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Sophia's Path > Haem > Flashcards

Haem Flashcards

1
Q

Anaemia Definition (Hb)

A

Men <135g/L

Women <115g/L

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2
Q

Causes of microcytic anaemia

A 
FAST
Fe-deficiency
Anaemia of chronic disease
Sideroblastic anaemia
Thalassaemia
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3
Q

Causes of macrocytic anaemia

A 
FATRBC(+M)
Fetus (pregnancy)
Antifolates (phenytoin)
Thyroid (Hypothyroidism)
B12 or folate deficiency
Cirrhosis (++alcohol)
Myelodysplastic syndromes
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4
Q

Causes of normocytic anaemia

A 
Acute blood loss
Anaemia of chronic disease
BM failure
Renal failure
Hypothyroidism
Haemolysis
Pregnancy
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5
Q

Fe deficiency anaemia - signs

A

koilonychia, atrophic glossitis, angular cheilosis

post cricoid webs - Plummer-Vinson Syndrome

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6
Q

Fe deficiency anaemia -blood film

A

microcytosis, hypochromic
anisytosis/ poikilocytosis
pencil cells

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7
Q

Fe deficiency anaemia - lab results

A

low Fe
low ferritin
high TIBC

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8
Q

Anaemia of chronic disease - pathophysiology

A

cytokine driven inhibition of RBC production

IFNs, TNF, Il1 = reduced EPO re3ceptor production (reduced EPO synthesis)

IL6 and LPS = stimulate liver to make hepcidin = decreases iron absorption (inhibits transferrin)

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9
Q

Anaemia of chronic disease - in renal failure

A

EPO deficiency

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10
Q

Sideroblastic anaemia

A

in BM - ineffective erythropoiesis - iron loading

causes endocrine/liver/ cardiac damage

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11
Q

Sideroblastic anaemia blood film

A

Ring sideroblasts

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12
Q

Sideroblastic anaemia - lab results

A

high Fe
high ferritin
N TIBC

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13
Q

Sideroblastic anaemia - causes

A 
ALCOHOL excess
myelodysplastic/ myeloproliferative disorders
post-chemo
irradiation
lead
anti-TB drugs
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14
Q

Sideroblastic anaemia - treatment

A

Pyridoxine - Vit B6 promotes RBC production

Remove cause

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15
Q

Vit B12 deficiency causes

A

Stomach - Pernicious anaemia (post-gastrectomy)

Terminal ileum - ileal resection (Crohn’s disease, bacteria overgrowth, tropical sprue, tape worms)

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16
Q

Vit B12 deficiency clinical features

A

glossitis, angular cheilosis

irritabiliy/ depression/ psychosis/ dementia

paraesthesiae, peripheral neuropathy (loss vibration/ proprioception 1st - absent ankle reflex, spastic paraperesis)

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17
Q

Pernicious Anaemia

A

anti-IF (50%)
anti-parietal cell (90%)
Schilling test

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18
Q

Vit B12 deficiency treatment

A

IM hydroxocobalamin (B12)

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19
Q

Folate deficiency causes

A

Diet
Pregnancy
Drugs - alcohol, anti-epileptics (phenytoin), methotrexate, trimethoprim

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20
Q

Hereditary spherocytosis genetics

A

autosomal dominant

SPECTRIN (or ankyrin) deficiency

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21
Q

Hereditary spherocytosis diagnosis

A

spherocytes
increase osmotic fragility
DAT +ve

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22
Q

Hereditary spherocytosis features

A

severe neonatal jaundice

splenomegaly - extravascular haemolysis

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23
Q

haemolytic anaemia complications

A

Parvovirus B19 susceptibility
- virus infects developing RBC and arrests maturation
if reduced RBC/RBC lifespan - may lead to severe anaemia
self limiting infection - may require transfusion

propensity to gall stones (pigmented)

increased risk of iron overload (increased intestinal absorption)
increased risk osteoporosis (unknown)

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24
Q

Gilbert Syndrome gene

A

genetic polymorphism

Mutation in UGT 1A1 (extradinucleotide on each allele = TA7/TA7)

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25
Q

Hereditary ellipotcytosis genetics

A

autosomal dominant - spectrin mutations

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26
Q

Hereditary pyropoikilocytosis (ellipotcytosis) genetics

A

abnormally snesitive to heat

autosomal recessive

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27
Q

South East Asian Ovalocytosis

A

recessive - heterozygous +/- malaria protection

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28
Q

G6PD Deifciency genetics

A

X linked (affect hereo males and homo females)

G6PD = enzyme catalyses first step in pentose phosphate pathway - maintains intracellular gluthianone (GSH)
GSH - protect RBCs against oxidative stress

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29
Q

G6PD Deifciency blood film

A

Heinz bodies - peripheral inclusions (methylviolet stain)
bite cells
nucleated RBCs, contracted cells

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30
Q

G6PD Deifciency presentation

A

rapid anaemia/ jaudice precipated by oxidants

drugs (primaquinine, sulfonamides, aspirin), broad/fava beans, acute stressors/infection, moth balls (naptholene0

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31
Q

Pyruvate kinase defiency

A

autosomal recessive

severe neonatal jaundice, splenomegaly, haemolytic anaemia

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32
Q

Sickle cell mutation

A

GAG -> GTG (Glu-> Val)
codon 6 on beta chain
HbA-> HbS

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33
Q

Sickle-haemaglobin C disease

A

HbSC
HbS from one parent
HbC from other parent (defective b chain)

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34
Q

Sickle beta thalassaemia

A

HbS/Beta
HbS from one parent
beta thalassaemia trait from other parent

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35
Q

SCD vaso-oclusion/ infarction SICKLED

A 
Stroke
Infection
Crises (splenic, sequestration, chest pain)
Kidney
Liver - gallstones
Eyes - retinopaty
Dactilitis

Mesenteric ischaemia
Priapism

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36
Q

SCD Haemolyis features

A 
anaemia 60-80
splenomegaly
folate deficiency
gallstones
aplastic crisis
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37
Q

SCD presentation

A

usually at 3-6 months (decrease HbF)
reduced O2 = HbS polymerisation = sickling

child - strokes, splenomegaly, splenic crisis, dactylitis
teens - impaired growth, gallstones, psych, priapism
adult - hyposplenism, CKD, retinopathy, pumonary HT

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38
Q

Beta thalassaemia

A

reduced beat chain synthesis

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39
Q

thalassaemia scull x ray

A

skull bossing
maxillary hypertrophy
hair-on-end

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40
Q

Beta thalassaemia treatment

A

blood transfusions + desferrioxamine (prevents iron overload)

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41
Q

Warm Autoimmune Haemolyitc Anaemia

A 
37C
IgG
DAT +ve
Spherocytes
Idiopathic, lymphoma, CLL, SLE, methyldopa
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42
Q

Cold Agglutinin Disease

A 
<37C
IgM
DAT +ve
Raynauds
Idiopathic, lymphoma, infections: EBV, mycoplasma
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43
Q

Warm Autoimmune Haemolyitc Anaemia Tx

A

steroids
splenectomy
immunosuppression

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44
Q

Cold Agglutinin Disease Tx

A

treat underlying condition
avoid cold
chlorambucil (chemo)

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45
Q

Paroxysmal Cold Haemoglobinuria

A

Hb in urine
Viral infection - measles, syphillis, VZV
Donath-Landsteiner Ab - stick to RBCs ub cold - complement mediated haemolysis in rewarming
(self limiting - IgG dissociate at higher temp than IgM)

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46
Q

Paroxysmal nocturnal haemoglobniuria

A

acquired loss of GPI surface markers (protective)
Complement mediated lysis
Chronic intravascular haemolysis (NIGHT)
morning haemoglobinuria, thrombosis(+ Budd Chiari Syndrome)
Immunophenotyping or Ham’s Test

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47
Q

Paroxysmal nocturnal haemoglobinuria treatment

A

iorn/folate supplement
Prophylactic vaccine, Ab
Eculizimab (expensive) - prevents complement binding

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48
Q

TTP Ab

A

anti-ADMTS13

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49
Q

TTP

A
  1. MAHA
  2. fever
  3. renal impairment
  4. focal neurology
  5. thombocytopenia
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50
Q

TTP pentad

A
  1. MAHA
  2. fever
  3. renal impairment
  4. focal neurology
  5. thombocytopenia
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51
Q

HUS pathophysiology

A

E.coli toxin damages endothelial cells = fibrin mesh and RBC destruction

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52
Q

HUS features

A

MAHA, diarrhoea, renal failure

Children/ elderly

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53
Q

Acute Leukaemia feautres

A

rapid onset
death in w-m if untreated
immature cells (blasts)
BM failure - anaemia, neutropenia, thrombocytopenia

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54
Q

Acute Pro Myelocytic Leukaemia mutation

A

t(15;17) translocation = PML-RARA fusion gene

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55
Q

AML Chromosomal translocations

A

t(15;17) = promyelocytic leukaemia (M3)
t(5;8)
inv(16)/ t(16;16) = good prognosis

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56
Q

AML Chromosomal duplications

A

+ chr 8

+ chr 21 (i.e. Trisomy 21) - increased risk AML and ALL

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57
Q

Chromosomal Loss/ deletion

A

5/5q - parital loss of chr 5

7/7q - total loss of chr 7

58
Q

AML molecular abnormalities

A

point mutations
NPM1 = good prognosis
CEBPA = good prognosis (both alleles)

partial duplication FLT3 = bad prognosis event

59
Q

Multiple hit hypothesis

A

AML requires at least 2 interacting molecular defects - synergise to give leukaemic phenotype

Type 1 abnormality - promote proliferation and survival
Type 2 abnormalities - block differentiation

(CML - single mutation may be sufficient to cause disease)

60
Q

Acute Pro Myelocytic Leukaemia clinical features

A

excess abnormal promyelocytes a/w DIC (due to hyperactive fibrinolysis)
must be diagnosed early to prevent death from haemorrhage

61
Q

Acute Pro Myelocytic Leukaemia blood film

A

blasts (no present in aleukaemic leukaemia)
multiple auer rods
hypergranular

62
Q

Multiple hit hypothesis - in acute pro myelocytic leukaemia

A

Type 1 abnormality - FLT3-ITD

Type 2 abnormalities - t(15;17) PML-RARA

63
Q

Multiple hit hypothesis - Core Binding factor (CBF) Leukaemias

A

Type 1 abnormality - KIT mutation

Type 2 abnormalities - CBF function mutation

64
Q

inv(16)/ t(16;16)

A

good prognosis

some maturation to abnormal eosinophil precursor - giant purple granules

65
Q

AML bloodfilm

A 
Auer rods (fusion of primary granules in crystal)
Granular cells
66
Q

AML cytochemistry (less used nowadays)

A 
Positive staining for
myeloperoxidase
non-specific esterase
sudan black B
- these stains are negative in ALL
67
Q

Immunopheonotyping ALL

A

Pre-B cell: CD19, Cd20, CD10, TdT
B cell: CD19, CD20, surface Ig
T cell: CD2, CD3, CD4, CD8, TdT

68
Q

Immunopheonotyping AML

A

MPO
CD13, CD33, CD14, CD15
Glycophorin E
Platelet Antigens

69
Q

Immunopheonotyping ALL and AML

A

CD34
CD45
HLA-DR

70
Q

DIC in leukaemia

A

DIC presents as vascular obstuction (even gangrene) and due to sepsis (BM failure)

In pro-myelocytic - due to hyperactive fibrinolysis (substances secreted from leukaemic cells)

71
Q

Clinical features of AML - eyes

A

retinal haemorrhages/ retinal exudates

due to hyperviscosity if WCC is +++ also due to thrombocytopenia

72
Q

Clinical features of ALL

A 
BM failure (anaemia, thrombocytopenia, neutropenia)
Lymphadenopathy ++
Thymic enlargment - T cell 
HSM
Testes, CNS, other site
Painful bones in children
73
Q

ALL mutations good prognosis

A

Hyperdiploidy = t(12;21), t(1;19)

t(9;22) - Ph+ Chr - prev poor prognosis - now GOOD with TK inhibitors - IMATINIB

74
Q

ALL mutations poor prognosis

A

Hypodiploidy = t(4;11)

75
Q

ALL B and T cell percentages

A 
T-lineage = 15%
B-lineage = 85%
76
Q

ALL Treatment - specific therapy

A

CNS directed therapy - propensity to cross BBB and migrate into CSF - drugs directly injected or high dose to cross BBB
(+ systemic chemo)

77
Q

PV gene mutation

A

JAK2 V617F mutation (100%)

