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Haem Flashcards

1
Q

4 causes of microcytic anaemia

A 
FAST
• Fe-deficiency anaemia
• Anaemia of chronic disease
• Sideroblastic anaemia
• Thalassaemia
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2
Q

7 causes of normocytic anaemia

A
  • Acute blood loss
  • Anaemia of chronic disease
  • Bone marrow failure
  • Renal failure
  • Hypothyroidism
  • Haemolysis
  • Pregnancy
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3
Q

6 causes of macrocytic anaemia

A
  • Fetus (pregnancy)
  • Antifolates
  • hypoThyroidism
  • Reticulocytosis
  • B12/folate deficiency
  • Cirrhosis

FATRBC

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4
Q

5 signs of iron-deficiency anaemia

A
  • Koilonychia
  • Atrophic glossitis
  • Angular cheilosis
  • Post-cricoid webs
  • Brittle hair and nails
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5
Q

Blood film of iron-deficiency anaemia

A
  • Microcytic
  • Hypochromic
  • Anisocytosis (unequal in size)
  • Poikilocytosis (abnormal shapes)
  • Pencil cells
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6
Q

Causes of iron-deficiency anaemia

A

Bleeding until proven otherwise - menorrhagia in young women

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7
Q

Leading cause of gastrointestinal blood loss in developing countries?

A

Hookworm infestation (ancylostoma duodenale)

Faecally contaminated soil - skin - alveoli - coughed and swallowed - intestine

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8
Q

Why can pregnancy cause iron-deficiency anaemia?

A

Increased utilisation

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9
Q

Why can iron-deficiency anaemia present post gastric surgery?

A
  • Rapid transit

* Less acid which helps Fe absorption

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10
Q

Why can coeliac disease cause iron-deficiency anaemia?

A

Absence of villous surface in duodenum

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11
Q

Investigations of iron-deficiency anaemia if no obvious cause

A
  • OGD + colonoscopy
  • Urine dip
  • Coeliac investigations
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12
Q

Treatment of iron-deficiency anaemia

A
  • Oral iron
  • IV iron if severe - risk of anaphylaxis
  • Blood transfusion if severe infection or sepsis as iron doesn’t absorb well and may fuel sepsis
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13
Q

What happens in ACD and how does it affect iron?

A

Cytokines reduce EPO receptor production by kidneys - reduced response to EPO - reduced red cell production

Cytokines stimulate liver hepcidin production, decreasing iron absorption and causing iron accumulation in macrophages - iron levels are therefore low

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14
Q

Ferritin and transferrin in ACD

A

High ferritin (intracellular protein - macrophages deprive invading bacteria of Fe)

Low transferrin

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15
Q

Management of ACD

A
  1. Treat disease

2. Recombinant EPO

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16
Q

What is sideroblastic anaemia and how is it diagnosed?

A
  • Enough iron but inability to put it into haemoglobin
  • Ineffective erythropoeisis
  • Bone marrow produces ring sideroblasts, which is seen in the marrow but not on film
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17
Q

Sideroblastic anaemia causes and treatment

A
  • Chemotherapy
  • Alcohol excess
  • Lead excess
  • Anti-TB drugs

Remove the cause + pyridoxine (Vit B6 promotes RBC production)

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18
Q 
The following is a sign of what
• Hypersegmented polymorphs
• Leucopenia
• Macrocytosis
• Anaemia
• Thrombocytopaenia
A

Megaloblastic blood film (B12/folate deficiency, cytotoxic drugs)

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19
Q

Source of vitamin B12

A

Meat and dairy

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20
Q
  • Glossitis
  • Angular cheilosis
  • Irriability, depression
  • Paraesthesiae
  • Peripheral neuropathy (loss of vibration and proprioception first, absent ankle reflex, SCDSC)

Features of what condition

A

B12 deficiency

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21
Q

Causes, diagnosis and treatment of pernicious anaemia

A

Autoimmune atrophic gasritis - lack of IF

Parietal cell antibodies, IF antibodies, Schilling test (outdated)

IM hydroxycobalamin (B12)

