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Phase 3 > Haematology > Flashcards

Haematology Flashcards

1
Q

How can myelodysplastic syndrome be distinguished from AML?

A

Percentage of blast cells in bone marrow
<20% myelodysplastic syndrome
>20% AML

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2
Q

What is an auer rod and which condition do you see them in?

A

Granular material that forms elongated needles in cytoplasm of myeloid leukaemic blasts
Myeloperoxidase aggregate

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3
Q

What are smudge/smear cells and what condition do you see them in?

A

Remnants of cells that lack identifiable cytoplasmic membrane or nuclear structure
Associated with fragile lymphocytes in conditions like CLL

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4
Q

Which leukaemia is response to tretinoin therapy?

A

Acute promyelocytic leukaemia

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5
Q

What is myelofibrosis?

A

Proliferation of abnormal clone of haematopoietic stem cells in bone marrow and other sites leads to fibrosis

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6
Q

What are signs and symptoms of myelofibrosis?

A 
Splenomegaly
Bone pain
Bruising and easy bleeding
Cachexia 
Hepatomagaly 
Fatigue 
Gout and high uric acid levels 
Increased susceptibility to infection
Pallor and SOB - anaemia
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7
Q

Why does malignant hypertension lead to haemolytic anaemia?

A

Mechanical - red blood cells rupture as they become damaged when forced at high pressures through microvasculature

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8
Q

What makes autoimmune haemolytic anaemias warm or cold?

A

Description of what temperatures the antibodies against the RBCs best work

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9
Q

What blood test abnormalities would you expect to find in DIC?

A

Increased PT and APTT
Anaemia
Thrombocytopenia
Increased fibrin degradation products

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10
Q

What are some causes of DIC?

A

Leukaemias
Serious infections - meningococcus, E. coli
Obstetric emergencies

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11
Q

What are signs and symptoms of an acute haemolytic transfusion reaction?

A

> 2 degrees rise in temperature
Abdominal pain
Hypotension

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12
Q

What features are typical of Von Willibrands disease?

A 
Lifelong tendency to easy bruising
Frequent epistaxis
Menorrhagia 
Purpura
Post op bleeding
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13
Q

Which clotting result would be deranged in Von willebrands disease?

A

Prolonged activated partial thromboplastin time

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14
Q

What can be precipitants of haemolysis in G6PD deficiency?

A

Consumption of fava beans
Antimalarials
Nitrofurantoin

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15
Q

What is marrow involvement in Hodgkin’s disease best treated with?

A

Chemotherapy

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16
Q

What can cause a raised ESR?

A

RA
SLE
Polymyalgia rheumatica
Myeloma

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17
Q

What happens to ESR with age?

A

Increases

ESR normally 1/2 age

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18
Q

What happens to ESR in polycythemia?

A

Low

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19
Q

A 6 month old boy was noticed to be pale but otherwise well and gaining weight. The liver was 3cm below costal margin and 2cm spleen palpable. Haemoglobin 80, reticulocyte count 4%, WCC normal. What is the likely diagnosis? Why?

A

Beta thalasseamia major

Anaemia, extramedullary haematopoiesis, hepatosplenomegaly and raised reticulocytes

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20
Q

What does chloramphenicol toxicity do to bone marrow?

A

Complete suppression

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21
Q

What clinical features does G6PD deficiency cause?

A

Episodes of haemolysis - jaundice, abdominal pain, dark urine

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22
Q

What is the commonest presenting symptom of myeloma?

A

Bone pain

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23
Q

What causes polyuria in myeloma?

A

Hypercalcaemia due to bone destruction

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24
Q

What are the main antigens that give rise to graft rejection?

A

ABO blood group
Human leukocyte antigen (HLA)
Minor histocompatibility antigens

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25
Q

What are some causes of hyperacute organ rejection?

A

Blood group ABO incompatibility

Pre existing HLA antibodies

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26
Q

What are the 4 most important HLA types in organ rejection?

A

HLA A
HLA B
HLA C
HLA DR

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27
Q

If a patient is HLA mismatched with their donor for organ transplant, what will happen?

A

T lymphocytes will recognise antigens bound to HLA molecules and become activated
Clonal expansion occurs with a response directed at that antigen

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28
Q

What are the different types of organ rejection?

A

Hyperacute: immediately through pre formed antigens
Acute: during first 6 months, usually T cell mediated, tissue infiltrates and vascular lesions, mononuclear cell infiltrates
Chronic: after 6 months, vascular changes predominate, myointimal proliferation leading to organ ischaemia, loss of acinar cells in pancreas, rapidly progressive CAD in cardiac transplants

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29
Q

Which transplants are at the highest risk of hyperacute organ rejection?

A

Renal

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30
Q

Give some examples of immune mediated blood transfusion reactions

A 
Pyrexia
Alloimmunisation
Thrombocytopenia 
Transfusion associated lung injury
Graft versus host disease
Urticaria
Acute or delayed haemolysis
ABO incompatibility
Rhesus incompatibility
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31
Q

Give some examples of non immune mediated blood transfusion reactions

A

Hypocalcaemia
CCF
Infections
Hyperkalaemia

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32
Q

What is graft versus host disease?

A

Lymphocyte proliferation causing organ failure

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33
Q

What is transfusion associated lung injury?

A

Neutrophil mediated allergic pulmonary oedema

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34
Q

What are some oral signs of AIDS?

A

Oral candidiasis
Hairy leukoplakia
Oral gingival regression
Kaposis sarcoma

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35
Q

What is wrong with the haemoglobin of patients with sickle cell anaemia? What is different with sickle cell trait?

A

Substitution of valine for glutamic acid at position 6 of the beta chain
Abnormal haemoglobin depolymerises at PO2 of 5-6 kPa which is found in normal venous blood so they are continuously sickling
Heterozygotes contain normal and abnormal Hb and only sickle at extremely low PO2 values of 2.5-4kPa

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36
Q

What is important in the peri operative management of sickle cell disease patients?

A 
Keep well oxygenated 
Keep warm
Keep them hydrated
Provide adequate analgesia with PCA
Avoid acidosis (venous stasis)
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37
Q

What can precipitate sickling in sickle cell disease?

A 
Hypoxaemia 
Hypothermia
Dehydration 
Infection 
Exertion
Stress
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38
Q

What are clinical features of multiple myeloma?

A 
Bone disease: pain, osteoporosis, pathological fractures, osteolytic lesions
Lethargy
Infection
Hypercalcaemia 
Renal failure
Amyloidosis 
Carpal tunnel syndrome
Hyperviscosity
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39
Q

What investigations should be done for multiple myeloma?

A

Monoclonal proteins: IgG or IgA in serum and urine (bence jones proteins)
Increased plasma cells in bone marrow
Bone lesions on skeletal survey

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40
Q

What are the criteria for diagnosis of multiple myeloma?

A

Major: plasmacytoma, 30% plasma cells in bone marrow sample, elevated levels of M protein in blood or urine
Minor: 10-30% plasma cells, minor elevations in M protein, osteolytic lesions, low levels of antibodies in blood
Diagnosis requires 1 major and 1 minor or 3 minor in someone with signs and symptoms

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41
Q

Why does hypercalcaemia occur in multiple myeloma?

