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Haematology Flashcards

1
Q

What are the parameters and general causes of anaemia

A

<13.5 g/dl Men
<11.5 g/dl Women
Increased loss of RBC, reduced production, volume expansion (eg pregnancy)

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2
Q

What are the signs and symptoms of anaemia?

A

Signs- Conjunctival pallor
Symptoms- fatigue, fainting, SOB, headaches, palpitations, anorexia tinnitus
Severe- hyperdynamic circulation- tachy flow murmur (ejection systolic at the apex), HF

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3
Q

What are the causes of low MCV anaemia?

A 
Normal RBC count with decreased actual content. 
F- Iron deficiency
A- anaemia of chronic disease
S- sideroblastic
T- Thalassaemia
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4
Q

What are the causes of normocytic anaemia?

A 
Anaemia of chronic disease
Pregnancy
Acute blood loss
Bone marrow failure
Hypothyroidism
Renal Failure
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5
Q

What are the causes of macrocytic anaemia?

A 
FAT RBC +
Pregnancy
Antifolates 
Hypothyroidism
Reticulocysosis
B12 deficiency + Folate
Cirrhosis
Myelodysplasia
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6
Q

Describe a blood film for iron deficiency anaemia

A

Microcytic, hypochromic, Poikilocytes, anisocytosis

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7
Q

What are the signs and symptoms specific to iron deficiency anaemia?

A

Brittle hair and nails, angular cheilosis. atrophic glossitis, koilinichynia
Post cricoid webs (plumbers Vinson -> dysphagia)

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8
Q

What are the causes of iron deficiency anaemia?

A

Blood loss - GI bleed ( peptic/ duodenal ulcers, NSAIDs, Ca, Crohn’s), menorrhagia, polyps, hookworm
Decreased intake - vegan/ vegetarian, poor diet, early life.
Increased usage- pregnancy, breastfeeding, infants
Poor absorptions - IBD, coeliac, tropical spruce, gastric band.
Intravascular haemorrhage- MAHA, PNH

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9
Q

What are the side effects associated with the treatment of iron deficiency anaemia?

A

Oral tables- abdominal discomfort, blood stool, constipation, nausea and diahorrea.
IV- high risk of anaphylaxis - therefore should not be used in sepsis.

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10
Q

Whata re the common causes of anaemia of chronic disease?

A 
Renal failure
Rheumatoid arthritis
Vasculitis
HIV, TB, Hepatitis, osetomyletis 
Malignancy
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11
Q

What is the pathophysiology of anaemia of chronic disease?

A
  1. In renal failure, increased INF, IL1 and TNF circulating due to chronic inflammation, leads to downregulation of the EPO receptor and inturn decreased EPO synthesis.
  2. IL6 and LPS increase hepcidin, which decreases iron absorbance, this causes increased sequestration of iron into macrophages -> deprives bacteria of iron
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12
Q

What is the cause of sideroblastic anaemia?

A

Ineffective erythropoesis -> iron loading -> haemosiderosis in heart, endocrine, liver
Lead toxicity, myelodysplasia, alcohol toxicity, cytotoxic drugs, post radio RIPE, myeloproliferative.

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13
Q

What are the blood film findings in sideroblastic anaemia?

A

Ring sideroblasts - erythroid precursors with iron deposits around the nucleus and mitochondria.

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14
Q

What is the treatment of sideroblastic anaemia?

A

Treat the cause +paroxidine

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15
Q

Why should you always check CRP with iron studies?

A

Ferritin is an acute-phase protein so may be altered in acute inflammation.

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16
Q

What are the iron studies for

a. Iron deficiency
b. anaemia of chronic disease
c. pregnancy
d. sideroblastic

A

Fe2+ TIBC Ferritin

a. v ^ v
b. v v ^
c. ^ ^ N
d. ^ N ^

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17
Q

What are the investigations for pancytopenia?

A 
Abdo exam - spleen
FBC - reticulocyte cound
Iron studies, B12, Folate
Blood film (Blasts, Hairy cell, dysplasia - leukaemia)
Parvovirus PCR
Myeloma screen
Consider BM biopsy if nil else found
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18
Q

What are the megaloblastic and non-megaloblastic causes of macrocytic anaemia?

A

Megaloblastic
v B12, v Folate, Cytotoxic drugs

Non-megaloblastic
Alcohol excess, Pregnancy, Hypothyroidism, reticulocytosis

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19
Q

What are the findings on a megaloblastic blood film?

A

Megaloblasts- hypersegmented polymorphs, macrocytic anaemia, leucopenia, thrombocytopenia

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20
Q

What are the sources of B12 and Folate and therefore their respective risk factors for deficiency?

A

B12- Meat and dairy
RF- Vegan/ vegetarian, poor diet, tropical spruce, IBD, post gastric surgery, lactose intolerance, pernicious anaemia, SIBO, tapeworm

Folate - Leafy green vegetables, nuts, yeast
RF- Poor diet, IBD, tropical spruce, pregnancy, antifolates- trimethoprim, phenytoin, methotrexate, ^ cell turnover (CA, Haemolysis, inflammation, CKD)

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21
Q

What are the signs and symptoms of b12 deficiency anaemia?

A

Glossitis, angular cheilosis
Psych- irritability, depression, dementia, psychosis
Neuro- paraesthesia, peripheral neuropathy, areflexia, spastic paresis, subacute degeneration of the spinal cord

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22
Q

Define pernicious anaemia

and what is its treatment?

A

Autoimmune destruction of the parietal cells or intrinsic factor, leading to B12 deficiency.
Hydroxocobalamin IM

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23
Q

What are the investigations and findings in hereditary spherocytosis?

