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Pathology and final year > Haematology > Flashcards

Haematology Flashcards

1
Q

Presence of tartrate-resistant acid phosphatase

A

Hairy cell leukaemia

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2
Q

how long does transfusion related lung injury occur following blood transfusion

A

within 6hrs

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3
Q

Dyspnoea, distended neck veins and pink frothy sputum following blood transfusion

A

Fluid overload
(transfusion associated circulatory overload-TACO)

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4
Q

Impaired degredation of factors 5a and 8a

A

protein S deficiency

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5
Q

factor V unable to be broken down by protein C due to a substitution mutaiton

A

factor V leiden

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6
Q

causes of howel jolly bodies

A

splenectomy
autosplenectomy from sickle cell disease

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7
Q

translocation between chrom 11 and 14 resulting in BCL-1 overexpression

A

mantle cell lymphoma

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8
Q

translocation between chromosome 14 and 18 leading to BCL2 overexpression

A

follicular lymphoma

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9
Q

what lymphoma is associated with centrocytes and centroblasts

A

follicular lymphoma

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10
Q

what knind of ig are RhD Abs

A

IgG
(can cross the placenta)

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11
Q

what is the complication of RhD negative pregnant mother previously exposed to RhD blood

A

haemolytic disease of the newborn

hydrops fatalis

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12
Q

what screening technique is used to determine blood group

A

indirect antiglobulin technique

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13
Q

what are the pillars of pt blood management

A

optimise haemopoesis (iron, B12, folate, EPO)
minimise blood loss/ bleeding (eg. tranexamic acid, blood sparing techniques, stop anticoag meds)
optimise physiological tolerance to anaemia

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14
Q

what is the shelf life of red cells

A

35 days at 4C

(must be transfused within 4hrs of leaving fridge)

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15
Q

what is the shelf life of platelets

A

stored at 20C for 7 days

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16
Q

transfusion rates of blood products

A

RBC –> 1 unit over 2-3 hours

Plts, FFP, cryo –> 1 unit over 20-30 mins

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17
Q

indications for blood transfusion

A

major blood loss if 30% loss
critical care Hb <70g/L
post chemo Hb <80g/L
symptomatic anaemia

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18
Q

when is platelet transfusion contraindicated

A

heparin induced thrombocytopenia thrombosis

TTP

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19
Q

indications for platelet transfusion

A
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20
Q

indications for FFP transfusion

A

massive transfusion
liver disease
replacement of single coag factor
DIC only if active bleeding
TTP

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21
Q

what is cryoprecipitate

A

FFP that has been centrifuged and precipitate collected

contains higher concentration of fibrinogen, fVIII and VWF, IgA, albumin

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22
Q

requirements of blood for severely immunocompromised

A

irradiated blood

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23
Q

requirements of blood for intra-uterine /neonatal transfusions and for elective transfusion in pregnant women

A

CMV negative

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24
Q

requirements of blood for severely allergic people

A

washed

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25
Q

examples of acute transfusion reactions

A

ABO incompatibility
anaphylaxis
infection (bacterial)
febrile non-haemolytic
Transfusion related circulatory overload

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26
Q

examples of chronic transfusion reactions

A

delayed hamolytic transfusion reaction
GVHD
infection (viral/HBV, malaria, CJD)
post transfutsion purpura
Iron overload

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27
Q

signs of acute transfusion reaction

A

early:
raide in temp
raise in HR
fall in BP

symptoms (dependent on cause):
rigors ,fever, flushing, vomiting, dyspnoea, loin pain, chest pain, urticaria

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28
Q

differentials if reaction is happening within minutes of transfusion

A

anaphylaxis
ABO incopatibility
febrile non-haemolytic transfusion reaction

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29
Q

describe febrile non-haemolytic transfusion reaction

A

During / soon after transfusion (blood or platelets),
rise in temperature of 10C, chills, rigors

