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Phase 2a - Jemima > Haemotology > Flashcards

Haemotology Flashcards

1
Q

what is anaemia

A

it is a lower than normal concentration of haemoglobin or red blood cells

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2
Q

what levels of haemoglobin suggests anaemia in
1. men
2. women

A
  1. less than 130
  2. less than 120
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3
Q

what is haemolytic anaemia

A

increased breakdown of red blood cells

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4
Q

what is anaplastic anaemia

A

decreased red blood cells, white cells and platelets

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5
Q

what is microcytic anaemia

A

a reduced mean corpuscular volume

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6
Q

what is a MVC blood test

A

An MCV blood test measures the average size of your red blood cells

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7
Q

what is macrocytic anaemia

A

a raised MCV

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8
Q

what are the general symptom of anaemia

A

fatigue, headache, dizziness, dyspnoea (especially on exertion)

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9
Q

what are signs of anaemia

A

tachycardia, skin pallor, conjunctiva pallor, intermittent claudication

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10
Q

What is koilonychia and what does it indicate

A

spoon shaped nails - iron deficiency

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11
Q

what is angular stomatitis a sign of

A

iron deficiency and B12 deficiency

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12
Q

what does lemon yellow skin indicate

A

B12 deficiency

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13
Q

what does jaundice or dark urine indicate

A

haemolytic anaemia

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14
Q

what is microcytic anaemia

A

it is iron deficiency with low Hb, and a low MCV (less than 80 fl)

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15
Q

what is seen in iron studies of someone with microcytic anaemia

A

there is low ferratin (unless active inflammation)
low serum iron
low transferrin saturation
raised transferrin

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16
Q

what is seen on a blood film with microcytic anaemia

A

small hypochromic cells

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17
Q

what are causes of microcytic anaemia

A

reduced absorption - low intake, malabsorption or drugs like PPIs
increased utilization - pregnancy
blood loss - stool, trauma, surgery, menorrhagia
Thalassemia
scleroblastic anaemia

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18
Q

in chronic microcytic anaemia what is seen on a full blood count

A

low Hb, normal or low MVC but high ESR

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19
Q

what do iron studies of someone with chronic microcytic anaemia look like

A

normal or raised ferratin
low serum iron
low transferrin saturation and transferrin

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20
Q

what might be causes of chronic microcytic anaemia

A

chronic infection
chronic inflammation (connective tissue disease)
neoplasia

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21
Q

what could be other causes of microcytic anaemia

A
  1. sickle cell
  2. Thalassemia
  3. Sideroblastic anaemia
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22
Q

what is sideroblastic anaemia

A

it is when the bodies iron levels are normal but the body cant insert the iron into Hb

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23
Q

what can cause normocytic anaemia

A

acute blood loss
bone marrow failure
pregnancy
Haemolytic anaemia
aplastic anaemia
chronic disease

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24
Q

what is the presentation of haemolytic anaemia

A

jaundice and dark urine

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25
Q

what investigations are done to test for haemolytic anaemia

A

raised reticulocytes (chronic), raised bilirubin, raised urobilinogen, schistocytes on blood film (fragmented RBCs)

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26
Q

what are causes of haemolytic anaemia

A
  1. autoimmunity
  2. sepsis or disseminated intravascular coagulopathy
  3. Sickle cell
  4. thalassemia
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27
Q

what is a type of macrocytic anaemia

A

B12 deficiency

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28
Q

what are causes of B12 deficiency

A

perncious anaemia - lack of intrinsic factor (cant absorb B12)
malabsorption - (coeliac, IBD, bowel resection, ileostomy)
decreased dietary intake
chronic nitrous oxide use

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29
Q

how does B12 deficiency present

A

Bloods: raised MCV, low Hb, low B12
Megaloblastic anaemia: defined by cell changes on blood smear
S&S: general anaemia presentation and a range of neurological symptoms as well

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30
Q

what is megablastic anaemia characterised by

A

oval shaped RBC’s, hyper segmented neutrophils

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31
Q

what could be other causes of macrocytic anaemia

A

diseases of the liver and spleen
haematological malignancy
alcohol

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32
Q

what can cause neutrophilia

A

infection
inflammation
CML

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33
Q

what can cause neutropenia

A

antibiotics
chemotherapy
marrow failure
liver disease

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34
Q

what can cause thrombocytosis

A

infection
inflammation
tissue injury
splenectomy
essential thrombocytopenia

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35
Q

what can cause thrombocytopenia

A

production failure - marrow failure (congenital/leptospirosis)
increased removal - ITP, TTP, DIC

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36
Q

what can cause lymphocytosis

A

EBV
CMV
hepatitis
malignancy - CLL, ALL, lymphoma
stress

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37
Q

what can cause lymphocytopenia

A

steroids
HIV
post - viral
marrow failure
chemotherapy

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38
Q

what is primary haemostasis

A

initiation and formation of a platelet plug
- platelet activation

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39
Q

what is secondary haemostasis

A

the formation of the fibrin clot
- intrinsic and extrinsic coagulation pathway

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40
Q

what causes initial platelet activation

A

collagen binding to GPVI/GPIIbIIIa via vWF

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41
Q

what causes amplification of platelet activation and clotting

A

thromboxane - binds TPa receptors
ADP binds P2Y12/P2Y1 receptors
other platelets form GPIIbIIIa cross bridges
thrombin - binds to PAR1/PAR4 receptors

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42
Q

what are the effects of platelet activation

A

platelets change shape
dense granules are released - contains ADP
alpha granules are released - inflammatory mediators and clotting factors

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43
Q

what drugs inhibits thromboxane formation

A

NSAIDS

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44
Q

what drugs inhibit P2Y12 binding

A

clopidogrel or ticagrelor

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45
Q

what drugs inhibits thrombin

A

dabigatran

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46
Q

what is the intrinsic clotting cascade

A

12 - 11 - 9 - 8 - 10 - 10a

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47
Q

what is the intrinsic clotting cascade initiated by

A

endothelial collagen exposure

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48
Q

what is the extrinsic clotting pathway

A

7 - 10 - 10a

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49
Q

what is the extrinsic clotting cascade initiated by

A

tissue factor - expressed on immune cells and endothelium

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50
Q

what is the common clotting cascade

A

10a - prothrombin (II) - thrombin (IIa) - fibrinogen - fibrin

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51
Q

what factor reinforces fibrin

A

factor 13

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52
Q

what does warfarin do

A

it blocks vitamin K - therefore vitamin K derived clotting factors - 2, 7, 9, 10 `

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53
Q

what is the action of heparin

A

acts via antithrombin III to prevent prothrombin activation

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54
Q

what is sickle cell

A

it is a genetic condition affecting the B globin chain (glutamic acid is substituted with valine)

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55
Q

what is the acute presentation of sickle cell

A
  1. MSK: bone and joint pain
  2. Infection
  3. Resp: dysponea, cough, hypoxia
  4. CNS: stroke
  5. GI: sequestration crisis
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56
Q

what is sequestration crisis

A

it is when the blood outflow of the spleen is blocked and blood accumulates leading to splenomegaly

