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Flashcards in Anaemia Deck (109):
1

Anaemia definition

reduction of total circulating red cell mass below normal limits - reduces oxygen carrying capacity of blood

2

reduced oxygen carrying capacity of blood could lead to

tissue hypoxia

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etiological clues to anaemia (3)

red cell size, shape, degree of haemoglobinisation

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3 red cell sizes

normocytic, microcytic, macrocytic

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2 degrees of haemoglobinisation

normochromic, hypochromic

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microcytic and hypochromic anaemia suggest

haemoglobin synthesis disorder

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macrocytic anaemia suggests

maturation in bone marrow abnormalities

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leg ulcers suggest

sickle cell anaemia

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bone deformities suggest

thalassaemia major

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jaundice suggests

haemolytic anaemia

11

koilonychia (spoon-shaped nails) suggests

iron deficiency anaemia

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3 features of haemolytic anaemias

premature destruction and short life span RBCs, accumulation of breakdown material, elevated erythropoietin and rate of erythropoiesis

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cause of extravascular haemolytic anaemia

alterations which make RBCs less deformable

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causes of intravascular haemolytic anaemia (4)

mechanical injury, complement fixation, intracellular parasites, exogenous toxic factors

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3 signs of extravascular haemolytic anaemia

anaemia, splenomegaly, jaundice (bilirubin)

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3 signs of intravascular haemolytic anaemia

anaemia, jaundice (bilirubin), haemoglobinaemia

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process extravascular haemolytic anaemia

can't navigate splenic pulp > red cell sequestration > phagocytosis within cords > some escapes

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process intravascular haemolytic anaemia

large amount Hb free from lysed cells > bound to HAPTOglobin > rapidly cleared by phagocytes > Hb oxidised to METHEMOglobin (brown) > some passes in urine

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Jaundice is from

iron build up in kidney > bilirubin

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genetics of sickle cell disease

maturation in 6th codon of beta globin > replacement of glutamate residue with valine residue

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in sickle cell disease, glutamate residue is replace with

valine residue

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haemoglobin type of sickle cell sufferers

HbS

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HbS heterozygous phenotype

sickle cell trait

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sickle cell trait protects against

malaria

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sickle cell trait symptoms

asymptomatic

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sickle cell disease and trait most common in people of what descent

african

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3 major pathological manifestations of sickle cell anaemia

chronic haemolysis, microvascular occlusions, tissue damage

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4 variables affecting sickling

interaction of HbS with other types of Hb in cell, mean cell Hb concentration, transit time of red cells through microvascular beds, intracellular pH

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sickle cell trait, percentage HbS and HbA

HbS = 40%, HbA = 60%

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HbA interferes with

HbS polymerisation

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RBCs of people with sickle cell TRAIT do not sickle unless under what conditions

hypoxic conditions

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intracellular what facilitates sickling

dehydration

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intracellular dehydration facilitates sickling because

increases mean cell Hb conc.

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transit times slow where (3)

spleen, bone marrow, inflamed vascular beds

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why might vascular beds be inflamed

increased adhesion molecules

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sickle red cells express higher than normal amounts of what

adhesion molecules

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decrease in pH reduced Hb affinity for what

oxygen

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reduced Hb oxygen affinity increases fraction of

deoxygenated HbS

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low pH increases tendency of

sickling

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sickle cell disease, splenomegaly can be seen in

children

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autosplenectomy seen in

adults with sickle cell disease

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progressive hypoxic tissue damage leads to

autosplenectomy

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how does sickling damage RBCs

HbS polymers grow > herniate membrane > influx calcium ions into RBC > efflux potassium ions and water > dehydration > increased density and rigidity

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sickle cell - damage to RBCs - influx of

calcium ions

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sickle cell - damage to RBCs - efflux of

potassium ions and water

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sickle cell is a form of what type of anaemia

haemolytic

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vaso-occlusive crises

episodes of infarction causing sever pain in affected region

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vaso-occlusive crises common in what tissue

bone

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chronic hypoxia in sickle cell responsible for

impaired growth and development

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sickle cell prognosis improved by (4)

DNA synthesis inhibitor drugs, bone marrow transplants, prophylactic antibiotics, blood transfusions

