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Haematology Flashcards

1
Q

Disseminated intravascular coagulation

Pathophysiology

A
  • Hemostasis goes out of control
  • Various blood clots form –> organ ischaemia (kidneys, liver, lungs, brain)
  • These clots consume platelets and clotting factors
  • Therefore the rest of the blood is low on these factors
  • Fibrin degradation products in the circulation (from breakdown of the clots) also interferes with new clot formation
  • Therefore resulting in bleeding with even the slightest damage to vessel walls

= Bleeding and clotting

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2
Q

Disseminated intravascular coagulation

Investigations

A
  • Decreased platelets
  • Decreased fibrinogen
  • Prolonged prothrombin time (PT)
  • Prolonged activated partial thromboplastin time (APTT)
    PT and PTT reflect low circulating coagulation factors
  • Elevated D-Dimer (fibrin degradation product)
  • Schistocytes due to microangiopathic haemolytic anaemia
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3
Q

Disseminated intravascular coagulation

When might you see chronic DIC

A
  • Solid tumours

- Large aortic aneurysms

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4
Q

Disseminated intravascular coagulation

What would you see on investigations

A
  • Relatively normall findings due to compensation
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5
Q

Disseminated intravascular coagulation

Management

A

Focus on underlying cause

  • Support underlying organs (ventilator, haemodynamic support, tranfusions if needed)
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6
Q

Disseminated intravascular coagulation

Causes

A
  • Sepsis
  • Trauma
  • Obstetric complications, e.g. HELLP syndrome, amniotic fluid embolism
  • Malignancy

Can all initially tip the balance in favour of clotting –> starting the process of DIC

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7
Q

Haemophilia

Pathophysiology

A
  • Deficiency of clotting factors

- Leading to bleeding

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8
Q

Haemophilia

Inheritance

A

X-linked recessive

  • All of the X chromosomes need to have the abnormal gene
  • Men only require one abnormal copy as they only have one X chromosome
  • Women require two abnormal copies
  • If they only have one copy –> carrier
  • Almost exclusively affects males as for a female to be affected it would require an affected father and an affected/carrier mother
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9
Q

Haemophilia

Features

A
  • Excessive bleeding in response to minor trauma
  • Risk of spontaneous hemorrhage
  • Haemoarthroses (bleeding into joints)
  • Haematomas
  • Prolonged bleeding after trauma/surgery
  • Cord bleeding in neonates
  • Bleeding of gums, GI tract, UT (haematuria), retroperitoneal space, intracranial

KEY presentation of severe disease = spontaneous bleeding into joints and muscles

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10
Q

Haemophilia

Investigations

A
  • Prolonged APTT
  • Bleeding time, thrombin time and prothrombin time all normal
  • Genetic testing
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11
Q

Haemophilia

Management

A

Prophylactically:

  • Replace clotting factors via IV
  • Complication = formation of antibodies against the clotting factor, making it ineffective Tx

Acutely:

  • Infusions of affected factor (VIII or IX)
  • Desmopressin to stimulate release of von Willebrand factor
  • Antifibrinolytics, e.g. tranexamic acid
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12
Q

Haemophilia

Types

A
  • Type A = deficiency in factor VIII

- Type B = deficiency in factor IX (Christmas disease)

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13
Q

Hyposplenism

Causes

A
  • Splenectomy
  • Sickle-cell
  • Coeliac disease, dermatitis herpetiformis
  • Graves’ disease
  • Systemic lupus erythematosus
  • Amyloid
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14
Q

Hyposplenism

Features on blood film

A
  • Howell-Jolly bodies

- Siderocytes

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15
Q

Splenectomy

Post-splenectomy risks

A
  • Pneumococcus
  • Haemophilus
  • Meningococcus
  • Capnocytophaga canimorsus (dog bites)
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16
Q

Splenectomy

Vaccination

A

If elective, should be done 2 weeks prior to operation:

  • Haemophilus influenza Type B (HiB)
  • Meningitis A&C

Also:

  • Annual influenza
  • Pneumococcal every 5 years
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17
Q

Splenectomy

Antibiotic prophylaxis

A
  • Penicillin V for at least 2 years or until 16 yrs old

- Can sometimes be for life

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18
Q

Splenectomy

Indications

A
  • Trauma (1/4 = iatrogenic)
  • Spontanous rupture (EBV)
  • Hypersplenism (hereditaory sphero/elliptocytosis)
  • Malignancy (lymphoma, leukaemia)
  • Splenic cysts, hydatid cysts, splenic abscesses
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19
Q

Splenectomy

Complications

A
  • Haemorrhage - from SHORT GASTRIC or SPLENIC HILAR vessels
  • Pancreatic fistula (from iatrogenic damage to tail)
  • Thrombocytosis - prophylactic aspirin
  • Encapsulated bacteria infection (strep pneumo, haem influenza, Neisseria meningitidis)
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20
Q

Splenectomy

Post-splenectomy changes

A
  • Platelets will rise first
  • Blood film will change after following weeks
  • Howell-Jolly bodies will appear on the film
  • May also see target cells, pappenheimer bodies
  • Increased risk of post-splenectomy sepsis -> prophylactic Abx and pneumococcal vaccine
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21
Q

Splenectomy

Post-splenectomy sepsis

A
  • Typically occur with encapsulated organisms
  • Risk is greatest in < 16 yrs and > 50 yrs
  • Tx = Penicillin V 500 mg BD and Amoxicillin 250 mg BD
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22
Q

Splenectomy

Travel

A
  • Asplenic individuals travelling to malaria endemic areas are at high risk and should have both pharmacological and mechanical protection
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23
Q

Myeloproliferative disorders

Umbrella term for what?

