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ΩΩ 2a Bateman - Systems Based > Haematology > Flashcards

Haematology Flashcards

1
Q

WHAT DOES CRYOPRECIPITATE CONTAIN?

A
  1. Factor 8
  2. Factor 13
  3. Fibrinogen
  4. Von Willebrand factor
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2
Q

WHAT IS MYELODYSPLASTIC SYNDROME?

A
  1. also known as myelodysplasia
  2. acquired neoplastic disorder of hematopoietic stem cells
  3. pre-leukaemia, may progress to AML
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3
Q

What are the features of myelodysplastic syndrome?

A
  1. more common with age
  2. presents with bone marrow failure (anaemia, neutropaenia, thrombocytopenia)
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4
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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5
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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6
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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7
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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8
Q

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

A

Monoclonal gammopathy of undetermined significance (MGUS)

  1. M-protein in serum <30 g/l
  2. Bone marrow clonal plasma cells <10 % and low level of plasma cell infiltration in a trephine biopsy (if done)
  3. No related organ or tissue impairment

​Asymptomatic myeloma (aMM)

  1. M-protein in serum >30 g/l and/or Bone marrow clonal plasma cells >10 %
  2. No related organ or tissue impairment

Symptomatic myeloma (sMM)

  1. M-protein in serum and/or urine
  2. Bone marrow (clonal) plasma cells or biopsy proven plasmacytoma
  3. Myeloma-related organ or tissue impairment (including bone lesions)
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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
Phosphate + Alk Phos Normal

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

What are the two different types of lymphoma?

A

Hodgkin’s

NHL.

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17
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

Most common type is nodular sclerosing

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18
Q

What is the epidemology of Hodgkin’s lymphoma?

A
  1. BIMODAL
  2. Young adults and elderly.
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19
Q

What are the risk factors for Hodgkin’s lymphoma?

A
  1. EBV
  2. SLE
  3. Post-transplantation
  4. Obesity
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20
Q

How does Hodgkins lymphoma present?

A

Enlarged lymph nodes

Hepatosplenomegaly

B symptoms:
Seen in approximately 25% of patients and includes

Unexplained fever >38°C

Drenching night sweats

Weight loss of >10% over 6 months

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21
Q

What can make lymph node pain worse?

A

Alcohol

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22
Q

How do you diagnose Hodgkins lymphoma?

A
  1. Lymph node biopsy
    • Reed-Sternberg cells
  2. Blood evaluation
    • FBC, ESR
  3. Chest radiography
  4. Staging with contrast
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23
Q

What is the staging system called for lymphoma?

A

Ann Arbor system

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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24
Q

What is the treatment for Hodgkin’s lymphoma?

A
  1. Chemo (ABVD) +/-RT
  2. Blood transfusions with irradiated packed red cells
  3. BMT for relapse
  4. Different amount of cycles depending on stage of disease
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25
Q

WHAT IS NHL?

A

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

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26
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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27
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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28
Q

What are some risk factors of NHL?

A
  1. Elderly
  2. Caucasians
  3. History of viral infection (specifically Epstein-Barr virus)
  4. Family history
  5. Certain chemical agents (pesticides, solvents)
  6. History of chemotherapy or radiotherapy
  7. Immunodeficiency (transplant, HIV, diabetes mellitus)
  8. Autoimmune disease (SLE, Sjogren’s, coeliac disease)
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29
Q

What are the symptoms of NHL?

A
  1. Painless lymphadenopathy
  2. Weight loss >10%
  3. Night sweats
  4. Pyrexia >38C
    • GI symptoms if small bowel lymphomas
  6. Skin involvement in T-cell lymphomas
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30
Q

What are the tests for NHL?

A

Marrow and node biopsy
Diagnostic

Blood
FBC, U&E, LFT

Cytology
LP for CSF cytology if CNS signs.

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31
Q

What is the treatment for NHL?

A

Low grade (follicular)
W&W

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

R-CHOP = Rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone.

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32
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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33
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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34
Q

What are the main differences between each leukaemia?

A
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35
Q

What is acute myeloid leukaemia a disease of?

A

Myeloblasts.

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36
Q

What is chronic myeloid leukaemia a disease of?

A

Basophils, Neutrophils and Eosinophils.

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37
Q

What is acute lymphoblastic leukaemia a disease of?

A

Lymphoblast.

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38
Q

What is chronic lymphocytic leukaemia a disease of?

A

B lymphocytes.

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39
Q

Which leukaemias affect children and which adults?

A

Children
ALL
AML

Adults
AML
CML
CLL

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40
Q

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

A

Sudan black stain

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41
Q

What can CLL develop into?

A

A lymphoma

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42
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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43
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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44
Q

What is the epidemology of acute myeloid leukaemia?

A

The commonest acute leukaemia of adults

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45
Q

What is the aetiology of acute myeloid leukaemia?

(Who is at increased risk)

A

Aetiology usually not obvious but risk increased in:

  1. Preceeding Haematological disorders
  2. Prior chemotherapy
  3. Exposure to ionising radiation
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46
Q

What are the symptoms of acute myeloid leukaemia?

