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Pathology > Haematology > Flashcards

Haematology Flashcards

1
Q

How do platelets bind to exposed subendothelial cells/matrix and what does this trigger?

A
  • GlpIa to exposed collagen
  • GlpIb to Von Willebrand factor
  • Release of ADP and thromboxane A2 trigger platelet aggregation
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2
Q

What factors are involved in platelet aggregation?

A

GlpIIb/IIIa crosslink platelets via fibrinogen

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3
Q

Which arachidonic acid metabolites affect platelet aggregation?

A

Thromboxane A2 induces platelet aggregation

Prostacyclin PGI2 inhibits platelet agggregation

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4
Q

What actions does thrombin have?

A
  • Cleaves Fibrinogen
  • Activates Platelets
  • Activates procofactors (FV and FVIII)
  • Activates zymogens (FVII, FXI and FXIII)
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5
Q

Describe the initiation phase?

A
  • Injury of vessels wall leads to contact between blood and subendothelial cells
  • Tissue factor (TF) is exposed and binds to FVIIa or FVII which is subsequently converted to FVIIa
  • The complex between TF and FVIIa activates FIX and FX
  • FXa binds to FVa on the cell surface
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6
Q

Describe the amplification phase?

A
  • The FXa/FVa complex converts small amounts of prothrombin into thrombin
  • The small amount of thrombin generated activates FVIII, FV, FXI and platelets locally. FXIa converts FIX to FIXa
  • Activated platelets bind FVa, FVIIIa and FIXa
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7
Q

Describe the propagation phase?

A
  • The FVIIIa/FIXa complex activates FX on the surfaces of activated platelets
  • FXa in association with FVa converts large amounts of prothrombin into thrombin creating a “thrombin burst”.
  • The “thrombin burst”leads to the formation of a stable fibrin clot.
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8
Q

What factor is activated to degrade fibrin?

A

Plasminogen is converted by tissue plasminogen activator (tPA) to plasmin which degrades fibrin.

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9
Q

What physiological anticoagulants are there?

A
  • Antithrombin III: main inhibitor of activated coagulation serine proteases. Also inactivates thrombin and activated factors X, IX, XI. ATIII activity is greatly accelerated (2000-fold) by heparin
  • Heparin co-factor II: complexes with thrombin inactivating it. Activity is amplified 1000-fold by heparin
  • Protein C + S: upon activation by thrombin in the presence of thrombomodulin, activated protein C inactivates VIIIa and Va, thus reducing the rate of thrombin generation. Protein S is a cofactor
  • Tissue factor pathway inhibitor: binds to Xa and TF:VIIa complex, inhibiting their proteloytic activity
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10
Q

Which substances mediate vasoconstriction in haemostasis?

A
  • adrenaline
  • ADP
  • kinins
  • thromboaxanes
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11
Q

Which coagulation factors are dependent on vitamin K for their normal function?

A

II, VII, IX, X, protein C and protein S

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12
Q

How does clopidogrel inhibit platelet aggregation?

A

Clopidogrel is a specifc, non-competive inhibitor of ADP- induced platelet aggregation, irreversibly inhibiting the binding of ADP to its platelet membrane receptors. Ultimately it inhibits the activation of the GPIIb/IIIa receptor, its binding to fibrinogen and further platelet aggregation.

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13
Q

What are the common causes of thrombocytopenia?

A 
Immune-mediated
	Idiopathic
	Drug-induced
	Connective tissue disease
	Lymphoproliferative disease
	Sarcoidosis
Non-immune mediated
	DIC
	Microangiopathic hemolytic anemia
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14
Q

What is the treatment for ITP?

A 
>50,000
- Nothing
20-50,000
- Not bleeding: None
- Bleeding	: Steroids + IVIG
<20,000
- Not bleeding: Steroids
- Bleeding	: Steroids, IVIG, Hospitalization
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15
Q

What are the clinical features of haemophilia?

A

X-linked congenital deficiency - Factor 8 (A) or 9 (B)
Bleeding – Haematoma, joint etc.
Prolonged aPTT but normal PT.
Clotting factor replacement-Life long.

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16
Q

What are the clinical features of von Willebrand Disease?

A 
Inheritance - autosomal dominant
Incidence - 1/10,000
Clinical features - mucocutaneous bleeding
Classification:
Type 1 - Partial quantitative deficiency
Type 2 - Qualitative deficiency
Type 3 - Total quantitative deficiency
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17
Q

What are the components of Virchow’s triad?

A

Vessel wall, blood, flow.

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18
Q

What inherited thrombophilia risk factors are there?

A
  • Anticoagulant deficiency (All rare): Protein C (0.3%), Protein S, Antithrombin (<0.2%)
  • Procoagulant excess (common): Factor VIII (10% of population), Factor II (2%), Fibrinogen
  • Additional factors: Factor V Leiden (5% caucasians), Lupus anticoagulant, Prothrombin G20210A
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19
Q

What is Lupus anticoagulant?

A

An immunoglobulin that binds to phospholipids and proteins associated with the cell membrane and increases the risk of thrombosis.

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20
Q

What acquired thrombophilia risk factors are there?

A

Age, obesity, previous DVT or PE, immobilisation, major surgery, long distance travel, malignancy (esp. pancreatic), pregnancy, COCP, HRT, antiphospholipid syndrome, polycythaemia, thrombocythaemia

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21
Q

What anticoagulant molecules are normally expressed by blood vessel walls?

A
  • Thrombomodulin: Directs thrombin to activate protein C
  • Endothelial protein C receptor: Presents PC to Thrombomodulin
  • Tissue factor pathway inhibitor: Inhibits Tissue factor
  • Prostacyclin (PGI2): Inhibits platelet activation
  • Nitirc Oxide: Inhibits platelet activation
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22
Q

What is used as DVT prophylaxis?

A

Chemical: low molecular weight heparin e.g. Tinzaparin, Clexane
Mechanical: TED stockings or intermittent compression devices

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23
Q

What is the treatment for a DVT/PE?

