Haematology Flashcards
(162 cards)
1
Q
Ix for anaemia
A
- Hb
- MCV = mean cell volume
- B12
- Folate
- Ferritin
- Blood film
- OGD and colonoscopy for unexplained iron deficiency anaemia – urgent cancer referral for suspected gastrointestinal ca
- Bone marrow biopsy – if cause remains unclear
2
Q
Causes of microcytic anaemias
A
MICROCYTIC ANAEMIA = TAILS • T = Thalassaemia • A = Anaemia of chronic disease • I = Iron deficiency anaemia • L = Lead poisoning • S = Sideroblastic anaemia
3
Q
Causes of normocytic anaemia
A
NORMOCYTIC ANAEMIA = 3 As and 2 Hs • A = Acute blood loss • A = Anaemia of chronic disease • A = Aplastic anaemia • H = Haemolytic anaemia • H = Hypothyroidism
4
Q
Causes of macrocytic anaemia
A
MACROCYTIC ANAEMIA = megaloblastic or normoblastic
• Megaloblastic – impaired DNA synthesis preventing the cell from dividing normally so it keeps growing into a larger abnormal cell
o B12 deficiency
o Folate deficiency
• Normoblastic anaemia o Alcohol o Reticulocytosis – from haemolytic anaemia or blood loss o Hypothyroidism o Liver disease o Azathioprine
5
Q
Anaemia symptoms
A
- Tiredness
- SOB
- Headaches
- Dizziness
- Palpitations
- Worsening of other conditions e.g., angina, HF, PVD
- Pale skin
- Conjunctival pallor
- Tachycardia
- Raised respiratory rate
6
Q
Causes of iron deficiency anaemia
A
- Blood loss e.g., menstruation in women
- Insufficient dietary iron
- Iron requirements increase e.g., in pregnancy
- Loss of iron e.g., from slow bleeding colon cancer, oesophagitis, gastritis, IBD
- Inadequate iron absorption e.g., PPIs, coeliac disease, Crohn’s disease
- Hookworm
7
Q
Ix for iron deficiency anaemia
A
FBC Blood film Total iron binding capacity - High Transferrin saturation - Low Serum iron - Low Ferritin- Low (Unless infection/malignancy, then high) Coeliac serology Gastroscopy/colonoscopy Stool microscopy - hookworm
8
Q
Specific signs of chronic iron deficiency anaemia
A
- Koilonychia
- Atrophic glossitis
- Angular cheilosis
- Plummer-Vinson syndromes: post-cricoid webs
9
Q
Define pernicious anaemia
A
an autoimmune condition where antibodies form against parietal cells or intrinsic factor therefore is a cause of B12 deficiency anaemia. B12 deficiency can be caused by insufficient dietary intake OR pernicious anaemia.
10
Q
Neurological symptoms of B12 deficiency
A
a. Peripheral neuropathy
b. Loss of vibration sensation or proprioception
c. Visual changes
d. Mood/cognitive changes
11
Q
Diagnosis of pernicious anaemia
A
o 1st line - Intrinsic factor antibody
o 2nd line - Gastric parietal cell antibody
12
Q
Tx for pernicious anaemia
A
- Cyanocobalamin – oral replacement for dietary deficiency of B12
- IM hydroxycobalamin – for pernicious anaemia as absorption is the problem
13
Q
In which order should you treat folate and B12 deficiency?
A
If there’s folate deficiency, treat the B12 deficiency first. Treating patients with folic acid when they have B12 deficiency can lead to subacute combined degeneration of the cord.
14
Q
Define haemolytic anaemia
A
Definition = destruction of RBCs (haemolysis) leading to anaemia. There’s inherited and acquired conditions that lead to haemolysis of RBCs.
