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Haematology Flashcards

1
Q

What is the Hb threshold for anaemia in men? (with units)

A

135 g/L

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2
Q

What is the Hb threshold for anaemia in women? (with units)

A

115g/L

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3
Q

What conditions are microcytic anaemias associated with?

A

FAST:

Fe-deficiency anaemia
Anaemia of chronic disease
Sideroblastic anaemia
Thalassaemia

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4
Q

What conditions are normocytic anaemia associated with?

A

HABHARP

Hypothyroidism
Acute blood loss
Bone marrow failure
Haemolysis
Anaemia of chronic disease
Renal Failure
Pregnancy
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5
Q

What conditions are macrocytic anaemia associated with?

A

HABARM

Hypothyroidism
Antifolate drugs (e.g. phenytoin)
B12 or folate deficiency
Alcohol excess or liver disease
Reticulocytosis
Myelodysplastic syndromes
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6
Q

What symptoms may somebody with anaemia complain of?

A 
Fatigue
Faintness
Headache
Tinnitus
Dyspnoea
Palpitations
Anorexia
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7
Q

What signs are associated with Iron-deficiency anaemia?

A 
Koilonychia
Brittle hair and nails
Angular cheilosis
Atrophic glossitis
Post-cricoid webs (Plummer-Vinson syndrome)
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8
Q

How can you categorise the causes of iron-deficiency anaemia?

A 
Decreased intake
Decreased absorption
Increased utilisation
Blood loss
Intravascular haemolysis
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9
Q

Causes of iron-defiency

A

Prematurity, infants/children/elderly (decreased intake)
Coeliac, post-gastric surgery (decreased absorption)
Pregnancy, infants/children during growth (increased utilisation)
Gastrointestinal loss (blood loss)
Haemolytic anaemia (intravascular haemolysis)

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10
Q

What conditions are microcytic anaemias associated with?

A

FAST:

Fe-deficiency anaemia
Anaemia of chronic disease
Sideroblastic anaemia
Thalassaemia

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11
Q

What conditions are normocytic anaemia associated with?

A

HABHARP

Hypothyroidism
Acute blood loss
Bone marrow failure
Haemolysis
Anaemia of chronic disease
Renal Failure
Pregnancy
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12
Q

What conditions are macrocytic anaemia associated with?

A

HABARM

Hypothyroidism
Antifolate drugs (e.g. phenytoin)
B12 or folate deficiency
Alcohol excess or liver disease
Reticulocytosis
Myelodysplastic syndromes
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13
Q

What symptoms may somebody with anaemia complain of?

A 
Fatigue
Faintness
Headache
Tinnitus
Dyspnoea
Palpitations
Anorexia
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14
Q

What signs are associated with Iron-deficiency anaemia?

A 
Koilonychia
Brittle hair and nails
Angular cheilosis
Atrophic glossitis
Post-cricoid webs (Plummer-Vinson syndrome)
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15
Q 
BLOOD FILM:
Microcytic
Hypochromic
Anisocytosis
Poikilocytosis (shape)
Pencil cells
A

Iron-deficiency anaemia

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16
Q

How can you categorise the causes of iron-deficiency anaemia?

A 
Decreased intake
Decreased absorption
Increased utilisation
Blood loss
Intravascular haemolysis
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17
Q

Causes of iron-defiency

A

Prematurity, infants/children/elderly (decreased intake)
Coeliac, post-gastric surgery (decreased absorption)
Pregnancy, infants/children during growth (increased utilisation)
Gastrointestinal loss (blood loss)
Haemolytic anaemia (intravascular haemolysis)

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18
Q

What happens to ferritin in anaemia of chronic disease?

A

It is raised

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19
Q

What causes anaemia of chronic disease in renal failure?

A

EPO deficiency

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20
Q

Which cytokine is raised in anaemia of chronic disease and what effect does this have on the body?

A

IL-6 is raised

leads to increased hepcidin production and release from the liver … leads to reduced ferroportin (therefore less iron access in the circulation)

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21
Q

What would you look for when diagnosing sideroblastic anaemia?

A

Ringed sideroblasts

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22
Q

Causes of sideroblastic anaemia?

