (__ on ea chromosome __). The α-globin chain is __ (b/c of btn __ bad genes) w/ no __ of any other chains. | 4, 2, 16, UNDERPRODUCED, 1-4, COMPENSATORY INCREASE |
Silent carriers of α-Thalassemia lack __ & have __. Patients with α-thalassemia trait lack __. | 1 α-GLOBIN GENE, NO PHYSICAL MANIFESTATIONS, 2 α-GLOBIN GENES |
Pts w/ thalassemia type HbH have __ & lack__. This is a __ hemolytic anemia. The Hb has very high__, so it’s almost useless in __. | ß4-TETRAMERS, 3 α-GLOBIN GENES, MILD-MODERATE, O2 AFFINITY, O2 DELIVERY |
Hb Barts pts have__ & lack__. This results in __ & intrauterine fetal death. | γ4-TETRAMERS, ALL 4 α-GLOBIN GENES, HYDROPS FETALIS, INTRAUTERINE FETAL DEATH |
α-Thalassemia is prevalent in __ & __. ß-Thalassemia is prevalent in __ populations. | ASIA, AFRICA, MEDITERRANEAN |
In ß-Thalassemia, there are only __ copies of ß-globin gene in ea cell (__ on ea chrom __). α-globin chains can’t form stable __ so they __ & __. α2γ2 (__) & γ4 (__) also occurs. | 2, 1, 11, TETRAMERS, PRECIPITATE, DIE PREMATURELY, HbF, Hb Bart’s |
in ß-Thalassemia minor/trait (__), the ß chain is __. __. | HETEROZYGOTE, UNDERPRODUCED, NO SPEC TX REQ’D |
In ß-Thalassemia major (__), the ß chain is __. Physical manifestations appear __ b/c ß-globin gene is __. ß-Thalassemia major pts are __ at birth, but soon __ req’s __. | HOMOZYGOTE, ABSENT, AFTER BIRTH, NOT EXPRESSED UNTIL LATE GESTATION, SEEMINGLY NORMAL, SEVERE ANEMIA, REGULAR TRANSFUSIONS |
Regular transfusions cause __ – death @ __yrs. Cardiac failure due to __. __ -> skeletal deformities. __ as transfusion alternative has helped extend life. In both cases, HbF production is compensatorily increased but is inadequate. | HEMOSIDEROSIS, 15-25, 2o HEMOCHROMATOSIS, MARROW EXPANSION, BM TRANSPLANTS |
HbS/ ß-Thalassemia has __ disease this is b/c thalassemia __ globin synthesis -> limited __. | MILD-MODERATE, DECREASES, MCHC |
Loss of blood =__, __ = Fe deficiency. Main cause of microcytic anemias is __ from __. You’ll see decreased serum __ & __, & increased __. Thalassemia is also __, but you’ll see __. __ & __ anemias are also microcytic. | MICROCYTIC, HYPOCHROMIC, Fe DEFICIENCY, BLOOD LOSS, IRON, FERRITIN, TIBC, MICROCYTIC, TGT CELLS, LEAD POISONING, SIDEROBLASTIC |
Under physiologic conditions, HbA is slowly & nonenzymatically __, the extent of which is dependent on the [plasma] of __. __ is the most abundant form of __ Hb. | GLYCOSYLATED, A PARTICULAR HEXOSE, HbA1c, GLYCOSYLATED |
HbA1c has glucose residues attached predominantly to the __groups of the __ of the beta-globin chains. Increased amts of HbA1c are found in __ b/c their HbA has contact w/ higher __ during the __ day lifetime. | NH2, N-TERMINAL VALINES, RBCs of DM PTS, [GLUCOSE], 120 |