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FINALS revision - @medicbotanist > Haematology > Flashcards

Haematology Flashcards

1
Q

What is polycythaemia?

A

A condition when you have too many RBC in the blood.

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2
Q

What is an example of true polycythaemia (primary)?

A

Myeloproliferative disorder i.e. rubra Vera polycythaemia

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3
Q

What is the gene that associated with rubra Vera polycythaemia?

A

Jack 2 gene

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4
Q

What is a cause of secondary true polycythaemia ?

A

Respiratory

CHD disease

Leads to tissue hypoxia and increased EPO release

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5
Q

What are some causes of apparent polycythaemia?

A

Dehydration

Diuretics

Alcohol consumption

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6
Q

What are

3 symptoms

3 signs of polycythaemia?

A

3 symptoms: red face, dizzy, headache, pruritus

3 signs: bleeding from mucosa, hepatomegaly and thrombosis of blood clots

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7
Q

What are the 2 things involved in polycythaemia treatment?

A
  1. Bone Marrow Suppression: chemotherapy

2. Aspirin: reduces the chance of clot formation

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8
Q

What factor is low in haemophilia A?

What would you see on blood tests in someone with haemophilia A ?

A

Factor 8

Low factor 8, prolonged APTT and PT time

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9
Q

How can haemophilia A present in neonates?

A
  1. Umbilical Cord bleeding
  2. Cephalohaematoma
  3. Iatrogenic bleeding

Later on you can soft tissue haematoma, joint bleeds and mucosal bleeds

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10
Q

How would you treat Haemophilia A

A. Long Term

B. Acutely

A

A. Factor 8 infusions

B. Desmopressin, Factor 8 and tranexamic acid

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11
Q

What is the factor which is low in Haemophilia B?

A

Factor 9

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12
Q

What process causes your reticulocytes to go down?

A

Anaemia

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13
Q

What two things cause reticulocytes levels to increase ?

A

Increased RBC destruction: sickle cell anaemia and haemolytic anaemia

Increased bone marrow activation

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14
Q

What would APTT and PT be like in a patient with VwF deficiency?

A

They would be normal

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15
Q

What is the purpose of VwF?

A

It stabilises Factor 8

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16
Q

What clotting factors does Vit K make?

A

2 7 9 and 10

Protein C and S

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17
Q

What will the APTT and PT appear like in a patient with Vit K deficiency

A

Prolonged APTT and PT

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18
Q

What are 5 causes of Iron deficiency anaemia?

A
  1. Hook worm
  2. Menstruation
  3. Coeliac and IBD
  4. Pregnancy
  5. Poor Diet
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19
Q

What are 3 symptoms and 3 signs of iron deficient anaemia?

A

3 symptoms: pallor, SOB, palpitations, pruritus and hair loss

3 signs: spoon shaped nails, dry hair, atrophic glottis and angular stomatitis

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20
Q

What will the blood tests in a patient with iron deficiency anaemia show?

A
  1. Low Ferritin
  2. Microcytic Anaemia
  3. High Transferrin
  4. High Total iron binding capacity
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21
Q

Macrocytic Anaemia:

What are 5 of causes of macrocytic anaemia?

A
  1. Pernicious Anaemia
  2. Vegan Diet
  3. Low Folate
  4. B12 deficiency
  5. Malabsorption
  6. Methotrexate and pregnancy
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22
Q

What are some symptoms of macrocytic anaemia that distinguish it from other anaemias?

A
  1. Peripheral Neuropathy
  2. Depression
  3. Weight Loss and SOB
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23
Q

What type of tongue will you have in low B12 or folate?

A

Smooth red and sore tongue

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24
Q

What investigations should you do in someone one you find out they have macrocytic anaemia?

A

Gastroscopy and look at anti-parietal cell antibodies

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25
Q

What are 3 causes of normocytic anaemia?

How do we treat it?

A

Haemorrhage, combined B12 and folate deficiency and chronic disease

Management: EPO enzyme

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26
Q

What is the inheritance of membranopathies?

A

Autosomal Dominant

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27
Q

What is the name given to a horizontal protein interaction?

What is the name given to a vertical protein interaction?

In relation to membranopathies?

A
  1. Elliptocytosis

2. Spherocytosis

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28
Q

What does parvo virus cause ?

A

Bone Marrow Supression

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29
Q

What is the inheritance pattern of Glucose 6 Phosphate Deficiency?

A

X linked inheritance

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30
Q

What are 3 triggers for someone with G6PD deficiency?

A

Broad Beans

Infections

Quinolines

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31
Q

How does G6PD deficiency present?

A

Jaundice

Haemolysis

Splenomegaly

Anaemia

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32
Q

When should you suspect G6PD deficiency?

A

In someone who has sudden onset jaundice, anaemia etc.