15
Q
Causes of inherited haemolytic anaemia
A
- Hereditary spherocytosis
- Hereditary elliptocytosis
- Thalassaemia
- Sick cell anaemia
- G6PD deficiency
16
Q
Causes of acquired haemolytic anaemia
A
- Autoimmune haemolytic anaemia
- Alloimmune haemolytic anaemia – transfusion reactions & haemolytic disease of the newborn
- Paroxysmal nocturnal haemoglobinuria
- Prosthetic valve related haemolysis
17
Q
Symptoms of haemolytic anaemias
A
- Anaemia
- Splenomegaly – spleen becomes filled with destroyed RBCs
- Jaundice – bilirubin released during the destruction of RBCs
18
Q
Ix for haemolytic anaemia
A
- FBC – normochromic anaemia
- Blood film – schistocytes (fragments of RBCs)
- Direct Coomb’s test - +ve in autoimmune haemolytic anaemia
19
Q
What is hereditary spherocytosis? (inheritance, pathology, presentation, diagnosis, tx)
A
- Inherited haemolytic anaemia
- Autosomal dominant
- Sphere shaped RBCs that get broken down easily in the spleen
- Presentation: gallstones, jaundice, splenomegaly, aplastic crisis (in the presence of pavovirus)
- Diagnosis: family hx and blood film
- Tx: folate, splenectomy, cholecystectomy if gallstones
20
Q
What is hereditary elliptocytosis?
A
- Inherited haemolytic anaemia
- Autosomal dominant
- RBCs ellipse shaped
- Presentation: gallstones, jaundice, splenomegaly, aplastic crisis
- Tx: folate, splenectomy
21
Q
What is G6PD deficiency and how is it diagnosed?
A
- X linked recessive condition
- More common in Mediterranean and African patients
- Defect in RBC enzyme G6PD
- Causes crises triggered by infections, medications (primaquine, ciprofloxacin, sulfonylureas, sulfasalazine & other sulphonamide drugs) and fava beans (broad beans)
- Presentation: jaundice, gallstones, anaemia, splenomegaly
- Diagnosis: Heinz bodies on blood film, G6PD enzyme assay
22
Q
What is autoimmune haemolytic anaemia?
A
• Antibodies are created against the patients RBCs = destruction of RBCs
• 2 types:
o Warm type autoimmune haemolytic anaemia – more common. Idiopathic.
o Cold type autoimmune haemolytic anaemia – AKA cold agglutinin disease. Antibodies cause RBCs to clump together (agglutination). Secondary to lymphoma, leukaemia, SLE, mycoplasma/EBV/CMV/HIV infection
• Ix: Coombs test
• Mx: blood transfusions, prednisolone, rituximab, splenectomy
23
Q
What is Allo-immune haemolytic anaemia?
A
- In transfusion reaction and haemolytic disease of the newborn
- Haemolytic transfusion reactions = antibodies produced against antigens on foreign RBCs that have been transfused into the patient = destruction of those RBCs
- Haemolytic disease of the newborn = antibodies cross the placenta from mother to fetus that target RBCs of the fetus
24
Q
What is paroxysmal nocturnal haemoglobinuria?
A
- Rare
- Gene mutation in haemopoietic stem cells in bone marrow
- Loss of proteins on surface of RBCs that inhibit the complement cascade = activation of complement cascade on the surface of RBCs
- Red urine in the morning (contains Hb and haemosiderin)
- Predisposed to VTE & smooth muscle dystonia (oesophageal spasm & erectile dysfunction)
- Mx: eculizumab, bone marrow transplant
25
What is microangiopathic haemolytic anaemia?
• Small blood vessels gave structural abnormalities that cause haemolysis of RBCs
• Secondary to:
o Haemolytic uraemic syndrome (HUS)
o DIC
o Thrombotic thrombocytopenia purpura (TTP)
o SLE
o Ca
26
What is prosthetic valve Haemolysis?
* Haemolytic anaemia is a complication of prosthetic heart valves
* Mx: monitoring, oral iron, blood transfusion, revision surgery
27
What is thalassaemia?
genetic diseases of unbalanced Hb synthesis, with under production (or no production) of one globin chain. Autosomal recessive conditions.
- Defects in alpha-globin chain = alpha thalassemia
- Defects in beta globin chain = beta thalassemia
28
Pathology of thalassaemia
1. RBCs more fragile as they're laking a global chain, the spleen destroys damaged RBCs therefore swells = splenomegaly
2. Bone marrow expands to produce extra RBCs to compensate for the chronic anaemia, therefore:
a. More susceptible to fractures
b. Pronounced forehead
c. Malar eminences – cheekbones
29
Diagnosing thalassaemia
1. FBC – microcytic anaemia
2. Hb electrophoresis – used to diagnose globin abnormalities
3. DNA testing – for genetic abnormalities
4. Screening in pregnant women
30
what is alpha thalassaemia, the chromosome involved and mx?
