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Phase 1c > Haem > Flashcards

Haem Flashcards

1
Q

what is mcv for microcytic anaemia and what does it suggest

A

MCV < 80
insufficient haemoglobin production

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2
Q

what is mcv for normocytic anaemia and what does it suggest

A

mcv 80-100
decreased blood volume or decreased erythropoiesis

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3
Q

what is mcv for macrocytic anaemia and what does it suggest

A

mcv > 100
defective DNA synthesis and repair

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4
Q

clinical features of anaemia

A

pallor
dyspnoea on exertion
fatigue
tachycardia (bcos need more blood)
koilonychia (iron deficiency)
pica

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5
Q

what would iron studies for iron deficiency anaemia show

A

low ferritin (confirms IDA)
raised transferrin
increased TIBA

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6
Q

what would iron studies show for anaemia of chronic disease

A

high ferritin
low transferrin
reduced TIBC
raised ESR
increased hepcidin

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7
Q

what investigation would you use to diagnose thalassaemia

A

haemoglobin electrophoresis

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8
Q

what investigation would you use to diagnose sickle cell disease

A

haemoglobin electrophoresis

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9
Q

what does increased HbA2 suggest

A

beta thalassemia trait

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10
Q

what does blood film show for iron deficiency anaemia

A

pencil poikilocytes
hypochromic and microcytic red cells
target cells
anisopoikilocytosis

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11
Q

investigations for macrocytic anaemia

A

vit b12 and folate levels
homocysteine and methylmalonic acid levels

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12
Q

what do hypersegmented neutrophils on blood film suggest

A

megaloblastic anaemia (b12/folate deficiency)

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13
Q

test for autoimmune haemolytic anaemia

A

DAT test (aka Coombs test)

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14
Q

lab results for haemolytic anaemia

(and first line treatment)

A

raised unconjugated bilirubin
raised LDH (raised when intracellular contents released)
raised reticulocytes
low haptoglobin
spherocytes on blood film
may be warm (IgG antibodies, SLE, CLL) or cold (IgM antibodies, mycoplasma, mononucleosis, lymphoma)
Tx with steroids (prednisolone)

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15
Q

management for anaemia

A

blood transfusion with RBCs if severe
nutrient replacement
IDA > ferrous sulfate

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16
Q

Ix for microcytic anaemia

A

FBC
blood film
blood chemistry
iron studies

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17
Q

mnemonic for types of microcytic anaemias

A

Find Those Small Cells Last

Fe deficiency
Thalassemia
Sideroblastic
Chronic disease
Lead poisoning

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18
Q

causes of iron deficiency anaemia

A

decreased intake:
-eating disorders
-dietary restrictions
-food insecurity

decreased absorption
-coeliac disease
-surgical resection of GI tract
-bariatric surgery
-excessive dietary calcium
-tannates
-oxalates

increased need and growth
-pregnancy, lactation
-growing children

increased loss of blood

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19
Q

complications of iron deficiency anaemia

A

high output heart failure
angina
cardioresp failure
impaired growth and development

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20
Q

signs and symptoms of iron deficiency anaemia

A

split into decreased oxygen to tissues and effects on epithelium

decreased oxygen to tissues
-pallor
-fatigue
-exertional dyspnoea
-angina
-compensation: palpitations, increased pulse, increased cardiac output, tachypnoea, shunting of blood to vital organs

effects on epthelium
-glossitis
-scaling, fissuring, dryness, lip scaling
-koilonychia
-oesophageal stricture

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21
Q

lab results for IDA

A

low RBC count
low/normal reticulocytes
low hb, haematocrit
hypochromic-microcytic erythrocytes
- low MCV, low MCH, low MCHC

blood film shows rbc with central pallor, anisocytosis, poikilocytosis, target cells

iron studies show:
decreased serum iron
decreased ferritin
high transferrin
increased total iron binding capacity

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22
Q

treatment for IDA

A

PO iron supplements (ferrous sulfate)
parenteral iron for severe persistent anaemia or intolerance or non adherence to PO iron

increase dietary iron
vit C increases absorption
calcium DECREASES absorption

blood transfusion

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23
Q

components of HbF

A

2 alpha globin + 2 gamma globin

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24
Q

components of HbA

A

2 alpha globin + 2 beta globin

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25
Q

components of HbA2

A

2 alpha globin + 2 delta globin

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26
Q

ethnic group beta thalassaemia most commonly seen

A

Mediterranean descent

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27
Q

ethnic group associated with alpha thalassaemia

A

asian and african

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28
Q

what types of beta thalassaemia are there

A

beta globin chains are coded by two alleles so two forms of the disease

beta thalassaemia minor (one defective allele)
beta thalassaemia major (two defective alleles)