A

Blood film - spherocytes, membrane osmotic fragility
Coombs test: DAT -negative
Flow cytometry of RBC

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24
Q

What are the genetic inheritance patterns of which mutation for spherocytosis and elliptocytosis?

A

AD spectrin or ankyrin deficiency - hereditary spherocytosis
AR spectrin mutation:
South-East Asian ovalocytosis
Pyropoikilocytosis
AD spectrin mutations: All other elliptocytosis

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25
Q

What are the precipitants of a G6P deficiency attack?

A

Mothballs, acute infection, acute stress, broad beans (<1 day)
Medications: Asprin, primaquine, sulphonamides ( reaction 2-3 days)

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26
Q

What are the acute and diagnostic investigations for G6P deficiency?

A

Acute- Blood film - Bite cells, Heinz bodies (Blue deposits of oxidised haemoglobin), LFTs- Rapid jaundice -^ bilirubin. FBC- anaemia
Diagnostic- should be found on the Guthrie test. If not enzyme assay 3 months post-attack.

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27
Q

What is the presenting complaint of pyruvate kinase deficiency?

A

Severe and rapidly developing anaemia and jaundice at birth, splenomegaly

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28
Q

What is the pathology of sickle cell?

A 
Single point mutation in the beta haemoglobin chain.GAG-> GTG, Glu -> Val at the 6th codon
SCD - Hb SS
Trait- Hb aS
other  rarer sickle cell phenotypes:
Haemoglobin C disease Hb SC, Hb S Thal
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29
Q

What are the signs and symptoms in SCD of haemolysis and vaso-occlusion/ infarction?

A 
Haemolysis:
Anaemia, splenomegaly, folate v, gallstones, Aplastic crisis
Vaso-oclusive/ infarction:
Stroke
Infections (hyposplenism and CKD) 
Crisis - splenic, painful& chest
Kidneys- papillary necrosis and nephrosis
Liver- gallstones
Eyes- retinopathy
Dactylitis (impaired growth)
\+mesenteric ischaemia and priapism
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30
Q

When do the symptoms of SCD present (age split)

A

Childhood - stroke, splenomegaly and splenic crisis, dactylitis
Teenagers- priapism, stunted growth, gallstones depression
Adults- hyposplenism, retinopathy, CKD, Pulmonary HTN

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31
Q

What are the investigations for SCD?

A 
Guthrie test 
Haemoglobin electrophoresis
FBC- microcytic anaemia
Blood film- sickle cells
sickle solubility test.
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32
Q

What is the acute and chronic management of SCD?

A

Acute: Opioid pain management, Exchange transfusion, + additional if <60g/L Hb

Chronic
Folate and B12
Prophylaxis- ABX - pen, RSV, vaccinations - including flu
Regular exchange transfusions (check for need using carotid doppler monitoring)
Crixonlizumab (anti P selectin)
Allosteric stem cell transplant - curative

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33
Q

What are the signs, symptoms and radiological findings of beta thalassaemia?

A

Hepatosplenomegaly

XR- skull bossing, maxillary hypertrophy, hair on end skull

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34
Q

What is the spectrum of disease in alpha thalassaemia?

A

Hydrops fetalis a 4x0
a thal - a0x3 a1 moderate anaemia and splenomegaly
trait -moderate a01a1a+ a1/0

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35
Q

What is the purpose of the DAT (Coombs test)?

A

Detects Immune-mediated haemolytic anaemia

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36
Q

What are the different types of bleeding disorders and their clinical signs?

A

Vascular abnormalities
- easy bruising, nose bleeds & mucus membranes, immediate bleeding post-injury
Coagulopathy
- Delayed but profound and prolongedbleeding disproportionate to injury, bleeding into deep tissue eg muscle & joints

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37
Q

What are the congenital and acquired vascular defects that cause clotting issues?

A

Congenital: Osler-weber-rendu syndrome, connective tissue disorders (eg Ehlers Danlos syndrome)
Acquired: Senile purpura, steroids, scurvy-

Scurvy presentation: perifollicular hemorrhages- around hair follicle on legs due to ^ pressure in capillaries, infection - (meningitis, measles, degue)

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38
Q

What are the causes of platelet disorders?

A

Dysfunction
- Acquired: aspirin, cardiac bypass, uremia
-Congenital: Storage pool disease, thrombasthenia (glycoprotein v)
Thrombocytopenia
-v production -bone marrow failure
-^ destruction: (AITP=ITP), drugs- heparin, DIC, HUS, TTP

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39
Q

What are the differences between acute and chronic Idiopathic thrombocytopenic purpura?

A 
Acute      Chronic
Age           2-6y/o       adults
F:M             1:1               3:1
pre-infection /              rare
Onset     sudden   abrupt-indolent
Plt count   <20k        <50k
duration    2-6 wks     longterm
remission spont uncommon - IVIg, steroids, splenectomy
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40
Q

When would you not give folate in the treatment of anaemia and why?

A

If you were not able to definitively rule out b12 deficiency as folate can exacerbate the neuropathies

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41
Q

Define haemolytic anaemia

A

Breakdown of RBC <120 days causing anaemia

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42
Q

What are erythroid hyperplasia states susceptible to?

A

Parvovirus - aplastic crisis

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43
Q

What do the blood tests for HA find generally?

A 
^Bilirubin- unconjugated
^urogilogen
^LDH
^haptoglobin
Methaemalbuminaemia
Blood film: Reticulocytosis, ^MCV, Free Hb
Hemoglobinuria
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44
Q

What are the heritable causes of HA?