Defects in alpha-globin chains
Chromosome 16
Mx:
- Monitor FBC
- Monitor for complications
- Blood transfusions
- Splenectomy
- Bone marrow transplant (can be curative)
31
what is beta thalassaemia, the chromosome involved and the 3 types (+their mx)?
Defects in beta-globin chain
Chromosome 11
3 types:
1. Thalassaemia major – homozygous for deletion genes, no functioning beta-globin chains. Severe anaemia and failure to thrive in early childhood.
a. Mx – regular transfusions, iron chelation, splenectomy, bone marrow transplant
2. Thalassaemia intermedia – two abnormal copies of gene, significant microcytic anaemia
a. Occasional blood transfusions
3. Thalassaemia minor – carries of gene, mild microcytic anaemia
a. Monitoring
32
Risk factors for DVT
- Immobility
- Recent surgery
- Long haul travel
- Pregnancy
- Hormone therapy with oestrogen – HRT/COCP
- Malignancy
- Polycythaemia
- SLE
- Thrombophilia :
o Antiphospholipid syndrome
o Factor V leiden
o Antithrombin deficiency
o Protein C or S deficiency
o Hyperhomocysteinaemia
o Prothrombin gene variant
o Activated protein C resistance
33
Presentation of DVT
* Unilateral
* Calf or leg swelling: measure the circumference of the calf 10cm below the tibial tuberosity, >3cm difference between calves is significant
* Dilated superficial veins
* Tenderness to calf – over the site of the deep veins
* Oedema
* Colour changes to the leg
* Ask PE symptoms
34
What is the Well's Score?
- Predicts the risk of a patient presenting with symptoms having a DVT or PE
- To calculate score:
o Clinical signs and symptoms of DVT (leg swelling with pain on palpation of deep veins)
o Alternative diagnosis is less likely
o Heart rate >100
o Recent surgery or immobilisation
o Previous PE/DVT
o Haemoptysis
o Malignancy
35
Diagnosis of DVT
1. D-dimer (sensitive but not specific) – good for excluding DVT where there is low suspicion
a. Other causes of raised d-dimer: pneumonia, malignancy, HF, surgery, pregnancy
2. Doppler US.
a. Repeat after 6-8 days if negative but positive D dimer and Well’s score is suggestive of a DVT
3. CTPA for PE or ventilation perfusion (VQ) scan
36
Ix for unprovoked DVT
- Ix for cancer – medical hx, baseline bloods and examination (?CT TAP)
- Antiphospholipid syndrome
- Hereditary thrombophilias
37
Mx for DVT
- Treatment dose apixaban/rivaroxaban
- Catheter directed thrombolysis in patients with symptomatic iliofemoral DVT & <14 days of symptoms
- Long term
o DOAC / warfarin / LMWH if pregnant
o Continue anticoagulation for
3 months if reversible cause
Beyond 3 months is cause unclear
3-6 months in active cancer
o IVC filters – device inserted into IVC to filter out blood clots in patients not suitable for anticoagulation
38
What is Budd-Chiari syndrome
: thrombosis in the hepatic vein, blocking outflow of blood from the liver. Causes acute hepatitis with the following triad of symptoms: abdominal pain, hepatomegaly and ascites.
39
What is thalassaemia?
A genetic defect in the protein chains that make up haemoglobin
Normal hb = 2 alpha and 2 beta globin chains
40
What is the genetic inheritance of thalassaemia?
Autosomal recessive
41
Pathology of thalassaemia
In thalassaemia the RBCs are more fragile and break down more easily.
The spleen collects all the destroyed RBCs and swells = splenomegaly
Bone marrow expands to produce extra RBCs to compensate for the chronic anaemia
= more susceptible to fracture & pronounced forehead/malar eminences
42
Two types of thalassaemia
Alpha thalassaemia - defects in alpha globin chains
| Beta thalassaemia - effects in beta globin chains
43
Signs and symptoms of thalassaemia
```
Microcytic anaemia
Fatigue
Pallor
Jaundice
Gallstones
Splenomegaly
Poor growth/development
Pronounced forehead
Pronounced malaria eminences - cheek bones
```
44
Diagnosis of thalassaemia
FBC - microcytic anaemia
HB electrophoresis - to diagnose globin abnormalities
DNA testing - for genetic abnormality
Pregnant women screened at booking
45
What is a complication of thalassaemia?