A

Increased osteoclastic bone resorption caused by local cytokines released by myeloma cells
Impaired renal function
Increased renal tubular calcium reabsorption and elevated PTHrP levels

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42
Q

What features might be seen on a blood film in a hyposplenic patient?

A 
Target cells
Howell Jolly bodies
Pappenheimer bodies
Siderotic granules
Acathocytes
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43
Q

What features might be seen on a blood film of a patient with iron deficiency anaemia?

A

Target cells

Pencil poikilocytes

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44
Q

What feature is seen on a blood film of a patient with myelofibrosis?

A

Tear drop poikilocytes

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45
Q

What feature is seen on blood film of a patient with intravascular haemolysis?

A

Schistocytes

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46
Q

What is seen on a blood film of a patient with megaloblastic anaemia?

A

Hypersegmented neutrophils

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47
Q

What is the most common inherited bleeding disorder?

A

Von Willebrands disease

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48
Q

What are common features of Von willebrands disease?

A

Epistaxis
Menorrhagia
Bleeding after tooth extraction

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49
Q

What are the different types of Von willebrands disease?

A

Type 1: partial reduction in vWF
Type 2: abnormal form
Type 3: total lack of (autosomal recessive)

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50
Q

How is Von willebrands investigated?

A

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

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51
Q

How is Von willebrands managed?

A

Tranexamic acid for mild bleeding
Desmopressin: raises levels of vWF by inducing release from Weibel-Palade bodies in endothelial cells
Factor VIII concentrate

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52
Q

What are the 4 different crises of sickle cell anaemia?

A

Thrombotic (painful)
Sequestration
Aplastic
Haemolytic

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53
Q

What is a thrombotic crisis in sickle cell anaemia?

A

Painful, vaso occlusive
Precipitated by infection, dehydration, deoxygenation
Infarction organs including bones

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54
Q

What is sequestration crisis in sickle cell anaemia?

A

Sickling within organs such as spleen or lungs causes pooling of blood with worsening of anaemia
Acute chest syndrome: dyspnoea, chest pain, pulmonary infiltrates, low PO2

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55
Q

What is the most common cause of death after childhood in sickle cell anaemia?

A

Acute chest syndrome

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56
Q

What is aplastic crisis in sickle cell anaemia?

A

Infection with parvovirus

Sudden fall in haemoglobin

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57
Q

What is a haemolytic crisis in sickle cell anaemia?

A

Rare

Fall in haemoglobin due to increased haemolysis

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58
Q

What are the histological classes of Hodgkin’s lymphoma? Which has the best and worst prognosis?

A

Nodular sclerosing - good prognosis
Mixed cellularity - good prognosis
Lymphocyte predominant - best prognosis
Lymphocyte depleted - worst prognosis

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59
Q

What are B symptoms of Hodgkin’s lymphoma?

A

Weight loss >10% in last 6 months
Fever >38
Night sweats

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60
Q

What factors are associated with poor prognosis of Hodgkin’s lymphoma?

A 
B symptoms
Age >45
Stage IV disease
Haemoglobin <10.5
Lymphocyte count <600
Male
Albumin <40
White cell count >15000
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61
Q

What is Burkitts lymphoma?

A

High grade B cell neoplasm
Endemic (Africa): maxilla or mandible
Sporadic: abdominal (ileo-caecal), commonly with HIV

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62
Q

What gene translocation is associated with Burkitts lymphoma?

A

C myc t(8:14)

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63
Q

Which virus is strongly implicated in the development of the African form of Burkitts lymphoma?

A

Epstein Barr virus

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64
Q

What are microscopy findings in Burkitts lymphoma?

A

Starry sky appearance: lymphocyte sheets interspersed with macrophages containing dead apoptotic tumour cells

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65
Q

How is Burkitts lymphoma managed? What problem can this cause?

A

Chemotherapy

Rapid response which may cause tumour lysis syndrome

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66
Q

What is given before chemo in Burkitts lymphoma to reduce the risk of tumour lysis syndrome occur if?

A

Rasburicase

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67
Q

What are complications of tumour lysis syndrome?

A 
Hyperkalaemia
Hyperphosphataemia
Hypocalcaemia
Hyperuricaemia 
Acute renal failure
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68
Q

What is the Philadelphia chromosome?

A

t(9:22)(q34;q11)
Associated with CML
Results in tyrosine kinase activity in excess of normal

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69
Q

A 68 year old male presents to GP with enlarged nodes in neck, axillae and groin. Blood tests show Hb 105, WBC 19. A blood film shows 60% white cells are small mature lymphocytes. What is the most likely diagnosis?

A

Chronic lymphocytic leukaemia

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70
Q

What are features of CLL?

A 
Often none
Constitutional: anorexia, weight loss
Bleeding
Infections
Lymphadenopathy
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71
Q

What are complications of CLL?

A

Hypogammaglobulinaemia leading to recurrent infections
Warm autoimmune haemolytic anaemia
Transformation to high grade lymphoma (Richters transformation)

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72
Q

What investigations are done for CLL?

A

Blood film: smudge cells/smear cells

Immunophenotyping

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73
Q

What is the Ann Arbor staging for non hodgkins lymphoma?

A

Stage 1: involvement of single lymph node region
Stage 2: involvement of 2 or more regions on same side of diaphragm
Stage 3: involvement of lymph node regions on both sides of the diaphragm
Stage 4: multifocal involvement of 1 or more extralymphatic sites +/- associated lymph nodes

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74
Q

Which vaccines are live attenuated and therefore shouldn’t be given to patients who are immunosuppressed?

A 
BCG
MMR
Oral polio
Yellow fever
Oral typhoid
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75
Q

By how much does 1 unit of blood increase Hb concentration?

A

10g/L

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76
Q

What are the most important symptoms/complications of myeloma?

A 
CRAB
Calcium raised
Renal failure
Anaemia 
Bone pain
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77
Q

What is the difference between warm and cold agglutinins?

A

Autoantibodies react with red cells at temps above 37 in warm and below 37 in cold
Both can be lymphoproliferative disorders
Warm primarily in spleen, IgG
Cold: infections (mycoplasma/EBV)

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78
Q

What is the diagnosis in a 7 year old boy presenting with haemarthrosis of his left knee. He has had repeated episodes of this since his early years. His APTT is prolonged but bleeding time is normal?

A

Haemophilia A

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79
Q

How are minor and major bleeds associated with haemophilia treated?

A

Minor: desmopressin which raises factor VIII levels
Major: factor VIII concentrate to raise levels above 50%

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80
Q

What is the management for an alcoholic patient presenting with ruptured oesophageal varices who has received 6 units of blood but has a prolonged prothrombin time?

A

Fresh frozen plasma to replace clotting factors

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81
Q

A day old baby who’s mother takes anticonvulsants is admitted with unexplained bruising. Clotting profile shows PT and APTT are prolonged. What is the likely diagnosis and what is the management?

A

Haemorrhagic disease of the newborn

Give Vit K to correct absence of Vit K dependent clotting factors

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82
Q

What is coombs test?

A

Direct and indirect
Direct used for autoimmune haemolytic anaemia
Indirect used for prenatal testing of pregnant woman and prior to transfusion

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83
Q

What are poor prognostic features of acute myeloid features?

