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haemotology and immunology > clinical > Flashcards

clinical Flashcards

1
Q

haemophilia signs and symptoms

A 
Haemarthrosis
muscle haematoma
CNS bleeding 
retroperitoneal bleeding 
post-surgical bleeding
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2
Q

haemophilia investigations

A 
Clinical 
prolonged APTT
normal PT
reduced FV 8/9
genetic analysis
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3
Q

haemophilia treatment

A 
Replacement 8/9
DDAVP 
tranexamic acid 
gene therapy 
splints, PT, synovectomy, joint replacement, analgesia
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4
Q

von Willebrand disease

A

Platelet type mucosal bleeding; epistaxis, purpura, menorrhagia, GI

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5
Q

Tx for Von willebrand disease

A

VWF or DDAVP, tranexamic acid, OCP

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6
Q

signs and symptoms of thrombocytopenia

A

Petechia, ecchymosis, mucosal bleeding, CNS bleeds

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7
Q

investigations for liver failure thrombocytopenia

A

Liver failure -> reduced factors, prolonged PT, APTT, reduced fibrinogen, Vit K depletion

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8
Q

Immune thrombocytopenia Tx

A

steroids, IgG, splenectomy, thrombopoietin analogues

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9
Q

liver failure thrombocytopenia Tx

A

factor replacement, replacement of fresh frozen plasma, Vit K

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10
Q

haemorrhagic disease of the newborn tx

A

Vit K

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11
Q

haemolytic disease of the newborn sign

A

anaemia and jaundice

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12
Q

Tx for haemolytic disease of the newborn

A

Prophylactic anti-D 28/40 and sensitising events

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13
Q

type 1 hypersensitivity signs and symptoms

A 
Anaphylaxis
angioedema
urticaria
rhinitis
asthma
dermatitis, eczema
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14
Q

type 1 hypersensitivity Tx

A

Beta2- adrenergic agonists, epinephrine, anti-histamines, corticosteroids

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15
Q

type 2 hypersensitivity signs

A

Takes several hours, haemolysis

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16
Q

type 2 hypersensitivity signs

A

Renal failure, glomerulonephritis, nephrotic syndrome

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17
Q

type 4 hypersensitivity signs

A

2-3 days, delayed

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18
Q

type 4 hypersensitivity treatment

A

NSAIDs, corticosteroids, drugs that block TNF, IL-6, antibodies against B cells, immunosuppressive drugs

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19
Q

primary immunodeficiency signs and symptoms

A

SCID

Unusual rashes, diarrhoea, early stages of life, FH of neonatal death, consanguinity

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20
Q

investigations for primary immunodeficiency

A

Low lymphocyte count, flow cytometry

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21
Q

antibody deficiency signs and symptoms

A

later in life chronic/ recurrent bacterial respiratory infections

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22
Q

antibody deficiency Dx

A

antibody levels; IgG, IgM, IgA, specific antibodies, complement and neutrophil function

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23
Q

neutropenic sepsis symptoms and signs

A

> 38.5C, rigors, chest infection, skin sepsis, UTI, septic shock

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24
Q

initial investigations for neutropenic sepsis

A

Blood cultures, urine output, serum lactate

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25
Q

further Dx for neutropenic sepsis

A

CXR, throat swab, sputum, FBC, renal, liver function, coagulation screen, CT if fungal suspected

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26
Q

neutropenic sepsis prophylaxis

A

ciprofloxacin, fluconazole, aciclovir, co-trimoxazole, G-CSF, stem cell transplant, laminar flow rooms, I.V. Ab, vaccine

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27
Q

neutropenic sepsis Tx

A

oxygen, antibiotics (Tazocin, gentimicin, vancomycin, teicoplanin maybe caspofungin) , I.V fluids

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28
Q

Acute myeloblastic Leukaemia symptoms

A

Bone marrow failure, rapidly fatal, anaemia, thrombocytopenic bleeding, infection

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29
Q

Acute myeloblastic Leukaemia Dx

A

Blood count and film, bone marrow aspirate, cytogenic, immunophenotyping,
CSF, targeted molecular genetics

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30
Q

Acute myeloblastic Leukaemia Tx

A

Daunorubicin, cytosine arabinoside, CPX-351, allogeneic stem cell transplant, arsenic trioxide, all-trans retinoic acid, midostaurin tyrosine kinase inhibitor targeted treatment

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31
Q

Chronic myeloid leukaemia symptoms and signs

A

Anaemia, splenomegaly, weight loss, hyperleukostasis , gout

hypercellular bone marrow, anaemia, high platelets, high WCC, basophils and cellular differentiation, t(9;22)

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32
Q

Chronic myeloid leukaemia Dx

A

FBC, blood film, bone marrow biopsy

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33
Q

Chronic myeloid leukaemia Tx

A

Tyrosine kinase inhibitors imatinib, dasatinib, direct inhibitors BCR-ABL 1st line, allogeneic transplantation in TKI failure

