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Flashcards in Anaemia Deck (89):
1

causes microcytic anaemia

iron deficiency, thalassaemia

2

causes normocytic anaemia

blood loss, haemolysis, chronic disease, marrow infiltration

3

causes macrocytic anaemia

megaloblastic anaemia

4

causes of iron deficient anaemia

bleeding from GI tract- benign, malignant; menorrhagia; pregnancy; malabsorption; malnutrition; bleeding urinary tract; hookworm, pulmonary haemosiderosis

5

symptoms/ signs iron deficient

koilonychia, glossitis, angular stomatitis, gastritis

6

investigations iron defi

endoscopy/colonoscopy, if GIT normal- test urine for haematuria; CXR

7

what can you give to correct iron deficiency

ferrous sulphate 200mg. give for 6 months

8

side effects ferrous sulphate

nausea, epigastric pain, diarrhoea constipation

9

what is the MCV for macrocytic

>98

10

MCV for normocytic

78-98

11

MCV for microcytic

12

what is megaloblastic anaemia caused by

B12 or folate deficiency

13

what are folate and B12 needed for

synthesis DNA

14

what is the name for B12

cobalamin

15

what do you get B12 from

animal sources- meat, fish, eggs, butter, milk

16

what does B12 combine with and where is it absorbed

combines with intrinsic factor (secreted by parietal cells), absorbed in the terminal ileum

17

causes of B12 deficiency

inadequate diet, intestinal malabsorption- ileal resection, crohns, congenital malabsorption, deficient gastric intrinsic factor- pernicious anaemia, gastrectomy

18

what is pernicious anaemia

autoimmune gastritis- reduced gastric secretion IF

19

presentation pernicious anaemia

patient is often early greying of the hair, blue eyes, fhx, associated autoimmune disorders- myxoedema, vitiligo, Addisons, thyrotoxicosis

20

features B12 deficiency

gradual onset symptoms. jaundice. glossitis and angular cheilosis. neuropathy- tingling in the feet, gait, visual.

21

treatment B12 deficiency

1mg hydroxycobalamin. then vit B12

22

where is folate in diet

in most foods. especially liver and green veg

23

where is folate absorbed

upper small intestine

24

causes of folate deficiency

malabsorption- coeliac, small bowel disease/resection, tropical sprue; dietary; liver disease; increased demand- pregnancy, haemolytic anaemia, malignancy, inflammatory diseases

25

features folate deficiency

same as B12 def but with no neuropathy

26

tests for causes B12 def

history, tests for IF antibody and parietal cell antibodies, upper GI endoscopy

27

tests for causes folate def

history, TTG and endomysial antibodies, duodenal biopsy. serum folate low, red cell folate low

28

treatment folate deficiency

5mg folic acid for 4 months

29

other causes raised MCV

alcohol, liver disease, myxoedema, reticulocytosis, cytotoxic drugs, aplastic anaemia, pregnancy, myeloma

30

normal red cell lifespan

120 days

31

what is haemolytic anaemia

abnormal destruction red blood cells, decreased lifespan RBCs. may be caused by fault in red cell or abnormality in its environment

32

inherited haemolytic anaemias

red cell membrane- hereditary spherocytosis, elliptocytosis; Hb- thalassaemia, sickling; G6PD

33

acquired haemolytic anaemias

immune- warm and cold autoimmune haemolytic anaemias; rhesus or ABO incompatability; trauma; infection; hypersplenism

34

features haemolytic

anaemia, jaundice, incr gallstones, splenomegaly, megaloblastic caused by decr folate

35

lab features haemolytic

extravascular- hb may be normal or reduced, raised reticulocyte, blood film, bone marrow shows incr erythropoiesis, bilirubin raised, haptogloibin absent. intravascular- raised Hb, positive Schumm test, haemosiderin in urine

36

causes of haemolytic anaemia- red cell fragmentation syndromes

cardiac valve, TTP, DIC, haemolytic uraemic syndrome

37

why is there jaundice in haemolytic

incr catabolism Hb releasing increased amounts bilirubin

38

what type of jaundice in haemolytic

uconjugated bilirubin so not present in urine

39

what is the most common inherited HA

hereditary spherocytosis

40

hereditary spherocytosis what is the defect in

red cell membrane protein- ankyrin

41

hereditary spherocytosis signs

splenomegaly, severe anaemia. fhx.

42

tests for hereditary spherocytosis

direct antiglobulin test- negative. EMA

43

treatment hereditary spherocytosis

splenectomy. folic acid. cholecystectomy- if gallstones

44

what happens in G6PD deficiency

red cells susceptible to oxidant stress- by drugs, infections, fava beans, neonatal period. X linked.

