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Haematology > Blood disorders > Flashcards

Blood disorders Flashcards

1
Q

What is thrombocytosis

A

Abnormally high platelet count usually >400*10^9/L

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2
Q

Causes of thrombocytosis

A

Reactive: platelets are an acute phase reactant- platelet count can increase in response to stress such as an severe infection, surgery
Iron deficiency anaemia can also cause a reactive thrombocytosis
Malignancy
Essential thrombocytosis or as part of another myeloproliferative disorder sch as CML or PCV

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3
Q

What is essential thrombocytosis

A

One of the myeloproliferative disorders which overlaps with chronic myeloid leukaemia, polycythaemia rubra vera and myelofirbosis.

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4
Q

Features of essential thrombocytosis

A

Features
platelet count > 600 * 109/l
both thrombosis (venous or arterial) and haemorrhage can be seen
a characteristic symptom is a burning sensation in the hands
a JAK2 mutation is found in around 50% of patients

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5
Q

Management of thrombocytosis

A

Hydroxyurea (hydoxycarbamide widely used to reduce the platelet count
Interferon- alpha is also used in younger patients
Low-dose aspirin may be used to reduce the thrombotic risk

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6
Q

Causes of massive splenomegaly

A 
Myelofibrosis
Chronic myeloid leukaemia
Visceral leishmaniasis
Malaria
Gaucher's syndrome
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7
Q

Other causes of splenomegaly

A

Portal hypertension- secondary to cirrhosis
Lymphoproliferative disease- CLL, Hodgkin’s
Haemolytic anaemia
Infection - hepatitis, glandular fever
Infective endocarditis
Sickle-cell thalassemia
Rheumatoid arthritis

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8
Q

Hereditary spherocytosis features

A

most common hereditary haemolytic anaemia in people of northern European descent
autosomal dominant defect of red blood cell cytoskeleton
the normal biconcave disc shape is replaced by a sphere-shaped red blood cell
red blood cell survival reduced as destroyed by the spleen

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9
Q

Presentation of hereditary spherocytosis

A

Failure to thrive

  • Jaundice, gallstones
  • Splenomegaly
  • Aplastic crisis precipitated by parvovirus infection
  • Degree of haemolysis variable
  • MCHC elevated
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10
Q

Diagnosis of hereditary spherocytosis

A

The osmotic fragility test - FIRST line

Diagnosis- equivocal - BJH recommend te EMA binding test and the cryohaemolysis test

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11
Q

Management

A

Acute haemolytic crisis- treatment is generally supportive
Transfusion if necessary

Longer term treatment
Folate replacement
Splenectomy

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12
Q

Beta-thalassemia trait

A

Group of genetic disorders charcterised by reduced production rate of either alpha or beta chains
Beta-thalassemia trait is an autosomal recessive condition characterised by a mild hypochromic, microcytic anaemia- USUALLY Asymptomatic

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13
Q

Features of Beta-thalassemia trait

A

Mild hypochromic, microcytic anaemia- microcystosis characteristically disproportionate to the anaemia
BhA2 raised- >3.5%

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14
Q

How does secondary polycythaemia in COPD work

A

Impaired oxygen exchange in the lungs - resulting in a low PaO2 which results in stimulation of EPO release from the kidneys.
EPO stimulates erythropoeises and increases red cell mass- resulting in polycythaemia.

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15
Q

Relative causes of polycythaemia

A

Dehydration

Stress: Gaisbock syndrome

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16
Q

Primary causes of Polycythaemia

A

Polycythaemia rubra vera

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17
Q

Secondary causes

A

COPD
Altitude
Obstructive sleep apnoea
Excessive erythropoeitin, cerebellar haemangioma, hypernephroma, hepatoma, uterine fibroids

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18
Q

Sickle-cell crises example

A
  • Thrombotic painful crises
  • Sequestration
  • Acute chest syndrome
  • Aplastic
  • Haemolytic
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19
Q

Thrombotic crises features

A

Painful crises or vaso-occlusive crises
Precipitated by infection, dehydration, deoxygenation
Painful vaso-occlusive crises- diagnoses CLINICALLY

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20
Q

Sequestration crises

A

Sickling within organs - such as spleen or lungs causes pooling of blood with worsening of the anaemia

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21
Q

Acute chest syndrome features

A

Dyspnoea, chest pain, pulmonary infiltrates, low pO2

The most common cause of death after childhood

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22
Q

Aplastic crisis features

A

Caused by infection with pravovirus

Sudden fall in haemoglobin

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23
Q

Haemolytic crises features

A

Rare

Fall in haemoglobin due to increased rate of haemolysis

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24
Q

Investigation of Von Willebrand disease results

A

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

25
Q

Management of Von Willebrand’s disease

A

Tranexamic acid for bleeding
Despmopressin (DDAVP) raises levels of vWF by inducing release of vWF from Weibel- Palade bodies in endothelial cells
Factor VIII concentrate

26
Q

Why are irradiated blood products used

A

To avoid transfusion-associated graft versus host disease

27
Q

Classical description of Reed-Sternberg cells

A

A blood film shows large cells with a bilobed nucleus and prominent eosinophilic inclusion-like nucleoli.

28
Q

When is CMV negative packed red cells recommended

A

They are recommended for pregnant and neonatal transfusion

29
Q

FFP use ?