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HbA has which 4 chains

2 alpha, 2 beta

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HbA alpha chains genes on chromosome

16

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HbA beta gene on chromosome

11

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thalassaemia consequences are from

Hb deficiency and excess of other globin chain

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thalassaemia CARRIERS are protected against

Malaria

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how many HbA alpha chain genes

2

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how many HbA beta chain genes

1

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two categories of beta thalassaemias

beta 0 and beta positive

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beta zero thalassaemia mutations associated with

absent beta globin synthesis

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beta positive thalassaemia mutations associated with

reduced beta globin synthesis

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most common cause of beta positive thalassaemia

splicing mutations

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most common cause of beta zero thalassaemia

chain terminator mutations

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mutations associated with beta positive thalassaemia

promotor region mutations (and splicing mutations)

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beta thalassaemia is (2)

hypochromic, microcytic

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two beta thalassaemia alleles

beta thalassaemia major

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one beta thalassaemia allele

beta thalassaemia minor

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beta thalassaemia minor is (2)

asymptomatic, microcytic

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beta thalassaemia major manifests when

6-9 months after birth (hbF-->HbA)

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major red cell Hb in beta thalassaemia major

HbF

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blood transfusion needed or death as child in

beta thalassaemia major

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bony prominences enlarged and distorted in

beta thalassaemia major

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only cure for beta thalassaemia major is

bone marrow transplant

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in newborns with alpha thalassaemia (hydrops fetalis), excess unpaired gamma globin chains form gamma 4 tetramers known as

haemoglobin barts

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in order children and adults with alpha thalassaemia, excess beta globin chains form beta 4 tetramers known as

HbH

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haemolysis and ineffective erythropoiesis less severe than

beta thalassaemia

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most common cause of reduced alpha chain synthesis and therefore alpha thalassaemia

gene deletion

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silent carrier state alpha thalassaemia

deletion of single alpha globin gene allele

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symptoms of silent carrier state alpha thalassaemia

asymptomatic

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silent carrier state alpha thalassaemia cell type

microcytic (slight)

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deletion of two alpha globin gene alleles

alpha thalassaemia trait

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deletion of two alpha globin gene alleles from single chromosome

asian

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deletion of two alpha globin gene alleles from two different chromosomes

african

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alpha thalassaemia trait cell type

microcytic

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symptoms of alpha thalassaemia trait

asymptomatic

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deletion of 3 alpha globin gene alleles

haemoglobin H disease (HbH disease)

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HbH disease most common in which populations

asian

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in HbH tetramers of what form

beta globin

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HbH has what kind of affinity for oxygen

very high

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deletion of all four alpha globin gene alleles

hydrops fetalis

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barts have what type of affinity for oxygen

very high

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hydrops fetalis have life long dependency on

blood transfusion

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only possible cure for hydrops fetalis

bone marrow transplant

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vitamin B12 deficiency anaemia is what type of anaemia (2)

megaloblastic, macrocytic

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pernicious anaemia

vitamin B12 deficiency anaemia resulting from inadequate gastric production / defective function of INTRINSIC FACTOR

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most common cause of vitamin B12 deficiency anaemia

long-standing malabsorption

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3 types of antibody found in pernicious anaemia

parietal canalicular, blocking, binding antibodies

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spinal cord disease associated with which anaemia

vitamin B12 deficiency anaemia

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treatment for vitamin B12 deficiency anaemia

hydroxycobalamin (vitamin B12)

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median age of diagnosis of pernicious anaemia

60

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folate deficiency anaemia very similar to vitamin B12 deficiency anaemia other than

no neurological abnormalities

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treatment for folate deficiency anaemia

oral folic acid

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most common cause of anaemia worldwide

iron deficiency anaemia

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percentage of world affected by iron deficiency anaemia

30%

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most common cause iron deficiency anaemia (developed world)

blood loss

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most common cause iron deficiency anaemia (developing world)

nutritional deficiencies

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iron absorbed where

duodenum

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cell type in iron deficiency anaemia (2)

microcytic, hypochromic

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long standing anaemia leads to

spooning of fingernails

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conditions carrying increased likelihood of iron deficiency (4)

pregnancy, adolescence, periods of rapid growth, intermittent history of blood loss