A
  • Primary myelofibrosis
  • Polycythaemia vera
  • Essential thrombocythaemia
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24
Q

Myeloproliferative disorders

Pathophysiology

A
  • Uncontrolled proliferation of a single type of stem cell

- Considered a type of bone marrow cancer

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25
Q

Myeloproliferative disorders

Cell lines and diseases

A
  • Primary myelofibrosis = fibroblasts (monocytes, eosinophils, neutrophils, basophils)
  • Polycythemia vera = erythrocytes (RBCs)
  • Essential thrombocythaemia = megakaryocytes (platelets)
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26
Q

Myeloproliferative disorders

Complications

A

Have the potential to progress and transform into acute myeloid leukemia (AML)

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27
Q

Myeloproliferative disorders

Associated mutations

A
  • JAK2
  • MPL
  • CALR
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28
Q

Myelofibrosis

Pathophysiology

A
  • Can be the result of primary myelofibrosis, polycythemia vera or essential thrombocytopenia
  • Proliferation of haematopoietic stem cell
  • Resultant release of platelet-derived growth factors –> stimulates fibroblast growth factor
  • Leads to fibrosis of the bone marrow, replaced by scar tissue
  • Can lead to low production of blood cells –> anaemia and leukopenia
  • Haematopoiesis starts occurring in liver and spleen (extramedullary haematopoiesis)
  • Can lead to hepatosplenomegaly and portal hypertension
  • If this occurs around the spine it can result in spinal cord compression
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29
Q

Myelofibrosis

Features

A
  • Symptoms of anaemia
  • Massive splenomegaly
  • Hypermetabolic symptoms - weight loss, night sweats
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30
Q

Myelofibrosis

Investigations

A
  • Anaemia
  • High WBC and platelet count in early disease
  • May be low in later disease
  • High urate and LDH (increased cell turnover)

Blood film:

  • TEARDROP POIKILOCYTES
  • Poikilocytosis (varying sizes)
  • Immature red and white cells (blasts)

Bone marrow biopsy:

  • Usually ‘dry’ as it has turned to scar tissue
  • Therefore, need trephine biopsy
  • Genetic testing: JAK2, MPL and CALR
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31
Q

Myelofibrosis

Management

A
  • Allogenic stem cell transplantation (potentially curative but carries risks)
  • Chemotherapy (improves symptoms and slow progression but not curative)
  • Supportive management of anaemia, splenomegaly, portal HTN
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32
Q

Polycythaemia vera

Pathophysiology

A
  • Clonal proliferation of a marrow stem cell, erythroid cells
  • Leads to an increased in red cell volume
  • Often accompanied by overproduction of neutrophils and platelets
  • INcidence peaks in 6th decade
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33
Q

Polycythaemia vera

Features

A
  • Hyperviscosity
  • Pruritus, typically after a hot bath
  • Splenomegaly
  • Haemorrhage (secondary to abnormal platelet function)
  • Plethoric appearance
  • Conjunctival plethora
  • Hypertension in a third of patients
  • Low ESR
  • Ruddy complexion
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34
Q

Polycythaemia vera

Investigations

A
  • FBC: raised haematocrit, neutrophils, basophils, platelets (in 1/2), raised red cell mass
  • JAK2 mutation (92%)
  • Serum ferritin
  • Renal and liver function test
  • Low ESR
  • Raised leukocyte alkaline phosphate
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35
Q

Polycythaemia vera

Management

A
  • Venesection to keep Hb in normal range = 1st line
  • Aspirin to reduce risk of blood clots
  • Chemo to control disease (hydroxyurea or phosphorus-32)
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36
Q

Polycythaemia vera

JAK-2 positive diagnostic criteria

A

Requires both

  • High haematrocrit (> 0.52 in men, > 0.48 in women) OR raised red cell mass (> 25% above predicted)
  • Mutation in JAK2
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37
Q

Polycythaemia vera

JAK-2 negative diagnostic criteria

A

Requires A1 + A2 + A3 + either another A or two B criteria

A1: Raised red cell mass (>25% above predicted) OR haematocrit >0.60 in men, >0.56 in women
A2: Absence of JAK2 mutation
A3: No cause of secondary erythrocytosis

A4: Palpable splenomegaly
A5: Presence of aquired genetic abnormality (excluding BRC-ABL) in haemopoietic cell
B1: Thrombocytosis (platelets > 450)
B2: Neutrophils > 10 in non-smokers, >12.5 in smokers
B3: Radiological evidence of splenomegaly
B4: Endogenous erythroid colonies or low serum erythropoietin

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38
Q

Essential thrombocytosis

Pathophysiology

A
  • Essential thrombocytosis is one of the myeloproliferative disorders which overlaps with chronic myeloid leukaemia, polycythaemia rubra vera and myelofibrosis.
  • Megakaryocyte proliferation results in an overproduction of platelets.
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39
Q

Essential Thrombocytosis

Features

A
  • Platelet count > 600
  • Both thrombosis and haemorrhage
  • Burning sensation in the hands
  • JAK2 mutation in 50%
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40
Q

Essential Thrombocytosis

Investigations

A

Raised platelet count (more than 600 x 10^9/l)

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41
Q

Essential Thrombocytosis

Management

A
  • Aspirin to reduce risk of thrombus formation
  • Chemo to control disease
  • Hydroxyurea to reduce platelet count
  • Interferon-alpha in younger patients
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42
Q

Thrombocytosis

Define

A

Thrombocytosis is an abnormally high platelet count, usually > 400 * 10^9/l

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43
Q

Thrombocytosis

Causes

A
  • Reactive: platelets are an acute phase reactant - platelet count can increase in response to stress such as a severe infection, surgery. Iron deficiency anaemia can also cause a reactive thrombocytosis
  • Malignancy
  • Essential thrombocytosis, or as part of another myeloproliferative disorder such as chronic myeloid leukaemia or polycythaemia rubra vera
  • Hyposplenism
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44
Q