A

Clotting studies

30
Q

Features of lead poisoning

A 
Abdominal pain
Peripheral neuropathy 
Fatigue
Constipation 
Blue lines on gum margin (only 20% of adults- very rare in children)
31
Q

Investigations

A

The blood lead level is usually used for diagnosis, levels greater than 10mcg/dl are considered significant
Full blood count: microcytic anaemia. Blood films- basophilic stippling and clover-leaf morphology
Raised serum and urine levels

32
Q

Management - various chelating agents are currently used

A

dimercaptosuccinic acid (DMSA)
D-penicillamine
EDTA
dimercaprol

33
Q

Autoimmune haemolytic anaemia classification

A

warm and cold

34
Q

AIHA Cooms test result

A

Positive result

35
Q

Causes of warm AIHA

A

autoimmune disease: e.g. systemic lupus erythematosus*

neoplasia: e.g. lymphoma, CLL
drugs: e.g. methyldopa

36
Q

Causes of cold AIHA

A

Causes of cold AIHA

neoplasia: e.g. lymphoma
infections: e.g. mycoplasma, EBV

37
Q

Indications

A
  • 1) Background a lymphoma (risk factor for cold AIHA)
  • 2) Raynaud’s phenomenon
  • 3) Symptoms worse in the cold
  • 4) New macrocytic anaemia, the macrocytosis here is occurring due to reticulocytosis (new immature RBCs which are larger) to compensate for the haemolysis.
38
Q

Which temperature does cold AIHA work at

A

<4 hours- IgM mediated haemolysis occurs

39
Q

G6PD deficiency

A

Male, African and Mediterranean descent
Neonatal jaundice
Infection/drugs precipitate haemolysis
Gallstones

Heinz bodies on the blood film
Dx- measure enzyme activity of G6PD

40
Q

Hereditary spherocytosis features

A 
Male and female- autosomal dominant 
Northern European descent 
Neonatal jaundice
Chronic symptoms although haemolytic crises may be precipitated by infection 
Gallstones
Splenomegaly is common

Spherocytes- round, lack of central pallor
EMA binding

41
Q

What is Richter’s transformation?

A

Leukaemia cells enter the lymph node and change into a high-grade, fast-growing non-Hodgkin’s lymphoma

Lymphnode swelling
Fever without infection 
Weight loss 
Night sweats 
Nausea
Abdominal pain
42
Q

Route of administration of transexamic acid

A

Given as IV bolus slowly

43
Q

MOA of transexamic acid

A

Synthetic derivative of lysine. Primary mode of action is an antifibrinolytic that reversibly binds to lysine receptor sites on plasminogen or plasmin.

Transexamic acid - most commonly prescribed to help treat menorrhagia

44
Q

Treatment of antiphospholipid syndrome in pregnancy

A

Aspirin and LMWH - enoxaparin

AVOID WARFARIN in pregnancy

45
Q

Which factors are affected with the administration of Heparin

A

Prevents activation of factors 2,9,10,11

46
Q

Warfarin- which factors does it affect

A

Affects synthesis of factors 2,7,9,10

47
Q

Which factors does DIC affect

A

Factors 1,2,5,8,11

48
Q

Liver disease clotting factors affected

A

Factors 1,2,5,7,9,10,11

49
Q

What is the beta- thalassemia trait?

A

It is an autosomal recessive condition characterised by a mild hypochromic, microcytic anaemia- usually asymptomatic

Features:- mild hypochromic, microcytic anaemia- microcytosis is characteristically disproportionate to the anaemia
HbA2 raised

50
Q

Infective causes of lympadenopathy

A 
infectious mononucleosis
HIV, including seroconversion illness
eczema with secondary infection
rubella
toxoplasmosis
CMV
tuberculosis
roseola infantum
51
Q

Neoplastic causes of lymphadenopathy

A 
infectious mononucleosis
HIV, including seroconversion illness
eczema with secondary infection
rubella
toxoplasmosis
CMV
tuberculosis
roseola infantum
52
Q

Other causes of generalised lymphadenopathy aside from neoplastic and infective

A

autoimmune conditions: SLE, rheumatoid arthritis
graft versus host disease
sarcoidosis
drugs: phenytoin and to a lesser extent allopurinol, isoniazid

53
Q

Contraindications to platelet transfusion

A

Chronic bone marrow failure
Autoimmune thrombocytopenia
Heparin-induced thrombocytopenia, or
Thrombotic thrombocytopenic purpura.

54
Q

Isolated thrombocytopenia on blood test leads to diagnosis of what in a middle aged woman with frequent menorrhagia and frequent nose-bleed

A

Idiopathic (immune) mediated thrombocytopenia

Antibodies are directed against the glycoprotein IIb/IIIa or Ib-V-IX complex.

55
Q

ITP presentation

A

may be detected incidentally following routine bloods
symptomatic patients may present with
petichae, purpura
bleeding (e.g. epistaxis)
catastrophic bleeding (e.g. intracranial) is not a common presentation

56
Q

Management of IP

A

first-line treatment for ITP is oral prednisolone
pooled normal human immunoglobulin (IVIG) may also be used
splenectomy is now less commonly used

57
Q

Components of cryoprecipitate

A

Factor VII, Fibrinogen, von Willebrand factor, Factor XIII

58
Q

Which combination of platelets and Fibrin degradation products make DIC most likely

A

Low platelets and raised FDP

59
Q

Drugs thought to cause haemolysis

A

anti-malarials: primaquine
ciprofloxacin
sulph- group drugs: sulphonamides, sulphasalazine, sulfonylureas

Haematology (3 decks)

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