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Haematology Flashcards

1
Q

features of acute intermittent porphyria

A

rare AD condition due to defective biosynthesis of haem - causes toxic accumulation of porphobilinogen and delta aminolaevulinic acid

presents with abdo pain and neuropsychiatric sx

abdominal: abdominal pain, vomiting
neurological: motor neuropathy
psychiatric: e.g. depression
hypertension and tachycardia common

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2
Q

investigation findings in acute intermittent porphyria

A

urine turns deep red on standing

raised urinary porphobilinogen

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3
Q

management of acute intermittent porphyria

A

avoiding triggers
acute attacks
IV haematin/haem arginate
IV glucose should be used if haematin/haem arginate is not immediately available

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4
Q

features of acute myeloid leukaemia

A

most common acute leukaemia in adults
may arise secondary to myeloproliferative disorder

sx related to bone marrow failure
anaemia: pallor, lethargy, weakness
neutropenia: whilst white cell counts may be very high, functioning neutrophil levels may be low leading to frequent infections etc
thrombocytopenia: bleeding
splenomegaly
bone pain

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5
Q

managing antiphospholipid syndrome in pregnancy

A

low dose aspirin once pregnancy is confirmed

low molecular weight heparin once a fetal heart is seen on ultrasound. This is usually discontinued at 34 weeks

without management:
recurrent miscarriage
IUGR
pre-eclampsia
placental abruption
pre-term delivery
venous thromboembolism

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6
Q

features of aplastic anaemia

A

hypoplastic bone marrow due to drugs, toxins, infections, radiation, or idiopathic causes

normochromic, normocytic anaemia
leukopenia, with lymphocytes relatively spared
thrombocytopenia

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7
Q

overview of warm autoimmune haemolytic anaemia

A

the antibody (usually IgG) causes haemolysis at body temperature
haemolysis tends to occur in extravascular sites e.g., spleen

caused by: idiopathic, AI disease, lymphoma, CLL, drugs

treat with steroids

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8
Q

overview of cold autoimmune haemolytic anaemia

A

IgM causes haemolysis best at 4C, mediated by complement, usually intravascular haemolysis

Features may include symptoms of Raynaud’s and acrocynaosis

caused by neoplasia and infections, poor response to steroids

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9
Q

definition of beta thalassemia major

A

absence of b globulin chains, presents in the first year of life with failure to thrive and hepatosplenomegaly- HbA2 and HbF raised, HbA absent

managed with repeated transfusions -> iron overload, organ failure

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10
Q

features of beta thalassemia trait

A

autosomal recessive condition characterised by a mild hypochromic, microcytic anaemia - microcytosis usually disproportionate to anaemia
HbA2 may be raised

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11
Q

blood film of hyposplenic patient

A

target cells
Howell-Jolly bodies
Pappenheimer bodies
siderotic granules
acanthocytes

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12
Q

blood film in iron deficiency anaemia

A

target cells
‘pencil’ poikilocytes
if combined with B12/folate deficiency a ‘dimorphic’ film occurs with mixed microcytic and macrocytic cells

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13
Q

blood films in myelofibrosis

A

‘Tear-drop’ poikilocytes

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14
Q

features and management of post-transfusion non-haemolytic febrile reaction

A

due to antibodies reacting with white cell fragments in the blood product and cytokines that have leaked from the blood cell during storage

causes fever and chills

mx - slow/stop transfusion and give paracetamol

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15
Q

features and management of post-transfusion minor allergic reaction

A

due to foreign plasma proteins

causes pruritis, urticaria

stop transfusion and give antihistamine

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16
Q

features and management of post-transfusion anaphylaxis

A

due to patients with IgA deficiency with anti-IgA antibodies

causes hypotension, dyspnoea, wheezing, angioedema

stop transfusion, IM adrenaline ABC support

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17
Q

features and management of post-transfusion acute haemolytic reaction

A

due to ABO-incompatible blood

causes fever, abdominal pain, hypotension

stop transfusion, confirm dx, confirm pt, send blood for coombs and repeat typing and X matching, supportive care

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18
Q

features and management of post-transfusion transfusion-associated circulatory overload (TACO)

