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2a Bateman - Phase 2a MASTER DECK > Haematology > Flashcards

Haematology Flashcards

1
Q

WHAT IS MULTIPLE MYELOMA?

https: //www.youtube.com/watch?v=jdytgW5wKa4
https: //www.youtube.com/watch?v=ghvoKhpAc64&t=471s

A

Cancer of plasma cells

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2
Q

What is the epidemology of myeloma?

A

Older age

Afro-Caribbeans more

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3
Q

What is the pathophysiology of multiple myeloma?

What is there an abnormal proliferation of?

What is secreted?

A

Abnormal proliferation of a single clone of plasma cells

Secretion of immunoglobulin (Ig) or an Ig fragment

Dysfunction of many organs (esp kidney).

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4
Q

What are the two proteins produced as a result of myeloma?

A

Paraprotein (just the light chain of Ig)

Bence Jones protein in the urine

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5
Q

How do osteoblasts and osteoclasts work?

What else does an osteoblasts do?

A

Osteoblasts secrete osteoid

Osteoclasts break down bone by HCl

Osteoblasts regulate osteoclasts by RANKL
When RANKL binds to RANK on osteoclasts it makes them work
Osteoblasts also secrete OPG which inhibit this

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6
Q

How do the amount of osteoclasts in multiple myeloma increase?

A

Bone marrow stromal cells which normally regulate haemotopoesis

Theese bone marrow stromal cells interact with multiple myeloma cells
Which activates cytokine mediated cell growth, durg resistance and migration

Secrete IL-3 stopping osteoblast production
DKK1 inhibit OPG
MIP1alpha and RANKL to increase osteoclast activity

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7
Q

What parts of B cell production damages what?

A

Recurrent infection
Monoclonal Igs

Renal impairment
Free Light Chains

Bone lesions
(IL-6) & ↑ Ca

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8
Q

What is the difference between MGUS, symptomatic and asymptomatic myeloma?

A

Monoclonal gammopathy of undetermined significance (MGUS)
Low amount of plasma cells in BM, no ROTI (related organ or tissue involvement), no evidence of amyloid or other LPD (lymphoproliferative disorder)

Asymptomatic myeloma (aMM)
>10% Moderate amount of plasma cells in BM, no ROTI

Symptomatic myeloma (sMM)
Paraprotein, >10% clonal plasma cells in BM or Bx proven plasmacytomata, ROTI nb CRAB

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9
Q

What are the symptoms for myeloma?

A

Remember babs the CRAB! Calcium, Renal, Anaemia, Bones

Hypercalcaemia

Renal impairment

Anaemia, neutropenia, or thrombocytopenia
Infiltration

Osteolytic bone lesions
Causing backache, fractures and vertebral collapse.

Recurrent bacterial infections
Immunoparesis, and neutropenia due to the disease and from chemotherapy.

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10
Q

What tests can you do for myeloma?

(Thick about the symptoms)

A

FBC (Full blood count)
Result in normocytic normochromic anaemia

ESR or PV (Erythrocyte sedimentary rate or plasma viscosity)
Both would be increased

U&E, Ca, albumin
All increased

Serum and urinary EP
Peak in IgG, M spike

XR of suspect areas
Lytic ‘punched-out’ lesions
Pepper-pot skull

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11
Q

What are the diagnostic features of myeloma?

A

Monoclonal protein band

Plasma cells increase

Evidence of end-organ damage from myeloma

Bone lesions

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12
Q

What Ig’s does myeloma normally produce?

A

IgG (2/3), IgA (1/3)

Rarely IgD, IgM or IgE

Monoclonal free light chains (Bence Jones proteins)

FLC only (15%)

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13
Q

What are the complications of myeloma?

A

Hypercalcaemia

AKI

Hyperviscosity

Cord compression

Amyloid

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14
Q

What is the treatment for myeloma?

A

C – Ca – hydration +/- bisphosphonates

R – Renal – hydration +/- dialysis

A – Anaemia– transfusion +/- EPO
Neutropenia - antibiotics

B – Bones – analgesia + bisphosphonates

Chemo +/- BMT

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15
Q

WHAT IS LYMPHOMA?

https: //www.youtube.com/watch?v=_QVO75CihYQ
https: //www.youtube.com/watch?v=FfuP7j4A1cs

A

Basically a malignant growth of white blood cells

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16
Q

Where does the growth of white blood cells in lymphoma normally happen?

A

Predominantly in lymph nodes

But also:
Blood, bone marrow
Liver, spleen
Anywhere.

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17
Q

What is the primary lymphoid organ?

A

Bone marrow

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18
Q

What is the secondary lymhpoid organ?

A

Lymph nodes

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19
Q

What happens in the bone marrow and lymph nodes (B cells)?

A

Precursor B cells turn into naive B cells

Then travel through the blood to the lymph nodes

Go to the cortex inside the lymph nodes

B cells can then divide into plasma cells which are found in the medulla (centre of lymph node)

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20
Q

What happens when a B cells antibody meets a particular antigen?

A

Can divide directly into plasma cells and secrete IgM

Go to centre of a primary follicle in the lymph node and differentiate into centroblasts and divide

This form a germinal centre, these have a rearrangement of their Ig genes and switch from forming IgM to IgG or IgA

The centrocyte in the middle of the centroblast can divide into plasma cells which go to the medulla or memory B cells which go to blood or MALT (mucosa assocaited lymphoid tissue)

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21
Q

What happens after the bone marrow to the thymus (T cells)?

A

Precursor T cells travel to thymus where they go into either

CD4 (helper)
OR
CD8 (cytotoxic)

These then circulate in blood and live in paracortex or lymph nodes

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22
Q

What are the two different types of lymphoma?

A

Hodgkin’s

NHL.

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23
Q

WHAT IS HODGKINS LYMPHOMA?