A 
Haemoglobinopathies
-SCD
-Thalassaemia
Enzyme deficiencies
-G6Pv
-Pyruvate kinase v
Membrane abnormalities
-Spherocytosis
-Elliptocytosis
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45
Q

What are the acquired causes of HA

A 
Immune
- ABO reaction
-Warm/ cold AI
Mechanical
-metal valves, trauma, 
drugs, MAHA, PNH, infection eg malaria
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46
Q

What is the pathology of hereditary spherocytosis and elliptocytosis?

A

Spherocytosis - AD spectrin or ankyrin deficiency

Elliptocytosis - All are AD spectrin mutations except Southeast Asian and ovalocytosis which are AR

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47
Q

What are the differences between warm and cold AI HA?

A 
Warm                              
Temp: >37, IgG, spherocytes     
Both can be idiopathic or lymphoma
Causes: CLL, SLE, methyldopa.     
Treatment:  Steroids, splenectomy, immunosuppressants

Cold:
<37 IgM, Raynauds
EBV, TB
Treatment: cold avoidance or cause

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48
Q

What are the causes of paroxysmal cold haemoglobinuria?

A

Infection: EBV, Measles, VZV, syphilis
Donath-Landsteiner Antibodies bind to RBC but dissociate in warmth, these cause complement-mediated haemolysis on rewarming.
Is self-limiting as IgG dissociates at warmer temperatures

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49
Q

Explain the pathology of B thalassaemia

A

B0- no expression B+ - some expression B1 - normal
Minor: B+, B+ or B0B+ asymptomatic/ mild
Intermediate - B+/B1 or B0/B+ moderate anaemia - splenomegaly, bone deformities, gallstones
Major B0 B0 - Severe anaemia at 3-6mnths, FTT, hepatosplenomegaly, bone deformity, HF

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50
Q

What is the pathology of paroxysmal nocturnal haemoglobinuria?

A

Acquired loss of GPI markers (which have a protective function on RBCs, Plts and neutrophils)
-> complement mediated lysis of RBCs-> chronic intravascular haemolysis at night

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51
Q

What are the investigations for paroxysmal nocturnal haemoglobinuria?

A

Morning -Urine dip haemoglobinuria
Coombs - DAT+
Immunophenotyping shows altered GPIs
(Ham’s test shows in vitro acid induce haemolysis)

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52
Q

Describe a blood film for MAHA and its pathology

A

Spherocytes- caused by RBCs getting damaged passing through fibrin - caused by HUS, TTP, eclampsia/ pre-eclampsia, DIC

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53
Q

What is the pathology of thrombotic thrombocytopenic purpura?

A

AI destruction of ADAMTS13 (this forms strands of VW factor that cuts through the RBCs)

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54
Q

What are the symptoms of TTP?

A

MAHA, Fever, renal impairment (worse in HUS), neurological abnormalities, thrombocytopenia

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55
Q

What is the pathology of haemolytic uraemic syndrome?

A

E.coli toxin destruction of endothelium -> fibrin mesh-> damaged RBC -> impaired eGFR +MAHA

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56
Q

What is the process of haemostasis from injury to stable plug (don’t include detail of clotting cascade)

A

Injury

  • vasoconstriction -> decreased blood flow = stable plug
  • platelet aggregation -> activates stable plug
  • direct TF stimulation of clotting cascade
  • crossing linking and fibrin stabilisation
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57
Q

Describe the clotting cascade

A

Extrinsic pathway
TF
7->7a
5->5a

Intrinsic
12->12a
   11->11a
      9->9a 
          8->8a
            10->10a
                5->5a
Common
5->5a
     prothrombin ->Thrombin
                        Fibrinogen -> fibrin
Plasminogen --TPA(Tissue plasminogen activators)--> plasmin ^ fibrin degregation
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58
Q

What parts of the coagulation cascade to aPTT, Pt and TT look at and their function

A

aPTT- activated partial thromboplastin time - Intrinsic pathway monitoring heparin
PT- Prothrombin time (INR), -Extrinsic Warfarin (INR)
TT- Thrombin time common pathway - starts at 5a

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59
Q

Describe the pathology of Haemoophalia A and B

A

Both are X-linked recessive
A- Factor VIII deficiency, 1/10Kmales
B- Factor IX deficiency 1/50Kmales

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60
Q

Describe the investigations for haemophilia A

A

^aPTT, normal PT and TT, v FVIII on assay

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61
Q

What is the management of

a. haemophilia A
b. haemophilia B
c. VWD

A

a. Avoid NSAIDs, IM injections.
Give desmopressin (as it causes VWF release, which carries factor VIII), F VIII replacement -life long
b. F IX replacement
c. May need prophylaxis, but treatment for acute bleeds is VWF and F VIII replacement, desmopressin

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62
Q

Describe the aetiology of von Willebrand’s disease

A

Type 1: defcicency in VWF
Type 2: impaired function of VWF
Type 3: Absent VWF (can present as haemophilia A)
All will have v plt function and v FVIII (as VWF carries F VIII)
AD- 1/10K

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63
Q

How does VW disease present + investigation findings?

A

Mainly as a platelet issue (mucosal, easy bruising), but can present as a clotting factor issue (deep bleeding)
^/-aPTT, normal PT, vF VIII, vVWF antigen (normal in T2), -plts

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64
Q

What is the cause of DIC?

A

Widespread activation of the coagulation leading to consumption of clotting factors and platelets
instigated by
- malignancy, sepsis, trauma, obstetric complications, toxins

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65
Q

What clotting functions are affected in liver disease?