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29
Q

what are the types of alpha thalassaemia

A

alpha globin chains are coded by four alleles so four forms of the disease

silent carrier (one defective)
alpha thalassaemia trait (two defective)
haemoglobin H disease (three defective)
haemoglobin bart disease (four defective alleles)

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30
Q

clinical features of beta thalassaemia

A

minor - mild microcytic hypochromic anaemia, symptomatic

major- severe haemolytic anaemia, hepatosplenomegaly, skeletal deformities

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31
Q

clinical features of alpha thalassaemia

A

silent carrier - asymptomatic

alpha thalassaemia trait - microcytic hypochromic red cells, no anaemia

haemoglobin H (HbH) disease - microcytic hypochromic anaemia with anaemia

haemoglobin bart disease - intrauterine death

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32
Q

Ix for thalassaemia

A

Hb - electrophoresis to check HbA2 levels and HbH
FBC (microcytic hypochromic anaemia)
raised reticulocytes

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33
Q

interpretation of Hb electrophoresis for thalassemia

A

minor alpha thalassemia:
normal or low MCV/MCH, normal or low HbA2, normal HbF

HbH disease:
low MCV/MCH, normal or low HbA2, normal or high HbF, HbH present

beta thalassemia:
low MCV/MCH, high HbA2, high HbF, absent HbH

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34
Q

management for thalassaemia

A

thalassaemia major - lifelong transfusion therapy - may lead to iron overload and organ failure so iron chelation therapy with desferrioxamine (deferoxamine)

genetic counselling and screening tests for relatives

folic acid supplements to support erythropoiesis

consultation with cardiology, other specialties

ongoing monitoring

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35
Q

complications of beta thalassaemia

A

haemolytic, microcytic, hypochromic anaemia:
- chronic leg tissue hypoxia
- leg ulcers
- high output heart failure
- hypermetabolic state > nutritional deficiency

extramedullary haematopoiesis:
- bone marrow hyperplasia which can lead to structural malformations

haemolysis
- increased bilirubin leads to gallstones

iron overload
- myocardium > arrythmia, restrictive cardiomyopathy, heart failure
- endocrinopathies
- liver cirrhosis and hepatocellular cancer
- renal insufficiency

treatment related complications

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36
Q

signs and symptoms of thalassemia

A

hypoxia:
-systemic: pallor, fatigue, activity intolerance
-cardiac: low bp, tachycardia, arrythmias

chronic haemolysis:
- jaundice, dark urine, hepatosplenomegaly

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37
Q

lab results thalassaemia

A

reduced serum Hb
hypochromic microcytic erythrocytes
blood smear (poikilocytosis, anisocytosis, erythroblasts, target cells)
increased LDH
increased unconjugated bilirubin
decreased haptoglobin
increased serum iron and ferritin

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38
Q

how does chronic disease cause anaemia

A

infective organism wants to utilise body’s iron
cytokines switch off iron transport via hepcidin in response
increases iron storage and decreases its presence in serum

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39
Q

is anaemia of chronic disease normocytic or microcytic

A

usually normocytic but eventually become microcytic when iron is depleted

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40
Q

point mutation for sickle cell disease

A

beta globin gene, Chr 11
autosomal recessive

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41
Q

sickling of cells is predisposed by

A

hypoxia
dehydration
acidosis
infection

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42
Q

lab results for sickle cell disease

A

blood film:
Howell-Jolly Bodies
Sickled cells

vaso-occlusion, haemolysis, splenic sequestration > decreased haemoglobin, haematocrit, RBC count

reticulocytosis

increased WBC count

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43
Q

what happens during sickle cell crisis

A

acute painful crisis
stroke
sequestration crisis
chronic cholecystitis

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44
Q

management of sickle cell crisis

A

mnemonic - Sickle Acute Painful Crisis
Supportive oxygen
Antibiotics (if needed)
Pain relief
Cannula (IV fluids) and Crizanlizumab for prevention

