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Haematology Flashcards

1
Q

Anaemia

  • Physiological effect
  • What is it
  • Classic symptoms
  • Classic sign
A

Decreased oxygen transport, Tissue hypoxia, compensatory changes (inc RBC prod.)

low Hb (low red cell mass or inc plasma vol e.g. preg)

FDF
Fatigue, dyspnoea, faint
(may also worsen any symptoms related to hypoxia e.g. IC/angina)

Conjunctival pallor

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2
Q

Normal Hb

A

Men: 13.5-17.5 g/dL (135-175g/L)

Women: 12.0-15.5 g/dL

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3
Q

What is severe anaemia?

Feature

A

less than 80g/L

Tachycardia, inc CO, flow murmur, Cardiac enlargement

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4
Q

Microcytic anaemia causes

A 
TICS
Thalassaemia
Iron deficiency
Chronic disease (20%)
Sideroblastic
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5
Q

Normocytic anaemia causes

A

Haemolytic anaemia
Pregnancy (increased plasma volume)

Bleeding	(e.g. menses)
			 Renal failure (decreased erythropoietin, may give recombinant)

Chronic disease (80%)

Primary marrow problem (Red cell aplasia, Myelodysplasia MM, infiltration)

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6
Q

Macrocytic anaemia causes

A

B12
Folate
Drugs/alcohol

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7
Q

Anaemia seen in Chronic disease

A

Microcytic in 20%

Normocytic in 80%

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8
Q

What does reticulocyte level tell you in normocytic anaemia

A

Reticulocytes high (haemolytic or bleed)

If low:
Evidence of renal or endocrine failure or chronic inflammation (chronic disease)?

If No consider Primary marrow problem (Red cell aplasia, Myelodysplasia MM, infiltration)

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9
Q

Where are Iron, Folate and B12 absorbed?

A 
IRON = DUODENUM/JEJUNUM
FOLATE = JEJUNUM
B12 = ILEUM
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10
Q

Iron transported by

Iron stored by (where is this)

A

Transferrin

Ferritin (intracellular)

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11
Q

Iron deficiency anaemia:

  • Causes
  • Presentation
A

Inadequate intake
Poor absorption
Excessive loss
Excessive iron requirement

FPFD
Fatigue, Pallor, Faint, dyspnoea, Nail changes (Koilonychia - spoon, brittle), Mouth changes (Angular stomatitis, atrophic glossitis)

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12
Q

Iron deficiency anaemia:

Inadequate intake

Poor absorption

Excessive loss

Excessive iron requirement

A

Diet

Surgery, Coeliac

GI bleed, Peptic ulcer (NSAID), surgery, Diverticulosis, Neoplasm, Menorrhagia.

Infancy, Pregnancy

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13
Q 
Iron deficiency:
Hb
MCV
MCHC
Iron studies (Serum iron, Ferritin, Transferrin, TIBC)
A

Hb
Less than 130g/L m, 120g/L f

MCV
Low - Microcytic

MCHC
Low - Hypochromic (Low Hb conc per cell)

Serum Fe: low
Ferritin: low
Transferrin Saturation: low
TIBC: increased

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14
Q

What happens to ferritin in infection

A

This is an acute phase protein so inc in inflammation, infection, malignancy

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15
Q

Iron deficiency anaemia Tx

SE

A

Oral Iron supplementation

(Ferrous sulphate
Continue for 3-6 months post Hb correction)

SE: constipation, black stools, vomiting

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16
Q

When to transfuse in iron def anaemia

A

Transfusion if symptomatic at rest, dyspnoea, chest pain

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17
Q

Sideroblastic anaemia

What is it

A

Ineffective erythropoiesis where iron can not be incorporated into Hb

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18
Q

What is a Sideroblast

A

Abnormal erythroblast with perinuclear iron accumulation (Cant incorporate)

Microcytic anaemia resistant to iron supps

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19
Q

Causes of Sideroblastic anaemia

A

Congenital (X-linked)

Acquired: Myeloma, B6 deficiency

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20
Q

Sideroblastic anaemia

Hb
MCV
MCHC
Blood smear

Serum iron
Ferritin
TIBC

Marrow aspirate

A

Low Hb

MCV and MCHC low (Microcytic and hypo chromic)

Mix of normal and hypo chromic blood cells

Serum iron and ferritin high, TIBC low (not a problem with iron levels)

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21
Q

Sideroblastic anaemia Tx

A

Mainly supportive

Iron chelation (removal) with Desferrioxamine
- there is inc absorption or iron in Sideroblastic anaemia
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22
Q

Chains seen in Hb:

HbA (normal adult)

HbF

A

2 x ⍺ + 2 x β

2 x ⍺ + 2 x ɣ (gamma)

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23
Q

Beta-Thalassemia

  • Pathophys
  • Type of anaemia
  • Mutation
  • Genetics
A

Decreased (β+) Or Absent (β0) beta globulins. Alpha overprod to compensate = membrane damage/cell destruction

Microcytic

Chr 11 (β-glob gene)

Autosomal recessive

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24
Q

Effect of Beta-Thalassemia

A

Cells can’t survive

Anaemia
-Microcytic

Erythroid hyperplasia

  • Skull bossing/bony changes
  • Hepatosplenomegaly (extra medullary hematopoiesis)
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25
Q

