Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

Cancer Care > Haematology > Flashcards

Haematology Flashcards

1
Q

What cells do lymphoid stem cells give rise to?

A

B cells
T cells
NK cells

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

What cells do myeloblasts give rise to?

A

Basophils
Neutrophils
Eosinophils
Macrophages

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

What is the progenitor cell for platelets?

A

Megakaryocytes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

When would you find Anisocytosis on blood film?

A

refers to a variation in size of the red blood cells. These can be seen in myelodysplasic syndrome as well as some forms of anaemia.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

When would you find target cells on blood film?

A

Target cells have a central pigmented area, surrounded by a pale area, surrounded by a ring of thicker cytoplasm on the outside. This makes it look like a bull’s eye target. These can be seen in iron deficiency anaemia, liver disease, haemaglobinopathies and post-splenectomy.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

When would you find Heinz bodies on blood film?

A

Heinz Bodies are individual blobs seen inside red blood cells caused by denatured globin. They can be seen in G6PD and alpha-thalassaemia.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

When would you find Howell-Jolly bodies on blood film?

A

Howell-Jolly bodies are individual blobs of DNA material seen inside red blood cells. Normally this DNA material is removed by the spleen during circulation of red blood cells. They can be seen in post-splenectomy and in patients with severe anaemia where the body is regenerating red blood cells quickly.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

When would you find reticulocytes on blood film?

A

Reticulocytes are immature red blood cells that are slightly larger than standard erythrocytes (RBCs) and still have RNA material in them. This percentage goes up where there is rapid turnover of red blood cells, such as haemolytic anaemia. They demonstrate that the bone marrow is active in replacing lost cells.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

When would you find schistocytes on blood film?

A

Schistocytes are fragments of red blood cells. They indicate the red blood cells are being physically damaged by trauma during their journey through the blood vessels. They may indicate networks of clots in small blood vessels caused by haemolytic uraemic syndrome, disseminated intravascular coagulation (DIC) or thrombotic thrombocytopenia purpura. They can also be present in replacement metallic heart valves and haemolytic anaemia.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

When do you find Sideroblasts on blood film?

A

Sideroblasts are immature red blood cells that contain blobs of iron. They occur when the bone marrow is unable to incorporate iron into the haemoglobin molecules. They can indicate a myelodysplasic syndrome.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

When do you find smudge cells on blood film?

A

Smudge cells are ruptured white blood cells that occur during the process of preparing the blood film due to aged or fragile white blood cells. They can indicate chronic lymphocytic leukaemia.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

When do you find spherocytes on blood film?

A

Spherocytes are spherical red blood cells without the normal bi-concave disk space. They can indicated autoimmune haemolytic anaemia or hereditary spherocytosis.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Causes of microcytic anaemia

A

TAILS

T – Thalassaemia
A – Anaemia of chronic disease
I – Iron deficiency anaemia
L – Lead poisoning
S – Sideroblastic anaemia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Causes of normocytic anaemia

A

There are 3 As and 2 Hs for normocytic anaemia:

A – Acute blood loss
A – Anaemia of Chronic Disease
A – Aplastic Anaemia
H – Haemolytic Anaemia
H – Hypothyroidism

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Causes of macrocytic anaemic

A

Megaloblastic anaemia is caused by:

B12 deficiency
Folate deficiency

Normoblastic macrocytic anaemia is caused by:

Alcohol
Reticulocytosis (usually from haemolytic anaemia or blood loss)
Hypothyroidism
Liver disease
Drugs such as azathioprine

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Signs of anaemia

A

Tiredness
Shortness of breath
Headaches
Dizziness
Palpitations
Worsening of other conditions such as angina, heart failure or peripheral vascular disease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

What type of anaemia would Koilonychia indicate?

A

Iron deficiency

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

What type of anaemia would Angular stomatitis indicate?

A

Iron deficiency

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

What type of anaemia would Atrophic glossitis indicate?

A

Iron deficiency

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

What type of anaemia would Jaundice indicate?

A

Haemolytic anaemia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

What type of anaemia would bone deformities indicate?

A

Thalassaemia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

What test would you do to investigate anaemia?