Myelodysplastic syndrome

Pathophysiology

A
  • Myeloid bone marrow cells do not mature properly
  • Therefore do not produce healthy blood cells
  • Pre-leukaemia –> may progress to AML
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45
Q

Myelodysplastic syndrome

Features

A

Bone marrow failure:

  • Anaemia - pallor, fatigue, SOB
  • Neutropenia (low neutrophils) - frequent/severe infections
  • Thrombocytopenia (low platelets) - purpura or bleeding
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46
Q

Myelodysplastic syndrome

Investigations

A
  • FBC: anaemia, neutropenia, thrombocytopenia
  • Blood film: Blasts
  • Bone marrow aspiration and biopsy
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47
Q

Myelodysplastic syndrome

Management

A
  • Watchful waiting
  • Supportive treatment with blood transfusions if severely anaemic
  • Chemotherapy
  • Stem cell transplantation
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48
Q

Myelodysplastic syndrome

Epidemiology

A
  • More common with age (> 60 yrs)

- More common in those who have previously had chemo or radio therapy

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49
Q

Polycythaemia

Types

A
  • Relative
  • Primary (polycythaemia vera)
  • Secondary
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50
Q

Causes of relative polycythaemia

A
  • Dehydration

- Stress - Gasibock syndrome

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51
Q

Causes of primary polycythaemia

A
  • Polycythaemia vera (proliferation of a marrow stem cell, erythroid cells)
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52
Q

Causes of secondary polycythaemia

A
  • COPD
  • Altitude
  • Obstructive sleep apnoea
  • Excessive erythropoietin:
    • Cerebellar hemangioma
    • Hypernephroma
    • Hepatoma
    • Uterine fibroids –> menorrhagia –> blood loss
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53
Q

How to differentiate between true (primary and secondary) and relative polycythaemia?

A

RED CELL MASS

In true polycythaemia the total red cell mass in males > 35 ml/kg and in women > 32 ml/kg

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54
Q

Thrombocytopenia

Pathophysiology

A
  • Low platelet count

- Can either be due to low production or excess destruction

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55
Q

Thrombocytopenia

Problems with production

A
  • Sepsis
  • B12 or folic acid deficiency
  • Liver failure –> reduced thrombopoietin production
  • Leukaemia
  • Myelodysplastic syndrome
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56
Q

Thrombocytopenia

Problems with destruction

A
  • Alcohol
  • ITP
  • TTP
  • Heparin-induced thrombocytopenia
  • Haemolytic-uraemic syndrome
  • DIC (using them up rather than destruction)
  • Medications:
    • Sodium valproate
    • Clozapine
    • Methotrexate
    • Isotretinoin
    • Antihistamines
    • PPIs
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57
Q

Thrombocytopenia

Features

A

Platelets < 50 x 109/L

  • Easy or spontaneous bruising and prolonged bleeding times
  • Nosebleeds, bleeding gums, heavy periods, easy bruising or blood in the urine or stools

Platelet counts < 10 x 109/L

  • High risk for spontaneous bleeding
  • Spontaneous intracranial haemorrhage or GI bleeds are particularly concerning
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58
Q

Differentials of abnormal or prolonged bleeding

A
  • Thrombocytopenia (low platelets)
  • Haemophilia A and haemophilia B
  • Von Willebrand Disease
  • Disseminated intravascular coagulation (usually secondary to sepsis)
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59
Q

Causes of severe thrombocytopenia

A
  • ITP
  • DIC
  • TTP
  • Haematological malignancy
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60
Q

Causes of moderate thrombocytopenia

A
  • HIT
  • Drug-induced
  • Alcohol
  • Liver disease
  • Hypersplenism
  • Viral infection (EBV, HIV, hepatitis)
  • Pregnancy
  • SLE
  • Antiphospholipid syndrome
  • Vit B12 deficiency
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61
Q

Immune thrombocytopenia

Pathophysiology

A
  • Antibodies are created against platelets

- Antibodies are directed against the glycoprotein IIb/IIIa or Ib-V-IX complex

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62
Q

Immune thrombocytopenia

Epidemiology

A
  • More common in older females
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63
Q

Immune thrombocytopenia

Features

A
  • May be detected incidentally
  • Petichae, purpura
  • Bleeding (e.g. epistaxis)
  • Catastrophic bleeding (e.g. intracranial) = rare
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64
Q

Immune thrombocytopenia

Investigations

A

Platelets

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65
Q

Immune thrombocytopenia

Management

A
  • Prednisolone = 1st line
  • IV normal human immunoglobulin (IVIG): raises platelet count quicker than Pred so may be used if active bleeding or urgent invasive procedure is required
  • Rituximab (monoclonal antibody against B cells)
  • Splenectomy (now less common)
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66
Q

Immune thrombocytopenia

Other names for it

A

Autoimmune thrombocytopenic purpura
Idiopathic thrombocytopenic purpura
Primary thrombocytopenic purpura

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67
Q

Thrombotic Thrombocytopenic Purpura

Pathophysiology

A
  • Abnormaly large or ‘sticky’ multimers of von Willebrand’s factor
  • Causes platelets to clump in the vessels
  • Deficiency of ADAMTS13 (normally present to break down multimers of von Willebrand’s factor)
  • Platelets are used up here and therefore can not form clots –> bleeding
  • The blood clots also break up RBCs leading to haemolytic anaemia
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68
Q

Thrombotic Thrombocytopenic Purpura

Features

A
  • Fever
  • Fluctuating neuro signs (microemboli)
  • Microangiopathic hemolytic anaema
  • Thrombocytopenia
  • Renal failure
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69
Q

Thrombotic Thrombocytopenic Purpura

Causes

A

Deficiency in the ADAMTS13 can be autoimmune disease or inherited genetic mutation

  • Post-infection, e.g. urinary, GI
  • Pregnancy
  • Tumours
  • SLE
  • HIV
  • Drugs:
    • Ciclosporin
    • Oral contraceptive pill
    • Penicillin
    • Clopidogrel
    • Aciclovir
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70
Q