Types of Beta-Thalassemia + effect

Silent (Carrier)
Minor
Intermediate
Major

A

Normal Haem, These are carriers (Autosomal recessive disease)

Minor
- Mild anaemia

Intermediate
- Progressive pallor, abdo distension, skull bossing

Major
β0/β0
- Failure to thrive

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26
Q

Beta thalassaemia

FBC
Blood smear
Hb analysis
Other investigations

A

Microcytic

Target cells, Reticulocytes (nucleated RBC - haemolytic)

No HbA in Major, some HbA in others. HbF elevated in all

LFT (haemolysis = inc total and unconjugated bilirubin), Skull XR, Abdo USS hepatosplenomegaly.

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27
Q

Beta thalassaemia Tx

A

Genetic counselling, Transfusion if symptomatic/Surgry/Infection, Chelation (desferrioxamine)

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28
Q

Beta thalassaemia complications

A

Thrombotic

Iron overload (arthropathy, arythmias + contractile dysfunction)

Transfusion reactions/infections (HIV, HBV/HCV)

Splenectomy

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29
Q

Why splenectomy in Beta thalassaemia

A

Extramedullary erythropoiesis and multiple transfusion leads to hypersplenism

= inc transfusion requirement, leukopenia + thrombocytopenia

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30
Q

Alpha thalassemia

  • Pathophys
  • Type of anaemia
  • Mutation
  • Genetics
A

microcytic anaemia caused by mutation in alpha-globin genes. Dec alpha glob leads to excess beta prod.

Chr 16

Autosomal recessive

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31
Q

Alpha thalassaemia Pathophsy

A

Ineffective erythropoiesis (Alpha chain), microcytic anaemia and haemolysis

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32
Q

Genetics in Alpha thalassemia

A

There are two genes coding for alpha globulin from each parent (4 total)

HbA can be see with some Beta tetramers after 3 deletions ( less than this is mild anaemia)

4 deletions (mutations) leads to death in utero

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33
Q

Haemolytic anaemia:

  • What is it
  • Where can it happen
  • Typical anaemia pattern
A

Premature destruction of RBC

1) intravascular (trauma e.g. mechanical heart valve, complement mediated/Autoimmune)
2) reticuloendothelial system (macrophages of liver, spleen, destruction due to immune tagging/opsonization)

Normocytic

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34
Q

Haemolytic Anaemia: Hereditary Vs Acquired Causes

A

Hereditary

  • Enzyme defect (G6PD deficiency)
  • Membrane defect (Spherocytosis)
  • Abnormal Hb (Sickle cell)

Acquired
- Immune or non-immune mediated

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35
Q

Immune mediated haemolytic anaemia

  • Cause
  • Test + result
  • Subtypes
A

Autoantibodies

Coombs antiglobulin +ve

Warm: IgG (Tx steroids)

Cold: IgM (Tx keep warm)

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36
Q

Non immune haemolytic anaemia

  • Test and result
  • Causes
A

Coombs negative

Infection (e.g. malaria), trauma, microangiopathic haemolytic anaemia (DIC, TTP, HUS, HELLP), hypersplenism, liver disease

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37
Q

Haemolytic anaemia presentation

RFs

A

Anaemia symptoms (Pallor, fatigue, dyspnoea, faintness)

Jaundice (inc bili due to haemolysis)

Autoimmune disease, Prosthetic valve, FH RBC defect, from Africa (malaria protection), Fava beans

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38
Q

Haemolytic anaemia

FBC
MCHC
Blood film
Other

A

Low Hb with reticulocytes
Inc MCHC suggests spherocytosis

sickle cells, spherocytes, heinz bodies (G6PDD)

Coombs test (+ve if immune), unconjugated bili mild elevation

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39
Q

Haemolytic anaemia

Differentiating between inc breakdown and increased production

A

Increased breakdown?
Increased bilirubin, increased urobilinogen, increased LDH

Increased production?
Increased MCV by increased reticulocytes, marrow hyperplasia

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40
Q

Haemolytic anaemia findings in

Extravascular Vs Intravascular

A

Intravascular:
- inc free plasma Hb and haemoglobinuria

Extravascular:
- Splenomegaly

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41
Q

G6PDD

  • pres
  • investigate
A

FPFD+Jaundice, dark urine (intravasc), AKI (Hb precip)

FBC (low Hb, normal MCV, MCHC), Blood film (heinz bodies)

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42
Q

G6PDD

  • Genetics
  • Protective of what
  • role of enzyme
  • What precipitation haemolysis
A

x-linked,

malaria protective

protects from oxidative stress

Things that cause oxidation
Drug(cephalosporins)/fava bean, infection.