A

Hb
MCV
B12
Folate
Ferritin
Blood film

Further tests:
- OGD
- Bone marrow biopsy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

Where is iron absorbed?

A

Duodenum and jejunum

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

Management of new iron deficiency anaemia

A

New iron deficiency anaemia without clear underlying causes warrants OGD and colonoscopy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

Management of anaemia with underlying cause

A

Blood transfusion
Iron infusion
Oral iron - 200mg TDS Ferrous sulphate

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

What is pernicious anaemia?

A

Autoimmune condition where antibodies form against parietal cells or intrinsic factor.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

What symptoms would you expect in people with B12 deficiency?

A

Peripheral neuropathy
Paraesthesia
Loss of vibration sense or proprioception
Visual changes
Mood or cognitive changes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
28
Q

What antibodies would you test for to diagnose pernicious anaemia?

A

Intrinsic factor antibodies
Gastric parietal cell antibodies

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
29
Q

How would you manage B12 deficiency?

A

IM hydroxocobalamin
PO Cyanobalamin

Treat B12 deficiency before treating folate because B12 can lead to subacute combined degeneration of the cord

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
30
Q

Examples of inherited haemolytic anaemias

A

Hereditary spherocytosis
Hereditary Elliptocytosis
Thalassaemia
Sickle cell anaemia
G6PD

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
31
Q

Examples of acquired haemolytic anaemias

A

Autoimmune haemolytic anaemia
Alloimmune haemolytic anaemia
Paroxysmal noctornal haemoglobinuria
Microangiopathic haemolytic anaemia
Prosthetic valve related haemolytic

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
32
Q

Features of red blood cell destruction

A

Anaemia
Splenomegaly
Jaundice

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
33
Q

What investigations would you do for haemolytic anaemia?

A

FBC
Blood film
Direct Coombs test

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
34
Q

What is hereditary spherocytosis

A

Autosomal dominant - sphere shaped RBC that are fragile and easy to break

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
35
Q

How would hereditary spherocytosis present?

A

Jaundice
Gallstones
Splenomegaly
Aplastic crisis - in presence of parvovirus

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
36
Q

How is hereditary spherocytosis diagnosed?

A

Blood film - spherocytosis
MCHC - Raised FBC
Raised reticulocytes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
37
Q

Treatment of hereditary spherocytosis

A

Folate supplementation and splenectomy
Removal of gallbladder is gallstones are a problem

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
38
Q

What is hereditary elliptocytosis

A

Autosomal dominant. RBC are elliptical in shape. Similar to hereditary spherocytosis in diagnosis and management.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
39
Q

What is the inheritance pattern of G6PD deficiency?

A

X-linked recessive.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
40
Q

What can trigger G6PD?

A

Infections, medications (primaquine - antimalarial, chirp, sulphonylureas, sulfasalazine) and Fova beans

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
41
Q

How would G6PD deficiency present?

A

Jaundice, gallstones, anaemia, splenomegaly and Heinz bodies.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
42
Q

How would G6PD deficiency be diagnosed?

A

G6PD enzyme assay

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
43
Q

What is autoimmune haemolytic anaemia? Types?

A

When antibodies form against patient’s red blood cells.

Warm and cold type autoimmune haemolytic anaemia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
44
Q

What is warm type autoimmune haemolytic anaemia?

A

Most common type. Idiopathic.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
45
Q

What is cold type autoimmune haemolytic anaemia?

A

Also called cold agglutinin disease. At lower temperature <10 degrees Celsius, RBC attach to each other via agglutination -> destruction by immune system.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
46
Q

Secondary causes of cold type AIHA

A

Lymphoma, leukaemia, SLE and mycoplasma, EBV, CMV and HIV infections

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
47
Q

Management of AIHA

A

Blood transfusions
Prednisolone
Rituximab
Splenectomy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
48
Q

What is alloimmune haemolytic anaemia?

A

Where there is either foreign red blood cells circulating in the patient’s blood causing an immune reaction that destroys those red blood cells or there is a foreign antibody circulating in their blood that acts against their own red blood cells and causes haemolysis. Two scenarios where this occurs are transfusion reactions and haemolytic disease of the newborn

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
49
Q

What is paroxysmal nocturnal haemoglobinuria?