Iron overload
46
Why do patients with thalassaemia suffer with iron overload?
as a result of faulty creation of red blood cells, recurrent transfusions and increased absorption of iron in response to the anaemia.
47
Monitoring for thalassaemia patients
Serum ferritin - to monitor for iron overload
48
What are the symptoms of iron overload?
```
Fatigue
Liver cirrhosis
Infertility and impotence
Heart failure
Arthritis
Diabetes
Osteoporosis and joint pain
```
49
What is the genetic abnormality in alpha thalassaemia?
defect on chromosome 16
50
Mx for alpha thalassaemia
```
Monitoring the full blood count
Monitoring for complications eg iron overload
Blood transfusions
Splenectomy may be performed
Bone marrow transplant can be curative
```
51
What is the genetic abnormality in beta thalassaemia?
Defect on chromosome 11
There may be abnormal copies of the gene for the beta globin chain so they retain some function or deletion of the gene
52
What are the 3 types of beta thalassaemia?
Thalassaemia minor / trait
Thalassaemia intermedia
Thalassaemia major
53
What happens in thalassaemia minor/trait?
carrier state - one normal and one abnormal gene
usually asymptomatic
there is mild anaemia that can worsen in pregnancy
54
What happens in thalassaemia intermedia?
Have 2 defective genes or one defective and one deletion gene
Moderate anaemia that doesn't usually require transfusions
55
what happens in thalassaemia major?
abnormalities in both b globin chain genes
Prevents in 1st year of life with severe microcytic anaemia
Get skull bossing, hepatosplenomegaly and osteopenia
Need lifelong blood transfusions, iron chelation, splenectomy and bone marrow transplants can be curative
56
what does a blood film show in thalassaemia major?
hypo chromic microcytic cells, target cells and nucleated RBCs
57
What is sickle cell anaemia?
a genetic condition that causes sickle (crescent) shaped red blood cells
Makes the RBCs fragile = easily destroyed = haemolytic anaemia
58
Genetic inheritance of sickle cell anaemia?
Autosomal recessive - abnormal gene for beta-globin on chromosome 11
One copy abnormal gene = carrier = sickle cell trait
Two copies abnormal gene = sickle cell disease
59
Diagnosis of sickle cell anaemia
Newborn screening heel prick test on day 5
60
Complications of sickle cell anaemia
```
Anaemia
Increased risk of infection
Stroke
Avascular necrosis in large joints such as the hip
Pulmonary hypertension
Painful and persistent penile erection (priapism)
Chronic kidney disease
Sickle cell crises
Acute chest syndrome
```
61
Mx of sickle cell anaemia
```
Avoid dehydration
Vaccinations
Abc prophylaxis with pen V
Hydroxycarbamide - stimulates production of fetal Hb
Blood transfusions in severe anaemia
Bone marrow transplant
```
62
How to manage a sickle cell crisis
Have a low threshold for admission to hospital
Treat any infection
Keep warm
Keep well hydrated (IV fluids may be required)
Simple analgesia such as paracetamol and ibuprofen
Penile aspiration in priapism
63
What is haemophilia?
Inherited severe bleeding disorder
64
What are the types of haemophilia?
```
Haemophilia A
Haemophilia B (Christmas disease)
```
65
what causes haemophilia A?
Deficiency in factor VIII
66
what causes haemophilia B?
Deficiency in factor IX
67
How is haemophilia inherited?
X linked recessive condition
- Men require one abnormal gene on their X chromosome
- women require 2 abnormal copies on both of their X chromosomes
Therefore haemophilia mostly affects males
68
Symptoms of haemophilia
Bleed excessively with minor trauma
Spontaneous haemorrhage without trauma - gums, GI, haematuria, retroperitoneal space, intracranial
Bleeding following procedures
Spontaneous bleeding into joints - haemoathrosis
Spontaneous bleeding into muscles
In neonates:
- Intracranial haemorrhage
- Haematomas
- Cord bleeding
69
Diagnosis of haemophilia
Prolonged APTT
Bleeding time, thrombin time and prothrombin time are normal
Bleeding scores
Coagulation factor assays
Genetic testing
70
Mx of haemophilia
Replace clotting factors (VII or IX) with IV infusions - prophylactically or with bleeding
In response to acute bleeding:
1. Infusions of affected factor
2. Desmopressin to stimulate release of von Willebrand factor
3. Antifibrinolytics e.g. tranexamic acid
71
Problem with treatment of haemophilia
Up to 10-15% of patients with haemophilia A develop antibodies to factor VIII treatment.
