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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
Tx for Von willebrands disease
1st line- desmopressin
26
What virus can illicit an aplastic crisis in sickle cell
Parovirus B19
27
Good standard investigation for sickle cell anemia
Hb electrophoresis
28
Treatment for acute sickle crisis
Oxygen Iv morphine IV fluids +/- penicillin
29
What is malaria
A disease caused by plasmodium Protozoa which is spread by the female anopheles mosquito
30
Which groups of people are protected from malaria
I was with sickle cell G6 PD deficiency an absence of Duffey antigens
31
Treatment for malaria
Quinolones/primaquine 
32
What investigation is required to make a diagnosis of malaria
Giemsa stained thick and thin blood films (3x required)
33
Risk factors for malaria
Travel to endemic area pregnancy immuno compromised lost immunity
34
What is the mode of inheritance of spherocytosis
Autosomal dominant
35
What is HIV
HIV is an RNA retrovirus
36
How can HIV be transmitted
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
37
What are the two markers that are used to monitor HIV infections
CD4 count and high viral load
38
What is the treatment for HIV
High active antiretroviral therapy (HAART)
39
What is immune thrombocytopenia purpura (ITP)
An autoimmune condition in which the body produces antibodies against platelet receptor (GP 2B/3A)
40
List 5 Sx of ITP
``` Easy bruising Gum bleeding Purpura Menorrhagia Epistaxis ```
41
List 4 signs of IDA
``` Angular stomatitis Atrophic glositis Koilonchyia- spoon shaped nails Subconjunctival pallor Brittle skin/nails ```
42
What Protozoa can cause relapases of malaria
P vivax and p.ovale
43
What criteria is required to charactise multiple myeloma
Excess plasma cells in bone marrow Lyric bone lesions on X-ray Monoclonal protein in serum/urine
44
What chromosomal abnormalities is associated with multiple myeloma
[t(11:14)] and abnormality of chr 13q associated with tx resistance and poor prognosis
45
What 2 RF are associated with AML
Down’s syndrome Radiation Myelodysplastic syndrome
46
What is a complication of CLL
Richters syndrome
47
List 3 treatments for AML
Chemotherapy Blood and platelet transfusion Bone marrow transplant
48
List 3 treatments for CML
Imatinib Hydroxyurea Interferon alpha
49
List 3 tx for ALL
Chemotherapy Blood and platelet transfusion Steroids Stem cell transplantation
50
List 3 tx for CLL
Chemotherapy Rituximab Bone marrow transplant Radiation
51
List 5 Sx and signs of ALL
``` Headaches Meningism Bone pain Splenomegaly Anaemia Vomiting ```
52
List 5 Sx and signs of AML
``` Anaemia Leukopenia Thrombocytopenia Gum hypertrophy Luekaemia skin cuts ```
53
List 5 Sx and signs of CML
``` Anaemia Leukopenia Thrombocytopenia Hepatosplenomegaly Bleeding Gout Bone pain Lymphadenopathy ```
54
List 5 Sx and signs of CLL
``` Often asymptomatic Weight loss Night sweats Fever Enlarged rubbery lymph nodes ```
55
What is Hodgkin’s lymphoma
Haematological malignancy arising form mature B cells
56
What staging is used for hodgkins and n-hondgkins lymphoma
Ann arbour
57
What may you see in a blood smear of multiple myeloma
Roleuax formation
58
What is the chemotherapy regimen for Hodgkin’s lymphoma
ABVD
59
List 4 causes of normoblastic macrocyclic anaemia
Liver disease Alcoholism Hypothyroidism Drugs- azathioprine
60
1st line Ix for pernicious anaemia
Ab against intrinsic factor
61
Tx for pernicious anaemia
Hydroxycobalmain injections
62
What is pathophysiology behind G6PD deficiency
Low G6PD — low amounts of reduced gluthathione— therefore oxidative damage to RBC
63
What is alpha thalassemia
A spectrum of diseases caused by non-functioning copies of 4alpha globin genes
64
List the types of alpha thalassemia
1 defective gene- silent carrier 2 defective genes- alpha thalassemia minor 3 defective genes- haemoglobin H 4defective genes- hb BARTS HYDROPS FETALIs
65
Name Sx of alpha thalassemia
Fatigue Skeleton deformity Hepatosplenomegaly Sob
66
Gold standard Ix for alpha thalassemia
Hb electrophoresis +/- genetic testing
67
Tx for alpha thalassemia
Blood transfusions +/- iron chelating agents (deferroxamine)
68
What is beta thalassemia
A spectrum of diseases caused by non functioning copies of 2 beta globin genes
69
Sx of beta thalassemia
Chipmunk fancies (frontal bossing enlarged cheekbones) Failure to thrive Skull (hair on end appearance)
70
What is the tx for hereditary spherocytosis
Splenectomy | + immunisations and life long -penicillin prohyplaxis
71
What is AML
Clonal expansion of myeloblasts in bone marrow
72
What virus is strongly associated with Hodgkin’s lymphoma
EBV
73
What disease often precedes myeloma
MGUS
74
Tx for multiple myeloma
Chemotherapy (bortezomib, thalidomide) | + bisphosphomates + analgesia
75
Name 4 causes of 2ndry polycythemia
High altitude COPD OSA excess EPO
76
1st line tx for polycythemia
Venesection | +aspirin, hydroxycarbamide (reduce thrombus) and chemo (can be used)
77
1st line tx for ITP
Oral prednisalone Or IVIG 2nd line- splenectomy
78
1st line tx for TTP
Plasma exchange and corticosteroids 2nd- immunosuppressive (rituximab) 3rd- splenectomy
79
Electrolyte abnormalities in tumour lysis syndrome
Hyperkalaemia Hyperphosphataemia Hyperuricaemia Hypocalcaemia
80
Tx for tumour lysis syndrome
Oral/IV allopurinolol
81
What is the triad associated with glandular fever/infectious mononucleosis
Sore throat Pyrexia Lymphadenopathy
82
What advice/management for infectious mononucleosis
Drink plenty fluids Avoid alcohol Analgesia Avoid contact sports for 4 weeks, to decrease risk of splenic rupture
83
What tx is given for DIC
Cryoprecipitate
84
Differential diagnosis of DVT
Cellulitis
85
Why might measuring serum ferritin be inaccurate at looking at iron levels
Because ferritin is an acute phase protein so will be raised in response to inflammation
86
3 chronic complications of sickle cell disease
Renal impairment Pulmonary hypertension Joint damage
87
3 acute complications of sickle cell
Vaso-occlusive crisis Aplastic crisis Stroke
88
List 3 levels that willl be increased in polycythemia
High haematocrit High red cell count High hb conc
89
Sx of polycythemia
``` Itchiness after hot bath Burning pain in hands and feet Headaches Dizziness Fatigue Visual disturbance Splenomegaly ```
90
List 5 causes of IDA
``` Blood loss Low intake Poor absorption Pregnancy Hook worms ```
91
List 4 Ix you would do in myeloma
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
What drug may you give someone to prevent painful sickle crises
Hydroxycarbamide
93
What can precipitate sickling
Trauma Stress Cold Exercise
94
What can cause ITP
Viral infections | Malignancy
95
What ab/immunoglobulin can be found in ITP
IgG Ab
96
3 signs of anaemia
Bounding pulse Tachycardia Pale skin/pallor
97
List 3 environmental causes of luekaemia
Radiation Drugs Chemicals- benzene compounds

Phase 2A (HV) (13 decks)

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