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Joshua's Path > Haematology > Flashcards

Haematology Flashcards

1
Q

What is the most common form of haemoglobin?

A

Hb A - made up of 2alpha chains and 2 beta chains. forms 90% of the haemaglobin

Hb A2 is made up of 2 alpha chains and 2 delta chains and makes up 1-3% of the haemoglobin. A raised level may indicate thallassaemia

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2
Q

what additional test should be ordered with iron studies?

A

CRP

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3
Q

What should you think of when patients have pre existing conditions in questions?

A

IS IT AUTOIMMUNE!!!

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4
Q

Sever complication of pernicious anaemia

A

subacute combined degeneration of the spinal cord

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5
Q

Name 4 inherited aplastic anaemias? what proportion of AA’s do they make up?

A

10% the other 70% are idiopathic and should be treated with immunosuppression a nd supportive care - transfusion abx and iron chelation if persitant. marrow stimulation and recovery may be used also e.g g-csf.

(D)DFS - to buy them a sofa
Fanconi's anaemia
dyskeratosis congenita
Schwachman diamond syndrome
Diamond Blackfan syndrome
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6
Q

Triad of dyskeratosis congenita

A

SON
skin pigmentation
oral leukoplakia
nail dystrophy

chromasome instability and telomere shortening - BM failure

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7
Q

Symptoms of schwachman diamond syndrome?

A

primarily neutrophilia - endocrine and pancreatic dysfunction

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8
Q

What blood results do you see in DIC? causes?

A

low platelets and fibrinogen, high fibrinogen degredation products, high d dimer, prolonged prothrombin time

Causes = malignancy, trauma, burns, toxins, obstetric complications.

Treatment = FFP, platelets, cryoprecipitate

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9
Q

What joint is most commonly affected by pseudogout?

A

Knee

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10
Q

what is another name for pseudogout

A

chondrocalcinosis

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11
Q

what type of bifringence do crystals show?

A

rhomboid shape with positive bifringence? BLUE WITH the axis.
pyrophosphate crystals

true gout is YELLOW WITH the axis

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12
Q

What counts as MASSIVE blood loss?

A

entire blood volume in 24 hours, >50% in 3 hours

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13
Q

What is the normal cutoff for platelet transfusion?

A

most people say <50 however some say 75 (x10^9)

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14
Q

What is the difference between red cell concentration and red cell mass

A

red cell concentration varies with hydration status and plasma volume. q

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15
Q

what drives anaemia of chronic disease

A

cytokines such as IFN, IL1 and TNF decrease EPO recepter expression

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16
Q

What are downs syndrome children at risk of (haem)

A

AML in first 3 years of life and then ALL

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17
Q

What is an acute leukaemia

A

Acute Neoplastic process of bone marrow and blood resulting in a rapidly progressing and fatal disease which has >20% blast cells on BM biopsy.

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18
Q

What is the best way to differentiate acute and chronic leukaemias clinically?

A

Chronic types are usually diagnosed incidentally, wheras acute have more severe presentations with BM failure and cytopenias - anaemia, petechiae, infections (neutropenia), and hepatosplenomegaly, cranial nerve palsies due to CNS involvement, gum hypertrophy (AMMoL often with hypoK) lymphadenopathy and bone bain.

However CML in blast phase can resemble an acute leukaemia.

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19
Q

TDT +ve

A

ALL - displayed on pre b

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20
Q

Philadelphia chromasome positive

A

usually CML but can be ALL in adults. (30% positive)

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21
Q

Name 2 subtypes of acute myeloid leukaemia

A

Acute promyleocytic leukaemia characterised by a t(15,17) with hypergranular promyelocytes with auer rods. it causes early DIC and haemorrhage. (DIC may occur with infection as a result of any leukaemia)
requires ATRA

Acute myelomonocytic leukaemia characterised by gum hypertrophy and

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22
Q

Are protein C and protein S, procoagulant or anticoagulant?

A

anticoagulant. along with TFPI, thrombomodulin, antithrombin (these are often expressed on the vessel wall)

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23
Q

how long does a flight need to be before it confers an increase thrombosis risk

A

9 hours

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24
Q

Which has the largest thrombosis risk:

Factor 5 leiden
Antithrombin deficiency
family history of thrombosis
Protein C/S deficnency

A

C/s and antithrombin deficiency are wrst followed by 5 leiden.

