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Phase 2A (HV) > Miscellaneous- Haem > Flashcards

Miscellaneous- Haem Flashcards

1
Q

What is TTP

A

A condition in which blood clots form in small blood vessels. It is characterised by microangiopathic haemolytic anaemia and thrombocytopenia purpura

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2
Q

What causes TTP

A

Lack or absence of ADAMTS-13 (protein that normally inactivates vwf)

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3
Q

Symptoms of TTP

A

Neuro signs
Fever
Purpura
Renal failure

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4
Q

Mode of inheritance of haemophilia a and b

A

X linked recessive

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5
Q

Mode of inheritance of G6PD

A

X linked recessive

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6
Q

Mode of inheritance of sickle cell anemia

A

Autosomal recessive

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7
Q

Mode of inheritance of thalssemia

A

Autosomal recessive

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8
Q

What clotting study is indicative of the external pathway

A

Prothrombin time

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9
Q

What would you expect the APTT, PT and platelets level to be in Bon willebrands disease

A

APTT- prolonged

Pt and platelets normal

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10
Q

What would you expect the APTT time to be in haemophilia

A

Prolonged

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11
Q

Sx of vwf disease

A

Epistaxis
Menorrhagia
GI bleeding
Easy bruising

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12
Q

What is the key Sx of hameophilia

A

Bleeding into the joints

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13
Q

What mutation is associated with polycythemia rubra Vera

A

JAK2

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14
Q

Moa of heparin

A

Activates antithrombin which then inhibits thrombin and factor 10a

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15
Q

What condition is poikilocytes associated with

A

Myelofibrosis

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16
Q

Name 3 complications of sickle cell anaemia

A

Osteomyelitis
Stroke
Aplastic anaemia
Poor growth

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17
Q

What aa change is seen in sickle cell disease

A

Valine replaces glutamic acid

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18
Q

What disease are Mediterranean people most likely to have

A

G6PD

Thallesemia

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19
Q

What antibody is associated with cold autoimmune haemolytic anemia

A

IgM

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20
Q

What antibody is associated with warm autoimmune haemolytic anemia

A

IgG

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21
Q

What histological feature is seen in G6PD

A

Heinz bodies and bite cells

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22
Q

What are the triggers for G6PD

A 
Fava beans 
Red wine 
Soy products 
Henna 
Drugs (NSAIDs, primaquine, notrofurantoin)
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23
Q

Where is iron absorbed

A

Duodenum/jejuni m

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24
Q

What disease causes itchiness after hot bath

A

Polycythemia rubra vera

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25
Q

Tx for Von willebrands disease

A

1st line- desmopressin

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26
Q

What virus can illicit an aplastic crisis in sickle cell

A

Parovirus B19

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27
Q

Good standard investigation for sickle cell anemia

A

Hb electrophoresis

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28
Q

Treatment for acute sickle crisis

A

Oxygen
Iv morphine
IV fluids
+/- penicillin

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29
Q

What is malaria

A

A disease caused by plasmodium Protozoa which is spread by the female anopheles mosquito

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30
Q

Which groups of people are protected from malaria

A

I was with sickle cell G6 PD deficiency an absence of Duffey antigens

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31
Q

Treatment for malaria

A

Quinolones/primaquine 

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32
Q

What investigation is required to make a diagnosis of malaria

A

Giemsa stained thick and thin blood films (3x required)

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33
Q

Risk factors for malaria

A

Travel to endemic area pregnancy immuno compromised lost immunity

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34
Q

What is the mode of inheritance of spherocytosis

A

Autosomal dominant

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35
Q

What is HIV

A

HIV is an RNA retrovirus

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36
Q

How can HIV be transmitted

A

Unprotected anal vagina and oral sex
Mother to child
Mucous membrane, blood are open and exposure to infected blood of bodily fluids e.g. sharing needles needlestick injuries et cetera

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37
Q

What are the two markers that are used to monitor HIV infections

A

CD4 count and high viral load

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38
Q

What is the treatment for HIV

A

High active antiretroviral therapy (HAART)

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39
Q

What is immune thrombocytopenia purpura (ITP)

A

An autoimmune condition in which the body produces antibodies against platelet receptor (GP 2B/3A)

40
Q

List 5 Sx of ITP

A 
Easy bruising 
Gum bleeding 
Purpura
Menorrhagia 
Epistaxis
41
Q

List 4 signs of IDA

A 
Angular stomatitis 
Atrophic glositis
Koilonchyia- spoon shaped nails 
Subconjunctival pallor
Brittle skin/nails
42
Q

What Protozoa can cause relapases of malaria

A

P vivax and p.ovale

43
Q

What criteria is required to charactise multiple myeloma

A

Excess plasma cells in bone marrow
Lyric bone lesions on X-ray
Monoclonal protein in serum/urine

44
Q

What chromosomal abnormalities is associated with multiple myeloma

A

[t(11:14)] and abnormality of chr 13q associated with tx resistance and poor prognosis

45
Q

What 2 RF are associated with AML

A

Down’s syndrome
Radiation
Myelodysplastic syndrome

46
Q

What is a complication of CLL

A

Richters syndrome

47
Q

List 3 treatments for AML

A

Chemotherapy
Blood and platelet transfusion
Bone marrow transplant

48
Q

List 3 treatments for CML

A

Imatinib
Hydroxyurea
Interferon alpha

49
Q

List 3 tx for ALL

A

Chemotherapy
Blood and platelet transfusion
Steroids
Stem cell transplantation

50
Q

List 3 tx for CLL

A

Chemotherapy
Rituximab
Bone marrow transplant
Radiation

51
Q

List 5 Sx and signs of ALL

A 
Headaches 
Meningism 
Bone pain
Splenomegaly 
Anaemia 
Vomiting
52
Q

List 5 Sx and signs of AML

A 
Anaemia 
Leukopenia
Thrombocytopenia
Gum hypertrophy 
Luekaemia skin cuts
53
Q

List 5 Sx and signs of CML

A 
Anaemia 
Leukopenia 
Thrombocytopenia 
Hepatosplenomegaly
Bleeding
Gout 
Bone pain 
Lymphadenopathy
54
Q

List 5 Sx and signs of CLL

A 
Often asymptomatic 
Weight loss
Night sweats 
Fever
Enlarged rubbery lymph nodes
55
Q

What is Hodgkin’s lymphoma

A

Haematological malignancy arising form mature B cells

56
Q

What staging is used for hodgkins and n-hondgkins lymphoma

A

Ann arbour

57
Q

What may you see in a blood smear of multiple myeloma

A

Roleuax formation

58
Q

What is the chemotherapy regimen for Hodgkin’s lymphoma

A

ABVD

59
Q

List 4 causes of normoblastic macrocyclic anaemia

A

Liver disease
Alcoholism
Hypothyroidism
Drugs- azathioprine

60
Q

1st line Ix for pernicious anaemia

A

Ab against intrinsic factor

61
Q

Tx for pernicious anaemia

A

Hydroxycobalmain injections

62
Q

What is pathophysiology behind G6PD deficiency

A

Low G6PD — low amounts of reduced gluthathione— therefore oxidative damage to RBC

63
Q

What is alpha thalassemia

A

A spectrum of diseases caused by non-functioning copies of 4alpha globin genes

64
Q

List the types of alpha thalassemia

A

1 defective gene- silent carrier
2 defective genes- alpha thalassemia minor
3 defective genes- haemoglobin H
4defective genes- hb BARTS HYDROPS FETALIs

65
Q

Name Sx of alpha thalassemia

A

Fatigue
Skeleton deformity
Hepatosplenomegaly
Sob

66
Q

Gold standard Ix for alpha thalassemia

A

Hb electrophoresis +/- genetic testing

67
Q

Tx for alpha thalassemia

A

Blood transfusions +/- iron chelating agents (deferroxamine)