A

v synthesis of II, V, VII, VIII,IX, XI
v Vitamin K absorption
abnormal platelet function

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66
Q

What are the bleeding risks associated with warfarin and why?

A

Warfarin is a vitamin K antagonist meaning there will be decreased F II, VII, IX and X.
It can also decrease protein S and C so may initially have a procoagulant effect but will ultimately be anticoagulant.

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67
Q

What can cause vitamin K deficiency and how can you treat it

A

Warfarin, vit K malabsorption/ malnutrition, Abx, biliary obstruction.
Treat with IV vit K or FFP in acute haemorrhage

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68
Q

What are the investigation findings in DIC?

A

Plts v, v fibrinogen, ^D-dimer, ^PT

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69
Q

Name 5 neonate specific causes of anaemia

A 
Birth trauma
Twin to twin transfusion (one ends up anaemic and one polycythaemic)
Foetal to maternal transfusion
Parvovirus
haemorrhage from the cord or placenta
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70
Q

What is congenital leukaemia

A

transient abnormal myelopoiesis
Associated with Downs
It can pass between twins

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71
Q

Define a thalsaemia and haemoglobinopathy

A

Thalassaemia is a reduced synthesis of one or more globin chains due to a genetic defect
Haemoglobinopathy - synthesis of structurally abnormal molecule

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72
Q

Which globin chains are on Chr11?

A 
Beta
Delta
Gamma
Locus control region for all these chains
Epsilon - embryonic
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73
Q

Which globin chains are on Chr16?

A

Alpha

Zeta

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74
Q

When are the different haemoglobin isoforms found in normal physiology?

A

A -aabb - normal adult + late foetal, children, infant
A2- aadd - very small amount infant, child, adult
F -aayy - Fetus and infant

75
Q

What is handfoot syndrome

A

Infarction of the long bones in the first few years of life (as these children have haemopoetic bm in long bones)

76
Q

Why does splenic sequestration occur only in children? and what are the consequences?

A

As SCD children get repeated splenic sequestration and infarction eventually resulting in a non-functional fibrotic splene.
This results in no more splenic sequestration but hyposplenisis

77
Q

Why are SCA children given folate (3 reasons)

A

Growth spurt require more folate in general
Theres a reduced RBC lifespan in SCD
Hyperplastic erythropoeisis

78
Q

What is a reason why strokes are more common in SCD children than adults?

A

Children have smaller cerebrovascular vessels which increases the risk of infarction.
prophylaxis is more thorough in adults

79
Q

When are the main changes in foetal haemoglobin during gestation?

A

<16 weeks - specfic fetal haemoglobin forms (Portland and Gower) epsilon and zeta

Alpha and gamma (maintained until 3 months )

> 32 Haemoglobin A begins to increase (about 1/3 of Hb at birth - rapid increase) alpha and beta rise

80
Q

What are the signs on blood film of oxidant damage

A

Heinz body (oxidised haemoglobin) and bite cells

81
Q

What are the differentials for haemophilia

A

Inherited thrombocytopaenia
Platelet dysfunction
Acquired defects eg ITP, acute laeukamia?
NAI

82
Q

What specific Hx question can you ask about to diagnose haemophilia?

A 
Bleed from the umbilical cord
Bleeding on vitamin K (haematoma) injection
Bleeding on heel prick test
Bleeding at circumcision
Family history
83
Q

What is yellow and red bone marrow?

A

Red bm = vascular bone contains haemopoetic precursors (metabolically active)
Yellow- fat

84
Q

What is the treatment for splenic sequestration?

A

Blood transfusion

85
Q

What is the pathology of acute chest crisis?

A

Infarction of the ribs and lungs

86
Q

What are the complications of poorly managed thalassaemia?

A

Anaemia-> HF and growth retardation
Erythropoesis-> bone expansion, hepatomegaly and splenomegaly
Iron overload-> HF, gonadal failure

87
Q

What is the management of beta thalassaemia?

A

Blood transfusions +/- chelation (deforioxamine deferiporone)
Family counselling
Monitor cerebral vessel narrowing
vaccines

88
Q

What are the differentials for ITP?

A 
Henchlochs purpura
NAI
Coagulation factor defect
Inherited thrombocytopaenia
Acute leukaemia
89
Q

What is the most common leukaemia in children?

A

ALL

AML can occur in infants

90
Q

What are the causes of congenital polycythaemia?

A

Twin to twin transfusion
Intrauterine hypoxia
placental insufficiency

91
Q

Give 4 ways haematology is different in children than adults

A

Different normal ranges
Growth spurts -> nutrient deficiencies
Lymphoid response to infection
Haematological issues can cause growth retardation, puberty failure

92
Q

How do the normal ranges for FBC differ in neonate, children and adults?

A

neonate ^WCC, ^neutrophils, ^ lymphocytes, ^Hb, ^MCV
^% of HbF
50% increase in G6PD (to protect against oxidant stress)

93
Q

What are the characteristics of acute leukaemia?

A 
Rapid onset and can result in early death if untreated
Bone marrow failure
-anaemia - fatigue, SOB, pallor
-Leukopenia- infection
Thrombocytopenia - bleeding risk
94
Q

Which chromosome does a derivative chromosome take its name from

A

From the chromosome that gives its centromere

95
Q

Explain chromosomal inversion and give an example

A

A flip from bottom to top (not whole of the chromosome chains therefore two point for chimerism) eg 16 AML - causes a fused core binding factor (regulator of haematopoiesis) with MYH11 which can no longer bind to DNA -> x differentiation so you get weird giant purple eosinophils

96
Q

How can chromosomal loss/ deletion result in leukaemia?