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22
Q

Source of folate and ‘medical’ causes of folate deficiency

A

Green vegetables, nuts, yeast, liver

Alcohol, methotrexate, phenytoin, trimethoprim

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23
Q

Treatment of folate deficiency

A

Oral folic acid, but treat B12 first if cause of anaemia is not known

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24
Q

Hereditary spherocytosis deficiency, pathogen susceptibility, and diagnosis

A

Spectrin or ankyrin deficiency (membrane protein)

Susceptible to effect of parvovirus B19

Diagnosis
• Spherocytes
• Increased osmotic fragility
• Eosin-5’-maleimide flow cytometry

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25
Q

Cause and blood film in hereditary elliptocytosis

A

Cause: spectrin mutations

Elliptical erythrocytes

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26
Q

How can someone with hereditary elliptocytosis present?

A

Asymptomatic to hydrops fetalis (oedema in at least 2 compartments)

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27
Q

Inheritance and benefit of South East Asian Ovalocytosis?

A

Autosomal recessive

Malaria protection

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28
Q

Precipitator and blood film of G6PD deficiency

A

Precipitated by acute stressors, moth balls, broad beans, antimalarials, steroids, certain antibiotics

Bite cells, Heinz bodies (blue deposits)

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29
Q

Clinical features and treatment of pyruvate kinase deficiency

A
  • Haemolytic anaemia
  • Severe neonatal jaundice
  • Splenomegaly

Most don’t require treatment

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30
Q

Which globins make up HbA (95% of normal haemoglobin)?

A

2 alpha and 2 beta

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31
Q

Which globins produce HbA2 and HbF?

A

HbA2 - 2 alpha and 2 gamma

HbF - 2 alpha and 2 delta

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32
Q

How many alpha and beta globin genes do we have?

A

4 alpha - 2 from each parent (Chr16)

2 beta - 1 from each parents (Chr11)

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33
Q

Inheritance of mutation in SCD

A

Autosomal recessive

Single base mutation
• Glutamic acid to valine at codon 6 of beta chain
• HbA -> HbS

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34
Q

Haemoglobin type in:
sickle cell anaemia
trait

A

Sickle cell anaemia - HbSS

Sickle cell trait - HbAS (asymptomatic unless stressed)

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35
Q

Most common invasive infection in sickle cell disease patients

A

Strep pneumoniae sepsis

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36
Q

What can (non-typhoidal) salmonella infection lead to in sickle cell disease patients?

A

Osteomyelitis

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37
Q

At what age does SCD manifest

A

3-6 months, coincides with decreasing HbF

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38
Q

Blood film findings of SCD

A

Sickle cells and target cells

Howell-Jolly bodies => hyposplenism

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39
Q

Acute and chronic treatment of SCD

Treatment in SCD for the following:
• Acute painful crises
• Acute severe crises

  • Prophylaxis
  • Increase HbF
  • Further chronic treatment
  • Childhood monitoring
A

Acute
• Opioids for painful crises
• Exchange tranfusion in severe crises

Chronic
• Penicillin V, pneumax, HiB vaccine
• Folic acid and hydroxycarbamide increases HbF
• Regular exchange transfusions
• Carotid doppler monitoring in early childhood

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40
Q 
Patient has the following features
• Skull bossing, maxillary hypertrophy, hairs on end skull X-ray*
• Hepatosplenomegaly*
• Heart failure**
• Gonadal failure**

What is the condition and the changes in the following in this condition: mutations, Hb changes, EPO, and iron.

A 
Beta-thalassaemia
• Point mutations - decreased beta-chain synthesis, excess alpha-chains
• Increased HbA2 and HbF
• Increased EPO*
• Iron overload**
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41
Q

Type of beta-thalassaemia with splenomegaly, bony deformity, gallstones, but no failure to thrive or heart failure.

A

Intermedia

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42
Q

Diagnosis and treatment of beta-thalassaemia

A

Diagnosis: Hb electrophoresis (Guthrie test at birth)

Treatment - blood transfusions with desferrioxamine to stop iron overload, folic acid

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43
Q

What does severity of alpha-thalassamia depend on?

