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Joshua's Path > Haematology > Flashcards

Haematology Flashcards

1
Q

What is the most common form of haemoglobin?

A

Hb A - made up of 2alpha chains and 2 beta chains. forms 90% of the haemaglobin

Hb A2 is made up of 2 alpha chains and 2 delta chains and makes up 1-3% of the haemoglobin. A raised level may indicate thallassaemia

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2
Q

what additional test should be ordered with iron studies?

A

CRP

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3
Q

What should you think of when patients have pre existing conditions in questions?

A

IS IT AUTOIMMUNE!!!

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4
Q

Sever complication of pernicious anaemia

A

subacute combined degeneration of the spinal cord

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5
Q

Name 4 inherited aplastic anaemias? what proportion of AA’s do they make up?

A

10% the other 70% are idiopathic and should be treated with immunosuppression a nd supportive care - transfusion abx and iron chelation if persitant. marrow stimulation and recovery may be used also e.g g-csf.

(D)DFS - to buy them a sofa
Fanconi's anaemia
dyskeratosis congenita
Schwachman diamond syndrome
Diamond Blackfan syndrome
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6
Q

Triad of dyskeratosis congenita

A

SON
skin pigmentation
oral leukoplakia
nail dystrophy

chromasome instability and telomere shortening - BM failure

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7
Q

Symptoms of schwachman diamond syndrome?

A

primarily neutrophilia - endocrine and pancreatic dysfunction

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8
Q

What blood results do you see in DIC? causes?

A

low platelets and fibrinogen, high fibrinogen degredation products, high d dimer, prolonged prothrombin time

Causes = malignancy, trauma, burns, toxins, obstetric complications.

Treatment = FFP, platelets, cryoprecipitate

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9
Q

What joint is most commonly affected by pseudogout?

A

Knee

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10
Q

what is another name for pseudogout

A

chondrocalcinosis

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11
Q

what type of bifringence do crystals show?

A

rhomboid shape with positive bifringence? BLUE WITH the axis.
pyrophosphate crystals

true gout is YELLOW WITH the axis

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12
Q

What counts as MASSIVE blood loss?

A

entire blood volume in 24 hours, >50% in 3 hours

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13
Q

What is the normal cutoff for platelet transfusion?

A

most people say <50 however some say 75 (x10^9)

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14
Q

What is the difference between red cell concentration and red cell mass

A

red cell concentration varies with hydration status and plasma volume. q

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15
Q

what drives anaemia of chronic disease

A

cytokines such as IFN, IL1 and TNF decrease EPO recepter expression

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16
Q

What are downs syndrome children at risk of (haem)

A

AML in first 3 years of life and then ALL

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17
Q

What is an acute leukaemia

A

Acute Neoplastic process of bone marrow and blood resulting in a rapidly progressing and fatal disease which has >20% blast cells on BM biopsy.

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18
Q

What is the best way to differentiate acute and chronic leukaemias clinically?

A

Chronic types are usually diagnosed incidentally, wheras acute have more severe presentations with BM failure and cytopenias - anaemia, petechiae, infections (neutropenia), and hepatosplenomegaly, cranial nerve palsies due to CNS involvement, gum hypertrophy (AMMoL often with hypoK) lymphadenopathy and bone bain.

However CML in blast phase can resemble an acute leukaemia.

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19
Q

TDT +ve

A

ALL - displayed on pre b

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20
Q

Philadelphia chromasome positive

A

usually CML but can be ALL in adults. (30% positive)

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21
Q

Name 2 subtypes of acute myeloid leukaemia

A

Acute promyleocytic leukaemia characterised by a t(15,17) with hypergranular promyelocytes with auer rods. it causes early DIC and haemorrhage. (DIC may occur with infection as a result of any leukaemia)
requires ATRA

Acute myelomonocytic leukaemia characterised by gum hypertrophy and

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22
Q

Are protein C and protein S, procoagulant or anticoagulant?

A

anticoagulant. along with TFPI, thrombomodulin, antithrombin (these are often expressed on the vessel wall)

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23
Q

how long does a flight need to be before it confers an increase thrombosis risk

A

9 hours

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24
Q

Which has the largest thrombosis risk:

Factor 5 leiden
Antithrombin deficiency
family history of thrombosis
Protein C/S deficnency

A

C/s and antithrombin deficiency are wrst followed by 5 leiden.

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25
Q

What cell lines should be seen on a CML Bone marrow

A

Hypercellular marrow with spectrum of immature myelocytes and mature granulocytic cells in the blood. neutrophils in blood are 50-500

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26
Q

what are ethe three phases of CML

A

Chronic <5% blasts
Accellerated >10% blasts - increasing splenomegaly
Blast >20% blasts resembling acute leukaemia
(ALL transformation can occur)

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27
Q

whats the difference between essential thrombocythemia and thrombocytopenic purpura

A

high and low

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28
Q

How do you differentiate hodgkins and non hodgkins lymphomas

A

hodgkins - pain on drinking alcohol in affected nodes, reed sternberg

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29
Q

what is nodular lymphocytic (predominant) pertain to?

