Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

Haematology > Haematology P3 > Flashcards

Haematology P3 Flashcards

1
Q

What qualifies as neutropenia?

A

< 1.5x10^9

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Causes of neutropenia:

A
  • viral: HIV, EBV, hepatitis
  • drugs: cytotoxics, carbimazole, clozapine
  • benign ethnic neutropenia: black African and Afro-Caribbean, no Tx
  • haematological malignancy: myelodysplastic, aplastic anaemia
  • rheumatological
  • SLE
  • RA (hypersplenism in Felty’s)
  • severe sepsis
  • haemodialysis
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

What causes neutropenic sepsis and what are the features? (how can you prevent)

A
  • 7-14 days post chemotherapy
  • neutrophil count <0.5x10^9
  • temp >=38 degrees
  • sepsis signs
  • prophylaxis: fluoroquinolone
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Management of neutropenic sepsis:

A
  • Abx immediately
  • piperacillin with tazobactam
  • if still unwell after 48 hours, meropenem (+vancomycin)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Causes of normocytic anaemia:

A
  • anaemia of chronic disease
  • chronic kidney disease
  • aplastic anaemia
  • haemolytic anaemia
  • acute blood loss
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Whats is paroxysmal nocturnal haemoglobinuria:

A
  • acquired disorder leading to haemolysis (mainly intravascular)
  • caused by increased sensitivity of cell membranes to complement due to lack of glycoprotein glycosyl-phosphatidylinositol (GPI)
  • patients more prone to venous thrombosis
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Features of paroxysmal nocturnal haemoglobinuria:

A
  • haemolytic anaemia
  • pancytopaenia may be present
  • haemoglobinuria: dark urine in morning
  • thrombosis e.g. Budd-Chiari syndrome
  • aplastic anaemia in some patients
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Diagnosis of paroxysmal nocturnal haemoglobinuria:

A

Ham’s test: acid induced haemolysis (not with normal red cells)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Management of paroxysmal nocturnal haemoglobinuria:

A
  • blood product replacement
  • anticoagulation
  • eculizumab, monoclonal antibody directed against terminal protein C5
  • stem cell transplant
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

When is platelet transfusion offered to a patient with active bleeding?

A
  • platelet count <30x10^9 with bleeding e.g. haematemesis, melaena, prolonged epistaxis
  • transfusion thresholds are higher: max <100x10^9 with severe bleeding or bleeding at critical sites e.g. CNS
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Platelet transfusions have the highest risk of what compared to other types of blood product:

A

bacterial contamination

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

When would platelet transfusions be used prophylactically?

A

-pre-invasive procedure
-for thrombocytopenia before surgery
-aim for platelet levels of >50x10^9/L for most patients
50-75x10^9/L if high risk bleeding
>100x10^9 if surgery at critical site

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

When would platelet transfusions be use without active bleeding or planned invasive procedure?

A

threshold of 10x10^9

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

CONTRA for platelet transfusions:

A
  • chronic bone marrow failure
  • autoimmune thrombocytopenia
  • heparin induced thrombocytopenia
  • thrombotic thrombocytopenia purpura
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

What is polycythaemia caused by?

A
  • relative: dehydration, stress (Gaisbock syndrome)
  • primary (rubra vera)
  • secondary: COPD, altitude, OSA, excessive erythropoietin ( cerebellar haemangioma, hypernephroma, hepatoma, uterine fibroids)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

How do you differentiate between the different causes of polycythaemia?

A

red cell mass studies

-true polycythaemia: >35ml/kg in men and >32ml/kg in women

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

What is polycythaemia rubra vera?