A

due to excessive rate of transfusion with pre-existing heart failure

causes pulmonary oedema, hypertension

slow/stop transfusion, consider IV loop diuretic e.g., furosemide with oxygen

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19
Q

features and management of post-transfusion transfusion related acute lung injury (TRALI)

A

non cardiogenic pulmonary oedema secondary to increased vascular permeability due to activated neutrophils

Hypoxia, pulmonary infiltrates on chest x-ray, fever, hypotension

stop transfusion, give oxygen and supportive care

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20
Q

indications for fresh frozen plasma

A

for ‘clinically significant’ but without ‘major haemorrhage’ in patients with a prothrombin time (PT) ratio or activated partial thromboplastin time (APTT) ratio > 1.5

prophylaxis for invasive surgery with significant bleeding risk

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21
Q

indications for using cryoprecipitate

A

small volume 15-20ml

contains concentrated Factor VIII:C, von Willebrand factor, fibrinogen, Factor XIII and fibronectin

treats ‘clinically significant’ but without ‘major haemorrhage’ who have a fibrinogen concentration < 1.5 g/L
disseminated intravascular coagulation, liver failure

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22
Q

indications for using prothrombin complex concentrate

A

emergency reversal of anticoagulation in patients with either severe bleeding or a head injury with suspected intracerebral haemorrhage

prophylaxis in patients with emergency surgery

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23
Q

red cell transfusions and indications

A

red blood cells should be stored at 4°C prior to infusion
in a non-urgent scenario, a unit of RBC is usually transfused over 90-120 minutes

threshold for ACS patients - 80g/L, without ACS 70g/L
aim to +20g/L

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24
Q

features of burkitt’s lymphoma

A

high-grade B-cell neoplasm affecting maxilla/mandible (endemic african form) or abdominal (sporadic, common form) - assx HIV

microscopy - ‘starry sky’ appearance: lymphocyte sheets interspersed with macrophages containing dead apoptotic tumour cells

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25
Q

treating burkitt’s lymphoma

A

chemotherapy, may cause tumour lysis syndrome (reduce chance with rasburicase)

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26
Q

complications of chronic lymphocytic leukaemia

A

anaemia
hypogammaglobulinaemia leading to recurrent infections
warm autoimmune haemolytic anaemia in 10-15% of patients
transformation to high-grade lymphoma (Richter’s transformation)

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27
Q

features of chronic lymphocytic leukaemia

A

often none: may be picked up by an incidental finding of lymphocytosis
constitutional: anorexia, weight loss
bleeding, infections
lymphadenopathy more marked than chronic myeloid leukaemia

FBC - lymphocytosis, anaemia, thrombocyopaenia
smudge cells / smear cells on blood film

treatment- imatinib

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28
Q

definitions of cryoglobulinaemias

A

Immunoglobulins which undergo reversible precipitation at 4 deg C, dissolve when warmed to 37 deg C. One-third of cases are idiopathic

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29
Q

features of cryoglobulinaemias

A

Raynaud’s only seen in type I
cutaneous
vascular purpura
distal ulceration
ulceration
arthralgia
renal involvement
diffuse glomerulonephritis

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30
Q

investigastions and management of cryoglobinaemia

A

low complement (esp. C4)
high ESR

treatment of underlying condition e.g. hepatitis C
immunosuppression
plasmapheresis

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31
Q

indications for using DOACs

A

prevention of stroke in non-valvular AF if:
prior stroke or transient ischaemic attack
age 75 years or older
hypertension
diabetes mellitus
heart failure

prevention of VTE following hip/knee surgery
treatment of DVT and PE

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32
Q

definition and causes of disseminated intravascular coagulation

A

coagulation and fibrinolysis are dysregulated causing widespread clotting with resultant bleeding

causes
sepsis
trauma
obstetric complications e.g. aminiotic fluid embolism or hemolysis, elevated liver function tests, and low platelets (HELLP syndrome)
malignancy

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33
Q

blood picture in disseminated intravascular coagulation

A

↓ platelets
↓ fibrinogen
↑ PT & APTT
↑ fibrinogen degradation products
schistocytes due to microangiopathic haemolytic anaemia