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57
Q

what are risk factors for sickle cell crisis

A

low oxygen
cold weather
parvovirus B19
exertion

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58
Q

what are chronic complications of sickle cell

A

avascular necrosis of joints
silent CNS infarct
retinopathy
nephropathy
ED

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59
Q

what investigations are done for sickle cell anaemia

A

FBC - low MCV and low Hb
Blood smear - sickles erythrocytes
sickle solubility test
Hb electrophoresis

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60
Q

what is the management for Sickle cell crisis

A
  1. Acute: Morphine, O2, IV fluids, transfusion exchange
  2. Chronic: hydroxycarbamide
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61
Q

What is thrombotic thrombocytopenic purpura

A

ADAM TS13 protein deficiency - vWF cleaving protease
- you cant break clumps of vWF into monomers and it can cause microvascular clots to form

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62
Q

what are the risks for developing thrombotic thrombocytopenic purpura

A

being adult
being female

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63
Q

how does thrombotic thrombocytopenic purpura present

A

fatigue
fever
jaundice
petechiae
purpura
neurological defects

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64
Q

how do you diagnose thrombotic thrombocytopenic purpura

A

FBC: raise white cell count, low Hb and low platelets
Other: raised bilirubin and raised creatinine
blood smear: schistocytes
Clotting: PT and APTT is normal

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65
Q

what is the treatment for thrombotic thrombocytopenic purpura

A

plasma exchange, IV methylprednisolone, monoclonal Abs

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66
Q

what is immune thrombocytopenic purpura

A

it is autoimmune IgG destruction of GPIIbIIIa meaning platelets cant activate and therefore causes problems with primary haemostasis

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67
Q

what are the risk factors for developing immune thrombocytopenic purpura

A

paediatric
post viral

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68
Q

what is the presentation of ITP

A

very similar to TTP - fatigue
fever
jaundice
petechiae
purpura
neurological defects
it is a diagnosis of exclusion really

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69
Q

what is seen when investigating ITP

A

FBC: raised white cell count, low Hb, low platelets
clotting: PT and APTT are normal
Blood smear is normal

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70
Q

What is the treatment for ITP

A

steroids or IV IgG

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71
Q

what is leukaemia

A

is the the cancer of bone marrow

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72
Q

what causes leukaemia

A

it is where immature blast cells proliferate uncontrollably, it takes up space within the bone marrow and then infiltrates into other tissues.
the lack of space within the bone marrow means fewer healthy cells can mature and be released into the blood.

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73
Q

what are the 4 types of leukaemia

A

acute myeloid leukaemia
acute lymphoblastic leukaemia
chronic lymphoblastic leukaemia
chronic myeloid leukaemia

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74
Q

what are features of acute lymphoblastic leukaemia

A

it is most common in children (0-4)
proliferation of immature lymphoblasts

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75
Q

how does acute lymphoblastic leukaemia present

A

general anaemia symptoms
bleeding/bruising
infections
hepatosplenomegaly
lymphadenopathy
CNS infiltration - headaches and palsies

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76
Q

How do you diagnose acute lymphoblastic leukaemia

A

FBC - anaemia, thrombocytopenia, neutropenia
blood film
bone marrow biopsy
imaging (CXR/CT) - lymphadenopathy

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77
Q

how do you manage acute lymphoblastic leukaemia

A

blood and platelet transfusions
chemotherapy (methotrexate)
steroids
stem cell or bone marrow transplant
antibiotics

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78
Q

how does anaemia present

A

fatigue
dizziness
palpitations
pallor
shortness of breath

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79
Q

what is acute myeloid leukaemia

A

it is the proliferation of immature myelobasts

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80
Q

what are features of acute myeloid leukaemia

A

it is present mostly in the elderly
it only has a 15-5% survival

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81
Q

how does acute myeloid leukaemia present

A

general anaemia symptoms
bleeding or bruising
infections
hepatosplenomegaly
gum hypertrophy

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82
Q

how do you diagnose acute myeloid leukaemia

A

FBC - anaemia and thrombocytopenia
blood film
bone marrow biopsy - auer rods

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83
Q

how do you manage acute myeloid leukaemia

A

blood and platelet transfusions
chemotherapy
stem cell or bone marrow transport
antibiotics

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84
Q

what is chronic lymphoblastic leukaemia

A

proliferation of B lymphocytes

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85
Q

what are the features of chronic lymphoblastic leukaemia

A

mostly affects those 60 plus
most common type of leukaemia in adults

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86
Q

how does chronic lymphoblastic leukaemia present

A

it is often asymptomatic
lymphadenopathy
may have night sweats and weight loss

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87
Q

how do you diagnose chronic lymphoblastic leukaemia

A

FBC - anaemia, thrombocytopenia, leukocytosis
blood film - smudge cells
bone marrow biopsy

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88
Q

how do you manage chronic lymphoblastic leukaemia

A

watch and wait in early stages
chemotherapy (rituximab)
stem cell or bone marrow transplant

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89
Q

what is chronic myeloid leukaemia

A

it is proliferation of myeloid blood cells

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90
Q

what are features of chronic myeloid leukaemia

A

most common in adults over 40
the vast majority of cases are associated with the Philadelphia chromosome

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91
Q

how does chronic myeloid leukaemia present

A

general anaemia symptoms
blooding or bruising
infections
hepatosplenomegaly
weight loss and night sweats
gout

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92
Q

how do you diagnose chronic myeloid leukaemia

A

FBC: anaemia, thrombocytopenia and leukocytosis
bone marrow biopsy
blood film
genetic testing

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93
Q

how do you manage chronic myeloid leukaemia

A

chemotherapy
stem cell or bone marrow transplant
tyrosine kinase inhibitors (Imatinib)

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94
Q

what is Hodgkin lymphoma

A

proliferation of lymphocytes in the lymph nodes

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95
Q

what is Hodgkin lymphoma associated with

A

EBV and immunosupression

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96
Q

what sort does incidence does Hodgkin lymphoma have

A

Bimodal distribution - peaks in early 20s and then in the 70s

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97
Q

what is the presentation of Hodgkin lymphoma

A

lymphadenopathy - painful upon drinking alcohol !!!
B-symptoms - fever, night sweats and weight loss

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98
Q

How do you diagnose Hodgkin lymphoma

A

ESR is raised
imaging is done (CXR/CT) - staging
lymph node biopsy - reed sternberg cells !!!