A

It is a rare condition that occurs when a specific genetic mutation in the haematopoietic stem cells in the bone marrow occurs during the patient’s lifetime. Mutation results in loss of proteins on the surface of RBC that inhibit the complement cascade. Loss of protection against the complement system results in activation of the complement cascade and destruction of the red blood cells.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
50
Q

What is microangiopathic haemolytic anaemia (MAHA)?

A

Is where the small blood vessels have structural abnormalities that cause haemolysis of the blood cells travelling through them.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
51
Q

What conditions is MAHA secondary to?

A

HUS
DIC
Thrombotic thrombocytopenia purpura (TTP)
SLE
Cancer

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
52
Q

What are types of thalassaemia?

A

Alpha thalassaemia
Beta thalassaemia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
53
Q

Pathophysiology of thalassaemia

A

Autosomal recessive defects in the alpha or beta global chains. Red blood cells are more fragile and break down more easily which is filtered through the spleen causing splenomegaly. Bone marrow expands to produce extra RBC to compensate for the chronic anaemia. Increased risk of fractures and prominent features such as pronounced forehead and malar eminences.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
54
Q

Potential signs and symptoms of thalassaemia

A

Microcytic anaemia
Fatigue
Pallor
Jaundice
Gallstones
Splenomegaly
Poor growth and development
Pronounced forehead and molar eminences

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
55
Q

Diagnosis of thalassaemia

A

FBC - showing microcytic anaemia
Hb electrophoresis. - diagnosis of globin abnormalities
DNA testing - to find genetic abnormality

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
56
Q

Types of beta thalassaemia

A

Thalassaemia minor
Thalassaemia intermedia
Thalassaemia major

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
57
Q

Thalassaemia minor

A

They have one abnormal and one normal gene. Thalassaemia minor causes a mild microcytic anaemia and usually patients only require monitoring and no active treatment

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
58
Q

Thalassaemia intermedia

A

Patients have two abnormal copies of the beta-globin gene. This can be either two defective genes or one defective gene and one deletion gene. This type causes more significant microcytic anaemia and patients require monitoring and occasional blood transfusions.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
59
Q

Thalassaemia major

A

Homozygous for the deletion genes. They have no functioning beta-globin genes at all. Most severe form and usually presents with severe anaemia and failure to thrive in early childhood.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
60
Q

What does thalassaemia major cause?

A

Severe microcytic anaemia
Splenomegaly
Bone deformities

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
61
Q

Pathophysiology of Sickle Cell Anaemia

A

Patients with sickle cell disease have an abnormal variant called haemoglobin S (HbS). Autosomal recessive - abnormal gene for beta-globin on chromosome 11.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
62
Q

How to diagnose Sickle cell anaemia

A

Newborn screening heel prick test at 5 days of age

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
63
Q

Complications of sickle cell anaemia

A

Anaemia
Increased risk of infection
Stroke
Avascular necrosis in large joints
Pulmonary hypertension
Priapism
CKD
Sickle cell crisis
Acute chest syndrome

64
Q

General management of sickle cell anaemia

A

Avoid dehydration and other triggers of crises
Ensure vaccines are up to date
Antibiotic prophylaxis to protect against infection with penicillin V
Hydroxycarbamide - to stimulate production of foetal haemoglobin (HbF)
Blood transfusion for severe anaemia
Bone marrow transplant - curative

65
Q

Triggers of sickle cell crisis

A

Spontaneous
Infection
Dehydration
Cold
Significant life events

66
Q

Management of sickle cell crisis

A

Treat any infection
Keep warm
Keep well hydrated
Simple analgesia
Penile aspiration in priapism

NSAIDs such as ibuprofen should be avoided where there is renal impairment

67
Q

Vaso-occlusive crisis pathophysiology

A

Caused by sickle shaped blood vessels clogging capillaries causing distal ischaemia. Associated with dehydration and raised haemltocrit. Symptoms are pain, fever. Can cause priapism in men.

68
Q

What is splenic sequestration crisis?

A

Caused by red blood cells blocking blood flow within the spleen. This causes an acutely enlarged and painful spleen. The pooling of blood in the spleen can lead to a severe anaemia and circulatory collapse. Management is supportive with blood transfusion and fluid resuscitation to treat anaemia and shock.