Common before blood was leucodepleted, now rarer

Have to stop or slow transfusion; may need to treat with
paracetamol

Cause: White cells can release cytokines during storage

mild-moderate

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30
Q

describe allergic transfusion reaction

A

Common especially with plasma
Mild urticarial or itchy rash sometimes with a wheeze
During or after transfusion
Usually have to stop or slow transfusion
IV antihistamines to treat (and prevent in future if recurrent)
Cause:
Allergy to a plasma protein in donor so may not recur again, depending on how common the allergen is
Commoner in recipients with other allergies and atopy

mild-moderate

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31
Q

describe ABO incompatibility (s&S and Mx)

A

Symptoms and signs of acute intravascular haemolysis- IgM
Restless, chest/ loin pain, fever, vomiting, flushing, collapse, haemoglobinuria (later);
↓BP & ↑HR (shock), ↑Temp
Stop transfusion – check patient / component
Take samples for FBC, biochemistry, coagulation,
Repeat x-match and Direct Antiglobulin Test (DAT)
Discuss with haematology doctor ASAP

severe-fatal

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32
Q

describe bacterial contamination reaction
(transfusion)

A

Restless, fever, vomiting, flushing, collapse.

↓BP & ↑HR (shock), ↑Temp

Bacterial growth can cause endotoxin production which causes immediate collapse
From the donor (low grade GI, dental, skin infection)
Introduced during processing (environmental or skin)
Platelets >red cells > frozen components (storage temp)

severe-fatal

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33
Q

describe anaphylactic transfusion reaction

A

↓BP & ↑HR (shock),
very breathless with wheeze,
often laryngeal &/or facial oedema

Mechanism:
IgE antibodies in patient cause mast cell release of granules & vasoactive substances
Most allergic reactions are not severe, but few are e.g. in IgA deficiency

severe-fatal

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34
Q

respiratory complications of transfusion

A

Transfusion Associated Circulatory Overload (TACO)- most common out of other resp complications

Transfusion Related Acute Lung Injury (TRALI)

Transfusion Associated Dyspnoea (TAD)

presents within 6hrs of transfusion

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35
Q

describe Transfusion Associated Circulatory Overload (TACO)

A

Pulmonary oedema / fluid overload;

SOB, low sats, raised HR, raised BP

fluid overload on CXR and cardiac failure

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36
Q

examples of transfusion transmitted infections

A

maleria
Hep B/C
zika
HIV1+2
HTLV1+2
parvovirus
CJD

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37
Q

describe delayed haemolytic transfusion reaction

A

within 1 week
IgG mediated extravascular haemolysis

Jaundice, dark urine, renal failure
DAT positive

38
Q

describe GvsHD

A

always fatal
in severely immuno compromised recipiants who cant kill donor lymphocytes so donor lymphocytes attack host

avoid by giving irradiated blood products

features: severe diarrhoea, liver failure, skin desquamation,
bone marrow failure

39
Q

describe the mechanism of haemolytic diseas eof the newborn

A

RhD -ve mother is pregnant for the first time with a RhD+ve fetus

blood enters circulation during pregnancy or labour, mother creates antibodies

anti Rh abs are IgG so can cross the placenta

If the fetus in the next pregnancy is Rh+ve then the Abs will attack fetus RBCs

leads to fetal anaemia and haemolytic disease of the newborn and hydrops fetalis

40
Q

prevension of haemolytic disease of the newborn

A

always give anti-D prophylaxis in Rh-ve mothers in first pregnancy within 72hrs of sensitising event (eg. bleed)

anti D coats Rh Ag on fetus RBC and they get removed by the spleen before sensitisation