A 
myelodysplastic disorders
myeloproliferative disease
post-chemotherapy
irradiation
alcohol excess
lead excess
anti-TB drugs
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23
Q

One way of treating sideroblastic anaemia?

A

Pyridoxine (vitamin B6 which promotes RBC production)

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24
Q

Iron: low
TIBC: raised
Ferritin: low

A

Iron deficiency

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25
Q

Iron: low
TIBC: low
Ferritin: raised

A

Anaemia of chronic disease

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26
Q

Iron: raised
TIBC: low
Ferritin: raised

A

Chronic haemolysis

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27
Q

Iron: raised
TIBC: low/normal
Ferritin: raised

A

Haemochromatosis

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28
Q

Iron: normal
TIBC: raised
Ferritin: normal

A

Pregnancy

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29
Q

Iron: raised
TIBC: normal
Ferritin: raised

A

Sideroblastic anaemia

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30
Q

What might you see on a megaloblastic blood film?

A

Hypersegmented polymorphs
Leucopenia
Macrocytosis
Thrombocytopenia

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31
Q

What are two causes of vitamin B12 deficiency?

A

Dietary (e.g. vegan)
Malabsorption:
1) STOMACH: Lack of intrinsic factor produced by gastric parietal cells leads to pernicious anaemia OR post-gastrectomy
2) Terminal ileum due to ileal resection, Crohn’s, bacterial overgrowth, tropical sprue, tapeworms

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32
Q

What test might you perform for pernicious anaemia?

A

Parietal cell antibodies
Intrinsic factor antibodies
Schilling test

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33
Q

How do you treat vitamin B12 deficiency?

A

IM hydroxycobalamin

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34
Q

What dietary sources contain folate?

A

Green veg
Nuts
Yeast
Liver

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35
Q

Causes of folate deficiency? and how do you treat?

A

Dietary
Increased demand: Pregnancy or increased cell turnover (e.g. haemolysis, malignancy, inflammatory disease, renal dialysis)
Malabsorption, e.g. coeliac, tropical sprue
Drugs: alcohol, anti-epileptics, methotrexate, trimethoprim

Give oral folic acid

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36
Q

What cluster of lab findings might make you suspect a haemolytic anaemia?

A

Increased bilirubin (unconjugated)
Increased urobilinogen
Increased LDH
Reticulocytosis (increased MCV and polychromasia)

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37
Q

What signs and symptoms might indicate a haemolytic anaemia specifically?

A

All those associated with anaemia plus

Dark red urine (haemogloburinia)
Jaundice

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38
Q

In terms of haemolytic anaemia what does ‘intrinsic’ and ‘extrinsic’ mean?

A

Intrinsic: factors related to the RBC
Extrinsic: factors outside of the RBC

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39
Q

What intrinsic causes of haemolytic anaemia are there? and give two examples of each.

A

Membrane defect: hereditary spherocytosis; hereditary elliptocytosis.
Enzyme defect: G6PD deficiency; Pyruvate kinase deficiency
Haemoglobinopathies: Sickle Cell Disease; Thalassaemias

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40
Q

What extrinsic causes or haemolytic anaemias are there and give examples of each.

A

Immune: Autoimmune (hot/cold); Alloimmune (haemolytic transfusion reactions).

Non-immune: Mechanical (metal heart valves, trauma); Paroxysmal nocturnal haemoglobinuria; Infections/Drugs

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41
Q

What is heparin’s mechanism of action?

A

Potentiates antithrombin III which then inactivates thrombin and other clotting factors (e.g. 10a)

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42
Q

What is the antidote for heparin?

A

Protamine sulfate

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43
Q

What are the clinical side-effects of heparin use? What might you see on lab results?

A

Heparin-induced-thrombocytopenia
Alopecia (rare!)
Osteoporosis (rare!)

Lab results:
elevated aminotransferase
hyperkalaemia

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44
Q

What is warfarin’s mechanism of action and what factors/proteins does this affect?

A

Vitamin K antagonist

Inhibition of factors 2, 7, 9 and 10
Inhibition of proteins C, S and Z

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45
Q

How might you reverse warfarin overdose?

A

IV vitamin K

Prothrombin complex concentrates (FII, VII, IX, X and Protein C and S)

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46
Q

In general what does a high INR put you at risk of? and a low INR?