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43
Q

G6PDD Tx

A

Avoid precipitants

  • Maintain fluid intake
  • Folic acid 5mg orally (for RBC production)
  • Blood transfusion if severe anaemia (<7g/dL)
  • Renal support if renal impairment by haemoglobinuria (EPO)
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44
Q

Anaemia of chronic disease

  • Description
  • Mechanism
  • types of disease assoc
A

Anaemia + evidence of immune system activation

Inflam (IL1/6)= decreased RBC prod and survival

Infection (acute and chronic), Neoplasm, autoimmune (RA, SLE), trauma, endocrine (DM), degenerative (CKD, CHF)

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45
Q 
Anaemia of chronic disease blood results
RBC/MCHC
Reticulocytes
Iron
TIBC
Transferrin saturation
Ferritin
A 
Normocytic normochromic/microcytic hypochromic
Low reticulocyte count
Low serum iron
High or normal TIBC (elevated ferritin)
Low transferrin saturation
Elevated ferritin
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46
Q

Anaemia of chronic disease

FBC
MCV + MCHC
Reticulocytes
Other investigations

A

Hb low, WCC/Pt may be raised

MCV/MCHC
Low or normal (Can be microcytic or normocytic)

Low Reticulocytes (Low prod.)

ESR/CRP

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47
Q

Anaemia of chronic disease Tx

A

Underlying cause

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48
Q

Aplastic anaemia

triad

A

1) Pancytopenia

Neutropenia: infections

Anaemia: fatigue, pallor, dyspnoea, faintness

Thrombocytopenia: bleeding bruising

2) BM biopsy has hypocellularity,
3) No abnormal cells: no dysplasia or blasts

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49
Q

Caused of Aplastic anaemia

A

Acquired:
idiopathic, post drug/virus

Inherited: Fanconi anaemia, Shwachman-Diamond syndrome

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50
Q

Aplastic anaemia Tx (if severe)

A

Allogenic BM transplant if severe

RBC + Pt transfusion (in the meantime)

Abx (Prophylactic)

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51
Q

Causes of Neutropenia

what at risk of

A 
Apalstic anaemia
Immunosuppression
Chemo
HIV
BM infiltration (MM)

Fatal infection (may also present atypically)

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52
Q

BM responsibility

Which bones?

A

Haematopoiesis

Axial skeleton (vertebrae, sternum, ribs, skull)

Long bones

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53
Q

Sickle cell

  • Autosomal …
  • effect of genetic mutation
A

Autosomal Recessive

Amino acid Glutamine replaced by Valine in beta chain of HbA resulting in sickling/cresent shape = HbS

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54
Q

Effect of RBC sickling

A

Disrupts blood flow/obstruction of small capillaries (shape)

Anaemia (varying degree) due to reduced RBC life and inc haemolysis

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55
Q

Effect of small vessel obstruction in SC

A

Painful crises
Organ damage
Increased vulnerability to infection

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56
Q

Sickle cell crisis precipitants

A

CHIDS

Cold
Hypoxia (extreme exercise)
Infection
Dehydration
Stress
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57
Q

Sickle cell epidemiology

A

Sub-Saharan africa (Malaria can live in sickled cells)

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58
Q

Sickle cell symptoms and signs

A

Anaemia + haemolysis (Fatigue, pallor, Faint, dyspnoea + Jaundice)

Vaso-occlusion: Dacytlitis, skeletal pain, chest/abdo pain, renal failure, CNS infarct, Priaprism

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59
Q

Sickle Cell Crisis

  • Chest
  • Bone
  • Abdo
A

Acute chest: sickling in pulm vasc = pneumonia like syndrome)

Bone infarct and avast necrosis (e.g. femoral head). Osteomyelitis with salmonella

Acute abdo: mesenteric ischaemia, Splenic sequestration (stops blood leaving = engorgement) inc susceptibility to encapsulated bact (S.Pneuonia, H.Inf)

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60
Q

Sickle cell diagnosis

A

Guthrie 5 day heel prick test

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61
Q

Sickle cell infection prophylaxis

A

Give pneumococcal vaccine and penicillin prophylactically for pneumococcus

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62
Q

Sickle cell crisis Tx

A

Cross-match blood

Analgesia - paracetamol, ibuprofen, codeine phosphate or morphine (IV) (+antihistamine for pruritus with opiates)

Supportive care

  • O2 (nasal cannula 2L/min) + treat cause e.g. correction of acidosis
  • IV fluids (correct dehydration)
  • Warmth

Antibiotics according to local guidelines

Give blood if Hb or reticulocytes fall quickly

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63
Q

Sickle cell chronic management

A

Manage chronic daily pain

Pneumococcal vaccination and penicillin prophylaxis

Trigger avoidance

Folic acid if severe haemolysis

Hydroxyurea (with monthly blood tests)
- Increases production of fetal haemoglobin - reduces painful crises

Repeat blood transfusion to maintain HbS below 30%

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64
Q

Sickle cell complications

A

Anaemia

Liver complications - jaundice, hepatomegaly and gallstones

Iron overload from chronic transfusion

Dactylitis

Leg ulcers

Priapism - requires urgent urological aspiration if >4 hours

CV - cardiomegaly

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65
Q

Causes of megaloblastic anaemia

A

B12/Folate deficiency

Alcohol

Drugs (hydroxyurea)

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66
Q

Causes of folate deficiency

A

Poor diet (in green veg, nuts, liver)
Inc demand (preg, renal disease)
Malabsorption (coeliac)
Drugs (Alcohol and anti-metabolites e.g. MTX)