72
What is von willebrands disease?
An inherited bleeding disorder that results in the deficiency, absence or malfunctioning of von willebrand factor
73
what is the most common inherited cause of abnormal bleeding (haemophilia)?
Von willebrand disease
74
Inheritance of von willebrands disease?
autosomal dominant, apart from type 3 which is recessive
| there are many different underlying genetic causes
75
What are the types of von willebrands disease?
Types are based on the underlying cause:
Type 1 - partial reduction in VWF
Type 2 - abnormal form of VWF
Type 3 - most severe, total lack of VWF (autosomal recessive)
76
What are the symptoms of von willebrands disease?
Unusually easy, prolonged or heavy bleeding
- Gums bleeding
- Epistaxis
- Menorrhagia
- Heavy bleeding during surgical operations
Positive family hx for bleeding
77
Diagnosis of von willebrands disease
History
Bleeding assessment tools
Prolonged bleeding time
APTT prolonged
Factor VIII levels may be moderately reduced
Defective platelet aggregation with ristocetin
78
Mx of von willebrands disease
In response to bleeding:
- Desmopressin - to stimulate the release of VWF
- VWF infusion
- Factor VIII infusion with plasma derived VWF
women with heavy periods:
- Tranexamic acid
- Mefanamic acid
- Noresthisterone
- COCP
- Mirena coil
- Hysterectomy in severe cases
79
Causes of hyposplenism
```
Splenectomy
Sickle cell anaemia
Coeliac disease, dermatitis herpetiformis
Grave's disease
SLE
Amyloid
```
80
Features of hyposplenism on a blood film
```
Howell-Jolly bodies
Siderocytes
Target cells
Pappenheimer bodies
Acanthocytes
```
81
What diseases are patients at risk of following a splenectomy?
Pneumococcus
Haemophilus
Meningococcus
Capnocytophaga canimorsus
82
What vaccinations should a patient have prior to splenectomy?
if elective, should be done 2 weeks prior to operation
Hib, meningitis ACWY
annual influenza vaccination
then pneumococcal vaccine every 5 years
83
what abx prophylaxis should someone have if they're having a splenectomy?
Penicillin V for at least 2 years or if not for life
84
Indications for splenectomy
Trauma: 1/4 are iatrogenic
Spontaneous rupture: EBV
Hypersplenism: hereditary spherocytosis or elliptocytosis etc
Malignancy: lymphoma or leukaemia
Splenic cysts, hydatid cysts, splenic abscesses
85
Complications of splenectomy
Haemorrhage (may be early and either from short gastrics or splenic hilar vessels
Pancreatic fistula (from iatrogenic damage to pancreatic tail)
Thrombocytosis: prophylactic aspirin
Encapsulated bacteria infection e.g. Strep. pneumoniae, Haemophilus influenzae and Neisseria meningitidis
86
what happens after a splenectomy?
Platelets will rise first (therefore in ITP should be given after splenic artery clamped)
Blood film will change over following weeks, Howell-Jolly bodies will appear
Other blood film changes include target cells and Pappenheimer bodies
Increased risk of post-splenectomy sepsis, therefore prophylactic antibiotics and pneumococcal vaccine should be given.
87
What is leukaemia?
cancer of a particular line of stem cells that usually begins in the bone marrow and results in high numbers of abnormal white blood cells.
These white blood cells are not fully developed and are called blasts or leukaemia cells
88
How is leukaemia classified?
Classified as acute or chronic according to the degree of cell differentiation.
Classified as myelogenous or lymphocytic according to the predominant type of cell involved – myeloid or lymphoid
89
What are the 4 types of leukaemia?