A

Immediate: Low molecular weight heparin until 2 days therapeutic INR
Ongoing: warfarin for 3-6 months, started simultaneously
In cancer patients continue LMWH not warfarin
THrombolysis reserved for life threatening PE or limb threatening DVT

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24
Q

How does heparin work, how is it administered and what long-term side effects are there?

A

Potentates antithrombin III which inactivates thrombin and factors 9, 10, 11
LMWH: SC OD, no monitoring (anti-Xa assay) except in renal failure and late pregnancy
Unfractionated: IV infusion, monitor APTT
Side effects: heparin induced thrombocytopenia and osteoporosis (more common with UFH)

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25
Q

What is the treatment for heparin overdose?

A

Protamine sulphate

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26
Q

How does warfarin work?

A

Inhibits regeneration of active vitamin K (via reductase enzymes) thus inhibits synthesis of factors 2, 7, 9, 10 and proteins C, S and Z

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27
Q

How does Rivaroxaban work?

A

It is an orally active direct factor Xa inhibitor

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28
Q

How does Dabigatran work?

A

Direct anti-thrombin inhibitor

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29
Q

What is factor V Leiden?

A

A polymorphic mutation which makes Factor V resistant to protein C. Affects 5% of white population

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30
Q

What are the consequences of red cell antibodies (e.g. anti-RhD) in pregnancy?

A

Only IgG antibodies can cross the placenta. If the maternal antibody level is high, they can destroy foetal red cells causing the foetus to become anaemic and jaundiced = Haemolytic Disease of the Newborn.
Rh D = most important cause of severe HDN, also only one with preventative treatment. Other blood groups can cause HDN e.g.: anti-Rh C, anti-K. IgG ABO antibodies can also cause HDN but this is usually not severe.

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31
Q

How is Haemolytic Disease of the Newborn prevented in a RhD negative women carrying an RhD positive foetus?

A

Giving her IM anti-D Ig at times when she is at risk of a feto-maternal haemorrhage;
- routine antenatal prophylaxis at 28 and 34 weeks has been shown to significantly reduce the sensitisation, due to ‘silent’ bleeds in the 3rd trimester
- during pregnancy if sensitising event occurs: abortion, miscarriage requiring medical/surgical evacuation, abdo trauma, external cephalic version, amniocentesis, stillbirth
- at delivery if the baby is RhD positive
Anti-D Ig only works if the mother is not already sensitised

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32
Q

What is a Kleihauer test?

A

A blood test used to measure the amount of foetal haemoglobin transferred from a foetus to a mother’s bloodstream. It is usually performed on Rhesus-negative mothers to determine the required dose of Rho(D) immune globulin to inhibit formation of Rh antibodies in the mother and prevent Rh disease in future Rh-positive children.

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33
Q

What are the two main causes of an immediate severe blood transfusion reaction?

A

1) Wrong (ABO incompatible) blood

2) Bacterial infection of the blood

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34
Q

What immediate adverse reactions to a blood transfusion can occur?

A 
Immune:
- ‘Wrong blood’ ABO incompatibility
- Febrile non-haemolytic
- Allergic / anaphylaxis
- Transfusion related acute lung injury
Non-immune:
- Bacterial infection
- Transfusion associated cardiac overload
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35
Q

What delayed adverse reactions to a blood transfusion can occur?

A 
Immune:
- Delayed haemolytic transfusion reaction
- Port-transfusion purpura
- Transplant -associated GVHD
Non-Immune:
- Viral infections + other
- Iron overload
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36
Q

What causes febrile non-haemolytic transfusion reactions?

A

White cell antibodies in the patient which react with white cells in the donor’s blood, causing fever and rigors. This is less common since leucodepletion of all donor blood.

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37
Q

What causes transfusion related acute lung injury?

A

Very rare. Occurs if the donor’s plasma contains potent white cell antibodies incompatible with the recipient’s white cells. Transfusion may cause a severe reaction characterised by chills, fever, a dry cough and breathlessness with cardiac failure. TACO more likely

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38
Q

What causes a delayed haemolytic transfusion reaction?

A

Many occur in patients who have antibodies (other than ABO), if specially selected blood is not given. Usually manifested by fever days to weeks after a transfusion, accompanied by a falling haemoglobin, and jaundice or haemoglobinuria.

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39
Q

What translation forms the RUNX1-RUNX1T1 fusion protein and what disease is it associated with?

A

t(8;21) = one of the most frequent karyotypic abnormalities in acute myeloid leukaemia (esp M2 subtype)

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40
Q

What is the two-hit model of AML pathogenesis?

A

That two different classes of mutation are needed;

  • Class I mutations confer proliferative and/or survival advantage, but do not affect differentiation e.g. tyrosine kinase mutations
  • Class II mutations serve primarily to impair haematopoietic differentiation and subsequent apoptosis
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41
Q

What are the common presenting features of chronic myeloid leukaemia?

A 
• M:F 1.4:1
• 40-60 years
• Weight loss, lethargy, night sweats
• Splenomegaly (often massive)
• Features of anaemia
• Bruising/bleeding
• Gout
100% BCR:ABL fusion gene
>95% of cases show Philadelphia chromosome
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42
Q

What are the phases of CML?

A

Chronic phase 20% blasts

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43
Q

What is the most sensitive method of assessing residual or relapsed CML?

A

Molecular RT-PCR followed by cytogenetic analysis then haematological response.

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44
Q

What is the first line treatment for CML?

A

Imatinib - BCR-ABL kinase inhibitor - first line therapy for CML in the chronic phase.
If blast crisis (or resistant to all TK inhibitors) - consider allogeneic stem cell transplant

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45
Q

What tyrosine kinase inhibitors are available for CML treatment?

A

Imatinib - first line
Dasatanib
Nilotinib

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46
Q

What disease is associated with t(15;17)?

A

Acute promyelocytic leukaemia

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47
Q

Which chromosomes are commonly duplicated or deleted in AML?

A

Duplicated: 8 and/or 21

Loss or deletion: 5/5q and/or 7/7q

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48
Q

What risk factors are there for AML?

A 
Unknown aetiology
Familial or constitutional predisposition
Irradiation
Anticancer drugs
Cigarette smoking
Benzene exposure
Down's syndrome
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49
Q

What are the clinical features of AML?