A

A loss of tumour suppressor gene
Or just insufficient amount to for normal haematopoiesis
A loss of DNA repair systems
Most common is 5/5q and 7/7q resulting in AML

97
Q

Whats the treatment of ITP?

A

Observation

Corticosteroids, high dose IV Ig, IV anti RhD (if RhD+)

98
Q

What are the leukaemogenic abnormalities?

A

Two hit that synergy’s
T1 - promote proliferation and survival
T2- blocking of differentiation (ordinarily cells would apoptose) instead accumulate as blast cell.

99
Q

What is the pathology of acute promyelotic leukaemia?

A

Type 1 -FLT3-ITD (mutated KIT)
Type 2. t15;17 translocation -> PML -RARA fusion gene
promyelocytes are abnormal - auer rods

100
Q

What are the clinical features of AML?

A

Bone marrow failure
-Anaemia, thrombocytopenia, leukopenia (infections eg necrotising fascitis and HSV)
Local Infiltration
(spleen, CNS and testicles are less common in AML)
Splenomegaly, hepatomegaly, Gums (monocytic), lymphadenopathy, skin, CNS (CN palsies) etc
Hyperviscosity - (M3=DIC and bleeding)

101
Q

What are the immunophenotype difference between ALL and AML

+ what they have in common

A

AML -MPO, CD13, 33, 14,15, glycophorin and plt antigens

ALL-
B- CD20, 19, TdT 10
T- CD2, 3, 4, 8 TdT

both- CD34, 45 and HLA DR

102
Q

What are the investigations for AML?

A

FBC - vHb, vPlt, ^WBC
Blood film - usually diagnostic -blasts, AUER RODS-pathognomic, granules (think basophils, eosinophils)

Flow cytometry - CD33, 12, MPO

If no blasts - bm aspirate
Molecular studies- for prognosis

Cytochemistry (usually in low resource settings- stain for myeloperoxidae, sudan black less specific and non-specific esterase)

103
Q

What is the management of AML (split into clinical presentations)

A

disease - cytotoxic drugs (Daunorubicin + cystarabin remission induction> consolidate with just cystarabin) OR
targeted molecular therapy- eg TKis or ATRA for APML, bm transplant if ^ risk of relapse
(because there is so much IV patient often have long lines

104
Q

What are the different types of chromosomal abnormalities?

A

Translocation
Deletions/ loss
Duplications
Inversions

105
Q

What is the epidemiology of AML?

A

incidence increases with age

<2y/o for children

106
Q

What type of leukaemia does DIC in a leukaemia presentation suggestion?

A

acute promyelocytic leukaemia (APML)
-acute onset haemorrhage caused by DIC and hyperactive fibrinolysis
(M3 AML)

107
Q

What genetic abnormalities can cause leukaemia in patients with normal chromosomes?

A

Point mutations
Cryptic deletions (fusion gene as a result of sticky ends joining)
Partial duplications of genes
Loss of tumour suppressor genes

108
Q

What is the epidemiology and prognosis of ALL?

A

Peak incidence in children (most common childhood malignancy)
(does still occur in adults)
Prognosis worsens - <2 or >10, WBC >20*10^9 at dx, male, non-caucasaian, T or B surface markers

109
Q

What are the clinical features of ALL?

A

Bone marrow failure
-anaemia
-Thrombocytopenia - bleeding
-leukopenia - increased infection > FEVER
Local infiltration
-Lymphadenopathy (+/-thymic enlargement), splenomegaly, hepatomegaly, gums, testes, kidneys infiltration, skin, CNS, bones (pain)
(More lymph node, CNS, testicles and thymus >aml)
(adults get less sequestering Sx)

110
Q

What are the supportive treatments for acute laekaemia?

A 
Blood products (replace v elements), folate, iron
Antibiotics (prophylaxis), vaccinations
Allopurinol
analgesics
Fluids, electrolytes to prevent TLS
111
Q

What are the leukaemogenic mechanisms in ALL?

A

Chromosomal translocation - fusion genes, (20-30% in adults are Philadelphia+)
promotors, dysregulation by proximity to T cell Recptor or Ig heavy chain loci
Unknown - hyperploidy

112
Q

What are the investigations for ALL?

A

FBC - anaemia, neutropenia (WBC can be high but all blasts), thrombocytopenia
Blood film -lymphoblasts, table tennis bats sign

(LFTs, creatinine, U&Es, calcium, phosphate, uric acid, coagulation screen)

Confirm with immunophenotyping -CD2/3/4/8/19,23

bm biopsy- lymphoblast infiltration (B or T)
Cytogenetics/ molecular - for prognosis and treatment

low resource setting - cytochemistry

113
Q

Whats the prognosis of AML and ALL?

A

AML- worse with age, 40% of adults cured
ALL - worse with age
Children 8-% disease free at 5 year, only 40% in adults

114
Q

What is the management of ALL?

A

Disease - cytotoxic agents,CNS therapy, molecular targets- eg TKis CAR-T cell, Rituximab - mAb CD20, imatinib for philidelphia
bm transplant if ^ risk of relapse.
Supportive

115
Q

What are the most common sites for chromosomal duplication in ALL?

A

Philadelphia - adults

8 and 21 (strong association with Downs)

116
Q

What presenting features should you suspect leukaemia?