Exchange blood transfusion

Splenectomy

Cholecystectomy

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45
Q

Diagnostic tests for sickle cell anaemia

A

Hb electrophoresis
blood film

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46
Q

complications of sickle cell anaemia

A

dactylitis
acute chest syndrome
haemolytic anaemia
priapism
aplastic crisis

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47
Q

management of sickle anaemia

A

conservative:
-trigger avoidance
-vaccination

medical:
-vaccinations
-hydroxyurea (increases cell deformability, decreases RBC endothelial adhesion, reduces sickling)
-hydroxycarbamide
-prophylactic Abx
- L-glutamate (reduced sickling)
- pain management

surgical:
-bone marrow transplant (curative)

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48
Q

what type of anaemia is sickle cell disease

A

normocytic haemolytic anaemia

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49
Q

causes of megaloblastic anaemia

A

cobalamin and/or folate deficiency
b12 deficiency
impaired DNA synthesis during erythropoiesis
insufficient diet or increased requirements
malabsorption

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50
Q

what causes pernicious anaemia

A

vitamin b12 deficiency secondary to intrinsic factor (IF) deficiency

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51
Q

signs and symptoms of megaloblastic anaemia

A

fatigue, activity intolerance, pallor
compensatory mechanisms: increased heart rate, bounding pulse

jaundice, splenomegaly

from neuronal demyelination: numbness, glove and stocking paraesthesia, weakness

hyporeflexia
rombergs +ve

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52
Q

lab results megaloblastic anaemia

A

increased MCV and MCH
hypersegmented neutrophils
anisocytosis and poikilocytosis
decreased serum hb and haematocrit
decreased serum b12 or folate levels
increased homocysteine and methylmalonic acid

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53
Q

causes of non-megaloblastic macrocytic anaemia

A

Myelodysplasia
Hypothyroidism
Liver disease
Alcohol

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54
Q

features of haemolytic anaemia

A

scleral icterus
pale conjunctivae and skin

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55
Q

blood test results haemolytic anaemia

A

Hb low
Haptoglobin low
Unconjugated bilirubin raised
LDH raised

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56
Q

Causes of hereditary haemolytic anaemias

A

membrane - hereditary spherocytosis
enzymes- G-6-P-D deficiency
haemoglobin - sickle cell, thalassaemia

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57
Q

causes of g6pd deficiency

A

genetic - x linked recessive
certain drugs, fava beans
infection
oxidative stress > build up of free radicals

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58
Q

signs and symptoms of g6pd deficiency

A

asymptomatic until exposed to oxidative stress
acute haemolysis:
-pallor, jaundice, dark urine
-abdo/back pain due to increased splenic activity
favism

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59
Q

investigations and results for g6pd deficiency

A

decreased hb
heinz bodies
bite cells
elevated bilirubin
elevated LDH
decreased haptoglobin
reticulocytosis

screening
g6pd assay

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60
Q

what could parvovirus b19 cause

A

aplastic crisis

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61
Q

signs and symptoms of hereditary spherocytosis

A

jaundice
anaemic signs and symptoms
splenomegaly

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62
Q

ix for hereditary spherocytosis

A

osmotic fragility test + coombs test negative
low hb
reticulocytosis
spherocytosis
schistocytes
elevated bilirubin, elevated ldh, decreased haptoglobih

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63
Q

treatment for hereditary spherocytosis

A

splenectomy
blood transfusions
phototherapy
folic acid supplements

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64
Q

what do you find on blood film for microangiopathic haemolytic anaemia

A

sheared RBCs - schistocytes

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64
Q

what do you find on blood film for microangiopathic haemolytic anaemia

A

sheared RBCs - schistocytes

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65
Q

haemolytic uraemic syndrome triad

A

thrombocytopenia
microangiopathic haemolytic anaemia
acute kidney injury

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66
Q

typical patient profile for haemolytic uraemic syndrome

A

child infected with shiga toxin-producing e.coli with bloody diarrhoea

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67
Q

what is atypical haemolytic uremic syndrome

A

no preceding diarrhoea

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68
Q

lab results for haemolytic uremic syndrome

A

requires triad
proteinuria, haematuria
schistocytes on blood film

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69
Q

differential diagnosis for haemolytic uraemic syndrome

A

TTP - measure ADAMTS13 activity in plasma
DIC - DIC panel (pTT, INR, d-dimer, fibrinogen)