A

High INR: High risk of bleeding

Low INR: High risk of clot formation

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47
Q

Who are more likely to develop ALL: children or adults?

A

Children

48
Q

What are the clinical features of ALL?

A

Bone Marrow Failure:
Anaemia
Thrombocytopenia (bleeding)
Neutropenia (infections)

Lymphadenopathy
Organ involvement (hepatomegaly, splenomegaly, CNS)
49
Q

What are you looking for in the blood of an ALL patient?

A

High WCC

Blast cells, lymphoblasts

50
Q

What is the aim of ‘remission induction’ chemotherapy? What agents are usually used?

A

Kill most tumour cells and get patient into remission (less than 5% of leukemic blasts in bone marrow, normal blood cells in blood and absence of other signs/symptoms).

Agents: 
Steroid (dexamethasone/prednisolone)
Vincristine
Asparaginase (better for paeds)
Daunorubicin (better in adults)
51
Q

What is the aim of ‘consolidation’ chemotherapy?

A

High dose multi-drug chemotherapy

Can target the CNS too

52
Q

What is the aim of ‘maintenance’ chemotherapy?

A

Kill off any remaining tumour cells that were not killed by the remission induction/consolidation phases, otherwise the patient may relapse.

53
Q

Auer rods are associated with which condition?

A

Acute Myeloid Leukaemia

54
Q

What investigation can you perform to distinguish AML from ALL?

A

Myeloperoxidase and Sudan black stains

55
Q

What signs/symptoms can be found in chronic myeloid leukaemia?

A

Fever
Bone pain
Splenomegaly

56
Q

You perform a blood count in a patient you suspect has CML. What are you looking for?

A

Elevated granulocytes: eosinophils; basophils; mast cells; neutrophils.

57
Q

What is the Philadelphia chromosome? What does it give rise to?

A

Abnormal chromosome 22. The result of a chromosomal translocation (9;22)

Philadelphia chromosome results in the BCR-ABL fusion gene.

58
Q

What are the three phases in CML and what order do they typically occur in?

A
  1. Chronic Phase
  2. Accelerated Phase
  3. Blast Crisis
59
Q

What is the first phase of CML? What signs and symptoms may the patient present with? What is the treatment in the first phase?

A

Chronic Phase.

Asymptomatic/mild symptoms
Fatigue
Left side pain
joint/hip pain
Abdominal fullness

Treatment:
Imatinib (a BCR-ABL tyrosine kinase inhibitor)
or, if resistant to imatinib, can use dasatinib/nilotinib.

60
Q

What is the second phase of CML? What signs/symptoms/lab findings might there be?

A

Accelerated phase

Incrasing splenomegaly 
Unresponsive to therapy
10-19% myeloblasts in blood/bone marrow
>20% basophils in the BM/blood
Platelets really high, or really low.
61
Q

What is the third and final phase of CML? What signs/symptoms/lab findings might there be?

A

Blast crisis.

> 20% myeloblasts/lymphoblasts in the blood/BM
Development of a chloroma (solid focus of leukaemia outside of the bone marrow)

62
Q

What condition is Binet Staging used in? How many stages are there and what are the features of each stage?

A

Chronic Lymphocytic Leukaemia

3 stages: A, B and C.
A: Less than 3 groups of enlarged lymph nodes and a raised WCC

B: Greater than 3 groups of enlarged lymph nodes and a raised WCC

C: Enlarged lymph nodes or spleen, raised WCC, low RBC/platelets.

63
Q

What condition is Rai Staging used in?

A

Chronic Lymphocytic Leukaemia

64
Q

Name some agents used in the treatment of chronic lymphocytic leukaemia.

A

Chlorambucil (nitrogen mustard alkylating agent)
Fludarabine (purine analog that interferes with DNA synthesis)
Alemtuzumab (monoclonal antibody, anti-CD52)

65
Q

Who is affected more in Hodgkin’s lymphoma … Males or Females?

A

Males.

66
Q

What age of incidence does Hodgkin’s lymphoma tend to have?

A

bimodal age incidence.

20-29 year olds
Greater than 60 year olds

67
Q

EBV is associated with what haematological condition?

