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CP3 Medicine > Haematology 1 > Flashcards

Haematology 1 Flashcards

1
Q

What is anaemia?

A

Decreased Hb in the blood such that there is inadequate oxygen delivery to tissues

Hb <135g/L in men
Hb <115g/L in women

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2
Q

What are the symptoms of anaemia?

A

non-specific: fatigue, weakness, headaches

CV: dyspnoea on exertion, angina, intermittent claudication, palpitations

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3
Q

What are the signs of anaemia?

A

pallor
tachycardia
systolic flow murmur
cardiac failure

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4
Q

What are the specific signs for certain types of anaemia?

A

kolionychia: spoon shaped nails in IDA
jaundice: in haemolytic anaemia
leg ulcers: often seen in sickle cell disease
bone marrow expansion: leading to abnormal facial structure or pathological fracture

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5
Q

What is MCV?

A

size of each red blood cell

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6
Q

What is MCH?

A

amount of haemoglobin each red blood cell

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7
Q

What is classed as low MCV?

A

<80fL (microcytic anaemia)

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8
Q

What are the causes of low MCV?

A 
T – Thalassaemia
A – Anaemia of chronic disease
I – Iron deficiency anaemia
L – Lead poisoning
S – Sideroblastic anaemia
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9
Q

What are the causes of normocytic anaemia (normal MCV)

A

acute blood loss
anaemia of chronic disease
renal anaemia
haemolytic anaemia: can be macrocytic due to reticulocytosis

marrow failure
pregnancy
connective tissue disease
dimorphic blood film: combined microcytic / microcytic processes

A – Acute blood loss
A – Anaemia of Chronic Disease
A – Aplastic Anaemia
H – Haemolytic Anaemia
H – Hypothyroidism
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10
Q

What are the causes of macrocytic anaemia >96fL

A 
B12 deficiency
Folate deficiency: coeliac disease
alcohol excess (or severe liver disease) 
myelodysplastic syndromes
Severe hypothyroidism (myxoedema, can be normocytic)
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11
Q

What clinical signs can be looked for with anaemia?

A

kolionychia
angular stomatitis
brittle nails / hair

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12
Q

What further tests can be done to confirm anaemia?

A

Iron studies: serum iron, ferritin, total iron binding capacity, serum soluble transferrin receptors

Blood film: microcytic anaemia = hypo chromic (pale on blood film)
Film may show sideroblasts / signs of thalassemia

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13
Q

Why is the body limited in taking up iron?

A

iron more readily taken up in the gut as ferrous iron (Fe2+) which is less abundant than the insoluble ferric Fe3+ iron

IDA develops when there is inadequate iron for haemoglobin synthesis

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14
Q

What are the causes of IDA?

A

Blood loss: hookworm is the most common cause worldwide

Heavy menstruation 
GI bleeds
Decreased absorption e.g. in coeliacs, patients on antacids 
increased demand; growth / pregnancy 
Inadequate intake
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15
Q

How is IDA diagnosed?

A

Blood film: microcytic, hypochromic cells with poikilocytosis and anisocytosis (pale and small and funny shaped)

serum iron: decreased

total iron binding capacity: increased

serum ferritin: decreased
represents amount of stored iron

Soluble transferrin receptor: increased - MOST SPECIFIC TEST

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16
Q

What are the findings for anaemia of chronic disease?

A

microcytic or normocytic

serum iron will be decreased
TIBC will be decreased and STR will be normal
Ferritin will be raised

FERRITIN Represents increased stored iron, BUT it is an acute phase reactant and raises in infection or malignancy

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17
Q

What are the further investigations for anaemia?

A

If menorrhagia hx - start oral iron
?coeliac

Non-obvious cause of bleeding, refer for GI investigation

OGD and colonoscopy

stool microscopy

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18
Q

What is the management of IDA?

A

Address underlying cause: menorrhagia

Oral ferrous sulphate: 200mg t.d.s and commence before awaiting ix results

Advise increased dietary intake of dark green vegetables, fortified bread/cereals, lean red meat and prunes or raising

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19
Q

How should patents who do not tolerate ferrous sulphate be handled?

A

Switch to ferrous gluconate,

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20
Q

What are teh common side effects of iron supplements?

A

GI related: cramping, bloating, nausea, vomiting, constipation, black stools

adverse effects can be decreased if taken with meals, offering laxatives for constipation or dose reduction

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21
Q

How should iron supplements be monitored?