Around the same time as having an infection

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33
Q

What can we measure in the blood to confirm G6PD deficiency?

A

Measure the amount of NADPH

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34
Q

What is pernicious anaemia?

What is the average age of presentation?

What is it associated with?

A

Autoimmune disorder.

60

Other Autoimmune disorders

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35
Q

How does Pernicious Anaemia present?

A

It presents with fatigue, pallor and SOB. Proceeds to get Headaches, palpitations and jaundice.

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36
Q

What type of anaemia will you get in pernicious anaemia?

What antibody will be present in the blood?

A

Megloblastic anaemia

Intrinsic factor antibody

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37
Q

What is the management of someone with pernicious anaemia?

A

Folate and B12 supplements

Hydroxocobalamin

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38
Q

Will you get higher or lower values of reticulocytes in haemolytic anaemia?

A

Higher level of reticulocytes

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39
Q

What are the two types of haemolytic anaemia?

Explain both..

A

Intravascular: within the circulation

Extravascular: spleen, liver, bone marrow and reticuloendothelial system.

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40
Q

What will happen to lactate, urobilinogen and bilirubin and lactate dehydrogenase in Haemolytic anaemia?

A

They will all increase!!

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41
Q

Is intravascular haemolytic anaemia described as warm or cold?

What are some common causes?

What sign do you get ?

How do you manage the condition?

A

Intravascular haemolytic anaemia is described as cold

Common causes: lymphoma, autoimmune and drug reactions

You get very cold hands and feet

Management: prednisolone and blood transfusions/

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42
Q

Is extravascular haemolytic anaemia warm or cold?

What are some common causes of it?

How do you manage it?

A

Warm

Cancer, HIV and UC

Prednisolone and splenectomy

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43
Q

What are some inherited conditions that are associated with haemolytic anaemia?

A

G6PD

Sickle Cell

Thalassaemia

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44
Q

What are two drugs that can cause acquired haemolytic anaemia?

A

Penicillin

Quinine

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45
Q

How do you diagnose haemolytic anaemia? 4 ways

A

Thin and Thick Blood Film: rules out malaria

Auto antibody test: direct Coombs test

FBC

Reticulocytes, LDH, lactate dehydrogenase, bilirubin and urobilinogen will all be raised.

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46
Q

What is the management of sickle cell anaemia?

A

Hydroxycarbamide

Give blood transfusions

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47
Q

How do you diagnose Sickle Cell anaemia?

A

Hb electrophoresis

Blood film

FBC: microcytic anaemia

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48
Q

What are the 3 different types of Alpha Thalassaemia?

A

Four gene deletion: not compatible with life can cause hydrops fetalis

Three gene deletion: moderate splenomegaly and anaemia

Two gene deletion: with or without mild anaemia

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49
Q

What is the treatment for alpha thalassaemia?

A

Blood transfusions and Iron chelating agents via ascorbic acid

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50
Q

What is the inheritance of thalassaemia?

A

Autosomal Recessive

51
Q

What are the 3 different types of Beta Thalassaemia? Describe the presentation of each:

A

Beta minor: mild microcytic anaemia

Beta intermedia: moderate anaemia, splenomegaly, recurrent leg ulcers and infections

Beta major: failure to thrive. Presents in first year of life. Severe anaemia, hepatosplenomegaly, extra medullary haematopoesis

52
Q

How do you investigate thalassaemia?

A

Haemoglobin electrophoresis

53
Q

What is the 4 step management of a patient in a sickle cell crisis?

A
  1. IV fluids + IV pain relief
  2. Oxygen
  3. Broad spectrum abx and a blood transfusion
54
Q

What is hyperviscosity?

How can it present?

How do you manage it?

A

Hyper viscosity: is when you get too much Ig M in the blood

Cause: myeloma

Symptoms: headaches, SOB and blurred vision, fatigue and mucosal bleeding

Management: fluids and on call haematologist

55
Q

How long do platelets last for ?

A

7 to 10 days

56
Q

What controls platelet formation? 2 things

A

Thrombopoieten

Stimulates Megakaryocytes stimulates the release of platelets

57
Q

What is released by platelet alpha granules?

A

VwF

Fibrinogen

PDGF

58
Q

What is released by platelet dense granules?

A

Serotonin

Ca2+

59
Q

What does thromboxane synthesis?

A

COX-1

60
Q

What is an inhibitor of GPIIb/IIIa ?

A

Tirofiban

61
Q

What are 3 examples of substances that lead to reduced platelet production?

A

Aplastic anaemia

Alcohol

Chemo

Low B12

62
Q

What are 3 causes of increased platelet destruction?

A

Hyper spleen

DIC

Warfarin

63
Q

What are the two treatment options for aplastic anaemia ?