78
Q

ET gene mutaiton

A

JAK2 - 60%
Calreticulin - 30%
MPL - 5%

79
Q

PMF gene mutaiton

A

JAK2 - 60%

Calreticulin - 30%

80
Q

PV Treatment

A

Venesection
Hydroxycarbamide
target HCT <45%

81
Q

ET Treatment

A

Aspirin - prevent thrombosis
Hydroxycarbamide - anti metabolite
Anagrelide - specifically inhibits platelet formation (SE - palpitations, flushing)

82
Q

MF investigations

A

Tear drop poikilocytes (dacrocyte)
Leukoerythroblasts (primitive cells)
BM dry tap

83
Q

Poor prognosis in MF

A

Severe anaemia <100g/L
Thrombocytopenia <100x10^19
Massive splenomegaly

Prognostic scoring system DIPPS
Score 0 - median survival 15y
Score 4-6 - median survival 1.3y

84
Q

MF treatment

A

Hydroxycarbamide, thalidomide
Supportive
Allogenic SCT
Ruxolotinib - high prognostic score cases

85
Q

MF cell signal inhibitor

A

Ruxolitinib (JAK2 inhibitor)

86
Q

ALL TK inhibitor

A

IMATINIB (PH+ ALL)

87
Q

Hodgkin Lymphoma staining

A

CD15

CD30

88
Q

Hodgkin Lymphoma tissue diagnosis - characteristic cell

A

Reed-Sternberg Cells (owl eye)

Bi/multi-nucleate

89
Q

Hodgkin Lymphoma Staging

A

Ann-Arbor
S1 One LN region
S2 Two or more LN regions on same side of diaphragm
S3 Two or more LN regions on opposite side of diaphragm
S4 extranodal sites (liver, BM)

A - no constitutional symptoms
B - constitutional symptoms (‘B’ symptoms)

90
Q

Hodgkin Lymphoma Treatment

A
  1. Combination chemo (ABVD)
    adriamycin, bleomycin, vinblastine, decarbgazine
  2. Radiotherapy (high risk breast cancer)
  3. Intensive Chemo and Autologous SCT
91
Q

NHL - H. Pylori

A

Gastric MALT (mucosa associated lymphoid tissue) Marginal Zone NHL of stomach

92
Q

NHL - Sjogrens

A

Marginal Zone NHL of Parotid lymphoma

93
Q

NHL - Coeliac disease

A

Small bowel T cell lymphoma

EATL (enteropathy associated T-Cell Non Hodgkin lymphoma)

94
Q

NHL - HTLV

A

HTLV1 infects T cells by vertical transmission
Carribean and Japan carriers
May develop Adult T cell leukaemia lymphoma (2.5% at 70 years) – very aggressive

95
Q

NHL - EBV

A

EBV infects B lymphocytes
Healthy Carrier state maintained by cytotoxic T cells - kill EBV antigen expressing B cells
Loss of T cells function give risk of EBV driven lymphomas
HIV
60 fold increase in lymphoma in HIV (high grade B-NHL)
Iatrogenic (renal heart pancreas transplant imunosuppression)
PTLD (post transplant lymphoproliferative disorder) – prednisolone or cyclosporin

96
Q

NHL Prognosis

A

LDH, B2 microglobulin – raised in rapidly dividing tumours
Albumen – low in sick patients
Kidney/BM function – is it obstructing?
Wider treatment options in younger patients

97
Q

Classical Hodgkin Lymphomas

A

Nodular sclerosing 80% Good prognosis (causes the peak incidence in young women)
Mixed cellularity 17% Good prognosis
Lymphocyte rich (rare) Good prognosis
Lymphocyte depleted (rare) Poor Prognosis

98
Q

Nodular Lymphocyte predominant HL

A

5% - disorder of the elderly multiple recurrences

99
Q

Hodgkin Lymphomas Treatment

A 
ABVD
Adriamycin			
Bleomycin			
Vinblastine 	
DTIC

ABVD, is given at 4-weekly intervals.