A

monitor for improvements in symptoms and blood parameters after 1 month; there should be a Hb increase of 20g/L in this time period

Treatment should continue for 3 months after blood parameters return to normal to replenish supplies

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22
Q

Describe the rule of 10s in anaemia?

A

The maximum rise in Hb concentration in one week is 10g/L

if more than 10g/L decline is seen over a week then blood is being lost

When transfusing, one bag will raise the Hb concentration by 10g/L

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23
Q

What is Plummer-Vinson syndrome?

A

Rare disease characterised by dysphagia, odynophagia, IDA, glossitis, chelitis and oesophageal webs

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24
Q

What is the management of Plummer-Vinson syndrome?

A

Iron supplementation and mechanical widening of the oesophagus generally provides an excellent outcome

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25
Q

What is thalassemia?

A

Genetic disorder of Hb synthesis, common in the Middle/Far East

caused by deficient alpha or beta chain synthesis, thus resulting in alpha or Beta thalassemia

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26
Q

What are the traits of B-thalassemia minor?

A

Carrier state: asymptomatic and gives mild microcytic anaemia that may worsen in pregnancy.

Often confused with IDA
HbA2 is raised, with slightly raised HbF also

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27
Q

What is the cause of B-thalassemia major?

A

Abnormality in both globin genes,

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28
Q

What is the presentation of B-thalassemia

A

presents within the first year with severe anaemia, hepatosplenomegaly and FTT

extra-medullary haematopoiesis results in facial deformities

survival is possible due to HbF

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29
Q

What does the blood film for B-thalassemia show?

A

Hypochromic, microcytic cells, also target cells ad nucleated RBCs

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30
Q

What is the management of B-thalassemia?

A

Lifelong blood transfusion

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31
Q

What is alpha thalassemia?

A

alpha chains are coded for by 4 genes
Deletion of all 4 alpha-globing genes, leading to HbBarts

Physiologically useless and leads to death in utero

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32
Q

What does deletion of 3 genes lead to? (alpha)

2 genes?

1 gene?

A

3 genes - moderate microcytic anaemia with features of haemolysis

2: asymptomatic carrier state with reduced MCV
1: clinically normal

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33
Q

What is sideroblastic anaemia?

A

bone marrow produces ringed sideroblasts rather than erythrocytes, which can be seen in the bone marrow

Can be a congenital disorder, or more commonly acquired in myelodysplastic syndrome

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34
Q

How should macrocytic anaemia be investigated

A

Blood film: hypersegmented neutrophils in B12/folate deficiency, may reveal other clues e.g. target cells in liver disease

LFTs/TFTs: thyroid or hepatic cause

Serum B12/folate levels: serum folate reflects recent intake so many labs do red cell folate

If B12 low: Anti-parietal cell Ab, anti-IF ab
Schilling test

Bone marrow biopsy: megaloblasts suggests B12/folate deficiency, also seen in myelodysplasia

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35
Q

What is schilling’s test?

A

distinguishes between pernicious anaemia and small bowel disease (radio labelled B12 given with and then without IF), amount of labelled B12 excreted in urine then detected

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36
Q

What is deoxyuridine suppression test used for?

A

Differentiate B12 folate deficiency in vitro after bone marrow biopsy

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37
Q

How do B12 and folate deficiency lead to megaloblastic anaemia?

A

B12 acts as a co-enzyme for the conversion of folate (B9) to activated folate

activated folate is required for DNA synthesis and thus if there is a deficiency in either B12 or folate, DNA synthesis malfunctions

In this case, the DNA fails to ‘stop’ erythrocyte development, leading to very large cells - which eventually are trapped and destroyed in the reticulo-endothelial system

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38
Q

Where do humans get B12 from?

A

Animal sources e.g. meat, fish, eggs and milk

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39
Q

Where is B12 stored?

A

LIVER, excreted in bile but around 70% is reabsorbed

Free vitamin B12 binds to R proteins in the upper GI tract, but these complexes are degraded by pancreatic proteases

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40
Q

What is IF secreted by?

A

Gastric parietal cells

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41
Q

What is the function of IF?

A

Binds free B12 with far less affinity than R proteins, but the IF-B12 complex is highly resistant to protease degradation.