A

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

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84
Q

What is Waldenstroms macroglobulinaemia?

A

Lymphoplasmacytic lymphoma
Affects B cells which produce excess immunoglobulin - monoclonal IgM paraproteinaemia
Bone marrow infiltration
Splenomegaly
Lymphadenopathy
Hyperviscosity of blood so risk of strokes/clots

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85
Q

Why does alcohol cause a large MCV?

A

Alcohol has effect on alkaline phosphatase on RBC membrane producing relative swelling of the cell

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86
Q

What condition is suggested by a prolonged APTT and thrombocytopenia with a background of recurrent DVT?

A

Antiphospholipid syndrome

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87
Q

Name some clotting disorders which make you pro thrombotic (thrombophilias)

A 
Factor V Leiden 
Prothrombin mutation
Antithrombin III deficiency 
Protein C deficiency 
Protein S deficiency 
Factor XIII mutation 
Familial dysfibrinogenaemia 
Antiphospholipid syndrome
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88
Q

What is hereditary spherocytosis?

A

Autosomal dominant abnormality of erythrocyte membrane proteins which interfere with cells ability to travel through capillaries
More prone to rupture, cells taken to spleen
Haemolytic anaemia
Howell Jolly bodies on blood smear

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89
Q

What is Virchows triad which describes factors that contribute to thrombosis?

A

Venous stasis
Endothelial damage
Blood hypercoagulability: hyperviscosity, deficiency of antithrombin III, nephrotic syndrome, disseminated malignancy, pregnancy, smoking

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90
Q

What should be done to manage a case of DIC?

A

Clotting studies and platelet count
Advice from haematologist
Up to 4 units of FFP and 10 units of cryoprecipitate whilst awaiting results

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91
Q

What is waldenstrons macroglobulinaemia?

A

Lymphoplasmocytic lymphoma
High levels of IgM
Preceded by IgM monoclonal gammopathy of undetermined significance
Weakness, fatigue, weight loss, chronic oozing of blood from the nose and gums

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92
Q

What is porphyria cutanea tarda?

A

Presents with skin manifestations later in life
Low levels of enzyme responsible for 5th step in heme production
Onycholysis and blistering of sun exposed skin

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93
Q

Which cytokine is involved in the reaction to a Mantoux test?

A

Interferon gamma secreted by t helper 1 cells which stimulate macrophage activity

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94
Q

In which condition are smudge cells seen on blood film?

A

Chronic lymphocytic leukaemia

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95
Q

What is the most common childhood malignancy?

A

Acute lymphoblastic leukaemia

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96
Q

Which cell lineage is most commonly affected in ALL?

A

B cell lineage

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97
Q

What are some causes of AML/ALL?

A

Ionising radiation
Chemicals
Viruses
Chromosomal abnormalities

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98
Q

What are clinical features of ALL?

A

Critically ill due to bone marrow failure: Anaemia, Infection, Haemorrhage
Signs of leucostasis
Bone/joint pain
Widespread lymphadenopathy
Mild to moderate hepatosplenomegaly + orchidomegaly
CNS involvement – meningism, facial nerve palsy

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99
Q

What investigations should be used for ALL and what might the results show?

A 
FBC – Hb, neutrophils and platelets low 
Total WCC high with blast cells on film 
Blood film – blast cells 
Bone marrow aspirate - >20% blast cells 
CXT/CT – mediastinal/abdominal lymphadenopathy
LP – CNS involvement
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100
Q

What is the management of ALL?

A

Resuscitation if septic / shock / haemorrhage
Supportive treatment
Chemotherapy: Aim to eliminate leukaemic cells and achieve complete haematological remission
4 continuous phases: Remission induction, Consolidation therapy, CNS prophylaxis, Maintenance therapy
Consideration of allogenic stem cell transplantation

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101
Q

What are poor prognostic factors in ALL?

A

Patient age
High leucocyte count
Cytogenetics
Long time to complete remission (>4 weeks)

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102
Q

What is the most common leukaemia in adults?

A

AML

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103
Q

What are associated conditions with AML?

A

Myelodysplasia, prior chemotherapy, radiation, chromosomal abnormalities (Down syndrome)

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104
Q

What are clinical features of AML?

A

Present critically ill due to bone marrow failure
Anaemia: Weakness, lethargy, breathlessness, palpitations
Infection: Chest, mouth, perianal, skin
Haemorrhage: Purpura, epistaxis, bleeding gums
Gum hypertropy / skin infiltration
Hepatosplenomegaly
Leucostasis: Hypoxia, confusion, CVA

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105
Q

What investigations should be done for AML?

A

Bloods: FBC – leukocytosis, anaemia, thrombocytopaenia
Blood film – presence of blast cells
Bone marrow aspirate - >20% blast cells
Differentiate from ALL with immunophenotyping or cytoplasmic myeloperoxidase (+ve in AML)

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106
Q

What is the management of AML?

A

Resuscitation – treat for neutropenic sepsis, haemorrhage
Fluids/allopurinol for tumour lysis syndrome
Chemotherapy: Aim to achieve complete haematological remission (<5% blast cells) + normalisation of peripheral blood count
2 phases: Remission induction – 2 cycles of combination chemo, Consolidation therapy – 2 cycles – reduce risk of relapse

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107
Q

What are poor prognostic factors for AML?

A

Advancing age >60 years
Specific cytogenetic abnormalities – ‘poor risk’ groups
Failure to achieve complete remission with 1st cycle

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108
Q

What is chronic lymphocytic leukaemia?

A

Progressive accumulation of mature appearing B lymphocytes in theperipheral blood, bone marrow, lymph nodes, spleen and liver

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109
Q

What are clinical features of chronic lymphocytic leukaemia?

A

Asymptomatic – incidental lymphocytosis on routine FBC
Advance disease associated with: Weight loss, fever, malaise, Lymphadenopathy, hepatosplenomegaly
Bone marrow failure due to infiltration: anaemia, neutropenia,
thrombocytopenia
Recurrent infections due to reduced antibody production: Hypogammaglobulinaemia

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110
Q

What investigations should be done for CLL? What might they show?

A

FBC – lymphocytosis > 5.0, Autoimmune haemolysis, Thrombocytopenia, anaemia, neutropenia
Blood film: Lymphocytosis, Presence of ‘smear’ / ‘smudge’ cells – damage to cells during preparation
Immunophenotyping
BM - >30% mature lymphocytes
Immunoglobulins – hypogammaglobulinaemia

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111
Q

What is the management of CLL?

A

Choice between observation, palliation or aim for complete remission
Management based on age, performance status, co-morbidities,
symptoms present and prognostic factors
Palliative approach treating symptomatology with minimal toxicity OR prolong disease-free survival
Palliative - alkylating agents

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112
Q

What is the prognosis of CLL?

A

Incurable disease

Early stage, asymptomatic patients – die of unrelated causes Advance stage - refractory disease and bone marrow failure

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113
Q

What are poor prognostic factors for CLL?

A

Male
Initial lymphocytosis >50 x 109/L
Lymphocyte levels doubling in <12 months
Specific cytogenetic abnormalities/ mutations
Poor response to therapy

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114
Q

What is CML?

A

Characterised by large granulocytes and presence of the Philadelphia chromosome

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115
Q

What are clinical features of CML?