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34
Q

definition of factor v leiden

A

Factor V Leiden (activated protein C resistance) - most common inherited thrombophilia

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35
Q

definition and features of fanconi anaemia

A

rare autosomal recessive bone marrow syndrome

features
haematological:
aplastic anaemia
increased risk of acute myeloid leukaemia
neurological
skeletal abnormalities:
short stature
thumb/radius abnormalities
cafe au lait spots

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36
Q

features of G6PD deficiency

A

commonest red blood cell enzyme defect
X linked recessive inheritance

neonatal jaundice is often seen
intravascular haemolysis
gallstones are common
splenomegaly may be present
Heinz bodies on blood films. Bite and blister cells may also be seen

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37
Q

triggers of haemolysis in G6PD deficiency

A

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

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38
Q

definition of acute graft vs host disease

A

Is classically defined as onset is classically within 100 days of transplantation*

Usually affects the skin (>80%), liver (50%), and gastrointestinal tract (50%)

Multi-organ involvement carries a worse prognosis**

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39
Q

definition of chronic graft vs host disease

A

May occur following acute disease, or can arise de novo
Classically occurs after 100 days following transplantation
Has a more varied clinical picture: often lung and eye involvement in addition to skin and GI, although any organ system may be involved

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40
Q

pathophysiology of graft vs host disease

A

multi-system complication of allogeneic bone marrow transplantation

due to T cells in the donor tissue (the graft) mount an immune response toward recipient (host) cells

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41
Q

hereditary causes of haemolytic anaemia

A

membrane: hereditary spherocytosis/elliptocytosis
metabolism: G6PD deficiency
haemoglobinopathies: sickle cell, thalassaemia

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42
Q

acquired causes of haemolytic anaemia

A

Acquired: immune causes (Coombs-positive)
autoimmune: warm/cold antibody type
alloimmune: transfusion reaction, haemolytic disease newborn
drug: methyldopa, penicillin

Acquired: non-immune causes (Coombs-negative)
microangiopathic haemolytic anaemia (MAHA): TTP/HUS, DIC, malignancy, pre-eclampsia
prosthetic heart valves
paroxysmal nocturnal haemoglobinuria
infections: malaria
drug: dapsone

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43
Q

features of haemophilia

A

Haemophilia A - deficient factor VIII
haemophilia B - deficient factor IX

Features
haemoarthroses
haematomas
prolonged bleeding after surgery or trauma

Blood tests
prolonged APTT
bleeding time, thrombin time, prothrombin time normal

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44
Q

features of hereditary angioedema

A

autosomal dominant condition

Symptoms
attacks may be proceeded by painful macular rash
painless, non-pruritic swelling of subcutaneous/submucosal tissues
may affect upper airways, skin or abdominal organs (can occasionally present as abdominal pain due to visceral oedema)
urticaria is not usually a features

45
Q

features of hereditary spherocytosis

A

normal biconcave disc shape is replaced by a sphere-shaped red blood cell and destroyed by the spleen

causes failure to thrive
jaundice, gallstones
splenomegaly
aplastic crisis precipitated by parvovirus infection
degree of haemolysis variable
MCHC elevated

46
Q

management of hereditary spherocytosis

A

acute haemolytic crisis:
treatment is generally supportive
transfusion if necessary

longer term treatment:
folate replacement
splenectomy

47
Q

features of hodgkins lymphoma

A

malignant proliferation of lymphocytes with reed-sternberg cell

painless, nontender, asymmetrical lymphadenopathy - neck, axillary, inguinal
alcohol induced lymph node pain
B symptoms - poor prognosis
weight loss > 10% in last 6 months
fever > 38ºC
night sweats

48
Q

staging of hodgkins lymphoma

A

I: single lymph node
II: 2 or more lymph nodes/regions on the same side of the diaphragm
III: nodes on both sides of the diaphragm
IV: spread beyond lymph nodes

Each stage may be subdivided into A or B
A = no systemic symptoms other than pruritus
B = weight loss > 10% in last 6 months, fever > 38c, night sweats (poor prognosis)