A

Malignancy of mature lymphocytes that arises in the lymphatic system

In Hodgkin’s lymphoma, characteristic cells with mirror-image nuclei are found, called Reed–Sternberg cells

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24
Q

What is the epidemology of HL?

A

BIMODAL

Young adults and elderly.

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25
Q

What are the risk factors for Hodgkin’s lymphoma?

A

EBV

SLE

Post-transplantation

Obesity

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26
Q

How does Hodgkins lymphoma present?

A

Enlarged lymph nodes

Hepatosplenomegaly

Sweats, weight loss

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27
Q

What can make lymph node pain worse?

A

Alcohol

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28
Q

How do you diagnose Hodgkins lymphoma?

A

Biopsy
Reed-Sternberg cells

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29
Q

What is the staging system called for lymphoma?

A

Ann Arbor system

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30
Q

How many stages does Hodgkin’s lymphoma have?

A

Four.

A - asymptomatic

  • *B - symptomatic**
  • *I** Confined to single lymph node region.
  • *II** Involvement of two or more nodal areas on the same side of the diaphragm.
  • *III** Involvement of nodes on both sides of the diaphragm.
  • *IV** Spread beyond the lymph nodes, eg liver or bone marrow.
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31
Q

What is the treatment for Hodgkin’s lymphoma?

A

Chemo +/-RT

BMT for relapse

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32
Q

WHAT IS NHL?

A

This includes all lymphomas without Reed–Sternberg cells —a diverse group.

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33
Q

What are some examples of NHL?

A

Low grade e.g. Follicular Lymphoma

High grade e.g. Diffuse Large B Cell Lymphoma

Very high grade e.g. Burkitt’s Lymphoma

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34
Q

Which cell line are most NHL derived from?

Which is the most common?

A

B-cells

Diffuse large B-cell lymphoma (DLBCL) is commonest.

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35
Q

What are some causes of NHL?

A

H.Pylori

HIV

Toxins

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36
Q

What are the symptoms of NHL?

A

Same as Hodgkin’s

+ GI symptoms if small bowel lymphomas

Skin involvement in T-cell lymphomas

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37
Q

What are the tests for NHL?

A

Blood
FBC, U&E, LFT

Marrow and node biopsy
Classification.

Cytology
LP for CSF cytology if CNS signs.

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38
Q

What is an example indolent or low grade lymphoma? What are its characteristics?

A

Follicular Lymphoma

Slow growing

Usually advanced at presentation

Incurable

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39
Q

What is an example high grade or aggressive NHL? What are its characteristics?

A

Diffuse Large B Cell Lymphoma

Usually nodal presentation

1/3 cases have extranodal involvement

Patient usually unwell

Often short history.

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40
Q

What is the treatment for NHL?

A

Low grade (follicular)
W&W

High grade (diffuse large B cell)
Chemo
BMT for relapse

Monoclonal antibodies
Rituximab

Steroids
Prednisolone

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41
Q

What is an example of a monoclonal antibody for lymphoma treatment?

A

Rituximab.

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42
Q

What does Rituximab do?

A

Monoclonal antibody

Anti CD-20

Targets CD20 expressed on cell surface of B-cells

Chimeric mouse/human protein

Minimal side-effects.

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43
Q

What is an example of a T cell engaging therapy?

A

Blinatumomab

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44
Q

What does Blinatumomab do?

A

bi-specific Antibody

Targets CD19 on B cells

And CD3 on T cells

Directs own immune system.

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45
Q

WHAT IS LEUKAEMIA?

https: //www.youtube.com/watch?v=itkRVTqfPsE
https: //www.youtube.com/watch?v=itkRVTqfPsE

A

Malignant proliferation of haemopoietic cells.

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46
Q

What are some different types of leukaemia?

https://www.youtube.com/watch?v=itkRVTqfPsE

A

Acute Myeloid leukaemia (AML)

Chronic Myeloid Leukaemia (CML)

Acute Lymphoblastic Leukaemia (ALL)

Chronic Lymphocytic Leukaemia (CLL).

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47
Q

What is acute myeloid leukaemia a disease of?

A

Myeloblasts.

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48
Q

What is chronic myeloid leukaemia a disease of?

A

Basophils, Neutrophils and Eosinophils.

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49
Q

What is acute lymphoblastic leukaemia a disease of?

A

Lymphoblast.

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50
Q

What is chronic lymphocytic leukaemia a disease of?

A

B lymphocytes.

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51
Q

What is the stain called which allows you to distinguish between myeloblasts and lymphoblasts?

A

Sudan black stain

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52
Q

WHAT IS ACUTE MYELOID LEUKAEMIA?

https://www.youtube.com/watch?v=itkRVTqfPsE

A

Clonal expansion of myeloblasts in BM, blood and other tissues

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53
Q

Above what percentage of blasts cells does there have to be for AML?

A

More than 20% blasts cells in bone marrow

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54
Q

What is the epidemology of acute myeloid leukaemia?

A

The commonest acute leukaemia of adults

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55
Q

What is the aetiology of acute myeloid leukaemia?

(Who is at increased risk)

A

Aetiology usually not obvious but risk increased in:

Preceeding Haematological disorders
Prior chemotherapy
Exposure to ionising radiation

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56
Q

What are the symptoms of acute myeloid leukaemia?

A

Marrow failure
Symptoms of anaemia, infection or bleeding.

Infiltration
Hepatomegaly and splenomegaly
GUM HYPERTROPHY + BLEEDING

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57
Q

How can you diagnose acute myeloid leukaemia?

A

FBC
↓RBC
↓PLT
WCC variable
Usually w/ neutropenia

Bone marrow biopsy
Blast cells may be few in the peripheral blood

Clotting screen
DIC may occur

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58
Q

What is diagnositc of AML from ALL?

A

Auer rods are diagnostic of AML

Myeloperoxidase makes this

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59
Q

What is the treatment for acute myeloid leukaemia?

A

Supportive

Treat Infection

Chemotherapy
(remission induction (regenerate) ➔ consolidation(intensification) ➔ maintenance: steroids

Bone Marrow Transplantation
During 1st remission

(blood/platelets/fluids)

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60
Q

What drug can be used to prevent tumour lysis syndrome?

Where else is this used?

A

Allopurinol

Gout

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61
Q

What is acute premylocitic leukaemia?

A

Build up of premyelocytes from a problem with the retinoic acid receptor

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62
Q

What is the cause of premyelocitic leukaemia?

What chromsome translocation?

A

Translocation between chromosome 15 and 17

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63
Q

What happens with premyelocitic leukaemia?

What do you get with all the premyelocytes?

What does this cause?

A

Build up of premyelocytes

Lots of auer rods

Increase coagualation risk

Medical emergency

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64
Q

What is the treatment of premyelocitic leukaemia?

A

All trans retionic acid ATRA

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65
Q

WHAT IS CHRONIC MYELOID LEUKAEMIA?

https://www.youtube.com/watch?v=Wn3fylOqUZU&t=6s

A

CML is characterized by an uncontrolled clonal proliferation of myeloid cells

Only partialy differentiate

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66
Q

What happens with the cells in CML?

Where do they go to?

A

Divide too quickly

Spill out into blood and crowd it

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67
Q

Where do the myeloid cells go to through the blood?

A

Liver and spleen

Cause hepatosplenomegaly

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68
Q

What is the epidemology of chronic myeloid leukaemia?

A

Usually 40-60yrs age

Rare in childhood

Slight male dominance

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69
Q

What is the key diagnostic feature of chronic myeloid leukaemia?

A

Philadelphia Chromosome t(9;22)

80%

BCR/ABL on 22

Causes an activated tyrosine kinase

Worse prognosis if absent

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70
Q

What are the symptoms and signs of chronic myeloid leukaemia?

A

Symptoms

Weight loss
Tiredness
Fever
Sweats
Bleeding (platelet dysfunction)
Abdominal discomfort (splenic enlargement).

Signs

Splenomegaly (>75%)—often massive.
Hepatomegaly, anaemia, bruising

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71
Q

What are the investigations for chronic myeloid leukaemia?

A

FBC
Raised WCC – all myeloid cells raised – neutrophils, macrophages, basophils, eosinophils).

Hb low or normal

Platlets variable

Cytogenetics (karyotype, FISH or PCR)
Philadelphia Chromosome

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72
Q

What is the treatment for chronic myeloid leukaemia?

A

Tyrosine kinase inhibitors
1st line Imatinib (Glivec)

2nd line Nilotinib, Dasatinib, Ponatinib.

Chemo

Stem cell transplant

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73
Q

WHAT IS ACUTE LYMPHOBLASTIC LEUKAEMIA?

https://www.youtube.com/watch?v=itkRVTqfPsE

A

Malignancy of B/T lymphocyte cell lines

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74
Q

What is the epidemology of acute lymphoblastic leukaemia?

A

Most common paediatric malignancy

Rarer in adults

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75
Q

What is thought to cause acute lymphoid leukaemia?

A

Genetic susceptibility
Translocations

+ an environmental trigger

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76
Q

What is the classification of acute lymphoid leukaemia?

A

Morphological
The FAB system divides ALL into 3 types (L1, L2, L3) by microscopic appearance.

Immunological
Surface markers are used to classify ALL into: • Precursor B-cell ALL • T-cell ALL • B-cell ALL.

Cytogenetic
Chromosomal analysis. Abnormalities are detected in up to 85%, which are often translocations.

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77
Q

What are the signs and symptoms for acute lymphoid leukaemia?

A

CNS involvement (cranial nerve palsies and meningism)

Lymphadenopathy

Orchidomegaly

+ Other leukaemia classics

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78
Q

What is the diagnosis for acute lymphoblastic leukaemia?

A

FBC
↓RBC, ↓PLT, WCC variable, usually w/ neutropenia

Blood film
Blasts cells unless confined to bone marrow (+ bone marrow aspiration)

Clotting screen
DIC may occur

Lumbar puncture
CNS involvement

Cytogenetics

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79
Q

How do you treat acute lymphoblastic leukaemia?

A

Supportive

Treat infection

Chemotherapy
(remission induction (regenerate) ➔ consolidation(intensification) ➔ maintenance: steroids

Bone Marrow Transplantation
(during 1st remission)

(blood/platelets/fluids)

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80
Q

WHAT IS CHRONIC LYMPHOBLASTIC LEUKAEMIA?

https://www.youtube.com/watch?v=Wn3fylOqUZU&t=6s

A

Accumulation of mature B cells that have escaped programmed cell death

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81
Q

What happens to the cells in CLL?

A

Don’t divide quickly enough

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82
Q

What is the epidemology of chronic lymphoblastic leukaemia?

A

Most common leukaemia

Generally elderly but 20% <55yrs

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83
Q

What are the signs and symptoms for chronic lymphoid leukaemia?

A

Symptoms
Often none, surprise finding on a routine FBC.
May be anaemic or infection-prone.
If severe: weight loss, sweats, anorexia.

Signs
Enlarged, rubbery, non-tender nodes.
Splenomegaly, hepatomegaly.

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84
Q

What are the different stages of chronic lymphocytic leukaemia?

A

Rai Staging

1-5 based on examination and FBC

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85
Q

What are the tests for chronic lymphoid leukaemia?

A

Increased WBCs

Film
Smudge cells
B cell that have been broken

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86
Q

What are the complications for chronic lymphoid leuklaemia?

A

Autoimmune haemolysis.

Increased Infection due to hypogammaglobulinaemia

Marrow failure.

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87
Q

What is the treatment for chronic lymphocytic leukaemia?

A

Rituximab

Chemotherapy

Bone marrow transplant + Radio

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88
Q

WHAT ARE SOME EXAMPLES OF DIFFERENT HAEMOGLOBINOPATHIES?

A

Disorders of quality (abnormal molecule or variant haemoglobins)
Sickle cell disease

Disorders of quantity (reduced production)
a or b thalassaemia

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89
Q

What does normal haemoglobin contain and foetal?

A

Normal Hb 2xa, 2xb

Foetal Hb 2xa, 2xg

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90
Q

What does haemoglobin change from to during development?

A

Haemoglobin F to haemoglobin A.

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91
Q

WHAT IS SICKLE CELL ANAEMIA?

What type of anaemia is it?

https://www.youtube.com/watch?v=1ql-X60CUNQ&t=6s

A

Sickle-cell anaemia is an autosomal recessive disorder causing production of abnormal beta globin chains.

Microcytic anaemia

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92
Q

What is haemoglobin S?

A

Variant haemoglobin arising because of a point mutation in the b globin gene.

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93
Q

What happens with a carrier of sickle cell disease?

A

Carriers of HbS are symptom free

Carriage offers protection against falciparum malaria

Sickle cell diseases arise in the homozygous state (SS) or in combined heterozygotes (SC or Sb thalassaemia).

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94
Q

What gene changes occur in sickle cell anaemia?

A

Thiamine for adenine in 6th codon

Beta globin gene

Glutamic acid leaves

Valine comes in

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95
Q

What is the pathology of sickle cell disease?

What happens to the red blood cell?

A

HbS polymerizes when deoxygenated

RBCs deform, producing sickle cells

Which are fragile and haemolyse, and also block small vessels

Sickling cells appear at acidosis at at time when they deposit lots of oxygen

96
Q

What are the symptoms of sickle cell disease?

Think about the crises’!

A

Fatigue and Anaemia

Pain Crises

Dactylitis and Arthritis

Bacterial Infections

Leg Ulcers

Aseptic Necrosis and Bone Infarcts

97
Q

What are the different crises for sickle cell disease?

A

Haemolytic

Vaso-occlusive ‘painful’ crisis

Aplastic crisis (sudden reduction in marrow production)

Sequestration crisis (There is pooling of blood in the spleen ± liver)

98
Q

What are the tests for sickle cell disease?

A

Screen neonate
blood/heel prick test

FBC
Low Hb, high reticulocyte count

Blood film
SICKLED erythrocytes

Hb ELECTROPHARESIS
For dx
HbSS present and absent HbA

99
Q

What are some common complications of sickle cell disease?

A

Acute complications
Painful crisis
Sickle chest syndrome
Stroke

Chronic complications
Renal impairment
Pulmonary hypertension
Joint damage

100
Q

What are the treatments for sickle cell anaemia?

A

Prophylactic ABX
Daily penicillin.
Pneumococcal & influenza vaccine
Folic acid

Pain relief for crisis
NSAIDs/paracetamol

Hydroxyurea
hydroxycarbamide
Increase conc. of HbF

101
Q

WHAT IS THALASSAEMIA?

https://www.youtube.com/watch?v=uK_uIBHnOWo&t=4s

A

The thalassaemias are genetic diseases of unbalanced Hb synthesis, with under-production (or no production) of one globin chain

102
Q

What is the pathology of thalassaemia?

What happens to the red blood cells?

A

Unmatched globins precipitate

Damaging RBC membranes

Causing their haemolysis while still in the marrow.

103
Q

What are the different types of Thalassaemia?

What different treatments do they require?

A

Thalassaemia Major
Transfusion dependent

Thalassaemia Intermedia
Less severe anaemia and can survive without regular blood transfusions

Thalassaemia Carrier/heterozygote
Asymptomatic.

104
Q

What type of anaemia is thalassaemia?

A

Microcytic

105
Q

WHAT IS BETA THALASSAEMIA?

What do you get less of?

What happens as a result?

A

In homozygous beta-thalassaemia, little/no normal beta chain production

EXCESS alpha chains.

Alpha chains combine w. whatever beta, delta or gamma chains available increased production of HbA2& HbF.

106
Q

What is the cause of beta thalassemia?

Which chromosome is it defected in?

A

Point mutations in beta-globin genes

Chromosome 11

Decreased beta-chain production (beta+) or absence (beta0)

107
Q

What is beta thalassemia major called?

A

Beta thalassaemia major (Cooley’s anaemia) denotes abnormalities in both beta-globin genes.

108
Q

When does beta thalassaemia major present?

A

6 -12 months

109
Q

What are the symptoms of beta thalassaemia major?

A

Failure to feed, listless, crying, pale.

Bones widen due to increased activity
Skull bossing

Anaemia

Hepatosplenomegaly
Lots of RBCs need to be destroyed

110
Q

What is beta thalassaemia minor or trait?

A

This is a carrier state

111
Q

What are the symptoms of beta thalassaemia minor?

A

Usually asymptomatic

Mild, well-tolerated anaemia (Hb >90g/L)

112
Q

What are the symptoms of beta thalassaemia intermedia?

A

Moderate anaemia but not requiring transfusions

There may be splenomegaly

113
Q

What are the tests for Beta thalassaemia?

A

FBC & film
Hypochromic & microcytic anaemia
Irregular and pale RBCs.
Increased reticulocytes and nucleated RBCs

Dx by Hb electrophoresis
shows increased HbF & absent/low HbA.

114
Q

What is the treatment for beta thalassaemia major?

A

BLOOD TRANSFUSIONS

Give iron chelation
Desferrioxamine
Decrease iron loading

Ascorbic acid
Increased urinary excretion of iron.

Long term folic acid supplements.

115
Q

What happens when a child with Thalassaemia major has treatment by continuous blood transfusions?

A

Children require lifelong blood transfusions.

Consequence is an progressive increase in body iron load.

Can’t eliminate the excessive iron

Patients inexorably develop a clinically worsening hemosiderosis

Liver and spleen, leading to liver fibrosis and cirrhosis.

Endocrine glands and the heart, resulting in diabetes, heart failure and premature death.

Death ultimately occurs, mainly due to cardiac hemosiderosis.

116
Q

What does degree of symptoms depends of in alpha Thalassaemia?

A

4 deletions
HbBarts, infants are STILLBORN(hydrops fetalis).

3 deletions
SEVERE anaemia.

2 deletions
Often ASYMP. carrier, may have mild anaemia.

1 deletion
Close to normal.

117
Q

WHAT ARE MEMBRANOPATHIES?

A

Autosomal dominant conditions.

Deficiency of red cell membrane proteins caused by a variety of genetic lesions.

118
Q

What are some examples of membranopathies?

A

Spherocytosis & elliptocytosis most common.

Neonatal jaundice.

119
Q

WHAT IS HAEMOLYTIC ANAEMIA?

https://www.youtube.com/watch?v=9DoUcEEthv8

A

Anemia due to hemolysis

Either in the blood vessels (intravascular hemolysis) or

elsewhere in the human body (extravascular)

120
Q

What are the causes of haemolytic anaemia?

A

Genetic
Red cell membrane abnormalities:
Hereditary spherocytosis, elliptocytosis

Haemoglobin abnormalities
Sickle cell anaemia, thalassaemia

Enzyme defects
G6PD deficiency, pyruvate kinase deficien

Acquired
Systemic lupus erythematosus (SLE)
Lymphoma
Chronic lymphatic leukaemia (CLL)

121
Q

What is Elliptocytosis? What causes this?

A

Where the red blood cells are shaped elliptically.

Horizontal interactions.

122
Q

What is Spherocytosis? What causes this?

A

Where the red blood cells are shaped spherically. Vertical interactions.

123
Q

What is the pathology of haemolytic anaemia?

What does the RBC release?

A

RBC haemolyse because of different causes

Haemolgobin released.

Haptoglobin binds to haemoglobin to recycle it

124
Q

What are some symptoms of haemolytic anaemia?

A

Pallor

Jaunidce

Splenomegaly

125
Q

What are the tests for haemolytic anaemia?

A

↑Unconjugated bilirubin

↑LDH

Coombs test +ve

Reduced reticulocytes

126
Q

What is the management for haemolytic anaemia?

A

Transfusion

Folic acid

Discontinue medications

Iron

Autoimmune haemolytic anaemia therapy

127
Q

WHAT ARE ENZYMOPATHIES?

https://www.youtube.com/watch?v=DZS-diLCa1g

A

Inherited enzyme deficiencies leading to shortened red cell lifespan from oxidative damage.

128
Q

What is the pathway for G6PD?

A

Pentose phosphate pathway

129
Q

What do the enzymes do in the cell?

A

Provides the fuel for the red cell
NAD+ to NAPH

Generates redox capacity to protect red cell
Gluthathione

130
Q

What does glucose 6 phosphate dehydrogenase deficiency have to do with haemolytic anaemia?

A

Common cause of haemolytic anaemia

131
Q

How is Glucose 6 phosphate dehydrogenase deficiency caused?

A

Caused by a wide variety of mutations within G6PD gene

X linked but women may also be affected

132
Q

What are the symptoms of Glucose 6 phosphate dehydrogenase deficiency?

A

Most asymptomatic

But may get oxidative crises due to decreased glutathione production, precipitated by drugs

In attacks, there is rapid anaemia and jaundice

133
Q

How is Glucose 6 phosphate dehydrogenase deficiency diagnosed?

A

Diagnosed by screening test for NADPH

Film
Bite- and blister-cells

134
Q

What is the treatment for glucose 6 phosphate dehydrogenase deficiency?

A

Avoid precipitants (eg, henna); transfuse if severe.

135
Q

WHAT ARE PLATELETS AND HOW ARE THEY FORMED?

A

Anucleate cells formed by fragmentation of megakaryocyte (MK) cytoplasm in bone marrow.

136
Q

What is important about platelets shape? What is their primary function?

A

Disc shape allows them to flow close to endothelium Important role in primary haemostasis

137
Q

How are old platelets recycled and after how long?

A

Life span 7-10 days

Old platelets are phagocytosed by splenic macrophages in red pulp.

138
Q

What does thrombopoietin do?

A

Stimulates production of platelets by megakaryocytes

Binds to platelet and MK receptors

↓plts = less bound TPO = ↑ free TPO able to bind to MK = ↑ Plt prodn.

139
Q

Where is thrombopoietin produced?

A

Liver.

140
Q

What happens after damage to the endothelium is done?

A

1) Platelets adhere to vascular endothelium via collagen & vWF (von Willebrand factor)
2) Binding of platelets to collagen stimulates shape change
3) Activation, leading to the release of platelet granule contents including ADP, fibrinogen, thrombin and calcium. Ends with production of fibrin
4) Aggregation of platelets then occurs, cross-linking by fibrin
5) Activated platelets also provide a negatively charged phospholipid surface, which allows coagulation factors to bind

141
Q

What do platelet granules contain? What is the mechanism of the clotting cascade?

A

Contents that help the formation of fibrin, and provide a surface for clotting factors Va and Xa to function better.

142
Q

What does Clopidogrel inhibit?

A

Platelets

P2Y12.

143
Q

What does Tirofiban inhibit?

A

Platelets

Bp IIbIIIa.

144
Q

What does Aspirin inhibit?

A

COX-1.

145
Q

What is COX-1 needed to make?

A

Thromboxane A2 from prostaglandin H2.

146
Q

What is needed to make Arachidonic acid?

A

P2Y12 and Gp IIbIIIa.

147
Q

What is the platelet dysfunction: clinical features?

A

Mucosal bleeding
Epistaxis, gum bleeding, menorrhagia

Easy bruising

Petechiae
Red or purple spot on the skin, caused by a minor bleed from broken capillary blood vessels

Purpura
Red or purple discolored spots on the skin that do not blanch on applying pressure

Traumatic haematomas
(inc subdural).

148
Q

What are some causes of platelet dysfunction? (TWO)

A

Reduced platelet number (thrombocytopenia).
Decrease in production.
Increase in destruction.

Normal numbers but reduced function.
Congenital abnormality in platelet function
Medication e.g aspirin
Von Willebrand disease (reduced VWF activity)
Uraemia

149
Q

What is thrombocytopenia?

A

Decreased platelet production.

150
Q

What are the different types of thrombocytopenia?

A

Congenital thrombocytopenia
Absent / reduced / malfunctioning megakaryocytes in BM

Infiltration of bone marrow
Leukaemia, metastatic malignancy, lymphoma, myeloma, myelofibrosis.

151
Q

What can cause a decreased production of platelets?

A

Reduced platelet production by bone marrow
Low B12 / folate
Reduced TPO (e.g. liver disease)
Medication: Methotrexate, chemotherapy
Toxins: e.g. Alcohol
Infections: e.g. viral (e.g. HIV) TB
Aplastic anaemia (auto immune)

Dysfunctional production of platelets in BM
Myelodysplasia.

152
Q

What are some causes of increased destruction of platelets?

A

Autoimmune
Immune thrombocytopenia (ITP)
Primary, or secondary

Hypersplenism
Portal hypertension, splenomegaly

Drug related immune destruction
E.g. Heparin induced thrombocytopenia.

153
Q

What are some diseases which increase destruction of platelets?

A

Disseminated intravascular coagulopathy (DIC)

Thrombotic thrombocytopenic purpura (TTP)

Haemolytic uraemic syndrome (HUS)

Haemolysis, elevated liver enzymes and low platelets (HELLP)

Major haemorrhage

154
Q

WHAT IS IMMUNE THROMBOCYTOPENIA?

A

IgG antibodies form to platelet and megakaryocyte surface glycoproteins

Opsonized platelets are removed by reticuloendothelial system

155
Q

What is primary ITP?

A

May follow viral infection / immunisation esp in children

156
Q

What is secondary ITP?

A

Occurs in association with some

Malignancies, such as Chronic Lymphocytic Leukaemia(CLL)

Infections e.g. HIV / HepC

157
Q

What is chronic ITP?

A

Chronic ITP runs a fluctuating course of bleeding, purpura, epistaxis and menorrhagia.

There is no splenomegaly.

158
Q

What are the symptoms for ITP?

A

Easy bruising

Purpura

Epistaxis (nose bleed)

Menorrhagia (heavy bleed at period)

159
Q

What are the tests for ITP?

A

Increased megakaryocytes in marrow

Antiplatelet autoantibodies often present

160
Q

How do you treat immune thrombocytopenia?

A

Corticosteroids i.e. prednisolone

Splenectomy

161
Q

WHAT IS DISSEMINATED INTRAVASCULAR CONGESTION?

https://www.youtube.com/watch?v=Gmh01S0msfY

A

Small blood clots develop throughout the bloodstream

Blocking small blood vessels maybe causing organ ischemia

Also depleting platelet supply leading to bleeding

162
Q

What is the cause of DIC?

A

Sepsis

Malginancy

Obstetric complications

Intravascular haemolysis

163
Q

What is DIC also known as and why?

A

Consumption coagulopathy, consumes platelets and clotting factors

164
Q

What is the normal response to injury?

A

Primary haemostasis
Vasocontriction

Platelets then adhere to the damaged endothelial wall

These then activate other platelets and form a plug

Coagulation cascade

One coagulation factor gets proteolytically cleaved which cleaves other coagulation factors

This then cleaves fibrinogen into fibin which forms a mesh

165
Q

What happens after a clot is formed?

A

Fibrinolysis

This stops the clot from becoming too big.

166
Q

What normally favours clotting?

A

Sepsis, malignancy, trauma, obstetrics complications.

These favour procoagulants
Tissue factor
Lipopolysaccharide.
Enzymes that cleave clotting factors.

167
Q

What is the pathology of DIC?

A

Underlying malignancy favours clotting
Widespread clot formation leading to
Ischaemia, necrosis and organ damage
Kidneys, Liver, Lungs and Brain.

Depletes clotting factors and platelets

Fibrin degradation products in circulation
Interferes with clot formation

168
Q

What are the symptoms of DIC?

A

Bleeding

Internal bleeding

Bruising

Ischaemia

169
Q

What are the investigations for DIC?

A

FBC
Thrombocytopenia
Low fibrinogen
Prolonged prothrombin time (PT)
Prolonged partial thrombopastin time (PTT)
High d-dimers - Fibrin degradation product

Chronic DIC
Could look normal due to compensation

170
Q

What does a prolonged prothrombin and prolonged partial thromboplastin time represent?

A

LOW circulating coagulation factors

171
Q

How do you treat DIC?

A

Platelets

FFP: contains clotting factors

Cryoprecipitate: contains fibrinogen and some clotting factors

Underlying cause

172
Q

WHAT IS THROMBOTIC THROMBOCYTOPENIC PRUPURA?

A

In TTP, blood clots form in small blood vessels throughout the body

173
Q

What is the pathophysiology of TTP (Thrombotic thrombocytopenic purpura)?

What is there a defect in?

A

There is a genetic or acquired deficiency of a protease (ADAMTS13) that normally cleaves multimers of von Willebrand factor (VWF).

Large VWF multimers form, causing platelet aggregation and fibrin deposition in small vessels, leading to microthrombi.

174
Q

What are the causes of TTP?

A

Idiopathic (40%)

Autoimmunity (eg SLE), cancer, pregnancy, drug associated (eg quinine), bloody diarrhoea prodrome (as childhood HUS), haematopoietic stem cell transplant.

175
Q

What are the tests for TTP?