A

Hodgkin’s lymphoma

68
Q

A patient presents to you complaining of a fever that gets worse and then eases off over a period of one or two weeks. What clinical sign is this and what condition is it associated with? How can you treat it?

A

Pel-Ebstein fever (1-2week, cyclical)

Associated with Hodgkin’s lymphoma (can get it in other conditions but then it isn’t called Pel-Ebstein)

Treat it with NSAIDs or treat the underlying Hodgkin’s.

69
Q

What effect might alcohol have on a patient with Hodgkin’s lymphoma?

A

Sudden development of pain in affected node after consuming alcohol.

70
Q

Describe a fibrin ring granuloma and what conditions it may appear in.

A

A central lipid vacuole surrounded by a dense fibrin ring and epithelioid macrophages.

You might see it in Q Fever, Hodgkin’s lymphoma and infectious mononucleosis.

71
Q

Over a game of jenga your friend asks you what cells are binucleated with prominent nucleoli and a CD30+, CD15+ immunophenotype? What condition are they associated with?

A

Reed-Sternberg Cells

Associated with Hodgkin’s lymphoma.

72
Q

Describe the different components of the Ann Arbor staging classification.

A

Stage 1: involvement of a single lymph node region … or single extralymphatic site

Stage 2: involvement of two or more LN regions on the SAME SIDE of the diaphragm … or one LN region and a contiguous extralymphatic site

Stage 3: LN regions on both sides of the diaphragm.

Stage 4: disseminated involvement of one or more extralymphatic organs.

‘A’=lack of systemic symptoms
‘B’=presence of systemic symptoms
‘S’=splenic involvement
‘X’=bulky disease

73
Q

What four agents are used in the treatment of Hodgkin’s lymphoma?

A

Adriamycin
Bleomycin
Vinblastine
Dacarbazine

74
Q

What risks are associated with allogeneic stem cell transplant versus autologous?

A

Allogeneic: Graft versus Host Disease risk, opportunistic infections, infertility, secondary malignancies.

75
Q

Patient with lymphoma, they drink alcohol, they don’t get pain. Do they have Hodgkin’s or non-Hodgkin’s?

A

Hodgkin’s

76
Q

What is Richter’s transformation?

A

B-cell CLL and hairy cell leukaemias convert into a diffuse large B-cell lymphoma.

77
Q

Mantle cell lymphoma is due to what chromosomal abnormality and what does this result in?

A

t(11:14) translocation … over-expression of cyclin D1

78
Q

What organism is MALT lymphoma associated with?

A

H. Pylori

79
Q

What happens in Sjogren’s syndrome?

A

WCC destruction of exocrine glands like salivary and lacrimal.

80
Q

What are the three types of Burkitt’s lymphoma?

A

Endemic
Sporadic
Immuno-deficiency

81
Q

What is sideroblastic anaemia?

A

BM produces ringed sideroblasts instead of healthy red cells.
Unable to incorporate iron into the haemoglobin.

82
Q

What are the clinical features of Multiple Myeloma?

A

CRAB

Calcium high - thirst, moans, groans, stones, bones
Renal failure (plus amyloidosis and nephrotic syndrome)
Anaemia (+panytopenia)
Bones - pain, osteoporosis, osteolytic lesions, fractures

+hyperviscosity syndrome

83
Q

The Durie-Salmon staging system is used in what condition?

A

Multiple Myeloma

84
Q

The presence of Bence-Jones protein in the urine is indicative of what conditions?

A

Multiple Myeloma

Woldenstrom’s macroglobunlinaemia

85
Q

What investigations do you want to perform in a multiple myeloma patient?

A

Serum electrophoresis - see a dense narrow band

Blood film - can see rouleaux
Urinalysis
Blood test - ESR will be high

86
Q

What are the three management categories in multiple myeloma?

A

Supportive for CRAB symptoms
Chemotherapy combinations
Steroids (dex or pred)

87
Q

Patients with monoclonal gammaglobinopathy of Unknown Significance are at increased risk of developing what condition?

A

Multiple Myeloma

88
Q

What are the three features of smouldering myeloma?

A

Greater than 30 g/L paraprotein
Less than 10% plasma cells in BM
No CRAB symptoms

89
Q

What antibody is attributed to Waldenstrom’s Macroglobulinaemia?