A

non classical type of hodgkins lymphoma. accounts for small number of cases and is indolent. not EBV associated. rarely has B symptoms. mainly lymphadenotpathy. it has atypical reed sternberg cells.

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30
Q

Describe the staging in hodgkins lymphoma

A

ANN ARBOR
stage 1: 1 lymph node region
stage 2 2 or more lymph node regions on same side of D
stage 3: 2 or more lymph nodes on opposite sides of D
Stage 4: extranodal sites e.g liver/BM

A- no constitutional sx B - fever/night sweat/weight loss

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31
Q

How can you treat von willebrand disease

A

Desmopressin can be used to increase the concentration of VwF and factor 8. this is only in type 1 and 2 disease. major disease - type 3 it would not be effective

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32
Q

clinical features of myelodysplastic syndromes

A

Fatigue, infection, bleeding. hypercellular marrow(as in CML)
qualitative cytopenias risk of AML transformation.

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33
Q

What is a ringed sideroblast? what myelodysplasias does it feature in?

A

abnormally nucleated blast cell with iron granule ring
occur in refractory anaemia with ringed sideroblasts. must have greater than 15% ringed sideroblasts. and erythroid dysplasia.

may also be part of refractory cytopenia with ringed sideroblasts. must have>15% ringed sideroblasts along with:

Cytopenia in 2 cell lines along with >10% dysplastic cells in 2 or more cell lines.

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34
Q

Define Refractory anaemia

A

anaemia, no blasts in blood. erythroid dysplasia with <5% blasts in BM

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35
Q

Define refractory anaemia with excess blasts 1

A

Blood:
cytopenias
<5% blasts
no auer rods

Bone Marrow
5-9% blasts
dysplasia

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36
Q

Define refractory anaemia with excess blasts 2

A

Blood:
cytopenias or 5-19% blasts or auer rods

Bone Marrow
dysplasia with
10-19% blasts, or
auer rods

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37
Q

what do MDS sufferers die from

A

International prognostic scoring system used to predict which takes into account rule of thirds

  • infection
  • bleeding
  • AML
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38
Q

Define MDS with 5q deletion

A

Blood
Anaemia
normal or increased platelets

Marrow
Hypolobulated nuclei of megakaryocytes
<5% blasts

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39
Q

Define unclassified MDS

A

complex -
Blood
cytopenias with no blasts or aur rods

BM
Myeloid or megakaryocytic dysplasia
<5% blasts

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40
Q

what nationality get adult t cell lymphoma

A

caribbean and Japanese
associated with HTLV1 infection
Hypercalcaemia and lymphocytosis. skin lesions and splenomeg

41
Q

Mantle cell lymphoma

A

t(11,14) BCL-1 locus causing overexertion of cyclin D1

They are aggressive lymphomas with angular nuclei on histology. occur in men more than women.

42
Q

Diffuse Large b cell lymphoma

A

germinal cell lymphoma - may have undergone richter’s transformation from other low grade germinal cell lymphoma or CLL.

Very aggressive high grade with sheets of large lymphoid cells which needs to be treated with RCHOP.

43
Q

What does RCHOP stand for and what is it used in?

A 
Rituximab
cyclophosphamide
vincristine
doxorubicin
prednisolone

DLBCL and mantle cell.

44
Q

What chemo is often used in hodgkins lymphoma

A

ABVD - adriamycin, bleomycin, vinblastine, decarbazine.

can use MOPP but makes sterile

45
Q

What is erythmelalgia?

A

painful burning or swelling sensation in the fingers

often associated with essential thrombocythemia - megakaryocytic in the bone marrow.
excess dysfunctional platelets. dizziness, headaches, stroke, MI gangrene thrombosis.

treat with anegralide
or hydroxycarbamide.

46
Q

what causes the hepatosplenomegaly in myelofibrosis

A

extramedullary haematopoeisis

47
Q

What is seen on myelofibrosis investigations?

A

Blood film - tear drop poikilocytes and leukoerthyroblasts

bone marrow - dry tap

48
Q

What is the cause of myelofibrosis

A

may be idiopathic or occur following Polycythemia or essential thrombocythaemia.

49
Q

antibody found in autoimmune type 1 diabetes

A

Anti - GAD

50
Q

CD55 and CD59 are found in what disease

A

PNH

51
Q

what does CK vary with

A

exercise and race

52
Q

what does albumin vary with

A

posture

53
Q

Where is THC NOT found

A

saliva

54
Q

Name 3 diseases which come about through constant antigenic stimulation

A

EATL - coeliac
Parotid lymphoma - sjogren’s
Gastric carcinoma - h.pylori

55
Q

Name 3 diseases which come about through direct action and viral drive from infection rather than via antigenic stimulation

A

HTLV1 - adult t cell lymphoma
EBV - burkitts +?
HIV -

56
Q

What CD are b cells associated with?