Receptors for the IF-B12 complex are present on the brush border of the terminal ileum where B12 is absorbed

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42
Q

Why is IF important?

A

Necessary for B12 ingestion

lack of = pernicious anaemia

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43
Q

How are patients with pernicious anaemia managed?

A

IM b12 or high dose PO supplementation

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44
Q

What are the causes of B12 deficiency?

A

Chronic low dietary intake: vegans

Impaired binding in the stomach: pernicious anaemia, congenital absence of IF, gastrectomy

Small bowel disease: resection, Chron’s, backwash ileitis in UC, bacterial overgrowth

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45
Q

What is pernicious anaemia?

A

Autoimmune disease resulting in severe B12 deficiency

3 autoantibodies that may contribute towards disease: autoantibodies against parietal cells
Blocking antibodies (prevent IF-B12 binding)
Binding antibodies (prevent IF binding to ileal receptors)
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46
Q

What is subacute combined degeneration of the cord?

A

Simultaneous posterior column (LMN) and CST (UMN) loss due to B12 deficiency and gives a combination of UMN and LMN signs

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47
Q

What is the presentation of subacute combined degeneration of the cord?

A

Peripheral neuropathy and on examination there is a classical triad of extensor plantars, brisk knee jerk but absent ankle jerks

Tone and power usually normal, gait may be ataxic

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48
Q

How does folate occur in nature?

A

dihydrofolate, tetraydrofolate

found in green vegetables or offal however cooking causes a loss of up to 90%

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49
Q

Where are dhf and thf converted to folate?

A

upper GIT and folate is absorbed in the jejunum

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50
Q

What are the causes of folate deficiency?

A

Poor nutritional intake: poor diet, alcohol excess, anorexia

Malabsorption: coeliac disease

Anti-folate drugs: trimethoprim, methotrexate, anti-convulsants

Excess physiological use: pregnancy, lactation, prematurity

Excess pathological use: excess erythrocyte production (e.g. in haemolysis, malignancy, inflammatory diseases

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51
Q

What is the treatment of folate deficiency?

A

Folic acid 5mg/day PO for 4 months, always with combined B12 unless the patient is known to have a normal B12 level
(folate can precipitate subacute combined degeneration of the cord)

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52
Q

What questions should be asked for normocytic anaemia?

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

is there acute blood loss?
Is there underlying chronic disease
is it haemolytic
are other cell lines affected i.e. bone marrow failures

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53
Q

What is anaemia of chronic disease?

A

normochromic or hypochromic, rarely severe

seen in chronic infection, malignancy, CKD and rheumatoid disorders

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54
Q

What are the lab findings of anaemia of chronic disease?

A

Predominant WBC production in the bone marrow

low serum iron, raised ferritin, low TIBC, normal STR

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55
Q

what are the lab findings of bone marrow failure?

A

HB, reticulocytes, WBC and platelets all equally low
Alterations in the blood film
?abnormal blasts in the marrow

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56
Q

What are the causes of bone marrow failure?

A 
Aplastic anaemia; idiopathic or due to drugs 
haematological malignancies 
metastatic disease
myelofibrosis
myelodysplasia 
parvovirus
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57
Q

What is haemolysis?

A

breakdown of RBC before the end of their normal lifespan (120 days)
The haemolysis can be intravascular or extravascular (reticuloedothelial system of the liver, spleen and bone marrow)

May be asymptomatic but haemolytic anaemia then develops if the bone marrow does not sufficiently compensate

58
Q

What are the inherited causes of haemolytic anaemia

A 
Hereditary Spherocytosis
Hereditary Elliptocytosis
Thalassaemia
Sickle Cell Anaemia
G6PD Deficiency
59
Q

What are the acquired causes of haemolysis?

A 
Autoimmune haemolytic anaemia
Alloimmune haemolytic anaemia (transfusions reactions and haemolytic disease of newborn)
Paroxysmal nocturnal haemoglobinuria
Microangiopathic haemolytic anaemia
Prosthetic valve related haemolysis
60
Q

What investigations should be done for haemolysis?

A

Increased Red cell breakdown signs:
Anaemia with raised MCV
Raised bilirubin: unconjugated, pre-hepatic
Raised serum LDH: released from RBCs

Increased RBC production: reticulocyte count >2% or 100 x 10^9

Blood film

61
Q

What blood film clues are there for haemolysis?