A

Number of the 4 alpha genes deleted

  • trait - 1-2 deleted, mild anaemia
  • HbH disease - 3 deleted, splenomegaly, anaemia
  • Hydrops foetalis - 4 deleted, incompatible with life
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44
Q

Name 2 autoimmune acquired haemolytic anaemias

A

Warm autoimmune haemolytic anaemia (WAIHA)

Cold agglutinin disease

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45
Q

Ig type and coombs test result in WAIHA and cold agglutinin disease

A

WAIHA - IgG, +ve Coombs test

Cold agglutinin disease - IgM or IgG, +ve Coombs test

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46
Q

WAIHA / Cold agglutinin intra or extravascular?

A

WAIHA - extravascular

Cold agglutinin - intravascular

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47
Q

Patient has syphilis and Donath-Landsteiner IgG seen sticking to RBCs.

Haemoglobin seen in urine. What is the condition?

A

Paroxysmal cold haemoglobinuria

Donath-Landsteiner IgG seen sticking to RBCs when cold, the complement-mediated haemolysis on rewarming.

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48
Q

Name 2 Coombs negative diseases

A

Paroxysmal nocturnal haemoglobinuria (PNH)

Microangiopathic haemolytic anaemia (MAHA)

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49
Q

Outline PNH

A
  • Loss of protective surface GPI markers on RBCs
  • Chronic complement-mediated intravascular haemolysis, especially at night
  • Morning haemglobinuria, thrombosis, Budd-Chiari syndrome (obstructed hepatic venous outflow)
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50
Q

Investigations for PNH

A
  • Altered GPI immunophenotype

* Ham’s test (in vitro acid-induced lysis)

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51
Q

Outline MAHA and its causes

A
  • RBC destruction after force through fibrin/platelet mesh in damaged vessels
  • Resultant schistocytes

HUS, TTP, pre-eclampsia, adenocarcinoma (release of procoagulant granules)

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52
Q

Outline the condition with antibodies against ADAMTS13

A

TTP
• Lead to long strands of vWF
• These cut up RBCs

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53
Q

Outline HUS and clinical features

A

E. coli Shiga toxin bind to endothelial cells

Clinical features
• Diarrhoea
• Renal failure
• Thrombocytopaenia

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54
Q

Why can abx lead to decreased vitamin K?

A

Vit K activated by bacteria - abx harm gut flora

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55
Q

Vitamin K dependent clotting factors

A

2, 7, 9, 10

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56
Q 
Where do these clotting cascade inhibitors act?
• TFPI
• Protein C
• Protein S
• Antithrombin III
A

TFPI - blocks VII to VIIa
Protein C - inactivates Va and VIIIa
Protein S - co-factor to Protein C
Antithrombin - Xa (and IXa/XIIa), thrombin

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57
Q

First factor of instrinsic pathway, test used to measure, and what therapy you should monitor with this

A

Factor XII

APTT

Heparin therapy

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58
Q

First factor of extrinsic pathway, test used to measure, and what therapy you should monitor with this

A

Factor VII

PT

Warfarin therapy (INR)

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59
Q

First factor of common pathway and test used to measure

A

Factor V

TT (thrombin time)

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60
Q

How does clopidogrel work?

A
  • Blocks ADP RECEPTOR

* Decreased platelet activation and fibrin cross-linking

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61
Q

Difference between acute and chronic ITP

A 
Acute
• Children 
• Preceding infection
• Lower plt
• Self-limiting
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62
Q

Haemophilia A factor deficiency, inheritance, presentation, diagnosis, management?

A 
Factor VIII deficiency
• X-linked recessive
• Presents early in life or prolonged bleeding after surgery/trauma (e.g. dental)
• High APTT
• Factor VIII concentrates
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63
Q

What should you avoid in haemophilia A (in terms of treatment)?

A

NSAIDs

IM injections

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64
Q

What is haemophilia B, inheritance, presentation, diagnosis, management?

A

Factor IX deficiency

The rest is like haemophilia A

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65
Q

What factor does VWD lead to a decrease in?