A

Adults - anaemia, new onset abnormal bruising/ bleeding, infection, organomegaly
Children - boney pain, limping, pallor, bruising, organomegaly

117
Q

What are the different methods of immunophenotyping?

A

immunocytochemistry (FISH- monoclonal antibodies)
immunohistochemistry
Flow cytometry

118
Q

What are the iron demands during pregnancy?

A 
300mg Foetus
500mg Mother (increased RBC size)
119
Q

What are the folate requirements in pregnancy

A

200mcg/day for growth and cell division

120
Q

What supplements are recommended in pregnancy

A

Folate - WHO 400mcg/day preconception and throughout pregancy to prevent neural tube defects
Iron - not routinely recommended but often taken 30mg/day required WHO 60mg/day

121
Q

What is the physiological fall of platelets in pregnancy?

What is the physiological cause of this drop?

A

10% fall,
Main fall is in the 3rd trimester due to increased activation and destruction (larger platelets released prematurely-automated counter can mistake them for RBC)

122
Q

What is the physiological fall of platelets in pregnancy?

What is the physiological cause of this drop?

A

10% fall,- gestational or incidental
Main fall is in the 3rd trimester due to increased activation and destruction (larger platelets released prematurely-automated counter can mistake them for RBC)

123
Q

What are the pathological causes of thrombocytopenia in pregnancy?

A

Pre-eclampsia (^activation and consumption 50% of PET) - HELLP
ITP (5% of pregnancies)
microangiopathic- MAHA, TTP, HUS
Non-gestational related- Bm failure, leukaemia, DIC, TTP, HUS

124
Q

What is the platelet threshold for normal delivery and epidural?

A

50 x10^9/L for delivery

70x 10^9/L for epidural

125
Q

Why may there be macrocytosis in pregnancy?

A

Physiological expansion

B12 or folate deficiency

126
Q

What changes may be seen on FBC in pregnancy?

A 
^red cell mass (20-30%)
^plasma volume (50% by end of 2nd tri)
^MCV
^neutrophils
vPlts (^size)
Dilution effect
127
Q

What is the management of ITP perinatal and post-natally?

A

IV Ig
Steriods
Anti-D if Rh +ve

Monitoring of chord gases and baby.
IV Ig if severe bleeding

128
Q

Why are pregnant women at a higher VTE risk?

A 
Hyperemesis (dehydration)
Bed rest obesity, PET, operative delivery, multiple pregnancy
Larger platelets
^clotting factors -FVIII and vWF (x3-%)
Fibrinogen x2
FVII 0.5x
Protein S v
^PAI-1 and PAI 2 (placenta;)
129
Q

What are the investigations for PE/DVT in pregnancy?

A

Calf doppler
CTPA (^maternal breast cancer risk) or V/Q (^childhood cancer risk)
D-dimer is not helpful as elevated in pregnancy

130
Q

What is the management of VTE risk in pregnancy?

A

Early mobilisation and hydration
High risk- LWMH OD/BD- doesn’t cross the placenta - stop when labour starts and 24 hrs after delivery resume
(DO NOT change to asprin as tetraogenic)
TEDs

131
Q

What are the indications to check for antiphospholipid syndrome?

A

Recurrent miscarriage >3 consecutive <10 weeks
>1 >10 weeks normal anatomy miscarriage
>1 preterm birth before 34 weeks due to placenta

132
Q

What are the names for a placenta that implants deeper than the endometrium?

A

Placenta accreta - through the endometrium
Placenta Incretra - into the uterine wall
Placenta pancreta- through the uterus and can attach to other organs

133
Q

What are the causes of post partum haemorrhage?

A

> 500ml blood loss, MOH >1L
Uterine Atony - biggest cause
Trauma- eg uterine Artery disection
Hameatological - DIC (resus, abruption, amniotic fluid embolism)

134
Q

What are the precipitants for DIC decompensation?

A 
Amniotic fluid embolism
Placental abruption
missed miscarriage
pre-eclampsia
sepsis
135
Q

What are the PC of amniotic fluid embolism?

A

3rd trimester severe rigors, shock, nausea and vomiting DIC

136
Q

What are the options for haemoglobinopathy screening?

A

<12 weeks FBC following questionnaire -microcytosis
HPLC - for beta thal
Maternal screening then if positive/ recessive partner screening
If positive:
CVS sampling 10-12 weeks
Amniocentesis 15-17 weeks - foetal blood sampling
USS - Hydrops

137
Q

What complications are associated with SCD in pregnancy?

A 
IUGR
Miscarriage
Pre-term labour
PET
VTE
138
Q

What is the presentation of CML?

A

Usually asymptomatic and incidental finding on FBC = ^neutrophils and basophils
(age 35-55)

General, malaise, fatigue, weightloss
worsening in accelerate and blast phase
BM suppression (often has sufficient room to still produce)
Massive hepatosplenomegaly, boney pain, CNS > nerve palsies, gum hypertrophy

139
Q

What are the investigations and findings for CML?

A

FBC - -/vHb, early^, late =vPlt, ^WBC 12-400- neutrophils and basophils (all maturities)
Film - Left shift - myeloblasts, undifferentiated neutrophils, basophilia

Immunotyping - PHILADELPHIA>90% t9,22, - BCR-ABL (confirm with PCR

BM biopsy = hypercellular - myelocytes, mature granulocytic cells

140
Q

What are the stages and appropriate treatment for CML?

A

Chronic - <5% blast cell in blood - IMATINIB
(Rarely needed 2nd line
swap to other TKi, 3rd allo HSCT + chemo)

Accelerated- >10% blasts, ^ S&S, may need more supportive therapy

Blast - >20% blasts (FLAWS) treat as AML - TKis, ATRA followed by HSCT.