A 
Accelerated phase characterised by blood count and organomegaly becoming refractory to therapy 
30% asymptomatic at diagnosis 
Lethargy, weight loss, sweats 
Splenomegaly, bruising/bleeding 
Occasional signs of leucostasis
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116
Q

What investigations should be done for CML and what might they show?

A

FBC: Raised WCC (often 100-300 x 109/L) – predominantly neutrophils, Anaemia, Platelets often unaffected or raised
Raised urate
BM: Hypercellularity – granulocyte hyperplasia, Chronic phase <10% blasts / accelerated >10% / crisis >20%
Cytogenetic analysis: Presence of Philadelphia chromosome

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117
Q

What is the management of CML?

A

Commence allopruinol
Consideration of leucapheresis in leucostasis
Tyrosine kinase inhibitor (Imatinib) – 1st line – turns off signalling cascade

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118
Q

What are the myeloproliferative disorders?

A

Polycythemia vera – excessive proliferation of the erythrocyte,
myeloid and megakaryocyte lineages
Essential thrombocythaemia – persistent thrombocytosis not due to any other cause
Primary myelofibrosis – neoplasm of early haematopoietic stem cell leading to marked hyperplasia of abnormal megakaryocytes and
monocytes stimulating marrow fibrosis

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119
Q

What are important features of management of polycythemia vera?

A

Venesection

Manage thrombosis risk

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120
Q

What are features of myeloma?

A

Usual presentation with bone pain (back) or pathological fracture
Weakness, fatigue, thirst, polyuria, oedema due to renal impairment
Acute hypercalcaemia
HyperCalcaemia, Renal impairment, Anaemia, Bone problems (CRAB)

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121
Q

What investigations should be done for myeloma and what might they show?

A

FBC – anaemia / U+Es – renal impairment
Serum protein electrophoresis – detect paraproteins
X-ray site of bone pain – pathological fracture / lytic lesions
BM aspirate – plasma cell infiltration

122
Q

What is the management of myeloma?

A

Not curable
Transient responses to chemotherapy
Pain control
Management of complications: Renal impairment, Hypercalcaemia, RTx for bone disease, Anaemia, Cord compression

123
Q

What are causes of DIC?

A

Sepsis/trauma/organ necrosis/malignancy/liver failure/transfusionin compatibility

124
Q

What factors can be used in a scoring system to help with diagnosis of DIC?

A

Plt count
D-dimer
Prolonged PT
Fibrinogen level

125
Q

What is the management of DIC?

A

Treat precipitating cause
Supportive therapy
Replacement therapy of platelets, cryoprecipitate, FFP

126
Q

What is thrombotic thrombocytopenic purpura?

A

Increased platelet aggregation and occlusion of arterioles and capillaries of the microcirculation due to antibodies that activate the immune system to inhibit the ADAMTS13 enzyme

127
Q

What are clinical features of TTP?

A 
Severe thrombocytopenia 
Neurological involvement 
Renal impairment 
Fever 
Haemolysis (micro-angiopathic haemolytic anaemia)
128
Q

What is the management of TTP?

A 
Haematological emergency
May require ITU level of care 
Plasmapheresis 
FFP for fluid replacement
Platelet transfusions can worsen condition therefore only give in major bleed
Steroids
129
Q

In which condition are Auer rods seen?

A

AML

130
Q

What are Howell jolly bodies? When are they seen?

A 
Blue black inclusions of red blood cells which represent residual nuclear fragments 
Seen in asplenic patients 
Haemolytic anaemia 
Leukaemia 
Thalassaemia
131
Q

What are pappenheimer bodies, when are they seen?

A

Violet staining granules in periphery of RBCs containing siderblastic bodies
Seen in sideroblastic anaemia, lead poisoning, thalassaemia, asplenic patients

132
Q

What is the pathophysiology behind beta thalassaemia major?

A

Not enough b chain produced
Alpha chains precipitate in erythroblasts and mature red cells
Ineffective erythropoiesis and haemolysis
Anaemia occurs at 3-6 months when switch from g to b chain production takes place
Marrow hyperplasia to compensate leads to thalassaemic facies: hair on end appearance of skull X-ray and thinning of cortex with increased risk of fractures

133
Q

Which atypical pneumonia can be associated with cold agglutinins and therefore Raynauds and autoimmune haemolytic anaemia?

A

Mycoplasma

134
Q

Which crises can occur with sickle cell anaemia?

A

Haemolytic crisis
Splenic sequestration crisis
Aplastic crisis

135
Q

To which bugs are patients with sickle cell susceptible?

A

Encapsulated bacteria due to hyposplenism: pneumococci, meningococci

136
Q

How long after a DVT are patients recommended not to fly for?

A

2 weeks

137
Q

What is the triad of symptoms in yellow nail syndrome?

A

Lymphoedema
Pleural effusions
Yellow discolouration of nails

138
Q

What are indications for red cell transfusion?

A

Acute blood loss: especially if >1.5 L in adult
Symptomatic anaemia with no easily treatable cause
Long term transfusion dependent anaemia
Radiotherapy patient Hb <100
Chemotherapy patient Hb <90

139
Q

What are potential complications from a blood transfusion?

A 
Acute haemolytic reaction
Anaphylaxis
Transfusion associated lung injury
Non haemolytic reaction
Allergic reaction
Fluid overload
140
Q

What is the name of the haemovigillance scheme that monitors adverse events in blood transfusion?

A

SHOT

141
Q

Which drugs should be co prescribed alongside a blood transfusion?

A

Hydrocortisone
Chlorpheniramine
Furosemide

142
Q

In which patients can giving vitamin K cause haemolysis?

A

G6PD deficiency

143
Q

What is octaplex used for?

A

Reversal of warfarin therapy or vit K deficiency

144
Q

What is octaplex?

A

Prothrombin complex concentrate

145
Q

After giving octaplex, when should you re check INR?

A

30 mins

146
Q

What causes transfusion related lung injury?

A

Donor Ab (esp HLA or neut) react with recipient WBCs leads to lung infiltrate
Occurs 1-6 hours post transfusion
Associated with FFP

147
Q

What are indications for FFP?

A

Coagulation factor deficiency where specific concentrates not available
Multiple clotting factor deficiency: liver disease, DIC, massive transfusion

148
Q

What is the greatest cause of transfusion death in the U.K.?

A

Transfusion associated circulatory overload

149
Q

What are causes of massive splenomegaly?

A 
Myelofibrosis
CML
Visceral leishmaniasis 
Malaria
Gauchers syndrome
150
Q

With which condition is the Philadelphia chromosome associated?

A

CML

151
Q

How does CML present?

A 
Middle age
Anaemia 
Weight loss
Abdo discomfort 
Splenomegaly 
Spectrum of myeloid cells in peripheral blood 
Decreased leukocyte alkaline phosphatase
152
Q

What is the management of CML?

A

Imatinib: tyrosine kinase inhibitor
Hydroxyurea
Interferon alpha
Allogenic bone marrow transplant

153
Q

What is the maximum time for transfusion of red cells?

A

3.5 hours

154
Q

When would you prescribe a platelet transfusion?

A

Prophylaxis if platelets <10
If septic, abnormality of coagulation <20
Bleeding/procedure/surgery <50
Brain/eye/bleeding surgery <100

155
Q

How does acute intermittent porphyria present?