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67
Q

Megaloblastic anaemia

Hb
MCV & MCHC

Blood smear

Other tests

A

Low Hb (may also have thrombocytopenia/neutropenia)

Elevated MCV/MCHC

Blood smear has macocytic RBS

Serum levels of B12/Folate will indicate the cause

IF antibody if pernicious anaem suspect

Other Alc related tests if suspect: Carbohydrate deficient transferrin

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68
Q

Folate deficiency Tx

A

ORal folic acid, Treat cause, packed red cell transfusion if severe/pancytopenia

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69
Q

B12

  • use
  • food
  • deficiency due to …
A

DNA synthesis (cofactor), nerves and red blood cells

Meat fish dairy

poor diet (Veg, vegan), old, surgery, Malabsorption (PPI/ H2 antagonists = acid needed to release B12 from food, pernicious anaemia = low intrinsic factor from parietal cells, coeliac, crohn’s)

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70
Q

What binds B12

A

Intrinsic factor

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71
Q

B12 deficiency symptoms

A

Anaemia (Fatigue, Pallor, Faint, Dyspnoea)

Neurological - Posterior column degenerative = Paresthesia, Ataxia, loss of vibration

Mouth - glossitis

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72
Q

B12 def Tx

A

IM hydroxycobalamin (for 3 months, may be needed life long) - this is Vit B12a

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73
Q

Pernicious anaemia

  • what is
  • Assoc
  • Complications
A

autoimmune disease with antibodies against IF or Parietal cells

Othe AI disease

Gastric cancer

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74
Q

Origins of haematological malignancy

A

BM: Myeloid

Lymphatic: Lymphoid (Can be B or T cell)

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75
Q

Haematological malignancy Tissue types

Leukaemia
Lymphoma
Myeloproliferative disorders

A

Leukaemia: Excess abnormal white cells (myeloid/from BM or lymphoid/lymphatic origin)

Lymphoma: Tumour presentation with lumps in lymphatic system

Myeloproliferative disorders: involve BM, liver and spleen

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76
Q

Haematological malignancy Clinical course types

A

Acute: rapid progression from onset. Life-threatening in weeks/months (AML = myeloid from BM, ALL = from lymphatics)

Chronic: slowly progressive, years between pres and clinical intervention

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77
Q

AML

  • what is it
  • Diagnostic feature
  • Epidemiology
A

Clonal expansion of myeloid blasts in BM. Unable to diffrentiate into neutrophils

Bone marrow blasts over 20% OR blasts in peripheral blood

Most common Leukaemia in adults

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78
Q

AML associated diseases

A

Myelodysplastic syndrome,
Down’s syndrome,
Bone marrow failure syndromes (e.g. Fanconi, Diamon-Blackfan, aplastic anaemia),

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79
Q

What can cause AML

A

AML is a complication of Chemotherapy

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80
Q

AML prognosis

A

5 year survival 25%

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81
Q

AML investigations:

A

FBC + blood film

  • Leukocytosis (inc WCC)
  • neutropenia, thrombocytopenia, anaemia

Coagulation panel
- PT & PTT prolonged (thrombocytopenia)

BM biopsy (Diagnostic)

  • Hypercellularity, over 20% blast cells
  • Auer rods (needles of granular material in cytoplasm of blast cells)

CXR
- Pulmonary infiltrates

82
Q

AML pres

A

Older person

Tiredness
Bruising/PEtechiae
Aching in bones (and boney tenderness)
Fever

83
Q

AML Tx

A

Chemo: Cytarabine and Daunorubicin (watch for tumour lysis syndrome

Allogenic (donor match) stem cell transplant

84
Q

Tumour lysis syndrome

A

Efflux of K+ = Hyperkalaemia (ECG)

Nucleic acid release -> purine catabolism -> high Uric acid = Uric acid crystals (AKI)

Phosphate (PO4) also released from intracellular. Causes HYPOCALCAEMIA (CATS) by binding to make calcium phosphate = stones = AKI

85
Q

ALL Presentation

A 
Younger child
Lethargy
Dyspnoea
Fever
Bruising

Hepatosplenomegaly

CXR: Mediastinal mass, pleural effusion

86
Q

ALL

  • What is it
  • Diagnosis
  • Epidemiology
  • What has poor prognosis
A

Malignancy of lymphoid cells (B&T cells). Uncontrolled proliferation.
Lymphoid cells replace haemopoetic cells in BM

BM blasts voer 20%
Blood smear with leukaemic lymphoblasts

Most common childhood leukaemia (75% under 6yrs)

Philedelphia Chr (BCR-ABL fusion oncogene) = Poor prognosis

87
Q

Symptoms of ALL CNS infiltration

A

Papilloedema
Nuchal rigidity
Meningism

Focal neurology: CN 3,4,6,7

88
Q

ALL Investigations

A

FBC
- Leukopeia, thrombocytopenia, anaemia

Blood smear: Leukaemic blasts

BM biopsy (diagnostic)

  • Hypercellularity
  • Lymphoblasts (over 0-25%)

CXR: Mediastinal widening

LP and Pleural tap also give leukaemic lymphoblasts

89
Q

ALL management

A

Chemo:
- Prednisolone + Vincristine + Daunorubicin (anthracycline) + TKinhib (imatinib)