70
Q

abx for haemolytic uraemic syndrome

A

none!
shiga-like toxin clears in days to week, abx not recommended as dead bacteria potentially release more toxins

71
Q

what is disseminated intravascular coagulation

A

severe concurrent clotting and bleeding

72
Q

causes of DIC

A

pancreatitis
sepsis
obstetric complications
cancers
trauma
ABO reaction

73
Q

features of DIC

A

bleeding features:
- petechiae
- ecchymoses
- haematuria

clotting features:
- prolonged APPT
- prolonged PT

haemolytic features:
- jaundice
- conjunctival pallor

+ features of underlying pathology

74
Q

lab results for DIC

A

low platelets
low Hb
low fibrinogen
low clotting factors
increased PT
increased aPPT
increased FDPs
increased d-dimer
schistocytes due to MAHA

75
Q

cause thrombotic thrombocytopaenic purpura

A

defunct ADAMTS-13 enzyme

76
Q

clinical signs for TTP

A

ADAMTS:

Antiglobulin negative
Decreased platelets
AKI
MAHA
Temperature
Swinging CNS signs

77
Q

DIC management

A

treat underlying disease
platelet + coagulation factors transfusion
anticoagulation - heparin

78
Q

complication for DIC

A

acute renal failure
life-threatening haemorrhage
haemothorax
gangrene

79
Q

blood cancer classification

A

leukaemia:
- myeloid
- lymphoid

lymphoma:
- Hodgkin
- non-Hodgkin

other:
- myelodysplasia
- myeloma
- polycythaemia vera, myelofibrosis, etc

80
Q

which cells are affected acute myeloid leukaemia

A

rapid proliferation of MYELOBLASTS
these crowd out bone marrow so you don’t get downstream production of neutrophils and other granulocytes

81
Q

risk factors for AML

A

incidence increases with age
down’s syndrome
irradiation
anti-cancer drugs

82
Q

signs and symptoms AML

A

bone marrow failure: pallor, bleeding, infections
tissue infiltration: swollen gums, mild splenomegaly

Auer rods on cytology
signs of neutropenia, anaemia, thrombocytopenia

83
Q

what is acute promyelocytic leukaemia

A

hyper-aggressive subtype of AML
genetic translocation - fuses RAR alpha gene with PML gene

Faggot cells on cytology- lots of Auer rods

84
Q

what cells are proliferated in ALL

A

lymphoblasts

85
Q

most common childhood blood cancer

A

ALL

86
Q

risk factors ALL

A

childhood
genetics
radiation
influenza

87
Q

signs and symptoms of ALL

A

bone marrow failure: pallor, bleeding, infections
tissue infiltration: lymphadenopathy, hepatosplenomegaly, swollen testes, tender bones

88
Q

how do signs and symptoms of ALL differ from AML

A

lymph node swelling common in ALL unlike AML
B symptoms often present in ALL (fever, night sweats, weight loss)

89
Q

hyperproliferation of which cell types in CML

A

myeloid stem cells (granulocyte precursor)

90
Q

3 stages of CML

A

chronic phase
accelerated phase
blast crisis

91
Q

risk factors for CML

A

male
Philadelphia chromosome (BCR-ABL1 fusion gene)

92
Q

signs and symptoms of CML

A

up to 50% asymptomatic
massive splenomegaly

hypermetabolic symptoms:
- weight loss, malaise, sweating

bone marrow failure:
- pallor, bleeding, infections

hyperviscosity symptoms: (too many rbcs)
- thrombotic events, headaches

93
Q

pathophysiology of CLL

A

progressive accumulation of functionally incompetent lymphocytes caused by failure of apoptosis

94
Q

ALL bone marrow biopsy result

A

> 20% lymphoblasts on bone marrow biopsy

95
Q

risk factors CLL

A

male
genetics

96
Q

signs and symptoms of CLL

A

50% asymptomatic
occasionally non-tender lymphadenopathy
occasional bone marrow failure symptoms

97
Q

how is CLL usually diagnosed

A

routine blood test - lymphocytosis
smear/smudge cells on blood film

98
Q

leukaemia investigations to order

A

FBC
- unusual cell counts

LDH
- elevated in cancer

Blood smear
- to look at cells

Bone marrow aspirate
- enables conclusive diagnoses

CXR
- to look for mediastinal lymphadenopathy (ALL)