A

Abdominal pain
Neuropathies
Constipation

156
Q

How can a diagnosis of acute intermittent porphyria be made?

A

Elevated level of porphobilinogen on a spot urine test during acute attack

157
Q

How is acute intermittent porphyria managed?

A

Mild: high doses of glucose
Severe: haematin

158
Q

What is acute intermittent porphyria?

A

Autosomal dominant disease resulting from defect in porphobilinogen deaminase so porphyrin precursors accumulate leading to neurological damage and psychiatric manifestations

159
Q

What is idiopathic thrombocytopenic purpura?

A

Immune mediated reduction in platelet count

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

160
Q

What is the most common type of Hodgkin’s lymphoma?

A

Nodular sclerosing

161
Q

Which factors are associated with a poor prognosis in Hodgkin’s lymphoma?

A 
Lymphocyte depleted type
B symptoms: weight loss, fever, night sweats
Age >45
Stage IV disease
Haemoglobin <10.5
Lymphocyte count <600
Male
Albumin <40
White cell count >15
162
Q

What are possible complications of CLL?

A

Hypogammaglobulinaemia leading to recurrent infections
Warm autoimmune haemolytic anaemia
Transformation to high grade lymphoma (richters transformation)

163
Q

What is beta thalassemia major?

A

Absent synthesis of beta haemoglobin chains due to mutation in HBB gene on chromosome 11, autosomal recessive
Inability to construct new beta chains leads to underproduction of HbA which leads to microcytic anaemia

164
Q

What is HbF and HbA2?

A

Different forms of haemoglobin which are present in beta thalassemia
HbF: alpha alpha gamma gamma
HbA2: alpha alpha delta delta

165
Q

What are the causes of DIC?

A 
Severe sepsis and septic shock
Malignancy
Obstetric disease: eclampsia, abruption, amniotic fluid embolism
Rheumatological disease
Severe liver disease
Transfusion reactions
166
Q

What does a schilling test assess? What is it?

A

Absorption of vitamin B12
Following overnight fast, body injected with non labelled B12. This is followed by oral labelled B12 with cobalt 58, and a second dose with cobalt 57 bound to intrinsic factor
Malabsorption if less than 10% labelled excreted in 24 hours
Pernicious anaemia if elevated ratio of Co-57 to Co58

167
Q

What is a typical finding of myelofibrosis on blood film?

A

Tear drop shaped cells

168
Q

What is used in mild Von willebrand disease if there is evidence of bleeding?

A

DDAVP (desmopressin)

169
Q

How is idiopathic thrombocytopenic purpura managed?

A

If symptomatic or platelet count below 20 then prednisolone

Splenectomy cures most cases

170
Q

How is a bleed on warfarin managed?

A

If significant active bleeding then octaplex (prothrombin complex concentrate)

171
Q

In which patients is pernicious anaemia most common?

A 
Fair haired
Elderly 
Women
Blue eyes 
Autoimmune conditions: thyroid disease and vitiligo
172
Q

How is pernicious anaemia managed?

A

IM vitamin B12/hydroxycobalamin

173
Q

What is the leading cause of death in ITP?

A

Intracranial haemorrhage

174
Q

What is the treatment choice for a patient with ITP and massive haemorrhage?

A

Platelet transfusion
IV methylprednisolone
IV immune globulin
Splenectomy

175
Q

In a patient presenting with an unprovoked DVT, other than commencing warfarin, what further actions are required?

A 
Chest X-ray
Bloods: FBC, calcium, LFTs
Urinalysis 
If all normal: abdo pelvis CT and mammogram for woman in all patients over 40
Check antiphospholipid antibodies
176
Q

What is haptoglobin?

A

Binds haemoglobin that has been released from erythrocytes

Can be used as measure of Haemolysis - levels will be decreased in intravascular

177
Q

What are provocative factors for a sickle cell crisis?

A 
Exercise
Infection
Fever
Anxiety
Hypoxia
Changes in temperature
178
Q

What is acute chest syndrome?

A

Vaso occlusive crisis of pulmonary vasculature seen in patients with sickle cell anaemia

179
Q

What are complications of sickle cell?

A 
Acute chest syndrome
Pulmonary infarction
Pneumonia 
Stroke
Renal papillary necrosis
Priapism
Aseptic necrosis of bones and joints
Digital infarction
Leg ulcers
180
Q

What can be given to induce release of Von willebrands factor from endothelial cells and therefore reduce bleed risk in theatre?

A

Desmopressin

181
Q

What is the most common inherited thrombophilia?

A

Activated protein c resistance (factor V Leiden)

182
Q

What are some inherited causes of hypercoagulable state?

A 
Factor V Leiden
Prothrombin gene mutation
Anti thrombin deficiency
Protein c and s deficiencies
Elevated homocysteine
Dysfibrinogenaemia
Sickle cell disease
183
Q

What are some causes of acquired hypercoagulable state?

A 
Antiphospholipid syndrome
Supplemental oestrogen use
Heparin induced thrombocytopenia and thrombosis (HITT)
Cancer
Obesity
Pregnancy
184
Q

What is the function of activated protein c?

A

Along with cofactor protein s it inactivates factor Va and VIIIa

185
Q

What is the mechanism of action of heparin?

A

Unfractioned - binds to antithrombin III to help inactivate thrombin
LMWH - factor Xa inhibitor

186
Q

What is waldenstroms macroglobulinaemia?

A

Lymphoplasmacytic lymphoma producing excess IgM
Presents with bone marrow infiltration, splenomegaly and lymphadenopathy, raynauds, visual disturbance due to hyperviscosity

187
Q

How is a diagnosis of hereditary spherocytosis made?

A

Osmotic fragility test

188
Q

What is the management of hereditary spherocytosis?

A

Folate replacement

Splenectomy

189
Q

Which factors are associated with increased bleeding risk when on anticoagulant drugs?

A 
Poor INR control
Hepatic or renal disease
EtOH abuse
Malignancy
Anaemia
Prior bleeding history
Age
Excessive fall risk
Prior history of stroke
Concomitant antiplatelets
190
Q

Which indication for anticoagaultion has a target INR higher than 2.5?

A

Recurrence of VTE while on warfarin

Mechanical prosthetic heart valve

191
Q

Why do warfarin and heparin need to overlap when treating acute venous thromboembolism with loading doses of warfarin?

A

Time delay in achieving an antithrombotic effect based on the elimination half-lives of the vitamin K dependent clotting factors
Protein C: Warfarin is initially prothrombotic due to decreasing protein c levels due to short half life

192
Q

What are the pharmacokinetics of warfarin?

A

Readily absorbed from the gastro-intestinal tract
Plasma half-life is about 40 hours
It is metabolised in the liver, and is excreted in the urine mainly as metabolites

193
Q

What are some non haemorrhagic adverse effects of warfarin?

A 
Rash
Alopecia
Nausea
Diarrhoea
Effects in utero: Congential abnormalities and foetal death if given to pregnant woman 6-14 weeks gestation
194
Q

Which foods and drinks does warfarin interact with?