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99
Q

how do you manage Hodgkin lymphoma

A

ABVD chemotherapy - Doxorubicin, bleomycin, vinblastine, dacarbazine
radiotherapy
steroids
stem cell or bone marrow transplant

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100
Q

What is associated with Non- Hodgkin lymphoma

A

associated with EBV and immunosuppression

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101
Q

when does Non-Hodgkin lymphoma typically present

A

predominantly affects adults - over the age of 40

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102
Q

How does Non-Hodgkin lymphoma present

A

painless lymphadenopathy
B symptoms are weight loss, night sweats, fever
can get hepatosplenomegaly

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103
Q

How do you diagnose Non-Hodgkin lymphoma

A

imaging (CXR/CT) for staging
Lymph node biopsy - no Reed Sternberg cells

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104
Q

how do you manage Non-Hodgkin lymphoma

A

RCHOP chemotherapy
- rituximab
- cyclophosphamide
- hydroxy-daunorubicin
- vincristine
- prednisolone
radiotherapy

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105
Q

how do you stage lymphoma

A

the ann arbor staging is used for lymphoma staging

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106
Q

what is stage 1 on the Ann arbor scale

A

the disease is only in one area only

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107
Q

what is stage 2 on the Ann arbor scale

A

the disease is in 2 or more areas on the same side of the diaphragm

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108
Q

what is stage three on the Ann Arbor scale

A

the disease is in 2 or more areas on both sides of the diaphragm

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109
Q

what is stage four on the Ann Arbor scale

A

the disease has spread beyond the lymph nodes

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110
Q

what age group does multiple myeloma occur in (normally)

A

predominantly occurs in those over the age of 40

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111
Q

what condition does multiple myeloma have a close link to

A

monoclonal gammopathy of undetermined significance

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112
Q

what is monoclonal gammopathy of undetermined significance

A

there is too much of an immunoglobulin released by abnormal plasma cells

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113
Q

how does multiple myeloma present

A

hypercalcaemia - polydipsia, polyuria, constipation, abdominal pain
renal impairement
anaemia - fatigue, dizziness
bone lesions - bone pain

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114
Q

how do you diagnose multiple myeloma

A

FBC - anaemia, rouleaux formation (aggregation of RBC)
ESR is raised
Blood film - rouleaux formation
Serum and urine electrophoresis - bence jones protein in urine !!!!
bone marrow biopsy
imaging (X-ray/CT) - bone lesions

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115
Q

how do you manage multiple myeloma

A

chemotherapy
stem cell transplant
analgesia
bisphosphonates
blood transfusions

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116
Q

what are common chemotherapy combinations

A

VCD - bortezomib, cyclophosphamide, dexamethasone
VTD - bortezomib, thalidomide, dexamethasone
MTP - melphalan, thalidomide, prednisolone

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117
Q

what is polycythaemia

A

a high concentration of erythrocytes in the blood

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118
Q

what are types of polycythaemia

A

absolute - split into primary and secondary
relative

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119
Q

what is relative polycythaemia

A

there are a normal number of erythrocytes but there is a reduction in plasma

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120
Q

what are causes of relative polycythaemia

A

causes include obesity, dehydration and excessive alcohol consumption

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121
Q

what is absolute polycythaemia

A

there is an increased number of erythrocytes

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122
Q

what is primary absolute polycythaemia

A

abnormality in the bone marrow
- caused polycythaemia vera

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123
Q

what is secondary absolute polycythemia

A

a disease outside the bone marrow causing overstimulation of the bone marrow

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124
Q

what causes secondary absolute polycythaemia

A

COPD, sleep apnoea, PKD, renal artery stenosis, kidney cancer

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125
Q

what is a myeloma

A

a malignant tumour of the bone marrow

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126
Q

what are the features of monoclonal gammopathy of undetermined significance

A

it is a myeloma made up of either solitary, multiple solitary or extramedullary plasmacytomas

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127
Q

what is multiple myeloma

A

neoplastic proliferation of bone marrow plasma cells

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128
Q

what is multiple myeloma characterised by

A

Monoclonal protein in serum or urine
lytic bone lesions/ CRAB end organ damage
excess plasma cells in bone marrow

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129
Q

what is the criteria for multiple myeloma diagnosis

A

protein in the blood
bone marrow plasma cells in excess of 10%
CRAB - cancer renal anaemia bone

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130
Q

what are common chromosomal abnormalities that can cause multiple myeloma

A

T(11:14) - most common
13q - associated with treatment resistance and poorer prognosis

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131
Q

What are common presenting features of multiple myeloma

A

tiredness and malaise
bone/back pain and more common fractures
infections
non specific symptoms

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132
Q

what lab results would you expect for someone with multiple myeloma

A

anaemia
abnormal FBC
renal failure
hypercalcaemia
raised globulins
raised ESR
serum or urine paraprotein

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133
Q

what is the main cause of death in myeloma

A

infection

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134
Q

what is amyloidosis

A

it is a group of rare serious conditions caused by a build up of an abnormal protein called amyloid in the organs and other tissues in the body

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135
Q

how can clonal expansion in MGUS lead to plasma cell leukemia

A

MGUS - early myeloma - late myeloma - plasma cell leukemia

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136
Q

what can plasmacytoma at a critical site lead to

A

cord compression
risk of fracture

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137
Q

what are the response rates in malignant myeloma treatment

A

many will respond to the treatment but will eventually relapse
can prolong survival by keeping people in plateau rather than try to cure it
both the disease and the treatment have a morbidity

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138
Q

what is leukaemia

A

malignant proliferation of haematopoietic cells

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139
Q

what might the history of a patient with leukaemia be

A
  1. anaemia - fatigue or shortness of breath
  2. infection - tonsillitis or fever
  3. thrombocytopenia - bruising, bleeding, rash
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140
Q

what does the blood of someone with leukaemia look like

A

low haemoglobin, low platelets, very low neutrophils, blast cells present with auer rods

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141
Q

what does blast cells present with auer rods suggest ?

A

someone has acute myeloid leukaemia

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142
Q

what is the differential diagnosis for leukaemia be

A
  • Acute leukaemia: AML, ALL, CML in blast crisis, myelofibrosis
  • severe sepsis
  • post operative
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143
Q

what blood cells type does acute myeloid leukaemia come from

A

myeloblasts (progenitor of basophil/eosinophil/neutrophil/monocytes)

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144
Q

what blood cell type does chronic myeloid leukaemia come from

A

basophil, neutrophil, eosinophil

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145
Q

what cell type does acute lymphoblastic leukaemia come from

A

lymphoblasts

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146
Q

what cell type does chronic lymphoblastic leukaemia come from

A

B lymphocytes

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147
Q

what is chronic myelomonocytic leukaemia

A

it is when there are too many monocytes in the blood

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148
Q

how do you diagnose chronic myelomonocytic leukaemia

A

blood tests - morphology, cytogenetics, flow cytometry, sequencing
Bone marrow aspirate and trephine biopsy
cytogenetics for prognosis
molecular genetics for prognosis
immunophenotyping

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149
Q

is chronic myeloid leukaemia slow or fast onset

A

slow onset

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150
Q

what is seen in the blood of someone with chronic myeloid leukaemia

A

a high white cell count
basophilia

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151
Q

what is a key diagnostic feature of chronic myeloid leukaemia

A

Philadelphia chromosome

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152
Q

what is the treatment for chronic myeloid leukaemia

A

you have targeted molecular therapy - tyrosine kinase inhibitors
- Imatinib

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153
Q

what is the treatment for acute myeloid leukaemia

A

chemotherapy and supportive measures
- need to take into account patient age, fitness, comorbidities, AML features, and clinical trials that may be happening

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154
Q

what is the most common paediatric malignancy

A

acute lymphoblastic leukaemia

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155
Q

what is the acute presentation of acute lymphoblastic leukaemia

A

bone marrow failure
organ infiltration

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156
Q

how do you treat ALL

A

CNS directed therapy
stem cell transplant
treatment phases - induction, consolidation, delayed intensification and maintenance

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157
Q

what is the most common type of leukaemia

A

chronic lymphocytic leukaemia

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158
Q

what is chronic lymphocytic leukaemia

A

there is gradual accumulation of B lymphocytes
- this accumulates in blood or bone marrow
- lymph glands including the spleen can accumulate