Immunophenotyping
- looking at cell markers and antigens to enable leukaemia classification

99
Q

how do patients with lymphoma present

A

lump + systemic symptoms (FLAWS)

100
Q

hodgkins lymphoma risk factors

A

bimodal age distribution (peaks between 20-30 and >50)
50% association with EBV (warning: EBV presence non specific)

101
Q

hodgkins lymphoma sign and symptoms

A

painless enlarging neck mass which may become painful after alcohol consumed

B symptoms

non tender rubbery lymphadenopathy with splenomegaly +/- hepatomegaly

neutrophilia

102
Q

diagnosis of Hodgkins lymphoma

A

Reed-Stenberg cells on lymph node biopsy
these are bi-nucleate lymphocytes

103
Q

Who’s going to ace their exams

A

chelsea, hannah, maz and wese

104
Q

risk factors for non Hodgkins lymphoma

A

EBV, HIV, SLE, Sjorgens
incidence increases with age

105
Q

signs and symptoms of non-Hodgkins lymphoma

A

painless enlarging mass in neck, axilla or groin
B symptoms (tho less common than HL)
organ involvement - skin rashes, headache, hepatosplenomegaly
neutropenia

106
Q

diagnosis of non hodgkins lymphoma

A

no reed-sternberg cells on lymph node biopsy

107
Q

Burkitt lymphoma usually cancer of what cells

A

b lymphocytes

108
Q

risk factors of Burkitt lymphoma

A

strong association with EBV infection
chronic malaria reduces resistance to EBV
HIV

109
Q

presentation of burkitt lymphoma

A

rapidly enlarging lymph node in jaw

110
Q

what does microscopy show in burkitt lymphoma

A

starry sky appearance

111
Q

how is lymphoma staged

A

Ann Arbor staging system

stage 1: single lymph node region or single organ

stage 2: two or more lymph node regions same side of diaphragm

stage 3: two or more lymph node regions above or below the diaphragm

stage 4: widespread disease, multiple organs with or without lymph node involvement

112
Q

cause of tumour lysis syndrome

A

metabolic abnormalities that arise as result of cancer treatment
release of contents from lysed cells causes a constellation of symptoms

113
Q

symptoms of tumour lysis syndrome and how they arise

A

released phosphate:
- forms calcium phosphate crystals
- leads to kidney failure and hypocalcaemia

released potassium:
- causes hyperkalaemia
- leads to arrythmia

released uric acid:
- forms urate crystals
- leads to gout

114
Q

which cells are proliferated in multiple myeloma

A

do not be fooled - not myeloid lineage

proliferation of plasma cells

115
Q

what do cancerous plasma cells produce in multiple myeloma

A

monoclonal immunoglobulin (IgG or IgA)

116
Q

risk factors for multiple myeloma

A

age > 70
Afro-Caribbean
ionising radiation
agricultural work (benzene, herbicides)
HIV

117
Q

presentation of multiple myeloma (hint: cancer star sign symbol)

A

CRAB

Calcium high: bones, stones, abdo groans
Renal impairment: worse prognosis
Anaemia: due to bone marrow crowding
Bone lesions: increased osteoclast activation (back/rib pain)

monoclonal Ig also crowds out production of normal polyclonal Ig causing infections

118
Q

multiple myeloma investigations

A

bloods:
-raised ESR, CRP, urea, Cr, Ca
- normal ALP (distinguish form bone malignancy where ALP high)

blood film:
- Rouleaux (rbc stacked like coins) formation

serum/urine electrophoresis:
- Bence Jones proteins

bone marrow aspirate:
- increased plasma cells > 10%

x-ray:
- to assess osteolytic lesions

119
Q

what is monoclonal gammopathy of unknown significance

A

pre-malignant condition with accumulation of some monoclonal plasma cells

absent CRAB features

120
Q

what is myelodysplasia

A

group of syndromes where the immature blood cells do not mature normally

121
Q

difference between primary and secondary myelodysplasia

A

primary: intrinsic bone marrow problem
secondary: previous chemotherapy/radiotherapy

122
Q

myelodysplasia signs and symptoms

A

may initially be asymptomatic
chronic pancytopaenia (anaemia, neutropenia, thrombocytopaenia)