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34
Q

hyperleukostasis causes

A

(fundal haemorrhage, venous congestion, altered consciousness, respiratory failure)

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35
Q

Polycythaemia Vera symptoms

A

Headaches, itches, vascular occlusion, thrombosis, TIA, stroke, splenomegaly

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36
Q

Polycythaemia Vera Dx

A

FBC, haematocrit, uric acid, blood film

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37
Q

Polycythaemia Vera Tx

A

Venesection, aspirin, hydroxycarbamide, ruxolitinib

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38
Q

polycythaemia vera labs

A

raised haematocrits and Hg, raised WCC, platelets, raised uric acid, raised red cell mass

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39
Q

Essential thrombocythemia signs and symptoms

A

Raised platelet count

arterial and venous thrombosis, digital ischaemia, gout, headache
mild splenomegaly

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40
Q

essential thrombocytopenia Tx

A

Aspirin, hydroxycarbamide, anagrelide

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41
Q

Essential thrombocythemia

Acute lymphoblastic leukaemia symptoms and signs

A

Rapid undifferentiated cells accumulating

short history of bone, joint pain and bone marrow failure, infections, sweats

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42
Q

Essential thrombocythemia

Acute lymphoblastic leukaemia Dx

A

Bone marrow biopsy, FBC

43
Q

Essential thrombocythemia

Acute lymphoblastic leukaemia Tx

A

Chemo, CNS and consolidation therapy, high risk then stem cell transplant, bi specific T cell engagers (Blinatumumab), CAR-T cells

44
Q

Chronic lymphocytic leukaemia symptoms

A

asymptomatic, bone marrow failure (anaemia, thrombocytopenia), lymphadenopathy, fever, sweats, maybe hepatomegaly, infections, weight loss

45
Q

essential thrombocytopenia labs

A

low Hg, high WCC, low platelets

46
Q

chronic lymphocytic leukaemia signs

A

Slow growing, low grade differentiated cells,

raised lymphocyte count of 5

47
Q

chronic lymphocytic leukaemia Tx

A

Watch and wait
cytotoxic chemo fludarabine, bendamustine
monoclonal antibody rituximab,
Tyrosine kinase inhibitors ibrutinib, PI3K inhibitor, BCL2 inhibitor

48
Q

lymphoma symptoms

A

Lymphadenopathy, hepatosplenomegaly, extra-nodal disease, bone marrow involvement, fever, night sweats, weight loss, IgM paraproteins

49
Q

staging for lymphoma

A

biopsy lymph node, CT, bone marrow aspirate, trephine biopsy

50
Q

Tx for lymphoma

A

Chemo, anti CD20 monoclonal antibodies,

51
Q

Hodgkin lymphoma investigation for responsiveness

52
Q

Tx for Hodgkin lymphoma

A

Chemo, RT, monoclonal antibodies anti-CD30, immunotherapy checkpoint inhibitors

53
Q

myeloma symptoms

A

IgG, IgA paraproteins, bone disease (lytic lesions, pathological fractures, cord compression, hypercalcaemia), bone marrow failure (Anaemia), infections, renal failure, hyper viscosity, amyloidosis

54
Q

Dx myeloma

A

CXR, MRI, biopsy of bone marrow with presence of excess plasma cells

55
Q

staging for myeloma

A

albumin and beta 2 microglobulin

56
Q

chemo for myeloma

A

proteasome inhibitors carfilzomib, bortezomib

57
Q

IMIDS for myeloma

A

lenalidomide, pomalidomide

58
Q

Tx for myeloma

A

monoclonal Ab,
bisphosphonate therapy, RT, steroids, pinning of long bones, decompression of the spinal cord, autologous stem cell transplant

59
Q

arterial thrombus Tx

A

Lifestyle modification, treatment of vascular risk factors, antiplatelet, anticoagulant drugs, thrombolytic therapy

60
Q

venous thrombus Tx

A

Prevent clot extension, prevent clot embolization, prevent clot recurrence in long term treatment.

anticoagulants (warfarin, DOAC’s, LMWH)
thrombolysis in massive PE

61
Q

venous thrombus Dx

A

Wells and Geneva score, D-dimer, Doppler, V/Q scan, CT pulmonary angiogram

62
Q

microvascular thrombus signs

A

DIC, diffuse ischaemia, gangrene, organ failure, bleeding

63
Q

hereditary spherocytosis symptoms

A

Anaemia, jaundice neonatal, splenomegaly, pigment gallstones

64
Q

hereditary spherocytosis Dx

A

FBC and film

65
Q

hereditary spherocytosis Tx

A

Folic acid, transfusion, splenectomy

66
Q

G6PD deficiency symptoms and signs

A

Blister and bite cells, neonatal jaundice, drug induced anaemia (intravascular haemolysis, haemoglobinuria), splenomegaly, pigment gallstones

67
Q

pyruvate kinase deficiency symptoms and signs

A

Anaemia, jaundice, gallstones

68
Q

thalassaemia beta major signs and symptoms

A

Beta major: expansion of ineffective bone marrow, bony deformities, splenomegaly, growth retardation