A

EtOH: Binge drinking
Vitamin supplements: Vitamin K containing
Diet: Foods rich in vitamin K eg broccoli, brussels sprouts, cabbage, kale, lettuce, spinach, watercress, and other green leafy vegetables

195
Q

What colours are the different doses of warfarin?

A

0.5mg white
1mg brown
3mg blue
5mg pink

196
Q

What baseline tests need doing before commencing warfarin?

A

FBC
LFTs
INR

197
Q

Why is it important to ensure the blue top bottle is filled correctly for calculating an INR?

A

Underfilled citrate bottle will cause high INR result as it will cause high citrate levels
Citrate binds calcium. Calcium is important for initiating clotting

198
Q

What patient advice should be offered when starting warfarin?

A

Individual dose is adjusted to achieve desired level of anticoagulation
Dose made up of tablets of different strengths and colours
INR monitored regularly so must attend for checks
Monitor for bleeding and know what to do if it happens
Keep record safe and up to date
Avoid medicines containing aspirin
Don’t start or stop new drugs including OTC without checking with dr or pharmacist first - huge number of interactions
Avoid binge drinking
Tell anyone involved in treating them that they are on warfarin

199
Q

What does an oral anticoagulant patient pack contain?

A

Yellow Book (treatment record)
Patient information
Patient alert card

200
Q

When should INR be checked after starting warfarin?

A

Day 5, 8 and 12

201
Q

What are options to reverse warfarin?

A

Vitamin K: IV or PO

Prothrombin Complex Concentrate eg octaplex/beriplex: Factors II, IV, IX, X, protein c and s

202
Q

How long does oral and IV vitamin K take to work?

A

Oral: Takes 24 hours to work
IV: Takes 6-8 hours to work

203
Q

What are different levels of thrombotic risk assessed for patients on warfarin before surgery? What does this mean for peri operative bridging therapy?

A

High risk: recent history of VTE (<3 months), or recurrent VTE on increased target INR (3.5) warfarin and severe prothrombotic disorder eg known Antiphospholipid Syndrome or Antithrombin
Deficiency. Need therapeutic LMWH
Moderate risk: history of recurrent VTE within last 3-12 months. Need prophylactic LMWH
Low risk: majority of AF patients and patients with past history of single episode of VTE >12 months ago. No bridging required

204
Q

What monitoring is required when a patient is on unfractionated heparin?

A

Need for regular APTTr and FBC every 6 hours

205
Q

In which patients is routine monitoring required for LMWH? What monitoring is done?

A 
Pregnancy
Infancy <1 year old 
Renal failure
Morbid obesity
Active bleeding with no apparent cause 
Anti Xa levels
206
Q

What are the pharmacokinetics of heparin?

A

LMWH: Rapidly and completed absorbed via S/C injection. Peak activity 1-4 hours post dose. Renal elimination primarily (half life 4-5 hours) although small degree of hepatic metabolism
IV heparin: Elimination as above, Half life 30 mins – 2 hours

207
Q

What are non bleeding adverse effects of heparin?

A 
Allergic
HITT 
Local skin reaction
Hyperkalaemia
Osteoporosis
208
Q

What are some indications for therapeutic LMWH?

A

Treatment of VTE / peripheral vascular surgery
Treatment of unstable angina and non-Q-wave myocardial infarction
Treatment of acute ST-segment elevation myocardial infarction
Prevention of extracorporeal thrombus formation during haemodialysis

209
Q

When should a sample be taken to check for anti Xa levels?

A

4 hours post dose

2 tubes required

210
Q

What can be used to reverse heparin?

A

Protamine

211
Q

What are contraindications to use of DOACs?

A 
Heart valves
Renal failure GFR <15
Strong CYP inhibitors eg systemic azoles
HIV drugs 
PGP inhibitors: Amiodarone, clarithromycin, colchicine, omeprazole, nifedipine, sertraline, tamoxifen, verapamil
212
Q

When should DOACs be stopped before surgery?

A

Minor surgery 24 hours
Major 48 hours
Renal failure 72 hours

213
Q

What is the novel antidote for dabigatran? When can it be used?

A

Idarucizumab (Praxbind)
Emergency major surgery (not to be used in elective or non-emergency cases)
Life-threatening or uncontrolled bleeding

214
Q

You are asked to see a 49 year old woman with a mechanical mitral heart valve on long term warfarin in the pre-op assessment clinic prior to laparascopic cholecystectomy.
How would you manage her peri-operative anticoagulation?

A

Stop warfarin 5 days pre op
Give bridging LMWH from 3 days pre op
Last dose 24 hours before surgery

215
Q

What are features of HIIT syndrome?

A 
DVT
PE
MI
Occlusion of limb arteries (possibly resulting in amputation)
Cerebrovascular accidents (stroke,TIA)
Skin necrosis
End-organ damage (adrenal, bowel, spleen, gallbladder or hepatic infarction, renal failure)
Death
216
Q

24 year old woman with x2 previous DVTs on long term warfarin admitted c/o abdominal discomfort. Routine pregnancy test positive. What action needs taking?

A

Stop warfarin immediately and commence LMWH / liaise with obstetrics

217
Q

76 year man, known atrial fibrillation on warfarin. Attends routine cardiology clinic. INR noted to be high (6.5). No bleeding apparent. What treatment is required?

A

PO vitamin K

218
Q

78 year old man with inoperable lung carcinoma is.During admission develops a swollen, painful leg. Ultrasound confirms a popliteal DVT. How do you manage his initial anticoagulation?

A

Start LMWH monotherapy

219
Q

What goes wrong that leads to leukaemogenesis?

A 
Loss of cell-cycle control
Autonomous proliferation
Increased self-renewal
Differentiation block 
Resistance to apoptosis: increased or decreased
Dissemination
Loss of tumour suppressor genes
220
Q

What is the management of neutropenic sepsis?

A

Treat as neutropenic without waiting for FBC result
Blood cultures
Broad spectrum antibiotics within 60 minutes of presentation
IV fluid resuscitation
Get help

221
Q

What living conditions are important when undergoing chemo for AML?

A 
Filtered air
No plants or flowers
No unnecessary visitors
Washed food – no salad or grapes or blackpepper
Antifungal prophylaxis
Mouthcare
222
Q

What are indications for bone marrow biopsy?

A

Diagnostic: Abnormal FBC, Paraproteinaemia, Bone lesions in pelvis accessible by this route, Pyrexia of unknown origin, Isolated splenomegaly with diagnosis unclear from peripheral blood
Staging: Hodgkin Lymphoma / Non Hodgkin Lymphoma
Treatment response: Leukaemia, Myeloma, Lymphoma

223
Q

What investigations needs to be done for multiple myeloma?

A

Protein electrophoresis serum/urine
Bone marrow examination
Skeletal survey

224
Q

What causes rouleaux formation in blood film?

A

Plasma protein concentration high, raised ESR

Caused by: infections, myeloma, waldenstrons macroglobulinaemia, connective tissue disorders, cancer

225
Q

What causes renal failure in myeloma?

A 
Light chain deposition in kidney
Hypercalcaemia
Hyperuricaemia
Dehydration
Non-steroidal anti-inflammatories
Plasma cell infiltration of kidney
226
Q

How does the Philadelphia chromosome lead to CML?

A

Translocation causes constitutive activation of a tyrosine kinase at basis of the disease
Constant activation of cell turnover

227
Q

What is the management of CML?