A

Hypochromic, microcytic cells: thalassemia
Sickle cells: sickle cell anaemia
Spherocytes: hereditary spherocytosis, or autoimmune haemolytic anaemia
eliptocyctes: hereditary eliptocytosis
Heinz bodies / ‘bite’ cells: G6PD deficiency
Schistocytes: microangiopathic haemolytic anaemia

62
Q

What further tests can be done for haemolysis?

A

Full blood count shows a normocytic anaemia
Blood film shows schistocytes (fragments of red blood cells)
Direct Coombs test is positive in autoimmune haemolytic anaemia

63
Q

Name the most common haemoglobinopathies

A

Thalassemia

sickle cell disease

64
Q

What is sickle cell anaemia?

A

autosomal recessive disorder causing production of abnormal b-globulin chains due to a single amino acid substitution (glu6val)

resulting in production of HbS rather than HbA

65
Q

In which patients is sickle cella anaemia more common?

A

African

66
Q

What are the two genotypes for sickle cell anaemia?

A

HBSS: sickle-cell anaemia phenotype

HbAS: sickle cell trait - HbAS confers protection from falciparum malaria

Rarely symptomatic BUT vaso-occlusive events may occur in hypoxia (when flying or under anaesthesia)

67
Q

Describe the pathogenesis of sickle cell anaemia

A

HbS polymerises when deoxygenated causing RBCs to form ‘sickle cells’
the sickle cells are fragile and haemolyse and also block small vessels

68
Q

How is diagnosis of sickle cell anaemia done?

A

Guthrie screening card
sickle cells are seen on the blood film
Hb electrophoresis can confirm the diagnosis and also distinguish variants

69
Q

What are the symptoms of sickle cell anaemia?

A

Often presents in the first few months of life - anaemia developing as HbF levels fall

acute haemolytic crises occurring causing bone infarcts and painful dactylitis in the fingers and toes

70
Q

What happens if haemolytic crises goes untreated?

A

repeated splenic infarction leading to hyposplenism, repeated renal infarction causing CKD and CVA

71
Q

What are the complications of SCA?

A 
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
72
Q

What is the chronic treatment of SCA?

A

lifelong folate supplementation
pneumococcal vaccination and prophylactic penicillin due to hyposplenism

Hydroxycarbamide can help by increasing HbF production and is advised if there are frequent crises

Regular lifelong transfusions (2-4 weekly) with iron chelators to prevent overload

Bone marrow transplantation is curative: but limited by availability of matched donors

73
Q

What are the causes of SCA vaso-occlusive crises

A

sickle shaped blood cells clog capillaries causing distal ischaemia

Micro-vascular occlusion, often affecting the bone marrow causing severe pain

74
Q

What triggers a crisis?

A

cold, infection, dehydration, hypoxia

75
Q

What is the presentation of a vaso-occlusive crisis?

A

pain in body

mesenteric ischaemia mimicking the acute abdomen, cerebral infarction, priapism

76
Q

What is an aplastic crisis caused by?

A

temporary loss of the creation of new blood cells. This is most commonly triggered by infection with parvovirus B19

77
Q

What is a sequestration crisis?

A

Pooling of blood in the spleen +/- liver, with organomegaly, severe anaemia and shock

urgent transfusions are required

78
Q

What is the management of a sickle cell crisis?

A

A-E resuscitation, high flow oxygen and IV fluids
Strong analgesia within 30 mins
FBC, reticulocytes, cross match blood
Secret for infection: blood cultures, MSU, CXR and treat early

prophylactic enoxaparin. give fully cross-matched blood transfusion if Hb or reticulocytes fall sharply

Exchange transfusion if rapidly deteriorating

79
Q

What is the cause of G6PD deficiency?

A

defect in the red blood cell enzyme G6PD

X linked inheritance

80
Q

What are the symptoms of G6PD deficiency?

A

Mostly asymptomatic, jaundice (usually in the neonatal period), gallstones, anaemia, splenomegaly and Heinz bodies on blood film

susceptible to oxidative crisis due to reduced glutathione production.
These attacks cause rapid anaemia and jaundice with ‘bite cells’ and ‘blister cells’ seen on the blood film

81
Q

What are the precipitants of attack from G6PD?