Thrombotic Thrombocytopenic Purpura

Investigations

A

Platelets
Hb (low)
Renal function

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71
Q

Thrombotic Thrombocytopenic Purpura

Management

A
  • Plasma exchange
  • Steroids
  • Rituximab (monoclonal antibody against B cells)
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72
Q

Heparin Induced Thrombocytopenia (HIT)

Pathophysiology

A
  • Development of antibodies against platelets in response to exposure to heparin
  • Specifically target platelet factor 4 (PF4)
  • Therefore are anti-PF4/heparin antibodies
  • HIT antibodies bind to platelets and activate clotting mechanisms -> hypercoagulable state -> thrombosis
  • BUT they also break down platelets causing thrombocytopenia

CLINICALLY: A patient on heparin has low platelets but forms unexpected blood clots –> HIT

73
Q

Heparin Induced Thrombocytopenia (HIT)

Diagnosis

A

Test for HIT antibodies

74
Q

Heparin Induced Thrombocytopenia (HIT)

Management

A
  • Stop heparin

- Use an alternative anticoagulant, guided by a specialist

75
Q

G6PD Deficiency

Pathophysiology

A
  • G6PD is responsible for helping to protect cells from damage by reactive oxygen species (ROS)
  • Reduced G6PD –> reduced NADPH –> reduced glutathione –> reduced RBC susceptibility to oxidative stress –> RBC haemolysis
  • Periods of acute stress lead to higher production of ROS –> acute haemolytic anaemia
  • X linked recessive pattern
76
Q

G6PD Deficiency

Triggers

A
  • Broad beans (fava beans)
  • Infection
  • Recent course one of the following drugs:
    • Anti-malarials - primaquine
    • Ciprofloxacin
    • Nitrofurantoin
    • Trimethoprim
    • Sulph-group drugs: sulphonamides, suphasalazine, sulphonylureas
77
Q

G6PD Deficiency

Epidemiology

A
  • More common in Mediterranean, Middle Eastern and African patients
  • Inherited in an X linked recessive pattern (usually affects males)
78
Q

G6PD Deficiency

Features

A
  • Anaemia
  • Intermittent jaundice (in response to triggers)
  • Neonatal jaundice
  • Intravascular haemolysis
  • Gallstones
  • Splenomegaly
79
Q

G6PD Deficiency

Investigations

A
  • Blood film: Heinz bodies (= blobs of denatured hemoglobin)
  • May also see bite and blister cells
  • Diagnosis = G6PD enzyme assay
    • Levels should be checked around 3 months after an acute episode of hemolysis
    • RBCs with the most severely reduced G6PD activity will have hemolysed → reduced G6PD activity → not be measured in the assay → false-negative results
80
Q

G6PD Deficiency

Management

A
  • Avoid triggers where possible

- Usually self-limiting

81
Q

Hereditary spherocytosis

Pathophysiology

A
  • Deficiency in RBC membrane proteins
  • Caused by genetic lesions
  • Spleen destroys them due to their odd shapes
  • Autosomal dominant
82
Q

Hereditary spherocytosis

Epidemiology

A
  • Autosomal dominant

- More common in Northern Europeans

83
Q

Hereditary spherocytosis

Features

A
  • Failure to thrive (children)
  • Jaundice
  • Gallstones
  • Splenomegaly
  • Asplastic crisis precipitated by PARVOVIRUS (more severe haemolysis, anaemia and jaundice, no response from bone marrow to make new cells)
  • Degree of haemolysis = variable
84
Q

Hereditary spherocytosis

Investigations

A
  • Family history
  • Blood film: spherocytes or elliptocytes (another membranopathy with similar patho)
  • Elevated mean corpuscular haemoglobin concentration (MCHC) on FBC
  • Reticulocutes will be raised due to rapid turnover of RBCs (NOT in an aplastic crisis)
85
Q

Hereditary spherocytosis

Management

A
  • Folate supplementation
  • Splenectomy
  • Removal of gallbladder (cholecystectomy) if required
  • Transfusions may be needed in acute crises
86
Q

Hereditary Elliptocytosis

A

Exactly the same as spherocytosis except ellipse shaped RBCs

Also autosomal dominant

87
Q

Von Willebrand Disease

Pathophysiology

A
  • von Willebrand = large glycoprotein that form massive mU\
  • Usually released from Weibel-Palade bodies in endothelial cells
  • Promotes platelet adhesion to damaged endothelium
  • Carrier molecule for factor VIII
    (ADAMST13 balances the adhesion and prevents multimers from getting too big)
88
Q

Von Willebrand Disease

Epidemiology

A
  • Most common inherited cause of abnormal bleeding

- Autosomal dominant (most of the underlying causes)

89
Q

Von Willebrand Disease

Types

A
  • Type 1: Partial reduction in vWF (80% - autosomal dominant)
  • Type 2: Abnormal form of vWF (autosomal dominant)
  • Type 3: Total lack of vWF (autosomal recessive)
90
Q

Von Willebrand Disease

Features

A
  • Bleeding gums with brushing
  • Epistaxis
  • Heavy menstrual periods
  • Heavy bleeding during surgical operations
  • Family history !!
91
Q

Von Willebrand Disease

Investigations

A
  • Prolonged bleeding time
  • APTT may be prolonged
  • Factor VIII levels may be moderately reduced
  • Defective platelet aggregation with ristocetin
92
Q

Von Willebrand Disease

Management

A
  • Desmopressin can stimulate the release of vWF from Weibel-Palade bodies in endothelial cells
  • VWF can be infused
  • Factor VIII infusion (along with plasma-derived VWF)
  • Tranexamic acid or mefenamic acid or mild bleeding
  • Could manage heavy periods with norethisterone, COCP, mirena coil, or ultimately hysterectomy
93
Q

What is Osler-Weber-Rendu syndrome also known as?