69
Q

What is aplastic crisis?

A

A situation where these is a temporary loss of the creation of new blood cells. Most commonly triggered by infection with parvovirus B19.

70
Q

Diagnosis of acute chest syndrome

A

Fever or respiratory symptoms
New infiltrates seen on a chest x-ray

This can be due to infection e.g. pneumonia or bronchiolitis or non-infective causes e.g. pulmonary vaso-occlusion or fat emboli

71
Q

Management and treatment of underlying cause

A

Antibiotics or antivirals for infections
Blood transfusions for anaemia
Incentive spirometry
Artificial ventilation

72
Q

What is leukaemia?

A

A form of cancer of bone marrow

73
Q

Types of leukaemia

A

Acute myeloid leukaemia
Acute lymphoblastic leukaemia
Chronic myeloid leukaemia
Chronic lymphocytic leukaemia

74
Q

Ages affected by leukaemia

A

ALL CeLLmates have CoMmon AMbitions

Under 5 and over 45 - Acute lymphoblastic leukaemia (ALL)
Over 55 - Chronic Lymphocytic leukaemia. (CeLLmates)
Over 65 - Chronic Myeloid leukaemia (CoMmon)
Over 75 - Acute Myeloid leukaemia (Ambitions)

75
Q

Presentation of leukaemia

A

Fatigue
Fever
Failure to thrive
Pallor due to anaemia
Petechiae
Abnormal bleeding
Lymphadenopathy
Hepatosplenomegaly

76
Q

Differentials for petechiae

A

Leukaemia
Meningococcal septicaemia
Vasculitis
HSP
ITP
Non-accidental injury

77
Q

Diagnosis of leukaemia

A

FBC
Blood film
LDH
Bone marrow biopsy
Chest xray
Lymph node biopsy
Lumbar puncture
CT, MRI and PET

78
Q

Bone marrow biopsy in leukaemia

A

Bone marrow aspiration
Bone marrow trephine - solid core sample of the bone marrow and provides a better assessment of the cells and structure
Bone marrow biopsy

79
Q

Acute lymphoblastic leukaemia

A

Under 5 and over 45
Acute proliferation of B-lymphocytes.
Associated with Down’s syndrome.
Blood film shows blast cells.
Associated with Philadelphia chromosome (9:22 translocation)

80
Q

Chronic lymphocytic leukaemia

A

Over 55
Chronic proliferation of B-lymphocytes.
Can present with infections, anaemia, bleeding and weight loss. Can cause warm autoimmune haemolytic anaemia.
CLL can transform into high-grade lymphoma (Richter’s transformation)
Blood film shows “smear” or “smudge” cells.

81
Q

Chronic myeloid leukaemia

A

Over 65
Three typical phases: chronic, accelerated and blast phase.

Chronic phase can last around 5 years and often asymptomatic and often diagnosed incidentally with a raised white cell count.

Accelerated phase - more symptomatic, develop anaemia and thrombocytopenia and become immunocompromised

Blast phase - high proportion of blast cells and blood. Severe symptoms and pancytopenia.

Associated with Philadelphian chromosome.

82
Q

Acute myeloid leukaemia

A

Over 75
It can result of transformation from a myeloproliferative disorder such as polycythaemia ruby vera or myelofibrosis.

A blood film will show a high proportion of blast cells.

Associated with Aeur rods

83
Q

Leukaemia management

A

Radiotherapy
Bone marrow therapy
Surgery

84
Q

Complications of chemotherapy

A

Failure
Stunted growth and development in children
Infections due to immunodeficiency
Neurotoxicity
Infertility
Secondary malignancy
Cardiotoxicity

85
Q

Tumour lysis syndrome

A

Caused by the release of uric acid from cells being destroyed by chemotherapy. Uric acid can form in kidneys causing AKI. Allopurinol or rasburicase used to reduce of high uric acid levels.

86
Q

Categories of lymphomas

A

Hodgkin’s and non-hodgkins lymphoma.

87
Q

What is Hodgkin’s lymphoma?