49
Q

management of hodgkin’s lymphoma

A

chemotherapy combinations

ABVD - (doxorubicin, bleomycin, vinblastine, and dacarbazine)

radiotherapy

chemo then radio

hematopoietic cell transplantation for relapsed or refractory classic Hodgkin lymphoma

50
Q

causes of hyposplenism

A

splenectomy
sickle-cell
coeliac disease, dermatitis herpetiformis
Graves’ disease
systemic lupus erythematosus
amyloid

51
Q

definition and features of immune thrombocytopenia

A

immune-mediated reduction in the platelet count with antibodies directed against the glycoprotein IIb/IIIa or Ib-V-IX complex

may be detected incidentally on bloods - isolated thrombocytopaenia

symptomatic patients may present with
petechiae, purpura
bleeding (e.g. epistaxis)
catastrophic bleeding (e.g. intracranial) is not a common presentation

52
Q

treatment of immune thrombocytopenia

A

first-line treatment for ITP is oral prednisolone

pooled normal human immunoglobulin (IVIG) may also be used - raises platelet count quicker than steroids

splenectomy if platelets < 30 after 3 months of steroid therapy

53
Q

causes of iron deficiency anaemia

A

Excessive blood loss
Inadequate dietary intake
Poor intestinal absorption
Increased requirements

53
Q

investigation findings in iron deficiency anaemia

A

FBC - hypochromic microcytic anaemia

low serum ferritin (low iron stores)
high TIBC and high transferrin

Blood film anisopoikilocytosis (red blood cells of different sizes and shapes) , target cells, ‘pencil’ poikilocytes

Endoscopy to rule out malignancy - especially in older, new unexplained IDA

54
Q

blood results in anaemia of chronic disease

A

low serum iron

low TIBC

low transferrin saturation

high ferritin

55
Q

features of lead poisoning

A

consider in questions giving a combination of abdominal pain and neurological signs

Features
abdominal pain
peripheral neuropathy (mainly motor)
neuropsychiatric features
fatigue
constipation
blue lines on gum margin (only 20% of adult patients, very rare in children)

56
Q

investigation findings for lead poisoning

A

blood lead level >10mcg/dL

FBC - microcytic anaemia, basophilic stippling and clover-leaf morphology

raised serum and urine levels of delta aminolaevulinic acid

57
Q

managing lead poisoning

A

dimercaptosuccinic acid (DMSA)
D-penicillamine
EDTA
dimercaprol

58
Q

causes of generalised lymphadenopathy

A

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

Neoplastic
leukaemia
lymphoma

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

59
Q

lymphatic drainage of ovaries, uterus and cervix

A

The ovaries drain to the para-aortic lymphatics via the gonadal vessels

The body of the uterus drains through lymphatics contained within the broad ligament to the iliac lymph nodes.

The cervix drains into external iliac nodes, presacral nodes, and internal iliac nodes

60
Q

causes of macrocytic anaemia

A

Megaloblastic causes of macrocytic anaemia
vitamin B12 deficiency
folate deficiency
e.g. secondary to methotrexate

Normoblastic causes of macrocytic anaemia
alcohol
liver disease
hypothyroidism
pregnancy
reticulocytosis
myelodysplasia
drugs: cytotoxics

61
Q

indications for urgent FBC

A

Pallor
Persistent fatigue
Unexplained fever
Unexplained persistent infections
Generalised lymphadenopathy
Persistent or unexplained bone pain
Unexplained bruising
Unexplained bleeding

62
Q

features of methaemoglobinaemia

A

describes haemoglobin which has been oxidised from Fe2+ to Fe3+ - causes tissue hypoxia due to inability to bind oxygen, moves oxygen dissociation curve to left

Features
‘chocolate’ cyanosis
dyspnoea, anxiety, headache
severe: acidosis, arrhythmias, seizures, coma
normal pO2 but decreased oxygen saturation

63
Q

management of methaemoglobinaemia

A

NADH methaemoglobinaemia reductase deficiency: ascorbic acid
IV methylthioninium chloride (methylene blue) if acquired