A

Factor VIII as vWF carries factor VIII in circulation

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66
Q

Clotting, factor change, antigen change, INR, platelets, and management of VWD

A
  • High APTT
  • Low Factor VIII
  • Low vWF Ag
  • Normal INR and platelets

Manage with desmopressin (increases VWF and Factor VIII), VWF and Factor VIII concentrates

67
Q

DIC platelets, fibrinogen, D-Dimer, PT, and treatment

A
  • Low platelets
  • Low fibrinogen
  • High FDP/D-Dimer
  • Long PT/INR

Treat the cause and give transfusions, heparin

68
Q

Why can warfarin be initially pro-coagulant?

A
  • Warfarin antagonises vit K
  • Vit K needed for synthesis of protein C/S (as well as the clotting factors) are normally anticoagulant
  • Therefore warfarin can stop the anticoagulant effect of vitamin K, creating a procoagulant state
69
Q

Most sensitive vitamin K-dependent clotting factor

A

Factor VII - first deficiency

70
Q

Treatment of vitamin K deficiency (and in acute haemorrhages)

A

IV vitamin K - FFP for acute haemorrhages

Prothrombin complex concentrate

71
Q

3 inherited causes of venous thrombosis

A
  • Antithrombin deficiency (highest risk)
  • Protein C deficiency
  • Protein S deficiency
72
Q

Most thrombogenic acquired condition

A

Obesity

73
Q

DVT prophylaxis

A
  • Daily subcutaneous LMWH

* TED stockings

74
Q

Initial treatment of DVT/PE

A
  • LMWH (treatment dose), then warfarin or DOAC

* LMWH stopped once INR 2-3 (due to warfarin being initially procoagulant)

75
Q

Long-term treatment time of VTE if:
• Known cause
• Recurrent VTE

A
  • Known cause - 3 months

* Recurrent VTE - lifelong

76
Q

How does heparin work?

A
  • Potentiates antithrombin III

* This inactivates thrombin, factor IX, X, XI

77
Q

What type of heparin is given in renal impairment?

A

IV unfractionated heparin

78
Q

Long-term side effect of heparin

A

Osteoporosis

79
Q

Why does haemoglobin drop in pregnancy?

A

Diluation anaemia

80
Q

What is HELLP syndrome and the management?

A

Haemolysis, elevated liver enzymes, low platelets

Supportive management, delivery of foetus

81
Q

Which Ig can cross placenta?

A

Only IgG

82
Q

In whom and how/when do you prevent anti-D formation?

A
  • RhD negative women
  • Give mother IM anti-D Ig if high risk of feto-maternal haemorrhage
  • Routine antenatal prophylaxis at 28 and 34 weeks
  • If sensitising event occurs e.g. abortion, miscarriage
  • At delivery if baby is RhD +ve
83
Q

In which leukaemia is testicular and thymic enlargement seen?

A

acute lymphoblastic leukaemia

84
Q

Which genetic disorder increases risk of acute leukaemia?

A

Down’s syndrome

85
Q

Which marker is seen in acute leukaemia

A

CD34 - blast cell marker

86
Q

When are auer rods seen?

A

AML

87
Q

Which marker is seen on flow cytometry in AML but not ALL?

A

MPO - myeloid cells

88
Q

What is the most common leukaemia in the west?

A

CLL

89
Q

General difference between CLL and small lymphocytic lymphoma (SLL)

A

Same disease process but slightly different presentations

CLL - bone marrow, SLL - lymph nodes

90
Q

Leukaemia which is mostly diagnosed on routine bloods, has symmetrical painless lymphadenopathy, and autoimmunity association

A

CLL

91
Q

What is Richter’s transformation

A

Transformation of CLL or SLL to more aggressive form of large cell LYMPHOMA

92
Q

Leukaemia with Philadelphia chromosome, massive splenomegaly, and high basophils

A

CML

93
Q

Binet staging for CML

A

A: < 3 groups of enlarged lymph nodes
B: 3+ groups of enlarged lymph nodes
C: anaemia or thrombocytopaenia

94
Q

Treatment for CML

A

Imatinib (BCR-ABL tyrosine kinase inhibitor) or dasatinib/nilotinib for resistance

95
Q

Which sex does Hodgkin’s lymphoma affect more and which virus is it associated with?