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159
Q

what age group does CLL normally affect

A

generally in the elderly
- 20% occurs in under 55s

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160
Q

what is the clinical course of CLL

A

it is variable - can get progressive lymphadenopathy or hepatosplenomegaly. You can get autoimmune issues with haemolysis and ITP, and you can get bone marrow failure due to the marrow replacement

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161
Q

how do you treat CLL

A

you can do nothing
chemotherapy
monoclonal antibodies
targeted therapy
bone marrow transplant

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162
Q

what is the action of chemotherapy

A

it damages highly proliferative cells (preferentially)

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163
Q

why is dosing important with chemotherapy

A

as it needs to be a balance between
1. Destroying leukemic cells
2. not causing irreversible toxicity to other normal cells

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164
Q

what are other things you can consider pre-chemotherapy

A

supportive measures - fertility cryopreservation
clinical trial availability

165
Q

what are side effects of chemotherapy

A

nausea
cytopenias = anaemia, neutropenia, low platelets and risk of bleeding
bystander organ damage - kidney, liver
temporary hair loss

166
Q

what is a lymphoma

A

it is a malignant growth of white blood cells - predominantly in the lymph nodes
- can also be in the blood, spleen, bone marrow, liver

167
Q

what is the aetiology (causes) of lymphoma

A
  1. primary immunodeficiency - ataxia telangiectasia
  2. secondary immunodeficiency - HIV, transplant recipients
  3. Infection - EBV, helicobacter pylori
  4. Autoimmune disorders
168
Q

what are signs and symptoms of lymphoma

A

common nodal disease symptoms
compression syndromes
systemic symptoms - B symptoms

169
Q

how do you diagnose lymphoma

A

blood film
bone marrow biopsy
immunophenotyping
cytogenetics (FISH)
molecular techniques - PCR

170
Q

how would you assess a new cause of lymphoma

A

staging - how bad is it
assessment of patient - how good is it
MDT

171
Q

how do you stage a lymphoma

A

blood tests - FBC, U/E, viral serology
CT scan or the chest, abdomen and pelvis
Echo
Bone marrow biopsy

172
Q

what are the performance status stages in a patient with lymphoma

A

0 = Asymptomatic
1 = symptomatic but completely ambulatory (able to carry out light work)
2 = Symptomatic <50% of the day in bed
3 = symptomatic >50% in bed but not bedbound
4 = bedbound
5 = death

173
Q

what are the subtypes of lymphoma

A

Hodgkins lymphoma
Non - hodgkins lymphoma

174
Q

what are the different types of Non-Hodgkin lymphoma

A

Low grade - follicular lymphoma
High grade - diffuse large B cell lymphoma
very high grade - burkitts lymphoma

175
Q

how does Hodgkin lymphoma present

A

painless lymphadenopathy
B symptoms - sweat, weight loss

176
Q

How do you diagnose Hodgkin lymphoma

A

Reed Sternberg cell - abnormal, large lymphocytes that may contain more than one nucleus
- 4 histological subtypes

177
Q

what is the treatment for stage 1-2A Hodgkins lymphoma

A

short course of combination chemotherapy followed by radiotherapy

178
Q

what is the treatment for stage 2B-4 Hodgkin lymphoma

A

combination chemotherapy

179
Q

what are the long term effects of chemotherapy

A

infertility
Anthracyclines - cardiomyopathy
Bleomycin - lung damage
Vinca alkaloids - peripheral neuropathy
second cancers
psychological issues

180
Q

what are the features of a low grade (indolent) non-hodgkin lymphoma

A

it is slow growing and is usually advanced at presentation

181
Q

what are the different types of low grade non-hodgkin lymphoma

A

Follicular lymphoma - MOST COMMON
mantle cell lymphoma
marginal zone lymphoma
small lymphocytic lymphoma
lymphoplasmacytic lymphoma
skin lymphoma

182
Q

what is the treatment for low grade non hodgkin lymphoma

A

alkylating agents
combination chemotherapy
purine analogues
monoclonal antibodies
radiotherapy
oral targeted agents
bone marrow transplant

183
Q

what are features of high grade (aggressive) non hodgkin lymphoma

A

they usualy have nodal presentation and 1/3 cases are extranodal as well

184
Q

what are the most common types of high grade non hodgkin lymphoma

A

diffuse large B cell lymphoma
Burkitt lymphoma
peripheral T cell lymphoma

185
Q

how do you treat aggressive non hodgkin lymphoma

A

early - use a short course of chemotherapy with radiotherapy
late - combination chemotherapy with monoclonal antibodies

186
Q

what is anaemia

A

it is reduced red cell mass - can also have a reduced haemoglobin concentration as well

187
Q

what are the consequences of anaemia

A

reduced oxygen transport
tissue hypoxia
compensatory changes - increased tissue perfusion, oxygen transfer to tissues and red cell production

188
Q

what are the pathological changes of anaemia

A

there can be myocardial fatty change
fatty change within the liver
it can aggravate angina or claudication
can cause atrophy of the skin and nails
can cause CNS cell death in the cortex and the basal ganglia

189
Q

what is the lifespan of red blood cells

190
Q

what is anaplastic anaemia

A

a condition that occurs when your bone marrow fails and stops making haematopoetic stem cells meaning your body stops producing enough new blood cells

191
Q

what can reduce circulating red blood cell levels

A

hypoplastic anaemias
post haemorrage anaemia
haemolytic anaemia
dyshaemopoietic anaemias

192
Q

what are examples of microcytic anaemia

A

iron deficiency
chronic disease
thalassaemia
rare diseases: congenital sideroblastic anaemia, lead poisoning

193
Q

how do you investigate iron deficiency

A

tests of deficiency - ferritin and iron studies
test of causes
treatment

194
Q

what are examples of normocytic anaemia

A

acute blood loss
anaemia of chronic disease
combined haematinic deficiency

195
Q

what is combined haemanitic deficiency

A

combined iron/vitamin B12 or folate deficiency

196
Q

what are examples of macrocytic anaemia

A

B12 or folate deficiency
alcohol excess or liver disease
hypothyroidism
antimetabolite therapy
haemolysis
bone marrow failure
bone marrow infiltration

197
Q

how do you investigate B12 deficiency

A

look for Intrinsic factor antibodies
the Schilling test
look for Coeliac antibodies
B12 replacement

198
Q

what is the schilling test

A

it is the Vitamin B12 absorption test

199
Q

what should you think when someone has combines haemanitic deficiency

A

they have malabsorption problems

200
Q

what investigations should be done when someone presents with anaemia

A
  • thorough history and examination
  • full blood count and blood film
  • reticulocyte count
  • U+Es, LFT and TSH
  • B12, folate and transferrin levels
201
Q

what can a small decrease in red cell survival be compensated by

A

increased EPO and a reduced apoptosis

202
Q

what is foetal haemoglobin

A

it is 2 alpha and two gamma chains

203
Q

what chromosome codes for beta globin chains

A

chromosome 11 - 2 genes

204
Q

what chromosome codes for A globin chain

A

chromosome 16 - 4 genes

205
Q

what is haemoglobin S

A

it is a variant of haemoglobin arising because of a point mutation on the beta globin gene

206
Q

what does the carrier status of haemoblobin S offer protection against

A

offers protection against falciparum malaria

207
Q

what are common complications of sickle cell disease

A

chronic haemolytic anaemia
infections
acute chest syndrome
stroke
avascular necrosis
pulmonary arterial hypertension
ocular disease

208
Q

what are acute complications of sickle cel disease

A

painful crisis
sickle chest syndrome
stroke

209
Q

what are chronic complications of sickle cell disease

A

renal impairment
pulmonary hypertension
joint damage

210
Q

what is disease modifying treatment for sickle cell disease

A

transfusion
hydroxycarbamide
stem cell transplant

211
Q

what is thalassaemia

A

it is a globin chain disorder which results in the diminished synthesis of one or more of the globin chains which then causes consequent reduction in the haemoglobin

212
Q

what is anaemia of chronic disease

A

it is a multifactorial anaeima. Diagnosis generally requires the presence of a chronic inflammatory condition, such as infection, autoimmune disease, kidney disease, or cancer. It is characterized by a microcytic or normocytic anemia and low reticulocyte count.