Effective treatment
Preserves fertility (unlike MOPP the original chemo)
Can cause (long term)
Pulmonary fibrosis
cardiomyopathy
100
Q

LN B Cell area

A

Lymphoid follicle
Mantle zone - naïve unstimulated B cells
Germinal center - B cells, Antigen presenting cells

This is where B cells which bind antigen epitopes are selected and activated

101
Q

LN T Cell area

A

T cells
Antigen presenting cells
High endothelial vessels (T cells interact with APCs)

This is where T cells which bind antigen epitopes are selected and activated

102
Q

Immunophenotyping - T and B cell markers

A

CD 20 = B cell marker

CD 3, CD 5 = T cell marker

103
Q

Protein expressed in B cells of Mantel Cell Lymphoma

A

Cyclin D1

104
Q

B cell clonality and malignancy

A

Light chain expression
Malignant B cells = monoclonal (kappa or lamda)
Normal B cells = poly clonal kappa and lamba light chain expression

105
Q

FISH: Mantle cell lymphoma

A

t (11;14)

106
Q

FISH: Follicular lymphoma

A

t (14;18)

107
Q

FISH: anaplastic large cell lymphoma

A

t (2;5) a/w better prognosis in anaplastic large cell lymphoma

108
Q

Immunophenotyping in CLL (poor prognosis)

A

CD38 staining

109
Q

Follicular Lymphoma

A

Lymphadenopathy middle aged/elderly
Follicular pattern - whole node is replaced with neoplastic follicles
Germinal centre cell origin = CD10, bcl-6+ positive staining
May transform to high grade

110
Q

Small lymphocytic lymphoma/CLL

A

Middle Aged/elderly; lymphadenopathy or high blood code

CD5, CD23 +

Multiple genetic abnormalities

Indolent, but can transform to high grade lymphoma (Richter transformation)

111
Q

Mantle cell lymphoma

A 
MA male predominence
Lymph nodes, GI tract
Disseminated disease at presentation
Aberrant CD5, cyclin D1 expression
11;14 translocation
Cyclin D1 over expression
Median SR 3-5 yrs
112
Q

Burkitt’s lymphoma

A

Jaw or abdominal mass children/young adults
Endemic, Sporadic, Immunodeficiency
EBV associated
Germinal center cell origin
“starry-sky” appearance
C-myc translocation (8:14, 2:8, 8;22) – removes break on cell cycle
Aggressive disease

113
Q

Diffuse large B cell lymphoma

A

MA/elderly
Germinal center or post-germinal center B cell
Germinal centre phenotype - CD10+ = better prognosis
Sheets of large lymphoid cells
Germinal center phenotype = good prognosis
p53 positive, high proliferation fraction = poor prognosis

114
Q

Diffuse large B cell lymphoma prognosis

A

CD10+ = better prognosis

p53 positive, high proliferation fraction = poor prognosis

115
Q

Hodgkin vs Non Hodgkin

A

Hodgkin
More often localised to a single nodal site
Spreads contiguously

Non-Hodgkin
More often involves multiple lymph node sites
Spreads discontinuously

116
Q

Classical Hodgkin Lymphoma

A

Young and MA (double peak)
Often involves just single lymph node group
Though to be germinal center/post germinal center B cell origin
EBV associated
Sclerosis, mixed cell population in which scattered Reed-Sternberg and Hodgkin cells with eosinophils
Moderately aggressive
CD 30 and CD 15 = diagnostic markers for HL

117
Q

Nodular LP (Non Classical) Hodgkin Lymphoma

A

Isolated lymphadenopathy
Germinal centre B cell (positive for some germinal centre B cell markers)
No association with EBV
B cell rich nodules with scattered L&H cells
Indolent
Can transform to high grade B cell lymphoma (i.e. transforms to Non-Hodgkin Lymphoma)
Reactive population = lymphocytes (eosinophils and macrophages not seen – unlike classical HL)
Express CD 20 strongly and diffusely (Negative for CD 30 and 15)

118
Q

CLL vs Small lymphocytic lymphoma (SLL)

A

CLL - primarily seen in BM

SLL - primarily seen in LN

119
Q

Evan’s syndrome

A

CLL a/w autoimmunity - AIHA, ITP

120
Q

CLL diagnosis

A

Lymphocytosis >5 (high % of WCC = small, mature lymphocytes)
Low serum Ig
SMEAR CELLS

121
Q

CLL prognostic factors - poor

A

Raised LDH
CD38+
11q23 deletion

122
Q

CLL prognostic factors - good

A

Hypermutated Ig Gene
Low ZAP-70 expression
13q14 deletion

123
Q

CLL Staging

A

Binet Staging
A = high WBC, <3 enlarged LN (no treatment)
B = >3 enlarged LN
C = anaemia or thrombocytopenia

124
Q

CLL treatment

A

watchful wairting if asymptomatic
Supportive
p53 +ve - first line = ALEMTUZUMAB

125
Q

Multiple Myeloma Features

A

CRAB
Calcium = high
Renal failure (+amyloidosis, nephrotic syndrome)
Anaemia (+pancytopenia)
Bones (pain, osteoporosis, osteolytic lesions, fractures)
(+hyperviscosity)

MONOCLONAL Ig (paraprotein) - Bence Jones protein in urine

126
Q

MM staging

A

Durie-Salmon Staging

127
Q

MM Investigations

A 
Narrow band on electrophoresis
Rouleaux on blood film
Bence-Jones protien in urine
ESR +++
>10% plasma cells in BM
128
Q

MM vs MGUS vs smouldering MM

A

MGUS
Monoclonal serum protein <30g/L
<10% clonal plasma cells
No CRAB

Smouldering MM
Monoclonal serum protein >30g/L
<10% clonal plasma cells
No CRAB

MM
Monoclonal serum protein >30g/L
Any population of clonal plasma cells
CRAB (>1)

129
Q

MM Bone Aspirate Morphology

A

Mature plasmacytic myeloma cells - clumped chromatin, low nuclear-cytoplasmic ratio, abundant cytoplasm, rare nucleoli

Immature plasmacytic myeloma cells - reticular chromatin, less abundant cytoplasm, prominent nucleoli
= WORSE PROGNOSIS

130
Q

MM Immunophenotyping

A

Stain positive for CD138
Stain negative for CD20 (unlike other B cell lymphomas)
Cytoplasmic Ig stain (other B cells stain for surface Ig)
Kappa/lamda = monoclona

131
Q

Myeloma nephropathy

A

Paraprotein light chain fragments crystalise in kidney - blockage
Proximal tubule necrosis
Fanconi syndrome (renal tubule acidosis)
Cast nephropathy

132
Q

MM treatment options

A 
Steroids
Chemotherapy (e.g. mephalan - high dose tx before SCT)
IMIDS (e.g. thalidomide)
Proteosome inhibtors (e.g. bortezomib)
133
Q

MM treatment (path guide)

A

Supportive + bisphosphonates
First line - Bortezomib (proteosome inhibitor)
Auto CT curative in young pt
Not suitable SCT - daratumumab

134
Q

MM treatment proteosome inhibitors

A

Build up of misfolded proteins
Fatal ER stress
E.g. bortezomib, carfilzomib

135
Q

MM treatment IMIDS

A

IMIDS (e.g. thalidomide)

TF inihibtors

136
Q

Myelodysplastic syndrome morphological feautres

A 
WBC
- Pelger-Huet anomaly (bilobed neutrophils)
- Hypogranulation
- Micro megakaryocytes
RBC - ring sideroblasts

> 5% blast cells in BM

137
Q

Myelodysplastic syndrome prognosis

A

1/3 die from infection
1/3 die from bleeding
1/3 die from acute leukaemia
(IPSS-R = prognosis)

138
Q

Fanconi Anaemia

A

Inherited AA - opanytopenia
Autosomal recessive
Skeletal abnormalities, renal malformations, microopthalmia, short stature, skin pigmentation

139
Q

Dyskeratosis Congenital

A

X linked most commonly
DKC1 gene - defective telomerase function

Autosomal dominant - mutated TERC gene
encodes telomerase RNA

140
Q

Dyskeratosis Congenital

A
  1. Skin pigmentaiton
  2. Nail dystrophy
  3. Oral leukoplaskia
    (+ BM failure)
141
Q

Blood transfusions acute adverse reactions <24h

A 
Acute haemolytic (ABO incompatibility)
Anaphylaxis/ allergy
Infection (bacterial)
Febrile non-haemolytic
TACO
TRALI
142
Q

Blood transfusions delayed adverse reactions >24h

A 
Delayed haemolytic (ABO incompatibility)
Infection (viral, malaria, VCJD)
TA-GVHD
Post transfusion purpura
Iron overload

Sophia's Path (8 decks)

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