A

IgM

90
Q

What stain is useful in diagnosing amyloidosis?

A

Congro-Red stain will appear apple-green under polarized light on microscopy.

91
Q

In what patient population is Fanconi Anaemia more frequently found in?

A

Ashkenazi Jewish

Afrikaners in S.A.

92
Q

What age does Fanconi Anaemia usually present at?

A

5-10 years

93
Q

What are signs/symptoms of Fanconi Anaemia?

A

‘FANCONIS’

Facial microcephaly
Abnormal testis n kidneys
Neutropenia
Cafe au lait
Ocular abnormality
No thumbs n radius
Increased risk of AML
Skin abnormality
94
Q

What condition are people with Fanconi’s at greater risk of progressing to?

A

Acute Myelogenous Leukaemia

95
Q

What triad of physical clinical features are characterisitic of Dyskeratosis Congenita?

A

Skin Pigementation
Nail dystrophy
Oral Leukoplakia

96
Q

What type of inheritance pattern does Dyskeratosis Congenita have?

A

X-linked

97
Q

What type of inheritance pattern does Schwachman-Diamond Syndrome have?

A

Autosomal recessive

98
Q

What signs/symptoms does Schwachman-Diamond usually exhibit?

A

SCHwachman

Short stature
Cells of the blood (neutropenia)
Hepatic impairment

Pancreatic dysfunction

99
Q

People with Schwachman-Diamond Syndrome are at greater risk of progressing to what condition?

A

Acute Myelogenous Leukaemia

100
Q

When does Diamond-Blackfan Syndrome usually develop?

A

Neonatal/1year

101
Q

What do you expect the MCV to be in Diamon-Blackfan?

A

Normal or micro

102
Q

What signs/symptoms do you expect to see in Diamond-Blackfan Syndrome?

A

Craniofacial malformations
Thumb/upper limb abnormalities
Urogenital malformations
Cleft palate

103
Q

The Philadelphia Chromosome is associated with what condition?

A

Chronic Myeloid Leukaemia (CML)

104
Q

What are common symptoms of Polycythaemia Rubra Vera?

A

PRV

Pruritis
Ringing in ears
Visual blurriness

105
Q

What is a Pseudo Polycythaemia?

A

Red cell mass is normal but plasma volume is reduced

106
Q

What can cause a pseudopolycythaemia?

A

‘Fire’
Burns, Cigarette Smoking

‘Water’
Dehydration, vomiting, diarrhoea

107
Q

What might you see on blood results in a patient with polycythaemia rubra vera? and in a secondary PRV?

A

Raised Hb
Raised hct
Raised platelets
Raised WCCs

secondary: only raised red cells

108
Q

What happens to the bone in myelofibrosis?

A

Fibrosis of the bone marrow

109
Q

Signs/Symptoms of myelofibrosis?

A 
Hepatomegaly
Splenomegaly
Weight Loss
Fever
Bone Pain
110
Q

What type of cells are in the bone marrow and what do they do in Essential Thrombocythaemia?

A

Megakaryocytes dominate in the BM. They produce a lot of platelets.

111
Q

What are people with essential thrombocythaemia at increased risk of?

A

venous/arterial thrombosis

haemorrhages

112
Q

Which Ig can cross the placenta?

A

IgG

113
Q

At how many weeks do we usually administer antiD Ig to pregnant women?

A

28 and 34 weeks

114
Q

What are some sensitising events during pregnancy that we should be aware of with regards to rhesus status?

A 
Abortion
Miscarriage
Abdominal trauma
External cephalic version 
amniocentesis
115
Q

What are some acute (

A

FITATAT

Febrile non-haemolytic
Infection (bacterial)
Transfusion associated circulatory overload (TACO)
Allergic/anaphylaxis
Transfusion related Acute Lung Injury (TRALI)
Acute haemolytic
Transfusion associated dyspnoea (TAD)

116
Q

What are some delayed (>24 hours) adverse reactions to blood transfusion?

A 
Antibody formation
Delayed haemolytic
Infection (viral, malaria)
TA-GvHD
Post transfusion purpura

Pathology (5 decks)

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