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Year 6 > Haematology > Flashcards

Haematology Flashcards

1
Q

Causes of microcytic anaemia

Causes of macrocytic anaemia

A 
Microcytic anaemia 
FAST
Fe deficiency
Anaemia of chronic disease
Sideroblastic anaemia
Thalassaemia

Macrocytic anaemia

Megalaloblastic: B12 or folate deficiency, Anti-folate drugs (phenytoin, methotrexate), Cytotoxics: Hydroxycarbamide

Non-megaloblastic
Reticulocytes
Alcohol
Liver disease
Pregnancy
Hypothyroid
Myelodysplasia, MPD, MM, AA
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2
Q

Causes of fe deficiency anaemia

Investigations and findings

Rx

A

Blood loss e.g. GI
Increased need: Pregnancy, Growth
Reduced intake (e.g. veg)
Decreased absorption e.g. Coeliac disease, gastrectomy. H.pylori infection. Could be Chron’s

Clinical features
Pallor, lethargy, SOB
Atrophic glossitis, Angular stomatitis, Koilonychia
Brittle hair, brittle nails
Post cricoid webs - Plummer vinson syndrome

Bloods - Haematinics

  • Low iron
  • low ferritin
  • High TIBC
  • Reduced transferrin saturation, inctreased levels
Blood film
- Hypochromic cells
- Anisocytosis (size)
- Poikilocytosis (shape)
- Pencil cells
COLONOSCOPY if unexplained anaemia (upper + lower) in >60yo. TTG if young.

Rx
Ferrous sulphate 200mg TDS - continue for 3 months after normalised levels

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3
Q

Causes of normocytic anaemia

A 
BM failure
Blood loss (acutely)
Hypothyroid
Haemolytic anaemia
Pregnancy
Anaemia of chronic disease
Renal failure
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4
Q

Anaemia with macrocytosis
Increased reticulocytes
Polychromasia

A

Haemolytic anaemia

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5
Q

Lab changes in haemolytic anaemia

Common

Specific to intravascular

Specific to extravascular

A

All:
Reticulocytes, polychromasia

Intravascular:
Hyperbillibrubinaemia
LDH high
Absent haptoglobins
Haemoglobinuria
Haemosiderinuria (brown urine)

Extravascular

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6
Q

Types of acquired haemolytic anaemia (just name)

A

Immune-mediated DAT +ve

  • AIHA: Warm, cold, PCH
  • Drugs: Penicillin, quinine, methyldopa
  • Alo-immuneL Acute transfusion retain, HDFN

PNH

Mechanical

  • MAHA: DIC, HUS, TTP
  • Heart valve

Infections
- Malaria

Burns

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7
Q

Types of hereditary haemolytic anaemia (just name)

A

Enzyme: G6PD and pyruvate kinase deficiency

Membrane: Hereditary spherocytosis, Hereditary Eliptocytosis

Haemoglobinopathy: SCD, Thalassaemia

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8
Q

Fe deficiency anaemia signs

Management

A

Koilonychia
Angular stomatitis/cheilosis
Post-cricoid webs

Ferrous suplhate 200mg PO TDS
SE: GI upset
Continue until 3 months symptom free

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9
Q

Symptoms of any anaemia

Signs

A 
Fatigue
Dyspnoea
Faintness
Palpitations
Headache
Tinnitus
Signs
Pallor
Hyperdynamic circulation
- Tachycardia
- Flow murmur: apical ESM
- Cardiac enlargement
Ankle swelling with HF
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10
Q

What is sideroblastic anaemia

Causes

Blood results

A

Ineffective erythropoeisis

  • Increased iron absorption
  • Iron loading in BM –> Ringed sideroblasts
  • Haemosiderosis: ends, liver and cardiac damage
Causes
Congenital
Acquired
- Myelodysplastic/myeloproliferative disease
- Drugs: Chemo, anti-TB, Lead