A

Hereditary hemorrhagic telangiectasia

94
Q

What is Osler-Weber-Rendu syndrome?

A

Characterized by (as the name suggests) multiple telangiectasia over the skin and mucous membranes

95
Q

The inheritance pattern of Osler-Weber-Rendu syndrome?

A

Autosomal dominant

96
Q

Diagnostic criteria of Osler-Weber-Rendu syndrome?

A

If 2 –> possible diagnosis
If 3+ –> definite diagnosis

  • Epistaxis (spontaneous, recurrent nosebleeds)
  • Telangiectases (multiple characteristic sights - lips, oral cavity, fingers, nose)
  • Visceral lesions (GI telangiectasia, pulmonary AV malformations, hepatic AVM, cerebral AVM, spinal AVM)
  • Fx - first-degree relative with HHT
97
Q

What is telangiectasia

A

Dilated or broken blood vessels located near the surface of the skin or mucous membranes

98
Q

Lymphoma

Pathophysiology

A
  • Group of cancers that affect the lymphocytes inside the lymphatic system
  • Cancerous cells proliferate within the lymph nodes
  • Cause the lymph nodes to become abnormally large (lymphadenopathy)
99
Q

Lymphoma

Types

A
  • Hodgkins

- Non-hodgkins (including Burkitt’s)

100
Q

Hodgkin’s Lymphoma

Epidemiology

A
  • 1 in 5 lymphomas = Hodgkins

- Bimodal age distribution: 20 yrs and 75 yrs

101
Q

Hodgkin’s Lymphoma

Risk factors

A
  • HIV
  • EBV
  • Autoimmune conditions, e.g. RA and sarcoidosis
  • Fx
102
Q

Hodgkin’s Lymphoma

Features

A
  • LYMPHADENOPATHY - sometimes pain when drinking alcohol, normally painless, non-tender, rubbery, asymmetrical
  • Fatigue
  • Itching
  • Cough/SOB
  • Abdo pain
  • Recurrent infections
  • Hepatosplenomegaly

B symptoms:

  • Fever (Pel-Ebstein: fever than rises and falls every 7-10 days)
  • Weight loss
  • Night sweats
103
Q

Hodgkin’s Lymphoma

Investigations

A
  • Raised LDH (not specific)
  • Normocytic anaemia
  • Eosinophilia
  • Lymph nodes biopsy
    • REED STERNBERG CELLS (abnormally large B cells that have multiple nuclei –> owl-looking)
  • CT, MRI, PET scan
104
Q

Hodgkin’s Lymphoma

Staging

A

ANN ARBOR STAGING

1: Confined to one region of lymph nodes
2: More than one region but same/one side of diaphragm
3: Affects lymph nodes both above and below diaphragm
4: Widespread involvements, including non-lymphatic organs, e.g. lungs, liver

105
Q

Hodgkin’s Lymphoma

Classification (A/B)

A

A = no systemic features (apart from pruritus)
B = B symptoms present
- Weight loss > 10% in last 6 months
- Fever > 38 degress
- Night sweats (poor prognosis)

106
Q

Hodgkin’s Lymphoma

Poor prognostic factors

A
  • B symptoms
  • Age > 45 yrs
  • Stage IV disease
  • Hb < 10.5
  • Lymphocytes < 600 or < 8%
  • Male
  • Albumin < 40
  • WBC > 15,000
  • Raised ESR
107
Q

Hodgkin’s Lymphoma

Histological types (and prognosis)

A

Nodular sclerosing

  • Most common
  • More common in women
  • Associated with lacunar cells
  • Good prognosis

Mixed cellularity

  • Around 20%
  • Associated with large numbers of RS cells
  • Good prognosis

Lymphocyte predominant
- Best prognosis

Lymphocyte depleted
- Worst prognosis

108
Q

Hodgkin’s Lymphoma

Management

A

Chemotherapy and radiotherapy

  • Chemo: risk of leukaemia and infertility
  • Radio: risk of cancer, damage to tissues, hypothyroidism

Chemo = AVBD

  • Doxorubicin hydrochloride (Adriamycin)
  • Bleomycin sulfate
  • Vinblastine sulfate
  • Dacarbazine
Early = 2-4 cycles
Advanced = 6-8 cycles
109
Q

Non-Hodgkins Lymphoma

Epidemiology

A
  • 6th most common cancer in UK (more common than hodgkins)
  • Typically in the elderly (> 75 yrs)
  • More common in men
110
Q

Non-Hodgkins Lymphoma

Risk factors

A
  • EBV
  • H.plyor (MALT lymphoma)
  • Hep B or C
  • Exposure to pesticides or specific chemical (trichloroethylene)
  • Fx
  • Hx of chemo/radiotherapy
  • Autoimmune diseases (SLE/sjogrens/coeliac)
  • Immunodeficiency (HIV/DM/tranplant)
111
Q

Non-Hodgkins Lymphoma

Features

A

Same as Hodgkins - only differentiated by biopsy

  • Painless lymphadenopathy (non-tender, rubbery, asymmetrical)
  • Constitutional/B symptoms (fever, weight loss, night sweats, lethargy)
  • Extranodal Disease - gastric (dyspepsia, dysphagia, weight loss, abdominal pain), bone marrow (pancytopenia, bone pain), lungs, skin, central nervous system (nerve palsies)
  • Signs of weight loss
  • Palpable abdominal mass - hepatomegaly, splenomegaly, lymph nodes
  • Testicular mass
  • Fever
112
Q

Non-Hodgkins Lymphoma

Investigations

A
  • Raised LDH
  • Raised ESR
  • Normocytic anemia
  • Lymph node biopsy
    • Burkitt’s may have starry sky appearance
  • CT TAP to assess staging
113
Q

