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Phase 2a Medicine - Hannah's version > Haematology > Flashcards

Haematology Flashcards

1
Q

What is myeloma?

A

Malignancy of plasma cells leading to progressive bone marrow failure.

It is associated with production of characteristic paraprotein, bone disease, hypercalcaemia and renal failure.

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2
Q

In order to make a diagnosis of myeloma, there must be evidence of mono-clonality. What is mono-clonality?

A

Abnormal proliferation of a single clone of plasma cell leading to excess production of a SINGLE TYPE of immunoglobulin and causing organ dysfunction especially to the kidney.

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3
Q

Myeloma results in immunoparesis. What is immunoparesis?

A

One type of immunoglobulin is produced in excess, and the others are underproduced - IMMUNOPARESIS.

Immunoparesis results in increased susceptibility to infections

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4
Q

What disease often precedes myeloma?

A

Monoclonal gammopathy of undetermined significance (MGUS).

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5
Q

What is MGUS?

A

Monoclonal gammopathy of undetermined significance

A common disease with paraprotein present in the serum but no myeloma.

Often asymptomatic.

<10% plasma cells in the bone marrow.

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6
Q

In approximately 2/3 of people with myeloma, what might their urine contain?

A

Immunoglobulin light chains with kappa or lamda lineage.

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7
Q

What is the clinical presentation of myeloma?

A

OLD CRAB

  • Old age
  • Calcium elevated
  • Renal failure
  • Anaemia
  • Bone lytic lesions
  • Recurrent bacterial infections
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8
Q

Give 3 symptoms of myeloma.

A
  1. Tiredness.
  2. Bone/back pain.
  3. Infections.
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9
Q

Give 4 signs of myeloma.

A

CRAB!

  1. Calcium is elevated.
  2. Renal failure.
  3. Anaemia.
  4. Bone lesions.
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10
Q

Why is calcium elevated in myeloma?

A

There is increased bone resorption and decreased formation meaning there is more calcium in the blood.

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11
Q

Why might someone with myeloma have renal failure?

A

Nephrotic syndrome - due to light chain deposition in kidneys

Results in THIRST due to lack of water retention

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12
Q

Why might someone with myeloma get bone lytic lesions / back pain?

A

Malignant plasma cells result in;

  • Activation of osteoclasts, thus increasing bone turnover and causing bone breakdown and lytic lesions
  • Inhibition of osteoblasts, thus decreasing new bone formation
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13
Q

Why might someone with myeloma have anaemia?

A

The bone marrow is infiltrated with plasma cells.

Consequences of this are anaemia, infections (neutropenia) and bleeding (thrombocytopenia).

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14
Q

What investigations might you do in someone who you suspect has myeloma?

A
  1. Blood count - hypercalcaemia, anaemia, thrombocytopenia, neutropenia
  2. Bone marrow aspirate (trephine biopsy) - looking for plasma cell infiltration
  3. Serum / urine electrophoresis - monoclonal protein band
  4. Skeletal survey - “punched out” bone lytic lesions
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15
Q

What would you expect to see on the blood film taken from someone with myeloma?

A

Rouleaux formation (aggregations of RBCs).

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16
Q

What would the a blood test of a patient with myeloma show?

A
  • Normocytic normochromic anaemia
  • Raised ESR
  • High calcium
  • High alkaline phosphatase
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17
Q

What are you looking for on a bone marrow biopsy taken from someone with myeloma?

A

Increased plasma cells.

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18
Q

What are you looking for on serum and urine electrophoresis in a patient with myeloma?

A

Monoclonal protein band.

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19
Q

What are you looking for on an X-ray taken from someone with myeloma?

A

Bone lesions.

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20
Q

What is the treatment for MGUS and asymptomatic myeloma?

A

Watch and wait.

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21
Q

Describe the treatment for symptomatic myeloma.

  • Pain
  • Fractures
  • Anaemia
  • Further renal damage prevention
  • Renal failure
  • Infections
  • Cancer
A
  • Analgesia for bone pain
  • Bisphosphonates to reduce fractures
  • RBC transfusion and erythropoietin for anaemia
  • 3L/day fluid to prevent renal damage
  • Dialysis for acute renal failure
  • Broad spectrum antibiotics for infections
  • Chemotherapy (CTD = unfit people, VAD = fit people)
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22
Q

What is lymphoma?

A

A malignant proliferation of lymphocytes which accumulate in lymph nodes and cause lymphadenopathy

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23
Q

Although predominantly in the lymph nodes, lymphoma is systemic. What other organs might it effect?

A
  1. Blood.
  2. Liver.
  3. Spleen.
  4. Bone marrow.
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24
Q

Give 4 risk factors for lymphoma.

A
  1. Primary immunodeficiency
  2. Secondary immunodeficiency (e.g. HIV)
  3. Infection (e.g. EBV, HTLV-1)
  4. Autoimmune disorders (e.g. SLE)
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25
Q

Describe the pathophysiology of lymphoma.

A

There is impaired immunosurveillance and infected B cells escape regulation and proliferate. (This is just a theory).

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26
Q

Give 4 symptoms of lymphoma.

A
  1. Cervical lymphadenopathy (feel ‘rubbery’)
  2. Symptoms of compression syndromes.
  3. General systemic ‘B’ symptoms (e.g. weight loss, night sweats, malaise)
  4. Hepatosplenomegaly
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27
Q

What investigations might you do in someone who you suspect has lymphoma?

A
  1. Bloods
    - High ESR / Low Hb
    - High serum lactate dehydrogenase
  2. Lymph node biopsy.
    - Miror image nuclei Reed-Sternberg cells
  3. CT / MRI chest, abdomen, pelvis - staging
  4. Immunophenotyping.
  5. Cytogenetics.
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28
Q

What are the two sub-types of lymphoma?

A
  1. Hodgkins lymphoma.
  2. Non-hodgkins lymphoma.
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29
Q

What are the symptoms of Hodgkins lymphoma?

A
  1. Painless lymphadenopathy.
  2. Presence of ‘B’ symptoms e.g. night sweats, weight loss.

(Some patients, particularly young women, present with cough due to mediastinal lymphadenopathy)

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30
Q

What is needed for diagnosis of Hodgkins lymphoma?

A

Presence of Reed-sternberg cells.

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31
Q

Describe the staging of Hodgkins lymphoma.

A

Stage 1: confined to a single lymph node region.

Stage 2: Involvement of two or more nodal areas on the same side of the diaphragm.

Stage 3: involvement of nodes on both sides of the diaphragm.

Stage 4: Spread beyond the lymph nodes e.g. liver.

Each stage is either ‘A’ (no systemic symptoms other than pruritis) ‘B’ (presence of B symptoms, e.g. fever, weight loss, etc.)

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32
Q

What is the treatment for stage 1A - 2A Hodgkins lymphoma?

A

Short course combination chemotherapy (ABVD) followed by radiotherapy.

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33
Q

What is the treatment for stage 2B - 4B Hodgkins lymphoma?

A

Longer course of combination chemotherapy (ABVD)

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34
Q

What is the ABVD combination chemotherapy treatment?

A

A - adriamycin

B - bleomycin

V - vinblastine

D - dacarbazine

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35
Q

What are the possible complications of treatment for Hodgkins lymphoma?

A
  1. Secondary malignancies (radiotherapy increases risk)
  2. IHD (radiotherapy)
  3. Infertility (chemotherapy)
  4. Nausea (chemotherapy)
  5. Alopecia (chemotherapy)
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36
Q

Describe low grade non-Hodgkins lymphoma.

A
  • Slow growing
  • Advanced at presentation
  • Systemic B symptoms
  • Pancocytopenia (anaemia, infection, bleeding)
  • Often incurable
  • Median survival is 10 years
  • e.g. follicular lymphoma
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37
Q

What is the treatment for low grade non-hodgkins lymphoma?

A

If asymptomatic - do nothing.

If symptomatic - Radiotherapy, combination chemotherapy and mAb may be used

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38
Q

Describe high grade non-hodgkins lymphoma.

A
  • Aggressive
  • Nodal presentation, patient unwell
  • Pancocytopenia
  • Systemic B symptoms
  • Often curable
  • e.g. diffuse large B-cell lymphoma (DLBCL)
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39
Q

Describe the treatment for high grade non-hodgkins lymphoma.

A

Early - 3 month R-CHOP chemotherapy and radiotherapy.

Advanced - 6 month R-CHOP chemotherapy and radiotherapy

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40
Q

What is R-CHOP chemotherapy regimen?

A

R - rituximab (monoclonal antibody - mAb)

C - cyclophosphamide

H - hydroxy-daunorubicin

O - vincristine (oncovin - brand name)

P - prednisolone

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41
Q

What is leukaemia?

A

Malignant proliferation of haemopoietic stem cells in the bone marrow which are non-functional

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42
Q

Name 4 sub-types of leukaemia.

A
  1. AML - acute myeloid leukaemia.
  2. CML - chronic myeloid leukaemia.
  3. ALL - acute lymphoblastic leukaemia.
  4. CLL - chronic lymphoblastic leukaemia.
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43
Q

Give 5 symptoms of leukaemia.

A
  1. Anaemia.
  2. Infection.
  3. Bleeding.
  4. Hepatomegaly.
  5. Splenomegaly.
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44
Q

Why are anaemia, infection and bleeding symptoms of leukaemia?

A

Bone marrow failure

Anaemia - low Hb

Infection - low WCC

Bleeding - low platelets

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45
Q

Why are hepatomegaly and splenomoegaly symptoms of leukaemia?

A

Liver / spleen infiltration

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46
Q

What investigations might you do on someone who you suspect has leukaemia?

A
  1. Blood film (WCC is high, blast cells on film and in bone marrow)
  2. Bone marrow biopsy.
  3. Lymph node biopsy.
  4. Immunophenotyping.
  5. Cytogenetics.
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47
Q

What is acute myeloid leukaemia?

A

Neoplastic proliferation of myeloblasts or myeloid stem cells

It causes death in 2 months if untreated

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48
Q

What can increase the risk of developing AML?

A
  1. Preceding haematological disorders.
  2. Prior chemotherapy.
  3. Exposure to ionising radiation.
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49
Q

How do you diagnose AML?

A
  • Raised WCC (but can be normal / low)
  • Few blast cells in peripheral blood so diagnose using bone marrow biopsy
  • Differentiate from ALL by microscopy, immunophenotyping and molecular methods
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50
Q

Describe the treatment for AML.

A
  • Supportive care.
  • Chemotherapy: curative v palliative.
  • Bone marrow transplant.
  • Prophylactic antimicrobials
  • Allopurinol (prevent tumour lysis syndrome)
  • IV fluids through Hickman line
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51
Q

What is CML?

A

Chronic myeloid leukaemia

There is uncontrolled clonal proliferation of myeloid cells (basophils, eosinophils and neutrophils)

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52
Q

What would the FBC and bone marrow aspirate from someone with CML look like?

A
  • Very high WBCs with whole spectrum of myeloid cells
  • Low Hb
  • Hypercellular aspirate
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53
Q

What chromosome is present in >80% of people with CML?

A

Philadelphia chromosome.

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54
Q

What is the treatment for CML?

A

Oral imatinib - tyrosine kinase inhibitors

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55
Q

What is ALL?

A

Acute lymphoblastic leukaemia

Uncontrolled proliferation of immature lymphoblast cells.

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56
Q

What is the treatment for ALL?

A
  • Blood / platelet transfusions
  • Prophylactic antimicrobials
  • Allopurinol (prevents tumour lysis syndrome)
  • IV fluids through Hickman line
  • Chemotherapy
  • Marrow transplant
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57
Q

What is CLL?

A

Chronic lymphocytic leukaemia

Proliferation of B lymphocytes leading to the accumulation of mature B cells that have escaped apoptosis

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58
Q

What is the treatment for CLL?

A
  1. Do nothing (cancer may regress)
  2. Chemotherapy / radiotherapy
  3. mAb - human IV immunoglobulins
  4. Bone marrow transplant.
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59
Q

Name the 3 broad categories of red cell disorders.

A
  1. Haemoglobinopathies.
  2. Membranopathies.
  3. Enzymopathies.
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60
Q

What is normal adult haemoglobin (HbA) made of?

A

Haem + 2 alpha chains + 2 beta chains

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61
Q

What is foetal haemoglobin (HbF) made of?

A

Haem + 2 alpha chains + 2 gamma chains

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62
Q

What is haemoglobin S (HbS)?

A

Haemoglobin S is a variant of Hb arising from a point mutation in the beta globin gene.

The mutation leads to a single amino acid change, valine -> glutamine.

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63
Q

What is sickle cell disease?

A

A haemoglobin disorder of quality.

HbS polymerises -> sickle shaped RBC.

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64
Q

What is the advantage of being a carrier of sickle cell disease?

A

Carriage offers protection against falciparum malaria.

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65
Q

Describe the inheritance pattern of sickle cell disease.

A

Autosomal recessive.

Sickle cell disease is homozygous SS.

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66
Q

If both parents are carriers of the sickle trait. What is the chance that their first child will have sickle cell disease?

A

Their offspring have a 1/4 chance of being affected with a sickle cell disease. (50% chance of being a carrier).

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67
Q

How long do sickle cells last for?

A

5-10 days - this explains why sickle cell disease is described as haemolytic.

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68
Q

Give 4 acute complications of sickle cell disease.

A
  1. Very painful crises (due to vaso-occlusion and avascular necrosis)
  2. Stroke in children (CNS infarction)
  3. Cognitive impairment (CNS infarction)
  4. Acute chest syndrome (caused by infections, fat emboli from necrotic bone marrow, and pulmonary infarctions)
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69
Q

Give 6 chronic complications of sickle cell disease.

A
  1. Renal impairment (chronic tubulointerstitial nephritis)
  2. Pulmonary hypertension.
  3. Joint damage.
  4. Spontaneous abortion (impaired placental blood flow)
  5. Retinopathy, vitreous haemorrhage, retinal detachments
  6. Hepatomegaly, liver dysfunction (due to trapping of sickle cells)
  7. Anaemia (chronic haemolysis)
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70
Q

How can you diagnose sickle cell disease?

A

Blood count - raised reticulocyte count

Blood films - sickled erythrocytes, positive sickle solubility test

Hb electrophoresis - 80%+ HbS and absent HbA

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71
Q

Describe the treatment for sickle cell disease.

How do you treat painful attacks?

A
  1. Transfusion (to treat acute chest syndrome, acute anaemia, stroke, HF)
  2. Hydroxycarbamide (to increase HbF concentration)
  3. Stem cell transplant.
  4. Avoid precipitating factors (e.g. cold, infection, dehydration) - prophylaxis vaccines
  5. Folic acid

Treat painful attacks with IV fluids, analgesia, oxygen and antibiotics (if required)

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72
Q

What is the significance of parvovirus for someone with sickle cell disease?

A

Parvovirus is a common infection in children.

It leads to decreased RBC production and can cause a dramatic drop in Hb in patients who already have a reduced RBC lifespan.

This can be dangerous for someone with sickle cell.

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73
Q

What is thalassaemia?

A

A haemoglobin disorder of quantity.

There is reduced synthesis of one or more globin chains, leading to reduced RBC

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74
Q

Describe beta thalassaemia

A
  • Very few beta chains
  • Excess alpha chains
  • Alpha chains combine with delta and gamma chains, resulting in increases HbA2 (Hb delta) and HbF (Hb gamma)
  • Caused by point mutations, resulting in production of highly unstable beta globins
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75
Q

What are the clinical classifications of beta thalassaemia?

A
  1. Thalassaemia major.
  2. Thalassaemia intermedia.
  3. Thalassaemia minor (carrier / heterozygote).
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76
Q

Describe beta-thalassaemia minor.

A
  • Heterozygote
  • Asymptomatic
  • Anaemia is mild / absent
  • Hypochromic microcytic RBCs with LOW MCV
  • Can be confused with iron deficiency, but distinguished since serum ferritin and iron stores are normal
  • Hb electrophoresis shows raised HbA2 and HbF
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77
Q

Describe beta-thalassaemia intermedia

A
  • Those who are symptomatic with moderate anaemia, but do not require regular transfusions
  • Splenomegaly
  • Bone deformities
  • Recurrent leg ulcers
  • Gallstones
  • Infections
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78
Q

Describe beta-thalassaemia major

A
  • Presents in children (<1yr) with homozygous beta-thalassaemia
  • Failure to thrive and recurrent bacterial infections
  • Severe anaemia from 3-6 months (when switch from gamma to beta chain production should occur)
  • Extramedullary haematopoiesis, resulting in hepatosplenomegaly and bone expansion, leading to thalassaemic faces
  • Life-long transfusion dependant
  • Hypertrophy of ineffective bone marrow, thus bone abnormalities
  • ‘Hair-on-end’ skull x-ray due to increased marrow activity
  • Very low MCV - microcytic
  • Irregular hypochromic RBCs
  • Normal serum ferritin
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79
Q

Why is it important to monitor iron levels in someone with beta thalassaemia major?

A

There is a risk of iron overload from the regular trasnfusions.

Excess iron will be deposited in various organs (e.g. the liver and spleen) and cause fibrosis.

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80
Q

Describe the inheritance pattern for membranopathies.

A

Autosomal dominant.

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81
Q

Name 2 common membranopathies.

A
  1. Spherocytosis.
  2. Elliptocytosis.
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82
Q

Briefly describe the physiology of membranopathies.

A

Deficiency of red cell membrane proteins caused by genetic lesions

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83
Q

What are enzymopathies?

A

Enzyme deficiencies lead to shortened RBC lifespan.

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84
Q

Name a common enzymopathy.

A

G6PD deficiency.

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85
Q

Give 3 signs of G6PD deficiency.

A

Crises characterised by:

  1. Haemolysis.
  2. Jaundice.
  3. Anaemia.
86
Q

What is anaemia?

A

A decrease in the amount of Hb in the blood below the reference range.

87
Q

What is the function of Hb?

A

It carries and delivers oxygen to tissues.

88
Q

What organs / events are responsible for removal of RBCs?

A
  1. Spleen.
  2. Liver.
  3. Bone marrow.
  4. Blood loss.
89
Q

Give 3 causes of microcytic anaemia.

A
  1. Iron deficiency.
  2. Anaemia of chronic disease.
  3. Thalassaemia.
90
Q

Give 3 causes of normocytic anaemia.

A
  1. Acute blood loss.
  2. Anaemia of chronic disease.
  3. Combined hematinic deficiency.
  4. Renal failure.
  5. Pregnancy.
91
Q

Give 3 causes of macrocytic anaemia.

A
  1. B12/folate deficiency (megaloblastic)
  2. Alcohol excess/liver disease (non-megaloblastic)
  3. Hypothyroid.

Megaloblastic = presence of erythroblasts with delayed nuclear maturation because of delayed DNA synthesis, resulting in large, anucleated cells

92
Q

Where is B12 absorbed?

A

The terminal ileum.

93
Q

Explain how pernicious anaemia leads to B12 deficiency.

A

Pernicious anaemia leads to a loss of parietal cells -> reduced intrinsic factor production -> vitamin B12 malabsorption.

B12 binds to intrinsic factor, produced by parietal cells, and is absorbed in the terminal ileum.

Pernicious anaemia leads to loss of parietal cells, thus there is less intrinsic factor produced, resulting in B12 malabsorption

94
Q

How do you diagnose pernicous anaemia?

A

Blood film / count

  • Macrocytic RBCs
  • Low serum B12
  • Low Hb
  • Low reticulocyte count
  • Intrinsic factor antibodies - DIAGNOSTIC but low sensitivity
95
Q

Give 5 causes of iron deficiency.

A
  1. Blood loss (menorrhagia, GI bleeding, hookworm)
  2. Poor absorption (Coeliac)
  3. Decreased intake in diet.
  4. Hook worm!
  5. Breastfeeding (low iron in breast milk)
96
Q

Give 5 signs and symptoms of anaemia.

A
  1. Fatigue.
  2. Faintness.
  3. Breathlessness.
  4. Reduced exercise tolerance.
  5. Headaches
  6. Pallor
97
Q

What investigations might you do in someone with anaemia?

A
  1. Blood tests: FBC and blood film.
  2. Biopsies.
  3. Reticulocyte count.
  4. B12 levels.
  5. Serum ferritin.
98
Q

What is the treatment for anaemia?

A

Treat the underlying cause

  • If iron deficient - ferrous sulphate
  • If folate deficient - folic acid tablets WITH B12
  • If low B12 due to malabsorption - injections
  • If low B12 because of diet - oral B12
  • To replenish B12 stores - IM hydroxycobalamin
99
Q

What is polycythaemia?

A

Increase in RBCs

  • Increased Hb
  • Increased haematocrit
  • Increased red cell count

(All these measurements are concentrations, thus depend on plasma volume as well)

100
Q

What hormone is responsible for regulating RBC production?

A

Erythropoietin.

101
Q

What stimulates EPO?

A

Tissue hypoxia.

102
Q

Name a primary cause of absolute polycythaemia. What is absolute polycythaemia?

A

Polycythaemia vera (PV) - over reactive bone marrow

Absolute - increase in RBC mass

103
Q

Give 3 secondary causes of absolute polycythaemia.

A

HYPOXIA

  1. Heavy smoking.
  2. Lung disease.
  3. Cyanotic heart disease.
  4. High altitude.
104
Q

Describe the pathophysiology for PV

A

Cells (RBCs, WBCs, platelets) do not need erythropoietin to avoid apoptosis, thus proliferate excessively, raising haematocrit and causing hyperviscosity and thrombosis

105
Q

Describe the clinical presentation for polycythaemia vera

A
  • May only be detected on FBC - asymptomatic
  • Symptoms due to hyperviscosity;
  • Headaches
  • Itching (especially when warm)
  • Tiredness
  • Dizziness
  • Tinnitus
  • Visual disturbance
  • Erythromelalgia (burning sensation in fingers and toes)
  • Gout
  • Hypertension
  • Angina
  • Intermittent claudication
  • Hepatosplenomegaly (distinguishes PV from secondary causes)
106
Q

What investigations do you do if you suspect a patient has polycythaemia vera?

A

Blood count

  • Raised WCC and platelets (distinguishes from secondary causes)
  • Raised Hb

Genetic screen

  • Presence of JAK2 mutation

Bone marrow biopsy

  • Prominent erythroid, granulocytic, and megakaryocytic proliferation
107
Q

What is the treatment for polycythaemia?

A
  1. If a secondary cause treat the underlying cause (e.g. smoking cessation)
  2. If a primary cause, treatment aims to maintain a normal blood count and prevent complications
    - Venesection (removal of ~450mL blood weekly)
    - Chemotherapy (if venesection is untolerated)
    - Low dose aspirin (with venesection / chemo)
108
Q

What is neutrophilia?

A

Too many neutrophils

109
Q

Give 3 causes of neutrophilia.

A
  1. Infection.
  2. Inflammation.
  3. CML.
  4. Cancer.
110
Q

What is lymphocytosis?

A

Too many lymphocytes.

111
Q

Give 3 causes of lymphocytosis.

A
  1. Viral infections.
  2. Inflammation.
  3. Malignancy.
  4. CLL.
112
Q

What is thrombocytopenia?

A

Not enough platelets.

113
Q

Give 2 causes of thrombocytopenia.

A
  1. Production failure (e.g. marrow suppression, marrow failure)
  2. Increased removal (e.g. immune response (ITP), consumption (DIC), splenomegaly)
114
Q

What is thrombocytosis?

A

Too many platelets.

115
Q

What is neutropenia?

A

Not enough neutrophils.

116
Q

What is the major risk associated with being neutropenic?

A

Susceptible to infection.

117
Q

Give 3 causes of neutropenia.

A
  1. Marrow failure.
  2. Marrow infiltration.
  3. Marrow toxicity.
118
Q

Where are platelets produced?

A

In the bone marrow - they are fragments of megakaryocytes.

119
Q

What hormone regulates platelet production?

A

Thrombopoietin - produced mainly in the liver.

120
Q

What is the lifespan of a a platelet?

A

7 - 10 days.

121
Q

What organ is responsible for platelet removal?

A

The spleen.

122
Q

What can cause platelet dysfunction?

A
  1. Reduced platelet number (thrombocytopenia).
  2. Reduced platelet function.
123
Q

Platelet dysfunction: what can cause decreased platelet production?

A
  1. Congenital causes e.g. malfunctioning megakaryocytes.
  2. Infiltration of bone marrow e.g. leukaemia.
  3. Alcohol.
  4. Infection e.g. HIV/TB.
124
Q

Platelet dysfunction: what can cause increased platelet destruction?

A
  1. Autoimmune e.g. ITP.
  2. Hypersplenism.
  3. Drug related e.g. heparin induced.
  4. DIC and TTP -> increased consumption.
125
Q

Platelet dysfunction: what can cause reduced platelet function?

A
  1. Congenital abnormality.
  2. Medication e.g. aspirin.
  3. VWF disease.
  4. Uraemia.
126
Q

Give 3 symptoms of platelet dysfunction.

A
  1. Mucosal bleeding.
  2. Easy bruising.
  3. Petechiae/purpura.
127
Q

Give 4 causes of bleeding.

A
  1. Trauma.
  2. Platelet deficiency e.g. thrombocytopenia.
  3. Platelet dysfunction e.g. aspirin induced.
  4. Vascular disorders.
128
Q

What is the definition of febrile neutropenia.

A

Temperature >38°C in a patient with neutrophil count <1x10^9/L.

129
Q

Give 4 risk factors for febrile neutropenia.

A
  1. If the patient had chemotherapy <6 weeks ago.
  2. Any patient who has had a stem cell transplant / high dose chemotherapy <1 year ago.
  3. Any haematological condition causing neutropenia.
  4. Bone marrow infiltration.
  5. Patients on methotrexate, carbimazole, clozapine
130
Q

What is the presentation of febrile neutropenia?

A
  1. Pyrexia, 38°C.
  2. Generally unwell - cough, sore throat, abdo pain, diarrhoea
  3. Confusion.
  4. Hypotensive.
  5. Tachycardic.
  6. Sweats / rigors
131
Q

Describe the management of febrile neutropenia.

A
  1. Thorough history and examination.
  2. Bloods.
  3. Broad spectrum IV antibiotics within 1 hour!
  4. DO NOT CATHETERISE IF NEUTROPENIC
132
Q

Give a risk factor for spinal cord compression.

A

Any malignancy that can cause compression e.g. bone metastasis, tumour extension, etc.

133
Q

Describe the presentation of spinal cord compression.

A
  1. Back pain.
  2. Weakness in legs.
  3. Inability to control bladder.
  4. Spastic paresis.
  5. Sensory level.
  6. Saddle paraesthesia
  7. Decreased perianal sensation and anal tone
134
Q

Describe the management of spinal cord compression.

A
  1. Bed rest.
  2. High dose steroids. - oral dexamethasone
  3. Analgesia.
  4. Urgent MRI of the whole spine.
135
Q

What is tumour lysis syndrome?

A

Life threatening metabolic derangement, occuring when malignant cells breakdown, resulting in neuro, cardio, and renal complications

Break down of malignant cells -> content release -> metabolic disturbances; can cause hyperuricaemia, hyperkalaemia, hyperphosphataemia, and hypocalcaemia.

136
Q

Give 3 risk factors for tumour lysis syndrome.

A
  1. High tumour burden.
  2. Pre-existing renal failure.
  3. Increasing age.
  4. Drugs that increase uric acid formation
137
Q

Describe the treatment of tumour lysis syndrome.

A
  1. Aggressive hydration.
  2. Monitor electrolytes.
  3. Drugs to reduce uric acid production e.g. allopurinol.
138
Q

What is hyperviscosity syndrome?

A

Increase in blood viscosity usually due to high levels of immunoglobulins.

Can also be due to high cell numbers (e.g. leukaemia, polycythaemia)

139
Q

Give 2 consequences of hyperviscosity syndrome.

A
  1. Vascular stasis.
  2. Hypoperfusion.
140
Q

Describe the presentation of hyperviscosity syndrome.

A
  1. Mucosal bleeding.
  2. Visual change (hypoperfusion to retina)
  3. Neurological disturbances (e.g. vertigo, hearing loss, ataxia)
  4. Breathlessness.
  5. Fatigue.
  6. Brusing
141
Q

What investigations might you do in someone who you suspect has hyperviscosity syndrome?

A
  1. FBC and blood film; look for rouleaux formation.
  2. U&E.
  3. Immunoglobulins.
  4. Plasma viscosity level
  5. CT scan (to exclude other causes of neurological signs)
142
Q

What is the treatment for hyperviscosity syndrome?

A
  1. Keep hydrated!
  2. Avoid blood transfusion (will make blood thicker)
  3. Treat the underlying cause.
  4. Plasmapharesis - remove circulating Ig (paraproteins) to decrease serum viscosity
143
Q

Give 5 signs/symptoms of hypercalcaemia.

A
  1. Confusion.
  2. Bone pain.
  3. Constipation.
  4. Nausea.
  5. Abdominal pain.
  6. Polyuria
  7. Renal stones
144
Q

What might you see in the ECG taken from someone with hypercalcaemia.

A

Shortened QT interval.

Hypercalcaemia = risk of MI.

145
Q

What is the treatment for hypercalcaemia?

A
  • IV hydration (3-4L/day)
  • Bisphosphonates (reduces Ca2+ production)
146
Q

What does rituximab target?

A

Targets CD20 on the surface of B.

147
Q

What is the characteristic genetic abnormality in chronic myeloid leukaemia?

A

t(9; 22) - philadelphia chromosome.

148
Q

How is myeloma bone disease usually assessed?

A

X-ray.

149
Q

What is the most common cause of microcytic anaemia?

A

Iron deficiency.

150
Q

What is the affect of sickle cell anaemia on reticulocyte count?

A

Reticulocyte count is raised.

151
Q

Why is reticulocyte count raised in sickle cell anaemia?

A

Sickle cell disease is haemolytic, there is increased degradation of RBC’s. Production therefore increases in order to keep up with degradation and so reticulocyte count is raised.

152
Q

What clotting factors depend on vitamin K?

A

2, 7, 9 and 10.

153
Q

Haemophilia A is due to deficiency of what clotting factor?

A

Factor 8 deficiency.

154
Q

Haemophilia B is due to deficiency of what clotting factor?

A

Factor 9 deficiency.

155
Q

Give 6 symptoms of ALL.

A
  1. Bone pain (bone marrow infiltration)
  2. Recurrent infections (neutropenia).
  3. Pale and tired (anaemia).
  4. Bruising (low platelets).
  5. Hepatosplenomegaly (liver / spleen infiltration)
  6. Lymphadenopathy (node infiltration)
  7. Headaches / cranial nerve palsies (CNS infiltration)
156
Q

Is ALL more common in adults or children?

A

ALL is mainly a childhood disease.

Most common between 2-4yrs

157
Q

What kind of anaemia could methotrexate cause?

A

Macrocytic due to folate deficiency.

Methotrexate is an anti-folate drug

158
Q

Give 4 causes of folate deficiency.

A
  1. Dietary.
  2. Malabsorption (e.g. Coeliac)
  3. Increased requirement )e.g. in pregnancy)
  4. Folate antagonists (e.g. methotrexate)
159
Q

Give 3 signs of haemolytic anaemia.

A
  1. Pallor.
  2. Jaundice.
  3. Splenomegaly.
  4. Leg ulcers
  5. Gallstones
160
Q

Give 4 causes of haemolytic anaemia.

A
  1. GP6D deficiency - enzymopathy
  2. Sickle cell anaemia / thalassaemia - haemoglobinopathies
  3. Spherocytosis / elliptocytosis - membranopathies
  4. Autoimmune haemolytic anaemia.
161
Q

Give 3 things that can cause coagulation disorders.

A
  1. Vitamin K deficiency.
  2. Liver disease.
  3. Congenital e.g. haemophilia.
162
Q

How does warfarin work?

A

It antagonises vitamin K and so you get a reduction in clotting factors 2, 7, 9 and 10.

163
Q

How does heparin work?

A

It activates antithrombin which then inhibits thrombin and factor Xa.

164
Q

What is disseminated intravascular coagulation (DIC)?

A

Systematic activation of the coagulation cascade -> widespread generation of fibrin -> thrombosis and multiorgan failure

Consumption of platelets and coagulation factor consumption in one area leads to thrombocytopenia in other areas -> increased bleeding

165
Q

Give 3 causes of disseminated intravascular coagulation (DIC).

A
  1. Sepsis.
  2. Major trauma.
  3. Malignancy.
166
Q

What is TT, PTT, and APTT? How are these affected in someone with DIC?

A

TT - thrombin time

PTT - prolonged thrombin time

APTT - activated partial thromboplastin time

All increased.

167
Q

What is the affect on fibrinogen and FDPs in someone with disseminated intravascular coagulation (DIC)?

A

Fibrinogen - decreased

Fibrin degradation products (FDP) - elevated, due to intense fibrinolytic activity stimulated by presence of fibrin in circulation

168
Q

Give 5 risk factors for DVT.

A
  1. Increasing age.
  2. Obesity.
  3. Pregnancy.
  4. OCP (hyper-coagulability).
  5. Major surgery.
  6. Immobility.
  7. Past DVT.
169
Q

Give 3 symptoms of DVT.

A
  • Unilateral warm, tender, painful, swollen leg.
  • Cyanotic discolouration of limb (with complete occlusion)
  • Severe oedema (with complete occlusion)
170
Q

What forms the differential diagnosis for a DVT?

A

Cellulitis.

171
Q

What investigations might you do in someone to see if they have a DVT?

A
  1. D-dimer in those patients with a low clinical probability - normal D-dimer excludes DVT
  2. Compression ultrasound
    - If you can compress popliteal vein = no DVT
    - If you cannot shut popliteal vein = DVT

D-dimer = fibrinogen degradation product released when clot starts dissolving

172
Q

What is the name of the score used to determine someones probability of having a DVT?

A

The Wells score.

173
Q

The Wells score determines someones clinical probability of having a DVT. Give 3 factors the score takes into account.

A
  1. Active cancer.
  2. Recently bedridden or major surgery.
  3. Tenderness along deep venous system.
  4. Swollen leg/calf.
  5. Unilateral pitting oedema.
174
Q

Describe the management for a DVT.

A

Aim of management is to prevent a PE!

  • Anticoagulants e.g. warfarin/heparin for minimum of 5 days
  • Oral warfarin (with target INR 2-3) for 6 months
  • Compression stockings
  • IVC filters (to reduce risk of PE)
175
Q

Explain why philadelphia chromosome causes CML.

A

Philadelphia chromosome leads to a fusion gene that has tyrosine kinase activity and enhanced phosphorylating activity -> stimulates cell division

176
Q

Where would you normally take a bone marrow biopsy from?

A

Posterior iliac crest.

177
Q

What is the most important medical treatment for DVT prophylaxis?

A

LMWH (low molecular weight heparin)

178
Q

What is the affect of iron deficiency anaemia on iron binding capacity?

A

Iron binding capacity will be raised.

179
Q

Why might measuring serum ferritin be inaccurate for looking at iron levels?

A

Ferritin is an acute phase protein and so its concentration will increase in response to inflammation.

180
Q

Describe the treatment for iron deficiency anaemia.

A

Ferrous sulphate tablets.

181
Q

What is aplastic anaemia?

A

When bone marrow stem cells are damaged -> pancytopenia.

182
Q

Give 3 symptoms of iron-deficiency anaemia.

A
  1. Koilonychia.
  2. Brittle hair and nails.
  3. Atrophic glossitis.
  4. Tiredness, reduced exercise tolerance.
  5. SOB.
183
Q

What is the difference between Raynaud’s disease and Raynaud’s phenomenon?

A

Raynaud’s disease is idiopathic.

Raynaud’s phenomenon can be due to SLE, scleroderma, RA, drugs e.g. beta blockers.

184
Q

Describe the pathophysiological mechanism behind Raynaud’s disease.

A

Peripheral digital ischaemia due to intermittent spasm in arteries that supply the fingers/toes.

Precipitated by cold/stress.

185
Q

Describe the colour changes that are seen in Raynaud’s.

A
  • Pale - due to vasoconstriction.
  • Cyanotic - due to deoxygenation.
  • Red - due to hyperaemia.
186
Q

Describe the treatment for Raynaud’s disease.

A
  1. Physical protection.
  2. Vasodilators.
  3. Nifedipine (CCB).
  4. Stop smoking.
187
Q

State two features with regards to red blood cell appearance that would make you think a patient had anaemia due to iron deficiency.

A
  1. Hypochromia (pale).
  2. Microcytosis.
  3. Poikilocytosis (variation in shape)
  4. Anisocytosis (variation in size)
188
Q

Suggest 3 ways in which multiple myeloma can lead to AKI.

A
  1. Deposition of light chain.
  2. Hypercalcaemia.
  3. Hyperuricaemia.
189
Q

In extreme cases, patients with myeloma can present with blurred vision, gangrene and bleeding. What is the pathology behind this?

A

Paraproteins form aggregates in the blood and change the viscosity.

190
Q

Why are patients with myeloma susceptible to recurrent infections?

A

There is a reduction in polyclonal immunoglobulin levels.

191
Q

What kind of anaemia is seen in patients with multiple myeloma?

A

Normochromic normocytic.

192
Q

What chemotherapy regime is used in patients with myeloma?

A

VAD or CTD.

VAD = Vincristine, Adriamycin, Dexamethasone

CTD = Cyclophosphamide, Thalidomide, Dexamethasone

193
Q

What combination chemotherapy regime is commonly used in patients with non hodgkin’s lymphoma?

A

RCHOP.

194
Q

Give 3 environmental causes of leukaemia.

A
  1. Radiation exposure.
  2. Chemicals e.g. benzene compounds.
  3. Drugs.
195
Q

Give 3 signs of anaemia.

A
  1. Pale skin and mucous membranes.
  2. Tachycardia.
  3. Bounding pulse.
196
Q

Describe the appearance of RBC’s seen in iron deficiency anaemia.

A

Hypochromic microcytic.

197
Q

What might you see on a blood count taken from someone with beta thalassaemia major?

A
  1. Raised reticulocyte count.
  2. Microcytic anaemia.
198
Q

What can precipitate sickling in sickle cell anaemia?

A

Trauma, cold, stress, exercise.

199
Q

Why does sickle cell anaemia not present until after 6 months of age?

A

HbF is not affected by sickle cell anaemia as it is made up of 2 alpha and 2 gamma chains.

200
Q

What drug can be used to prevent painful crises in people with sickle cell anaemia?

A

Hydroxycarbamide.

201
Q

Give 3 clinical features of a patient with a membranopathy.

A
  1. Jaundice.
  2. Anaemia.
  3. Splenomegaly.
  4. Leg ulcers
  5. Gall stones
202
Q

Why does a deficiency in glucose-6-phosphate dehydrogenase lead to shortened red cell lifespan?

A

G6PD is necessary for providing NADPH to RBCs, which is used with glutathione to protect RBCs against oxidative damage (from H2O2)

203
Q

What 3 blood test values would be increased in someone with polycythaemia?

A
  1. Hb.
  2. RCC.
  3. PCV.
204
Q

Give 3 symptoms of polycythaemia.

A
  1. Itching.
  2. Headache.
  3. Dizziness.
  4. Visual disturbance.
205
Q

What can cause immune thrombocytopenia purpura (ITP)?

A
  1. Viral infection (acute)
  2. Malignancy (chronic)
  3. Autoimmune disorders (chronic)
206
Q

What antibodies are often found in ITP?

A

Platelet autoantibodies - IgG.

207
Q

Are PTT and APTT increased or decreased in DIC?

A

Increased.

208
Q

Are fibrinogen and platelets increased or decreased in DIC?

A

Decreased.

209
Q

Name 2 things that are increased and 2 that are decreased in DIC.

A

Increased: PTT and APTT

Decreased: fibrinogen and platelets.

210
Q

How would you treat haemophilia A?

A

IV infusion of factor 8.

211
Q

What is sickle cell vaso-occlusive crises?

A

Acute pain in hands and feet (dactylitis) due to vaso-occlusion of small vessels and avascular necrosis of the bone marrow in children

Phase 2a Medicine - Hannah's version (9 decks)

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