41
Q

examples of RhD sensitising events

A

spontaneous miscarriages if surgical evacuation needed and therapeutic terminations

amniocentesis and chorionic villous sampling

abdominal trauma (falls and car accidents)

external cephalic version (turning the fetus)

stillbirth or intrauterine death

42
Q

complications of anti-kell Abs on fetus

A

Kell causes reticulocytopenia in fetus as well as haemolysis

usually less severe as anti-D

43
Q

what is basophilic stippling a sign of

A

lead poisoning
alcohol excess
sideroblastic anaemia

44
Q

hypochromic microcitic RBC with anisopoikilocytosis

A

iron deficienct anaemia

45
Q

hypersegented neutrophil (>5 segments) sign of

A

B12/ folic acid deficiency
anti DNA synthesis meds eg. methotrexate

46
Q

what are target cells a sign of

A

iron deficiency
hyposplenism
thalassaemia
liver disease

47
Q

what are howell jolly bodies? and what are they a sign of

A

remnant of nucleus- usually removed by spleen when it filters the RBCs

sign of hyposplenism

48
Q

features of megaloblastic anaemia

A

hypersegmented neutrophil
large MCV

49
Q

features of hyposplenism

A

howell jolly bodies
target cells

50
Q

causes of iron deficiency anaemia

A

chronic blood loss
malabsorption
poor diet

51
Q

causes of B12 def

A

malabsorption
pernicious anaemia
poor diet

52
Q

causes of hyposplenism

A

IBD
Coeliac
sickle cell
SLE

splenectomy
trauma

53
Q

what deficiencies seen in coeliac

A

Iron, B12, folate, fat, calcium

54
Q

what deficiencies are seen in IBD

A

B12, bile salts (Fe deficincy due to chronic diseas/ bleeding)

55
Q

What description is given to red blood cells which are typically polychromatic and stain heavily for the presence of RNA?

A

reticulocyte

56
Q

small molecule drug used in the treatment of CLL and MOA

A

ibrutinib
Tyrosine kinase inhibitor

57
Q

revesible agent of rivaroxiban

A

andexenet alfa

58
Q

mutation in hereditary spherocytosis

A

Spectrin
ANK1/ ankyrin

59
Q

mode of inheritance of G6PD def and hereditary spherocytosis

A

G6PD- X linked RECESSIVE
hereditary sphero- aut dominant

60
Q

second most common genetic cause of pancreatic insufficiency leading to malabsorption and steatorrhoea (2nd to CF)

A

Shwachman diamond syndrome

61
Q

Heparin induced thrombocytopenia is associated with the formation of antibodies directed against what autoantigen?

A

platelet factor 4

62
Q

causes of acanthocytes (spike cells )

A

Liver disease, hyposplenism

63
Q

causes of basophilic stippling

A

Lead poisoning
megaloblastic anaemia
myelodysplasia
thalassaemia

64
Q

causes of burr cells (echinocyte)

A

artefact if sat in EDTA prior film
uraemia
renal failure
GI bleeding
stomach carcinoma

65
Q

causes of heinz bodies

A

G6PD deficiency
chronic liver disease

66
Q

causes of howell jolly bodies

A

post splenectomy, hyposplenism (sickle cell disease, coeliac, IBD, amyloidosis)
megaloblastic anaemia
hereditary spherocytosis

67
Q

What is a pelger huet cell

A

hyposegmented neutrophil with 2 lobes like a dumbell

congenital
myeloid leukaemias, myelodysplastic syndromes

68
Q

what is a right shift and what causes it

A

increased production of hypersegmented neutrophils (hypermature)

megaloblastic anaemia

69
Q

Anaemia cut off Hb values for men women and pregnant women

A

men <135g/L
women <115g/L

cut off for ferrous sulphate treatment
1st trim women <110g/L
2/3rd trim <105g/L
posr partum <100g/L

70
Q

causes of microcytic anaemia

A

iron deficiency
anaemia of chronic disease
sideroblastic anaemia
thalassaemia

71
Q

causes of normocytic anaemia

A

acute blood loss
anaemia of chronic disease
BM failure
renal failure
hypothyroidism
hameolysis
pregnancy

72
Q

casues of macrocytosis

A

non-megaloblastic
pregnancy
hypothyroidism
reticulocytosis
alcohol excess
cirrhosis

megaloblastic
B12/ folate deficiency
antifolates (eg. phenytoin)
cytotoxic drugs

73
Q

clinical signs of iron deficiency anameia

A

angular stomatitis
koilonychia
brittle hair
plummer vinson syndrome (web-like growth of membranes in the throat that makes swallowing difficult)

74
Q

describe anaemia of chronic disease

A

inhibition of RBC production driven by chronic cytokine release

IFN, TNF and IL1 reduce EPO receptor production

IL6 stimulates upregulation of hepcidin which decreases iron absorption from the gut (as it inhibits transferrin)

ferritin (intracellular protein store) high as Fe sequestered in macrophages to deprive pathogen from getting Fe

75
Q

what is sideroblastic anaemia and what are the signs

A

ineffective erythropoisis leading to iron loading casueing haemosiderosis

can be congential (eg. X linked ALAS2 mutation)
aquired (myelodysplastic syndromes, lead poisoning, alcohol XS, anti TB drugs)

signs:
ringed sideroblasts in BM (erythroid precursurs with iron deposition in mitochondria in a ring around the nucleus)

76
Q

describe hereditary spherocytosis

A

AD
spectrin or ankyrin deficiency
defect in RBC membrane
effect suseptibility with parvovirus B19 infection
extravascular haemolysis - splenomagaly

77
Q

describe G6PD deficiency

A

glucose-6-phosphate dehydrogenase
most common inherited RBC enzyme defect

X linked recessive

attacks- rapid anaemia with jaundice
triggers- fava beans/ broad beans (1 days after eating), drugs (primaquine, aspirin, sulphonamides- ~2/3 days after starting), acute stressors eg infection

78
Q

difference between warm and cold haemolytic anaemias

A

warm- occurs at body temperature, IgG, spherocytes
casues: primary idiopathic, lymphoma, CLL, SLE, methyldopa

cold occurs <37C, IgM, associated with raynauds
casues: primary idiopathic, EBV, mycoplasma

79
Q

describe TTP

A

Abs against ADAMTS13 or inherited deficiency
enzyme responsible for vWF breakdown so leads to build up of vwF in long strands which then act as a ‘cheese wire’ cutting up RBCs (MAHA) and increased activation of plts

pentad of:
MAHA
fever
renal impairment
neuro signs
thrombocytopenia

80
Q

What protein is typically defective in hereditary elliptocytosis?

A

spectrin

81
Q

The classical triad is of nail dystrophy (nail loss, longitudinal ridging), oral leukoplakia (white patches in the mouth) and skin pigmentation (lace like hyperpigmentation of skin creases) is suggestive of

A

dyskeratosis congenita

82
Q

What scoring system can be used to calculate the probability of developing heparin induced thrombocytopenia?

A

4T score

83
Q

what might be given for somoone with high risk of neutropenia during chemo

A

filgrastim- a granulocyte- colony stimulating factor

84
Q

what are the features of post thrombotic syndrome and what is the management

A

painful, heavy calves
pruritus
swelling
varicose veins
venous ulceration

Mx: graduated compression stockings

85
Q

what is the revaersal agent of dabigatran

A

idarucizumab

86
Q

Most common inherited thrombophilia (procoagulative state)

A

heterozygous factor V leiden

mutation in factor V making it resistant to breakdown by protein C

87
Q

where is vitamin B12 absorbed and what condition causes reduced B12 absorption

A

terminal ileum

chrons

88
Q

test used to diagnose paroxysmal nocturnal haemoglobinuria

A

Ham’s test

88
Q

causes of massive splenomegaly

A

CML
PRV
Kala-azar

89
Q

test used to investigate hereditary spherocytosis.

A

osmotic fragility test

90
Q

stain used which is useful in the differentiation of AML and ALL

A

sudan black B

91
Q

what is Tartrate-resistant acid phosphatase stain used for

A

diagnosing hairy cell leukaemia

Pathology and final year (21 decks)

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