A

drugs: aspirin, primaquine, sulphonamides
broad bean consumption
illness

82
Q

How is diagnosis of G6PD crisis done?

A

Enzyme assay 3 months after initial crisis

delay as young RBCs may have enough enzyme, and appaear normal.
treatment = precipitant avoidance, plus transfusion if severe

83
Q

What is the cause of PK (pyruvate kinase deficiency)?

A

Autosomal recessive condition - with reduced ATP production, shortening the RBC lifespan

84
Q

What is the production of PK deficiency?

A

Neonatal jaundice, and later chronic jaundice with hepatosplenomegaly

diagnosis = with enzyme assay

often well tolerated, so no specific therapy is needed, although splenectomies may help

85
Q

What is hereditary spherocytosis?

A

autosomal dominant condition. It causes sphere shaped red blood cells that are fragile and easily break down when passing through the spleen.

86
Q

What is hereditary eliptocytosis?

A

Hereditary elliptocytosis is very similar to hereditary spherocytosis except that the red blood cells are ellipse shaped. It is also autosomal dominant. Presentation and management are the same.

87
Q

What is the management of hereditary spherocytosis / eliptocytosis =?

A

Folate

Splenectomy is curative, but reserved for severe dissed

88
Q

What is autoimmune haemolytic anaemia?

A

antibodies are created against the patient’s red blood cells. These antibodies lead to destruction of the red blood cells. There are two types based on the temperature at which the auto-antibodies function to cause the destruction of red blood cells.

89
Q

What are the classifications of AHA?

A

Warm AHA: IgG mediated, haemolysis occurs at 37 degrees (normal temperature)
Treated with steroids or immunosuppressants +/- splenectomy

Cold AHA: IgM mediated,
At lower temperatures (e.g. less than 10ºC) the antibodies against red blood cells attach themselves to the red blood cells and cause them to clump together. This is called agglutination. This agglutination results in the destruction of the red blood

often associated with Raynaud’s
Treated with cold avoidance +/- chlorambucil

90
Q

What is drug induced haemolysis?

A

penicillin based drugs can cause formation of RBC antibodies, whereas drugs such as quinine cause production of immune complexes

91
Q

Of the extrinsic causes of haemolysis, which are immune mediated?

Non-immune mediated?

A

Immune mediated: AHA, Drug induced

Non-immune mediated: Infection, Microangiopathic anaemia

Alloimmune reactions (transplant, transfusion or rhesus reaction) - immuned mediated but Coomb’s negative

92
Q

What infections cause acute haemolysis?

A

Malaria and other intracellular parasites

93
Q

What is microangiopathic anaemia caused by?

A

Microangiopathic haemolytic anaemia (MAHA) is where the small blood vessels have structural abnormalities that cause haemolysis of the blood cells travelling through them. Imagine a mesh inside the small blood vessels shredding the red blood cells. This is usually secondary to an underlying condition:

Haemolytic Uraemic Syndrome (HUS)
Disseminated Intravascular Coagulation (DIC)
Thrombotic Thrombocytopenia Purpura (TTP)
Systemic Lupus Erythematosus (SLE)
Cancer

94
Q

What is the most common abnormality seen in pre-op patients?

A

Anaemia:
<60g/L will require transfusion
<100g/L may require transfusion depending on cardiac risk and anticipated blood loss

95
Q

What are the cells included in a WBC differential?

A 
Neutrophils 
Lymphocytes 
Eosinophils 
Monocytes 
Basophils
96
Q

What to neutrophils do?

A

Ingest and kill bacterial, fungi and cellular debris

97
Q

What do lymphocytes do?

A

Produce antibodies for cell mediated immunity

98
Q

What do eosinophils do?

A

Role in allergic reactions, and defence of parasitic infection

99
Q

What do monocytes do?

A

precursor of tissue macrophages

100
Q

What do basophils do?

A

Release histamine in inflammatory reactions

101
Q

What is leucocytosis?

A

Increase in the number of white blood cells in the blood

102
Q

What are the differentials for neutrophilia?

A 
Bacterial infection
Inflammatory reaction 
Disseminated malignancy
Stress e.g. surgery or burns 
Myeloproliferative conditions
Corticosteroid therapy
103
Q

What are the differentials for neutropenia?

A 
Viral infections
Severe sepsis 
Neutrophil antibodies e.g. SLE
Bone marrow failure 
Hypersplenism e.g. Felty's 
Cytotoxic drugs
104
Q

What is agranulocytosis?