213
Q

what is the pathology of thalassaemia

A

Insufficient global chains cause precipitation of dominant chain
Causes abnormalities in the red cells
These red cells are then destroyed
Additional ineffective erythropoiesis

214
Q

what are the clinical classifications of beta thalassaemia

A

thalassaemia major - transfusion dependent
thalassaemia intermedia - less severe and can survive without regular blood transfusions
thalassaemia carrier - asymptomatic

215
Q

what is beta thalassaemia major

A

A rare, genetic disease that causes severe anaemia due to the inadequate production of haemoglobin. Children who are afflicted with thalassaemia major require lifelong blood transfusions if they develop to adulthood.

216
Q

at what age do people start to present with beta thalassaemia major

A

6-12 months of age

217
Q

what are the clinical presentations of beta thalassaemia

A

failure to feed
listless
crying
pale

218
Q

what would blood results show in someone with beta thalassaemia

A

Hb 40-70g/L, with low MCV and MCH
the blood film will show large and small very pale red cells and nucleated red blood cells

219
Q

how do you treat beta thalassaemia

A

regular transfusion
iron chelation
endocrine supplementation
support bone health

220
Q

what monitoring needs to occur in beta thalassaemia

A

ferritin
cardiac and liver MRIs
endocrine testing - gonadal function, diabetes screening, growth and puberty, thyroid function, Vitamin D, Calcium and PTH
DEXA scanning for fertility

221
Q

what is a major consequence of transfusions in thalassaemia major

A

someone can get morbidity and mortality iron overload from transfusions

222
Q

how might someone get iron overload with thalassaemia major

A

due to lifelong blood transfusions there will be a progressive increase in the body iron load. There is no means for the body to eliminate the excess iron and these patients develop clinically worsening hemosiderosis

223
Q

what happens as a consequence of iron overload in thalassaemia major

A

excess iron is mainly deposited in the liver and spleen leading to liver fibrosis and cirrhosis. deposits in the endocrine glands and heart leads to diabetes, heart failure and eventually premature death

224
Q

what are the four types of alpha thalassaemia

A

alpha thalassemia silent carrier - one gene missing
alpha thalassaemia carrier - two genes missing, mild anaemia
hemoglobin H disease - three genes are missing
alpha thalassemia major - all 4 genes missing, severe anaemia

225
Q

what is hereditary RBC membranopathies

A

mutations in genes encoding skeletal proteins that alter the membrane complex structure

226
Q

what types of inheritance pattern are membranopathies

A

autosomal dominant conditions

227
Q

what are the most common types of membranopathies

A

spherocytosis and elliptocytosis

228
Q

what is the pathology of membranopathies

A
  • neonatal jaundice
  • mold to moderate haemolytic anaemia (exacerbation during infection)
  • gallstones
  • folic acid and splenectomy
229
Q

what is a common and serious complication of parvovirus B19

A

haemolytic anaemia

230
Q

what age demographic is parvovirus + haemolytic anaemia common in

231
Q

what is Glucose - 6- phosphate dehydrogenase deficiency

A

a genetic disorder that affects red blood cells, which carry oxygen from the lungs to tissues throughout the body. In affected individuals, a defect in an enzyme called glucose-6-phosphate dehydrogenase causes red blood cells to break down prematurely.
- haemolytic anemia

232
Q

what ethnic groups is glucose-6-phosphate dehydrogenase deficiency more common in

A

african, middle eastern, mediterranean, s. asian

233
Q

what is the genetic inheritance of glucose 6 phosphate dehydrogenase deficiency

A

it is X linked but mostly women are affected

234
Q

what is glucose 6 phosphate dehydrogenase deficiency precipitated by

A

broad beans
infections
drugs

235
Q

what drugs shouldnt be given to those with glucose 6 phosphate dehydrogenase deficiency

A

primaquine
sulphonamides
nitrofurantoin
quinolones
dapsone

236
Q

what is pyruvate kinase deficiency

A

a condition in which red blood cells break down faster than they should

237
Q

what is the inheritance pattern of pyruvate kinase deficiency

A

autosomal recessive

238
Q

what are the clinical features of pyruvate kinase deficiency

A

variable chronic haemolysis
can be apparent in neonates, or in later life
they are prone to aplastic crisis if they get parvovirus B19

239
Q

what is the treatment for pyruvate kinase deficiency

A

folic acid
transfusion during severe crisis
consider splenectomy if there are high transfusion requirements

240
Q

what are secondary causes of anaemia

A

smoking
lung disease
cyanotic heart disease
altitude
EPO or androgen excess

241
Q

what is polycythaemia Rubra vera

A

it is a myeloproliferative disorder which causes ‘overreactive bone marrow’, making too many red blood cells

242
Q

what mutation makes up 95% of polycythaemia rubra vera

A

JAK2 mutations

243
Q

what is the clinical presentation of polycythaemia rubra vera

A

plethoric appearance
thrombosis
itching (especially after a bath)
Burning in the fingers and toes
headache
splenomegaly
abnormal full blood count

244
Q

how do you treat polycythaemia rubra vera

A

aspirin
venesection
bone marrow suppressive drugs

245
Q

what are causes of neutrophilia

A

reactive - infection, inflammation, malignancy
primary - CMV

246
Q

what is neutrophilia

A

it is when you have too many white blood cells

247
Q

what is lymphocytosis

A

too many white blood cells - normal neutrophil count

248
Q

what are causes of lymphocytosis

A

reactive - infection, inflammation, malignancy
primary - CLL

249
Q

what is thrombocytopaenia

A

it is when there is not enough platelets

250
Q

what is thrombocytosis

A

it is when you have too many platelets

251
Q

what are causes of thrombocytosis

A

reactive - infection, inflammation, malignancy
primary - essential thrombocythemia (make too many platelets)

252
Q

what is neutropenia

A

when there is not enough neutrophils

253
Q

what are causes of neutropenia

A

underproduction - marrow failure, infiltration or toxicity (drugs)
increased removal - autoimmune, felty’s syndrome, cyclical

254
Q

what is the physiology of platelets

A

they are fragments of megakaryocytes (produced in the bone marrow), and they are regulated by thrombopoietin (produced in the liver)