If CNS disease: intrathecal MTX

Maintainence chemo (years):
- Mercaptopurine (daily) + MTX (weekly) + vincristine&amp;prednisalone (monthly)
90
Q

CML

  • What is it
  • Pres/symptoms
A

Uncontrolled proliferation of myeloid cells in bone marrow

1/3 asymptomatic
Malaise, Fever, Wt loss, splenomegaly (elevated WBC), night sweats, Arthralgia (inc uric acid from inc cell turnover)

91
Q

What confirms Diagnosis of CML

A

Presence of Philadelphia chromosome/ BCR-ABL fusion gene confirms Dx

92
Q

CML pathophys

A

Philedelphia chromosome oncogene causes active TK on myeloid cell surface = unregulated cell division

93
Q

Complication of CML

A

May transform to accelerated (symptomatic) or blast phase (AML)

94
Q

CML Investigation

A

Bloods: Leukocytosis (inc WCC), Anaemia

BM biopsy: Granulocytic hyperplasia

95
Q

CML Tx

A

TK inhibitor: Imatinib

Allogenic haemopoeitic stem cell transplant + Chemo cytarabine + daunorubicin

96
Q

Philedelphia chromosome and prognosis

A

In CML = GOOD

in ALL = BAD

Philedelphia not good for kids…

97
Q

What is a blast cell crisis

A

Seen in AML and CML

Leads to pancytopenia

98
Q

CLL

  • What is it
  • What can trigger and in who
A

Malignancy of B lymphocytes that escape apoptosis. Involves BM, peripheral blood + haemopoietic organs (hepatosplenomegaly)

Pneumonia can trigger.
Risk increases with age. This is most common type of leukaemia.

99
Q

AML
ALL
CML
CLL

A

Adults, myeloid cant make neutrophils

Kids, Philly = bad, years of chemo, malignant B&T from lymph replace haemopoietic in BM (pancytopenia)

Same as AML but slower rate, can switch to AML picture. Seen with Philadelphia chr

Most common leukaemia. B-cell apoptotic loss, hepatosplenomegaly (infiltration)

100
Q

CLL symptoms

of anaemia
of infiltration
Other (remember the cell mainly involved)

A

SOB, fatigue

Hepatosplenomegaly,
Lymphadenopathy (painless + rubbery)

B-symptoms: 
Fever/Chills
Night sweats
Weight loss
Fatigue
101
Q

CLL investigation

A

FBC: MARKED lymphocytosis, low Hb, low pt

Blood film: smudge and smear cells (lymphocyte damage in slide prep

CT: hepatosplenomegaly, mediastinal lymph nodes

102
Q

DDX CLL

A

Leukaemic phase of lymphoma

103
Q

CLL Tx

A

Watch and wait if Hb and Pt normal (monitor FBC)

if anaemia/thrombocytopenia/lymphadenopathy
Chemo: Rituximab (B-CELL!) + cyclophosphamide + fludarabide + stem cell transplant (50% remission but high relapse)

104
Q

CLL complications

A

Inc infection risk: lymphocytes cant produce antibodies (hypogammaglobulinaenia in IgA, IgG, IgM) - monthly IVIg

Autoimmune haemolytic anaemia: give prednisolone

105
Q

Difference between leukaemia and lymphoma

A

Lymphoma is solid, leukaemia is circulating

106
Q

Non-Hodgkin lymphoma name 4 types

A 
Diffuse large B-cell (commonest - mainly B-cell lines)
Follicular
Burkitt's
Primary CNS
MALT
T-cell
107
Q

What Lymphoma most common

A

Non-Hodgkin lymphoma 5X more common than Hodgkin

108
Q

Non-Hodgkin lymphoma assoc disease

A

Associated with autoimmune disorders (Sjogren’s RA, SLE, Coeliac, Pernicious anemia)

Assoc with immunodeficiency

109
Q

Non-Hodgkin lymphoma presentation

A

Nodal: rapid lymphadenopathy

Extra-nodal: Pancytopenia, hepatosplenomegaly, dry cough (mediastinal mass)
Skin (t-cell)

B-symptoms:
Night sweats
Fever
Weight loss (over 10%)
Fatigue
110
Q

Non-Hodgkin lymphoma Investigations

A

FBC: Pancytopenia (BM affected)

Lymph node core biopsy/BM biopsy
- Cytogenetics and tumour surface markers (diff in diff types)

Lactate raised

CXR: mediastinal nodes

111
Q

What is used to stage Non-Hodgkin lymphoma

What does staging look at

What carries bad prognosis?