A

Blood and protein in urine
Haematuria/proteinuria

Blood film
Fragmented RBC
Decrease platelets
Decrease Hb

Clotting tests are normal

176
Q

What is the treatment for TTP?

A

Plasma exchange

Steroids

177
Q

WHAT ARE SOME CAUSES OF OVER-ANTICOAGULATION?

A

Warfarin and Heparin

178
Q

What is HTP?

A

IgG antibody against platelet-heparin complex

IgG/plt/heparin complex causes pltactivation and THROMBOSIS

179
Q

When does HTP occur?

A

After Cardiac bypass surgery

180
Q

What are the symptoms of over-anticoagulation?

A

Bruising

Bleeding

Melena

Epistaxis

Hematemesis

Haemoptysis

181
Q

What is the treatment for HTP and warfarin problems?

A

Stop drug straight away

182
Q

WHAT IS ANAEMIA?

A

Reduced red cell mass +/- reduced haemoglobin concentration

183
Q

What are the different types of anaemia?

What are the MCVs for each?

A

Microcytic
<80

Normocytic
80-100

Macrocytic
>100

184
Q

What are the different microcytic anaemias?

A

Iron deficiency

Haemoglobinopathies e.g. Thalassaemia, sickle cell

Anaemia of chronic disease e.g. CKD and so lack of EPO

185
Q

What are the causes of normocytic anaemia?

A

Acute blood loss

Combined haematinic deficiency

Anaemia of chronic disease e.g. CKD and so lack of EPO

186
Q

What are the different causes of macrocytic anaemia?

A

B12/Folate deficiency

Alcohol excess/liver disease

Metabolic disease e.g. hypothyroidism

(Megaloblastic)

187
Q

What is the normal haemoglobin range?

A

Male Hb = 131 – 166 g/L

Female Hb = 110 – 147 g/L

188
Q

What are the physiological consequences of anaemia?

A

Reduced O2 transport

Tissue hypoxia

Compensatory changes

  • Increase tissue perfusion
  • Increase O2 transfer to tissues
  • Increase red cell production
189
Q

What are some pathological consequences of anaemia?

A

Myocardial fatty change

Fatty change in liver

Aggravate angina/claudication

Skin and nail atrophic changes

CNS cell death (Cortex and basal ganglia)

190
Q

Where are red blood cells removed?

A

Spleen. Liver. Bone marrow. Blood loss.

191
Q

How long do red blood cells last?

A

120 days.

192
Q

What are the different types of anaemias?

A

Microcytic.

Normocytic.

Macrocytic.

193
Q

What are the causes of microcytic anaemia?

A

Iron deficiency.

Chronic disease.

Thalassaemia.

Rarely
Congenital sideroblastic anaemia.
Lead poising.

194
Q

WHAT IS IRON-DEFICIENCY ANAEMIA?

A

Anaemia form a lack of iron

195
Q

What type of anaemia is iron-deficiency anaemia?

A

Microcytic

196
Q

What are the causes of iron-deficiency anaemia?

A

Blood loss
eg menorrhagia or GI bleeding

Poor diet
May cause IDA in babies or children (but rarely in adults), those on special diets, or wherever there is poverty.

Malabsorption
(eg coeliac disease) is a cause of refractory IDA.

Increased demand
Pregnant women and child/adolesence

197
Q

What are the signs of iron-deficiency anaemia?

A

Koilonychia
Spoon shaped nails

Atrophic glossitis
Painful tongue

Angular stomatitis
Corners of mouth inflammed

Brittle hair and nails

RARELY
Post-cricoid webs (Plummer-Vinson syndrome).

198
Q

How can you investigate iron deficiency?

A

FBC & film
MCV<80
Variation in size (anisocytosis) & shape (poikilocytosis) of cells.
Low serum iron & ferritin
Low reticulocytes (due to reduced Hb production)

High TIBC

Ix of GI tract

Coeliac serology

199
Q

What is the treatment of iron-deficiency anaemia?

A

Treat the cause.

Oral iron, eg ferrous sulfate

200
Q

What does an elevated ferritin show?

A

Inflammation.

201
Q

What is a megaloblast?

A

A megaloblast is a cell in which nuclear maturation is delayed compared with the cytoplasm.

This occurs with B12 and folate deficiency, as they are both required for DNA synthesis.

202
Q

WHAT IS FOLATE-DEFICIENCY ANAEMIA?

A

Deficiency in folate

203
Q

Where is folate found?

A

Green veg, liver, nuts and yeast.

204
Q

What are the causes of folate deficiency?

A

Dietary

Malabsorption

Increased req (e.g. pregnancy); Folate antagonists

205
Q

What are the tests for folate deficiency?

A

Low folate

Low Hb

Film
Hypersegmented polymorphs and Megaloblasts

206
Q

What is the treatment of folate-deficiency anaemia?

A

Folic acid supplements

With B12

207
Q

WHAT IS B12 DEFICIENCY?

A

Deficiency in B12

208
Q

What food is B12 in? How long do our body stores of B12 last?

A

Found in meat, fish & dairy products. NOT in plants!!

Body stores last 4yrs! (in liver)

209
Q

How is B12 abosrbed and what is produced in a deficiency?

A

Binds to intrinsic factor & absorbed in terminal ileum

Prod of DNA impaired w deficiency
Decreased RBC prod.

210
Q

What are the causes of B12 deficiency?

A

Dietary
Vegans

Malabsorption
Crohn’s
Coelaic
Gastritis

Congenital metabolic errors

211
Q

How can you investigate B12 deficiency?

A

Blood film:
Megoblasts and hypersegmented polymorphs

Bone marrow biopsy:

IF antibodies

Schilling test (medical procedure used to determine whether you’re absorbing vitamin B-12 properly)

212
Q

What is the treatment of B12 deficiency?

A

Vitmain B12 injections or tablets

213
Q

WHAT IS PERNICIOUS ANAEMIA?