Bloods

  • Microcytic anaemia, not responsive to iron
  • High ferritin, high serum iron, normal TIBC

Treatment

  • Remove cause
  • Pyridoxine may help
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11
Q

Complications of regular iron transfusions in beta Thalassaemia major

How are they avoided

A

Iron deposition – the most important (all patients):
• Heart – cardiomyopathy
• Liver – cirrhosis
• Pancreas – diabetes
• Pituitary gland – impaired growth and sexual maturation
• Skin – hyperpigmentation

Antibody formation (10% of children)
•	Allo-antibodies to transfused red cells in the patient make finding compatible blood very difficult

Infection – now uncommon (<10% of children)
• Hepatitis A, B, C
• HIV
• Malaria
• Prions (e.g. new variant Creutzfeldt-Jakob disease)

Venous access (common problem)
• Often traumatic in young children
• Central venous access device (e.g. Portacath) may be required; these predispose to infection

Avoided by giving SC desferrioxamine Fe chelation

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12
Q

Beta Thalassaemia major features

Management

Blood results

A 
From 3-6 months age:
Severe anaemia
Jaundice
FTT
Extra-medullary erythropoiesis:
- Frontal bossing
- Maxillary overygrowth
- Hepatosplenomegaly
Signs of iron deposition
- Heart: Cardiomyopathy
- Liver: Cirrhosis
- Pituitary
- Pancreas: Diabetes
- Skin: Hyperpigmentation

Management

  • Life long transfusions
  • Desferrioxamine SC Fe chelation
  • BM transplant is only curative option: Require HLA identical sibling

B0/B0, B0/B+, B+/B+

bloods
Low Hb, Low MCV, increased reticulocytes
Hb electrophoresis: Increased HbF, HbA2 variable
Film: Target cells and nucleated RBCs

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13
Q

Alpha Thalassaemia types

A

Alpha Thalassaemia major/ Hb Barts
0 alpha globin gene: –/–
Hydrops fetalis –> death in utero

HbH disease
1 alpha globin gene a-/–
Moderate anaemia; may need transfusions
Haemolysis: Hepatosplenomegaly, jaundice

Train
2 alpha globin genes: –/aa or a-/a-
Asymptomatic
Hypochromic microcytes

Normal is aa/aa - 4 alpha globin

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14
Q

Beta Thalassaemia trait/hetrozygosity
Genes
Features
Blood results

A

B/B+ or B/B0
Mild anaemia which is usually harnless
Low MCV
Increased HbA2 and increased HbF

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15
Q

Hypersegmented PMN

Oval macrocytes

A

B12/Folate deficiency

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16
Q

Investigations for microcytic anaemia

A

LFTs
TFTs
Se B12
Red cell folate: Reflects body stores over 2-3mon

BM biopsy if cause not revealed by above

  • Megaloblastic erythropoiesis
  • Giant metamyelocytes
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17
Q

Folate deficiency

Folate sources

Causes

Absorption location

Blood + blood film results

Management

A

Folate sorce: Green veg, nuts, liver
Stores 4months

Absorption: Proximal JEJENUM

Causes of folate deficiency

  • Reduced intake: Poor diet
  • Increased demand: Pregnancy, Haemolysis, Malignancy
  • Malabsorption: Coeliac, Chrons
  • Drugs: EtOH, Phenytoin, Methotrexate

Bloods:
As for microcytic anaemia: LFT, TFT, SeB12, Red cell folate
Film: Hypersegmented PMN, Oval macrocytes

Management

  • Assess for underlying cause
  • Give B12 first unless B12 known to be normal - may precipitate or worsen SACD
  • Folate 5mg/d PO
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18
Q

B12 deficiency

Source
Stores
Absorption

Causes of b12 deficiency

Features

Investigations

A

Source: Meat, fish and dairy (vegans at risk)
Stores: 4y
Absorption: Terminal ileum bound to intrinsic factor (released from gastric parietal cells)
Role: DNA and myelin synthesis

Causes

1) Reduced intake: Vegan
2) Reduced intrinsic factor: Pernicious anaemia, Post-gastrectomy
3) Terminal ileum: Chron’s, ileal resection, bacterial overgrowth

Features

1) General
- Anaemia symptoms
- Lemon tinge: pallor and mild jaundice
- Glossitis: Beefy red tongue
2) Neuro
- Paraesthesia
- Peripheral neuropathy
- Optic atrophy
- SACD = subacute combined degeneration of the cause

Investigations

  • IF antibodies: Specific but lower sensitivity
  • Patietal cell Abs: 90% +ve in PA but reduced specificity
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19
Q

What is subacute combined degeneration of the cord

Cause

Pathology

Features

A

Cause = pernicious anaemia
= Combined symmetrical dorsal column loss and corticospinal tract loss
–> Distal sensory ataxia esp joint position + vibration
–> Ataxia with wide gait and +ve Romberg

Mixed UMN and LMN signs

  • Spastic paraparesis
  • Brisk knee jerks
  • Absent ankle jerks
  • Upgoing plantars

Pain and temperature remain intact

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20
Q

B12 deficiency managament

A

Malabsorption ==> Hydroxocobalmin parenteral

  • Replenish 1mg/48h IM
  • Maintain: 1mg IM every 3 months

Dietary ==> Oral B12 (Cyancobalamin)

Parenteral B12 reverses neuropathy but not SACD

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21
Q

CLL
Epidemiology

Aetiology

Features

Investigations

A

Commonest leukaemia WW
M>F: 2:1
Elderly 70s

Aetiology
- Clones of mature B cells (memory cells)

Features

  • often asymptomatic incidental finding
  • Symmetrical PAINLESS lymphadenopathy
  • Hepatosplenomegaly
  • B symptoms: Weight loss, fever, night sweats

Investigations

  • Increased WCC: Lymphocytes
  • Smear cells
  • Decreased serum Ig - (infections)
  • +ve DAT!!!
  • Rai or Binet staging
  • Immunophenotype!!!
22
Q

CLL

Complications

Treatment

Staging scale

A

Complications

  • Autoimmune Haemolysis
  • Infection
  • Marrow failure/infiltration

Natural hx

  • Some remain stable for yrs
  • Nodes usually enlarge slowly +/- obstruction
  • Death often due to infection
  • RICHTER transformation: CLL –> LArge B cell lymphoma

Treatment

Indications to treat

  • symptomatic
  • Ig genes un-mutated (bad prognosis)
  • 17p deletion (bad prognosis)

Supportive
Chemo
Radiotherapy: Relieve LN or splenomegaly

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

Binet
A: <3 areas of lymphadenopathy
B: 3 or more areas of lymphadenopathy
C: Hb <10 or Put <100

23
Q

Massive leucocytosis 50-200
Neutrophils and myelocytes
Basophils

Hepatosplenomegaly

A

CML
9;22

Chronic phase –> Advanced phase –>Blast crisis
<5% blasts 10-19% 20 + blasts

Management
- Imatinib

24
Q

AML vs ALL diagnosis

A

20% BLAST BM cells
Cytology: AML have auer rods
Cytochemistry: AML +ve staining for myeloperoxidase, sudan black, non-specific esterase

Immunophenotyping:
AML
- MPO, CD13, CD33, CD14, CD15, glycophorin
ALL
- Precursor B cell - CD19, CD20, TdT, CD10 +/-ve
- B cell - CD19, CD20, sIg
- T-cell: CD2, CD3, CD4, CD8, TdT

Both AML and ALL have CD-34, CD45, HLA-DR

25
Q

AML clinical features

AML diagnosis

A

BM failure: Anaemia, thrombocytopenia, neutropenia
Local infiltration: Hepatosplenomagaly, gum infiltration, lymphadenopathy (much less than ALL), Skin or other sites

Blood count and film
BM morphology
Immunophenotyping - more so to follow up disease
Cytogenic analysis on BM - affects Rx and guides prognosis
Molecular studies (when needed)

26
Q

AML treatment

A

Supportive
- Red cells (in anaemia), platelets (if <10 or bleeding), FFP (if DIC), Antibiotics, Long line, Allopurinol (prevent breakdown of blood products leading to renal failure), fluid and electrolyte balance

Chemo - damages DNA of leukaemic cells as they are continuously dividing and lack cell cycle checkpoint control
- usually combination chemo 4-5 courses ~ 6 months

Consider transplant if poor prognosis

Nb: ATRA for promyelocytic leukaemia (DIC)
TK inhibitors for rare PH +ve

27
Q

ALL features

A

PB: anaemia, thrombocytopenia, neutropenia

BM and other organs - lymphadenopathy (v often), Hepatosplenomegaly, Tests, CNS, kidneys, Bone (pain in long bones)

28
Q

Hodgkin lymphoma staging

A 
Ann arbor staging
I; one group of nodes
II: >1 group nodes same side diaphragm
III; nodes above and below diaphragm
IV: extra-nodal spread
B if fever, night sweats, unexplained weight loss
Chemo - preserves fertility. 2-6 cycles
Adriamycin (se cardiomyopathy)
Bleomycin (se pulmonary fibrosis)
Vinblastine
DTIC

+/-

Radiotherapy: field only
(risk of breast Ca after 25y, Leukaemia 3% after 10yrs, lung/skin cancer)

PET-CT after 2 cycle ABVD to assess response and guide radiotherapy need at end of chemo

Prognosis ~ 50-90% cure
Stage I or II: 80% cured
IV: Only 50% cured

29
Q

Hodgkin lymphoma epidemiology

Features

Investigations

A

M>F: 2:1
Bimodal age incidence 20-29 and >60

NB HL only 20% of lymphoma

Features

1) Lymphadenopathy
- Painless
- Asymmetric
- Spreads CONTINUOUSLY to adjacent LNs
- May be alcohol LN induced pain

2) B symptoms
- Fever
- NIght sweats
- Weight loss (>10% over 6 monthS)

3) Other
- Itch
- Pel ebstein fever (cyclical)
- Hepato and or splenomegaly

Biopsy: reed stein berg cells!!!
CT/MRI CAP

30
Q

G6PD

Pathophysiology

Features

Diagnosis

Causes

A

Glucose-6-phosphate dehydrogenase (G6PD) deficiency is the commonest red blood cell enzyme defect. It is more common in people from the Mediterranean and Africa and is inherited in an X-linked recessive fashion. Many drugs can precipitate a crisis as well as infections and broad (fava) beans

Pathophysiology
↓ G6PD → ↓ glutathione → increased red cell susceptibility to oxidative stress

Features
neonatal jaundice is often seen
intravascular haemolysis
gallstones are common
splenomegaly may be present
Heinz bodies on blood films. Bite and blister cells may also be seen

Diagnosis is made by using a G6PD enzyme assay

Some drugs causing haemolysis
anti-malarials: primaquine
ciprofloxacin
sulph- group drugs: sulphonamides, sulphasalazine, sulfonylureas

Some drugs thought to be safe
penicillins
cephalosporins
macrolides
tetracyclines
trimethoprim
31
Q

Platelet transfusion indications

Pre-procedure

A

ffer platelet transfusions to patients with a platelet count of <30 x 10 9 with clinically significant bleeding (World Health organisation bleeding grade 2- e.g. haematemesis, melaena, prolonged epistaxis)

Platelet thresholds for transfusion are higher (maximum < 100 x 10 9) for patients with severe bleeding (World Health organisation bleeding grades 3&4), or bleeding at critical sites, such as the CNS.

It should be noted that platelet transfusions have the highest risk of bacterial contamination compared to other types of blood product.

Pre-invasive procedure (prophylactic)

Platelet transfusion for thrombocytopenia before surgery/ an invasive procedure. Aim for plt levels of:
> 50×109/L for most patients
50-75×109/L if high risk of bleeding
>100×109/L if surgery at critical site

If no active bleeding or planned invasive procedure

A threshold of 10 x 109 except where platelet transfusion is contradindicated or there are alternative treatments for their condition

For example, do not perform platelet transfusion for any of the following conditions:
Chronic bone marrow failure
Autoimmune thrombocytopenia
Heparin-induced thrombocytopenia, or
Thrombotic thrombocytopenic purpura.
32
Q

DIC features

Causes

Treatment

A

low platelets, low fibrinogen
prolonged prothrombin time, APTT and bleeding time
Schistiocytes

Causes

  • Sepsis
  • Malignancy (esp APML)
  • Trauma
  • Obs

Treatment

  • FFP
  • Platelets
  • Heparin
33
Q

Tumour lysis syndrome criteria

A 
Positive laboratory TLS requires 2 or more of the below within 7 days of chemotherapy or 3 days before:
uric acid > 475umol/l or 25% increase
potassium > 6 mmol/l or 25% increase
phosphate > 1.125mmol/l or 25% increase
calcium < 1.75mmol/l or 25% decrease

Positive clinical TLS requires any one of:
increased serum creatinine (1.5 times upper limit of normal)
cardiac arrhythmia or sudden death
seizure

34
Q

Causes of thrombocytopenia

A 
Causes of severe thrombocytopenia
ITP
DIC
TTP
haematological malignancy
Causes of moderate thrombocytopenia
heparin induced thrombocytopenia (HIT)
drug-induced (e.g. quinine, diuretics, sulphonamides, aspirin, thiazides)
alcohol
liver disease
hypersplenism
viral infection (EBV, HIV, hepatitis)
pregnancy
SLE/antiphospholipid syndrome
vitamin B12 deficiency
35
Q

Myeloma cause

A 
The most likely underlying diagnosis is myeloma. Myeloma typically presents with the CRAB features:
Elevated calcium
Renal failure
Anaemia
Bone pain

The stem states this lady is in the early stages of disease. Therefore it is unlikely there are any bony metastases. In the early stages of disease the most likely biochemical pattern to see in myeloma as:
Raised calcium
Normal or high phosphate
Normal alkaline phosphate

36
Q

Types of Hodgkin lymphoma

A 
Type	Frequency	Prognosis	Notes
1) Nodular sclerosing	
Most common (around 70%)
Good prognosis	
More common in women. 
Associated with lacunar cells

2) Mixed cellularity
Around 20%
Good prognosis
Associated with a large number of Reed-Sternberg cells

3) Lymphocyte predominant
A*round 5%
Best prognosis

3) Lymphocyte depleted
Rare
Worst prognosis

37
Q

Aplastic anaemia

Characteristics

Causes

A

Characterised by pancytopenia and a hypoplastic bone marrow

Peak incidence of acquired = 30 years old

Features
normochromic, normocytic anaemia
leukopenia, with lymphocytes relatively spared
thrombocytopenia
may be the presenting feature acute lymphoblastic or myeloid leukaemia
a minority of patients later develop paroxysmal nocturnal haemoglobinuria or myelodysplasia

Causes
idiopathic
congenital: Fanconi anaemia, dyskeratosis congenita
drugs: cytotoxics, chloramphenicol, sulphonamides, phenytoin, gold
toxins: benzene
infections: parvovirus, hepatitis
radiation

38
Q

vWD

Types

Investigation

Management

A

Most common inherited bleeding disorder. The majority of cases are inherited in an AUTOSOMAL DOMINANT and characteristically behaves like a platelet disorder i.e. epistaxis and menorrhagia are common whilst haemoarthroses and muscle haematomas are rare

Role of von Willebrand factor
large glycoprotein which forms massive multimers up to 1,000,000 Da in size
- promotes platelet adhesion to damaged endothelium (via gp1b)
- carrier molecule for factor 8

Types
type 1: partial reduction in vWF (80% of patients)
type 2*: abnormal form of vWF
type 3**: total lack of vWF (autosomal recessive)

Investigation
prolonged bleeding time
APTT may be prolonged
factor VIII levels may be moderately reduced
defective platelet aggregation with ristocetin

Management

  • Transexamic acid for mild bleeding
  • Desmopressin (DDAVP): raises levels of vWF by inducing release of vWF from Weibel-Palade bodies in endothelial cells
  • Factor VIII concentrate
39
Q

What is thrombophilia

Investigations

A

Coagulopathy predisposing to a thrombosis, usually venous

Inherited

  • Factor V Leiden/APC resistance
  • Prothrombin Gene Mutation
  • Protein C and S deficiency

Acquired

  • Progesterones in OCP
  • Anti-phospholipid syndrome

Investigations

  • FBC, Clotting, Fibrinogen conc
  • Factor V Leiden/APC resistance
  • Lupus anticoagulant and ant–cardiolipin Abs
  • Assays for AT, Protein C and S deficiencies
  • PCR for prothrombin gene mutation
40
Q

Factor V Leiden other name

Pathophysiology

A

APC resistance

Protein C normally deactivates F5 and F8

41
Q

Immediate blood transfusion reactions

A

1) Haemolytic (mins)
- Agitation, fever, abdo/chest pain,
- Hypotension –> shock
- DIC –> haemorrhage
- Renal failure
Mechanism = ABO incompatibility –> IV Haemolysis

Rx: Stop transfusion. Tell lab, FLUID RESUS, Treat DIC

2) Non haemolytic febrile reaction
- Fever, rigors, chills
Mechanism: Due to WBC HLA antibodies (of recipient)

Rx: Slow transfusion. Paracetamol 1g

3) Allergic reaction (immediate)
- Urticaria, itch - but ranges in severity:
- Angiodema
- Anaphylaxis
Mechanism: Recipient IgA deficiency, Anti-IgA IgE

Rx: Discontinue and give antihistamine e.g. chlorephenamine 10mg/IV/IM. Can restart transfusion after symptoms settle. If more severe - discontinue and treat urgently

4) TRALI
A rare but potentially fatal complication of blood transfusion.
- Development of hypoxaemia / acute respiratory distress syndrome within 6 hours of transfusion. Features include:
- hypoxia
- pulmonary infiltrates on chest x-ray
- fever
- hypotension
Mechanism: Anti-WBC Abs in donor plasma

Rx: Stop transfusion, Rx ARDS

5) TACO
A relatively common reaction due to fluid overload resulting in pulmonary oedema. As well as features of pulmonary oedema the patient may also be HYPERTENSIVE a key difference from patients with TRALI.

42
Q

Warfarin patients with High INR managemet

A

Major bleeding

  • Stop warfarin
  • Give intravenous vitamin K 5mg
  • Prothrombin complex concentrate - if not available then FFP*

INR > 8.0 with Minor bleeding

  • Stop warfarin
  • Give IV vitamin K 1-3mg
  • Repeat dose of vitamin K if INR still too high after 24 hours
  • Restart warfarin when INR < 5.0

INR > 8.0 No bleeding

  • Stop warfarin
  • Give vitamin K 1-5mg by mouth, using the intravenous preparation orally
  • Repeat dose of vitamin K if INR still too high after 24 hours
  • Restart when INR < 5.0

INR 5.0-8.0 Minor bleeding

  • Stop warfarin
  • Give IV vitamin K 1-3mg
  • Restart when INR < 5.0

INR 5.0-8.0 No bleeding

  • Withhold 1 or 2 doses of warfarin
  • Reduce subsequent maintenance dose
43
Q

What is FFP used for

Universal donor

A

‘Clinically significant’ but without ‘major haemorrhage’ in patients with a prothrombin time (PT) ratio or activated partial thromboplastin time (APTT) ratio > 1.5
typically 150-220 mL

Can be used prophylactically in patients undergoing invasive surgery where there is a risk of significant bleeding

In contrast to red cells, the universal donor of FFP is AB blood because it lacks any anti-A or anti-B antibodies

44
Q

What does cryoprecipitate contain

Uses

A

Contains concentrated Factor VIII:C, von Willebrand factor, fibrinogen, Factor XIII and fibronectin, produced by further processing of Fresh Frozen Plasma (FFP).

Clinically it is most commonly used to replace fibrinogen
much smaller volume than FFP, typically 15-20mL
most suited for patients for ‘clinically significant’ but without ‘major haemorrhage’ who have a fibrinogen concentration < 1.5 g/L

Example use cases include

  • DIC
  • Liver failure
  • Hypofibrinogenaemia secondary to massive transfusion.

It may also be used in an emergency situation for haemophiliacs (when specific factors not available) and in von Willebrand disease
Can be used prophylactically in patients undergoing invasive surgery where there is a risk of significant bleeding where the fibrinogen concentration < 1.0 g/L

45
Q

What is haemophilia

Types

Features

Blood tests

Managements

A

Haemophilia is a X-linked recessive disorder of coagulation. Up to 30% of patients have no family history of the condition.

Haemophilia A is due to a deficiency of factor VIII whilst in Haemophilia B (Christmas disease) there is a lack of factor IX

Features
haemoarthroses, haematomas
prolonged bleeding after surgery or trauma

Blood tests
Prolonged APTT - only abnormal
bleeding time, thrombin time, prothrombin time normal

Up to 10-15% of patients with haemophilia A develop antibodies to factor VIII treatment.

Management

  • Avoid NSAIDs and Im injections
  • Minor bleeds: Desmopressin + transexamic acid
  • Major bleeds: rhF8 (if A)
46
Q

Schistiocyte causes

A

MAHA

Mechanical valve

47
Q

Treatment of DVT/PE

A

1) LMWH (tinzaparin 175) + warfarin
- stop LMWH once INR >2 for 2 days
- Continue for 3-6 months

2) Start DOAC and continue for 3-6 months

If cancer pt, continue LMWH not warfarin

48
Q

Target cells causes

A

Hyposplenism
Thalassaemia
Liver disease

49
Q

RBC with a purple spot in it

Name
Conditions that cause it

A

Howell-Jolly bodies
- nuclear remnants

1) Post splenectomy
2) Hyposplenism
(E.g. sickle cell disease, coeliac, IBD, myeloproliferative, amyloid)
3) Megaloblastic anaemia

50
Q

Platelets >600 x 10^9

Thrombosis/bleeding, headaches

Diagnosis
Mutations
Management

A

ET

Mutations in JAK2, calreticulin and MPL

Rx: Aspirin
Hydroxycarbamide
Anagrelide (not commonly)

51
Q

Non-hodgkin lymphoma

Features

A

80% of lymphoma

1) Lymphadenopathy
- PAINLESS
- SYMMETRIC
- Spreads DISCONTINUOUSLY
- Multiple sites

2) Extranodal
- Skin esp T cell lymphomas
- CNS
- Oropharynx + GIT
- Splenomegaly

3) B Symptoms
- Fever
- Night sweats
- Weight loss (>10% in 6 months)

4) Blood
- Pancytopenia
- Hyperviscosity

52
Q

Non-hodkin lymphoma investigations

A 
FBC, U&amp;Es, LFT, LDH
- LDH high = worse prognosis
Film
- Normal or circulating lymphoma cells 
- +/- pancytopenia
Classification: LN and BM biopsy 
Staging: CT/MRI CAP
- Ann Arbor staging
I; one group of nodes
II: >1 group nodes same side diaphragm
III; nodes above and below diaphragm
IV: extra-nodal spread
B if fever, night sweats, unexplained weight loss

Year 6 (21 decks)

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