clinical Flashcards
1
Q
haemophilia signs and symptoms
A
Haemarthrosis muscle haematoma CNS bleeding retroperitoneal bleeding post-surgical bleeding
2
Q
haemophilia investigations
A
Clinical prolonged APTT normal PT reduced FV 8/9 genetic analysis
3
Q
haemophilia treatment
A
Replacement 8/9 DDAVP tranexamic acid gene therapy splints, PT, synovectomy, joint replacement, analgesia
4
Q
von Willebrand disease
A
Platelet type mucosal bleeding; epistaxis, purpura, menorrhagia, GI
5
Q
Tx for Von willebrand disease
A
VWF or DDAVP, tranexamic acid, OCP
6
Q
signs and symptoms of thrombocytopenia
A
Petechia, ecchymosis, mucosal bleeding, CNS bleeds
7
Q
investigations for liver failure thrombocytopenia
A
Liver failure -> reduced factors, prolonged PT, APTT, reduced fibrinogen, Vit K depletion
8
Q
Immune thrombocytopenia Tx
A
steroids, IgG, splenectomy, thrombopoietin analogues
9
Q
liver failure thrombocytopenia Tx
A
factor replacement, replacement of fresh frozen plasma, Vit K
10
Q
haemorrhagic disease of the newborn tx
A
Vit K
11
Q
haemolytic disease of the newborn sign
A
anaemia and jaundice
12
Q
Tx for haemolytic disease of the newborn
A
Prophylactic anti-D 28/40 and sensitising events
13
Q
type 1 hypersensitivity signs and symptoms
A
Anaphylaxis angioedema urticaria rhinitis asthma dermatitis, eczema
14
Q
type 1 hypersensitivity Tx
A
Beta2- adrenergic agonists, epinephrine, anti-histamines, corticosteroids
15
Q
type 2 hypersensitivity signs
A
Takes several hours, haemolysis
16
Q
type 2 hypersensitivity signs
A
Renal failure, glomerulonephritis, nephrotic syndrome
17
Q
type 4 hypersensitivity signs
A
2-3 days, delayed
18
Q
type 4 hypersensitivity treatment
A
NSAIDs, corticosteroids, drugs that block TNF, IL-6, antibodies against B cells, immunosuppressive drugs
19
Q
primary immunodeficiency signs and symptoms
A
SCID
Unusual rashes, diarrhoea, early stages of life, FH of neonatal death, consanguinity
20
Q
investigations for primary immunodeficiency
A
Low lymphocyte count, flow cytometry
21
Q
antibody deficiency signs and symptoms
A
later in life chronic/ recurrent bacterial respiratory infections
22
Q
antibody deficiency Dx
A
antibody levels; IgG, IgM, IgA, specific antibodies, complement and neutrophil function
23
Q
neutropenic sepsis symptoms and signs
A
> 38.5C, rigors, chest infection, skin sepsis, UTI, septic shock
24
Q
initial investigations for neutropenic sepsis
A
Blood cultures, urine output, serum lactate
25
further Dx for neutropenic sepsis
CXR, throat swab, sputum, FBC, renal, liver function, coagulation screen, CT if fungal suspected
26
neutropenic sepsis prophylaxis
ciprofloxacin, fluconazole, aciclovir, co-trimoxazole, G-CSF, stem cell transplant, laminar flow rooms, I.V. Ab, vaccine
27
neutropenic sepsis Tx
oxygen, antibiotics (Tazocin, gentimicin, vancomycin, teicoplanin maybe caspofungin) , I.V fluids
28
Acute myeloblastic Leukaemia symptoms
Bone marrow failure, rapidly fatal, anaemia, thrombocytopenic bleeding, infection
29
Acute myeloblastic Leukaemia Dx
Blood count and film, bone marrow aspirate, cytogenic, immunophenotyping,
CSF, targeted molecular genetics
30
Acute myeloblastic Leukaemia Tx
Daunorubicin, cytosine arabinoside, CPX-351, allogeneic stem cell transplant, arsenic trioxide, all-trans retinoic acid, midostaurin tyrosine kinase inhibitor targeted treatment
31
Chronic myeloid leukaemia symptoms and signs
Anaemia, splenomegaly, weight loss, hyperleukostasis , gout