A

19? often 20 used as rituximab is common therapy

57
Q

What CD are t cell lymphomas associated with

A

3+5

58
Q

what marker is often used in DLBCL staging

A

LDH, age

59
Q

t(8,14)

A

burkitts lymphoma

60
Q

t(15,17)

A

APML

61
Q

t(14,18)

A

follicular lymphoma

62
Q

t(11,18)

A

MALToma

63
Q

t(11,14)

A

mantle cell lymphoma

64
Q

Acanthocytes

A

also known as spurr cells

signify:
liver disease
hyposlenism
abetalipoproteinaemia

65
Q

What is basophilic RBC indicative of?

A

Increased rate of erythropoeisis or defective synthesis.

likely due to megaloblastic anaemia or myelodysplasia or LEAD POISINING

66
Q

Heinz bodies

A

inclusions within red blood cells which give rise to bite cells - G6PD

67
Q

Howell jolly body

A

basophilic staining of the cytoplasm and nuclear remnants in RBC

hyposlenism
sickle cell

68
Q

Leucoerythroblastic anaemia

A

nucleated RBCs and WBC’s in the periphery.

marrow infiltration i.e myelofibrosis/malignancy

69
Q

Pelger huet cells

A

hyposegmented neutrophils

myelodisplastic/leukaemia

70
Q

polychromasia

A

reticulocytosis - gey blue cells.

increases in haemolytic anaemia and decreases in aplastic anaemia or with chemotherapy

71
Q

rouleaoux

A

stacking

myeloma and paraproteinaemia

72
Q

spherocytes

A

round - usually hereditary spherocytosis or may be due to AIHA (warm)

73
Q

target cells

A

bulls eyes

HHH
hyposplenism
hepatic pathology
haemaglobinopathies.

74
Q

What is the normal range of anaemia in men and women

A

135 in men and 115 in women.

75
Q

What are the GI causes of bleefding

A

meckels diverticulum
peptic ulcers
polyps/cancers
hookworm

76
Q

What is the mechanism for anaemia of chronic disease?

A

normocytic

reduction of EPO - IL! TNF etc

reduction of iron transportation out of gut cells - IL6

77
Q

What is high in anaemia of chronic disease

A

ferritin - iron store

78
Q

what is a sideroblastic anaemia

A

iron cannot be correctly incorporated into the cells and so is dotteda round the place - occurs as part of myelodysplasia and genetic conditions

79
Q

what is the treatment for sideroblastic anaemia

A

remove the cause and give vitamin b6 to increase production

80
Q

What antibodies do perncicious anaemia sufferers have

A

anti parietal cell - 90
anti intrinsic factor - 50

schilling test but is outdated.

81
Q

Removal of what organs gievs youpernicious anaemia?

A

stomach removal or terminal ilium.

may be after fat or chrons surgery.
TROPICAL SPRUE

82
Q

what are the signs of pernicious anaemia

A

b12 def
glossitis angular cheilosis

irritability, depression psychosis

tingling neuropathy, spastic paraparesis and SACD spinal cord ABSENT ANKLE REFLEX

83
Q

What will make a b12 neuropathy worse

A

folic acid.

84
Q

What are the indications of intravascular hameolysis?

A

free plasma hb
haemagolbinurea
reduced haptoglobin
methaemaglobinaemia

85
Q

What are the indications of extravascular hameolysis

A

splenomegaly

86
Q

How do you recognise all haemolysis

A

unconjugated bilirubin goes up, urobilinogen goes up(clear excreted in urine and faeces)
LDH goes up

GALL STONES

87
Q

When do you get pencil cells

A

hereditary eliptocytosis but also in IDA

88
Q

When would you see spectrin mutations

A

both hereditary spherocytosis and eliptocytosis

89
Q

What are echinocytes

A 
burr cells (not spurr)
pyruvate kinase deficiency and EDTA change
90
Q

what goes down in HUS

A

platelets

91
Q

What does TXA2 do?

A

causes platelet aggregation

92
Q

what does a2 macroglobulin do?

A

inhibits 10

93
Q

what does uraemia do to platelets

A

reduces their function

94
Q

What are the main things associated with Haemophilia A

A

factor 8 deficiency

causes prolonged bleeding and haemarthroses

can have varying degrees of loss.

95
Q

What is VWf function in the blood stream?

A

to carry factor 8

when there is von willebrands disease they will have reduced factor 8 aswell as reduced platelt functioning which would be BLEEDING such as nosebleeds.

96
Q

what is more common haemophilia A or B

A

A is 5 times more common

x linked recessive so is in men

97
Q

how do you treat von willebrands

A

gove low purity factor 8 or desmopressin

98
Q

what is d dimer also known as

A

type of FDP.

99
Q

what factors are not made when there is liver disease?

A

2 5 7 9 10 11

platelts reduced function

Joshua's Path (9 decks)

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