123
Q

presentation of haemophilia

A

usually presents early in life or after surgery/trauma
deep bleeding and bruising (secondary haemostasis)
- haemarthrosis
- haematoma
- excessive bruising and haematuria
x-linked recessive (male)

124
Q

which clotting factor deficient in haemophilia A

A

factor 8

125
Q

which clotting factor deficient in haemophilia B

A

factor 9

126
Q

diagnosis of haemophilia

A

prolonged APTT (factor 8 + 9 in intrinsic pathway)
factor assay to confirm diagnosis

127
Q

which clotting factor does vWF stabilise

A

factor 8

128
Q

what is type 1 von willebrand syndrome

A

reduced levels of normal vWF
autosomal dominant

129
Q

what is type 2 von willebrand syndrome

A

adequate levels of abnormal vWF
autosomal dominant

130
Q

what is type 3 von willebrand syndrome

A

complete lack of vWF and reduced factor 8
autosomal recessive

131
Q

presentation von willebrand syndrome

A

superficial bleeding
bruising, epistaxis, menorrhagia
prolonged gum bleeding after dental procedures
prolonged bleeding from minor wounds
type 3 more severe and causes deep bleeding into joints and soft tissues

132
Q

diagnosis of von willebrand syndrome

A

depends on type

prolonged bleeding time
prolonged APTT and normal PT (factor 8 reduction)
reduced vWF (except in type 2)
normal platelets

133
Q

difference between acute overt DIC and chronic non-overt DIC

A

Acute overt:
emergency and life-threatening
significant platelet and clotting factor depletion > bleeding

Chronic non-overt:
slower rate, time for compensatory responses
hypercoagulable state but less bleeding

134
Q

what is haemochromatosis

A

autosomal recessive disorder of iron absorption and metabolism resulting in iron accumulation

135
Q

risk factors for haemochromatosis

A

genetics
male
> 40 yrs

136
Q

types of haemochromatosis and how they are caused

A

primary:
- hereditary (autosomal recessive)
- mutations on the HFE gene (hint: H(igh) Fe)
- most common form

secondary:
- caused by iron overload
- transfusion related

137
Q

clinical features of haemochromatosis

A

most asymptomatic
triad (fatigue, arthralgia, erectile dysfunction)
signs of secondary diabetes
tanning (bronze pigmentation)
liver - cirrhosis, hepatomegaly
cardiac failure
loss of libido due to hypogonadism

138
Q

Ix for haemochromatosis

A

kinda opposite to IDA results

high serum iron
serum transferrin saturation >45%
transferrin low
raised serum ferritin
low TIBC
genetic tests

139
Q

management of haemochromatosis

A

dietary change (low iron diet)
therapeutic phlebotomy: (1st line)
- regular venesection weekly
- target ferritin and transferrin levels
drug-induced iron chelation with deferoxamine (2nd line)

140
Q

complication of haemochromatosis

A

cirrhosis
diabetes mellitus
heart failure
hepatocellular carcinoma (may need USS)
hypogonadism

141
Q

what is Wilson’s disease

A

autosomal recessive disorder leading to excess copper deposition in tissues hence LOW COPPER IN SERUM

142
Q

presentation of Wilson’s disease

A

onset usually between 10-25 yrs old
combination of liver and neurological disease

liver - hepatitis, cirrhosis
neurological - basal ganglia degeneration, asterixis, chorea, dementia
Kayser-Fleischer rings

143
Q

classification of haemophilia based on severity

A

mild:
- haematomas following severe trauma
- factor 8/9 activity between 5-50%

moderate:
- haematomas following mild trauma
- factor 8/9 activity between 1-5%

severe:
- spontaneous haematomas
- factor 8/9 activity <1%

144
Q

management of haemophilia

A

substitution of clotting factors (factor concentrates)

desmopressin - may be given for mild haemophilia A. triggers release of vWF leading to increase of factor 8

antifibrinolytic therapy (aminocaproic acid or tranexamic acid)

145
Q

management of von willebrand disease

A

tranexamic acid for mild bleeding
desmopressin
factor 8 concentrate

146
Q

causes of hyposplenism

A

operative splenectomy
sickle cell anaemia
coeliac disease
CLL
IBD
bone marrow transplant
congenital asplenia