A

Immunosupression

Bone Marrow Transplant

64
Q

What are 3 conditions that lead to the destruction of platelets via consumption?

A
  1. DIC
  2. TTP
  3. Haemolytic Uraemic Syndrome
65
Q

Who is thrombotic thrombocytopenia purpura more common in- males or females?

What are two main causes of it?

A

Females in 40s +

Two main causes: postpartum, cancer and congenital

66
Q

What are the 3 pathophysiological steps that make up thrombotic thrombocytopenic purpura?

A

Small (thrombotic) clots in the vessels

Low platelets and

Red Blood Cell Destruction.

67
Q

What gene is associated with TTP?

A

ADAMTS13

68
Q

What are 5 ways that TTP can present?

A

Purple Petechiae Bruises and Thrombocytopenia

Renal impairment

Haemolytic anaemia

Fever

Abdominal and chest pain

Confusion/Headache

69
Q

What levels will be high in TTP?

A

High reticulocytes

High bilirubin

High LDH

70
Q

What is the 4 step management of TTP?

A
  1. Full plasma tranfusion
  2. High Dose Steroids
  3. Monoclonal Antibody
  4. Splenectomy
71
Q

Idiopathic Thrombocytopenic Purpura:

What are the auto antibodies against ?

A

Glycoproteins IIb/IIIa

72
Q

When does Idiopathic Thrombocytopenic Purpura present?

A. Children

B. Adults

A

A. Normally 2 weeks post viral infection. Common in those 5-7 yrs old

B. Autoimmune related, HIV or Hep C

73
Q

What viruses will you want to test in a patient with ITP?

A

Hep C

HIV

74
Q

How do you treat ITP in:

  1. Children
  2. Adults
A
  1. Prednisolone and platelet infusions

2. IV immunoglobulin and IV anti D

75
Q

How does ITP present?

A
  1. Bruising
  2. Nose Bleeds
  3. Gastric Bleeds + pain
  4. Heavy Periods
76
Q

What are 3 causes of DIC?

A

Sepsis

Complications of pregnancy: pre eclampsia or placental abruption

Malignancy: leukaemia or Cancer of pancreas, lung or prostate

77
Q

What are 5 signs of DIC ?

A

Petechiae and Necrosis of the tissue

Bleeding

Fever

Signs of haemorrhage: low BP and sweaty

Confusion

78
Q

What are blast cells?

A

Non functioning cells that build up in the bone marrow

79
Q

What are the two ways you can classify Acute Lymphoblastic Leukaemia (ALL)?

A
  1. FAB system.

2. Immunological

80
Q

What are 4 ways that ALL normally presents?

A
  1. Bilateral parotid infiltration
  2. Anaemia
  3. Infection
  4. Bleeding
81
Q

What investigations would you like to do in a patient with suspected ALL?

A

FBC

Blood Film

Bone Marrow Biopsy

CXR and CT to look for lymphadenopathy

LP to check for CNS involvement

82
Q

How do you treat ALL?

A

Blood and Platelet transfusions

Allopurinol and IV fluids to prevent tumour lysis syndrome

Chemotherapy: IV and into the spine

Prophylactic: anti- virals, antibacterials and anti-fungal

Bone Marrow transplantation

83
Q

Who is AML more common in?

A

Adults

84
Q

Which version of leukaemia do you get CNS involvement in?

A

ALL

85
Q

What is the common presentation of AML leukaemia?

A

Gum hypertrophy

Bruising, anaemia and infection

Hepatomegaly

86
Q

What are people with AML prone to get? ( 2 things)

A
  1. DIC

2. Rash

87
Q

What leukaemia do you get Auer Rods seen on Blood film in?

A

Acute Myeloid Leukaemia (AML)

88
Q

What form of leukaemia is associated with the Philadelphia chromosome?

A

Chronic Myeloid Leukaemia (CML)

89
Q

What is the most common form of Leukaemia?

A

Chronic Lymphocytic Leukaemia (CLL)

90
Q

What age is CLL common in?

A

60-70s

More common in men

91
Q

How does CLL normally present?

A

Normally asymptomatic and just appears on routine FBC

However, it can also present with enlarged rubbery or tender nodes with hepato or splenomegaly

92
Q

Describe the categorisation of CLL : i.e. Stage A B and C

A

Stage A no anaemia or thrombocytopenia Less than 3 enlarged nodes

Stage B no anaemia or thrombocytopenia. More than 3 enlarged nodes

Stage C anaemia or thrombocytopenia. Doesn’t matter about enlarged nodes.

93
Q

How do you treat CLL?

A

Stage A: watch and wait

Stage B and Stage C: treat with chemotherapy and alkylating agents

94
Q

Is haemolytic anaemia more common in CML or CLL ?

A

CLL need to fo a direct Coombs test

95
Q

How does CML present?

