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galvan Flashcards

1
Q

anaemia is when haemoglobin is below what

A

120 in females and 130 in males

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2
Q

what is the normal response to anaemia

A

to make more blood cells (reticulocytosis )

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3
Q

describe reticulocytes

A

still have remnants of RNA, stain purple/deeper red, blood oil appears polychromatic and larger than average red cells

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4
Q

when will there be a low reticulocyte count

A

when there is decreased production

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5
Q

when might have anaemia but high reticulocyte count

A

bleeding or haemolysis

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6
Q

what murmur can be a sign of anaemia

A

systolic

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7
Q

reticulocyte assesses response of what

A

marrow

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8
Q

what anaemia is caused by deficient haemoglobin synthesis

A

microcytic

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9
Q

most common cause of microcytic anaemia

A

iron deficiency

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10
Q

in anaemia of chronic disease what is released and increases production of hepcidin. subsequently downregulating ferroportin

A

IL-6

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11
Q

what anaemia can be due to problems with porphyrin synthesis

A

sideroblastic

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12
Q

what has an excess iron build up in mitochondria due to failure to incorporate iron into haem

A

sideroblastic anaemia

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13
Q

acquired caused of sideroblastic anaemia

A

myelodysplastic syndrome, lead poisoning, alcohol excess

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14
Q

what are global deficiencies

A

Thalassaemia

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15
Q

circulating iron is bound to

A

transferrin

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16
Q

tissues that especially express transferrin receptors

A

erythroid marrow

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17
Q

what is increased in genetic haemochromatosis

A

% saturation of transferrin with iron (measures iron supply)

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18
Q

where is iron stored

A

in ferritin mainly in the liver

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19
Q

what is an indirect measure of storage iron

A

serum ferritin. Low ferritin means iron deficiency

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20
Q

what blocks ferroportin mediated release of iron

A

hepcidin

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21
Q

what falls when iron deficient erythropoiesis

A

MCV

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22
Q

what is koilonhcyia

A

spoon shaped nails

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23
Q

what reflects hypo chromic

A

MCH - mean corpuscular haemoglobin

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24
Q

microcytic is determine by

A

MCV

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25
what is poikilocytosis
variation in shape of red cells
26
what is anisocytosis
variation in size of red blood cells
27
what correlates with total iron body stores
serum ferritin
28
improving gastric what can improve iron intake
gastric acidity
29
typically need iron supplements for how long to replenish stores
2-3 months
30
what anaemia has defective DNA synthesis
megaloblastic
31
characterised by the presence in the bone marrow of
erythroblasts
32
folate is found in what
leafy green vegetables
33
drugs that can cause folate deficiency
anticonvulsants
34
what is a megaloblast
abnormally large nucleated red cell precursor with an immature nucleus
35
what anaemias are characterised by a lack of red cells
megaloblastic
36
a macrocyte lacks what
nucleus
37
a megaloblast has what
immature nucleus
38
folate and B12 are absorbed where
folate - duodenum and jejunum B12- ileum
39
source of B12
animal
40
what does fit B12 bind to
intrinsic factor
41
what glycoprotein transports fit B12
transcobalamin II
42
patients with what anaemia may appear mildly jaundiced
macrocytic
43
how might B12/folate deficiency present
like anaemia, weight loss, sore tongue, diarrhoea
44
blood film shows macrovalocytes and hypersegmented neutrophils (3-5 nuclear segments)
macrocytic anaemia
45
does low levels of B12 and folate in serum mean deficiency
no and normal levels may not always indicate it is normal
46
what antibodies are specific for macrocytic anaemia
anti intrinsic factor
47
in what anaemia do you need vitamin B12 injections for life
pernicious anaemia
48
causes of non megaloblastic macrocytosis WITH anaemia
alcohol, liver disease, hypothyroidism
49
what marrow failures are causes of macrocytosis without megaloblastic anaemias that are associated with anaemia
myelodysplasia, myeloma, anaplastic anaemia
50
what happens in spurious (false) macrocytosis
volume of the mature red cell is normal, but the MCV is measured as high
51
examples of when spurious macrocytosis may occur
reticulocytosis and cold agglutinins
52
thalassaemia have what inheritance pattern
recessive
53
having a thalassaemia trait is believed to confer some protection against what
falciparum malaria
54
consequence of alpha thalassaemia
microcytic hypochromic anaemia
55
how can you differ alpha thalassaemia trait from iron deficiency
in alpha thalassaemia the ferritin will be normal. in beta it can be normal or high but won't be low
56
there is normally how many alpha genes
4
57
clinical features of thalassaemias
jaundice, fatigue and facial bone deformities
58
FBC in thalassaemias
microcytic hypochromic anaemia
59
what is needed to make the diagnoses in thalassaemias
genetic analysis
60
alpha genes are from what chromosome
16
61
mutations affecting Beta global synthesis are from what chromosome
11
62
what is diagnostic of beta thalassaemia minor trait
HbA2
63
blood film shows target cells and basophilic stippling
beta thalassaemia minor
64
blood film shows marked anisopoikilocytosis, target cells and nucleated RBCs
Beta thalassaemia major
65
management of severe thalassaemias
lifelong blood transfusions, splenectomy is an option particularly in those with HbH disease, bone marrow transplant( if carried out before complications develop)
66
biggest complication of thalassaemia
iron overload from transfusion becomes the main cause of mortality
67
iron overload from transfusion for thalassaemias can be prevented by
iron chelating agents eg desferrioxamine
68
what are sickle syndromes
group of genetic disorders that affect the structure of haemoglobin
69
the sickle beta is due to mutation on codon 6 that substitutes glutamine to
valine
70
clinical features of sickle cell
acute pain in hands and feet, acute chest syndrome, pulmonary hypertension, anaemia
71
bone marrow aplasia can occur in sickle syndrome and most commonly follows infection with
parvovirus B19
72
management of sickle crises
oxygen, fluids and analgesia. red cell exchange transfusion in severe crisis and antibiotics if evidence of infection
73
what prophylaxis for sickle cell
penicillin
74
what supplementation for long term management of sickle anaemia
folic acid
75
what can be given if severe sickle crises occur
hydroxycarbamide
76
is autoimmune haemolytic anaemia Coombs positive or negative
positive
77
do red blood cells have a mitochondria
no
78
extravascular haemolytic anaemia occurs where
spleen and liver
79
feature of haemolytic anaemia
anaemia, splenomegaly and jaundice
80
spherocytes suggest
membrane damage
81
heinz bodies
oxidative damage due to G6PD deficiency
82
first line mx for warm autoimmune haemolytic anaemia
prednisolone
83
which AIHA has IgM
cold
84
mx for cold AIHA
supportive. steroids not shown to have a benefit
85
what are schistocytes
fragments of RBCs

My haem (8 decks)

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