A

Initial control of very high white cell count to prevent stroke
– aspirin
Drugs to control white cell count
Leucopheresis
Imatinib: inhibitor of the BCR-ABL tyrosine kinase inhibitor

228
Q

What levels of haematocrit (PCV) are high?

A

PCV>0.60 – men

PCV>0.56 – women

229
Q

Which mutation is found in association with primary polycythemia?

A

JAK2
Signals from EPO into cell
Red cell production in absence of growth factor when mutated

230
Q

What is management of primary polycythemia?

A

Reduce risk of cardiovascular events as result of increased abnormal red cell production:
Venesections
Aspirin
Drugs to suppress bone marrow function

231
Q

What are myeloproliferative disorders?

A

Clonal, pre-leukaemic
Uncontrolled proliferation of one or more bone marrow lineages:
Red cells – primary polycythaemia
Platelets – essential thrombocythaemia
White cells (myeloid) – chronic myeloid leukaemia
Fibroblasts - myelofibrosis

232
Q

What are malignant causes of hyperviscosity?

A

Paraprotein (IgM > IgA > IgG)
High WCC (CML / AML > CLL)
High red cell mass (polycythaemia)
Raised platelet count (>1,000, myeloproliferative rather than reactive)

233
Q

What are causes of splenomegaly?

A

Haematological: Chronic myeloid leukaemia, Myelofibrosis, Chronic lymphatic leukaemia, Acute lymphoblastic leukaemia, Lymphoma
Infective: EBV, Chronic malaria, Visceral Leishmaniasis
Liver: HCV / HBV with portal hypertension, Any cause cirrhosis with portal hypertension

234
Q

What are indications for lymph node biopsy?

A

Generalised lymphadenopathy, FBC unhelpful
Palpable cervical LN with mediastinal LN on CXR
Isolated lymphadenopathy – no obvious pathology in anatomical region drained, (ENT: nasendoscopy NAD, FNA unhelpful)
Regional lymphadenopathy with obvious primary pathology inaccessible to biopsy

235
Q

What are some possible findings from lymph node biopsy?

A 
Reactive
Necrotic 
Granulomatous – TB, Sarcoid?
HIV
Metastatic Carcinoma
Metastatic Melanoma
Lymphoma
236
Q

What are risks of chemo and radiotherapy?

A 
Breast cancer
Thyroid cancer
Secondary leukaemia / myelodysplasia
Infertility
Other endocrine failure - early menopause
Bones
Cardiac damage (chemo + radiotherapy)
237
Q

What are causes of anaemia?

A 
Physiological 
Impaired production: BM failure, Dyserythropoesis- eg thalassaemia, Aplasia, leukaemia, Anaemia of chronic disease 
Reduced haematinics (Fe, Folate, B12)
Increased breakdown (Haemolysis) 
Increased loss (Haemorrhage) 
Multifactorial
238
Q

What are the stages of development of iron deficiency anaemia?

A

Normal
Low iron stores: low ferritin
Pre-clinical iron deficeiency: low MCV
Clinical iron deficiency: low Hb

239
Q

What are causes of iron deficiency anaemia in children?

A

Dietary: especially infants and toddlers, Fussy eaters, High milk intake, High requirements/prematurity
Malabsorption
Increased loss: GIT, menstruation

240
Q

How should haemolytic anaemia be investigated?

A

Reticulocytes: increased
LDH: high levels
Haptoglobin: absent (intravascular haemolysis)
Direct Coombs Test (autoimmune haemolysis, Looking for immunoglobulins on red cells)
Blood Film for medical review
Thalassemia and haemoglobinopathy screen as appropriate
Further testing depending on results of initial tests

241
Q

How can haemolytic anaemia present?

A

Anaemia
Jaundice
Splenomegaly
Hyperbilirubinaemia

242
Q

What are some causes of haemolytic anaemia?

A

Intrinsic: Membrane defects: hereditary spherocytosis
Abnormal Hb: Sickle cell, Thalassaemia
Enzyme defect: G6PD
Extrinsic: Haemolytic Disease of newborn: Rh Dx and ABO incompatibility
Drug induced
HUS

243
Q

How can you prevent megaloblastic crisis in hereditary spherocytosis?

A

Folate supplementation

244
Q

What causes red cell aplasia in hereditary spherocytosis?

A

Parvovirus infection

245
Q

Which populations are mainly affected by sickle cell disease?

A

African, Middle Eastern, Asian

246
Q

What is sickle cell disease?

A 
Amino acid substitution in Beta-globulin chain of haemoglobin 
Autosomal Recessive 
Normal (adult) Hb AA 
Carrier trait AS 
Sickle cell Diagnosis: Hb SS, SC, SD
247
Q

How can sickle cell patients present?

A

Crisis: Pain- limbs, chest, abdomen, Aplastic, Sequestration

248
Q

What are complications of sickle cell disease?

A 
Anaemia Hb ~80 
Splenic infarction – hyposplenism 
Infection 
Poor growth 
Leg ulcers, stroke
249
Q

What is the management of sickle cell disease?

A

Prevention: antenatal diagnosis
Prophylaxis: folate, antibiotics, avoid cold and dehydration
Crisis: fluids, antibiotics, analgesia, exchange transfusion, Mobile auromated red cell exchange service
Longer term management: Hydroxycarbamide, Bone marrow transplantation
Experimental: gene therapy, anti P-selectin monoclonal antibody, Crizanlizumab

250
Q

What is thalassaemia?

A

Due to reduced globin chain synthesis
Normal: HbA (alpha 2 and beta 2) +small amounts of HbF and A2)
Beta-thalassaemia: Genes on Chromosome 11, Thalassemia major– HbF (alpha 2 and gamma 2) and Hb A2 (alpha 2 and delta 2) – Thalassemia intermedia, Thalassemia minor
Alpha-thalassemia: 2 alpha globin genes on each chromosome 16, Loss of 1 or 2 alpha genes: asymptomatic: alpha Thal Trait, Loss of 3 genes: HbH Disease, Loss of 4 genes: HbBarts Hydrops Foetalis

251
Q

What is the management of beta thalassaemia?

A 
Genetic counselling  
Regular blood transfusions 
Fe overload- complications: Liver, heart, pancreas, endocrinopathy
Fe chelation 
BM Transplantation
252
Q

What is the prognosis for acute leukaemia in children?

A

Prognosis >80% cured (age 2-10, female, WCC<50, no CNS involvement)

253
Q

What are causes of bruising in children?

A

Trauma: Accidental, non-accidental
Vascular: HSP, scurvy, ehlers danlos
Platelets: ITP, leukaemia, Von willebrand
Coagulation abnormality: haemorrhagic disease of newborn, haemophilia

254
Q

What is Von willebrand factor?

A

Secreted by endothelium/platelets
Carrier for FVIII:c
Facilitates platelet adhesion

255
Q

What tests and results would you expect in Von willebrand disease?

A

Prolonged bleeding time, PTT
Low FVII:C, vWF, ristocetin cofactor assay
vWF multimer analysis
DNA tests

256
Q

What are auer rods?

A

Material seen in cytoplasm of AML cells

Can also be seen in megaloblastic anaemia, leukaemia, iron deficiency anaemia hyposplenism - coeliac, thalassaemia

257
Q

What are Howell jolly bodies?