A

Very much like lymphoma:

Fever, Fatigue. Sweats. Weight loss

Chronic and insidious

Anaemia, hepatomegaly and bruising

96
Q

What is the management of CML?

A

Tyrosine kinase inhibitor (imatinib)

Allogenic stem cell transplant

97
Q

What is Richter’s Transformation?

A

This is the progression of CLL to diffuse Large Cell B Lymphoma

Rule of 3s

1/3 never progress
1/3 progress with time
1/3 actively progress

98
Q

What form of lymphoma are reed sternberg cells associated with?

A

Hodgkin’s Lymphoma

99
Q

What is the Ann Arbor classification used to stage?

A

Hodgkin’s Lymphoma

100
Q

What age groups is Hodgkin’s Lymphoma associated with?

A

It has two peaks

One peak: 20-35

Second peak- 50-70s

101
Q

What are 3 RF of Hodgkin’s lymphoma?

A

Primary immunodeficiency: SLE
Secondary immunodeficiency: HIV or transplant

EBV virus

102
Q

Which lymphoma is associated with an increased risk of SVOC?

A

Hodgkin’s Lymphoma

103
Q

How does Hodgkin’s lymphoma present?

A

Enlarged rubbery non painful lymph nodes

Alcohol induced lymph node pain

Weight loss, fever and night sweats

SVCO

104
Q

Explain the 4 stages of the Ann Arbor Classification:

A

1: confined to one lymph node
2. >2 lymph nodes same diaphragm side

  1. > 2 lymph nodes on different diaphragm sides
  2. Spread beyond lymph nodes

A or B (presence of weight loss + other systemic symptoms)

105
Q

How do you manage Hodgkin’s Lymphoma?

A

Radiotherapy

Chemotherapy in those in stage 2 or higher or those with B symptoms + also give Rituximab

106
Q

What leukaemia is associated with non Hodgkin’s lymphoma?

A

Chronic LL

107
Q

Is non Hodgkin’s lymphoma more common in males or females?

What age does it typically present?

A

Males

65-75 yrs old

108
Q

What virus is associated with non hodgkins associated with?

A

EBV

109
Q

What is the difference between

Low Grade Follicular Non Hodgkin’s Lymphoma and

High Grade Diffuse Non Hodgkin’s lymphoma ?

A

Low grade: low growth and asymptomatic

High Grade: high growth rate and symptoms like weight loss, fever, night sweats

110
Q

Describe the course of disease for low grade and high grade non Hodgkin’s lymphoma..

A
  1. Low Grade: common to get relapses. Normally do nothing. Treatment if you do is with rituximab and chemo
  2. High Grade: need to treat! With CHOP (chemo + pred), radiotherapy, rituximab and a stem cell transplant.
111
Q

What type of proteins are paraproteins?

A

M proteins

112
Q

What is MGUS?

A

Gammopathy of the blood with no symptoms

Present in those over 50

High chance of developing myeloma

113
Q

How would you tell myeloma if you did electrophoresis?

A
  1. Large M spike

2. Lots of Ig M and Ig G

114
Q

When does myeloma become multiple myeloma?

A

When it affects the bones

115
Q

What is the pathophysiology of myeloma?

A

You get malignant cells in the bone marrow. Causes low RBC

Stimulates osteoclasts activity. Hypercalcaemia and lytic lesions. Can lead to spinal cord compression

Increased paraprotein leads to hyper-viscosity and can cause kidney damage.

Increased

116
Q

What diagnostic tests would you do on myeloma?

A

Serum electrophoresis: show M spike, high Ig M and Ig G and Bence Jones Proteins

Blood Film: rouleaux of RBC

Skeletal Survery

Bone Marrow Biopsy

FBC

117
Q

How do you treat myeloma?

A
  1. Chemotherapy
  2. Radiotherapy
  3. Bisphosphonates
  4. Plasmapheresis
  5. Thalidomide: anti angiotensin effects
118
Q

What clinical presentation would you get with a patient who has P Vivax and P OVALE ?

A

Slowly developed anaemia and tender hepatomegaly

119
Q

What symptoms would you get with P Malariae?

A

Mild Symptoms but for a longer amount of time

Malaise, Headache , Vomiting and Diarrhoea

120
Q

How would P Falciparum present?

A
  1. Less marked fever
  2. Cerebral oedema, causes confusions, convulsions and coma
  3. Biggest cause of death
121
Q

What test would you want to do in someone with suspected malaria ?

A

Thin and Thick Blood film

122
Q

What is a good abx prophylactic for malaria?

A

Doxycycline

Chloroquine

123
Q

How would treat a complicated form of malaria?

A

IV quinine dihydrochloride

124
Q

What is the treatment for uncomplicated malaria ?

A

ORAL quinine + doxycycline

FINALS revision - @medicbotanist (20 decks)

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