A

Males

EBV

96
Q

Clinical presentation of H. lymphoma

A
  • Asymmetrical painless lymphadenopathy
  • Fever >38
  • Drenching night sweats
  • Weight loss >10% in 6 months
  • Pain after alcohol
97
Q

Diagnosis of H. lymphoma

A
  • CT-PET
  • LN or BM biopsy - CD15 and CD30
  • Reed-Sternberg cell (‘owl eyed’) surrounded by fibrotic collagen bands
98
Q

Most common subtype of H. lymphoma

A

Nodular sclerosing

99
Q

Ann-Arbor staging of H. lymphoma

A

1 - one LN region (includes spleen)
2 - two or more LN regions on same side of diaphragm
3- two or more LN regions on opposite sides of the diaphragm
4 - extranodular sites

A - no constitutional symptoms
B - constitutional symptoms

100
Q

H. lymphoma combination chemo treatment

A

ABVD (adriamycin, bleomycin, vinblastine, dacarbazine)

101
Q

What is autologous stem cell transplant?

A
  • Own SCs harvested
  • High dose treatment to eradicate malignant cells at cost of bone marrow ablation
  • SCs reintroduced
102
Q

What is the most common lymphoma?

A

Non-Hodgkin’s (80%)

103
Q

How is the presentation of NH lymphoma different to H?

A

No pain after alcohol

104
Q

Translocation and gene overexpressed in Burkitt’s lymphoma (NH, B cell)?

A

t(8;14) translocation

c-myc oncogene

105
Q

Histology of Burkitt’s lymphoma

A

Starry sky appearance

106
Q

Histology of diffuse large B-cell (NH, B cell)

A

Sheets of large lymphoid cells

107
Q

Angular nuclei - hearts/crests in cells, are seen in which subtype of NH B cell lymphoma?

A

Mantle cell

108
Q

Cause of gastric and parotid MALT (NH, B cell) lymphoma

A

Chronic antigen stimulation
• H. pylori => gastric
• Sjogren’s => parotid lymphoma

109
Q

Treatment for B cell lyphomas

A

Rituximab (or remove antigenic stimulus in MALT)

110
Q

Alk-1 protein expression is seen in which lymphoma?

A

Anaplastic large cell lymphoma (T cell)

111
Q

Cause of adult T cell leukaemia/lymphoma and population greatly affected

A

HTLV-1 infection

Caribbean and Japanese

112
Q

Blood film of adult T cell leukaemia/lymphoma

A

Flower cells

113
Q

Disease associations with enteropathy-associated T cell lymphoma and cutaenous T cell lymphoma

A

EATL - coeliac disease

Cutaneous - mycosis fungoides (red rashes)

114
Q

Treatment for T cell lymphomas

A

Alemtuzumab (anti-CD52)

115
Q

What is multiple myeloma?

A

Neoplasia of plasma cells of bone marrow - production of monoclonal immunglobulin (IgG most common)

116
Q

Ethinicity with increased incidence of MM

A

Afro-caribbeans

117
Q

Clinical features of MM

A

C - high calcium (groans, moans, stones, thirst)
R - renal failure
A - anaemia
B - pain, osteoporosis, osteolytic lesions, hyperviscosity syndrome

myeloma cells release osteoclast activating / osteoblast inhibiting factors

Fatigue - must be MM over MGUS or smouldering MM

118
Q

MM diagnosis

A
  • Dense narrow band on serum electrophoresis (in gamma region)
  • Rouleaux on blood film
  • Bence-Jones protein in urine
  • High ESR
  • > 10% plasma cells in BM
119
Q

Treatment for MM

A

Supportive for CRAB symptoms

Induce remission for autologous SCT:

  1. Bortezomib (proteosome inhibitor), dexamethasone (steroids cytotoxic to lymphoma)
  2. auto-SCT or daratumumab
120
Q

Amount of M-spike in MGUS, smouldering myeloma and MM

A

MGUS + smouldering myeloma <30g/l

MM >30g/l

121
Q

Clonal plasma cells in MGUS, smouldering myeloma and MM

A

MGUS <10%
Smouldering myeloma >10%
MM - any clonal plasma cell population

122
Q

Is treatment needed in MGUS, smouldering myeloma and MM?

