Haematology Flashcards

(61 cards)

1
Q

How would you further investigate a microcytic anaemia?

A

haematinics (iron studies, B12 and folate)
coeliac screen - TTG antibodies

consider endoscopy and colonoscopy if old or RF for bowel CA

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2
Q

what results of blood tests would you expect a patient suffering from DIC to have?

A

low platelets
low fibrinogen
high PT and APTT
high D-dimer and fibrin degradation products

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3
Q

what are hereditary causes of haemolytic anaemia?

A

hereditary spherocytosis
hereditary elliptocytosis

G6PD deficiency, pyruvate kinase deficiency

sickle cell disease, thalassaemia

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4
Q

what are acquired causes of a haemolytic anaemia?

A

autoimmune (SLE)
some drugs
infection
MAHA

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5
Q

What would be the differential for a patient present with backache with hypercalcaemia, low PT and normal ALP ?

A

multiple myeloma

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6
Q

What features do patients with multiple myeloma have?

A

CRAB

Calcium is high
Renal impairment
Anaemia
Bone (pain/ache or fracture)

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7
Q

What might patients with polycythaemia present with?

A

headache
pruritis post hot bath
blurred vision
tinnitus
thombosis
grangrene
choreiform movements

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8
Q

what causes a low reticulocyte count?

A

parvovirus B19 infection (-> aplastic crisis)

aplastic crisis 2nd to sickle cell
blood transfusion

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9
Q

What is the most common cause of aplastic anaemia?

A

idiopathic acquired aplastic anaemia

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10
Q

What are some secondary causes of aplastic anaemia?

A

infection - parvovirus B19
cytotoxic drugs

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11
Q

What do Heinz bodies suggest?

A

oxidized Hb

GLUCOSE-6-PHOSPHATE DEHYDROGENASE

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12
Q

causes of macrocytosis

A

B12/folate deficiency
alcoholism
myelodysplastic disorders
hypothyroidism
liver disease

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13
Q

What does a positive Coombs test indicate?

A

confirms the presence of antibodies to RBC indicating a autoimmune haemolytic anaemia

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14
Q

What do Burr cells indicate?

A

uraemia

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15
Q

what do target cells indicate?

A

IDA

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16
Q

What do reed-sternberg cells indicate?

A

hodgkin’s lymphoma

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17
Q

What is the philadelphia chromosome associated with?

A

CML

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18
Q

What does a positive Hams test indicate?

A

paroxysmal nocturnal haemoglobinuria

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19
Q

what is paroxysmal nocturnal haemoglobinuria?

A

acquire RBC defect making them more susceptible to being destroyed by complement

PC: abdo pain, haemolytic anaemia, dark coloured urine at night or early morning

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20
Q

What are hyperviscosity symptoms?

A

blurry vision from retinal haemorrhages
headaches
bleeding
severe pruritus post bath

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21
Q

what is the tumour marker for a seminoma?

A

beta-HCG

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22
Q

What cancer is EBV a RF for?

A

lymphoma

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23
Q

what is haemophilia?

A

an inherited deficiency in clotting factors leading to a bleeding disorder

rarely an acquired form may occur whereby an individual makes autoantibodies to clotting factors

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24
Q

What are the main types of haemophilia?

A

haemophilia A - factor 8 deficiency
haemophilia B - factor 9 deficiency
haemophilia C - factor 11 deficiency

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25
what is the most common type of haemophilia?
haemophilia A
26
Epidemiology of acute lymphoblastic leukaemia?
most common malignancy in children peak incidence 2-5 years of age second peak of incidence in the elderly
27
what are the stages of CML?
1. relatively stable chronic phase (4-6 years) 2. accelerated phase (3-9 months) 3. acute leukaemia phase characterised by blast transformation
28
Describe the ann arbor staging system for lymphoma
``` I = single LN region II = 2+ LN regions on one side of diaphragm III = LN regions on both sides of diaphragm IV = extranodal involvement ``` ``` A = no B symptoms B = B symptoms S = spleen involvement ```
29
Give features of Hodgkin's lymphoma
15% of all lymphomas Reed-sternberg cells are diagnostic EBV infection is a risk factor bimodal incidence - 20-30s and \>50s M\>F
30
What are some presenting features of Hodgkin's lymphoma?
enlarged mass in neck/axilla/groin which may spontaneously enlarge or reduce in size mass painful post alcohol pruritis, cough, SOB B symptoms: fever, night sweats, weight loss
31
what are the B symptoms?
fever, night sweats, weight loss
32
is extra nodal involvement more common in Hodgkin's or non-Hodgkin's?
non hodgkin's
33
RF for Non-hodgkin's lymphoma
radiotherapy, immunosuppresion, chemotherapy, HIV, HBV, HCV, oncogenic viruses (EBV)
34
Presentation of non-Hodgkin's lymphoma
painless enlarged mass in neck/axilla/groin systemic symptoms (FLAWS) extra-nodal - skin rash, headache, sore throat, abdo pain, testicular swelling BM failure -\> anaemia, infections, bleeding hypercalcaemia
35
what is multiple myeloma?
haematological malignancy characterised by proliferation of plasma cells causing increased monoclonal immunoglobulin production (usually IgG or IgA)
36
what monoclonal immunoglobulin is most commonly implicated in multiple myeloma?
IgG accounts for 2/3 of cases
37
What would the blood results and blood film show for a patient with multiple myeloma?
low Hb high creatinine high calcium high ALP rouleux formation on blood film
38
what might you find in urine analysis of a multiple myeloma patient?
serum paraprotein BENCE JONES PROTEIN
39
what is myelofibrosis?
obliteration of the bone marrow with fibrosis due to increased fibroblast activity
40
what conditions are associated with myelofibrosis?
30% of myelofibrosis patients have a Hx of polycythaemia rubra vera or essential thrombocytopenia
41
What are the symptoms and signs of myelofibrosis?
weight loss, fever, loss of appetite, night sweats, pruritis splenomegaly, hepatomegaly, symptoms of BM failure
42
what are the symptoms of polycythaemia?
headache, blurred vision, tinnitus SOB pruritis after a hot bath burning pain in fingers and toes night sweats thrombotic events
43
what are the signs of polycythaemia?
splenomegaly plethoric complexion [red and ruddy] scratch marks from ithcing HTN
44
what is the inheritance pattern of thalassaemia?
autosomal recessive
45
what does the impaired Hb synthesis in thalassaemia patients lead to?
1. impaired erythropoiesis 2. haemolysis 3. anaemia 4. extramedullary haematopoiesis
46
how does alpha thalassaemia 3 gene deletion present? what signs on blood film are there?
severe anaemia, splenomegaly, jaundice and abnormal RBC indices very early life HEINZ BODIES
47
how does beta thalassaemia major present?
3-6 months of age when foetal haemoglobin becomes adult Hb failure to thrive, recurrent infections, anaemia
48
what are the signs of major beta thalassaemia?
pallor, malaise, SOB, mild jaundice, frontal bossing, thalassaemia facies, hepato-splenomegaly
49
what is the pentad of thrombotic thrombocytopenic purpura?
MAHA thrombocytopenia renal involvement (impairment) neurological abnormalities fever note majority of patients with TTP don't have the pentad
50
what are the causes of TTP?
idiopathic (40%) autoimmune e.g. SLE cancer pregnancy or post-partum (10-25%) malignancy drug related (quinine) bloody diarrhoea prodrome type (progression of HUS from E.coli infection)
51
what are the symptoms and signs of TTP?
epistaxis, bruising, petechiae, haematuria, menorrhagia NEURO: confusion, headache, vision changes, aphasia, fits fever, pallor, myalgia, chest/abdo pain purpura, jaundice (2nd to haemolysis), severe HTN, splenomegaly
52
what are the functions of vWF?
1. binds to exposed subendothelium 2. aids platelet activation and binding to one another 3. stabilises factor VIII preventing it's degradation
53
what are the type of von willebrands disease
type 1 = most common, autosomal dominant, deficiency in vWF type 2 = 2nd most common, abnormal vWF, autosomal dominant type 3 = undetectable vWF levels, autosomal recessive
54
what clotting factor abnormalities do vWF disease patients have ?
low VIII vWF abnormality/deficiency prolonged clotting time (high APTT)
55
What malignancy are auer rods associated with?
acute myeloid leukaemia
56
what is aplastic anaemia?
it is a global pancytopenia where cells are normal in morphology but deficient. BM biopsy shows hypocellular marrow at least 2 peripheral deficiencies must be detected of either: Hb \< 100, platelets \< 50 or neutrophil \< 1.5 x 10^9
57
what are some acquired causes of aplastic anaemia?
parvovirus B19 medications such as methotrexate chemicals such as benzene or DDT paroxysmal nocturnal haemoglobinuria
58
what are inherited causes of aplastic anaemia?
fanconi's anaemia
59
what is the diagnosis of DIC based on?
presence of _\>_ 1 known underlying condition causing DIC + abnormal global coagulation tests * reduced paltelet count * increased PT * increased fibrin-related marker (d-dimer, fibrin degradation production) * decreased fibrinogen levels
60
what drugs trigger G6PD deficiency?
sulpha drugs * sulphonamides - trimethoprim, sulfasalazine * sulphasalazine * sulphonylureas - glipizide, gliclazide, glimepiride
61
who gets irradiated transfusion and who gets CMV -ve