1. Acute lymphoblastic leukaemia
2. Acute myeloid leukaemia
3. Chronic myeloid leukaemia
4. Chronic lymphoid leukaemia
90
Pathology of leukaemia
Form of cancer of cells in the bone marrow
A genetic mutation in one of the precursor cells in the bone marrow leads to excessive production of a single type of abnormal white blood cell
The excessive production of a single type of cell = suppression of the other cell lines = pancytopenia (anaemia, leukopenia, thrombocytopenia)
91
Who gets what type of leukaemia? (by age)
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)
92
What are the symptoms of leukaemia?
```
Fatigue
Fever
Failure to thrive in children
Pallor - anaemia
Petechiae / abnormal bruising - thrombocytopenia
Abnormal bleeding - thrombocytopenia
Lymphadenopathy
Hepatosplenomegaly
```
93
Differentials for petechiae
```
Leukaemia
Meningococcal septicaemia
Vasculitis
HSP
ITP
Non accidental injury
```
94
Diagnosis of leukaemia
FBC - do within 48 hours in suspected leukaemia
Blood film - abnormal cells and inclusions
Lactate dehydrogenase - raised in leukaemia + other cancers
**Bone marrow biopsy
CXR - mediastinal lymphadenopathy
Lymph node biopsy - Ix for lymph node involvement/lymphoma
LP - of CNS involvement
CT/MRI/PET
95
How are bone marrow biopsies done?
Bone marrow aspiration - liquid sample of cells from bone marrow
Bone marrow trephine - core sample of bone marrow
Bone marrow biopsy - from iliac crest, need LA & special needle
96
When should you immediately refer children/young adults to hospital if you suspect leukaemia?
If they have petechiae or hepatosplenomegaly
97
What cell is implicated in ALL?
Malignant change in one of the lymphocyte precursor cells, usually B-lymphocytes
98
What is the peak age for ALL?
2-4 you
| Can also affect adults >45 yo
99
Associations with ALL
Down's syndrome
| Philadelphia chromosome = t(9:22) translocation
100
what does the blood film show in ALL?
Blast cells
101
What is the cell implicated in CLL?
chronic proliferation of a single type of well differentiated lymphocyte, usually b-lymphocytes
102
Who gets CLL?
Adults aged over 55
| Its the most common leukaemia in adults overall
103
Symptoms of CLL
```
Asymptomatic
Infections
anaemia
Bleeding
wEight loss
Warm autoimmune haemolytic anaemia
```
104
What are the complications of CLL?
CLL can transform into high-grade lymphoma. This is called Richter’s transformation.
anaemia
hypogammaglobinaemia = recurrent infections
warm autoimmune haemolytic anaemia
105
What is Richter's transformation?
When CLL transforms into a high grade lymphoma
leukaemia cells enter the lymph node and change into a high-grade, fast-growing non-Hodgkin's lymphoma. Patients often become unwell very suddenly.
106
What is seen on blood films of CLL?
Smear or smudge cells
107
What are the phases of illness seen in chronic myeloid leukaemia?
1. Chronic phase - lasts around 5 years, asymptomatic, diagnosis incidental on raised WCC
2. Accelerated phase - abnormal blast cells take up a high proportion of cells in the bone marrow & blood (10-20%) = pancytopenia
3. Blast phase - involves an even higher proportion of blast cells in the bone marrow and blood (>30%) = severe pancytopenia symptoms (often fatal)
108
What chromosome abnormality is associated with CML?
Philadelphia chromosome t(9:22) translocation
| = translocation of genes between chromosome 9 and 22
109
Which is the most common acute leukaemia seen in adults?
AML
110
What is seen on blood film of AML?
Blast cells - high proportion. The blast cells have Rods inside their cytoplasm = AUER RODS
111
What can cause AML?
Can be the result of transformation from a myeloproliferative disorder like polycythemia ruby vera or myelofibrosis
112
Mx of leukaemia
Chemotherapy & steroids to induce remission
To ensure remission is consolidated, do a bone marrow transplant
Can do radiotherapy and surgery
Closely monitor for 3 years to ensure remission is maintained
113
complications of chemotherapy
```
Failure
Stunted growth and development in children
Infections due to immunodeficiency
Neurotoxicity
Infertility
Secondary malignancy
Cardiotoxicity
Tumour lysis syndrome
```
114
What is lymphoma?