A

MGUS and smouldering MM: no treatment needed

MM: treatment needed

123
Q

In whom does Waldenstrom’s macroglobinaemia (lymphoplasmacytoid lymphoma - LPL) more commonly present?

A

Elderly men

124
Q

What is LPL?

A
  • Low-grade NHL (lymphoplasmacytic)

* Lymphoplasmacytoid cells produce monoclonal serum IgM that infiltrates the LNs/BM

125
Q

Lymphoma with hyperviscosity syndrome (visual problems, confusion etc.) and is treated with chlorambucil?

A

LPL (Lymphoplasmacytic) - Waldenström’s macroglobulinaemia is the most common one

126
Q

What causes AL amyloidosis and which ratio is abnormal

A

Mis-folded light chains deposit in tissues

Abnormal kappa:lambda light chain ratio (although increased lambda, and decreased ratio = MM)

127
Q

Diagnosis of AA amyloidosis

A
  • Biopsy of affected organ
  • Congo-red stain
  • Apple green birefringence
128
Q

What is myelodysplastic syndrome and how is it different to aplastic anaemia?

A

Differentiation of abnormally maturing myeloid stem cells (give rise to RBCs, neutrophils, platelets, macrophages, basophils etc.) and ineffective proliferation.

Aplastic anaemia is a quantitative deficiency rather than functional. The bone marrow stops producing new cells, rather than producing abnormal cells.

129
Q

What does myelodysplastic syndrome risk transforming into?

A

Acute myeloid leukaemia (AML)

130
Q

What does the bone marrow look like in myelodysplastic syndrome and describe the RBCs, WBCs and platelets?

A

Hypercellular BM

  • RBCs - ring sideroblasts
  • WBCs - hypogranulation, Pseudo-Pelger-huet anomaly (hyposegmented neutrophil)
  • Platelets - micromegakaryocytes, hypolobated nuclei
131
Q

What does a ring sideroblasts look like and what causes it?

A
  • Increased ferritin as ineffective erythropoiesis

* Nucleated erythroblast with granules of iron in the mitochondria surrounding the nucleus

132
Q

What are hypomethylating agents useful for treating?

A

Myelodysplastic syndromes and AML

133
Q

Scoring system for prognosis of myelodysplastic syndrome

A

International prognostic scoring system
• BM blast %
• Karyotype - degree of cytopenia

134
Q

What are the classifications of aplastic anaemia?

A

Primary
• Idiopathic (70%)
• Inherited (10%)

Secondary (10-15%) - malignancy, radiation, drugs (e.g. chloramphenicol), chemo, viruses, autoimmune

135
Q

Which inherited aplastic anaemias are responsible for these presentations?

1.
• pancytopaenia
• skeletal abnormalities, short stature, cafe-au-lait spots, hypogonadism, microcephaly, developmental delay

2.
• Chromosome instability - telomere shortening
• Skin pigmentation, nail dystrophy, oral leukoplakia

3.
• Primary neutrophilia
• Skeletal abnormalities, endocrine and pancreatic dysfunction, hepatic impairment, short stature

4.
• Red-cell aplasia
• Dysmphorphology

A
  1. Fanconi anaemia
  2. Dyskeratosis Congenita
  3. Schwachman-Diamond syndrome
  4. Diamond-Blackfan syndrome
136
Q

What can myeloproliferative disorders all progress to?

A

Myelofibrosis

137
Q

Name a philadelphia chromosome positive myeloproliferative disroder

A

CML

138
Q

Name 3 ph (philadelphia chromosome)-negative myeloproliferative disorders

A
  • Polycythaemia vera
  • Myelofibrosis
  • Essential thrombocytosis
139
Q

What mutations are ph -ve mp disorders associated with?

A

JAK2 mutations

140
Q

What are the primary and secondary causes of polycythaemia?

A

Primary (abnormality in BM)
• Polycythaemia vera
• Familial polycythaemia

Secondary (EPO)
• Diseases states (renal cancer)
• High altitude
• Chronic hypoxia

141
Q

What is relative (pseudo) polycythaemia and what can cause it?