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25
What cell lines should be seen on a CML Bone marrow
Hypercellular marrow with spectrum of immature myelocytes and mature granulocytic cells in the blood. neutrophils in blood are 50-500
26
what are ethe three phases of CML
Chronic <5% blasts Accellerated >10% blasts - increasing splenomegaly Blast >20% blasts resembling acute leukaemia (ALL transformation can occur)
27
whats the difference between essential thrombocythemia and thrombocytopenic purpura
high and low
28
How do you differentiate hodgkins and non hodgkins lymphomas
hodgkins - pain on drinking alcohol in affected nodes, reed sternberg
29
what is nodular lymphocytic (predominant) pertain to?
non classical type of hodgkins lymphoma. accounts for small number of cases and is indolent. not EBV associated. rarely has B symptoms. mainly lymphadenotpathy. it has atypical reed sternberg cells.
30
Describe the staging in hodgkins lymphoma
ANN ARBOR stage 1: 1 lymph node region stage 2 2 or more lymph node regions on same side of D stage 3: 2 or more lymph nodes on opposite sides of D Stage 4: extranodal sites e.g liver/BM A- no constitutional sx B - fever/night sweat/weight loss
31
How can you treat von willebrand disease
Desmopressin can be used to increase the concentration of VwF and factor 8. this is only in type 1 and 2 disease. major disease - type 3 it would not be effective
32
clinical features of myelodysplastic syndromes
Fatigue, infection, bleeding. hypercellular marrow(as in CML) qualitative cytopenias risk of AML transformation.
33
What is a ringed sideroblast? what myelodysplasias does it feature in?
abnormally nucleated blast cell with iron granule ring occur in refractory anaemia with ringed sideroblasts. must have greater than 15% ringed sideroblasts. and erythroid dysplasia. may also be part of refractory cytopenia with ringed sideroblasts. must have>15% ringed sideroblasts along with: Cytopenia in 2 cell lines along with >10% dysplastic cells in 2 or more cell lines.
34
Define Refractory anaemia
anaemia, no blasts in blood. erythroid dysplasia with <5% blasts in BM
35
Define refractory anaemia with excess blasts 1
Blood: cytopenias <5% blasts no auer rods Bone Marrow 5-9% blasts dysplasia
36
Define refractory anaemia with excess blasts 2
Blood: cytopenias or 5-19% blasts or auer rods Bone Marrow dysplasia with 10-19% blasts, or auer rods
37
what do MDS sufferers die from
International prognostic scoring system used to predict which takes into account rule of thirds - infection - bleeding - AML
38
Define MDS with 5q deletion
Blood Anaemia normal or increased platelets Marrow Hypolobulated nuclei of megakaryocytes <5% blasts
39
Define unclassified MDS
complex - Blood cytopenias with no blasts or aur rods BM Myeloid or megakaryocytic dysplasia <5% blasts
40
what nationality get adult t cell lymphoma
caribbean and Japanese associated with HTLV1 infection Hypercalcaemia and lymphocytosis. skin lesions and splenomeg
41
Mantle cell lymphoma
t(11,14) BCL-1 locus causing overexertion of cyclin D1 They are aggressive lymphomas with angular nuclei on histology. occur in men more than women.
42
Diffuse Large b cell lymphoma
germinal cell lymphoma - may have undergone richter's transformation from other low grade germinal cell lymphoma or CLL. Very aggressive high grade with sheets of large lymphoid cells which needs to be treated with RCHOP.
43
What does RCHOP stand for and what is it used in?
``` Rituximab cyclophosphamide vincristine doxorubicin prednisolone ``` DLBCL and mantle cell.
44
What chemo is often used in hodgkins lymphoma
ABVD - adriamycin, bleomycin, vinblastine, decarbazine. can use MOPP but makes sterile
45
What is erythmelalgia?
painful burning or swelling sensation in the fingers often associated with essential thrombocythemia - megakaryocytic in the bone marrow. excess dysfunctional platelets. dizziness, headaches, stroke, MI gangrene thrombosis. treat with anegralide or hydroxycarbamide.
46
what causes the hepatosplenomegaly in myelofibrosis
extramedullary haematopoeisis
47
What is seen on myelofibrosis investigations?
Blood film - tear drop poikilocytes and leukoerthyroblasts | bone marrow - dry tap
48
What is the cause of myelofibrosis
may be idiopathic or occur following Polycythemia or essential thrombocythaemia.
49
antibody found in autoimmune type 1 diabetes
Anti - GAD
50
CD55 and CD59 are found in what disease
PNH
51
what does CK vary with
exercise and race
52
what does albumin vary with
posture
53
Where is THC NOT found
saliva
54
Name 3 diseases which come about through constant antigenic stimulation
EATL - coeliac Parotid lymphoma - sjogren's Gastric carcinoma - h.pylori
55
Name 3 diseases which come about through direct action and viral drive from infection rather than via antigenic stimulation
HTLV1 - adult t cell lymphoma EBV - burkitts +? HIV -
56
What CD are b cells associated with?
19? often 20 used as rituximab is common therapy
57
What CD are t cell lymphomas associated with
3+5
58
what marker is often used in DLBCL staging
LDH, age
59
t(8,14)
burkitts lymphoma
60
t(15,17)
APML
61
t(14,18)
follicular lymphoma
62
t(11,18)
MALToma
63
t(11,14)
mantle cell lymphoma
64
Acanthocytes
also known as spurr cells signify: liver disease hyposlenism abetalipoproteinaemia
65
What is basophilic RBC indicative of?
Increased rate of erythropoeisis or defective synthesis. likely due to megaloblastic anaemia or myelodysplasia or LEAD POISINING
66
Heinz bodies
inclusions within red blood cells which give rise to bite cells - G6PD
67
Howell jolly body
basophilic staining of the cytoplasm and nuclear remnants in RBC hyposlenism sickle cell
68
Leucoerythroblastic anaemia
nucleated RBCs and WBC's in the periphery. marrow infiltration i.e myelofibrosis/malignancy
69
Pelger huet cells
hyposegmented neutrophils | myelodisplastic/leukaemia
70
polychromasia
reticulocytosis - gey blue cells. | increases in haemolytic anaemia and decreases in aplastic anaemia or with chemotherapy
71
rouleaoux
stacking myeloma and paraproteinaemia
72
spherocytes
round - usually hereditary spherocytosis or may be due to AIHA (warm)
73
target cells
bulls eyes HHH hyposplenism hepatic pathology haemaglobinopathies.
74
What is the normal range of anaemia in men and women
135 in men and 115 in women.
75
What are the GI causes of bleefding
meckels diverticulum peptic ulcers polyps/cancers hookworm
76
What is the mechanism for anaemia of chronic disease?
normocytic reduction of EPO - IL! TNF etc reduction of iron transportation out of gut cells - IL6
77
What is high in anaemia of chronic disease
ferritin - iron store
78
what is a sideroblastic anaemia
iron cannot be correctly incorporated into the cells and so is dotteda round the place - occurs as part of myelodysplasia and genetic conditions
79
what is the treatment for sideroblastic anaemia
remove the cause and give vitamin b6 to increase production
80
What antibodies do perncicious anaemia sufferers have
anti parietal cell - 90 anti intrinsic factor - 50 schilling test but is outdated.
81
Removal of what organs gievs youpernicious anaemia?
stomach removal or terminal ilium. may be after fat or chrons surgery. TROPICAL SPRUE
82
what are the signs of pernicious anaemia
b12 def glossitis angular cheilosis irritability, depression psychosis tingling neuropathy, spastic paraparesis and SACD spinal cord ABSENT ANKLE REFLEX
83
What will make a b12 neuropathy worse
folic acid.
84
What are the indications of intravascular hameolysis?
free plasma hb haemagolbinurea reduced haptoglobin methaemaglobinaemia
85
What are the indications of extravascular hameolysis
splenomegaly
86
How do you recognise all haemolysis
unconjugated bilirubin goes up, urobilinogen goes up(clear excreted in urine and faeces) LDH goes up GALL STONES
87
When do you get pencil cells
hereditary eliptocytosis but also in IDA
88
When would you see spectrin mutations
both hereditary spherocytosis and eliptocytosis
89
What are echinocytes
``` burr cells (not spurr) pyruvate kinase deficiency and EDTA change ```
90
what goes down in HUS
platelets
91
What does TXA2 do?
causes platelet aggregation
92
what does a2 macroglobulin do?
inhibits 10
93
what does uraemia do to platelets
reduces their function
94
What are the main things associated with Haemophilia A
factor 8 deficiency causes prolonged bleeding and haemarthroses can have varying degrees of loss.
95
What is VWf function in the blood stream?
to carry factor 8 when there is von willebrands disease they will have reduced factor 8 aswell as reduced platelt functioning which would be BLEEDING such as nosebleeds.
96
what is more common haemophilia A or B
A is 5 times more common | x linked recessive so is in men
97
how do you treat von willebrands
gove low purity factor 8 or desmopressin
98
what is d dimer also known as
type of FDP.
99
what factors are not made when there is liver disease?
2 5 7 9 10 11 | platelts reduced function

Joshua's Path (9 decks)

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