255
Q

in normal physiology what happens when you have low platelets

A

there is reduced bound TP, meaning there is increased free TPO. This causes an increased megakaryocyte stimulation and therefore increases platelet production

256
Q

what are features of platelets

A
  • they are anucleated cells
  • they have a lifespan of 7-10 days
  • normal count is 150-400x10^9/L
257
Q

what are the surface proteins found on platelets

A

ABO
HPA
HLA class 1
glycoproteins - GP1a

258
Q

what are platelets activated by

A
  • Adhesion to collagen via GP1a
  • Adhesion to vWF via GP1b and IIa/IIIb
259
Q

what does platelet activation lead to

A
  • release of alpha granules containing PDGF, fibrinogen, vWF, PF4
  • Dense granules - nucleotides (ADP), Ca2+, serotonin
  • membrane phospholipids activate clotting factors
260
Q

what is the steps taken by platelets following damage to he endothelium

A
  1. Platelets adhere to vascular endothelium via collagen & vWF (von Willebrand factor)
  2. Binding of platelets to collagen stimulates cytoskeleton shape change within the platelets, and they spread out
  3. This increases their surface area and results in their activation, leading to the release of platelet granule contents including ADP, fibrinogen, thrombin and calcium. These components facilitate the clotting cascade ending with the production of fibrin
  4. Aggregation of platelets then occurs, which involves the cross-linking of activated platelets by fibrin
  5. Activated platelets also provide a negatively charged phospholipid surface, which allows coagulation factors to bind and enhance the clotting cascade
261
Q

what are the different ways you can test platelet function

A
  1. number - full blood count
  2. appearance - blood film
  3. function - PFA - response to aggregating agents
  4. surface proteins - flow cytometry
262
Q

what problems can cause bleeding

A

injury
vascular disorders
low platelets
abnormal platelet function
defecting coagulation
clinical patterns - platelet type v haemophiliac

263
Q

what are the clinical features of platelet dysfunction

A

mucosal bleeding
easy bruising
petechiae - pinpoint spots that appear on the skin due to bleeding
purpura - small blood vessels leaking under skin
traumatic haemotomas

264
Q

what are aquired reasons why platlet production might fail

A

drugs
marrow suppression
marrow failure
marrow replacement

265
Q

what can cause increased removal of platelets

A

immune dysfunction
consumption
splenomegaly

266
Q

what is an artefactual reason for low platelets

A

EDTA induced clumping

267
Q

what are platelet disorders which cause impaired function

A
  1. storage pool disorders
  2. Glanzmann - reduction/deficiency of GPIIb/IIIa
  3. Bernard soulier - reduction/deficiency of GP1b
  4. Von Willebrand disease
268
Q

what are some acquired causes of impaired platelet function

A

uraemia
drugs

269
Q

what is congenital thrombocytopenia

A

it is absent/reduced/malfunctioning megakaryocytes in the bone marrow, meaning there is affected platelet production

270
Q

what infiltrations of the bone marrow could cause a reduced platelet production

A

leukaemia
metastatic malignancy
lymphoma
myeloma
myelofibrosis

271
Q

what can cause a reduced platelet production from the bone marrow

A
  • low B12 or folate
  • reduced TPO
  • medication: methotrexate or chemotherapy
  • toxins: alcohol
  • infections: viral
  • aplastic anaemia
272
Q

what is myelodysplasia

A

a group of cancers in which immature blood cells in the bone marrow do not mature or become healthy blood cells. The different types of myelodysplastic syndromes are diagnosed based on certain changes in the blood cells and bone marrow

273
Q

what can cause dysfunctional production of platelets in the bone marrow

A

myelodysplasia

274
Q

what are diagnostic features of dysfunctional platelets

A

do a blood smear - will see abnormal sizes of platelets (small, large, giant) and an absence of platelet alpha granules

275
Q

what autoimmune conditions can cause thrombocytopenia

A

immune thrombocytopenia - primary or secondary

276
Q

what can cause thrombocytopenia

A

autoimmune
hypersplenism - portal hypertension and splenomegaly
drugs related - heparin induced

277
Q

what can cause increased consumption of platelets

A

disseminated intravascular coagulopathy
thrombotic thrombocytopenic purpura
haemolytic uraemic syndrome
haemolysis, elevated liver enzymes and low platelets
major haemorrhage

278
Q

what medications affect platelets

A
  1. clopidogrel - inhibits P2Y12
  2. Tirofiban - inhibits GpIIb/IIIa
  3. Aspirin - inhibits COX1
279
Q

what causes immune thrombocytopenia

A

IgG antibodies form to platelet and megakaryocyte surface glycoproteins
Opsonized platelets are removed by reticuloendothelial system

280
Q

what are reasons for primary immune thrombocytopenia

A

may follow viral infection/immunisation especially in children

281
Q

what are reasons for secondary immune thrombocytopenia

A

occurs in association with malignancies such as CLL, and infections such as HCV/HIV
occurs in adults, often adult women

282
Q

what is the treatment for immune thrombocytopenia

A

immunosuppression (steroids)
treat the underlying cause
if bleeding then give platelets
tranexamic acid - inhibits fibrin breakdown (good for mucosal bleeding)

283
Q

what is disseminated intravascular coagulopathy

A

a rare but serious condition that causes abnormal blood clotting throughout the body’s blood vessels. You may develop DIC if you have an infection or injury that affects the body’s normal blood clotting process

284
Q

what is the pathophysiology of disseminated intravascular coagulopathy

A

it is provoked due to systemic inflammatory response syndrome (i.e sepsis, severe shock, septic shock, multiple organ dysfunction syndrome). Cytokines are released causing systemic activation of the clotting cascade leasing to microvascular thrombosis and organ failure. Because of this you have have an increased consumption of platelets and clotting factors leading to bleeding

285
Q

what investigations are done for disseminated intravascular coagulopathy

A

find the underlying cause - sepsis or malignancy and treat
provide platelets, FFP (contains clotting factors) and cryoprecipitate (contains fibrinogen and clotting factors)

286
Q

what investigations are done for DIC

A

what is the underlying cause
look at if there is evidence of organ failure
test for thrombocytopenia
prolonged PT and APTT
look at fibrinogen levels
look at d-dimer levels

287
Q

what is thrombotic thrombocytopenic purpura

A

Spontaneous platelet aggregation in microvasculature
Brain, kidney, heart

288
Q

what are signs and symptoms of TTP

A

Bleeding into the skin or mucus membranes.
Confusion.
Fatigue, weakness.
Fever.
Headache.
Pale skin color or yellowish skin color.
Shortness of breath.
Fast heart rate (over 100 beats per minute)

289
Q

what genetic abnormality can lead to TTP

A

Reduction in a protease enzyme - ADAMTS13
Acquired TTP - due to antibodies against ADAMTS13
ADAMTS13 breaks down vWF into smaller pieces
Failure to break down high molecular weight vWF multimers

290
Q

what is the treatment for TTP

A

Urgent plasma exchange (replaces ADAMTS 13 and removes antibody)
Immunosuppression (reduce antibody level)
Do not give platelets - increases thrombosis