A

An Arbor

Lymph node involvement

B-symptoms = worse prognosis

112
Q

Non-Hodgkin lymphoma Tx

A

Stage 1/2 = Radiotherapy

Chemo or stage 3/4

R-CHOP-21
rituximab, cyclophosphamide, hydroxy-duanorubicin, vincristine (oncovin) Prednisolone
21 days

113
Q

Non-Hodgkin lymphoma chemo SE

A

Haematological (BM suppression) Infection (neutropenia) Cardiac (doxorubicin) Alopecia, chills, fever, hypotension (rituximab cd20 mAb)

114
Q

Diffuse large B-cell lymphoma

A

80% cure

115
Q

Hodgkin’s lymphoma

  • Cell type
  • Common presentation
  • Assoc cell
A

B-cells

Cervical or supraclavicular lymphadenopathy (painless)

30% have B-symptoms

Reed-Sternberg cells (multinucleate B-cells)

116
Q

Hodgkin Lymphoma Causes&RFs

A

Immunodeficiency
- Primary (ataxia-telangectasia) or secondary (HIV, transplant)

Infection (EBV)

Autoimmune (SLE)

117
Q

Hodgkin Lymphoma pathophys

A

Impaired immunosurveillance of B-cells (EBV infected esp). B cells escape regulation and proliferate

118
Q

Hodgkin Lymphoma Presentation

A

Painless lymphadenopathy (supraclavic/cervical)

B-symp (25%) - night sweats, fever, weight loss

Pruritus

Alcohol induced pain

mediastinal lymphadenopathy (dry cough, SVC obstruct, chest pain)

119
Q

Diagnosis Hodgkin Lymphoma

A

ESR - Raised = poor prognosis

CXR - mediastinal mass

Lymph node biopsy - Reed sterberg

120
Q

Hodgkin Lymphoma Tx

A

Chemo BDDV + Radiotherapy

Bleomycin
Doxorubicin
Dacarbazine
Vincristine

121
Q

Multiple myeloma 3 main features

A

1) Osteolytic bone disease (inhibitor of osteoprogenitor cells and thus osteoblasts in BM) + hypercalcemia
2) Anaemia - accumulation of plasma cells in BM
3) Renal disease - Bence-Jones proteins (immunoglobulin light chain found in urine of MM)

122
Q

Multiple Myeloma

  • Description
  • Common symptoms
A

Clonal proliferation of plasma cells in BM assoc with monoclonal banding of Ig in serum/urine (bence-jones)

Bone pain, Anaemia, Fatigue, Hypercalcaemia

123
Q

MM diagnosis

A

Serum and urine protein electrophoresis shows monoclonal bands of Immunoglobuilin (M-proteins)

M-Protein over 30g/L OR BM clonal cells over 30%

124
Q

Tissue/Organ involvement in MM

A

CRAB

Calcium elevation

Renal insufficiency (Creatinine over 173mmol/L)

Anaemia

Bone disease (lytic or osteopathic)

125
Q

MM Osteolysis, BM infiltrate and renal pathophys

A

Osteolytic due to Osteoclastic stimulation and low osteoblastic progenitors (BM) - Backache, pathological fracture, vertebral collapse

Pancytopenia: BM infiltration. neutropenia = infection

Renal impairment due to light chain depsition and precipitation with Tamm-horsfall protein in ascending loop of Henle

126
Q

MM investigations

A

Serum and urine is gold standard:

  • spike of IgG / IgM
  • light chain urinary excretion (Bence-Jones

Skeletal survey (Bone scan)
Osteopenia
Osteolytic lesions (Raindrop skull)
Pathological fracture

BM aspirate: Plasma cell infiltrate over 30% = major

FBC = Anaemia
Serum Ca raised
Urine = Cr raised

127
Q

MM Tx

A

Thalidomide + Dexamethasone + autologous stem cell transplant

Bone disease - bisphosphonates
Anaemia - EPO, transfuse
Infection - Abx, pneumococcal/flu vaccine
AKI - rehydration

128
Q

What is Polycythemia

A

Inc proportion of Hb in blood

Relative = dec plasma vol

Absolute = Inc RBC number

129
Q

Polycythemia cause

A

Overactive BM (predominantly RBC but also others)

130
Q

Polycythemia complications

A

Hyperviscosity: thrombosis (Stroke, MI, DVT), haemorrhage

Myelofibrosis, AML

131
Q

Polycythemia mutation

A

95% JAK2

132
Q

Polycythemia presentation

A

Plethoric (RED and full) appearance

Pruritus, redness and pain in fingers/toes after hot bath

133
Q

Investigations for Polycythemia vera

A

Raised Hb
Raised haemocrit

Raised Pt and WCC

BM: hypercellularity

134
Q

Polycythemia Tx

A

Venesection, low dose aspirin,

135
Q

Myelodysplastic syndrome

  • Description
A

1) dysplastic changes in one or more cell lineages
2) Pancytopenia (anaemia, infection, bleeding)
3) predilection to develop AML

136
Q

Myelodysplastic syndrome Causes

A

90% are primary

Radio/chemo causes 10% of Cases

137
Q

What is Fanconi anaemia

A

Autosomal recessive BM failure syndrome

138
Q

Cells which produce platelets

A

Megakaryocytes

139
Q

Bleeding causes

A

Reduced platelet number

Reduced platelet function

140
Q

Causes of dec platelet production

A
  • BM infiltrate (leukaemia, myeloma, myelofibrosis)
  • Liver disease (Thrombopoietin dec)
  • Dec BM prod. (aplastic anaemia, B12/folate)

Dysfunctional Pt: Myelodysplasia

141
Q

Causes of inc Pt destruction

A

Autoimmune - ITP
Hypersplenism
Drug - Heparin
Inc Consumption - DIC, TTP, HUS, Haemorrhage