A

Proliferation of lymphocytes.
Peaks at 20s and 75.

88
Q

Presentation of Hodgkin’s lymphoma

A

Lymphadenopathy is key symptom. Characteristically non-tender and feel ‘rubbery’.

B symptoms: Fever, weight loss, night sweats

Other symptoms:
Fatigue
Itching
Cough
SOB
Abdominal pain
Recurrent infections

89
Q

Investigations for lymphoma

A

LDH
Lymph node biopsy - key diagnostic test
Reed-sternberg is key finding from lymph node biopsy

CT, MRI and PET for diagnosis and staging

90
Q

Management of lymphoma

A

Chemotherapy and radiotherapy are key treatments.

Chemotherapy - risks of leukaemia and infertility
Radiotherapy - risk of cancer and damage to tissues and hypothyroidism

91
Q

Risk factors for non-hodgkins lymphoma

A

HIV
EBV
H-pylori (MALT Lymphoma)
Hep B or C
Exposure to pesticides
Family history

92
Q

What is myeloma?

A

Cancer of plasma cells.

93
Q

What is multiple myeloma?

A

Where the myeloma affects multiple areas of the body

94
Q

What is monoclonal gammopathy of undetermined significance (MGUS)?

A

Where there is an excess of a single type of antibody or antibody components without other features of myeloma or cancer.

95
Q

What is smouldering myeloma?

A

Where there is progression of MGUS with higher levels of antibodies or antibody components. Premalignant and more likely to progress to myeloma.

96
Q

What is Waldenstrom’s macroglobulinaemia?

A

A type of smouldering myeloma where there is excessive IgM

97
Q

Myeloma pathophysiology

A

Genetic mutation causing plasma cells to multiply rapidly and uncontrollably. These plasma cells produce on type of antibody called immunoglobulins. Most commonly IgG.

98
Q

Myeloma and anaemia

A

Invasion of bone marrow by cancerous plasma cells. Causes suppression of the development of other blood cell lines leading to anaemia, neutropenia. and thrombocytopenia

99
Q

What is myeloma bone disease?

A

Plasma cells released cytokines which react with bone stroll cells causing increased osteoclast activity and suppressed osteoblast activity. Bone becomes imbalanced.

100
Q

What is myeloma renal disease?

A

Patients with myeloma often develop renal impairment due to:

High levels of immunoglobulins
Hypercalcaemia
Dehydration

101
Q

Key features of myeloma

A

CRAB

Calcium (elevated)
Renal failure
Anaemia
Bone lesions/pain

102
Q

Risk factors for myeloma

A

Older age
Male
Black African ethnicity
Family history
Obesity

103
Q

Testing for myeloma

A

BLIP

Bence-jones protein (urine electrophoresis)
L - serum free light chain assay
I - serum immunoglobulins
P - protein electrophoresis

Bone marrow biopsy

X-ray - punched out lesions, lytic lesions, raindrop skull

104
Q

Myeloma and hyperviscosity

A

Plasma viscosity increases due to more immunoglobulins in the blood. Causes:

Easy bruising
Easy bleeding
Reduced or loss of sight due to vascular disease in the eye
Purple discolouration to the extremities
HF

105
Q

What are common sites for myeloma bone disease?

A

Skull
Spine
Long bones
Ribs

106
Q

What are the three important myeloproliferative disorders?

A

Primary myelofibrosis
Polycythaemia vera
Essential thrombophilia

107
Q

What is primary myelofibrosis?

A

Proliferation of haematopoetic stem cells

108
Q

What is polycythaemia vera?

A

Proliferation of erythroid cell line

109
Q

What is essential thrombocythaemia?

A

Proliferation of megakaryocytic cell line

110
Q

Myeloproliferative disorders can progress to what?

A

Acute myeloid leukaemia

111
Q

What mutations are myeloproliferative conditions associated with?

A

JAK2
MPL
CALR

112
Q

What is myelofibrosis?

A

Myelofibrosis can be caused by primary myelofibrosis, polycythaemia vera or essential thrombocythaemia.