68
Q

What is beta thalassemia

A

A spectrum of diseases caused by non functioning copies of 2 beta globin genes

69
Q

Sx of beta thalassemia

A

Chipmunk fancies (frontal bossing enlarged cheekbones)
Failure to thrive
Skull (hair on end appearance)

70
Q

What is the tx for hereditary spherocytosis

A

Splenectomy

+ immunisations and life long -penicillin prohyplaxis

71
Q

What is AML

A

Clonal expansion of myeloblasts in bone marrow

72
Q

What virus is strongly associated with Hodgkin’s lymphoma

A

EBV

73
Q

What disease often precedes myeloma

A

MGUS

74
Q

Tx for multiple myeloma

A

Chemotherapy (bortezomib, thalidomide)

+ bisphosphomates + analgesia

75
Q

Name 4 causes of 2ndry polycythemia

A

High altitude
COPD
OSA
excess EPO

76
Q

1st line tx for polycythemia

A

Venesection

+aspirin, hydroxycarbamide (reduce thrombus) and chemo (can be used)

77
Q

1st line tx for ITP

A

Oral prednisalone
Or IVIG

2nd line- splenectomy

78
Q

1st line tx for TTP

A

Plasma exchange and corticosteroids
2nd- immunosuppressive (rituximab)
3rd- splenectomy

79
Q

Electrolyte abnormalities in tumour lysis syndrome

A

Hyperkalaemia
Hyperphosphataemia
Hyperuricaemia
Hypocalcaemia

80
Q

Tx for tumour lysis syndrome

A

Oral/IV allopurinolol

81
Q

What is the triad associated with glandular fever/infectious mononucleosis

A

Sore throat
Pyrexia
Lymphadenopathy

82
Q

What advice/management for infectious mononucleosis

A

Drink plenty fluids
Avoid alcohol
Analgesia
Avoid contact sports for 4 weeks, to decrease risk of splenic rupture

83
Q

What tx is given for DIC

A

Cryoprecipitate

84
Q

Differential diagnosis of DVT

A

Cellulitis

85
Q

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

A

Because ferritin is an acute phase protein so will be raised in response to inflammation

86
Q

3 chronic complications of sickle cell disease

A

Renal impairment
Pulmonary hypertension
Joint damage

87
Q

3 acute complications of sickle cell

A

Vaso-occlusive crisis
Aplastic crisis
Stroke

88
Q

List 3 levels that willl be increased in polycythemia

A

High haematocrit
High red cell count
High hb conc

89
Q

Sx of polycythemia

A 
Itchiness after hot bath
Burning pain in hands and feet
Headaches
Dizziness
Fatigue
Visual disturbance 
Splenomegaly
90
Q

List 5 causes of IDA

A 
Blood loss
Low intake
Poor absorption 
Pregnancy 
Hook worms
91
Q

List 4 Ix you would do in myeloma

A

FBC, U&E
serum or urine electrophoresis- paraprotein spike (igG)
serum free light chain essay (bence jones protein)
Skeletal X-ray
Bone marrow aspirate and biopsy ** (>10% plasma cells)
CRP, LDH (high levels poor prognostic marker)

92
Q

What drug may you give someone to prevent painful sickle crises

A

Hydroxycarbamide

93
Q

What can precipitate sickling

A

Trauma
Stress
Cold
Exercise

94
Q

What can cause ITP

A

Viral infections

Malignancy

95
Q

What ab/immunoglobulin can be found in ITP

A

IgG Ab

96
Q

3 signs of anaemia

A

Bounding pulse
Tachycardia
Pale skin/pallor

97
Q

List 3 environmental causes of luekaemia

A

Radiation
Drugs
Chemicals- benzene compounds

Phase 2A (HV) (13 decks)

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