291
Q

what are the risk factors for developing disseminated intravascular coagulation

A

sepsis
crush trauma
maliganacy

292
Q

how does DIC present

A

bleeding
epistaxis
bruising
rash
GI bleed
ARDS

293
Q

what is the pathology of DIC

A

there is widespread activation of the coagulation cascade ad platelet activation, caused by inflammation or injury. This causes mircovascuar thrombosis all over the body, initially causing clotting. Then as all clotting factors are consumed, the body can no longer clot and lead to bleeding

294
Q

what is seen on blood test of a patient with DIC

A

there are low platelets
low fibrinogen
high D dimer
long PT
long APTT

295
Q

what is seen on the blood smear of a patient with DIC

A

schistocytes - fragmented parts of red blood cells

296
Q

what does APTT measure

A

measures the intrinsic and common clotting pathway

297
Q

what does the prothrombin time measure

A

the extrinsic pathway

298
Q

how do you treat DIC

A

treat the underlying cause
treat low fibrinogen with cryoprecipitate
treat low platelets with a platelet transfusion

299
Q

What are the two types of haemophilia

A

type A and type B

300
Q

what is type A haemophilia

A

X linked recessive disorder which causes factor VIII deficiency (affects the intrinsic clotting pathway), causing secondary haemostasis

301
Q

what is type B haemophilia

A

it is a rare genetic condition which causes factor IX deficiency (affecting the intrinsic clotting pathway)

302
Q

how does haemophilia present

A

present with a soft tissue bleeding - bleed into muscles and joints leading to haematoma formation

303
Q

what is seen in bloods for haemophilia

A

APTT is long
PT may be normal

304
Q

what is required for haemophilia testing

A

genetic testing - look at factor IX or VII

305
Q

how do you treat haemophilia

A

recombinant factor VIII or IX treatment

306
Q

what is von willebrands disease

A

disease which causes a defect in the quantity or quality of vWF
- many different subtypes

307
Q

What is the most common type of von Willebrand’s disease

308
Q

How is Type 1 vW disease inherited

A

autosomal dominant

309
Q

what is the pathogenesis of Von Willebrands disease

A

Due to a deficiency in either the quality or quantity of vWF there is an inability for blood to clot. Furthermore vWF protect factor VII from liver protein C destruction. Without vWF factor 8 deficiency can occur as well

310
Q

what id the definition of neutrophilia

A

neutrophil levels over 7.5 x10^9/L

311
Q

what is the definition of neutropenia

A

neutrophil levels under 2 x 10^9/L

312
Q

what is the definition of lymphocytosis

A

lymphocyte levels over > 3.5 x 10 ^9/L

313
Q

what is the definition of lymphocytopenia

A

lymphocyte levels under <1.3 X 10 9/L

314
Q

what is the definition of thrombocytosis

A

when platelet levels are over 400 x10^9/L

315
Q

what is the definition of thrombocytopenia

A

when platelet levels are under <150 x10^9/ L

316
Q

what is the normal range for neutrophils in the blood

A

2-7.5 x109/L

317
Q

what is the normal range of lymphocytes in the body

A

1.3-3.5X109/L

318
Q

what is the normal range for platelets in the body

A

150-400 x109/L

319
Q

what is the normal INR range

A

between 0.8 and 1.2

320
Q

what is the normal INR range if someone is on warfarin

321
Q

why might someones INR increase

A

if they are on anticoagulants
if they have liver disease
if they have vitamin K deficiency
if they have disseminated intravascular coagulation

322
Q

what is the normal APTT

A

between 35-45 seconds

323
Q

what is the APTT of someone on heparin

324
Q

What is APTT affected by

A

haemophilia A
haemophilia B
Von Willebrand’s disease

325
Q

what is the mean corpuscular volume

A

it measures the average size and volume of you red blood cells

326
Q

What MCV level is microcytic anaemia

A

anything less than 80

327
Q

what MCV level is nromocytic anaemia

328
Q

what MCV level is macrocytic anaemia

A

anything higher than 95

329
Q

what can cause non haemalytic normacytic anaemia

A

anplastic anaemia
chronic disease - CKD
pregnancy
myelophthisic anaemia

330
Q

what is often seen in bloods for haemolytic normocytic anaemia

A

an increase in reticulocytes and schistocytes

331
Q

what are causes of haemolytic normocytic anaemia

A

sickle cell disease
GPDH deficiency
AHA
hereditary spherocytosis

332
Q

when would you consider someone with anaemia for a transfusion

A

when haemoglobin is under 70g/L or under 80g/L with cardiac myopathy

333
Q

what are causes of iron deficiency anaemia

A

infants = malnutrition, prolonged breastfeeding
children = malnutrition and malabsorption
adults = malnutrition, malabsorption and menorrhagia
elderly = this is rare and a red flag for colon cancer bleeding

334
Q

what is the pathophysiology of iron deficient anaemia

A

iron normally circulated bound to transferrin and is stored as ferritin. however in iron deficiency anaemia there is low haemoglobin synthesis and leads to microcytic anaemia

335
Q

what are Howell jolly bodies

A

nucleated red blood cells

336
Q

what does iron studies look like in an iron deficient patient

A

low serum iron
low ferritin
low transferrin saturation
high TIBC

337
Q

what is thalassemia

A

it is an autonomic recessive haemoglobinopathy

338
Q

what is the pathology of alpha thalassemia

A

this is associated with a decrease in the amount of alpha chains resulting in less haemoglobin. It also leads to the production of unstable beta globin molecules which increases red blood cell destruction

339
Q

what is alpha thalassemia associated with

A

HbH - when there is an abnormal Hb isoform where beta chain tetramers form

340
Q

how many genes encode for alpha haemoglobin

A

4 genes found on chromosome 16

341
Q

how many genes encode for beta globin

A

two genes on chromosome 11

342
Q

what is beta thalassemia

A

where there is a mutation in beta thalassemia gene resulting in normal haemoglobin isoforms but a depletion of the beta chains

343
Q

what are symptoms of beta thalassemia

A

it can cause major issues in the first year of life with general anaemia symptoms
- can cause chipmunk facies where there is a large forehead and cheekbones
- hepatosplenomegaly
- failure to thrive
- gallstones

344
Q

how do you diagnose thalassemia

A

Full blood count - hypochromic RBCs, target cells, microcytic anaemia, increased reticulocytes
Blood film
Haemoglobin electrophoresis (Gold standard)

345
Q

how do you treat thalassemia

A

regular transfusions
iron chelation (desferrioxamine) to prevent iron overload
splenectomy
folate supplements

346
Q

how do you diagnose sideroblastic anaemia

A

FBC and blood film = microcytic cells with ringed sideroblasts
iron studies show increased serum iron, ferritin, transferrin saturation and decreased TIBC

347
Q

what is acute chest crisis

A

when there is pulmonary vessel occlusion in sickle cell disease which causes respiratory distress
- a medical emergency

348
Q

What is a common side effect of sickle cell disease

A

Osteomyelitis - caused by salmonella

349
Q

What is G6PDG deficiency

A

X linked recessive enzymopathy which causes 1/2 lifespan as well as red blood cell degeneration

350
Q

what is G6PDHs normal function

A

It is involved in glutathione synthesis, and is protective against oxidative damage

351
Q

what are the symptoms of G6PDH deficiency

A

it is mostly asymptomatic unless its precipitated
can be caused by
Nephthelene
antimalarials
aspirin
fava beans