A

Marrow failure
Symptoms of anaemia, infection or bleeding.

Fever

Infiltration
Hepatomegaly and splenomegaly
GUM HYPERTROPHY + BLEEDING

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47
Q

How can you diagnose acute myeloid leukaemia?

A
  1. FBC
    • ↓RBC
    • ↓PLT
    • WCC variable
    • Usually w/ neutropenia
  2. Bone marrow biopsy
    • Blast cells may be few in the peripheral blood
    • 20 out of 100 white blood cells
  3. Clotting screen
    • DIC may occur
  4. Blood film
    • ​​Aurer rods
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48
Q

What is diagnositc of AML from ALL?

A
  1. Auer rods are diagnostic of AML
  2. Myeloperoxidase makes this
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49
Q

What is the treatment for acute myeloid leukaemia?

A
  1. Supportive
    • (blood/platelets/fluids)
  2. Treat Infection
  3. Chemotherapy
    • (remission induction (regenerate) ➔ consolidation(intensification) ➔ maintenance: steroids
  4. Bone Marrow Transplantation
    • During 1st remission
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50
Q

What drug can be used to prevent tumour lysis syndrome?

Where else is this used?

A

Allopurinol

Gout

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51
Q

What is acute premylocitic leukaemia?

A

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

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52
Q

What happens with premyelocitic leukaemia?

What do you get with all the premyelocytes?

What does this cause?

A
  1. Build up of premyelocytes
  2. Lots of auer rods
  3. Increase coagualation risk
  4. Medical emergency
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53
Q

What is the treatment of premyelocitic leukaemia?

A

All trans retionic acid ATRA

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54
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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55
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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56
Q

Where do the myeloid cells go to through the blood?

A
  1. Liver and spleen
  2. Cause hepatosplenomegaly
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57
Q

What is the epidemology of chronic myeloid leukaemia?

A

Usually 40-60yrs age

Rare in childhood

Slight male dominance

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58
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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59
Q

What are the symptoms and signs of chronic myeloid leukaemia?

A

Symptoms

  1. Weight loss
  2. Tiredness
  3. Fever
  4. Sweats
  5. Bleeding (platelet dysfunction)
  6. Abdominal discomfort (splenic enlargement).

Signs

  1. Splenomegaly (>75%)— OFTEN MASSIVE
  2. Hepatomegaly, anaemia, bruising
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60
Q

What are the investigations for chronic myeloid leukaemia?

A
  1. FBC
    • Raised WCC >30x10^9 – all myeloid cells raised – neutrophils, macrophages, basophils, eosinophils).
    • Hb low or normal
    • Platlets variable
    • can cause thrombocytosis
  2. Cytogenetics (karyotype, FISH or PCR)
    • Philadelphia Chromosome
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61
Q

What is the treatment for chronic myeloid leukaemia?

A
  1. Matinib is now considered first-line treatment
    • Inhibitor of the tyrosine kinase associated with the BCR-ABL defect
    • Very high response rate in chronic phase CML
  2. Hydroxyurea
  3. Interferon-alpha
  4. Allogenic bone marrow transplant
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62
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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63
Q

What is the epidemology of acute lymphoblastic leukaemia?

A

Most common paediatric malignancy

Rarer in adults

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64
Q

What is thought to cause acute lymphoid leukaemia?

A

Genetic susceptibility
Translocations

+ an environmental trigger

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65
Q

What are the signs and symptoms for acute lymphoid leukaemia?

A
  1. CNS involvement (cranial nerve palsies and meningism)
  2. Anaemia, bleeding, vulnerability to infection
  3. Lymphadenopathy
  4. Orchidomegaly
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66
Q

What is the diagnosis for acute lymphoblastic leukaemia?

A
  1. FBC
    • ↓RBC, ↓PLT, WCC variable, usually w/ neutropenia
  2. Blood film
    • Blasts cells unless confined to bone marrow (+ bone marrow aspiration) 30 in 100 cells
    • Terminal deoxynucleotide transferase
  3. Clotting screen
    • DIC may occur
  4. Lumbar puncture
    • CNS involvement
  5. Cytogenetics
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67
Q

How do you treat acute lymphoblastic leukaemia?

A
  1. Chemotherapy
    Remission induction (regenerate) ➔ consolidation (intensification) ➔ maintenance: steroids
  2. Bone Marrow Transplantation
    During 1st remission
  3. Supportive
    Blood/platelets/fluids
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68
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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69
Q

What happens to the cells in CLL?

A
  • Don’t divide quickly enough
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70
Q

What is the epidemology of chronic lymphoblastic leukaemia?

A
  1. Most common leukaemia
  2. Generally elderly but 20% <55yrs
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71
Q

What are the signs and symptoms for chronic lymphoid leukaemia?

A

Symptoms

  1. Often none, surprise finding on a routine FBC.
  2. May be anaemic or infection-prone.
  3. If severe: ‘B’ symptoms - weight loss, sweats, anorexia.

Signs

  1. Enlarged, rubbery, non-tender nodes.
  2. Splenomegaly, hepatomegaly.
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72
Q

What are the different stages of chronic lymphocytic leukaemia?

A
  1. Rai Staging
  2. 1-5 based on examination and FBC
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73
Q

What are the tests for chronic lymphoid leukaemia?

A
  1. Increased WBCs greater than 15000 x 10^9 per litre
  2. Film
    Smudge cells (also know as smear cells)
    B cell that have been broken
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74
Q

What are the complications for chronic lymphoid leuklaemia?

A
  1. Autoimmune haemolysis
  2. Increased Infection due to hypogammaglobulinaemia
  3. Marrow failure
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75
Q

What is the treatment for chronic lymphocytic leukaemia?

A
  1. Rituximab
  2. Chemotherapy
  3. Bone marrow transplant + Radio
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76
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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77
Q

What does normal haemoglobin contain and foetal?

A

Normal Hb 2xa, 2xb

Foetal Hb 2xa, 2xg

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78
Q

What does haemoglobin change from to during development?

A

Haemoglobin F to haemoglobin A.

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79
Q

WHAT IS SICKLE CELL ANAEMIA (GENETICS)?

What type of anaemia is it?

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

A
  1. Sickle-cell anaemia is an autosomal recessive disorder causing production of abnormal beta globin chains.
  2. Microcytic anaemia
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80
Q

What is haemoglobin S?

A

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

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81
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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82
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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83
Q

What is the pathology of sickle cell disease?

What happens to the red blood cell?

A
  1. HbS polymerizes when deoxygenated
  2. RBCs deform, producing sickle cells
  3. Which are fragile and haemolyse, and also block small vessels
  4. Sickling cells appear at acidosis at at time when they deposit lots of oxygen
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84
Q

What are the symptoms of sickle cell disease?

Think about the crises’!

A
  1. Fatigue and Anaemia
  2. Pain Crises
  3. Dactylitis and Arthritis
  4. Bacterial Infections
  5. Leg Ulcers
  6. Aseptic Necrosis and Bone Infarcts
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85
Q

What are the different crises for sickle cell disease?

A
  1. Haemolytic
  2. Vaso-occlusive ‘painful’ crisis
  3. Aplastic crisis (sudden reduction in marrow production) - reduced reticulocytes
  4. Sequestration crisis (There is pooling of blood in the spleen ± liver) - increased reticulocytes
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86
Q

What are the tests for sickle cell disease?

A
  1. Hb ELECTROPHARESIS
    • For dx
    • HbSS present and absent HbA
  2. Screen neonate
    • blood/heel prick test
  3. FBC
    • Low Hb, high reticulocyte count
  4. Blood film
    • SICKLED erythrocytes
    • Howell-Jolly bodies
    • Taget cells
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87
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

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88
Q

What are the treatments for sickle cell anaemia?

A
  1. Prophylactic ABX
    • Daily penicillin.
    • Pneumococcal & influenza vaccine
    • Folic acid
  2. Pain relief for crisis
    • NSAIDs/paracetamol
  3. Hydroxyurea
    • Hydroxycarbamide - REDUCE COMPLICATIONS
    • Increase conc. of HbF
  4. Transfusions
    • Bone marrow transplant
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89
Q

What bacteria for osteomyelitis are sickle cell patients susceptable to?

A

Salmonella enteridis

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90
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

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91
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.

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92
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.

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93
Q

What type of anaemia is thalassaemia?

A

Microcytic

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94
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.

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95
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)

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96
Q

What is beta thalassemia major called?

A

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

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97
Q

When does beta thalassaemia major present?

A

6 -12 months

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98
Q

What are the symptoms of beta thalassaemia major?

A
  1. Failure to feed, listless, crying, pale.
  2. Bones widen due to increased activity
    • Skull bossing
  3. Anaemia
  4. Hepatosplenomegaly
    • Lots of RBCs need to be destroyed
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99
Q

What is beta thalassaemia minor or trait?

A

This is a carrier state

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100
Q

What are the symptoms of beta thalassaemia minor?

A
  1. Usually asymptomatic
  2. Mild, well-tolerated anaemia (Hb >90g/L)
    • Mild hypochromic, microcytic anaemia - microcytosis is characteristically disproportionate to the anaemia
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101
Q

What are the symptoms of beta thalassaemia intermedia?

A

Moderate anaemia but not requiring transfusions

There may be splenomegaly

102
Q

What are the tests for Beta thalassaemia?

A
  1. FBC & film
    • Hypochromic & microcytic anaemia
    • Target cells are seen
    • Reduced reticulocytes and nucleated RBCs
  2. Dx by Hb electrophoresis
    • Absent/low HbA.
    • HbA2 & HbF raised
103
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.

104
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.

105
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.

106
Q

WHAT ARE MEMBRANOPATHIES?

A

Autosomal dominant conditions.

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

107
Q

What are some examples of membranopathies?

A

Spherocytosis & elliptocytosis most common.

Neonatal jaundice.

108
Q

WHAT IS HAEMOLYTIC ANAEMIA?

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

A
  1. Anemia due to hemolysis
  2. Either in the blood vessels (intravascular hemolysis) or
  3. Elsewhere in the human body (extravascular)
109
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)

110
Q

What is Elliptocytosis? What causes this?