A
  • Bone marrow failure: Anaemia, Neutropenia, Thrombocytopenia
  • Local infiltration:
    Splenomegaly
    Hepatomegaly
    Gum infiltration (if monocytic)
    Lymphadenopathy (only occasionally)
    Skin, CNS or other sites
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50
Q

How is AML often diagnosed?

A

Peripheral blood film

  • circulating blasts
  • Auer rods
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51
Q

What are the principles of treatment for AML?

A
  • Supportive care to correct the effects of inadequate bone marrow function (red cells, platelets, antibiotics)
  • Combination chemotherapy
  • Possibly targeted molecular therapy (strongly indicated for acute promyelocytic leukaemia)
  • Possibly immunotherapy
  • Possibly haemopoietic stem cell transplantation
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52
Q

What are the clinical features of ALL?

A
  • Peak incidence in childhood
  • Bone marrow failure: Anaemia, Neutropenia, Thrombocytopenia
  • Local infiltration:
    Lymphadenopathy (± thymic enlargement)
    Splenomegaly
    Hepatomegaly
    Testes, CNS, kidneys or other sites
    Bone (causing pain)
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53
Q

What cytogenetic factors are good / bad prognostic indicators for ALL?

A

Hyperdiploidy - good prognosis
Hypodiploidy - poor prognosis
Philadelphia chromosome - poor prognosis

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54
Q

Which is more common, T- or B-lineage ALL?

A 
B-lineage = 85%
T-lineage = 15%
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55
Q

What are the general principles of treatment for ALL?

A 
Specific therapy:
- systemic chemotherapy (girls 2 yrs, boys 3 yrs)
- CNS directed therapy
Supportive care:
- blood products
- antibiotics
- general medical care
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56
Q

What is the cause of an iron deficiency anaemia and what lab findings would there be?

A 
Bleeding until proven otherwise
- GI tract
- Urinary tract
Laboratory findings:
- Reduced ferritin and transferrin saturation
- Raised TIBC
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57
Q

What are the cancer-associated anaemias?

A
  • Iron deficiency
  • Anaemia of inflammation
  • Leucoerythroblastic anaemia
  • Acquired haemolytic anaemia
    > Immune mediated
    > Fragmentation (microangiopathic) mediated
    Cancer may cause polycythaemia e.g. renal cell cancer
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58
Q

What is the pathogenesis of anaemia of inflammation and what would the laboratory findings be?

A
  • Adequate iron stores but these are not released to the developing erythroid precursors
  • Iron is sequestered and retained in macrophages
    > Normo- or microcytic anaemia
    > Normal or high ferritin
    > Normal or low iron and TIBC
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59
Q

How and why is iron sequestered in macrophages in anaemia of inflammation?

A

Hepcidin binds iron as a defence mechanism against bacterial infection

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60
Q

What are the features of leucoerythroblastic anaemia?

A
  • normocytic normochromic anaemia with numerous poikilocytes
  • normoblasts (nucleated red cells)
  • low-grade reticulocytosis (2-5%)
  • circulating immature white cells, generally myelocytes and promyelocytes
  • thrombocytopaenia is more common than thrombocythaemia
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61
Q

What are the causes of a leucoerythroblastic anaemia?

A

Bone marrow infiltration

  • Cancer: haemopoietic, non-haemopoietic
  • Severe infection: miliary TB, severe fungal infection
  • Myelofibrosis
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62
Q

What are the common lab features of haemolysis?

A
  • anaemia (though may be compensated)
  • reticulocytosis
  • raised Bilirubin (unconjugated)
  • raised LDH
  • reduced haptoglobins
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63
Q

What test is used to distinguish between types of acquired haemolytic anaemia?

A 
Direct Antiglobulin (DAT or Coombs test)
Distinguishes between immune (+) or non-imune (-)
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64
Q

What are the causes of a non-immune acquired haemolytic anaemia?

A

Infection (malaria)
Micro-angiopathic
Paroxysmal nocturnal haemoglobinuria

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65
Q

What features are there on a blood film with microangiopathic haemolytic anaemia?

A

Red cell fragments
Thrombocytopenia
Schistocytes

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66
Q

What are the causes of a microangiopathic haemolytic anaemia?

A

Mechanical RBC destruction by fibrin mesh in vessels

  • haemolytic uraemic syndrome
  • thrombotic thrombocytopenic purpura
  • DIC (adenocarcinomas - low grade DIC)
  • pre-eclampsia
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67
Q

What is paroxysmal nocturnal haemoglobinuria?

A

Marchiafava-Micheli syndrome: a rare, acquired loss of protective surface GPI markers on RBCs leading to complement-induced intravascular haemolytic anaemia, red urine and thrombosis.

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68
Q

What are the causes of a neutrophilia?

A
  • infection
  • corticosteroids
  • underlying neoplasia
  • tissue inflammation (e.g.colitis, pancreatitis)
  • myeloproliferative/ leukaemic disorders
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69
Q

Which infections characteristically do not produce a neutrophilia?

A
  • brucella
  • typhoid
  • many viral infections
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70
Q

What are the causes of a reactive eosinophilia?

A
  • parasitic infestation
  • allergic diseases e.g. asthma, rheumatoid, polyarteritis, pulmonary eosinophilia.
  • Underlying Neoplasms, esp. Hodgkin’s, T-cell NHL (reactive eosinophilia)
  • Drugs (reaction erythema mutiforme)
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71
Q

What are the causes of a monocytosis?

A

Rare but seen in certain chronic infections and primary haematological disorders:

  • TB, brucella, typhoid
  • Viral; CMV, varicella zoster
  • sarcoidosis
  • chronic myelomonocytic leukaemia (MDS)
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72
Q

An ESR >100 mm/hr is suggestive of what?

A
  • Myeloma
  • Systemic autoimmune disease
  • Active TB
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73
Q

What is plasma cell myeloma?

A

A neoplastic proliferation of bone marrow plasma cells associated with a monoclonal protein in serum and/or urine = 1% of all cancers, average survival 3-5 years

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74
Q

What are the clinical features of myeloma?