A 
Spherical blue black inclusions of red blood cells which represent residual nuclear fragments 
Commonly seen in asplenic patients 
Also severe haemolytic anaemia
Pernicious anaemia
Leukaemia
Thalassaemia
258
Q

What are pappenheimer bodies?

A 
Violet staining granules in periphery of RBCs 
Sideroblastic anaemia
Lead poisoning
Thalassaemia
Asplenic patients
259
Q

With which condition are smudge cells associated?

A

CLL

260
Q

How is a diagnosis of CLL confirmed?

A

Lymphocytosis
Characteristic lymphocyte morphology (smudge cells)
Immunophenotyping

261
Q

What are features of polycythemia rubra Vera?

A 
Hyperviscosity
Pruritis (typically after hot bath)
Splenomegaly
Haemorrhage (2ary to abnormal platelets)
Plethoric appearance
HTN
262
Q

What investigations should be done for polycythemia rubra Vera?

A 
FBC 
Blood film
JAK2 mutation
Serum ferritin
U and Es
LFTs 
If JAK2 negative: red cell mass, arterial O2 sats, abdo USS, serum EPO, bone marrow aspirate and trephine, cytogenetic analysis, erythroid burst forming unit culture
263
Q

What is the mechanism of action of DDAVP in Von willebrands disease?

A

Stimulates release of vWF from endothelial cells

Can increase levels long enough for surgery or dental procedures to be performed

264
Q

Who should be screened for thrombophilia?

A

Unprovoked DVT or PE with first degree relative also had DVT or PE

265
Q

What would you expect on a blood film in a patient with G6PD?

A

Heinz bodies

266
Q

What is the diagnostic test for hereditary spherocytosis?

A

Osmotic fragility test

267
Q

What is the inheritance pattern of G6PD deficiency?

A

X linked recessive

268
Q

What are presenting features of hereditary spherocytosis?

A 
Failure to thrive
Jaundice
Gallstones
Splenomegaly
Aplastic crisis precipitated by parvovirus 
MCHC elevated
269
Q

What is management of hereditary spherocytosis?

A

Folate replacement

Splenectomy

270
Q

What blood results would you expect to see with hypersplenism?

A

Pancytopenia

Raised reticulocytes

271
Q

What is paroxysmal nocturnal haemoglobinuria?

A

Acquired clonal defect of red cells resulting in production of erythrocytes with increased susceptibility to lysis by complement
Intermittent passage of bloody urine
Prothrombotic tendency caused by complement induced platelet aggregation

272
Q

What is protein S?

A

Vit K dependent anticoagulant which acts as non enzymatic co factor to activated protein c in proteolytic degradation of factor Va and VIIIa

273
Q

What is factor V Leiden?

A

Hypercoagulability disorder resulting from variant of factor V which cannot be inactivated by activated protein c

274
Q

What are features of fanconi anaemia?

A 
Autosomal recessive
Aplastic anaemia 
Increased risk of AML
Neurological problems
Short stature
Cafe au lait spots
275
Q

What are antibodies directed against in ITP?

A

Glycoprotein IIb/IIIa complex

276
Q

What are indications for splenectomy?

A

Traumatic rupture
ITP
Spherocytosis

277
Q

What prophylaxis do splenectomy patients need?

A

Vaccines 4 weeks before: pneumococcus, meningococcus, HIB

Lifelong Pen V

278
Q

What investigations need to be done for myeloma?

A

Serum electrophoresis, serum free light chains
Bone marrow examination
Skeletal survey

279
Q

What does vitamin K do?

A

Gamma carboxylation of factors II, VII, IX and X

280
Q

Where does vitamin K come from?

A

50% from food (leafy vegetables, eggs)

50% from bacteria in gut (why antibiotics affect warfarin control)

281
Q

Why don’t we use FFP in warfarin reversal any more?

A

High volume required in order to raise factor levels by small amounts

282
Q

Which is the only DOAC that can be reversed?

A

Dabigatran (can be dialysed and has a Mab antidote)

283
Q

What type of antibodies are anti A/B?

A

IgM (complement fix)

284
Q

What is a delayed transfusion reaction?

A

Later onset, due to minor antibodies in the transfused blood
Secondary immune response
Not ABO incompatibility

285
Q

What are important questions with regards to spine treatment for vertebral collapse in myeloma?

A

Have they had radiotherapy?

Have they had vertebroplasty?

286
Q

What are markers of red cell breakdown?

A

Bilirubin
Haptoglobin (absent)
LDH

287
Q

What are markers of increased red cell production?

A

Reticulocytosis

288
Q

How do you investigate a cause of Haemolysis?

A

Blood film

Direct Coombs test (look for antibodies)

289
Q

What are spherocytes?

A

Red cells more round as their membrane is lost

E.g. Autoimmune haemolytic anaemia, macrophages eat membrane

290
Q

What are different causes of haemolytic anaemia?

A

Congenital: G6PD deficiency, sickle cell, hereditary spherocytosis
Immune: autoimmune, alloimmune (ABO incompatibility)
Non immune: intravascular, DIC, HUS/TTP, mechanical valve, accelerated HTN

291
Q

What are causes of retinal haemorrhages?

A

Diabetes
HTN
Haematology: CML/AML hyperviscosity, paraproteinaemia IgM, polycythemia, raised platelet count

292
Q

What are causes of splenomegaly?

A

Haem: CML, CLL, ALL, lymphoma
Infective: EBV, chronic malaria, visceral leishmaniasis
Liver: HCV/HBV with portal HTN, cirrhosis
RA
SLE

293
Q

What is haptoglobin?

A

Binds free Hb with high affinity and so inhibits oxidative activity

294
Q

What is the definition of a massive transfusion?

A

Entire patients blood volume/10 units of blood in 24 hours

295
Q

What are early and late transfusion complications?

A

Early: acute haemolysis, bacterial contamination, allergic reaction, anaphylaxis, TRALI, fluid overload, non haemolytic febrile transfusion reaction
Late: iron overload, graft vs host disease, post transfusion purpura, infection

296
Q

What are complications of a massive transfusion?

A

Hypocalcaemia
Hypothermia
Hyperkalaemia
Dilutional effect on other blood components eg thrombocytopenia

297
Q

What are non megaloblastic causes of macrocytic anaemia?

A 
Alcohol
Liver disease
Hypothyroidism 
Pregnancy
Reticulocytosis
298
Q

What drug is given to treat B12 deficiency?

A

Intramuscular hydroxycobalamin (B12)

299
Q

What are causes of folate deficiency?

A

Poor diet: vegans
Increased demand: pregnancy
Malabsorption: coeliac disease
Anti folate drugs: methotrexate

300
Q

What might be examination findings in beta thalassaemia major?

A 
Hepatosplenomegaly 
Frontal bossing
Jaw enlargement 
Dental malocclusion
Flow murmur 
Generalised pallor
Conjunctival pallor
301
Q

What is the common name for beta thalassaemia major?

A

Cooleys anaemia

302
Q

What advice should be given to haemophiliacs?

A

Wear medic alert bracelet
Avoid NSAIDs and IM injections
Avoid contact sports and other activities

Phase 3 (23 decks)

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