A

Where the red blood cells are shaped elliptically.

Horizontal interactions.

111
Q

What is Spherocytosis? What causes this?

A

Where the red blood cells are shaped spherically.

Vertical interactions.

112
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

113
Q

What are some symptoms of haemolytic anaemia?

A

Pallor

Jaunidce

Splenomegaly

114
Q

What are the tests for haemolytic anaemia?

A
  1. ↑Unconjugated bilirubin
  2. ↑LDH
  3. Coombs test +ve
    • Detect antibodies stuck on surface of RBC
  4. Reduced reticulocytes
115
Q

What is the management for haemolytic anaemia?

A
  1. Transfusion
  2. Folic acid
  3. Discontinue medications
  4. Iron
  5. Autoimmune haemolytic anaemia therapy
116
Q

WHAT ARE THE FEATURES OF HEREDITARY SPHEROCYTOSIS?

A
  1. failure to thrive
  2. jaundice, gallstones
  3. splenomegaly
  4. aplastic crisis precipitated by parvovirus infection
  5. degree of haemolysis variable
  6. MCHC elevated
117
Q

What are the investigations for hereditary spherocytosis?

A
  1. Patients with a family history of HS, typical clinical features and laboratory investigations (spherocytes, raised mean corpuscular haemoglobin concentration [MCHC], increase in reticulocytes) do not require any additional tests
  2. If the diagnosis is equivocal the BJH recommend the EMA binding test and the cryohaemolysis test
  3. For atypical presentations electrophoresis analysis of erythrocyte membranes is the method of choice
118
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.

119
Q

What do the enzymes do in the cell?

A
  1. Provides the fuel for the red cell
    • NAD+ to NAPH
  2. Generates redox capacity to protect red cell
    • Gluthathione
120
Q

How is Glucose 6 phosphate dehydrogenase deficiency caused?

Which drugs can precipitate an attack?

A
  1. Caused by a wide variety of mutations within G6PD gene
  2. X linked but women may also be affected

Drugs

  1. Sulphonamides
  2. Nitrofurans
  3. Antimalarials - chloroquine, primaquine
  4. Antipyretics
  5. Aspirin
  6. Chloramphenicol
121
Q

What are the symptoms of Glucose 6 phosphate dehydrogenase deficiency?

A
  1. Most asymptomatic
  2. But may get oxidative crises due to decreased glutathione production, precipitated by drugs
  3. In attacks, there is rapid anaemia and jaundice
122
Q

How is Glucose 6 phosphate dehydrogenase deficiency diagnosed?

A

G6PD enzyme assay

Film
Heinz boides
Bite- and blister-cells

123
Q

What is the treatment for glucose 6 phosphate dehydrogenase deficiency?

A
  1. Avoid precipitants (eg, henna)
  2. Transfuse if severe
124
Q

WHAT ARE PLATELETS AND HOW ARE THEY FORMED?

A

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

125
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

126
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.

127
Q

What does Clopidogrel inhibit?

A

Platelets

P2Y12.

128
Q

What does Aspirin inhibit?

A

COX-1.

129
Q

What is the platelet dysfunction: clinical features?

A
  1. Mucosal bleeding
    • Epistaxis, gum bleeding, menorrhagia
  2. Easy bruising
  3. Petechiae
    • Red or purple spot on the skin, caused by a minor bleed from broken capillary blood vessels
  4. Purpura
    • Red or purple discolored spots on the skin that do not blanch on applying pressure
  5. Traumatic haematomas
    • (inc subdural)
130
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

131
Q

What is thrombocytopenia?

A

Decreased platelet production.

132
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.

133
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.

134
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.

135
Q

WHAT IS IDIOPATHIC/IMMUNE THROMBOCYTOPENIA?

A
  1. IgG antibodies form to platelet and megakaryocyte surface glycoproteins
  2. Antibodies are directed against the glycoprotein IIb/IIIa or Ib-V-IX complex.
  3. Opsonized platelets are removed by reticuloendothelial system
136
Q

What is primary ITP?

A

May follow viral infection / immunisation esp in children

Varicella

137
Q

What is secondary ITP?

A

Occurs in association with some malignancies, such as:

  1. Chronic Lymphocytic Leukaemia(CLL)
  2. Infections e.g. HIV / HepC
138
Q

What is chronic ITP?

A

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

There is no splenomegaly.

139
Q

What are the symptoms for ITP?

A
  1. Easy bruising
  2. Purpura
  3. Epistaxis (nose bleed)
  4. Menorrhagia (heavy bleed at period)
140
Q

What are the tests for ITP?

A
  1. FBC
    • Thrombocytopenia
  2. Blood film
  3. Increased megakaryocytes in marrow
  4. Antiplatelet autoantibodies often present
141
Q

How do you treat immune thrombocytopenia?

A
  1. First-line treatment for ITP is oral prednisolone
  2. Pooled normal human immunoglobulin (IVIG) may also be used
    • It raises the platelet count quicker than steroids, therefore may be used if active bleeding or an urgent invasive procedure is required
  3. Splenectomy is now less commonly used
142
Q

WHAT IS DISSEMINATED INTRAVASCULAR CONGESTION?

https: //www.youtube.com/watch?v=Gmh01S0msfY
https: //www.youtube.com/watch?v=U3VbjS7WqOE

A
  1. Small blood clots develop throughout the bloodstream
  2. Blocking small blood vessels maybe causing organ ischemia
  3. Also depleting platelet supply leading to bleeding
143
Q

What is the cause of DIC?

A

Sepsis

Malginancy

Obstetric complications

Intravascular haemolysis

144
Q

What is DIC also known as and why?

A

Consumption coagulopathy, consumes platelets and clotting factors

145
Q

What is the normal response to injury?

What happens after a clot is formed?

A

Primary haemostasis

  1. Vasocontriction
  2. Platelets then adhere to the damaged endothelial wall
  3. These then activate other platelets and form a plug

Coagulation cascade

  1. One coagulation factor gets proteolytically cleaved which cleaves other coagulation factors
  2. This then cleaves fibrinogen into fibin which forms a mesh

Fibrinolysis

  1. This stops the clot from becoming too big.
146
Q

What conditions normally favour clotting?

A
  1. Sepsis
  2. Malignancy
  3. Trauma
  4. Obstetrics complications.
147
Q

What is the pathology of DIC?

A
  1. Underlying malignancy favours clotting
  2. Widespread clot formation leading to
  3. Ischaemia, necrosis and organ damage
  4. Kidneys, Liver, Lungs and Brain.
  5. Depletes clotting factors and platelets
  6. Fibrin degradation products in circulation
  7. Interferes with clot formation
148
Q

What are the symptoms of DIC?

A
  1. Bleeding
  2. Internal bleeding
  3. Bruising
  4. Ischaemia
149
Q

What are the investigations for DIC?

A

FBC

  1. ↓ platelets
  2. ↓ fibrinogen
  3. ↑ PT & APTT
  4. ↑ fibrinogen degradation products
  5. schistocytes due to microangiopathic haemolytic anaemia

Chronic DIC
Could look normal due to compensation

150
Q

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

A

LOW circulating coagulation factors

151
Q

How do you treat DIC?

A
  1. FFP: contains clotting factors - FIRST LINE
  2. Platelets
  3. Cryoprecipitate: contains fibrinogen and some clotting factors
  4. Underlying cause
152
Q

WHAT IS THROMBOTIC THROMBOCYTOPENIC PRUPURA?

https://www.youtube.com/watch?v=U3VbjS7WqOE

A

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

153
Q

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

What is there a defect in?

A
  1. Abnormally large and sticky multimers of von Willebrand’s factor cause platelets to clump within vessels
  2. ADAMTS13 (a metalloprotease enzyme) which breakdowns (‘cleaves’) large multimers of von Willebrand’s factor

​

  1. Overlaps with haemolytic uraemic syndrome (HUS)
154
Q

What are the causes of TTP?

A
  1. Idiopathic (40%)
  2. Post-infection e.g. urinary, gastrointestinal
  3. Pregnancy
  4. Drugs: ciclosporin, oral contraceptive pill, penicillin, clopidogrel, aciclovir
  5. Tumours
  6. SLE
  7. HIV
155
Q

What are the symptoms of TTP?

A
  1. Rare, typically adult females
  2. Fever
  3. Fluctuating neuro signs (microemboli)
  4. Microangiopathic haemolytic anaemia
  5. Thrombocytopenia
  6. Renal failure
156
Q

What are the tests for TTP?

A

Blood and protein in urine
Haematuria/proteinuria

Blood film
Fragmented RBC - Schistiocytes
Decrease platelets
Decrease Hb

Clotting tests are normal

157
Q

What is the treatment for TTP?

A

Plasma exchange using FFP

Steroids - prednisolone

158
Q

WHAT ARE SOME CAUSES OF OVER-ANTICOAGULATION?

A

Warfarin and Heparin

159
Q

What is heparin-induced thrombocytopenia?

A

IgG antibody against platelet-heparin complex

IgG/plt/heparin complex causes pltactivation and THROMBOSIS

160
Q

When does heparin-induced thrombocytopenia occur?

A

After Cardiac bypass surgery

161
Q

What are the symptoms of over-anticoagulation?

A

Bruising

Bleeding

Melena

Epistaxis

Hematemesis

Haemoptysis

162
Q

What is the treatment for heparin and warfarin overdose?

A
  1. Stop drug straight away
  2. Heparin - Protamine
  3. Warfarin - Vitamin K/ Phytomenadione
163
Q

WHAT ARE THE DIFFERENT TYPES OF ANAEMIA?

What are the MCVs for each?

A

Microcytic
<80

Normocytic
80-100

Macrocytic
>100

164
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

165
Q

What are the causes of normocytic anaemia?

A
  1. Acute blood loss
  2. Combined haematinic deficiency
  3. Anaemia of chronic disease e.g. CKD and so lack of EPO
166
Q

What are the different causes of macrocytic anaemia?

A

B12/Folate deficiency

Alcohol excess/liver disease

Metabolic disease e.g. hypothyroidism

(Megaloblastic)

167
Q

What is the normal haemoglobin range?

A

Male Hb = 131 – 166 g/L

Female Hb = 110 – 147 g/L

168
Q

WHAT TYPE OF ANAEMIA IS IRON DEFICIENCY ANAEMIA?

A

Microcytic

169
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

170
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).

171
Q

How can you investigate iron deficiency?

A
  1. FBC & film
    • MCV<80
    • Variation in size (anisocytosis) & shape (poikilocytosis) of cells.
    • Low serum iron & ferritin
    • Low reticulocytes (due to reduced Hb production)
  2. High TIBC
  3. Ix of GI tract
  4. Coeliac serology

IF HAEMOGLOBIN <10 WOMEN AND <11 MEN THEY NEED URGENT UPPER AND LOWER ENDOSCOPE

172
Q

What is the treatment of iron-deficiency anaemia?

A
  1. Oral iron, eg ferrous sulfate
  2. IV ferrous sulfate for surgery
    • If deficient and interval for surgery short
173
Q

What does an elevated ferritin show?

A

Inflammation

174
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.

175
Q

WHAT FOODS IS FOLATE FOUND?

A

Green veg, liver, nuts and yeast.

176
Q

What are the causes of folate deficiency?

A

Dietary

Malabsorption

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

177
Q

What are the tests for folate deficiency?

A

Low folate

Low Hb

Film
Macrocytic anaemia - hypersegmented polymorphs and Megaloblasts

178
Q

What is the treatment of folate-deficiency anaemia?

A

Folic acid supplements

With B12

179
Q

WHAT IS B12 DEFICIENCY?

A

Deficiency in B12

180
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)

181
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.

182
Q

What are the causes of B12 deficiency?

A
  1. Pernicious anaemia: most common cause
  2. Post gastrectomy
  3. Vegan diet or a poor diet
  4. Disorders/surgery of terminal ileum (site of absorption)
  5. Crohn’s: either diease activity or following ileocaecal resection
183
Q

How can you investigate B12 deficiency?

A
  1. Blood film:
    • Megoblasts and hypersegmented polymorphs
  2. Bone marrow biopsy:
    • IF antibodies
    • Schilling test (medical procedure used to determine whether you’re absorbing vitamin B-12 properly)
184
Q

What is the treatment of B12 deficiency?

A

Vitmain B12 injections or tablets

185
Q

WHAT IS PERNICIOUS ANAEMIA?

A

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

186
Q

What is pernicious anaemia associated with?

A

Other autoimmune diseases
Thyroid disease
Vtiligo
Addison’s disease
Hypoparathyroidism.

187
Q

What are the tests for pernicious anaemia?

A
  1. Intrinsic factor (IF) antibodies
  2. MCV increase
  3. Serum B12 lower
  4. Reticulocytes lower or normal as production impaired,
  5. Hypersegmented polymorphs
  6. Megaloblasts in the marrow
188
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.

189
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.

190
Q

What is pancytopenia?

A

Pancytopenia is reduction in all the major cell lines:

Red cells

White cells

Platelets

191
Q

WHAT IS APLASTIC ANAEMIA?

A

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

192
Q

What is the pathology of aplastic anaemia?

A

Decreased number of pluripotent stem cells.

BM replaced by fat.

193
Q

What are the causes of aplastic anaemia?

A
  1. Mostly AI
  2. Triggered by drugs (cytotoxics, chloramphenicol, sulphonamides, phenytoin, gold)
  3. Irradiation
  4. Fanconi anaemia (=inherited form)
  5. Infections (hepatitis)
194
Q

What is the test for aplastic anaemia?

A
  1. Bone marrow exam for dx
    • HYPOCELLULAR
  2. FBC
    • Pancytopenia
195
Q

What is the treatment for aplastic anaemia?

A

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

  1. Blood & platelet transfusions
  2. Prophylactic ABX/prompttx of infections

<40yo (curative)

  1. Bone marrow transplant

>40yo

  1. Immunosuppression w. anti-thymocyteglobulin & ciclosporin.
196
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.

197
Q

What are the different myeloproliferative disorders?

A

RBC
Polycythaemia rubra vera (PRV)

WBC
Chronic myeloid leukaemia (CML, p352)

Platelets
Essential thrombocythaemia

Fibroblasts
Myelofibrosis

198
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.

199
Q

What is the cause of polycythaemia rubra vera?

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

200
Q

How does polycythaemia rubra vera present like?

A

May present with no symptoms

May have

Easy bleeding/bruising
Fatigue
Dizziness
Headaches

201
Q

What are the investigations for polycythaemia rubra vera?

A
  1. FBC
  2. Bone marrow biopsy
  3. Genetic testing for JAK2 gene
202
Q

How can you treat polycythaemia rubra vera?

A
  1. Aspirin
    Reduces the risk of thrombotic events
  2. Venesection
    First-line treatment to keep the haemoglobin in the normal range
  3. Chemotherapy
    Hydroxyurea - slight increased risk of secondary leukaemia
    Phosphorus-32 therapy
203
Q

What can polycythaemia ruba vera progress to?

A
  1. 5-15% of patients progress to myelofibrosis
  2. 5-15% of patients progress to acute myeloid leukaemia (risk increased with chemotherapy treatment)
204
Q

WHAT IS MALARIA?

A

Malaria is a disease caused by Plasmodium protozoa which is spread by the female Anopheles mosquito. There are four different species which cause disease in man:

Plasmodium falciparum

Plasmodium vivax

Plasmodium ovale

Plasmodium malariae

205
Q

What is fever paroxysms in malaria?

What are the phases?

A

Fever paroxysms reflect synchronous release of flocks merozoites from mature schizonts

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
206
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.

207
Q

What is the diagnosis of malaria?

A

Microscopy of films

Rapid diagnostic test

208
Q

What is the treatment for malaria?

A

Chloroquine

209
Q

WHAT IS A DVT?

A

Deep vein thrombosis, a subset of venous thromboembolism (VTE), occurs in normal veins, usually the deep calf veins of the leg and propagates more proximally

210
Q

What are the clinical features of a DVT?

A
  1. Pain and tenderness
  2. Pain may vary from an ache to cramping, from dull to sharp and from mild to severe, be intermittent or constant
  3. Aggravated by movement and standing up and relived by elevating the leg
  4. Local swelling and warmth
  5. Mild pyrexia
  6. Persistent tachycardia
211
Q

What is the diagnosis of a DVT?

A

Well’s score

D-dimer

212
Q

What should you do if the D-dimer is positive but the doppler is negative?

A

Stop anticoagulation and repeat scan in 6-8 days

213
Q

What is the treatment for a DVT?

A

NOAC

  1. 3 months - provoked
  2. 6 months - unprovoked

UNLESS

  1. Severe renal impairment <15/min
  2. Antiphospholipid syndrome

then LMWH

214
Q

What is the management of different high INRs?

A

INR>8 or active bleeding
STOP warfarin
Give vit K
Restart warfarin when <5

INR 5-8

  1. Minor bleeding - STOP warfarin, give vit K
  2. No bleeding - withhold 1 or 2 does of warfarin
215
Q

What are the reversal agents for each DOAC?

A

Apixaban or rivaroxiban - Andexanet alfa

Dabigatran - Idarucizumab

Haemodialysis if treatment methods don’t work

216
Q

What is the reversal agent for heparin, enoxaparin or dalteparin?

A

Protamine sulphate

217
Q

What are the side effects of erythropoietin?

A
  1. Accelerated hypertension potentially leading to encephalopathy and seizures (blood pressure increases in 25% of patients)
  2. Bone aches
  3. Flu-like symptoms
  4. Skin rashes, urticaria
  5. Pure red cell aplasia* (due to antibodies against erythropoietin)
  6. Raised PCV increases risk of thrombosis (e.g. Fistula)
  7. Iron deficiency 2nd to increased erythropoiesis
218
Q

WHAT IS NEUTROPENIC SEPSIS

A

Neutropenic sepsis is a relatively common complication of cancer therapy, usually as a consequence of chemotherapy?

219
Q

What are the features of neutropenic sepsis?

A
  1. It most commonly occurs 7-14 days after chemotherapy.
  2. It may be defined as a neutrophil count of < 0.5 * 109 in a patient who is having anticancer treatment and has one of the following:
    • A temperature higher than 38ºC or
    • Other signs or symptoms consistent with clinically significant sepsis
220
Q

What is the treatment for neutropenic sepsis?

A
  1. Piperacillin with tazobactam (Tazocin)
  2. Started asap - do not wait for blood results
221
Q

WHAT IS HAEMOPHILIA?

A

Haemophilia is an X-linked recessive disorder of coagulation

222
Q

What factor is haemaphilia A deficient in?

A

VIII (8)

223
Q

What factor is haemaphilia B (christmas disease) deficient in?

A

IX (9)

224
Q

What are the features of haemophilia?

A
  1. Haemoarthroses (blood in joints)
  2. Haematomas
  3. Prolonged bleeding after surgery or trauma
225
Q

What are the investigations for haemophilia?

A
  1. Prolonged APTT
  2. Bleeding time, thrombin time, prothrombin time normal
226
Q

WHAT IS HAEMOLYTIC URAEMIC SYNDROME?

A
  1. Haemolytic uraemic syndrome is generally seen in young children and produces a triad of:
  2. acute kidney injury
  3. microangiopathic haemolytic anaemia
  4. thrombocytopenia
227
Q

What are the investigations for HUS?

A
  1. full blood count: anaemia, thrombocytopaenia, fragmented blood film
  2. Decreased haptoglobins
  3. U&E: acute kidney injury
  4. stool culture
    • looking for evidence of STEC infection
    • PCR for Shiga toxins
228
Q

What is the treatment of HUS?

A
  1. Treatment is supportive e.g. Fluids, blood transfusion and dialysis if required
  2. There is no role for antibiotics, despite the preceding diarrhoeal illness in many patients
  3. The indications for plasma exchange in HUS are complicated. As a general rule plasma exchange is reserved for severe cases of HUS not associated with diarrhoea
  4. Eculizumab (a C5 inhibitor monoclonal antibody) has evidence of greater efficiency than plasma exchange alone in the treatment of adult atypical HUS
229
Q

WHAT IS ACUTE HAEMOLYTIC REACTION SYMPTOMS?