64
Q

features of monoclonal gammopathy of undetermined significance

A

usually asymptomatic
no bone pain or increased risk of infections
around 10-30% of patients have a demyelinating neuropathy
normal immune function
lower and stable level of paraproteinaemia than myeloma
no clinical features of myeloma

65
Q

features of myelodysplastic syndrome

A

ineffective hematopoiesis leading to peripheral cytopenias despite a typically hypercellular bone marrow

fatigue, weakness, and pallor due to anaemia; recurrent infections due to neutropenia; and easy bruising or bleeding due to thrombocytopenia

66
Q

features of myelofibrosis

A

a myeloproliferative disorder caused by hyperplasia of abnormal megakaryocytes - resultant release of platelet derived growth factor is thought to stimulate fibroblasts
haematopoiesis develops in the liver and spleen

Features
e.g. elderly person with symptoms of anaemia e.g. fatigue (the most common presenting symptom)
massive splenomegaly
hypermetabolic symptoms: weight loss, night sweats etc

67
Q

features of myeloma

A

haematological malignancy characterised by plasma cell proliferation

CRABBI

hypercalcaemia - constipation, nausea, anorexia and confusion

renal - monoclonal production of Ig causes light chain deposition within the renal tubules - renal damage - dehydration, thirst

anaemia - bone marrow crowding suppresses erythropoiesis leading to anaemia

bones - bone marrow infiltration by plasma cells causes lytic bone lesions causing pain

bleeding - bone marrow crowding results in thrombocytopenia, increasing risk of bleeding and bruising

infection - a reduction in the production of normal immunoglobulins results in increased susceptibility to infection

68
Q

causes of neutropenia

A

viral
HIV
Epstein-Barr virus
hepatitis
drugs
cytotoxics
carbimazole
clozapine
myelodysplastic malignancies
aplastic anemia
rheumatological conditions
systemic lupus erythematosus

68
Q

definition of neutropenia

A

low neutrophil counts, < 1.5 * 109. A normal neutophil count is 2.0 - 7.5 * 109.

Mild 1.0 - 1.5 * 109
Moderate 0.5 - 1.0 * 109
Severe < 0.5 * 109

predisposes to severe infection

69
Q

features of non-hodgkin lymphoma

A

Painless lymphadenopathy (non-tender, rubbery, asymmetrical)
Constitutional/B symptoms occur later (fever, weight loss, night sweats, lethargy)
Extranodal Disease more common - gastric (dyspepsia, dysphagia, weight loss, abdominal pain), bone marrow (pancytopenia, bone pain), lungs, skin, central nervous system (nerve palsies)

differentiated from hodgkin lymphoma via biopsy

70
Q

investigating non-hodgkin lymphoma

A

Excisional node biopsy
CT chest, abdomen and pelvis (to assess staging)
HIV test - risk for NHL
FBC and blood film - normocytic anaemia, rule out other haem malignancies
ESR - prognostic indicator
LDH - marker of cell turnover

71
Q

management of non hodgkin lymphoma

A

watchful waiting, chemotherapy or radiotherapy - depends on sub type

Rituximab often used

flu/pneumococcal vaccine

72
Q

causes of normocytic anaemia

A

anaemia of chronic disease
chronic kidney disease
aplastic anaemia
haemolytic anaemia
acute blood loss

73
Q

features of paroxysmal nocturnal haemoglobinuria

A

acquired disorder leading to haemolysis due to increased sensitivity of cell membranes to complement - increased risk of VTE

Features
haemolytic anaemia
red blood cells, white blood cells, platelets or stem cells may be affected therefore pancytopaenia may be present
haemoglobinuria: classically dark-coloured urine in the morning (although has been shown to occur throughout the day)
thrombosis e.g. Budd-Chiari syndrome
aplastic anaemia may develop in some patients

74
Q

management of paroxysmal nocturnal haemoglobinuria

A

blood product replacement
anticoagulation
eculizumab, a monoclonal antibody directed against terminal protein C5, is currently being trialled and is showing promise in reducing intravascular haemolysis
stem cell transplantation

75
Q

platelet thresholds for platelet transfusion before invasive procedure

A

> 50×109/L for most patients
50-75×109/L if high risk of bleeding
100×109/L if surgery at critical site

76
Q

platelet thresholds for platelet transfusion in active bleeding

A

platelet count of <30 x 10 9 with clinically significant bleeding grade 2 e.g. haematemesis, melaena, prolonged epistaxis)

thresholds for transfusion are higher (maximum < 100 x 10 9) for patients with severe bleeding (WHO grades 3&4), or bleeding at critical sites, such as the CNS.