141
Q

What is the presentation of CLL?

A

Usually asymptomatic - incidental finding on FBC - ^WBC (lymphocytes)
Symmetrical painless lymphadenopathy, SOB
BM failure
FLAWS - poor prognostic
Hepatosplenomegaly (NOT massive than CML)

142
Q

What is the difference between CLL and SLL?

A

CLL and SLL are the same disease but CLL is predominantly found in the bone marrow. SLL is predominantly found in lymphnodes

143
Q

What are the RF for CLL?

A

> 65, male, white, AI - Evans syndrome (AIHA and ITP), Trans form TO >DLBC lymphoma = Richter’s transformation

144
Q

Describe the investigations and findings for CLL

A

FBC - HB-/v(more aggressive), -/- Plt, ^WBC - lymphocytes >100 (no neutrophils)
FIlm - SMUDGE/ smear cells +/- spherocytes, polychromasia, small mature lymphocytes
Flow cytometry - all same markers (eg all Kappa or Lambda = clonal)
Immunotyping - C23+, CD5+
BM - lymphocytic replacement

145
Q

What are the poor progonist indicators for CLL?

A

vHb, vPLt, ^LDH, CD38+ve

11q23 deletion

146
Q

Describe the staging and appropriate treatment for CLL

A

A- ^WBC, <3 groups of lymphnodes - watch and wait
may need supportive

B - ^WBC, >3groups of lymph nodes - consider Tx (especially if AI eg ITP or AIHA)

C- anaemia and thrombocytopenia
Treats Atemtutzumab + HSCT or clinical trial eg rituximab, FCR chemo now biologics

147
Q

What is the SBA difference between hodkin’s and non-hodkins lymphoma?

A

Hodkins hurts after alcohol
- Soreness of Ln
NH does not

148
Q

Describe the ann-arbor staging

A

Stage 1- 1 collection of LN (can include spleen)
Stage 2 - 2+ collections of LN on one side of the diaphragm
STage 3 - 2+ collections of lympnodes either side of the diaphragm
Stage 4- extranodal

149
Q

Describe the presentation of Hodkin’s lymphoma

A

Asymmetrical painless lymphadenopathy.
Obstructive symptoms - SVC syndrome - cough, SOB, distended neck veins, facial plethora.
Abdo pain, chest pain, puritis, hepatosplenomegaly, tonsillar enlargement
FLAWS
pain on drinking alcohol

150
Q

Describe the histology of immunohistology of Hodkin’s lymphoma

A

Reed Sternberg cells (binucleated cells on background of lymphocytosis and reactive cells)
CD15,30

151
Q

What are the different types of Hodkin’s lymphoma?

A 
Nodular Sclerosisng CD20+ (cd45-)
Mixed cellularity
Lymphocyte rich
Lymphocytes depleted
Nodular predominant -cd20- and CD45+
152
Q

What is the management of hodkin’s lymphoma?

A

Chemo - ABVD (adriamycin, bleomycin, vinblastine, dacarbazine)
Radio adjunct

Allo or auto HSCT

153
Q

What haematological malignancy is associated with starry sky histology?

A

NH Lymphoma - very Aggressive Burkitt’s

154
Q

Describe the presentation of non-hodkin’s lymphoma

A

Heterogeneous dependet on site
]Asymmetrical painless lymphadenopathy, often muti site
FLAWS

155
Q

What are the different types of NH lymphomas?

A

Very aggressive - Burkitt’s

aggressive - Mantle, Diffused large B cell

Low grade - Follicular, marginal zone, SLL, MALToma

T cell- anaplastic, peripheral, adult, eneropathy-associated, cutaneous.

156
Q

What haematological malignancies are associated with EBV?

A

HL, Burkitt’s - Endemic and sporadic (not immundef)

157
Q

What is the treatment for

a. Burkitt’s Lymphoma
b. DLBC
c. Mantle
d. folliclar
e. MALTomas
f. T cell

A

a. Rituximab (+ leaukamia tx)
b. Riximab-CHOP (autoHSCT for relapse)
c. Rituximab- CHOP (autoHSCT for relapse)
d. Watch and wait (or rituximab)
e. treat cause eg H.pylori
f. Alemtuzumab = antiCD25

158
Q

What are the presenting symptoms of multiple myeloma?

A

Calcium - bones, stones, groans and mones

Renal failure - nephrOtic syndrome (prOtein) +/- amylodosis (deposits - macroglossia, splenomegaly, restrictive cardiomyopathy, peripheral neuropathy)

Anaemia - normocytic +/- pancytopenia (BM infiltration sx)

Bones - osteolytic lesions, - boney pain or pathological fractures

Hyperviscoity - blurry vision, nose bleeds

159
Q

What are the investigations for MM?

A 
Bloods
FBC - anaemia, plt v/-, WBC v/-
Bone profile - ^ Ca, v PTH, ^ALP
U&Es - ^ creatinine
^ESR
Immunoglobulins - 1 thick line will indicate Ig G>A>M
Blood film - roulouxs
Urinalysis - bence jones proteins
Skeletal survey (CT or MRI) - osteolytic lesions of the long bones, spine and skull
BM biopsy - >10% plasma cells
160
Q

What are the treatment options for MM?

A

Symptoms - Bisphosphonates, glucocorticoids, fluids, abx, immune modulators - linaliomide, thalidomide

remission induction
Chemo
- ciclophosphamide, Bortezombi +/- dex
Allo HSCT

161
Q

Describe the mechanism of renal failure in MM

A

Bence Jones proteins are freely filtered but not resobed, they are toxic to the PCT
They form a proteination block in the DCT > ^ calcium > calcinosis

162
Q

What are the differences between MM, smoldering MM and MGUS?