A group of cancers that affect the lymphocytes inside the lymphatic system
Cancerous cells proliferate within the lymph nodes and cause lymphadenopathy
115
What are the 2 main categories of lymphoma?
Hodgkin's lymphoma - specific disease
| Non-Hodgkin's lymphoma - encompasses all other lymphomas
116
What is Hodgkins lymphoma?
Caused by proliferation of lymphocytes
117
who gets Hodgkins lymphoma?
Bimodal age distribution
| Peaks at 20 and 75 years
118
Risk factors for Hodgkins lymphoma
HIV
Epstein-Barr Virus
Autoimmune conditions such as rheumatoid arthritis and sarcoidosis
Family history
119
Symptoms of Hodgkins lymphoma
Lymphadenopathy - non tender rubbery, pain in the lymph nodes when drinking alcohol
```
Fever
Weight loss
Night sweats
Fatigue
Itching
coughing
SOB
Abdominal pain
Recurrent infections
```
120
Ix for Hodgkins lymphoma
Lactate dehydrogenase (LDH) - raised in HL but can be raised in other cancers
Lymph node biopsy
Reed-Sternberg cells on lymph biopsy
CT/MRI/PET scans for staging
121
How is lymphoma staged?
Ann Arbor staging
About whether affected lymph nodes are above or below the diaphragm
- stage 1 = confined to one region of lymph nodes
- Stage 2 = in more than one region but same side of the diaphragm
- Stage 3 = affects lymph nodes both above and below the diaphragm
- Stage 4 = Widespread involvement including non lymphatic organs like the lungs or liver
122
Mx of Hodgkins lymphoma
Chemotherapy
Radiotherapy
Remission is usually achieved but there is risk of relapse
123
Risks of chemotherapy in lymphoma mx
Leukaemia
| Infertility
124
Risks of radiotherapy in lymphoma mx
cancer due to damage to tissues
| hypothyroidism
125
Name 3 important non-hodgkins lymphomas
1. Burkitt lymphoma
2. MALT lymphoma (mucosa associated lymphoid tissue)
3. Diffuse large B cell lymphoma
126
What are infections associated with Burkitt lymphoma?
EBV
Malaria
HIV
127
Where is MALT lymphoma and what is the infection its associated with?
Mucosa associated lymphoid tissue around the stomach
| Is associated with H pylori
128
How does diffuse large B cell lymphoma present?
Rapidly growing painless mass in over 65 yo
129
Risk factors for non-Hodgkins lymphoma
```
HIV
Epstein-Barr Virus
H. pylori (MALT lymphoma)
Hepatitis B or C infection
Exposure to pesticides and a specific chemical called trichloroethylene used in several industrial processes
Family history
```
130
Mx of non-hodgkins lymphoma
```
Watchful waiting
Chemotherapy
Monoclonal antibodies such as rituximab
Radiotherapy
Stem cell transplantation
```
131
What are the 3 types of polycythaemia?
Relative
Primary (polycythaemia rubra vera)
Secondary
132
Causes of relative polycythaemia
Dehydration
| Stress - Gaisbock syndrome
133
Causes of secondary polycythaemia
COPD
altitude
obstructive sleep apnoea
excessive erythropoietin: cerebellar haemangioma, hypernephroma, hepatoma, uterine fibroids*
134
How do you differentiate between primary/secondary polycythaemia and relative polycythaemia?
Red cell mass studies
| Males >35 and female >32 in true polycythaemia
135
symptoms of polycythaemia vera
```
hyperviscosity
pruritus, typically after a hot bath
splenomegaly
haemorrhage (secondary to abnormal platelet function)
plethoric appearance
hypertension in a third of patients
low ESR
```
136
What is the mutation associated with polycythaemia vera?
Mutation in JAK2 is present in 95% of patients
137
what is polycythaemia vera?
a myeloproliferative disorder caused by clonal proliferation of a marrow stem cell leading to an increase in red cell volume, often accompanied by overproduction of neutrophils and platelets.