77
Q

contraindications for platelet transfusion

A

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

78
Q

types of polycythaemia

A

Relative causes
dehydration
stress: Gaisbock syndrome

Primary
polycythaemia rubra vera

Secondary causes
COPD
altitude
obstructive sleep apnoea
excessive erythropoietin: cerebellar haemangioma, hypernephroma, hepatoma, uterine fibroids*

79
Q

features of polycythaemia vera

A

myeloproliferative disorder caused by clonal proliferation of a marrow stem cell leading to an increase in red cell volume, and increased neutrophils and platelets
JAK2 mutation

pruritus, typically after a hot bath
splenomegaly
hypertension
hyperviscosity - arterial and venous thrombosis
haemorrhage (secondary to abnormal platelet function)
low ESR

80
Q

management of polycythaemia vera

A

aspirin
reduces the risk of thrombotic events

venesection
first-line treatment to keep the haemoglobin in the normal range

chemotherapy
hydroxyurea - slight increased risk of secondary leukaemia
phosphorus-32 therapy

81
Q

managing DVT and PE in pregnancy

A

S/C low-molecular weight heparin preferred to IV heparin (less bleeding and thrombocytopenia)
warfarin C/I

increased clotting due to increase in factors VII, VIII, X and fibrinogen, decrease in protein S, uterus presses on IVC causing venous stasis in legs

82
Q

features of primary immunodeficiency

A
83
Q

definition of sickle cell disease

A

autosomal recessive condition that results for synthesis of an abnormal haemoglobin chain termed HbS

sx arise at 4-6months

normal haemoglobin: HbAA
sickle cell trait: HbAS
homozygous sickle cell disease: HbSS

84
Q

management of sickle cell anaemia

A

Crisis management
analgesia e.g. opiates
rehydrate
oxygen
consider antibiotics if evidence of infection
blood transfusion
exchange transfusion: e.g. if neurological complications

Longer-term management
hydroxyurea - increases HbF

85
Q

types of sickle cell crisis

A

thrombotic, ‘vaso-occlusive’, ‘painful crises’
acute chest syndrome
anaemic - aplastic, sequestration
infection

86
Q

definition of sequestration crises

A

sickling within organs such as the spleen or lungs causes pooling of blood with worsening of the anaemia
associated with an increased reticulocyte count

87
Q

definition of aplastic crises

A

caused by infection with parvovirus
sudden fall in haemoglobin
bone marrow suppression causes a reduced reticulocyte count

88
Q

definition of acute chest syndrome

A

vaso-occlusion within the pulmonary microvasculature → infarction in the lung parenchyma
dyspnoea, chest pain, pulmonary infiltrates on chest x-ray, low pO2
management
pain relief
respiratory support e.g. oxygen therapy
antibiotics: infection may precipitate acute chest syndrome and the clinical findings (respiratory symptoms with pulmonary infiltrates) can be difficult to distinguish from pneumonia
transfusion: improves oxygenation

89
Q

causes and definition of sideroblastic anaemia

A

red cells fail to completely form haem - leads to red cells fail to completely form haem

Acquired causes
myelodysplasia
alcohol
lead
anti-TB medications

90
Q

investigation results for sideroblastic anaemia

A

full blood count
hypochromic microcytic anaemia (more so in congenital)

iron studies
high ferritin
high iron
high transferrin saturation

blood film
basophilic stippling of red blood cells

bone marrow
Prussian blue staining will show ringed sideroblasts

91
Q

causes of splenomegaly

A

myelofibrosis
chronic myeloid leukaemia
visceral leishmaniasis (kala-azar)
malaria
Gaucher’s syndrome
portal hypertension e.g. secondary to cirrhosis
lymphoproliferative disease e.g. CLL, Hodgkin’s
haemolytic anaemia
infection: hepatitis, glandular fever