A

Complete absence of circulating neutrophils

Side effect of carbimazole (exam q)

105
Q

What are the differentials for lymphocytosis?

A

viral infections
Chronic infections (TB, hepatitis)
Myeloproliferative conditions

106
Q

What are the differentials for lymphopenia?

A 
Bone marrow failure
Corticosteroid failure 
SLE
Uraemia
HIV infection 
Cytotoxic drugs
107
Q

What is a leukaemia?

A

Malignant proliferation of blood forming cells.
Either acute or chronic
either myeloid or lymphoid

108
Q

hamopoetic stem cell –> myeloid or lymphoid progenitor.

Then, What do MYELOID progenitor cells divide to form?

A

erythrocytes
mast cells
megakaryocytes
myeloblasts

which go on to form monocytes, basophils, neutrophils and eosinophils

109
Q

What do LYMPHOID progenitor cells divide to form?

A

NK cells, T and B lymphocytes

110
Q

Name the three myeloproliferative conditions

A

due to uncontrolled proliferation of a single type of stem cell: considered a type of bone marrow cancer

Myelofibrosis
Essential thrombocythaemia
Polycythaemia vera - considered pre-leukaemic

111
Q

What is ALL?

A

Malignancy of lymphoid cells of either B or T cell lineages, leading to uncontrolled proliferation of immature blast cells. Excessive proliferation of these cells causes them to replace the other cell types being created in the bone marrow, leading to a pancytopenia

Leads to eventual bone marrow failure and tissue infiltration

112
Q

What is the most common malignancy in childhood?

A

ALL - more common in certain genetic syndromes e.g. Down’s

113
Q

What are the good and poor prognostic factors in ALL?

A

Prognosis is good in children below 10 - with cure rates of >80%

Poor prognosis is suggested by older age of presentation, male sex, B cell disease and presence of the Philedelphia chromosome (9:22 translocation)

114
Q

What is AML?

A

Malignancy of Blast cells from the marrow myeloid elements

115
Q

How does AML occur?

A

Can arise de novo or on a background of a myeloproliferative condition, previous chemotherapy, ionising radiation or genetic syndromes

116
Q

What is the general age of presentation of AML?

A

65 years old

Incidence increases with age

117
Q

What Is the prognosis for AML?

A

Rapidly progressive - only 20% 3-year survival after chemotherapy

118
Q

What are the symptoms of acute leukaemia?

A

GENERALLY B-symptoms, bone pain etc

B-symptoms: Fatigue, weight loss, night sweats, fevers, pruritus
Bone pain

Symptoms related to bone marrow failure:
Anaemia: SOB, weakness
Leukopenia: recurrent infections
Thrombocytopenia: bleeding and bruising - more common in AML

Hepato/splenomegaly

119
Q

Why do you get leukopenia symptoms when the presentation = leucocytosis?

A

Cells = immature and non-functioning blast cells

thus symptoms of leucopenia are seen

120
Q

What are the investigations for acute leukaemias?

A

FBC
Blood film: blasts diagnostic, with lineage identified morphologically and confirmed with immunotyping

CXR: T cell ALL classically shows mediastinal widening

Bone marrow aspiration: to confirm diagnosis and confirm lineage

PET scanning: check for metastatic disease

U&Es, LFTs, cardiac function testing ECG/echo are then essential for planning therapy

121
Q

What are the supportive care steps in managing leukaemias?

A

Nurse with full barrier nursing
Hickman line - venous access
high calorie diet
frequent blood and platelet transfusions required

Allopurinol to prevent tumour lysis syndrome due to chemotherapy

122
Q

What antibiotics are given in management of acute leukaemia and why?

A

If temperature >38 degrees on two occasions greater than an hour apart, assume sepsis and start broad spectrum Abx until afebrile for 72 hours

Often a cephalosporin plus gentamicin

123
Q

What is the management of ALL?

A

High dose chemotherapy to induce remission
Consolidation with high/medium dose ‘blocks’ over many weeks

2 years of maintenance therapy

Consider a marrow transplant if poor prognosis or relapse

Transplant necessary to cure those with philedelphia chromosome

124
Q

What is the management of AML?