45

who is G6PD common in

Mediterranean, middle eastern, oriental

46

what does the blood film show in G6PD

in a crisis can be absent Hb- bite and blister cells. Heinz bodies (denatured Hb)

47

what is autoimmune haemolytic anaemia

autoantibodies against red cell membrane

48

what temp does warm AIHA work

37 degrees

49

what temp does cold AIHA work

0-5 degrees (4)

50

features warm AIHA

splenomegaly. DAT scan positive (differentiating from hereditary spherocytosis where is it negative). IgG

51

treatment warm AIHA

prednisolone 1mg/kg PO. transfusion if necessary, immunosuppressive drugs, splenectomy if fails, remove cause, treat underlying disease

52

warm AIHA antibody

IgG

53

cold AIHA antibody

IgM

54

DAT scan in cold AIHA

positive with C3d

55

features cold AIHA

acrocyanosis (purplish skin), Raynaud phenomenon. may be intravascular haemolysis and if severe- rigors, haemoglobinuria, renal failure

56

why is Hb high in intravascular haemolysis

as RBCs are broken down and released into the blood stream

57

causes of intravascular haemolysis

mismatched blood transfusion, G6PD, red cell fragmentation, autoimmune haemolytic, drug and infection

58

what are present in cold AIHA

cold agglutinins directed against red cells.

59

treatment cold AIHA

keep the patient warm, immunosuppression, plasma exchange to lower antibody titre

60

what is red cell fragmentation syndrome

when red cells are exposed to an abnormal surface eg artificial heart valve, DIC, damaged small vessels eg TTP. haemolysis is intra and extra vascular

61

what is thalassaemia

autosomal recessive. disorders of alpha or beta globin chain synthesis

62

alpha thalassaemia severity depends on

number of a genes deleted or dysfunctional there are 4 genes 2 on each chromosome 16

63

what happens if all 4 alpha genes are inactive

hydrops foetalis. fetus cant make fetal or adult Hb. death in utero or neonatally

64

what is the lab of thalassaemia

hypochromic microcytic

65

what is it called when 3/4 a genes are inactive

haemoglobin H disease

66

what do you get if one or 2 a genes are inactive

a thalassaemia trait

67

what is thalassaemia major

complete or almost complete failure B globin chain synthesis. severe imbalance a:b chains and a chains are deposited in erythroblasts. innefective eryhthropoiesis, severe anaemia, extra medullary haemopoiesis

68

features thalassaemia major

anaemia (3-6m), failure to thrive, infection, pallor, jaundice, hepato and splenomegaly, frontal bossing and hair on end appearance on x ray. iron overload

69

management thalassaemia major

regular tranfusions- packed red cells, iron chelation- desferrioxamine (SC) 5-7 nights weekly. oral vit C incr iron excretion; deferiprone (oral)

70

why does anaemia present at 3-6m in thal major

switch from y to b chain synthesis

71

what is the presentation of thalassaemia intermedia

less severe. present later (2-5 years), normal bones changes and growth

72

b thalassaemia trait

asymptomatic hypochromic microcytic anaemia

73

if both parents have b thal trait, what is the chance of having thal major

25%

74

what is sickle cell disease

point mutation in B gene- substitution valine for glutamic acid. insolubility of HB S- crystallise causing sickling and vascular occlusion

75

what are the B like in sickle cell anaemia and trait

2 x BS in anaemia, BS with BA in trait

76

what are the crises in sickle cell

vaso-occlusive, sequestration, aplastic

77

what happens in vaso occlusive crises

incr sickling with blockage small vessels. infection, dehydration, acidosis, deoxygenation. abdominal pain, bone pain, CNS

78

what happens in sequestration crises

sickling with pooling red cells in liver, spleen, lungs. acute chest syndrome

79

what happens in aplastic crisis

follows infection by B19 parvovirus. temp arrest erythropoiesis. severe anaemia need transfusion

80

clinical features sickle cell

increased susceptibility to infection, gallstones, legulcers, avascular necrosis fem and hum heads, cardiomyopathy, pulm hypertension

81

diagnosis sickle cell

Hb 70-90, blood film- sickle cells, screening for sickling. can do cranial Doppler to assess for stroke, retinal exam

82

treatment sickle cell

avoid precipitants of crisis, vaccination, folic acid. red cell transfusion in severe anaemia.

83

how is vaso occlusive crisis treated

hydration, saline, analgesia

84

how is sequestration crisis treated

exchange transfusion to reduce HbS

85

what medication can be given in sickle cell to reduce freq sickle cell crises

hydroxycarbamide

86

how does sickle trait present

benign condition without anaemia, asymptomatic. can be haematuria in oxygen deprivation

87

conditions causing anaemia of chronic disease

chronic infections eg tb,osteomyelitis, HIV; ronic inflam- RA, SLE; malignant- carcinoma, lymphoma; CCF; endo- hypo and hyper thyroid

88

what is released by the liver in anaemia of chronic disease

hepicidin

89

what cells are seen on blood film in sickle cell

target cells, pencil cells