A
  1. Fever
  2. Abdominal pain
  3. Hypotension
230
Q

WHAT IS THE CAUSE FOR ANAPHYLAXIS IN BLOOD TRANSUFSIONS?

A

Low IgA

231
Q

WHAT IS VON WILEBARND DISEASE?

A
  1. Von Willebrand’s disease is the most common inherited bleeding disorder.
  2. The majority of cases are inherited in an autosomal dominant fashion* and characteristically behaves like a platelet disorder i.e. epistaxis and menorrhagia are common whilst haemoarthroses and muscle haematomas are rare
232
Q

What is the role of von willebrand factor?

A
  1. large glycoprotein which forms massive multimers up to 1,000,000 Da in size
  2. promotes platelet adhesion to damaged endothelium
  3. carrier molecule for factor VIII
233
Q

What are the investigations von willebrand disease?

A
  1. prolonged bleeding time
  2. APTT may be prolonged
  3. factor VIII levels may be moderately reduced
  4. defective platelet aggregation with ristocetin
234
Q

What is the management of von willebrand disease?

A
  1. tranexamic acid for mild bleeding
  2. desmopressin (DDAVP): raises levels of vWF by inducing release of vWF from Weibel-Palade bodies in endothelial cells
  3. factor VIII concentrate
235
Q

WHAT ARE THE SYMPTOMS OF A FAT EMBOLISM?

A
236
Q

What is the imaging used for a fat emboli?

A
  1. May be normal
  2. Fat emboli tend to lodge distally and therefore CTPA may not show any vascular occlusion, a ground glass appearance may be seen at the periphery
237
Q

What is the treatment for fat emboli?

A
  1. Prompt fixation of long bone fractures
  2. Some debate regarding benefit Vs. risk of medullary reaming in femoral shaft/ tibial fractures in terms of increasing risk (probably does not).
  3. DVT prophylaxis
  4. General supportive care
238
Q

WHAT IS THE MANAGEMENT OF CKD ANAEMIA?

A
  1. The 2011 NICE guidelines suggest a target haemoglobin of 10 - 12 g/dl
  2. Determination and optimisation of iron status should be carried out prior to the administration of erythropoiesis-stimulating agents (ESA). Many patients, especially those on haemodialysis, will require IV iron
  3. ESAs such as erythropoietin and darbepoetin should be used in those ‘who are likely to benefit in terms of quality of life and physical function’
239
Q

WHY ARE IRRADIATED BLOOD PRODUCTS USED?

A

To prevent graft versus host disease

240
Q

IF A PATIENT HAS JUST DRANK ALCOHOL BEFORE A BLOOD TEST WHAT WOULD YOU SEE?

A

Macrocytic anaemia and thrombocytopenia

241
Q

What conditions are involved in the intrinsic pathway?

A
  1. Haemophilia
  2. Von Wilebrand’s disease
  3. Vitmain K deficiency
242
Q

What conditions are involved in the extrinsic pathway?

A
  1. Vitamin K deficiency
243
Q

WHAT IS FACTOR V LEIDEN?

A

Factor V Leiden (activated protein C resistance) is the most common inherited thrombophilia, being present in around 5% of the UK population.

244
Q

What is the other name for factor V leiden?

A

Protein C resistance

245
Q

WHAT IS TUMOUR LYSIS SYNDROME?

A
  1. Tumour lysis syndrome (TLS) is a potentially deadly condition related to the treatment of high-grade lymphomas and leukaemias.
  2. It can occur in the absence of chemotherapy but is usually triggered by the introduction of combination chemotherapy.
  3. On occasion, it can occur with steroid treatment alone
246
Q

What are the symptoms of tumour lysis syndrome?

A
  1. A high potassium
  2. High phosphate
  3. Low calcium
247
Q

What is the prophylaxis for tumour lysis syndrome?

A
  1. Patients at high risk of TLS should be given
    • IV allopurinol or
    • IV rasburicase

Immediately prior to and during the first days of chemotherapy.

248
Q

WHAT IS SIDEROBLASTIC ANAEMIA?

A

Sideroblastic anaemia is a condition where red cells fail to completely form haem, whose biosynthesis takes place partly in the mitochondrion. This leads to deposits of iron in the mitochondria that form a ring around the nucleus called a ring sideroblast. It may be congenital or acquired.

249
Q

What are the investigations for sideroblastic anaemia?

A
  1. full blood count
    • hypochromic microcytic anaemia (more so in congenital)
  2. iron studies
    • high ferritin
    • high iron
    • high transferrin saturation
  3. blood film
    • basophilic stippling of red blood cells
  4. bone marrow
    • Prussian blue staining will show ringed sideroblasts
250
Q

What is the treatment for sideroblastic anaemia?

A
  1. supportive
  2. treat any underlying cause
  3. pyridoxine may help
251
Q

What does MCH stand for?

A

Mean corpuscular haemoglobin

ΩΩ 2a Bateman - Systems Based (11 decks)

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