A

Intensive chemotherapy
In disease with poor prognosis, allogenic marrow transplant from HLA-matched siblings is indicated after first-round therapy

This then allows further high dose chemotherapy

In intermediate prognosis disease, autologous marrow transplants may be used (cells grown from own bone marrow)
Further chemotherapy must be at lower doses

125
Q

When does each leukaemia onset?

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)

126
Q

What % of people with CML have the philedelphia chromosome?

A

95% - better prognosis than those without

127
Q

What are the symptoms of CML?

A

Insidious B symptoms
Others = gout due to purine breakdown and abdominal discomfort due to scenic enlargement

Splenomegaly, Hepatomegaly and signs of anaemia/thrombocytopenia

128
Q

What Ix are done for CML?

A

FBC: WCC very high, raised across the whole spectrum of myeloid cells
Bone marrow biopsy: hyper cellular
CT/PET
Cytogenic analysis of blood/marrow for the Philedelphia chromosome

129
Q

What is the management of CML?

A

Imatinib chemotherapy has response rates >90% and is first line

Stem cell transplantation is teh only treatment that may achieve remission, but carries significant mortality / morbidity

130
Q

What are the three phases of CML?

A

chronic phase, the accelerated phase and the blast phase.

The chronic phase - 5 years, is often asymptomatic and patients are diagnosed incidentally with a raised white cell count.

The accelerated phase occurs where the abnormal blast cells take up a high proportion of the cells in the bone marrow and blood (10-20%).

Patients become more symptomatic, develop anaemia and thrombocytopenia and become immunocompromised.

The blast phase - even higher proportion of blast cells and blood (>30%). This phase has severe symptoms and pancytopenia. It is often fatal.

131
Q

Which is the most common leukaemia for all ages?

A

Chronic lymphocytic leukaemia - twice as common in males

Median age of presentation: 70 years

132
Q

Describe how CLL occurs?

A

Accumlation of mature B cells that have escaped apoptosis and this increasing mass of immune-competent cells leads to bone marrow failure

133
Q

What are the symptoms of CLL?

A

Often none: surprise finding on a routine FBC
Patient may be anaemic or infection prone or if severe there can be B symptoms

On examination there are enlarged non-tender lymph nodes and hepatosplenomegaly

134
Q

What investigations are done for CLL?

A

FBC: markedly raised lymphocytes, may be a sign of bone marrow failure
Autoimmune haemolysis develops later
Blood film: predominant smudge cells (small mature lymphocytes)

135
Q

What is the management of CLL?

A

Without tx, 1/3 never progress, 1/3 eventually progress and 1/3 actively progress from diagnosis

Treatment is only indicated if symptomatic, or there are cytogenic markers of poor prognosis

Tx = chemotherapy or radiotherapy o relive hepatosplenomegaly

136
Q

What is the prognosis of CLL?

A

Stage 0: lymphocytosis alone; >13 years mean survival
Stage 1: lymphocytosis and lymphadenopathy, 8 years
Stage 2: lymphocytosis and hepato-splenomegaly: 5 years
Stage 3: lymphocytosis and anaemia: 2 years
Stage 4: lymphocytosis and thrombocytopenia, 1 year

137
Q

What is the cause of death in CLL?

A

Usually due to an infection, or transformation to an aggressive lymphoma (Richter’s syndrome)

138
Q

What is myeloma?

A

Myeloma is a cancer of the plasma cells. These are a type of B lymphocyte that produce antibodies. Cancer in a specific type of plasma cell results in large quantities of a single type of antibody being produced.

139
Q

How does myeloma occur?

A

Normally, many different plasma cells produce a range of immunoglobulins i.e. they are polyclonal

In myeloma, a single clone of plasma cells produce a single immunoglobulin which can be seen as a monoclonal band, or paraprotein, on serum/urine electrophoresis

140
Q

What are the clinical features of myeloma?

A 
CRAB: calcium, renal, anaemia, bone 
C – Calcium (elevated)
R – Renal failure
A – Anaemia (normocytic, normochromic) from replacement of bone marrow.
B – Bone lesions/pain

Osteolytic bone lesions due to osteoclast activation
Backache
pathological fractures
hypercalcaemia (bones, stones, moans and groans)

Bone marrow failure:
infection
symptoms of anaemia
bleeding

Renal impairment:
Seen in 20% at diagnosis, due to light chain deposition

CP3 Medicine (19 decks)

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