A
  • myeloproliferative disorder
  • clonal proliferation of marrow stem cells leading to increased RCV
  • overproduction of neutrophils and platelets
  • mutation of JAK2 in 95% of patients
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

Features of polycythaemia rubra vera:

A
  • hyperviscosity
  • pruritus, typically after hot bath
  • splenomegaly
  • haemorrhage
  • plethoric appearance
  • HTN
  • low ESR
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

Criteria for JAK2 positive polycythaemia vera:

A

A1 - high haematocrit or raised red cell mass

A2 - mutation in JAK2

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

Criteria for JAK2 negative polycythaemia vera:

A

requires A1, 2 and 3 with either another A or 2 B criteria
A1 - raised red cell mass or haematocrit
A2 - absence of JAK2 mutation
A3 - no cause of secondary erythrocytosis
A4 - palpable splenomegaly
A5 - presence of acquired genetic abnormality in haematopoietic cells
B1 - thrombocytosis
B2 - neutrophil leucocytosis
B3 - radiological evidence of splenomegaly
B4 - endogenous erythroid colonies or low serum erythropoietin

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

Management of polycytheamia vera:

A
  • aspirin
  • venesection - first line
  • hydroxyurea - slightly increased risk of secondary leukaemia
  • phosphorus-32 therapy
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

Prognosis of polycytheamia vera:

A
  • thrombotic events
  • 5-15% myelofibrosis
  • 5-15% acute leukaemia
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

Post-thrombotic syndrome symptoms and management:

A
  • painful heavy calves
  • pruritus
  • swelling
  • varicose veins
  • venous ulceration
  • compression stocking once syndrome has developed and keep leg elevated
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

How do DVT/PE come about in pregnancy?

A
  • hypercoagulable state
  • majority in last trimester
  • increase in VII, VIII, X and fibrinogen
  • decrease in protein S
  • uterus presses on IVC causing venous stasis in legs
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

Treatment of DVT/PE in pregnancy:

A
  • warfarin contraindicated

- LMWH preferred to IV

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

Neutrophil disorders:

A
  • chronic granulomatous disease
  • Chediak-Higashi syndrome
  • Leukocyte adhesion deficiency
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

What happens in chronic granulomatous disease:

A
  • lack of NADPH oxidase reduces ability of phagocytes to produce ROS
  • recurrent pneumonias and abscesses (esp catalase positive bacteria e.g. staph aureus and fungi e.g. aspergillus)
  • negative nitro blue tetrazolium test
  • abnormal dihydrorhodamine flow cytometry test
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
28
Q

What happens in Chediak-Higashi syndrome:

A
  • microtubule polymerisation defect which leads to decrease in phagocytosis
  • partial albinism and neuropathy
  • recurrent bacterial infections
  • giant granules in neutrophils and platelets
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
29
Q

What happens in leukocyte adhesion deficiency:

A
  • defect of LFA-1 intern (CD18) protein on neutrophils
  • recurrent bacterial infections
  • delay in umbilical cord sloughing may be seen
  • absence of neutrophils/pus at sites of infections
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
30
Q

B cell disorders:

A
  • common variable immunodeficiency
  • Bruton’s (x-linked) congenital agammaglobulinaemia
  • Selective immunoglobulin A deficiency
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
31
Q

Common variable immunodeficiency:

A
  • many causes
  • hypogammaglobulinaemia
  • predisposes to autoimmune disorders and lymphoma
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
32
Q

Bruton’s congenital agammaglobulinaemia:

A
  • defect in Bruton’s tyrosine kinase (BTK) gene that leads to severe block in B cell development
  • x-linked recessive
  • recurrent bacterial infections
  • absence of B cells with reduced immunoglobulins
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
33
Q

Selective immunoglobulin A deficiency:

A
  • maturation defect in b cells
  • most common primary Ab deficiency
  • recurrent sinus and respiratory infections
  • associated with coeliac disease and may cause false negative coeliac Ab screen
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
34
Q

DiGeorge syndrome:

A
  • T cell disorder
  • 22q11.2 deletion
  • failure to develop 3rd and 4th pharyngeal pouches
  • congenital heart disease (e.g. tetralogy of fallot), learning difficulties, hypocalcaemia, recurrent viral/fungal diseases, cleft palate
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
35
Q

Combined B and T cell disorders:

A
  • severe combined immunodeficiency
  • ataxic telangiectasia
  • Wiskott-Aldrich syndrome
  • Hyper IgM syndromes
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
36
Q

Severe combined immunodeficiency:

A
  • most common x-linked due to defect in common gamma chain, protein in receptors for IL-2
  • also due to adenosine deaminase deficiency
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
37
Q

Ataxic telangiectasia:

A
  • defect in DNA repair enzymes
  • autosomal recessive
  • cerebellar ataxia, telangiectasia, recurrent chest infections
  • 10% risk malignancy, lymphoma or leukaemia
38
Q

Wiskott-Aldrich syndrome:

A
  • WASP gene
  • x-linked recessive
  • recurrent bacterial infections, eczema, thrombocytopenia
  • low IgM levels
  • increased risk of autoimmune disorders and malignancy
39
Q

Hyper IgM syndromes:

A
  • mutations in CD40 gene
  • infection/pneumocystis pneumonia
  • hepatitis
  • diarrhoea
40
Q

What is sickle cell anaemia?

A
  • auotosomal recessive
  • synthesis of abnormal haemoglobin chain HbS
  • more common in African descent (heterozygous condition offers protection against malaria)
  • only symptomatic if severely hypoxic
  • symptoms in homozygotes not until 4-6 months when HbS takes over from foetal haemoglobin
  • sickle cells are fragile and haemolyse; block small blood vessels
41
Q

Investigations for sickle cell anaemia:

A

haemoglobin electrophoresis

42
Q

Sickle cell crisis management:

A
  • analgesia
  • rehydrate
  • oxygen
  • Abx if evidence of infection
  • blood transfusion
  • exchange transfusion e.g. if neurological complications
43
Q

Longer term management of sickle cell anaemia:

A
  • hydroxyurea - increases HbF levels

- receive pneumococcal polysaccharide vaccine every 5 years

44
Q

Types of sickle cell crises:

A
  • thrombotic (painful)
  • sequestration
  • acute chest syndrome
  • aplastic
  • haemolytic
45
Q

Sickle cell thrombotic crisis:

A
  • painful/vaso-occlusive crisis
  • precipitated by infection, dehydration, deoxygenation
  • diagnosed clinically
  • e.g. avascular necrosis of hip, hand-foot syndrome in children, lungs, spleen and brain
46
Q

Sequestration crisis in sickle cell:

A

sickling within organs such as spleen or lungs causes pooling of blood with worsening of anaemia

47
Q

Acute chest syndrome in sickle cell:

A
  • dyspnoea
  • chest pain
  • pulmonary infiltrates
  • low pO2
  • most common cause of death after childhood
48
Q

Aplastic crisis in sickle cell:

A
  • caused by infection with parvovirus
  • sudden all in Hb
  • low reticulocyte
49
Q

Haemolytic crises in sickle cell:

A
  • rare
  • fall in Hb due to increased rate of haemolysis
  • high reticulocyte
50
Q

What is sideroblastic anaemia?

A
  • red cells fail to completely form haem
  • deposits of iron in mitochondria; ring around nucleus called ring sideroblast
  • congenital or acquired
51
Q

Congenital cause of sideroblastic anaemia:

A

delta -aminolevulinate synthase-2 deficiency

52
Q

Acquired causes of sideroblastic anaemia:

A
  • myelodysplasia
  • alcohol
  • lead
  • anti-TB meds
53
Q

Investigations for sideroblastic anaemia:

A
  • hypochromic microcytic anaemia

- bone marrow: sideroblasts and increased iron stores

54
Q

Management of sideroblastic anaemia:

A
  • supportive
  • treat any underlying cause
  • pyridoxine may help
55
Q

Causes of massive splenomegaly:

A
  • myelofibrosis
  • chronic myeloid leukaemia
  • visceral leishmaniasis
  • malaria
  • Gaucher’s syndrome
56
Q

Other causes of splenomegaly:

A
  • portal HTN
  • lymphoproliferative disease
  • haemolytic anaemia
  • infection: hepatitis, glandular fever
  • infective endocarditis
  • sickle cell, thalassaemia
  • RA (Felty’s)
57
Q

Causes of severe thrombocytopenia:

A
  • ITP
  • DIC
  • TTP
  • haematological malignancy
58
Q

Causes of moderate thrombocytopenia:

A
  • heparin induced thrombocytopenia
  • drug induced (quinine, diuretics, sulphonamides, aspirin, thiazides)
  • alcohol
  • liver disease
  • hypersplenism
  • viral infection
  • pregnancy
  • SLE/antiphospholipid syndrome
  • vit B12 deficiency
59
Q

What is pseudothrombocytopenia associated with?

A

EDTA as anticoagulant

60
Q

What qualifies as thrombocytosis:

A

> 400x10^9/L

61
Q

Causes of thrombocytosis:

A
  • reactive e.g. infection, surgery, iron def anaemia
  • malignancy
  • essential thrombocytosis
  • hyposplenism
62
Q

Essential thrombocytosis:

A
  • one of myeloproliferative disorders
  • overlaps with chronic myeloid leukaemia, polycythaemia rubra vera and myelofibrosis
  • megakaryocyte proliferation results in overproduction of platelets
63
Q

Features of essential thrombocytosis:

A
  • platelet >600x10^9/L
  • both thrombosis and haemorrhage can be seen
  • burning sensation in hands
  • JAK2 mutation in 50% patients
64
Q

Management of essential thrombocytosis:

A
  • hydroxyurea to reduce platelet count
  • interferon alpha in younger patients
  • low dose aspirin to reduce thrombotic risk
65
Q

Thrombophilia inherited causes:

A

Gain of function polymorphisms:

  • factors V Leiden (activated protein C resistance) - most common
  • prothrombin gene mutation - second most common
  • antithrombin III def
  • protein C def
  • protein S def
66
Q

Which thrombophilias have the highest relative risk of VTE?

A
  • Factor V Leiden homozygous
  • Protein C def
  • antithrombin III def
67
Q

Causes of acquired thrombophilia:

A
  • antiphospholipid syndrome

- COCP

68
Q

What is TTP?

A
  • abnormally large and sticky multimers of VWF
  • clumps in vessels
  • deficiency of ADAMTS13 (metalloprotease enzyme)
  • overlaps with HUS
69
Q

Features of TTP:

A
  • rare, typically adult females
  • fever
  • fluctuating neuro signs (micro emboli)
  • microangiopathic haemolytic anaemia
  • thrombocytopenia
  • renal failure
70
Q

Causes of TTP:

A
  • post infection
  • pregnancy
  • drugs: ciclosporin, COCP, penicillin, clopidogrel, acyclovir
  • tumours
  • SLE
  • HIV
71
Q

What is a thymoma?

A
  • most common tumours of anterior mediastinum
  • assoc: myasthenia gravis (30-40%), red cell aplasia, dermatomyositis, SLE, SIADH
  • causes of death: compression of airway, cardiac tamponade
72
Q

What is tranexamic acid used for?

A
  • synthetic derivation of lysine
  • antifibrinolytic that reversibly binds to lysine receptor site on plasminogen or plasmin
  • commonly for menorrhagia
  • IV bolus followed by infusion for major haemorrhage
73
Q

What is tumour lysis syndrome:

A
  • related to high grade lymphomas and leukaemias
  • usually with chemotherapy (occasionally with steroids)
  • prophylaxis: IV allopurinol or IV rasburicase
  • lower risk: oral allopurinol
  • breakdown of tumour cells leads to hyperkalaemia and hyperphosphataemia with hypoclacaemia
  • present with AKI and hyperuricaemia
  • clinical tumour lysis syndrome: lab tumour lysis syndrome with either increased creatinine (1.5 times) or cardiac arrhythmia/sudden death or seizure
74
Q

General risk factors for VTE:

A
  • increased risk with advancing age
  • obesity
  • family history of VTE
  • pregnancy
  • immobility
  • hospitalisation
  • anaesthesia
  • central venous catheter: femoral»subclavian
75
Q

Underlying condition risk factors for VTE:

A
  • malignancy
  • thrombophilia
  • heart failure
  • antiphospholipid
  • Behcet’s
  • polycythaemia
  • nephrotic syndrome
  • sickle cell
  • paroxysmal nocturnal haemoglobinuria
  • hyperviscosity syndrome
  • homocystinuria
76
Q

Medication risk factors for VTE:

A
  • COCP
  • HRT: higher with oestrogen and progesterone combined
  • raloxifene and tamoxifen
  • antipsychotics especially onlanzapine
77
Q

What is B12 used for and how is it absorbed?

A
  • red blood cell development and maintenance of nervous system
  • absorbed after binding to IF secreted from parietal cells in stomach
  • absorbed in terminal ileum
  • small amount absorbed passively
78
Q

Causes of vitamin B12 deficiency:

A
  • pernicious anaemia: most common
  • post gastrectomy
  • vegan/poor diet
  • disorders of terminal ileum (Crohn’s)
  • metformin (rare)
79
Q

Features of vit B12 deficiency:

A
  • macrocytic anaemia
  • sore throat and mouth
  • neurological symptoms: dorsal column affected first prior to distal paraesthesia
  • neuropsychiatric symptoms
80
Q

Management of vit B12 deficiency:

A
  • if no neurological involvement 1mg of IM hydroxocobalamin 3 times each week for 2 weeks then every 3 months
  • if also deficient in folic acid, treat B12 first to avoid precipitating subacute combined degeneration of cord
81
Q

What is VW disease?

A

-most common inherited bleeding disorder
-autosomal dominant
-vWF is carrier molecule for factor VIII, promotes platelet adhesion to damaged endothelium, large glycoprotein which forms massive multimers
type I: partial reduction in vWF (most common)
type II: abnormal form of vWF
type III: total lack of vWF (autosomal recessive)

82
Q

Investigations for VW disease:

A
  • prolonged bleeding time
  • APTT may be prolonged
  • factor VIII levels may be moderately reduced
  • defective platelet aggregation with ristocetin
83
Q

Management of VW disease:

A
  • tranexamic acid for mild bleeding
  • desmopressin: raised levels of vWF by inducing release of vWF from Weibel-Palade bodes in endothelial cells
  • factor VIII concentrate
84
Q

What is Waldenstrom’s macroglobulinaemia?

A
  • in older men

- lymphoplasmacytoid malignancy characterised by secretion of monoclonal IgM paraprotein

85
Q

Features of Waldenstrom’s macroglobulinaemia:

A
  • monoclonal IgM paraproteinaemia
  • systemic upset: weight los, lethargy
  • hyperviscosity syndrome e.g. visual disturbances (pentameric configuration of IgM increases serum viscosity)
  • hepatosplenomegaly
  • lymphadenopathy
  • cryoglobulinaemia e.g. Raynaud’s
86
Q

What is Wiskott-Aldrich syndrome and what are the features:

A
  • primary immunodeficiency due to combined B and T cell dysfunction
  • inherited in X-linked recessive fashion
  • mutation in WASP gene
  • recurrent bacterial infections e.g. chest, eczema, thrombocytopaenia, low IgM levels
87
Q

Complication of ileocaecal resection in Crohn’s:

A

vit B12 def

88
Q

What is disproportionate microcytic anaemia?

A
  • microcytic anaemia
  • drop in MCV much more than Hb
  • beta thalassaemia
89
Q

What type of anaemia does sickle cell cause?

A

normocytic (low Hb, normal MCV, increased reticulocytes)

90
Q

What causes an isolated rise in Hb?

A

polycythaemia vera

91
Q

What does IgA deficiency put you at increased risk of with transfusions?

A

anaphylactic reaction

Haematology (4 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions