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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
Q

what is poikilocytosis

A

variation in shape of red cells

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26
Q

what is anisocytosis

A

variation in size of red blood cells

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27
Q

what correlates with total iron body stores

A

serum ferritin

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28
Q

improving gastric what can improve iron intake

A

gastric acidity

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29
Q

typically need iron supplements for how long to replenish stores

A

2-3 months

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30
Q

what anaemia has defective DNA synthesis

A

megaloblastic

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31
Q

characterised by the presence in the bone marrow of

A

erythroblasts

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32
Q

folate is found in what

A

leafy green vegetables

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33
Q

drugs that can cause folate deficiency

A

anticonvulsants

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34
Q

what is a megaloblast

A

abnormally large nucleated red cell precursor with an immature nucleus

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35
Q

what anaemias are characterised by a lack of red cells

A

megaloblastic

36
Q

a macrocyte lacks what

37
Q

a megaloblast has what

A

immature nucleus

38
Q

folate and B12 are absorbed where

A

folate - duodenum and jejunum
B12- ileum

39
Q

source of B12

40
Q

what does fit B12 bind to

A

intrinsic factor

41
Q

what glycoprotein transports fit B12

A

transcobalamin II

42
Q

patients with what anaemia may appear mildly jaundiced

A

macrocytic

43
Q

how might B12/folate deficiency present

A

like anaemia, weight loss, sore tongue, diarrhoea

44
Q

blood film shows macrovalocytes and hypersegmented neutrophils (3-5 nuclear segments)

A

macrocytic anaemia

45
Q

does low levels of B12 and folate in serum mean deficiency

A

no and normal levels may not always indicate it is normal

46
Q

what antibodies are specific for macrocytic anaemia

A

anti intrinsic factor

47
Q

in what anaemia do you need vitamin B12 injections for life

A

pernicious anaemia

48
Q

causes of non megaloblastic macrocytosis WITH anaemia

A

alcohol, liver disease, hypothyroidism

49
Q

what marrow failures are causes of macrocytosis without megaloblastic anaemias that are associated with anaemia

A

myelodysplasia, myeloma, anaplastic anaemia

50
Q

what happens in spurious (false) macrocytosis

A

volume of the mature red cell is normal, but the MCV is measured as high

51
Q

examples of when spurious macrocytosis may occur

A

reticulocytosis and cold agglutinins

52
Q

thalassaemia have what inheritance pattern

53
Q

having a thalassaemia trait is believed to confer some protection against what

A

falciparum malaria

54
Q

consequence of alpha thalassaemia

A

microcytic hypochromic anaemia

55
Q

how can you differ alpha thalassaemia trait from iron deficiency

A

in alpha thalassaemia the ferritin will be normal. in beta it can be normal or high but won’t be low

56
Q

there is normally how many alpha genes

57
Q

clinical features of thalassaemias

A

jaundice, fatigue and facial bone deformities

58
Q

FBC in thalassaemias

A

microcytic hypochromic anaemia

59
Q

what is needed to make the diagnoses in thalassaemias

A

genetic analysis

60
Q

alpha genes are from what chromosome

61
Q

mutations affecting Beta global synthesis are from what chromosome

62
Q

what is diagnostic of beta thalassaemia minor trait

63
Q

blood film shows target cells and basophilic stippling

A

beta thalassaemia minor

64
Q

blood film shows marked anisopoikilocytosis, target cells and nucleated RBCs

A

Beta thalassaemia major

65
Q

management of severe thalassaemias

A

lifelong blood transfusions, splenectomy is an option particularly in those with HbH disease, bone marrow transplant( if carried out before complications develop)

66
Q

biggest complication of thalassaemia

A

iron overload from transfusion becomes the main cause of mortality

67
Q

iron overload from transfusion for thalassaemias can be prevented by

A

iron chelating agents eg desferrioxamine

68
Q

what are sickle syndromes

A

group of genetic disorders that affect the structure of haemoglobin

69
Q

the sickle beta is due to mutation on codon 6 that substitutes glutamine to

70
Q

clinical features of sickle cell

A

acute pain in hands and feet, acute chest syndrome, pulmonary hypertension, anaemia

71
Q

bone marrow aplasia can occur in sickle syndrome and most commonly follows infection with

A

parvovirus B19

72
Q

management of sickle crises

A

oxygen, fluids and analgesia. red cell exchange transfusion in severe crisis and antibiotics if evidence of infection

73
Q

what prophylaxis for sickle cell

A

penicillin

74
Q

what supplementation for long term management of sickle anaemia

A

folic acid

75
Q

what can be given if severe sickle crises occur

A

hydroxycarbamide

76
Q

is autoimmune haemolytic anaemia Coombs positive or negative

77
Q

do red blood cells have a mitochondria

78
Q

extravascular haemolytic anaemia occurs where

A

spleen and liver

79
Q

feature of haemolytic anaemia

A

anaemia, splenomegaly and jaundice

80
Q

spherocytes suggest

A

membrane damage

81
Q

heinz bodies

A

oxidative damage due to G6PD deficiency

82
Q

first line mx for warm autoimmune haemolytic anaemia

A

prednisolone

83
Q

which AIHA has IgM

84
Q

mx for cold AIHA

A

supportive. steroids not shown to have a benefit

85
Q

what are schistocytes

A

fragments of RBCs

My haem (8 decks)

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