142
Q

What is ITP

Pres

Tx

A

Idiopathic thrombocytic purpura

Autoimmune antibodies to platelets

Petechiae, mucosal bleeding, menorrhagia

Immunosuppression
-> IVIG+Corticosteroid+Pt trasfuse
(chronic cases get Rituximab)

143
Q

What is TTP

Why important

A

Absent VW breakdown enzyme (autoimmune) = spontaneous Pt aggregate in micro vasc (brain, kidney, heart)

Uses up Pt

95% fatal

144
Q

TTP Pentad Pres

A

Due to microvasc Thrombophilia

  1. Fever
  2. Renal failure 3. Haemolytic anaemia
  3. Thrombocytopenia 5. Neurological change (coma, focal, seizure)
145
Q

TTP Tx

A

Urgent Plasma exchange + Prednisolone + Antiplatelet (aspirin)

146
Q

DIC pathophys

A

Coagulation pathways activated (e.g. due to endotoxin)

Diffuse fibrin deposition in microvasculature

Consumption of coal factors & Its (Bleeding)

147
Q

DIC Pres

A

Systemic collapse: Oliguria, Hypotension, Tachycardia

Bleeding: bruis, purpura (fulminant = necrotic), haemorrhage, petechiae, haematuria

148
Q

What is increased in DIC

A

PT (prothrombin time - extrinsic pathway)

aPTT (activated partial thromboplastin time - intrinsic pathway)

D-dimer

Fibrin degradation products

149
Q

What is decreased in DIC

A

Platelet count (thrombocytopenia)
Fibrinogen
Factor V, Factor 8

150
Q

DIC Tx

A

Treat underlying cause (usually sepsis)

To ITU

Supportive care:

  • Pt transfusion
  • FFP: replace coagulation factors
151
Q

Haemophilia

  • Genetics
  • Pres
A

X-linked recessive (Only in boys)

Bleeding pattern to muscles and joints

Presents in Toddlers

152
Q

VWD

  • Genetics
  • Pres
A

Autosomal dominant

Bleeding into mucus membranes/skin

153
Q

What is Haemophilia A and B

A

A = no Factor VIII

B = no Factor IX

154
Q

Haemophilia Tx & what to avoid

A

Recombinant F8 (A) and F9 (B)

Desmopressin (DDAVP - trade name) for mild type A

Avoid IM injections, Aspirin and NSAIDs

155
Q

vWD

  • Pathophys
  • Pres
  • Tx
A

Defective vWF = defective platelet plug and factor 8 deficiency (vWF is protective usually)

Bruis, prolonged bleeding, mucosal bleed, epistaxis, menorrhagia

DDVAP (Demopressin)

Recombinant F8 if severe

156
Q

Clotting products synthesised by liver?

A

Coal factors

Fibrinogen

157
Q

Vit K required for which factors

A

1972

Factors: II, VII, IX, X

158
Q

Cause of Vit K deficiency + Tx

A

Malabsorption (fat soluble vit so esp in obstructive jaundice - bile for fat)

Tx = Vit K

159
Q

Intrinsic pathway initiating factor

A

Factor XII –> XIIa

160
Q

Extrinsic pathway initiating factor

A

Tissue factor

Factor VII–>VIIa

161
Q

Coagulation cascade common pathway

A

X–>Xa

Xa =
Prothrombin –> Thrombin

Thrombin =
Fibrinogen –> fibrin

162
Q

What is the coat pathway

A

Proteolytic enzyme cascade circulating in inactive state

Activation generates Thrombin that cleaves fibrinogen to fibrin = stable fibrin clot

163
Q

Platelets:

  • activated by
  • adhere via
  • bound together by
A

Endothelial damage (TF, vWF)

Collagen, vWF and GP1b

Cross linking of Pt with fibrin

164
Q

What occurs on Pt activation

A

Shape change to active form

Degranulation

  • alph: PDGF, TGF-beta
  • delta: ADP, ATP, Ca2+
165
Q

Aspirin&Clopidogrel
Vs
Heparin&Warfarin

A

Affect Pt function

Affect coagulation cascade

166
Q

Aspirin mech

A

Irreversibly inhibits COX1

Prevent Thromboxane A2 thus prevent Pt aggregation and vasoconstriction

167
Q

Clopidogrel mech

A

Irreversible P2y12 antagonist (usually activated by ADP)

Inhibits ADP induced PT aggregation

168
Q

Heparin Vs Warfarin

Factors affected and testing effect

A

Heparin = Intrinsic/common pathway: Xa and thrombin (aPTT)
(LMWH: Dalteparin only does Xa - less risk bleed)

Warfarin = extrinsic pathway: 1972 (Vit K anatag)

169
Q

Risk of Heparin

A

Heparin induced thrombocytopenia: Bleeding

170
Q

What is INR?