A
  • asymptomatic
  • Calcium high
  • Renal failure (plus amyloidosis and nephrotic syndrome)
  • Anaemia (and pancytopenia)
  • Bone pain, lytic lesions and fractures
  • hyperviscosity syndrome
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75
Q

What staging system is used for plasma cell myeloma?

A

Durie-Salmon, I-III

Also International Staging System (ISS) based on beta-2-microglobulin

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76
Q

When is the ESR normal in a patient with myeloma?

A

When the myeloma produces only free light chains and no serum paraprotein or is non-secretory (3%)

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77
Q

What are the general aspects of treatment for myeloma?

A
  • Hypercalcaemia: rehydration, intravenous infusion of a bisphosphonate, e.g. pamidronate.
  • Renal failure: correct dehydration, treat hypercalcaemia, dialysis if required
  • Anaemia: usually improves when the disease responds. Blood transfusions may be needed
  • Bone disease: local radiotherapy for pain, long-term bisphosphonate.
  • Infection: treat promptly and vigorously, influenza vaccine annually.
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78
Q

What are the specific treatment options for myeloma?

A
  • Chemotherapy often combined with steroids. e.g. CTD (cyclophosphamide, thalidomide and dexamethasone), melphalan and prednisolone.
  • Bortezomib is used as second-line therapy and lenalidomide is used as third-line therapy.
  • Radiotherapy for local areas of disease, e.g. for pain or cord compression
  • High-dose therapy with stem cell support in younger patients (autologous or allogeneic)
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79
Q

What are myelodysplastic syndromes?

A

A heterogenous group of progressive disorders featuring ineffective proliferation and differentiation of abnormally maturing myeloid stem cells characterised by:
- peripheral cytopenia
- qualitative abnormalities of cell maturation
- risk of AML transformation
Typically seen in elderly, symptoms develop over weeks & months

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80
Q

What is Pelger-Huet anomaly?

A

Hyposegmented neutrophil - bilobed nucleus

Pseudo-Pelger-Huet anomaly can be seen in MDS

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81
Q

What abnormal blood and bone marrow morphological features can be seen in MDS?

A
  • Hypercellular bone marrow (normal < 5% blasts)
  • Defective cells
    > WBCs: Pseudo-Pelger-Huet anomaly and dysganulopoieses of neutrophils, myelokathexis
    > Dyserythropoiesis of RBCs e.g. ring sideroblasts
    > Dysplastic megakaryocytes e.g. micro-megakaryocytes
82
Q

What is myelokathexis?

A

Retention of neutrophils in the bone marrow - often have hypersegmented nuclei

83
Q

What stain is used to show ringed sideroblasts?

A

Prussian blue stain

84
Q

How is prognosis worked out in MDS?

A

International prognostic scoring system, depends on BM blast %, karyotype, degree of cytopenia.
Mortality ‘rule’: 1/3 die from infection, 1/3 bleeding and 1/3 acute leukaemia

85
Q

What is the general treatment for MDS?

A 
Supportive care:
- Blood product support
- Antimicrobial therapy
- Growth factors (Epo, G-CSF)
Biological Modifiers:
- Immunosuppressive therapy
- Azacytidine
- Lenalidomide
Chemotherapy: similar to AML
Allogenic SCT
86
Q

What are the blood and bone marrow features of refractory anaemia? (RA)

A

Blood: anaemia, no blasts
BM: erythroid dysplasia with <5% blasts

87
Q

What are the blood and bone marrow features of refractory anaemia with ringed sideroblasts? (RA +RS)

A

Blood: anaemia, no blasts
BM: erythroid dysplasia with >15% ringed sideroblasts

88
Q

What are the blood and bone marrow features of refractory cytopenia with multi-lineage dysplasia? (RCMD)

A

Blood: Bicytopenia or pancytopenia
BM: Dysplasia in >10% cells in 2 or more cell lines

89
Q

What are the blood and bone marrow features of refractory cytopenia with multi-lineage dysplasia and ringed sideroblasts? (RCMD +RS)

A

Blood: Bicytopenia or pancytopenia
BM: Dysplasia in >10% cells in 2 or more cell lines and >15% ringed sideroblasts

90
Q

What are the blood and bone marrow features of refractory anaemia with excess blasts-1? (RAEB I)

A

Blood: Cytopenias, <5% blasts, no Auer rods
BM: Dysplasias, 5-9% blasts

91
Q

What are the blood and bone marrow features of refractory anaemia with excess blasts-2? (RAEB II)

A

Blood: Cytopenias or 5-19% blasts or Auer rods
BM: Dysplasias, 10-19% blasts or Auer rods

92
Q

What are the blood and bone marrow features of MDS with 5q deletion?

A

Blood: Anaemia, normal or increased platelets
BM: Megakaryocytes with hypolobulated nuclei and <5% blasts

93
Q

What are the blood and bone marrow features of Myelodysplasia Syndrome Unclassified?

A

Blood: Complex - cytopenias, no blasts, no Auer rods
BM: Complex - myeloid or megakaryocytic dysplasia, <5% blasts

94
Q

Blood: anaemia, no blasts
BM: erythroid dysplasia with <5% blasts

A

refractory anaemia (RA)

95
Q

Blood: anaemia, no blasts
BM: erythroid dysplasia with >15% ringed sideroblasts

A

refractory anaemia with ringed sideroblasts (RA +RS)

96
Q

Blood: Bicytopenia or pancytopenia
BM: Dysplasia in >10% cells in 2 or more cell lines

A

refractory cytopenia with multi-lineage dysplasia (RCMD)

97
Q

Blood: Bicytopenia or pancytopenia
BM: Dysplasia in >10% cells in 2 or more cell lines and >15% ringed sideroblasts

A

refractory cytopenia with multi-lineage dysplasia and ringed sideroblasts (RCMD +RS)

98
Q

Blood: Cytopenias, <5% blasts, no Auer rods
BM: Dysplasias, 5-9% blasts

A

refractory anaemia with excess blasts-1 (RAEB I)

99
Q

Blood: Cytopenias or 5-19% blasts or Auer rods
BM: Dysplasias, 10-19% blasts or Auer rods

A

refractory anaemia with excess blasts-2 (RAEB II)

100
Q

Blood: Anaemia, normal or increased platelets
BM: Megakaryocytes with hypolobulated nuclei and <5% blasts

A

MDS with 5q deletion

101
Q

Blood: Complex - cytopenias, no blasts, no Auer rods
BM: Complex - myeloid or megakaryocytic dysplasia, <5% blasts

A

Myelodysplasia Syndrome Unclassified

102
Q

What inherited bone marrow failure syndromes are there?