Myelofibrosis is where the proliferation of the cell line leads to fibrosis of the bone marrow. The bone marrow is replaced by scar tissue. This is in response to cytokines that are released from the proliferating cells. One particular cytokine is fibroblast growth factor. This fibrosis affects the production of blood cells and can lead to anaemia and low white blood cells (leukopenia).

When the bone marrow is replaced with scar tissue the production of blood cells (haematopoiesis) starts to happen in other areas such as the liver and spleen. This is known as extramedullary haematopoiesis and can lead to hepatomegaly and splenomegaly. This can lead to portal hypertension. If it occurs around the spine it can lead to spinal cord compression.

113
Q

How can myelofibrosis present?

A

Initially asymptomatic

They can present with systemic symptoms such as fatigue, weight loss, night sweats and fever

There may be signs and symptoms of underlying complications: anaemia (except in polycythaemia), splenomegaly (abdominal pain), portal hypertension (ascites, varies and abdominal pain), low platelets (bleeding and petechiae), thrombosis, raised red blood cells (thrombosis and red face), low white blood cells (infections)

114
Q

Three key signs on examination in someone with suspected polycythaemia vera

A

Conjunctival plethora (excessive redness to the conjunctiva in the eyes)
Ruddy complexion
Splenomegaly

115
Q

FBC findings in polycythaemia vera

A

Raised Hb >185 in men and >165 in women

116
Q

FBC findings in Primary thrombocythaemia

A

Raised platelet count more than 600 x 10^9

117
Q

What does blood film in myelofibrosis show?

A

Teardrop-shaped RBC with varying sizes of red blood cells and immature red and white blood cells

118
Q

How to diagnose myelofibrosis?

A

Bone marrow biopsy is test of choice

Bone marrow aspiration - usually dry as bone marrow changed to scar tissue

Test for JAK2, MPL and CALR

119
Q

Management of primary myelofibrosis

A

Allogenic stem cell transplantation

Chemo

Supportive management

120
Q

Management of polycythaemia vera

A

Venesection - first line treatment

Aspirin - to reduce the risk of developing blood clots

Chemo

121
Q

Management of essential thrombocythaemia

A

Aspirin - reduce the risk of developing blood clots

Chemo

122
Q

What is thrombocythaemia?

A

Low platelet count. They can be split into problems with production or destruction

123
Q

Problems with production in thromocythaemia

A

Sepsis
B12 or folic acid deficiency
Liver failure causing reduced thrombopoeitin
Leukaemia
Myelodysplastic syndrome

124
Q

Problems with destruction in thrombocythaemia

A

Medications - sodium valproate, methotrexate, antihistamine, PPI
Alcohol
Immune thrombocytopaenic purpura
Thrombotic thrombocytopenia purpura
Heparin-induced thrombocytopenia
HUS

125
Q

Presentation of thrombocythamia

A

Mild usually asymptomatic and found incidentally on FBC
Platelet counts below 50x10^9. will result in easy on spontaneous bruising and prolonged bleeding times. They may present with nosebleeds, bleeding gums, heavy periods, easy bruising or blood in the urine or stools

Platelet counts below 10x10^9 are high risk for spontaneous bleeding. Spontaneous intracranial bleeding or GI bleeds are particularly concerning

126
Q

Differential diagnosis of abnormal or prolonged bleeding

A

Thrombocytopenia
Haemophilia A and B
Von willebrand disease
DIC

127
Q

Immune thrombocytopenia purpura

A

A condition where antibodies are created against platelets. This causes an immune response against platelets, resulting in the destruction of platelets and low platelet count

128
Q

Management of immune thrombocytopenia purpura

A

Prednisolone
IV immunoglobulins
Rituximab (monoclonal antibody against B cells)
Splenectomy

129
Q

Presentation of TTP

A

Fever
Altered mental state
Haemolytic anaemia
Reduced renal output
HTN

130
Q

What is Thrombotic thrombocytopenia purpura?

A

Where tiny blood clots develop throughout the small vessels of the body using up platelets and causing thrombocytopaenia, bleeding under the skin and other systemic issues.

Blood clots develop due to a problem with specific protein called ADAMTS13. It inactivates von willebrand factor and reduces platelet adhesion to vessel walls and clot formation. Platelets used up leading to thrombocytopenia. Blood clots break up red blood cells leading to haemolytic anaemia.

131
Q

Treatment of Thrombotic thrombocytopenia purpura

A

Plasma exchange
Steroid
Rituximab

132
Q

What is Heparin induced thrombocytopenia?

A

Involves the development of antibodies against platelets in response to exposure to heparin. These heparin induced antibodies targets proteins on the platelets called platelet factor 4. HIT antibodies bind to platelets and activate clotting mechanisms causing a hyper coagulable state and leads to thrombosis. They also break down platelets causing thrombocytopenia.

133
Q

Diagnosis of Thrombotic thrombocytopenia purpura

A

HIT antibodies

134
Q

Management of Thrombotic thrombocytopenia purpura

A

Stopping heparin and using an alternative anticoagulant guided by a specialist

135
Q

What is myelodysplasic syndrome?

A

When myeloid bone marrow cells not maturing properly and therefore not producing healthy RBC.

136
Q

How does myelodysplastic affect the myeloid cell line?

A

Anaemia
Neutropenia
Thrombocytopenia (low platelets)

137
Q

Myelodysplastic syndrome affects what category of people?

A

Patients above 60 who have previously had chemo or radio

138
Q

Presentation of myelodysplastic syndrome

A

Mostly asymptomatic and incidentally diagnosed on DBC

May present with symptoms of anaemia. (fatigue, pallor, or SOB), neutropenia or thrombocytopenia

139
Q

Diagnosis of myelodysplastic syndrome

A

FBC - may be blast on the blood film

Diagnosis is confirmed by bone marrow aspiration and biopsy.

140
Q

Management of myelodysplastic syndrome

A

Watchful waiting
Supportive treatment with blood transfusions
Chemo
Stem cell transplantation

141
Q

What is Von willebrand disease?

A

Most common inherited cause of abnormal bleeding. Autosomal dominant. Malfunctioning von willebrand factor. Three types. Type 3 most common

142
Q

Presentation of von willebrand disease

A

Bleeding gums with bruising
Nose bleeds
Heavy menstrual bleeding
Heavy bleeding during surgical operations

143
Q

Diagnosis of von willebrand disease

A

History of abnormal bleeding, family history, bleeding assessment tools and laboratory investigations.

144
Q

Management of von willebrand disease

A

Desmopressin - stimulates release of the VWF
VWF can be infused
Factor VII

Women with VWD that suffer from heavy periods can be managed by:
Tranexamic acid
Mefanamic acid
Noresthisterone
COCP
Mirena coil

Hysterectomy

145
Q

What deficiency causes Haemophilia A?

A

Factor VIII

146
Q

What deficiency causes Haemophilia B?

A

Factor IX

147
Q

What is the inheritance pattern of Haemophilia A and B?

A

X linked recessive

148
Q

Signs and symptoms of haemophilia A and B

A

Most cases present in neonates or early childhood with intracranial haemorrhage, haematomas and cord bleeding

Spontaneous bleeding into joins and muscles is classic feature of severe haemophilia

Abnormal bleeding of:
Gums
GI tract
Urinary tract causing haematuria
Retroperitoneal space
Intracranial
Following procedures

149
Q

Diagnosis of haemophilia

A

Bleeding scores, coagulation factor assay and genetic testing

150
Q

Management of haemophilia

A

Clotting factors VII or IX replaced intravenously.
Desmopressin to stimulate release of VWF
Antifibrinolytics such as tranexamic acid

151
Q

Thrombophilias that can predispose to development of clots

A

Anti-phospholipid syndrome
Factor V Leiden
Antithrombin deficiency

152
Q

First line management for DVTs

A

DOAC not LMWH

153
Q

How would you manage a symptomatic iliofemoral DVT?

A

Catheter-directed thrombolysis

154
Q

How should tranexamic acid be administered in major haemorrhages?

A

Tranexamic acid is given as an IV bolus followed by an infusion in cases of major haemorrhage.

155
Q

Pathophysiology of Factor V Leiden

A

Resistance to action of protein C

156
Q

When to transfuse platelets?

A

Offer platelet transfusions to patients with a platelet count of <30 x 10 9 with clinically significant bleeding

Cancer Care (3 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions