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Blood disorders Flashcards

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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

Study These Flashcards
A

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

25
Management of Von Willebrand's disease
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
Why are irradiated blood products used
To avoid transfusion-associated graft versus host disease
27
Classical description of Reed-Sternberg cells
A blood film shows large cells with a bilobed nucleus and prominent eosinophilic inclusion-like nucleoli.
28
When is CMV negative packed red cells recommended
They are recommended for pregnant and neonatal transfusion
29
FFP use ?
Clotting studies
30
Features of lead poisoning
``` Abdominal pain Peripheral neuropathy Fatigue Constipation Blue lines on gum margin (only 20% of adults- very rare in children) ```
31
Investigations
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
Management - various chelating agents are currently used
dimercaptosuccinic acid (DMSA) D-penicillamine EDTA dimercaprol
33
Autoimmune haemolytic anaemia classification
warm and cold
34
AIHA Cooms test result
Positive result
35
Causes of warm AIHA
autoimmune disease: e.g. systemic lupus erythematosus* neoplasia: e.g. lymphoma, CLL drugs: e.g. methyldopa
36
Causes of cold AIHA
Causes of cold AIHA neoplasia: e.g. lymphoma infections: e.g. mycoplasma, EBV
37
Indications
- 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
Which temperature does cold AIHA work at
<4 hours- IgM mediated haemolysis occurs
39
G6PD deficiency
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
Hereditary spherocytosis features
``` 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
What is Richter's transformation?
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
Route of administration of transexamic acid
Given as IV bolus slowly
43
MOA of transexamic acid
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
Treatment of antiphospholipid syndrome in pregnancy
Aspirin and LMWH - enoxaparin | AVOID WARFARIN in pregnancy
45
Which factors are affected with the administration of Heparin
Prevents activation of factors 2,9,10,11
46
Warfarin- which factors does it affect
Affects synthesis of factors 2,7,9,10
47
Which factors does DIC affect
Factors 1,2,5,8,11
48
Liver disease clotting factors affected
Factors 1,2,5,7,9,10,11
49
What is the beta- thalassemia trait?
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
Infective causes of lympadenopathy
``` infectious mononucleosis HIV, including seroconversion illness eczema with secondary infection rubella toxoplasmosis CMV tuberculosis roseola infantum ```
51
Neoplastic causes of lymphadenopathy
``` infectious mononucleosis HIV, including seroconversion illness eczema with secondary infection rubella toxoplasmosis CMV tuberculosis roseola infantum ```
52
Other causes of generalised lymphadenopathy aside from neoplastic and infective
autoimmune conditions: SLE, rheumatoid arthritis graft versus host disease sarcoidosis drugs: phenytoin and to a lesser extent allopurinol, isoniazid
53
Contraindications to platelet transfusion
Chronic bone marrow failure Autoimmune thrombocytopenia Heparin-induced thrombocytopenia, or Thrombotic thrombocytopenic purpura.
54
Isolated thrombocytopenia on blood test leads to diagnosis of what in a middle aged woman with frequent menorrhagia and frequent nose-bleed
Idiopathic (immune) mediated thrombocytopenia | Antibodies are directed against the glycoprotein IIb/IIIa or Ib-V-IX complex.
55
ITP presentation
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
Management of IP
first-line treatment for ITP is oral prednisolone pooled normal human immunoglobulin (IVIG) may also be used splenectomy is now less commonly used
57
Components of cryoprecipitate
Factor VII, Fibrinogen, von Willebrand factor, Factor XIII
58
Which combination of platelets and Fibrin degradation products make DIC most likely
Low platelets and raised FDP
59
Drugs thought to cause haemolysis
anti-malarials: primaquine ciprofloxacin sulph- group drugs: sulphonamides, sulphasalazine, sulfonylureas

Haematology (3 decks)

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