A

Fanconi anaemia
Dyskeratosis congenita
Schwachman-Diamond syndrome
Diamond-Blackfan syndrome

103
Q

What are the acquired causes of aplastic anaemia?

A 
Idiopathic (70%)
Marrow infiltration:
> Haematological (leukaemia, lymphoma, myelofibrosis)
> Non-haematological (Solid tumours)
Radiation
Drugs
Chemicals (benzene)
Autoimmune (SLE)
Infection (Parvovirus, Viral hepatitis)
104
Q

What is the general management of aplastic anaemia?

A

> Supportive - transfusions, antibiotics, iron chelation
Drugs to promote marrow recovery - growth factors, oxymetholone
Immunosuppressants (idiopathic AA ~70%)
Stem cell transplant

105
Q

What is aplastic anaemia and how is it classified?

A

Aplastic anaemia is defined as pancytopenia with a hypocellular bone marrow in the absence of an abnormal infiltrate and with no increase in reticulin.
It is classified as non-severe, severe, or very severe on the basis of the degree of peripheral-blood pancytopenia

106
Q

What is Fanconi anaemia?

A

Commonest form of inherited (AR or X) aplastic anaemia.
Multiple genes responsible: normally contribute to genomic stability.
Blood counts and marrow cellularity often normal until 5-10 yrs then pancytopenia develops, 10% progress to AML.
Skeletal abnormalities (thumbs), renal malformations, microcephaly, hypogonadism, short stature, skin pigmentation.

107
Q

What is dyskeratosis congenita?

A

X-linked disorder characterised by marrow failure, cancer predisposition and somatic abnormalities.
Classic triad: skin pigmentation, nail dystrophy, oral leukoplakia

108
Q

What is Schwachman-Diamond syndrome?

A

A rare AR congenital disorder characterized by exocrine pancreatic insufficiency, bone marrow dysfunction (neutropenia +/- others), skeletal abnormalities, and short stature.

109
Q

What is Diamond-Blackfan Syndrome?

A

A congenital hypoplastic anaemia that usually presents in infancy with pure red-cell aplasia, normal WCC and platelets. Often other congenital anomalies.

110
Q

What are the blood count changes in pregnancy?

A

Mild anaemia: RCM 120-130%, plasma volume 150% = net dilution
Macrocytosis: MCV rises 5-10 fl
Neutrophilia
Thrombocytopenia: increased platelet size

111
Q

What supplements are recommended during pregnancy?

A

Iron: 3x increase in requirements, need extra 800mg, RDA 30mg. No routine supplementation in UK
Folate: additional 200mcg/day required
WHO recommends 60mg Fe +400mcg folic acid daily during pregnancy

112
Q

What possible effects are there of iron deficiency during pregnancy?

A

Preterm delivery
Low birth weight,
Possibly placental abruption,
Increased peripartum blood loss,

113
Q

What happens to the platelet count during pregnancy?

A

Decrease ~10% - can drop into thrombocytopenic range.

Combination of haemodilution and increased platelet activation and clearance.

114
Q

What are the causes of thrombocytopenia in pregnancy?

A
  • Physiological
  • Pre-eclampsia
  • Immune thrombocytopenia (ITP)
  • Microangiopathic syndromes
  • All other causes: bone marrow failure, leukaemia, hypersplenism, DIC etc.
115
Q

What is the relationship between pre-eclampsia and thrombocytopenia?

A

50% get thrombocytopenia: proportionate to severity.
Probably due to increased activation and consumption.
Usually remits following delivery.

116
Q

What are the treatment options for immune thrombocytopenia in pregnancy?

A 
When symptomatic or count <20
IV immunoglobulin
Steroids etc.
(Anti-D where Rh D +ve)
Baby may be affected for up to 5 days post delivery (IVIG if low)
117
Q

What are the main coagulation changes in pregnancy?

A

Increase in vWF (x3-5), fibrinogen (x2) and factors 7, 8, 10
50% decrease in protein S
Also increase in plasminogen activator inhibitors 1+2
Net effect = procoagulant state

118
Q

When are pregnant women at risk of thromboembolism?

A

From the very beginning of pregnancy until well into postpartum period

119
Q

What factors increase the risk of thrombosis in pregnancy?

A 
Bed rest
Obesity
Pre-eclampsia
Operative delivery
Previous thrombosis/thrombophilia
Age
Parity
Multiple pregnancy
IVF: ovarian hyperstimulation
120
Q

How should pregnant women with VTE risk factors be managed?

A 
- Prophylactic heparin +TED stockings
> Either throughout pregnancy
> Or in peri-post- partum period 
> Highest risk get adjusted dose LMWH heparin
- Mobilise early
- Maintain hydration
121
Q

How should pregnant women with a VTE be managed?

A
  • LMWH as for non-pregnant
  • Do not convert to warfarin (teratogenic)
  • After 1st trimester monitor anti Xa
  • Stop for labour or planned delivery, esp. for epidural
122
Q

What is the definition of post partum haemorrhage and what are the major factors?

A

> 500 ml blood loss
- uterine atony
- trauma
haematological factors minor except
- dilutional coagulopathy after resuscitation
- DIC in abruption, amniotic fluid embolism etc

123
Q

What conditions can precipitate decompensation to DIC in pregnancy?

A 
Amniotic fluid embolism
Abruptio placentae
Retained dead fetus
Pre-eclampsia (severe) 
Sepsis
124
Q

What possible consequences of haemolytic anaemia are there?