A

This is caused by an autoimmune atrophic gastritis, leading to achlorhydria and lack of gastric intrinsic factor secretion.

214
Q

What is pernicious anaemia associated with?

A

Other autoimmune diseases: thyroid disease, vitiligo, Addison’s disease, hypoparathyroidism.

215
Q

What are the tests for pernicious anaemia?

A

Intrinsic factor (IF) antibodies

MCV increase

Serum B12 lower

Reticulocytes lower or normal as production impaired,

Hypersegmented polymorphs

Megaloblasts in the marrow

216
Q

What is the treatment of pernicious anaemia?

A

Treat the cause if possible.

If a low B12 is due to malabsorption, injections are required.

Replenish stores with hydroxocobalamin (B12)

If the cause is dietary, then oral B12 can be given after the initial acute course.

217
Q

WHAT DOES BONE MARROW DO?

A

The marrow is responsible for haemopoiesis. In adults, this normally takes place in the central skeleton (vertebrae, sternum, ribs, skull) and proximal long bones. In some anaemias (eg thalassaemia), increased demand induces haematopoiesis beyond the marrow (extramedullary haematopoiesis), in liver and spleen, causing organomegaly.

218
Q

What is pancytopenia?

A

Pancytopenia is reduction in all the major cell lines:

Red cells

White cells

Platelets

219
Q

WHAT IS APLASTIC ANAEMIA?

A

PANCYTOPENIA w. HYPOCELLULARITY (aplasia) of the bone marrow.

220
Q

What is the pathology of aplastic anaemia?

A

Decreased number of pluripotent stem cells.

BM replaced by fat.

221
Q

What are the causes of aplastic anaemia?

A

Mostly AI

Triggered by drugs

Irradiation

Fanconi anaemia (=inherited form)

Infections.

222
Q

What is the test for aplastic anaemia?

A

BM exam for dx
HYPOCELLULAR

FBC
Pancytopenia

Film

223
Q

What is the treatment for aplastic anaemia?

A

Withdrawal of offending agent, supportive care & some definitive tx:

Blood & platelet transfusions

Prophylactic ABX/prompttx of infections

<40yo (curative)
Bone marrow transplant

>40yo
immunosuppression w. anti-thymocyteglobulin & ciclosporin.

224
Q

WHAT ARE THE MYELOPROLIFERATIVE DISORDERS?

A

These are caused by proliferation of a clone of haematopoietic myeloid stem cells in the marrow.

While the cells proliferate, they also retain the ability to differentiate into RBCS, WBCS or platelets.

225
Q

What are the different myeloproliferative disorders?

A

RBC
Polycythaemia rubra vera (PRV)

WBC
Chronic myeloid leukaemia (CML, p352)

Platelets
Essential thrombocythaemia

Fibroblasts
Myelofibrosis

226
Q

What is the haematicrit?

A

Cells that sink to the bottom of a tube when centrifuged.

227
Q

WHAT IS POLYCYTHAEMIA RUBRA VERA?

https://www.youtube.com/watch?v=vOPuAPCioE4

A

Deficiency of the bone marrow, produces too many red blood cells.

Genetic disorder.

228
Q

What is the cause of PRV?

What is the gene in primary?

A

Primary
Polycythaemia Rubra Vera (PRV)
JAK2 gene - increased sensitivty to EPO

Reactive/secondary
EPO excess, altitude, lung disease

229
Q

How does PRV present like?

A

May present with no symptoms

May have

Easy bleeding/bruising
Fatigue
Dizziness
Headaches

230
Q

What are the investigations for PRV?

A

FBC

Bone marrow biopsy

Genetic testing for JAK2 gene

231
Q

How can you treat PRV?

A

Polycythaemia vera
Blood letting
Aspirin

Secondary
Treat the cause!

232
Q

WHAT IS MALARIA?

A

Biology Spread: fig 2. Plasmodium protozoa injected by 
Anopheles mosquitoes multiply in RBCS causing haemolysis, RBC sequestration and cytokine release.

233
Q

What is fever paroxysms?

A

Fever paroxysms reflect synchronous release of flocks mero-
zoites from mature schizonts (fig 2).

3 phases:

1 Shivering (1h): “I feel so cold.”

2 Hot stage (2–6h): T≈41°C, flushed, dry skin; nausea/vomiting; headache.

3 Sweats (~3h) as T° falls.95

234
Q

What are the signs of malaria?

A

Anaemia, jaundice, and hepatosplenomegaly. No rash or lymphadenopathy.

Anaemia is common, eg from haemolysis of parasitized RBCS (often serious in children). Thrombocytopenia.

235
Q

What is the diagnosis of malaria?

A

Serial thin & thick blood films (needs much skill, don’t always believe –ve reports, or reports based on thin film examination alone); if P. falciparum, you must know the level of parasitaemia. Rapid stick tests are available if microscopy cannot be performed or previously treated seriously ill patient: see p383 for ParaSight F®. Serology is not useful. Other tests: FBC (anaemia, thrombocytopenia), clotting (DIC, p346), glucose (hypoglycaemia), ABG/lactate (lactic acidosis), U&E (renal failure), urin- alysis (haemoglobinuria, proteinuria, casts), blood culture to rule out septicaemia.

236
Q

What is the treatment for malaria?

A

If the patient has taken prophylaxis, don’t use the same drug for treat- ment. If species unknown or mixed infection, treat as P. falciparum. Nearly all P. falciparum is resistant to chloroquine and in many areas also to Fansidar® (py- rimethamine + sulfadoxine). If in doubt, consider as resistant. Chloroquine103 is 1st choice for benign malarias in most parts of the world, but chloroquine-resistant P. vivax occurs in Papua New Guinea, Indonesia, parts of Brazil, Colombia and Guy- ana.104Neverrelyonchloroquineifusedaloneasprophylaxis.

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