A

This is way of monitoring Warfarin derived from PT

Aim = 2-3
higher range (e.g. mechanical valve) 3-4.5
171
Q

NOACs

  • examples
  • effect
  • indications
  • benefit
  • problem
A

Apixiabn, rivaroxiban, dabigatran

Direct Xa or Thrombin inhibitors

DVT/PE and AF

Require no monitoring

NOT easily reversible

172
Q

Difference between arterial and venous thrombosis

A

Arterial - Platelet rich.
Seen with atherosclerosis + RF

Venous - fibrin rich (DVT/PE)

173
Q

Tx of DVt/PE

A

LMWH s/c then oral Warfarin 3 months if provoked, 6 if unprovoked)

174
Q

Wirkows triad

A

Contributors to Thrombosis (arterial and venous)

Venous stasis
Hypercoagulable state
Endothelial injury

175
Q

Acquired hyper coagulable state

A 
95%
Antiphospholipid syndrome
Cancer
Slow flow - sickle cell, PRV (viscous)
Nephrotic syndrome
Obesity
Pregnancy
176
Q

Inherited hyper coagulable state (5%) - Screen these for DVT

A

Protein C deficiency (break clot)

Protein S deficiency (break clot)

Factor V Leiden (AD)
Factor 5 is a cofactor for factor Xa to activate prothrombin to thrombin
In Leiden factor 5 is resistant to degradation

Antithrombin deficiency (break clot)

177
Q

Blood type A (can sub for B)

  • antigen
  • antibody in plasma
  • Can receive from
  • Can donate to
A

A

Anti-B

A & O

A and AB

178
Q

Blood type AB

  • antigen
  • antibody in plasma
  • Can receive from
  • Can donate to
A

A and B

Neither

O, A, B & AB

AB only

179
Q

Blood type O

  • antigen
  • antibody in plasma
  • Can receive from
  • Can donate to
A

None

Anti-A and Anti-B

O only

All

180
Q

What needs to be checked in women before giving blood

A

Rhesus status

Rhesus positive = fine

Rhesus -ve cant get +ve blood

181
Q

Group and Screen:

  • what it looks at
  • when might be needed
A

Group - ABO and Rh (C,D,E) status
Screen - Screen’s for antibodies to other antigens

Any chance might need blood: trauma, haemorrhage, surgery

182
Q

When is whole blood given

A

Correcting hypovolaemia rapidly

e.g. following blood loss

183
Q

When are packed red cells given

How much does 1 unit raise Hb

A

Acute haemorrhage and chronic anaemia

Not given unless Hb less than 70g/L

1 unit raises by 10g/L

184
Q

when are Pts given

A

Thrombocytopenia + Bleeding

185
Q

When is FFP given

A

Factor V and VII in plasma

Given in DIC, Liver disease

186
Q

What can be given to cancer/chemo patient unresponsive to Abx

A

Granulocytes

187
Q

Blood transfusion complications (3 categories)

A

Infection

Acute (non-infectious)

Delayed (non-infectious)

188
Q

Blood transfusion related infection

A

HBV, HCV, HIV

189
Q

Acute transfusion reactions

A

Haemolytic: ABO incompatible

Febrile non-haemolytic: antibodies to leukocytes

Anaphylaxis

Transfusion related lung injury

190
Q

Delayed reactions

A

Iron overload

Delayed haemolytic reaction

191
Q

Acute haemolytic reaction Pres and Tx

A

Fever, Chest pain, infusion site pain

discontinue blood + give Fluids

192
Q

Allergic transfusion reaction (anaphylaxis)

A

Flushing, dyspnoea, pruritus, hypotension

discontinue blood + adrenaline + secure airway (intubate) + salbutamol ± antihistamine

193
Q

Febrils transfusion reaction

A

Fever

Stop blood, Antipyretic

194
Q

Definition of Hypersplenism

A

pancytopenia in patients with an enlarged spleen.

Causes CHINA - MMM

195
Q

CHINA MMM causes for hypersplenism

A 
Congestion (Portal HTN, Budd-Chiari)
Haematological (haemolysis, Sickle, PRV)
Infection (Malaria, EBV, HIV)
Neoplasm (CML, Lymphoma)
Autoimmune/connective tissue (RA, SLE, Sarcoid)

chronic Myeloid leukaemia
Myelofibrosis
Malaria

196
Q

Hyposplenism:

  • what is it
  • causes
  • complications
A

Hyposplenism (physiological loss of function)
e.g. sickle cell (splenic infarct) Coeliac
Splenectomy

This leads to an increased risk of infection from encapsulated bacteria (strep)

197
Q

Hyposplenism Management

A

Oral phenoxymethylpenicillin + or macrolides (azithromycin/clarithromycin)
Pneumococcal vaccine and influenza vaccine
Carry health alert card

198
Q

Types of BM transplant

A

Autologous (own BM harvested

  • reduced chance reject
  • lower risk infection

Allogenic (healthy donor)

  • must match HLA to patient (at 3 loci)
  • normally sibling/relative
199
Q

BM transplant procedure

A

1) BM Harvest under GA (Self or Donor) / Stem cells collected form blood
2) ablative chemo/radiotherapy
3) BM/stem cells given IV as inpatient

200
Q

BM transplant complications

A

infection - post procedure immunosuppression

Mucositis (chemo damages mouth/throat

Graft Vs Host if non-self (donated marrow recognises body as non-self and immune response ensues)

201
Q

Use of hydroxycarbamide

A

Sickle cell

decreased production so upreg fatal Hb

Final (26 decks)

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