69
Q

thalassaemia beta major Tx

A

Chronic transfusions, chelation therapy with desferrioxamine, bone marrow transplant

70
Q

sickle cell anaemia symptoms and signs

A

Haemolysis, cation loss, dehydration, vaso-occlusion

painful bone crisis, stroke, infection, hyposplenism, gallstones, aplastic crisis, sequestration crises

71
Q

chest crisis investigation for sickle cell anaemia

A

Chest crisis-> spirometry , CXR

72
Q

painful crisis sickle cell anaemia tx

A

hydration, oxygen, antibiotics

73
Q

chest crisis sickle cell anaemia Tx

A

respiratory support, IV fluids, antibiotics, transfusion

74
Q

long term sickle cell anaemia Tx

A

vaccination, penicillin, folic acid, hydroxycarbamide, bone marrow transplant, gene therapy

75
Q

anaemia symptoms

A

Tiredness, breathlessness, pallor, ankle swelling, dizziness, chest pain potentially jaundice, splenomegaly, lymphadenopathy, weight loss, diarrhoea, bleeding

76
Q

hypochromic microcytic anaemia follow up

77
Q

normochromic normocytic anaemia follow up

A

reticulocyte

78
Q

macrocytic anaemia follow up

A

B12/folate/bone marrow

79
Q

haemolytic anaemia appearance on a blood film

A

schistocytes

80
Q

haemolytic anaemia Dx

A

Direct antibody test
immune mediated then; warm, cold or allo
blood film, FBC, serum bilirubin, LDH, serum haptoglobin, urine

81
Q

Tx for haemolytic anaemia

A

Folic acid, immunosuppression, steroids, splenectomy, treat underlying cause, consider transfusion

82
Q

megaloblastic anaemia symptoms and signs

A

Neurological symptoms, yellow tinge, LDH, friable red cells

83
Q

megaloblastic anaemia Dx

A

Autoantibodies against intrinsic factors or parietal cells, B12 levels, folate, bone marrow

84
Q

Tx for megaloblastic anaemia

A

Replace B12, folate oral

85
Q 
Haemarthrosis
muscle haematoma
CNS bleeding 
retroperitoneal bleeding 
post-surgical bleeding associated with
A

haemophilia

86
Q

Petechia, ecchymosis, mucosal bleeding, CNS bleeds, epistaxis, purpura, menorrhagia, GI associated with

A

thrombocytopenia

87
Q 
Anaphylaxis
angioedema
urticaria
rhinitis
asthma
dermatitis, eczema  associated with
A

type 1 hypersensitivity

88
Q

Renal failure, glomerulonephritis, nephrotic syndrome associated with

A

type 3 hypersensitivity

89
Q

SCID

Unusual rashes, diarrhoea, early stages of life, FH of neonatal death, consanguinity, associated with

A

primary immunodeficiency

90
Q

Takes several hours, haemolysis associated with what hypersensitivity reaction

91
Q

later in life chronic/ recurrent bacterial respiratory infections consider

A

antibody deficiency

92
Q

2-3 days, delayed type of hypersensitivity reaction

93
Q

SCID Tx

A

Stem cell transplantation, prophylaxis and avoid live vaccines, gene therapy

94
Q

prophylaxis and treatment generally for primary immunodeficiency

A

Prophylactic antibiotics, in severe antibody deficiency then immunoglobulin replacement

95
Q

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

A

chronic myeloid leukaemia

96
Q

Headaches, itches, vascular occlusion, thrombosis, TIA, stroke, splenomegaly

raised haematocrits and Hg, raised WCC, platelets, raised uric acid, raised red cell mass
associated with

A

polycythaemia vera

97
Q

Rapid undifferentiated cells accumulating

short history of bone, joint pain and bone marrow failure, infections, sweats

low Hg, high WCC, low platelets associated with

A

acute lymphoblastic leukaemia

98
Q

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

A

chronic lymphocytic leukaemia

99
Q

Lymphadenopathy, hepatosplenomegaly, extra-nodal disease, bone marrow involvement, fever, night sweats, weight loss, IgM paraproteins associated with

100
Q

IgG, IgA paraproteins, bone disease (lytic lesions, pathological fractures, cord compression, hypercalcaemia), bone marrow failure (Anaemia), infections, renal failure, hyper viscosity, amyloidosis associated with

101
Q

Blister and bite cells, neonatal jaundice, drug induced anaemia (intravascular haemolysis, haemoglobinuria), splenomegaly, pigment gallstones associated with

A

G6PD deficiency

102
Q

Haemolysis, cation loss, dehydration, vaso-occlusion

painful bone crisis, stroke, infection, hyposplenism, gallstones, aplastic crisis, sequestration crises associated with

A

sickle cell anaemia

103
Q

Tiredness, breathlessness, pallor, ankle swelling, dizziness, chest pain potentially jaundice, splenomegaly, lymphadenopathy, weight loss, diarrhoea, bleeding associated with

haemotology and immunology (3 decks)

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