Non-Hodgkins Lymphoma

Staging

A

ANN ARBOR STAGING

1: Confined to one region of lymph nodes
2: More than one region but same/one side of diaphragm
3: Affects lymph nodes both above and below diaphragm
4: Widespread involvements, including non-lymphatic organs, e.g. lungs, liver

Plus A or B for absence/presence of B symptoms

114
Q

Non-Hodgkins Lymphoma

Management

A
  • May just be watchful waiting
  • Chemo: R-CHOP
    • Rituximab
    • Cyclophosphamide
    • Doxorubicin Hydrochloride
    • Vincristine (Oncovin)
    • Prednisolone
  • Flu/pneumococcal vaccines
  • Neutropenia may require prophylactic Abx
  • Stem cell transplantation
115
Q

Non-Hodgkins Lymphoma

Complications

A
  • Bone marrow infiltration causing anaemia, neutropenia or thrombocytopenia
  • Superior vena cava obstruction
  • Metastasis
  • Spinal cord compression
  • Complications related to treatment e.g. Side effects of chemotherapy
116
Q

Non-Hodgkins Lymphoma

Prognosis

A
  • Low-grade non-Hodgkin’s lymphoma has a better prognosis

- High-grade non-Hodgkin’s lymphoma has a worse prognosis but a higher cure rate

117
Q

Non-Hodgkins Lymphoma

Types and cells involved and additional symptoms

A
  • Mantle cell (Mature B cell lymphoma)
  • B-cell follicular (Mature B cell lymphoma)
  • Diffuse Large B-Cell (B cell) - bowel symptoms
  • Burkitt’s (B cell) - abdo/testicle/CNS mass
  • T and NK cell (T cell)
118
Q

Lymphoma

Factors that might help differentiate between Hodgkins and non-Hodgkins before biopsy

A
  • Lymphadenopathy in Hodgkin’s lymphoma can experience alcohol-induced pain in the node
  • ‘B’ symptoms typically occur earlier in Hodgkin’s lymphoma and later in non-Hodgkin’s lymphoma
  • Extra-nodal disease is much more common in non-Hodgkin’s lymphoma than in Hodgkin’s lymphoma
119
Q

Burkitt’s Lymphoma

Types

A

Endemic (African):
- Maxilla or mandible

Sporadic form:

  • Abdo (ileo-caecal) tumours are most common
  • Most common in HIV patients
120
Q

Burkitt’s Lymphoma

Genetics

A
  • Associated with c-myc gene
  • Translocation t(8:14)
  • EBV is strongly implicated in African form
121
Q

Burkitt’s Lymphoma

Microscopy findings

A
  • Starry sky appearance

- Lymphocyte sheets interspersed with macrophages containing dead apoptotic tumour cells

122
Q

Burkitt’s Lymphoma

Management

A
  • Chemo
  • Produced rapid response which may lead to TUMOUR LYSIS SYNDROME
  • Give Rasburicase before chemo to reduce chance of this
123
Q

Chemotherapy side effects

A
  • Alopecia
  • Nause and vomiting
  • Fatigue
  • Neutropenia

Use ONDANSETRON (5HT3 antagonist)

124
Q

Myeloma

Pathophysiology

A
  • Proliferation of plasma cells
  • Type of B lymphoycte that produce antibodies
  • Cancer in a specific type of plasma cell results in large quantities of a single type of antibody being produced (antibodies = immunoglobulins)
  • Multiple myeloma = where myeloma affects multiple areas of the body
125
Q

Myeloma

Risk factors

A
  • Older age
  • Males
  • Black African ethnicity
  • Fx
  • Obesity
126
Q

Myeloma

Features

A

CRABBI

C: Calcium - hypercalcemia (bones, groans, psych moans, stones)

  • Occurs due to increased osteoclast activity within bones
  • Leads to constipation, nausea, anorexia, confusion

R: Renal failure

  • Light chain deposition within renal tubules (BENCE JONES)
  • Renal damage -> dehydration and thirst
  • Other causes: amyloidosis, nephrolithiasis, nephrocalcinosis

A: Anaemia

  • Bone marrow crowding suppresses erythropoiesis
  • Fatigue and pallor

B: Bone lesions/pain

  • Bone marrow infiltration by plasma cells and cytokine-mediated osteoclast overactivity –> lytic bone lesions
  • Pain (espeically back), increased fragility fractures

B: Bleeding/bruising
- Bone marrow crowding –> thrombocytopenia

I: Infection

  • Reduction in production of normal immunoglobulins –> increased susceptibility to infection
  • Infiltration of bone marrow –> neutropenia
127
Q

Myeloma

Investigations

A
  • FBC: thrombocytopenia, neutropenia, anaemia (low RBCs), raised ESR
  • U&Es: Raised urea and creatinine
  • Raised calcium
  • Plasma viscosity

If any of above +ve or myeloma still suspected: BLIP

  • B: Bence Jones protein in urine (urine electophoresis)
  • L: serum Light chain assay
  • I: serum Immunoglobuilins
  • P: serum Protein electrophoresis

Bone marrow biopsy needed to confirm the diagnosis (significantly raised plasma cells)

Whole-body MRI or CT or skeletal survey for bony lesions

128
Q

Myeloma

General management

A

Myeloma = chronic relapsing and remitting malignancy which is currently deemed incurable. Management aims to control symptoms, reduce complications and prolong survival.

For those suitable for stem cell transplantation, induction therapy:
- Bortezomid and Dexamethasone

For those NOT suitable:
- Thalidomide + Alkylating Agent + Dexamethasone

For relapses:

  • Bortezomib monotherapy
  • May be suitable for repeat stem cell transplant

VTE prophylaxis

129
Q

What are the different type of immunoglobulins

A

They come in 5 main types: A, G, M, D and E

130
Q

What are immunoglobulins

A
  • Complex molecules made up of two heavy chains and two light chains arranged in a Y shape
  • They help the immune system recognize and fight infections by targeting specific proteins on the pathogen.
131
Q

What are immunoglobulins

A
  • Complex molecules made up of two heavy chains and two light chains arranged in a Y shape
  • They help the immune system recognize and fight infections by targeting specific proteins on the pathogen.
132
Q

What can occur as a result of hyperviscotiy in myeloma?