138
Peak incidence of polycythaemia
60's
139
Ix for polycythaemia vera
```
FBC - raised haemltocrit, neutrophils, basophils, platelets
Blood film
JAK2 mutation
Serum ferritin
U&Es
LFTs
```
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Mx of polycythaemia vera
1. aspirin = reduces the risk of thrombotic events
2. venesection = first-line treatment to keep the haemoglobin in the normal range
3. chemotherapy
- hydroxyurea - slight increased risk of secondary leukaemia
- phosphorus-32 therapy
141
Complications of polycythaemia vera
thrombotic events
progression to myelofibrosis
progression to acute leukaemia (risk increased with hydroxyurea chemotherapy)
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what are the blood products used in the reversal of warfarin?
1. Stop warfarin
2. Vitamin K - IV takes 4-6hrs to work, oral takes 24 hrs to work
3. Fresh frozen plasma
4. Human prothrombin complex = reversal within 1 hour
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How fast should a unit of RBCs be transfused in a non urgent scenario?
90-120 minutes
144
What do you see on a blood film for iron deficiency anaemia?
target cells
'pencil' poikilocytes
if combined with B12/folate deficiency a 'dimorphic' film occurs with mixed microcytic and macrocytic cells
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What do you see on a blood film for myelofibrosis?
'tear-drop' poikilocytes
146
what do you see on a blood film for Haemolysis?
Schistocytes
147
What is neutropenic sepsis?
a relatively common complication of cancer therapy, usually as a consequence of chemotherapy.
It may be defined as a neutrophil count of < 0.5 * 109 in a patient who is having anticancer treatment and has one of the following:
a temperature higher than 38ºC or
other signs or symptoms consistent with clinically significant sepsis
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When does neutropenic sepsis tend to occur?
7-14 days after chemotherapy
149
Prophylaxis for neutropenic sepsis?
Given if patients have a neutrophil count of less than < 0.5 * 109 as a consequence of their treatment
FLUOROQUIOLONE
150
Mx for neutropenic sepsis
Start Abx immediately, don't wait for WCC
Piperacillin + tazobactam IV
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What suggests a poor prognosis in Hodgkins lymphoma?
'B' symptoms imply a poor prognosis
- weight loss > 10% in last 6 months
- fever > 38ºC
- night sweats
152
What are the key symptoms of a transfusion associated circulatory overload?
Pulmonary oedema, Hypertension, raised jugular venous pulse, afebrile, S3 present.
153
What are the key symptoms of a transfusion related acute lung injury?
Hypoxia, pulmonary infiltrates on CXR, Hypotension, pyrexia, normal/unchanged JVP
154
symptoms of richter transformation
CLL > NHL
```
lymph node swelling
fever without infection
weight loss
night sweats
nausea
abdominal pain
```
155
What is multiple myeloma?
a neoplasm of the bone marrow plasma cells
156
when is the peak incidence for multiple myeloma?
60-70 year olds
157
Symptoms of multiple myeloma
Bone disease - bone pain, osteoporosis, pathological fractures, osteolytic lesions
Lethargy
Infection
Hypercalcaemia
Renal failure
Other features:
- amyloidosis
- macroglossia
- carpal tunnel syndrome
- neuropathy
- hyper viscosity
158
Ix for multiple myeloma
Monoclonal antibodies in the serum and urine = Bence Jones proteins
Increased plasma cells in bone marrow
MRI whole body - for bone lesions
Xray = rain drop skull
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What is the diagnostic criteria for multiple myeloma?
one major and one minor criteria or three minor criteria in an individual who has signs or symptoms of multiple myeloma
MAJOR:
- plasmocytoma
- 30% plasma cells in bone marrow sample
- Elevated levels of M protein in blood/urine
MINOR:
- 10-30% plasma cells in bone marrow
- Minor elevations in level of M protein in blood/urine
- Osteolytic lesions
- Low levels of antibodies in blood
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What is myelofibrosis?
a myeloproliferative disorder
thought to be caused by hyperplasia of abnormal megakaryocytes
the resultant release of platelet derived growth factor is thought to stimulate fibroblasts
haematopoiesis develops in the liver and spleen
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Symptoms of myelofibrosis
- anaemia
- massive splenomegaly
- weight loss, night sweats
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Ix findings for myelofibrosis
Anaemia
High WBC and platelet count
Tear drop poikilocytes on blood film
Unattainable bone marrow biopsy - 'dry tap' = do trephine biopsy
high rate and LDH (due to increased cell turnover)