A

MM
CRAB with organ failure
>30% plasm cell on BM, M spike >30g/l, treat

Smoldering
no Sx or organ failure
>10% plasma cells, >30g/l, monitor high change rate

MGUS
no Sx
<10% plasma cells, <30g/l clonal protein, no tx just monitor

163
Q

What syndrome os associated with elevated IgM

A

Waldenstrom’s macroglobulinaemia

  • causes hyperviscosity, weightloss anf fatigue
  • Tx Rituximab
164
Q

What is the clinical presentation of MDS?

A

heterogeneous due to which myeloid maturation is defective
Slow development of BM failure
anaemia, thrombocytopenia and leucopenia

165
Q

What are the possible investigation findings in MDS?

A

FBC - vHb, Pltv/-, wbc v/-
Film - sideroblasts - nucleated with perinuclear granules, hyposegmented neutrophils and v granulation of WBC, megakaryocytes (hypolobulated), <20% blasts

166
Q

Describe the management of MDS

A 
supportive
- Blood products
Disease modifying
-Lenalidomide (5q deletion)
-Azacytidine
-Chemo (daunorubicin and cytrarabine)
Definitive - HSCT
167
Q

How is MDS different from AML?

A

AML has >20% in BM

MDS <20%

168
Q

What are the mutations commonly associated with myeloproliferative disorders (2)

A 
JAK2
Philadelphia (CML)
169
Q

What are myeloproliferative disorders? (+ examples)

A

Essential thrombocythaemia
Polycythaemia Vera
Myelofibrosis

170
Q

What are the different causes for polycythaemia (3 categories)

A

Primary: Ruba vera and familial Polycythaemia
Secondary - ^EPO - chronic hypoxia - COPD and high altitude or exogenous
Pseudo (v plasma volume eg dehydration)

171
Q

What are the causes of Myelofibrosis?

A

JAK2
Primary = idiopathic
Secondary =
Development from polycythaemia ruba vera and Essential thrombocythaemia or leukamia

172
Q

What is the presenting complaint for myelofibrosis?

A

BM Failure
anaemia, neutropenia, thrombocytopenia
Splenomegaly (can be massive - extra medullary haematopoesis)
weightloss, fever
Budd-Chiari ( clots blocking hepatic vein > nutmeg liver)

173
Q

Describe the investigation finding of myelofibrosis

A

FBC - vHb, vWBC, vPlt
FIlm - tear drops, poikilocytes, leukoerythoblasts
BM - dry tap

174
Q

Describe the management of myelofibrosis

A

HPST - curative (must be done early)
Ruloxitinib - JAKi
or hydroxycarbamide, thalidomide or steroids

175
Q

Describe the pc of essential thrombocytosis

A

Often incidental finding of Plt >450 consistentely

VTE, gangrene, haemorrhage, erythomegalgia (burning on heat exposure)
splenomegaly, dizziness, head aches, visual disturbances

176
Q

Describe the investigations for essential thrombocytosis

A

FBC - Plt >450 consistently or >600
Blood film = megakaryocytes and fragmnents
Bm- ^ megakaryocytes

177
Q

Describe the management of essential thrombocytosis

A

Asprin (prophylactic)
Hydroxycarbamide therapeutic
anagrelid (v formation of plt)

178
Q

Describe the presentation of polycythaemia ruba vera

A

Hyperviscosity, hypervolaemia, hypermetabolism

Plethora, blurred vsion, red nose, gout, retinal vein engorgement, splenomegaly
^ VTE risk - abnormal site thrombosis
Aquagenic purritis

179
Q

Describe the investigations and management of polycythaemia ruba vera

A

FBC - ^ Hct >0.53M and >0.48F, often ^Plt
Management - Asprin prophylaxis
Venesection
Hydroxycarbamide

180
Q

What are the different types of aplastic anaemia

primary and secondary

A

Primary

  • Fanconi’s anaemia
  • Dyskeratosis congenita
  • Schwachman-DIamond syndrome
  • DIamond blackfan syndrome

Secondary
-malignant infiltration, radio, chemo, AI (SLE), viral

181
Q

What is the management of aplastic anaemia?

A

Supportive - replacement transfusions, iron chelation and antibiotics

Immunosuppressant if idiopathic
HSCT

Promotion of BM recover = growth factor and oxymethalone

182
Q

Describe the pc of

a. fanconi’s anaemia
b. Dyskeratosis congenita
c. Schwachman-Diamon syndrome
d. Diamond black syndrome

A

a. anaemia, facial abnormalities, pancytopenia, skeletal abnormalities (thumbs), horseshoe kidney, short, Intellectual deficit, hypo and hyperpigmentation
b. skin pigment, nail dystrophy, oral leukoplakia, bm failure
c. skeletal abnormalities, ^risk of AML, pancreatic dysfunction, hepatic dysfunction, short
d. thumb deformities, coarctation, VSD, ptosis and stribismus

183
Q

Where do the leukaemia’s arise (along normal blood celll maturation)

A

Acute - either haemocytoblasts or common lymphoid/ myeloid progenitor
Chronic
CLL = small lymphocytes (or more differentiated
CML= Common myeloid progenitor
Myeloma = plasma cells

184
Q

What are the causes of massive splenomegaly?

A

CML
Myelofibrosis
Lishmaniasis

Pathology (6 decks)

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