A
  • Easy bruising
  • Easy bleeding
  • Reduced or loss of sight due to vascular disease in the eye
  • Purple discolouration to the extremities (purplish palmar erythema)
  • Heart failure
133
Q

What can occur as a result of hyperviscosity in myeloma?

A

Results in vascular stasis and hypo-perfusion

  • Easy bruising
  • Easy bleeding
  • Reduced or loss of sight due to vascular disease in the eye
  • Purple discolouration to the extremities (purplish palmar erythema)
  • Heart failure
134
Q

Myeloma

X-Ray signs

A
  • Punched out lesions
  • Lytic lesions
  • Raindrop skull - caused by many punched out (lytic) lesions throughout the skull, gives appearance of raindrops splashing on surface (salt and pepper too)
135
Q

Myeloma

Complications

A
  • Pain (Tx = analgesia)
  • Pathological fracture
  • Infection
  • Renal failure
  • Anaemia
  • Hypercalcaemia
  • Peripheral neuropathy
  • Spinal cord compression
  • Hyperviscosity
136
Q

Myeloma

Management of bone disease

A
  • Bisphosphonates (Zolendronic Acid)
  • Radiotherapy to bone lesions
  • Orthopaedic surgery can stabilize bones (e.g. inserting prophylactic intramedullary rod)
  • Cement augmentation (inject cement into vertebral fractures or lesions to improve spine stability and pain)
137
Q

Myeloma

Prognosis

A
  • Incurable

- Patients always relapse

138
Q

Leukaemia

Pathophysiology

A

Rapid proliferation of immature blood blast cells (precursors of RBCs, WBCs, platelets) in the bone marrow that are non-functional (defective)

  • A genetic mutation in one of the precursor cells in the bone marrow leads to excessive production of a single type of abnormal white blood cell
  • -> Less energy, space and food for creating and maintaining healthy cells
  • Results in a PANCYTOPENIA - low RBCs (anaemia), low WBCs (leukopenia) and low platelets (thrombocytopenia)
139
Q

Acute myeloid leukemia

Epidemiology

A
  • Most common acute leukaemia in ADULTS
  • Myeloid blast cell proliferation
  • Associated with radiation, Downs syndrome
  • Can be a long-term complication of chemo (e.g. lymphoma)
  • Can be the result of myeloproliferative disorders, e.g. polycythemia vera/myelofibrosis
140
Q

Acute myeloid leukemia

Features

A

Bone marrow failure:

  • Anaemia (low RBCs)
  • Infection, fever, mouth ulcers (low WBCs)
  • Bleeding + bruising (low platelets)

Marrow infiltration:

  • Bone pain
  • Splenomegaly
  • Gum hypertrophy and bleeding
141
Q

Acute myeloid leukemia

Investigations

A
  • Blood film: BLAST CELLS and AUER RODS

- Same as ALL apart from HIGH WCC and low neutrophils

142
Q

Acute myeloid leukemia

Management

A
  • Chemo (daunorubicin, cytarabine)
  • Supportive - tranfusions
  • Allopurinol to prevent tumour lysis syndrome
  • Treat infections
  • Bone marrow transplant
143
Q

Acute myeloid leukemia

Poor prognostic factors

A
  • > 60 years
  • > 20% blasts after first course of chemo
  • Cytogenetics: deletions of chromosome 5 or 7
144
Q

Chronic myeloid leukaemia

Epidemiology/associations

A
  • Most often in adults (40-70 yrs)
  • Slight male predominance
  • > 80% have Philadelphia chromosome = t(9:22)
145
Q

Chronic myeloid leukaemia

Features

A

INSIDIOUS ONSET

  • Anaemia
  • Weight loss
  • Fatigue
  • Fever
  • Sweats
  • Gout
  • Bleeding
  • SPLENOMEGALY - often massive
146
Q

Chronic myeloid leukaemia

Phases

A
  • Chronic phase - can last around 5 yrs, often asymptomatic, may be diagnosed incidentally
  • Accelerated phase - abnormal blast cells take up a high proportion of cells in bone marrow and blood (10-20%), more symptoms, anaemia, immunocompromised
  • Blast phase - even higher proportion of blast cells (> 30%), severe symptoms, pancytopenia, often fatal
147
Q

Chronic myeloid leukaemia

Investigations

A
  • PHILADELPHIA CHROMOSOME
  • Decreased leukocyte alkaline phosphate
  • Very high WCC - whole spectrum of myeloid cells - neutrophils, basophils, eosinophils, myelocytes
148
Q

Chronic myeloid leukaemia

Managements

A
  • IMATINIB (inhibitor of tyrosine kinase) = 1st line
  • Hyroxyurea
  • Interferon-alpha
  • Allogenic bone marrow transplant
149
Q

What is the philadelphia chromosome?