352
Q

what are the symptoms of a G6PDH deficiency attack

A

you get a rapid anaemia and jaundice - intravascular haemolysis

353
Q

how do you diagnose G6PDH deficiency

A

FBC and a blood film
- normal between attacks
- Attach causes increased normocytic blood with increased reticulocytes, Heinz bodies and bite cells
- will see low G6PDH levels in the blood

354
Q

how do you treat G6PDH deficiency

A

you avoid the precipitants
blood transfusions when attacks occur

355
Q

what is hereditary spherocytosis

A

it is an autonomic dominant membranopathy which causes deficiency in the structural membrane protein spectrin, causing red blood cells to be more spherical and rigid

356
Q

What is the pathogenesis of hereditary spherocytosis

A

there is an increase in splenic recycling (extravascular haemolysis) which causes splenomegaly as rigid cells get stuck
- risk of autosplenectomy

357
Q

what are the symptoms of hereditary spherocytosis

A

general anaemia
neonatal jaundice
splenomegaly
gall stones (50%)

358
Q

how do you diagnose hereditary spherocytosis

A

FBC and blood = Normocytic normochromic and increased reticulocytes. Spherocytes also seen

359
Q

what is the treatment for hereditary spherocytosis

A

splenectomy (decreased extravascular haemolysis)
- need to wait until they are 6 to reduce sepsis risk
- folate supplements
- transfusions

360
Q

how do you treat neonatal jaundice

A

phototherapy

361
Q

what is there a risk of if neonatal jaundice is untreated

A

risk of kernicterus if untreated - when bilirubin accumulates in the basal ganglia leading to CNS dysfunction and death

362
Q

what is autoimmune hemolytic anaemia

A

it is when autoantibodies bind red blood cells and lead to intra or extra haemolysis

363
Q

what are the two subtypes of autoimmune hemolytic anaemia

A

warm and cold subtypes

364
Q

what specialist test can you do for autoimmune hemolytic anemia

A

the coombs tests - you are direct coombs positive

365
Q

what is the coombs test

A

causes agglutination of the red blood cells with coombs reagent

366
Q

what is myelophthisic process

A

it is when the bone marrow is replaced with something else such as a malignancy

367
Q

why can chronic kidney disease cause anaemia

A

because you can get haemolytic anaemia due to low EPO

368
Q

what are the causes of aplastic anaemia

A

idiopathic acquired mostly, perhaps with infection or congenital

369
Q

what is the FBC of someone with aplastic anaemia

A

normocytic anemia with low reticulocytes

370
Q

what are the symptoms of pernicious anaemia

A

general anemia +
- lemon yellow skin
- angular stomatitis and glossitis
- neurological symptoms
- muscle weakness
- symmetrical paraesthesia

371
Q

what are causes of folate deficient anemia

A

malnutrition
malabsorption
pregnancy
trimethoprim
methotrexate

372
Q

what are the symptoms of folate deficient anemia

A

general anemia + angular stomatitis and glossitis

373
Q

what is leukemia

A

neoplastic proliferation of a white blood cell line resulting in a decreased production of other haematopoetic cells

374
Q

what are the general symptoms of leukemia

A

BM failure
- bone pain
- bleeding
- infections
- anaemia symptoms
- TATT

375
Q

what is the common chromosomal mutation in acute myeloid leukaemia

A

translocation (15:17)

376
Q

what is the common chromosomal mutation in chronic myeloid leukaemia

A

translocation (9:22)
- BCR-ABL

377
Q

what does the 9:22 translocation in CML cause

A

tyrosine kinase to be irreversibly switched on
- increases cell proliferation

378
Q

what is the most common genetic abnormality in ALL

A

translocation (12:21)

379
Q

what cells most commonly cause ALL

380
Q

what is a complication of CLL

A

Richter transformation: B cells massively accumulate in the lymph nodes causing lymphadenopathy (aggressive lymphoma)

381
Q

How do you diagnose polycythemia vera

A

Full blood count = increased which blood cells, platelets and red blood cells. Increased haemoglobin
Genetic tests: JAK 2 mutation positive

382
Q

what can cause arterial thrombosis

A

atherosclerosis

383
Q

what are the symptoms of an arterial blockage

A

the organs or tissues become cyanotic and cold

384
Q

what can an arterial thrombosis lead to

A

coronary vascular disease
cerebrovascular disease
peripheral vascular disease

385
Q

what is the symptoms of a venous thrombosis

A

the affected area is red and warm

386
Q

what causes venous thrombosis

A

remember virchaus triad
- change in coagulation
- change in vessel wall
- change in flow

387
Q

what can a venous thrombosis lead to

A

DVT
pulmonary embolism

388
Q

how do you diagnose Von Willebrands disease

A

normal prothrombin time and an increased APTT. there will be a reduction in vWF

389
Q

How do you treat vWF disease

A

use desmopressin to increase the release of vWF from endothelial weibelpalade bodies

390
Q

what are symptoms of immune thrombotic purpura

A

purpuric rash

391
Q

what is seen in bloods when diagnosing immune thrombotic purpura

A

thrombocytopenia and increased BM megakaryoblasts

392
Q

what is the 1st line treatment of immune thrombocytic purpura

A

prednisolone

393
Q

what are the 4 types of malaria to be aware of

A

plasmodium faliparum
plasmodium ovale
plasmodium vivax
plasmodium malariae

394
Q

what is the pathophysiology of malaria

A

sporozoites in mosquito saliva go into human host
multiply inside hepatocytes as merozoites
then go into RBC and become trophozoites, then schizont and then merozoites
RBC rupture and cause infection

395
Q

what are symptoms of malaria

A

FEVER AND EXOTIC TRAVEL
anemia
blackwater fever
hepatosplenomegaly

396
Q

how do you treat malaria

A

quinine and doxycycline

397
Q

what cancers is EBV closely related to

A

Hodgkins
Burketts
Nasopharyngeal carcinoma

398
Q

what is the pathophysiology of HIV

A

HIV gp120 binds to CD4 on Th cells
endocytosis and release contents
RNA to DNA via reverse transcriptase
integrase allows viral DNA to integrate with host DNA
protein synthesis
virus forms and exocytosis

399
Q

what are symptoms of HIV

A

fever
diarrhoea
night sweats
minor opportunistic infections

400
Q

what are the defining conditions of AIDS

A

CMV - colitis
Pneumocystis jirovecci pneumonia
cryptosporidium (fungal) infection
TB
Karposi sarcoma
toxiplasmosis
lymphomas

401
Q

what does it mean if lactate dehydrogenase is high on a blood test

A

that there is an increased cell turnover

402
Q

what is the coombs test used for

A

to differentiate between autoimmune and non autoimmune causes of haemolytic anaemia

403
Q

what is seen in bloods which is specific for AML

404
Q

what is a diagnostic feature of CML

A

it has the Philadelphia chromosome

405
Q

what is seen on a blood smear in CLL

A

SMUDGE CELLS

406
Q

what is a complication of CLL

A

it can transform into lymphoma - the Richter reaction

407
Q

what is ferritin on an iron study

A

iron storage

408
Q

what is transferrin

A

the protein which facilitates iron absorption

409
Q

what is total iron binding capacity

A

the affinity for iron to bind to protein

Phase 2a - Jemima (11 decks)

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