hypercellular bone marrow, anaemia, high platelets, high WCC, basophils and cellular differentiation, t(9;22)
32
Chronic myeloid leukaemia Dx
FBC, blood film, bone marrow biopsy
33
Chronic myeloid leukaemia Tx
Tyrosine kinase inhibitors imatinib, dasatinib, direct inhibitors BCR-ABL 1st line, allogeneic transplantation in TKI failure
34
hyperleukostasis causes
(fundal haemorrhage, venous congestion, altered consciousness, respiratory failure)
35
Polycythaemia Vera symptoms
Headaches, itches, vascular occlusion, thrombosis, TIA, stroke, splenomegaly
36
Polycythaemia Vera Dx
FBC, haematocrit, uric acid, blood film
37
Polycythaemia Vera Tx
Venesection, aspirin, hydroxycarbamide, ruxolitinib
38
polycythaemia vera labs
raised haematocrits and Hg, raised WCC, platelets, raised uric acid, raised red cell mass
39
Essential thrombocythemia signs and symptoms
Raised platelet count
arterial and venous thrombosis, digital ischaemia, gout, headache
mild splenomegaly
40
essential thrombocytopenia Tx
Aspirin, hydroxycarbamide, anagrelide
41
Essential thrombocythemia
| Acute lymphoblastic leukaemia symptoms and signs
Rapid undifferentiated cells accumulating
short history of bone, joint pain and bone marrow failure, infections, sweats
42
Essential thrombocythemia
| Acute lymphoblastic leukaemia Dx
Bone marrow biopsy, FBC
43
Essential thrombocythemia
| Acute lymphoblastic leukaemia Tx
Chemo, CNS and consolidation therapy, high risk then stem cell transplant, bi specific T cell engagers (Blinatumumab), CAR-T cells
44
Chronic lymphocytic leukaemia symptoms
asymptomatic, bone marrow failure (anaemia, thrombocytopenia), lymphadenopathy, fever, sweats, maybe hepatomegaly, infections, weight loss
45
essential thrombocytopenia labs
low Hg, high WCC, low platelets
46
chronic lymphocytic leukaemia signs
Slow growing, low grade differentiated cells,
| raised lymphocyte count of 5
47
chronic lymphocytic leukaemia Tx
Watch and wait
cytotoxic chemo fludarabine, bendamustine
monoclonal antibody rituximab,
Tyrosine kinase inhibitors ibrutinib, PI3K inhibitor, BCL2 inhibitor
48
lymphoma symptoms
Lymphadenopathy, hepatosplenomegaly, extra-nodal disease, bone marrow involvement, fever, night sweats, weight loss, IgM paraproteins
49
staging for lymphoma
biopsy lymph node, CT, bone marrow aspirate, trephine biopsy
50
Tx for lymphoma
Chemo, anti CD20 monoclonal antibodies,
51
Hodgkin lymphoma investigation for responsiveness
PET
52
Tx for Hodgkin lymphoma
Chemo, RT, monoclonal antibodies anti-CD30, immunotherapy checkpoint inhibitors
53
myeloma symptoms
IgG, IgA paraproteins, bone disease (lytic lesions, pathological fractures, cord compression, hypercalcaemia), bone marrow failure (Anaemia), infections, renal failure, hyper viscosity, amyloidosis
54
Dx myeloma
CXR, MRI, biopsy of bone marrow with presence of excess plasma cells
55
staging for myeloma
albumin and beta 2 microglobulin
56
chemo for myeloma
proteasome inhibitors carfilzomib, bortezomib
57
IMIDS for myeloma
lenalidomide, pomalidomide
58
Tx for myeloma
monoclonal Ab,
bisphosphonate therapy, RT, steroids, pinning of long bones, decompression of the spinal cord, autologous stem cell transplant
59
arterial thrombus Tx
Lifestyle modification, treatment of vascular risk factors, antiplatelet, anticoagulant drugs, thrombolytic therapy
60
venous thrombus Tx
Prevent clot extension, prevent clot embolization, prevent clot recurrence in long term treatment.