A

Anaemia(+/-)

  • Erythroid hyperplasia with increased rate of RBC production and circulating reticulocytes
  • Increased folate demand
  • Susceptibility to effect of parvovirus B19
  • Increased risk of gallstones, iron overload, osteoporosis
125
Q

What are the main inherited causes of haemolytic anaemia?

A
  • Membrane defects: hereditary spherocytosis, hereditary elliptocytosis
  • Enzyme defects: G6PD deficiency, pyruvate kinase deficiency
  • Haemoglobinopathies: sickle cell disease, thalassaemias
126
Q

What is hereditary spherocytosis?

A

75% AD cause of extravascular haemolysis
Spectrin or ankyrin deficiency
Suseptible to parvovirus B19 and gallstones
Diagnosed by blood film and osmotic fragility test
Treat with splenectomy and folic acid

127
Q

What is hereditary elliptocytosis?

A

AD spectrin mutations

Broad range in severity

128
Q

What does G6PD do?

A

Glucose-6-phosphate dehydrogenase catalyses first step in pentose phosphate pathway - generates NADPH required to maintain intracellular glutathione (GSH)

129
Q

Where is G6PD deficiency prevalent?

A

In areas of malarial endemicity i.e.. African, Mediterranean and Middle Eastern populations

130
Q

What are the clinical features of G6PD deficiency?

A 
X-linked RBC enzyme deficiency
Neonatal jaundice
Acute haemolytic attacks with bite cells, Heinz bodies, dark urine
Attacks precipitated by oxidants
Chronic haemolytic anaemia (rare)
131
Q

What agents may provoke haemolysis in G6PD deficiency?

A
  • Anti-malarials: Primaquine
  • Antibiotics: Sulphonamides, Ciprofloxacin, Nitrofurantoin
  • Other drugs: Dapsone, Vitamin k, Aspirin
  • Fava beans
  • Mothballs
132
Q

For which haemolytic anaemias is there substantial benefit from splenectomy?

A 
PK deficiency and some other enzymopathies
Hereditary spherocytosis
Severe elliptocytosis/pyropoikilocytosis
Thalassaemia syndromes
Immune haemolytic anaemia
133
Q

What are the indications/criteria for splenectomy as a treatment for haemolytic anaemia?

A 
Transfusion dependence
Growth delay
Physical limitation Hb <  8g/dl
Hypersplenism
Age 	not < 3 yrs but before 10 yrs to maximise prepubertal growth
134
Q

What is pyruvate kinase deficiency?

A

Majority autosomal recessive
Severe neonatal jaundice, splenomegaly, haemolytic anaemia
Most do not require treatment but may include transfusion or splenectomy

135
Q

What is the incidence of lymphoma?

A

1 new case per 5000 each year
Non-Hodgkin’s Lymphomas 80%
Hodgkin Lymphoma 20%

136
Q

What risk factors are there for lymphoma?

A
  • Majority of case no identifiable risk factors
  • Constant antigenic stimulation
  • Infection(viral infection of cells)
  • Immunosupression (HIV and immunosuppresants)
137
Q

What three inherent characteristics of the immune system make it susceptible to malignant change?

A

Rapid cell proliferation in immune response
> Proliferating cells risk DNA replication error
Dependent on apoptosis (90% of normal cells die!)
> Apoptosis is “switched off” in germinal centre
DNA molecules are cut and rejoined plus undergo deliberate point mutations
> Potential for rejoining errors and new point mutations

138
Q

What is the epidemiology of Hodgkin’s Lymphoma?

A

M>F
20-29 peak, second smaller peak >60
EBV-associated

139
Q

What is the classical clinical presentation of Hodgkin’s Lymphoma?

A
  • Asymmetrical painless lymphadenopathy =/- obstructive/mass effect symptoms
    +/- Constitutional (B) symptoms; fever, night sweats, weight loss, pruritis
  • Rarely alcohol induced pain
  • Pel-Ebstein fever: cyclical 1-2wk in minority
  • reed-sternberg cell
140
Q

What subtypes of Hodgkin’s Lymphoma are there?

A 
Nodular sclerosing 80%
Mixed cellularity 17%
Lymphocyte rich (rare)
Lymphocyte depleted (rare, poor prog)
Nodular Lymphocyte predominant 5% (not classical HL)
141
Q

How is Hodgkin’s Lymphoma staged?

A

I - one group of nodes (includes spleen)
II - >1 group of nodes same side of the diaphragm
III - nodes above and below the diaphragm
IV - extra nodal spread (liver, BM)
Plus A if no constitutional symptoms, B if there are any
Same staging system used for NHL too.

142
Q

What is the commonest chemotherapy regime used to treat Hodgkin’s Lymphoma?

A

ABVD: Adriamycin, Bleomycin, Vinblastine, Dacarbazine (DITC)

  • given at 4-weekly intervals
  • preserves fertility
  • can cause pulmonary fibrosis and cardiomyopathy later
143
Q

What is non-Hodgkin’s lymphoma?

A 
All lymphomas other than Hodgkin's,
~ 40 different subtypes
Can be classified as;
- mature or immature
- high or low grade
- B or T cell lineage
144
Q

What are the clinical features of non-Hodgkin’s lymphoma?

A

Presentation varies significantly from subtype to subtype
Similarities: painless lymphadenopathy, often involving multiple sites, constitutional symptoms, no pain after alcohol
Staging as per Hodgkin’s

145
Q

Examples of indolent, low grade non-Hodgkin’s lymphomas?

A
  • Follicular lymphoma
  • Small lymphocytic/CLL
  • Mucosa associated (MALT)
146
Q

Examples of aggressive, high grade non-Hodgkin’s lymphomas?

A 
Aggressive
- Diffuse Large B cell
- Mantle cell
Very aggressive
- Burkitt Lymphoma 
- T or B cell Lymphoblastic leukaemia/lymphoma
147
Q

What is the commonest subtype of non-Hodgkin’s lymphoma and what is a standard treatment regime?

A 
Diffuse large B cell lymphoma 30-40% of all NHL
6-8 cycles of R-CHOP
- Rituximab
- Cyclophosphamide
- Adriamycin (Hydroxydaunomycin)
- Vincristine (Oncovin)
- Prednisolone
148
Q

How is prognosis calculated for diffuse large B cell lymphoma?