147
Q

indications for splenectomy

A

trauma
spontaneous rupture
hypersplenism
neoplasia

148
Q

most common organisms associated with severe infection in hyposplenism

A

NHS or No Happy Spleen

N.meningitidis
H.influenzae type b
S.pneumoniae (pneumococcus)

149
Q

presentation of hyposplenism

A

sepsis following splenectomy due to infection (pyrexia, hypotension, tachycardia)

150
Q

Ix for hyposplenism

A

blood film:
howell jolly bodies, target cells, siderocytes

imaging:
USS, CT, MRI

following splenectomy - thrombocytosis (may need prophylactic aspirin)

151
Q

Mx for hyposplenism

A

immunisations
prophylactic antibiotics (penicillin or amoxicillin) - recommended in patients at high risk of pneumococcal infections

152
Q

what are the myeloproliferative disorders

A

CML
polycythaemia vera
essential thrombocytosis
primary myelofibrosis

153
Q

what is essential thrombocytosis

A

aka primary thrombocythaemia

chronic myeloproliferative disorder associated with sustained dysregulated megakaryote proliferation in the bone marrow increasing the number of circulating platelets causing thrombocytosis

154
Q

risk factors for essential thrombocytosis

A

50-70 years
JAK2 mutation

155
Q

main conditions with massive splenomegaly

A

CML and myelofibrosis

156
Q

signs and symptoms of myelofibrosis

A

massive splenomegaly
pancytopenia
dry tap - failure of bone marrow aspirate
tear drop poikiloctes (dactrocytes) on blood film

157
Q

clinical features of myleproliferative disorders

A

erythromelalgia - burning pain and dusky congestion of extremities, pain worse with heat

splenomegaly

arterial and venous thrombosis

bleeding

158
Q

Ix for myeloproliferative disorders

A

FBC - platelet count > 450 x 10^9/L
JAK2 mutation testing

159
Q

Mx of myeloproliferative disorders

A

hydroxycarbamide - used to reduce platelet count
antiplatelet therapy - aspirin

160
Q

causes of pancytopenia

A

aplastic anaemia
myelofibrosis
multiple myeloma
methotrexate
leukaemia
chemotherapy
radiotherapy
b12 or folate deficiency
lymphoma

161
Q

what is polycythaemia

A

increased hb conc

162
Q

difference between relative and absolute (true) polycythaemia

A

relative: normal cell mass but low plasma volume caused by dehydration or stress

absolute: increased cell mass

163
Q

difference between polycythaemia vera (primary) and secondary polycythaemia

A

primary is EPO independent
secondary driven by excess EPO

164
Q

risk factor for polycythaemia vera

A

Budd-Chiari syndrome

165
Q

what can polycythaemia vera lead to

A

AML or myelofibrosis

166
Q

causes on increase in EPO

A

appropriate: chronic hypoxia
inappropriate: hepatocellular carcinoma, renal carcinoma, EPO abuse by athletes

167
Q

clinical features of polycythaemia

A

constitutional symptoms - weight loss, fatigue, sweating
hyperviscosity syndrome - mucosal bleeding + neurological symptoms + visual changes
facial redness
tenderness or painful burning and redness of fingers palm, heels or toes
pruritis - worse when skin touches water
splenomegaly

168
Q

Ix and results for polycythaemia

A

hb elevated
hct elevated
JAK2 gene mutation screen
elevated platelets
decrease EPO in polycythaemia vera
increased EPO in secondary polycythaemia (check for EPO secreting tumours)

169
Q

Mx for polycythaemia

A

phlebotomy (venesection)
antiplatelet prophylaxis (aspirin)
cytoreductive therapy
JAK2 inhibition (Ruxolitinib)

170
Q

common anticoagulants

A

oral:
vit K antagonist (warfarin)
DOACs (apixaban)

parenteral:
heparin

171
Q

how does warfarin work

A

vit K antagonist
vik K responsible for production of factors 2,7,9,10
has biggest effect on factor 7 hence prolonged PT

can be reversed by replacement of vit K

172
Q

warm autoimmune haemolytic anaemia is associated with which immunoglobulin

A

IgG

warm weather makes me Glad

173
Q

cold autoimmune haemolytic anaemia is associated with which immunoglobulin

A

IgM

cold weather makes me Mad

Phase 1c (13 decks)

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