anticoagulants (warfarin, DOAC’s, LMWH)
thrombolysis in massive PE
61
venous thrombus Dx
Wells and Geneva score, D-dimer, Doppler, V/Q scan, CT pulmonary angiogram
62
microvascular thrombus signs
DIC, diffuse ischaemia, gangrene, organ failure, bleeding
63
hereditary spherocytosis symptoms
Anaemia, jaundice neonatal, splenomegaly, pigment gallstones
64
hereditary spherocytosis Dx
FBC and film
65
hereditary spherocytosis Tx
Folic acid, transfusion, splenectomy
66
G6PD deficiency symptoms and signs
Blister and bite cells, neonatal jaundice, drug induced anaemia (intravascular haemolysis, haemoglobinuria), splenomegaly, pigment gallstones
67
pyruvate kinase deficiency symptoms and signs
Anaemia, jaundice, gallstones
68
thalassaemia beta major signs and symptoms
Beta major: expansion of ineffective bone marrow, bony deformities, splenomegaly, growth retardation
69
thalassaemia beta major Tx
Chronic transfusions, chelation therapy with desferrioxamine, bone marrow transplant
70
sickle cell anaemia symptoms and signs
Haemolysis, cation loss, dehydration, vaso-occlusion
painful bone crisis, stroke, infection, hyposplenism, gallstones, aplastic crisis, sequestration crises
71
chest crisis investigation for sickle cell anaemia
Chest crisis-> spirometry , CXR
72
painful crisis sickle cell anaemia tx
hydration, oxygen, antibiotics
73
chest crisis sickle cell anaemia Tx
respiratory support, IV fluids, antibiotics, transfusion
74
long term sickle cell anaemia Tx
vaccination, penicillin, folic acid, hydroxycarbamide, bone marrow transplant, gene therapy
75
anaemia symptoms
Tiredness, breathlessness, pallor, ankle swelling, dizziness, chest pain potentially jaundice, splenomegaly, lymphadenopathy, weight loss, diarrhoea, bleeding
76
hypochromic microcytic anaemia follow up
ferritin
77
normochromic normocytic anaemia follow up
reticulocyte
78
macrocytic anaemia follow up
B12/folate/bone marrow
79
haemolytic anaemia appearance on a blood film
schistocytes
80
haemolytic anaemia Dx
Direct antibody test
immune mediated then; warm, cold or allo
blood film, FBC, serum bilirubin, LDH, serum haptoglobin, urine
81
Tx for haemolytic anaemia
Folic acid, immunosuppression, steroids, splenectomy, treat underlying cause, consider transfusion
82
megaloblastic anaemia symptoms and signs
Neurological symptoms, yellow tinge, LDH, friable red cells
83
megaloblastic anaemia Dx
Autoantibodies against intrinsic factors or parietal cells, B12 levels, folate, bone marrow
84
Tx for megaloblastic anaemia
Replace B12, folate oral
85
```
Haemarthrosis
muscle haematoma
CNS bleeding
retroperitoneal bleeding
post-surgical bleeding associated with
```
haemophilia
86
Petechia, ecchymosis, mucosal bleeding, CNS bleeds, epistaxis, purpura, menorrhagia, GI associated with
thrombocytopenia
87
```
Anaphylaxis
angioedema
urticaria
rhinitis
asthma
dermatitis, eczema associated with
```
type 1 hypersensitivity
88
Renal failure, glomerulonephritis, nephrotic syndrome associated with
type 3 hypersensitivity
89
SCID
| Unusual rashes, diarrhoea, early stages of life, FH of neonatal death, consanguinity, associated with
primary immunodeficiency
90
Takes several hours, haemolysis associated with what hypersensitivity reaction
type 2
91
later in life chronic/ recurrent bacterial respiratory infections consider
antibody deficiency
92
2-3 days, delayed type of hypersensitivity reaction
type 4
93
SCID Tx
Stem cell transplantation, prophylaxis and avoid live vaccines, gene therapy
94
prophylaxis and treatment generally for primary immunodeficiency
Prophylactic antibiotics, in severe antibody deficiency then immunoglobulin replacement
95
Anaemia, splenomegaly, weight loss, hyperleukostasis (fundal haemorrhage, venous congestion, altered consciousness, respiratory failure), gout
hypercellular bone marrow, anaemia, high platelets, high WCC, basophils and cellular differentiation, t(9;22) associated with
chronic myeloid leukaemia
96
Headaches, itches, vascular occlusion, thrombosis, TIA, stroke, splenomegaly
raised haematocrits and Hg, raised WCC, platelets, raised uric acid, raised red cell mass
associated with
polycythaemia vera
97
Rapid undifferentiated cells accumulating
short history of bone, joint pain and bone marrow failure, infections, sweats
low Hg, high WCC, low platelets associated with
acute lymphoblastic leukaemia
98
Slow growing, low grade differentiated cells,
raised lymphocyte count of 5
asymptomatic, bone marrow failure (anaemia, thrombocytopenia), lymphadenopathy, fever, sweats, maybe hepatomegaly, infections, weight loss associated with
chronic lymphocytic leukaemia
99
Lymphadenopathy, hepatosplenomegaly, extra-nodal disease, bone marrow involvement, fever, night sweats, weight loss, IgM paraproteins associated with
lymphoma
100
IgG, IgA paraproteins, bone disease (lytic lesions, pathological fractures, cord compression, hypercalcaemia), bone marrow failure (Anaemia), infections, renal failure, hyper viscosity, amyloidosis associated with
myeloma
101
Blister and bite cells, neonatal jaundice, drug induced anaemia (intravascular haemolysis, haemoglobinuria), splenomegaly, pigment gallstones associated with
G6PD deficiency
102
Haemolysis, cation loss, dehydration, vaso-occlusion
painful bone crisis, stroke, infection, hyposplenism, gallstones, aplastic crisis, sequestration crises associated with
sickle cell anaemia
103
Tiredness, breathlessness, pallor, ankle swelling, dizziness, chest pain potentially jaundice, splenomegaly, lymphadenopathy, weight loss, diarrhoea, bleeding associated with
anaemia