A
  • Translocation between the long arm of chromosome 9 and 22 –> t(9:22)
  • Results in part of the ABL proto-oncogene from chromosome 9 being fused with the BCR gene on chromosome 22
  • Outcome = BCR-ABL gene
  • Codes for a fusion protein that has excessive tyrosine kinase activity
150
Q

Acute lymphoblastic leukemia

Epidemiology

A
  • Most common in children
  • Peak = 2-5 yrs old
  • Boys slightly more commonly affected
  • Usually B-lymphocytes
  • Associated with Down’s syndrome and ionising radiation during pregnancy
151
Q

Acute lymphoblastic leukemia

Features

A

Marrow failure:

  • Anaemia
  • Infection
  • Bleeding

Organ infiltration

  • Bone pain
  • Hepatosplenomegaly
  • Lymphadenopathy
  • Headache and cranial nerve palsy
  • Mediastinum masses
  • testicular swelling
  • Fever is present in up to 50% of new cases
152
Q

Acute lymphoblastic leukemia

Investigations

A
  • Blast cells on blood film
  • Philadelphia chromosome in 30% of adults, 3-5% of children (poor prognostic factor)
  • LP to look for CNS involvement
153
Q

Acute lymphoblastic leukemia

Types

A

If all B-cells –> Children

If all T-cells –> Adult

154
Q

Acute lymphoblastic leukemia

Management

A
  • Supportive - transfusion
  • Chemo (vincristine, pred)
  • If CNS –> Methotrexate
  • Allopurionl to prevent tumor lysis
  • Treat infections quickly
  • Bone marrow transplant
155
Q

Acute lymphoblastic leukemia

Poor prognostic factors

A
  • Age < 2yrs r > 10 yrs
  • WBC > 20 at diagnosis
  • T or B cell surface markers
  • Non-Caucasian
  • Male sex
156
Q

Chronic lymphocytic leukaemia

Epidemiology

A
  • MOST COMMON LEUKAEMIA
  • Most common in the elderly
  • Accumulation of mature B-lymphocytes that have escaped programmed cell death and undergone cell cycle arrest
157
Q

Chronic lymphocytic leukaemia

Features

A
  • Oftrn asymptomatic!
  • Anaemic or infection prone
  • If severe: weight loss, sweats, anorexia
  • Hepatosplenomegaly
  • Lymphadenopathy - more marked than CML
158
Q

Chronic lymphocytic leukaemia

Investigations

A
  • FBC: lymphocytosis, anaemia

- SMUDGE CELLS or SMEAR CELLS (during process of preparing the film, fragile WBCs rupture and leave smudge on film)

159
Q

Chronic lymphocytic leukaemia

Complications

A
  • Can transform into high-grade lymphoma. This is called Richter’s transformation.
  • Can cause WARM AUTOIMMUNE HAEMOLYTIC ANAEMIA
  • Hypogammaglobulinemia (recurrent infections)
160
Q

What is Richter transformation

A

Ritcher’s transformation occurs when leukaemia cells enter the lymph node and change into a high-grade, fast-growing non-Hodgkin’s lymphoma. Patients often become unwell very suddenly.

Ritcher’s transformation is indicated by one of the following symptoms:

  • Lymph node swelling
  • Fever without infection
  • Weight loss
  • Night sweats
  • Nausea
  • Abdominal pain
161
Q

Chronic lymphocytic leukaemia

Management

A
  • 1st line = Fludarabine and Rituximab +/- Cyclophosphamide
  • Human IV immunoglobulin
  • Blood transfusions
  • Bone marrow transplant
162
Q

Chronic lymphocytic leukaemia

Prognosis

A

Rule of three

  • 1/3 never progress
  • 1/3 progress slowly
  • 1/3 progress rapidly
163
Q

Age of different leukaemias

A

ALL CeLLmates have CoMmon AMbitions

  • <5 and >45: ALL
  • Over 55: CLL
  • Over 65: CML
  • Over 75: AML
164
Q

Investigations for all leukaemias

A
  • FBC
  • Blood film
  • LDH - not specific
  • Bone marrow biopsy
  • CXR - infection of mediastinum lymphadenopathy
  • Lymph node biopsy
  • LP is CNS involvement
  • CT, MRI, PET to stage and assess
165
Q

Types of bone marrow biopsy

A
  • Aspiration: take liquid sample full of cells

- Trephine: solid core sample - better assessment of cells and structure

166
Q

Where is the bone marrow biopsy usually taken from?

A

The iliac crest

167
Q

Causes of pathological fractures

A
  • Metastatic tumor (breast, lung, thyroid, renal, prostate)
  • Bone disease (osteogenesis imperfecta, osteoporosis, metabolic bone disease, Paget’s disease)
  • Local benign condition (chronic osteomyelitis, solitary bone cyst)
  • Primary malignant tumours (chonqdrosarcoma, osteosarcoma, Ewing’’s tumour)
  • Myeloma (salt and pepper/raindrop skull)
168
Q

Which vessels might bleed following a splenectomy?

A
  • Short gastric

- Splenic hilar

169
Q

What would prompt an urgent FBC in young people?

A
  • Pallor
  • Persistent fatigue
  • Unexplained fever
  • Unexplained persistent infections
  • Generalised lymphadenopathy
  • Persistent or unexplained bone pain
  • Unexplained bruising
  • Unexplained bleeding
170
Q

How quickly do you prescribe RBCs in a non-urgent scenario?

A

90-120 minutes

171
Q

Transfusion threshold for patients without ACS?

A

70 g/L

172
Q

Transfusion threshold for patients with ACS?

A

80 g/L

173
Q

Target after transfusion for those without ACS?

A

70-90 g/L

174
Q

Target after transfusion for those with ACS?

A

80-100 g/L

175
Q

Inherited causes of thrombophilia?

A
  • Most common = Factor V Leiden (activated protein C resistance)
  • 2nd most common = prothrombin gene mutation
  • Antithrombin III deficiency
  • Protein C deficiency
    Protein S deficiency
176
Q

Acquired causes of thrombophilia?

A
  • Antiphospholipid syndrome

- COCP

177
Q

What are irradiated blood products?

A
  • Depleted of T-lymphocytes
  • Avoid transfusion-associated graft versus host disease
  • Used in intra-uterine, neonates, bone marrow/stem cell transplants, immunocompromised, patients with/prev Hodgins Lymphoma
178
Q

Tx of neutropenic sepsis?

A

IV piperacillin with tazobactam

Finals (21 decks)

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