A
  • Red cell mass normal but plasma volume reduced (normal Hb)

* Dehydration, burns, vomiting, diarrhoea, smoking

142
Q

What causes polycythaemia rubra vera?

A
  • Erythroid precursors dominate the BM

* Point mutation JAK2

143
Q

Clinical features of polycythaemia rubra vera

A
  • Blurred vision
  • Headache
  • Plethoric, red nose
  • Gout
  • Splenomegaly
  • Aquagenic pruritis
144
Q

Treatment for polycythaemia rubra vera

A
  • Venesection - younger patients
  • Hydroxycarbamide - maintenance
  • Aspirin - decrease thrombosis risk
145
Q

Clinical features of myelofibrosis

A
  • Pancytopaenia-related
  • Hepatomegaly
  • Massive splenomegaly
  • Fever
  • Budd-Chiari syndrome
146
Q

Patient has dry tap from bone marrow. What would you expect to see on blood film and what mutations would you find?

A

Blood film - tear-drop poikilocytes (dacrocyte), leukoerythroblasts (primitive cells)
JAK2 mutation, MPL mutation

(myelofibrosis)

147
Q

Treatment for myelofibrosis

A

Supportive - blood producs, splenectomy
Cytoreductive - ruloxitinib (JAKi)
Curative - SCT

148
Q

What are 50% of essential thrombocythaemias associated with?

A

JAK2 mutations

149
Q

Which cell type dominates the BM in essential thrombocythaemia?

A

Megakaryocytes

150
Q

Clinical features in thrombocythaemia?

A

Often incidental finding
Venous and arterial thrombosis
Erythromelalgia (redness and burning in feet, hands, ears)

151
Q

Investigation findings in essential thrombocythaemia

A

aka thrombocytosis, so:
• Platelet count >600
• Blood film - large platelets and megakaryocyte fragments

152
Q

Treatment for essential thrombocythaemia

A
  • Aspirin
  • Anagrelide (reduces platelet formation)
  • Hydroxycarbamide (chemo)
  • Interferon (<40yo)
153
Q

What is the red cell transfusion threshold if asymptomatic and symptomatic?

A

Asymptomatic - 70g/l

Symptomatic - 80g/l

154
Q

Do you tranfuse red cells first or treat iron/folate/B12 deficiency?

A

Treat deficiency first unless active bleeding

155
Q

When should platelets be transfused?

A
  • Consumptive disorders e.g. DIC, TTP

* Only if active bleeding

156
Q

What is the universal blood type when transfusing red cells?

A

O- (red cells can’t be destroyed by anti-A or anti-B antibodies)

157
Q

What is the universal blood type when transfusing FFP?

A

AB (no anti-A or anti-B antibodies in plasma)

158
Q

How long does FFP need to thaw?

A

30 mins

159
Q

Which acute (<24 hours) transfusion reactions present with the following:

  1. Pulmonary oedema
  2. IgM-mediated intravascular haemolysis: restlessness, chest/loin pain, vomiting, collapse
A
  1. Transfusion-related circulatory overload

2. ABO incompatibility

160
Q

What are potential delayed (>24 hours) reactions to transfusions?

A

Delayed-haemolytic transfusion reaction
• Within 1 week
• Extravascular haemolysis - IgG-mediated

GVHD
• Donor lymphocytes recognise recipient’s HLA as foreign
• Diarrhoea, liver failure, skin desquamation, BM failure

Delayed infections
• CMV
• Parvovirus
• Variant CJD

161
Q

Outline haemolytic disease of the newborn

A

1) RhD sensitising event
2) Maternal anti-D crosses placenta (IgG) and coats fetal RhD +ve cells
• Cells destroyed in fetal spleen and liver

162
Q

How is HDN prevented?

A
  • Anti-D Ig given to mother if she is RhD negative (so has anti-RhD antibodies) and foetus is RhD positive
  • This destroys the fetal cells in the mother, bypassing the mother’s immune response
  • Given at delivery, foeto-maternal haemorrhage, spontaenous miscarriage, during abdo trauma or ECV
163
Q

Most common cytogenic abnormality in myeloma

A

Hyperdiploid karyotype

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