A

International Prognostic Index, a point for each of;

  • Age > 60y
  • serum LDH > normal
  • performance status 2-4
  • stage III or IV
  • more than one extranodal site
149
Q

What is the commonest indolent non-Hodgkin’s lymphoma and what is it associated with?

A

Follicular lymphoma ~35% of NHL
Associated with t(14;18) which results in over-expression of bcl2 an anti-apoptosis protein
Incurable, median survival 12-15 years

150
Q

What is a MALT lymphoma?

A

A Marginal zone NHL involving extranodal lymphoid tissue due to chronic antigen stimulation;
- Sjogrens syndrome ; parotid lymphoma
- H.Pylori ; Gastric MALT lymphoma
Can be pathogen dependent so antibiotics cure

151
Q

What sections can the lymphoreticular system be classified into?

A
  • Generative: Bone marrow and thymus
  • Reactive: Lymph nodes and spleen
  • Acquired: Extranodal lymphoid tissue
152
Q

What is the definition of a lymphoma?

A

Neoplastic proliferation of lymphoid cells forming discrete tissue masses.
Arise in and involve lymphoid tissues (including acquired lymphoid tissue).

153
Q

What immunohistochemistry and translocations identify a folicular lymphoma?

A
  • CD10, bcl-6 (GC) and bcl-2

- 14;18 translocation involving bcl-2 gene

154
Q

What immunohistochemical profile does small lymphocytic lymphoma/CLL have?

A

CD5, CD23 +

155
Q

What immunohistochemical profile and translocations does mantle cell lymphoma have?

A
  • CD5, cyclin D1 over expression

- 11;14 translocation

156
Q

What are the features and associations of Burkitt’s lymphoma?

A
  • Jaw or abdominal mass children/young adults
  • EBV associated
  • “starry-sky” appearance on histology
  • CD10, bcl6 (GC)
  • C-myc translocation (8:14, 2:8, 8;22)
157
Q

Which has a better prognosis, germinal center or post-germinal center diffuse large B cell lymphoma?

A

Germinal center phenotype

158
Q

What type of lymphoma is associated with HTLV-1?

A

Human T-lymphotropic virus type I causes adult T cell leukaemia and lymphoma

159
Q

What translocation and protein expression is associated with anaplastic large cell lymphoma?

A

2;5 translocation
Alk-1 protein expression
CD30 +, T cell or null phenotype

160
Q

What is the histological picture of Hodgkin’s lymphoma?

A

Sclerosis, mixed cell population in which scattered Reed-Sternberg and Hodgkin cells with eosinophils

161
Q

What is chronic lymphocytic leukaemia and how does it normally present?

A

Progressive accumulation of malignant mature B lymphocytes in the blood, bone marrow and lymphoid tissues. Commonest leukaemia in western world.
70-80% incidental diagnosis, majority over 60.
Bone marrow failure, recurrent sinopulomary infection (hypogamaglobulinaemia) and auto-immune phenomena (AIHA, ITP)

162
Q

What peripheral blood and bone marrow findings are there in chronic lymphocytic leukaemia?

A
  • Lymphocytosis between 5 and 300 x 10^9/L
  • Smear cells
  • Normocytic normochromic anaemia
  • Thrombocytopenia
    Bone marrow: lymphocytic replacement of normal marrow elements
163
Q

What does immunophenotyping usually reveal in chronic lymphocytic leukaemia?

A 
CD5
CD19
CD20
CD23
CD79a
Surface IgM
164
Q

What staging systems are used for CLL?

A

Rai staging (0-IV) or Binet staging (A-C)

165
Q

What chromosomal abnormalities detected via FISH are prognostic for CLL?

A

Deletion of 13q - good prognosis, better than normal
Trisomy 12 - good
Deletion of 11q (ATM) - poor
Deletion of 17p (TP53) - very poor

166
Q

What carries a better prognostic value, mutated or unmutated IgH gene in CLL?

A

Unmutated worse prognosis

167
Q

What is Richter’s syndrome?

A

Aka Richter’s transformation, is a complication of CLL and hairy cell leukaemia in which the leukaemia changes into a fast-growing diffuse large B cell lymphoma or Hodgkin’s lymphoma. Happens to 5-10% of CLL patients. Treat with CHOP-R

168
Q

What are the indications for treatment of CLL?

A
  • Progressive lymphocytosis >50% increase over 2 months or lymphocyte doubling time <6 months
  • Progressive marrow failure
  • Massive or progressive lymphadenopathy/splenomegaly
  • Systemic (B) symptoms
  • Autoimmune cytopenias (treat with steroids or Rituximab)
169
Q

What antibodies are sometimes used as therapies for CLL and when?

A 
Alemtuzemab/Campath: anti-CD52
> 1st line: 17p deleted patients 
> 2nd line: relapsed refractory patients
> Toxicity - T-cell immunosuppression, CMV reactivation, fevers
Rituximab: anti-CD20
> part of FCR standard 1st line
170
Q

What oral chemotherapy drug is used in CLL?

A

Chlorambucil

171
Q

What is FCR combination chemotherapy?

A

Fludarabine, cyclophosphamide and rituximab.

Used to treat CLL

172
Q

What are the causes of polycythaemia in the foetus or neonate?

A
  • Twin-to-twin transfusion
  • Intrauterine hypoxia
  • Placental insufficiency
173
Q

What are some unique causes of anaemia in the foetus or neonate?

A
  • Twin-to-twin transfusion
  • Fetal-to-maternal transfusion
  • Parvovirus infection (virus not cleared by immature immune system)
  • Haemorrhage from the cord or placenta
174
Q

When does the first mutation that leads to acute lymphobastic leukaemia usually occur?

A

in utero.

Pre-leukaemic cells carrying this mutation can even spread from one twin to another

175
Q

What type of leukaemia solely affects children with Down’s syndrome?

A

Transient myeloproliferative disease aka transient abnormal myelopoiesis (TAM)
Affects 3–10% of infants, not typically serious, often resolves without treatment.
20–30% risk of developing acute lymphoblastic leukemia

176
Q

Why is sickle cell anaemia not clinically apparent at birth?

