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Yr4 - Cancer Care > Haematological Malignacies > Flashcards

Haematological Malignacies Flashcards

1
Q

Define myelomas

A

Proliferation of plasma cells

  • terminal cell of B lymphocyte
  • release immunoglobulins - non-functioning
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2
Q

Features of myelomas

A

Bone marrow infiltration -> anaemia/thrombocytopenia
Cause bone and renal disease
Can form solid tumours of plasma cells (plasmacytomas)
Impaired immune system
Treatable but not curable

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3
Q

Myeloma presentation

A 
Bones
- generalised osteopenia
- pathological fractures
Hypercalcaemia
- polyuria, polydipsia, abdo pain, constipation, lethargy, confusion
Bone marrow infiltration
- anaemia
- thrombocytopenia
- neutropenia
Infections
Hyperviscotisy syndrome
- visual disturbance 
- headache
- - - -
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4
Q

What define myeloma

A 
Myeloma defining events
S - 60% BM plasmacytosis
Li - light chain ratio > 100
M - > 1 focal lesions on MRI > 5 mm
Evidence of end organ damage
C - calcium elevation > 2.75
R - renal impairment Cr>117
A - anaemia Hb < 100
B - bone lesion
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5
Q

Define haemopoiesis

A

Formation of blood cells from a hemopoietic stem cell

  • cell isolated from blood or bone marrow can renew itself
  • able to differentiate to a variety of specialised cells
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6
Q

Where does haemopoiesis go wrong

A 
Loss of normal tight controls on haemopoiesis
- too many cells proliferating 
- don't apoptose/die when should
- don't differentiate (maturation arrest)
Myeloid 
- acute myeloid leukaemia
- chronic myeloproliferative neoplasms
Lymphoid
- acute lymphoblastic leukaemia
- chronic lymphoblastic leukaemia
- lymphoma
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7
Q

Leukaemia vs lymphoma

A

Leukaemia - affecting mainly bone marrow with or without released circulating neoplastic cells in blood
Lymphoma - predominantly nodal or organ-based

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8
Q

Define leukaemia

A

Cancer of stem cell line in bone marrow

Increase in number of white blood cells

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9
Q

Classification of leukaemia

A

Myeloid vs lymphoid
- depends on which lineage is involved
Acute vs chronic
- based on maturity of cells
- acute has proliferation of immature blast cells
- chronic has proliferation of more mature cells
- pace of disease faster in chronic

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10
Q

Define myeloproliferative neoplasms

A

Chronic proliferation of myeloid cells
Red = polycythaemia
Platelets = thrombocythemia
White = leukaemia (CML)

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11
Q

Features of bone marrow

A

Where blood cells develop

Mostly found in pelvis, vertebrae, ribs and sternum

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12
Q

Development of blood cells

A

Pluripotent haematopoietic stem cells
- undifferentiated cells that have the potential to become - myeloid stem cells, lymphoid stem cells and dendritic cells
Red blood cells
- develop from reticulocytes that come from myeloid stem cells
- survive up to 3 months
Platelets
- made by megakaryocytes
- lifespan of 10 days
White blood cells
- myeloid stem cells become promyelocytes
- monocytes then macrophages
- neutrophils
- eosinophils
- mast cells
- basophils
- lymphocytes come from lymphoid stem cells - become B and T cells
- B lymphocytes mature in the bone marrow and differentiate into plasma or memory B cells
- t lymphocytes mature in thymus gland and differentiate into CD4 cells (T helper), CD8 cells (cytotoxic T cells) and natural killer cells

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13
Q

Features of blood films

A

Anisocytosis - variation in size of red blood cells
- myelodysplastic syndrome as well as anaemia
Target cells - central pigmented area surrounded by pale area surrounded by ring of thicker cytoplasm
- iron deficiency anaemia and post-splenectomy
Heinz bodies - individual blobs seen inside RBC caused by denatured globin
- G6PD and alpha-thalassaemia
Howell-Jolly bodies - individual blobs of DNA material seen in RBC, normally removed by spleen
- post-splenectomy and severe anaemia
Reticulocytes - larger immature RBC, still have RNA material
- haemolytic anaemia
Schistocytes - fragments of RBCs
- haemolytic uraemic syndrome, DIC, thrombotic thrombocytopenia purpura, metallic heart valves, haemolytic anaemia
Sideroblasts - immature RBC that contain blobs of iron, occur when bone marrow unable to incorporate iron into haemoglobin
- myelodysplasic syndrome
Smudge cells - ruptured WBC, occur during process of preparing the blood film due to aged or fragile WBC
- chronic lymphocytic leukaemia
Spherocytes - spherical RBCs
- autoimmune haemolytic anaemia or hereditary spherocytosis

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14
Q

Define anaemia

A

Low level of haemoglobin

  • women 120-165
  • men 130-180
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15
Q

Causes of microcytic anaemia

A

TAILS

  • thalassaemia
  • anaemia of chronic disease
  • iron deficiency anaemia
  • lead poisoning
  • sideroblastic anaemia
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16
Q

Causes of normocytic anaemia

A

3As and 2Hs

  • acute blood loss
  • anaemia of chronic disease
  • aplastic anaemia
  • haemolytic anaemia
  • hypothyroidism
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17
Q

Causes of macrocytic anaemia

A 
Megaloblastic
- result of impaired DNA synthesis preventing cell from dividing normally
- B12 or folate deficiency
Normoblastic
- alcohol
- reticulocytosis
- hypothyroidism
- liver disease
- azathioprine
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18
Q

Symptoms of anaemia

A 
Fatigue
SOB
Headaches
Dizziness
Palpitations
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19
Q

Specific symptoms of iron deficiency anaemia

A

Pica - dietary cravings

Hair loss

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20
Q

Signs of anaemia

A 
General
- pale skin
- conjunctival pallor
- tachycardia
- raised RR
Specific
- koilonychia, angular cheilitis, atrophic glossitis - iron deficiency
- jaundice - haemolytic
- bone deformities - thalassaemia
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21
Q

Ix for anaemia

A 
Haemoglobin
Mean Cell Volume (MCV)
B12
Folate
Ferritin
Blood film
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22
Q

Causes of iron deficiency anaemia

A 
Blood loss - most common in adults
- menorrhagia
- GI tract cancer
Dietary insufficiency - most common in growing children
Poor iron absorption
Increased requirements during pregnancy
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23
Q

Features of iron deficiency

A

Ferritin - form of iron when deposited and stored in cells
- low suggestive of iron deficiency
- high is difficult to interpret as related to inflammation
Serum iron - varies throughout day
Total iron binding capacity - marker for how much transferrin is in blood
Transferrin saturation - good indication of total iron in body

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24
Q

Mx of iron deficiency anaemia

A

Blood transfusion
Iron infusion
Oral iron

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25
Q

Pathophysiology of pernicious anaemia

A

Autoimmune condition

Antibodies form against parietal cells or intrinsic factor preventing absorption of vitamin B12

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26
Q

Symptoms of B12 deficiency

A

Peripheral neuropathy with numbness or paraesthesia
Loss of vibration sense or proprioception
Visual changes
Mood or cognitive changes

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27
Q

Ix for B12 deficiency

A

Testing for auto-antibodies

  • intrinsic factor antibody - 1st line
  • gastric parietal cell antibody
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28
Q

Mx of B12 deficiency anaemia

A

Dietary deficiency - oral replacement - cyanocobalamin

Pernicious anaemia - 1mg IM hydroxycobalamin 3x weekly for 2 weeks, then every 3 months

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29
Q

Define autoimmune haemolytic anaemia

A

Antibodies created against patients RBCs

Leads to destruction of RBC

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30
Q

Types of autoimmune haemolytic anaemia

A 
Warm
- more common
- haemolysis occurs at normal or above normal temperatures
- idiopathic
Cold
- antibodies attach at lower temperatures causing RBC to clump together - agglutination
-> destruction of RBC
- secondary to other conditions 
     - lymphoma
     - leukaemia
     - SLE
     - EBV
     - CMV
     - HIV
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31
Q

Mx of autoimmune haemolytic anaemia

A

Blood transfusions
Prednisolone
Rituximab - monoclonal antibody against B cells
Splenectomy

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32
Q

Define thalassaemia

A

Genetic defect in protein chains that make up haemoglobin
- defects in alpha-globin -> alpha thalassaemia - chromosome 16
- defects in beta-globin -> beta thalassaemia - chromosome 11
Both autosomal recessive

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33
Q

Clinical features of thalassaemia

A 
Microcytic anaemia (low mean corpuscular volume)
Fatigue
Pallor
Jaundice
Gallstones
Splenomegaly
Poor growth and development
Pronounced forehead and malar eminences
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34
Q

Ix for thalassaemia

A

FBC - microcytic anaemia
Haemoglobin electrophoresis
DNA testing

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35
Q

Complications of thalassaemia

A

Iron overload
- faulty RBC, recurrent transfusions and increased iron absorption
Monitor serum ferritin
Mx involves limiting transfusions and iron chelation

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36
Q

Mx of alpha-thalassaemia

A 
Monitoring the full blood count
Monitoring for complications
Blood transfusions
Splenectomy may be performed
Bone marrow transplant can be curative
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37
Q

Types of beta-thalassaemia

A

Minor
- one normal and one abnormal gene
- causes mild microcytic anaemia
Intermedia
- two defective genes or one defective and one deletion
- more significant microcytic anaemia
Major
- homozygous for deletion genes
- severe anaemia and failure to thrive in early childhood
- causes severe microcytic anaemia, splenomegaly and bone deformities

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38
Q

Define sickle cell anaemia

A

Genetic condition that causes sickle shaped red cells

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39
Q

Pathophysiology of sickle cell anaemia

A

Foetal haemoglobin replaced by haemoglobin A at around 6 weeks
Patients with sickle cell have abnormal haemoglobin variant S
- causes red blood cells to sickle
Autosomal recessive
Abnormal gene for beta-globin on chromosome 11

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40
Q

Complications of sickle cell anaemia

A 
Anaemia
Increased risk of infection
Stroke
Avascular necrosis in large joints such as the hip
Pulmonary hypertension
Painful and persistent penile erection (priapism)
Chronic kidney disease
Sickle cell crises
Acute chest syndrome
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41
Q

Mx of sickle cell

A

Avoid dehydration and other triggers of crises
Ensure vaccines are up to date
Antibiotic prophylaxis to protect against infection with penicillin V (phenoxymethypenicillin)
Hydroxycarbamide can be used to stimulate production of fetal haemoglobin (HbF). Fetal haemoglobin does not lead to sickling of red blood cells. This has a protective effect against sickle cell crises and acute chest syndrome.
Blood transfusion for severe anaemia
Bone marrow transplant can be curative

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42
Q

Define sickle cell crisis

A

Umbrella term for spectrum of acute crises related to the condition
Occur spontaneously of triggered by stresses
- infection
- dehydration
- cold
- significant life events

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43
Q

Mx of sickle cell crisis

A

Have a low threshold for admission to hospital
Treat any infection
Keep warm
Keep well hydrated (IV fluids may be required)
Simple analgesia such as paracetamol and ibuprofen
Penile aspiration in priapism

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44
Q

Types of leukaemia

A

Acute myeloid leukaemia
Acute lymphoblastic leukaemia
Chronic myeloid leukaemia
Chronic lymphocytic leukaemia

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45
Q

Pathophysiology of leukaemia

A

Genetic mutation in one of the precursor cells leads to excessive production of single type of abnormal WBC
Suppression of the cell lines -> pancytopenia
- low RBC - anaemia
- low WBC - leukopenia
- low platelets - thrombocytopenia

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46
Q

Epidemiology of leukaemia

A

Under 5 and over 45 - ALL
Over 55 - CLL
Over 65 - CML
Over 75 - AML

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47
Q

Presentation of leukaemia

A 
Non-specific - need urgent FBC
Fatigue
Fever
Failure to thrive (children)
Pallor due to anaemia
Petechiae and abnormal bruising due to thrombocytopenia
Abnormal bleeding
Lymphadenopathy
Hepatosplenomegaly
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48
Q

Differential diagnosis of petechiae

A

Caused by thrombocytopenia

  • leukaemia
  • meningococcal septicaemia
  • vasculitis
  • idiopathic thrombocytopenia purpura
  • non-accidental injury
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49
Q

Ix for leukaemia

A 
FBC - within 48 hrs
Blood film
LDH - raised but not specific
CXR - infection or mediastinal lymphadenopathy
Lymph node biopsy
Lumbar puncture - if CNS involvement
CT/MRI/PET for staging and assessing
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50
Q

Types of bone marrow biopsy

A 
Bone marrow aspiration
- taking liquid sample full of cells
Bone marrow trephine
- solid core sample of bone marrow
- better assessment of cells and structure
Bone marrow biopsy
- usually taken from iliac crest
- local anaesthetic and specialist needle
- samples can be examined straight away
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51
Q

Define Acute Lymphoblastic Leukaemia

A

Malignant change in one the lymphocyte precursor cells

  • > acute proliferation of a single type of lymphocyte - usually B lymphocytes
  • > replace other cell types created by bone marrow - pancytopenia
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52
Q

Epidemiology of ALL

A 
Most common cancer in children
- peaks 2-4 years
Also affects adults over 45
Associated with Downs syndrome
Associated with Philadelphia chromosome translocation
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53
Q

Features of blood film of ALL

A

Blast cells - large cell with very large nucleus

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54
Q

Define chronic lymphocytic leukaemia

A

Chronic proliferation of single type of well differentiated lymphocyte - usually B lymphocytes

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55
Q

Epidemiology of CLL

A

Adults over 55

Can transform into high-grade lymphoma - Richter’s transformation

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56
Q

Clinical features of CLL

A 
Asymptomatic
Infection
Anaemia
Bleeding
Weight loss
Can cause warm autoimmune haemolytic anaemia
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57
Q

Blood film features of CLL

A

Smear or smudge cells

- aged or fragile WCC rupture leaving a smudge on the film

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58
Q

Features of chronic myeloid leukaemia

A 
3 phases
- chronic
- accelerated
- blast
Associated with Philadelphia chromosome
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59
Q

Feature of chronic phrase of CML

A

Lasts around 5 years

Often asymptomatic - patients diagnosed incidentally with raised WCC

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60
Q

Features of accelerated phase of CML

A

Occurs when abnormal blast cells take up high proportion of cells in bone marrow and blood
Patients become symptomatic, develop anaemia and thrombocytopenia and become immunocompromised

61
Q

Features of the blast phrase of CML

A

Involves high proportion of blast cells and blood
Severe symptoms and pancytopenia
Often fatal

62
Q

Features of acute myeloid leukaemia

A

Many different types all with slightly different cytogenetic differences and differences in presentation

63
Q

Epidemiology of AML

A

Most common acute leukaemia in adults
Can present at any age - normally middle age onwards
Can result from a transformation from myeloproliferative disorder - polycythaemia ruby vera or myelofibrosis

64
Q

Features of blood film of AML

A

High proportion of blast cells

- Auer rods - rods inside cytoplasm

65
Q

Mx of leukaemia

A

Mainly chemotherapy and steroids

Allogenic stem cell transplant

66
Q

Complications of leukaemia

A 
Failure
Stunted growth and development in children
Infections due to immunodeficiency
Neurotoxicity
Infertility
Secondary malignancy
Cardiotoxicity
Tumour lysis syndrome
67
Q

Pathophysiology of tumour lysis syndrome

A

Causes by release of uric acid from cells that are being destroyed by chemotherapy
Uric acid can form crystals in interstitial tissue and tubules of kidneys

68
Q

Mx of tumour lysis syndrome

A

Allopurinol or rasburicase used to reduce high uric acid levels

69
Q

Define lymphomas

A

Group of cancers that affect lymphocytes inside lymphatic system
Cancerous cells proliferate within lymph nodes -> lymphadenopathy

70
Q

Categories of lymphoma

A

Hodgkin’s
- specific disease
Non-Hodgkin’s
- encompasses all other lymphomas
- B cell or T cell in origin
- either low (aggressive, large cells seen) or high grade (small cells)
- high grade tumours have high mortality in first few years but then good prognosis as less likely to reoccur

71
Q

Epidemiology of Hodgkin’s lymphoma

A

1 in 5 lymphomas
Caused by proliferation of lymphocytes
Bimodal age distribution with peaks around 20 and 75

72
Q

Risk factors for Hodgkin’s lymphoma

A

HIV
EBV
Autoimmune conditions - RA and sarcoidosis
FHx

73
Q

Clinical features of Hodgkin’s lymphoma

A 
Lymphadenopathy
- enlarged lymph nodes
- neck, axilla or inguinal region
- non-tender and rubbery
- pain in nodes when drinking
Fatigue
Itching
Cough
Shortness of breath
Abdominal pain
Recurrent infections
B symptoms
- fever
- weight loss
- night sweats
74
Q

Ix of lymphoma

A 
Lactate dehydrogenase
- raised in Hodgkin's but not specific
Lymph node biopsy
- Reed-Sternberg cells - abnormally large B cells with multiple nuclei with nucleoli inside them - owl face
CT, MRI or PET for stagin
75
Q

Staging of lymphoma

A

Ann Arbor Staging used for all lymphomas
Stage 1 - confined to one region of lymph nodes
Stage 2 - in more than one region but on same side of diaphragm
Stage 3 - affects lymph nodes both above and below the diaphragm
Stage 4 - widespread involvement including non-lymphatic organs such as lungs or liver
A - asymptomatic
B - b symptoms present
E - presence of extranodal site

76
Q

Mx of Hodgkin lymphoma

A

Chemotherapy (ABVD - doxorubicin (Adriamycin), bleomycin, vinblastine and dacarbazine (DTIC)) and radiotherapy

  • aim to cure
  • usually successful
  • risk of relapse
77
Q

Side effects of mx of Hodgkin lymphoma

A 
Chemotherapy
- leukaemia
- infertility
Radiotherapy
- cancer
- damage to tissues
- hypothyroidism
78
Q

Types of non-Hodgkin Lymphoma

A

Burkitt lymphoma - associated with EBV, malaria and HIV
MALT lymphoma - affects mucosa-associated lymphoid tissue, usually around the stomach, a/w H.pylori
Diffuse large B cell lymphoma - presents as rapidly growing painless mass in patients over 65 years

79
Q

Risk factors for non-Hodgkin’s lymphoma

A 
HIV
Epstein-Barr Virus
H. pylori (MALT lymphoma)
Hepatitis B or C infection
Exposure to pesticides and a specific chemical called trichloroethylene used in several industrial processes
Family history
80
Q

Mx of follicular lymphoma

A 
Stage 2A 
- radiotherapy
- watch and wait if unable
Stage 3/4 (advanced)
- A - rituximab
- S - rituximab with chemotherapy
      - cyclophosphamide, vincristine and prednisolone (CVP)
      - cyclophosphamide, doxorubicin, vincristine and prednisolone (CHOP)

Stem cell transplantation

81
Q

Define myeloma

A

Cancer of the plasma cells

-> large quantities of single type of antibody being produced

82
Q

Define multiple myeloma

A

Myeloma that affects multiple areas of the body

83
Q

Define MGUS

A

Monoclonal Gammopathy of Undetermined Significance
Excess of a single type of antibody or antibody components without other features of myeloma or cancer
Often incidental finding in otherwise healthy person
May progress to myeloma

84
Q

Define smouldering myeloma

A

Progression of MGUS with higher levels of antibodies or antibody components
Premalignant

85
Q

Pathophysiology of myeloma

A

Genetic mutation causing a plasma cell to rapidly and uncontrollably multiply
Produce one type of antibodies (immunoglobulins)
One type (A, G, M, D and E) significantly abundant
- monoclonal paraprotein
- IgG more than 50% of the time
Bence Jones protein can be found in the urine of many myeloma patients
- subunit of the antibody - light chains

86
Q

Complications of myeloma

A

Anaemia
Myeloma bone disease
Myeloma renal disease
Hyperviscocity

87
Q

Features of anaemia of myeloma

A

Cancerous plasma cells invade the bone marrow - bone marrow infiltration
Suppression of development of other blood cell lines
- anaemia - low RBC
- neutropenia - low neutrophils
- thrombocytopenia - low platelets

88
Q

Features of myeloma bone disease

A

Result of increased osteoclast activity and suppressed osteoblast activity
Metabolism of bone is imbalanced
- more bone reabsorbed than constructed
- cytokines released from plasma cells and stromal cells
Commonly occurs in skull, spine, long bones and ribs
- some areas of bone becomes thin - osteolytic lesions
- lead to pathological fractures
Osteoclast activity causes a lot of calcium to be released into blood
- hypercalcaemia
Development of plasmacytomas
- individual tumours made up of cancerous plasma cells

89
Q

Features of myeloma renal disease

A

Development of renal impairment

  • High levels of immunoglobulins (antibodies) can block the flow through the tubules
  • Hypercalcaemia impairs renal function
  • Dehydration
  • Medications used to treat the conditions such as bisphosphonates can be harmful to the kidneys
90
Q

Features of hyperviscocity of myeloma

A

Viscosity increases due to increased proteins
Leads to
- easy bruising
- easy bleeding
- reduced or loss of sight due to vascular disease
- purple discolouration to the extremities
- heart failure

91
Q

4 key features of myeloma

A 
CRAB
Calcium elevated
Renal failure
Anaemia - normocytic, normochromic
Bone lesions/pain
92
Q

Risk factors for myeloma

A 
Older age
Male
Black African ethnicity
Family history
Obesity
93
Q

Ix for myeloma

A 
Suspect in anyone over 60 with persistent bone pain, particularly back pain or unexplained fractures
FBC - low WCC
Calcium - raised
ESR - raised 
Plasma viscosity - raised
94
Q

Testing for myeloma

A 
B - Bence-Jones protein - request urine electrophoresis
L - serum-free light chain assay
I - serum immunoglobulins
P - serum protein electrophoresis
Bone marrow biopsy - confirm diagnosis
Imaging
- whole body MRI
- whole body CT
- skeletal survery
95
Q

Features of myeloma on x-ray

A

Punched out lesions
Lytic lesions
Raindrop skull

96
Q

Mx of myeloma

A 
Aim of treatment to control disease - often takes relapsing-remitting course
Chemotherapy
- bortezomid
- thalidomide
- dexamethasone
Stem cell transplantation
VTE prophylaxis
97
Q

Mx of myeloma bone disease

A

Bisphosphonates - suppress osteoclast activity
Radiotherapy - improve pain
Orthopaedic surgery - stabilise bones
Cement augmentation - injecting cement into vertebral fractures/lesions to improve spine stability and pain

98
Q

Complications of myeloma

A 
Infection
Pain
Renal failure
Anaemia
Hypercalcaemia
Peripheral neuropathy
Spinal cord compression
Hyperviscocity
99
Q

Define myeloproliferative disorder

A

Type of bone marrow cancer
Occur due to uncontrolled proliferation of a single type of stem cell
Have the potential to progress and transform into acute myeloid leukaemia
Associated with mutations of JAK2, MPL and CALR genes

100
Q

Types of myeloproliferative disorders

A

Primary myelofibrosis - proliferation of hemopoietic stem cells
Polycythaemia vera - proliferation of erythroid cell line
Essential thrombocythemia - proliferation of megakaryocytic cell line

101
Q

Define myelofibrosis

A

Proliferation of cell line leads to fibrosis of bone marrow
Response to cytokines released by proliferating cells
Haematopoiesis occurs in other areas such as liver and spleen (extramedullary haematopoiesis)
-> hepatomegaly and splenomegaly -> portal hypertension

102
Q

Presentation of myeloproliferative disorders

A 
Initially asymptomatic
Systemic symptoms
- fatigue
- weight loss
- night sweats
- fever
Underlying complications
- anaemia - except in polycythaemia
- splenomegaly (abdo pain)
- portal hypertension ( ascites, varices and abdo pain)
- low platelets (bleeding and petechiae
- thrombosis
- raised RBC
- low WCC (infections)
103
Q

Key features of examination of polycythaemia vera

A

Conjunctival plethora - excessive redness
Ruddy complexion
Splenomegaly

104
Q

FBC findings for myeloproliferative disorders

A 
Polycythaemia vera
- raised Hb
Primary thrombocythemia
- raised platelet count
Myelofibrosis 
- anaemia
- leucocytosis or leukopenia
- thrombocytosis or thrombocytopenia
- tear-dropped shaped RBCs, varying sized RBC (poikilocytosis) and blast cells on blood film
105
Q

Ix for myeloproliferative disorders

A

Bone marrow biopsy
- bone marrow aspiration dry due to fibrosis
Testing for JAK2, MPL and CALR genes

106
Q

Mx of primary myelofibrosis

A

Those with mild disease monitored
Allogenic stem cell transplantation - curative but carries risk
Chemotherapy - help control disease, improve symptoms and slow progression but not curative alone
Supportive

107
Q

Mx of polycythaemia ver

A

Venesection - used to keep haemoglobin in normal range
Aspirin - reduce thrombosis risk
Chemotherapy - control disease

108
Q

Mx of essential thrombocythaemia

A

Aspirin - reduce risk of thrombus formation

Chemotherapy - control disease

109
Q

Features of myelodysplastic syndrome

A

Caused by myeloid bone marrow cells not maturing properly and producing healthy blood cells
Causes low level of blood components that originate from the myeloid cell line
- anaemia
- neutropenia
- thrombocytopenia
Increased risk of transforming into acute myeloid leukaemia

110
Q

Epidemiology of myelodysplastic syndromes

A

More common in patients over 60

Patients who have previously had treatment with chemotherapy and radiotherapy

111
Q

Clinical features of myelodysplastic syndromes

A

May by asymptomatic - incidentally diagnosed on FBC
Present with symptoms of
- anaemia - fatigue, pallor or SOB
- neutropenia - frequent of severe infections
- thrombocytopenia - purpura or bleeding

112
Q

Ix of myelodysplastic syndromes

A

FBC abnormal
Blasts on blood film
Diagnosis confirmed by bone marrow aspiration and biopsy

113
Q

Mx of myelodysplastic syndromes

A

Watchful waiting
Supportive treatment with blood transfusions if severely anaemic
Chemotherapy
Stem cell transplantation

114
Q

Define thrombocytopenia

A

Low platelet count

115
Q

Causes of thrombocytopenia

A 
Problems with production
- sepsis
- B12 or folic acid deficiency
- liver failure
- leukaemia
- myelodysplastic syndrome
Problems with destruction
- medications - sodium valproate, methotrexate, antihistamines, PPIs
- alcohol
- immune thrombocytopenic purpura
- heparin-induced 
- haemolytic uraemic syndrome
116
Q

Presentation of thrombocytopenia

A

Mild may be asymptomatic
Spontaneous bruising and prolonged bleeding times
- nosebleeds, bleeding gums, heavy periods, blood in urine/stools

117
Q

Ddx of abnormal or prolonged bleeding

A

Thrombocytopenia
Haemophilia
Von Willebrand disease
DIC

118
Q

Define immune thrombocytopenic purpura

A

Antibodies created against platelets

  • destruction of platelets
  • low platelet count
119
Q

Mx of ITP

A

Prednisolone
IV immunoglobulins
Rituximab
Splenectomy

120
Q

Define thrombotic thrombocytopenic purpura

A

Tiny blood clots develop throughout small vessels of body

- using up platelets

121
Q

Pathophysiology of TTP

A

Problem with ADAMTS13 protein
- normally inactivated von Willebrand factor reducing platelet adhesion
Shortage of protein leads to von Willebrand factor overactivity
Clots break up RBC -> haemolytic anaemia

122
Q

Mx of TTP

A

Referral to haematologist
Plasma exchange
Steroids
Rituximab

123
Q

Pathophysiology of heparin induced thrombocytopenia

A

Development of antibodies against platelets in response to heparin exposure
- target platelet factor 4 - anti-PF4/heparin antibodies
Bind to platelets and activate clotting mechanisms
-> thrombosis
Breakdown of platelets
-> thrombocytopenia

124
Q

Mx of heparin induced thrombocytopenia

A

Diagnosis by HIT antibody testing
Stop heparin
Use alternative anticoagulant

125
Q

Features of von Willebrand disease

A

Most common inherited cause of abnormal bleeding
Manly autosomal dominant
Deficiency, absence or malfunctioning of glycoprotein von Willebrand factor

126
Q

Mx of von Willebrand factor

A 
Desmopressin - stimulates release of VWF
VWF infusion
Factor VIII
Mx of heavy periods
- tranexamic acid
- mefanamic acid
- norethisterone
- COCP
- Mirena coil
- hysterectomy
127
Q

Features of haemophilia A and B

A

A caused by deficiency of factor VIII
B caused by deficiency of factor IX
X-linked recessive - much more common in males

128
Q

Presentation of haemophillia

A 
Spontaneous haemorrhage
- hemarthrosis
- bleeding into muscles
Presentation in neonatal/early period
- intracranial haemorrhage
- haematomas
- cord bleeding
129
Q

Ix for haemophillia

A

Bleeding scores
Coagulation factor assays
Genetic testing

130
Q

Mx of haemophillia

A

Replacement of clotting factors by IV infusion
- prophylactically
- in response to bleeding
- can result in antibody formation against clotting factor - becomes ineffective
Desmopressin - stimulates release of von Willebrand factor
Antifibrinolytics

131
Q

Risk factors for VTE

A 
Immobility
Recent surgery
Long haul flights
Pregnancy
Hormone therapy with oestrogen (combined oral contraceptive pill and hormone replacement therapy)
Malignancy
Polycythaemia
Systemic lupus erythematosus
Thrombophilia
132
Q

DVT presentation

A 
Unilateral 
Calf or leg swelling
Dilated superficial veins
Tenderness to the calf (particularly over the site of the deep veins)
Oedema
Colour changes to the leg
133
Q

Ix for VTE

A 
Wells score
D-dimer - also raised
- pneumonia
- malignancy
- heart failure
- surgery
- pregnancy
USS doppler of leg
CTPA or VQ for PE
134
Q

Mx of VTE

A

Initial management with LMWH
- started immediately before confirming diagnosis
Long term anticoagulation
- warfarin, NOAC (apixaban, dabigatran or rivaroxaban) or LMWH
Continue anticoagulation for
- 3 months in obvious reversible cause
- beyond 3 months is cause unclear, recurrent VTE or irreversible underlying cause
- 6 months is active cancer

135
Q

Ix for unprovoked DVT

A 
Screen for cancer
- history + examination
- CXR
- bloods - FBC, calcium + LFTs
- urine dipstick
- CT abdo and pelvis in patients over 40
- Mammogram in women over 40
Antiphospholipid antibodies to check for antiphospholipid syndrome
If FHx test for hereditary thrombophilias
136
Q

Define Budd-Chiari syndrome

A

Blood clot develops in hepatic vein blocking outflow of blood
-> acute hepatitis

137
Q

Features of Budd-Chiari syndrome

A

Abdo pain
Hepatomegaly
Ascites

138
Q

Presentation of ALL

A

Pancytopenia

Bone pain

139
Q

Mx of ALL

A

Multi-drug chemotherapy

140
Q

Complications of CLL

A

Infiltration of BM
Reduction in repertoire of antibodies
Altered T cell immunity
Autoimmune phenomena - due to immune dysregulation
- AIHA
- ITP
Transformation to high grade lymphoma - Richters transformation

141
Q

Mx of CLL

A

Not all need treatment - undergo watch and wait period
Treatable but not curable
Treated when clinically indicated
• Hb<10
• Plt<100
• Significant lymphadenopathy, massive splenomegaly
• Doubling time <6 months (time for lymphocyte count to double, only
significant once the lymphocyte count is more than 30)
• Infections
• Autoimmune phenomena
Treatment includes chemotherapy, targeted therapies such as monoclonal antibodies and tyrosine kinase inhibitors

142
Q

Secondary causes of high platelets

A 
Bleeding
Iron deficiency
Inflammation
Infection
Post-surgery
Splenectomy
143
Q

Secondary causes of high RBC

A 
Chronic hypoxia
Smoking
Obstructive sleep apnoea
Altitude
Renal/hepatic tumours
Congenital heart disease
144
Q

Role of lymphatic system

A

Defence against infection
Return of tissue fluid to blood
Absorption of fats and vitamins from GI tract

145
Q

Define aplastic anaemia

A

Failure of all lines resulting in pancytopenia

  • anaemia
  • thrombocytopenia
  • neutropenia
146
Q

Clinical features of aplastic anaemia

A

Anaemia
Bleeding
Infection

147
Q

Ix for aplastic anaemia

A 
FBC
Reticulocyte count
B12 and folate levels
Infection screen
LFTs and U+Es
Thyroid function
Bone marrow aspirate for diagnosis
148
Q

Mx of aplastic anaemia

A

Supportive

  • reduce infection risk
  • treat infection aggressively
  • platelet and red cell transfusions as appropriate
  • ATG and ALG immunotherapy
149
Q

Causes of splenomegaly

A 
Massive
- myeloproliferative disorders - CML, AML and myelofibrosis
- tropical infections - malaria
- HIV
Moderate
- myeloproliferative disorders
- lymphoproliferative disorders - CLL, lymphoma
- amyloidosis
Mild 
- myelo and lymphoproliferative disorders
- portal hypertension
- infections - IE, EBV, viral hepatitis
- haemolytic anaemia
- autoimmune - SLE

Yr4 - Cancer Care (12 decks)

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