A

Clinical features manifest as gamma chain production and haemoglobin F synthesis decrease and betaS and haemoglobin S production increase.

177
Q

Why/how does sickle cell anaemia in the infant and child differ from the same disease in the adult?

A

> The distribution of red bone marrow (susceptible to infarction) differs - the hand/foot syndrome
The infant still has a functioning spleen - splenic sequestration can occur
The infant has an immature immune system and has not developed immunity to pneumococcus or parvovirus
The infant and child is growing rapidly and has a greater need for folic acid

178
Q

What is splenic sequestration?

A

> Acute pooling of a large percentage of circulating red cells in the spleen, causes the spleen to enlarge and the Hb to fall acutely and death can occur.
Doesn’t happen in older children and adults because recurrent infarction has left the spleen small and fibrotic

179
Q

Why is infection with parvovirus B19 more serious in children with sickle cell disease than in a normal child?

A

Parvovirus infection nearly completely prevents red blood cell production for 2-3 days. In normal individuals, this is of little consequence, but the shortened red cell life of sickle-cell patients results in an abrupt, life-threatening anaemia that may require transfusion.

180
Q

Why does folic acid matter more in a child with sickle cell disease than in a normal child or an adult?

A
  • Hyperplastic erythropoiesis requires folic acid
  • Growth spurts require folic acid
  • Red cell life span is shorter so anaemia can rapidly worsen
181
Q

How do we manage sickle cell anaemia and other forms of sickle cell disease in the infant and child?

A

Accurate diagnosis
Educate parents
Vaccinate
Prescribe folic acid and penicillin

182
Q

Siblings with sickle cell anaemia present simultaneously with severe anaemia and a low reticulocyte count—likely diagnosis?

A

Parvovirus B19 infection

183
Q

What is beta thalassaemia?

A

A condition resulting from reduced synthesis of beta globin chain and therefore haemoglobin A. It often becomes apparent in the first 3-6 months of life.
Beta thalassaemia homozygosity causes a severe anaemia that, in the absence of blood transfusion, is fatal in the first few years if life.

184
Q

What are the clinical effects of poorly treated thalassaemia major?

A

Anaemia > heart failure, growth retardation
Erythropoietic drive > bone expansion, hepatomegaly, splenomegaly
Iron overload > heart failure, gonadal failure

185
Q

How do we manage an infant/child with beta thalassaemia major?

A
  • Accurate diagnosis and family counselling
  • Blood transfusion
  • Once iron overload starts to occur, chelation therapy (desferioxamine, deferiprone)
  • Consideration of the child as an individual and as part of a family
186
Q

What specific questions can be asked in the history of a child with suspected inherited defect of coagulation?

A
  • Was there umbilical cord bleeding or bleeding when a heel-prick was done for Guthrie spot?
  • Was there haematoma formation after vitamin K injection of vaccinations?
  • Was there bleeding after circumcision?
187
Q

A 1-year-old boy presents with joint bleeding, Hb, WBC and platelet count are normal, aPTT is prolonged, PT is normal, bleeding time normal—most likely diagnosis?

A

Haemophilia A (more common than B)

188
Q

What is the treatment for autoimmune thrombocytopenia purpura?

A
  • Observation (often remits spontaneously)
  • Corticosteroids
  • High dose intravenous immunoglobulin
  • Intravenous anti Rh D (if Rh-positive)
189
Q

What cell marker is specific for haematopoetic stem cells?

A

CD34

190
Q

What complications can occur after a stem cell transplant?

A
  • Graft failure
  • Infections
  • Graft-versus-host disease (GVHD): allografting only
  • Relapse
191
Q

What different phases of immune defects are there following an allogenic bone marrow transplant?

A 
Aplastic phase (0-3 wks)
GVHD phase (3 wks - 6 mths)
Late phase (6 mths - yrs)
192
Q

What pathogens is a patient particularly susceptible to in the aplastic phase following a bone marrow transplant?

A 
Bacteria gram +/-
Candida
HSV
RSV
Influenza
193
Q

What pathogens is a patient particularly susceptible to in the GVHD phase following a bone marrow transplant?

A 
CMV
VZV
HHV-6
Aspergillus (10-15% deaths)
Candida
Adenovirus
194
Q

What pathogens is a patient particularly susceptible to in the late phase following a bone marrow transplant?

A

Pneumococci
H. influenzae
VZV
RSV

195
Q

What are some of the treatment options for GVHD?

A 
Corticosteroids
Cyclosporin A
FK506
Mycophenylate mofetil
Monoclonal antibodies
Photopheresis
Total lymphoid irradiation
196
Q

What treatments are used to prevent GVHD?

A 
Methotrexate
Corticosteroids
Cyclosporin A
CsA plus MTX
FK506
T-cell depletion
197
Q

What drugs are frequently used as a conditioning regimen prior to stem cell transplantation and what are the common side effects?

A
  • melphalan, busulphan and cyclophosphamide
  • side effects include alopecia, gastro-intestinal problems of nausea, vomiting, diarrhoea and mucositis, cardiotoxicity, pulmonary fibrosis, haemorrhagic cystitis, liver toxicity known as veno-occlusive disease and gonadal failure.
198
Q

What therapy is most commonly used as a conditioning regimen prior to allogenic-SCT and what common side effects are there?

A
  • total body irradiation (TBI)
  • side effects include gastro-intestinal disturbance, pulmonary damage, pancreatitis, parotitis, gonadal failure and veno-occlusive disease
199
Q

How is acute GCHD defined?

A

Occurs within 100 days of SCT and usually affects the skin, gastro-intestinal tract and liver
Fatal in 10% of recipients of sibling grafts and in 20% of recipients of unrelated volunteer cells

200
Q

What are the transplant related mortality figures for stem cell transplants?

A

Risk of death due to the transplant as opposed to the disease:
~5% in auto-SCT,
20-30% in sibling allo-SCT
30-50% in unrelated allo-SCT.

201
Q

What are the normal ranges for WBC, Hb, MCV, and platelet count in adults?

A

WBC
Hb
MCV
Platelets

Pathology (5 decks)

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