92
Q

causes of thrombocytopenia

A

heparin induced thrombocytopenia (HIT)
drug-induced (e.g. quinine, diuretics, sulphonamides, aspirin, thiazides)
alcohol
liver disease
hypersplenism
viral infection (EBV, HIV, hepatitis)
pregnancy
SLE/antiphospholipid syndrome
vitamin B12 deficiency

93
Q

causes of thrombocytosis

A

reactive: platelets are an acute phase reactant - platelet count can increase in response to stress such as a severe infection, surgery. Iron deficiency anaemia can also cause a reactive thrombocytosis

malignancy

essential thrombocytosis (see below), or as part of another myeloproliferative disorder such as chronic myeloid leukaemia or polycythaemia rubra vera

hyposplenism

94
Q

features of essential thrombocytosis

A

myeloproliferative disorders which overlaps with chronic myeloid leukaemia, polycythaemia rubra vera and myelofibrosis. Megakaryocyte proliferation results in an overproduction of platelets.

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

95
Q

treating essential thrombocytosis

A

hydroxyurea (hydroxycarbamide) is widely used to reduce the platelet count
interferon-α is also used in younger patients
low-dose aspirin may be used to reduce the thrombotic risk

96
Q

features of thrombotic thrombocytopenic purpura

A

abnormally large and sticky multimers of von Willebrand’s factor cause platelets to clump within vessels

rare, typically adult females
fever
fluctuating neuro signs (microemboli)
microangiopathic haemolytic anaemia
thrombocytopenia
renal failure

97
Q

features in thymoma

A

Associated with
myasthenia gravis (30-40% of patients with thymoma)
red cell aplasia
dermatomyositis
also : SLE, SIADH

multimers of von Willebrand’s factor cause platelets to clump within vessels

98
Q

cause of tumour lysis syndrome

A

occurs from the breakdown of the tumour cells and the subsequent release of chemicals from the cell. It leads to a high potassium and high phosphate level in the presence of a low calcium

suspect in any patient presenting with an acute kidney injury in the presence of a high phosphate and high uric acid level

99
Q

causes of vitamin b12 deficiency

A

pernicious anaemia: most common cause
post gastrectomy
vegan diet or a poor diet
disorders/surgery of terminal ileum (site of absorption)
Crohn’s: either diease activity or following ileocaecal resection
metformin (rare)

100
Q

prophylaxis of of tumour lysis syndrome

A

IV fluids
patients are higher risk should receive either allopurinol or rasburicase
increased serum creatinine (1.5 times upper limit of normal)

101
Q

features of vitamin b12 deficiency

A

macrocytic anaemia
sore tongue and mouth
neurological symptoms
the dorsal column is usually affected first (joint position, vibration) prior to distal paraesthesia
neuropsychiatric symptoms: e.g. mood disturbances

1 mg of IM hydroxocobalamin 3 times each week for 2 weeks, then once every 3 months

102
Q

features of von willebrand disease

A

most common inherited bleeding disorder, autosomal dominant

behaves like platelet disorder - epistaxis and menorrhagia

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

103
Q

types of von willebrand disease

A

type 1: partial reduction in vWF (80% of patients)
type 2*: abnormal form of vWF
type 3**: total lack of vWF (autosomal recessive)

104
Q

management of von willebrand disease

A

tranexamic acid for mild bleeding
desmopressin (DDAVP): raises levels of vWF by inducing release of vWF from Weibel-Palade bodies in endothelial cells
factor VIII concentrate

105
Q

features of waldenstrom’s macroglobulinaemia

A

uncommon condition seen in older men - lymphoplasmacytoid malignancy characterised by the secretion of a monoclonal IgM paraprotein

Features
systemic upset: weight loss, lethargy
hyperviscosity syndrome e.g. visual disturbance
hepatosplenomegaly
lymphadenopathy
cryoglobulinaemia e.g. Raynaud’s

106
Q

diagnosis and management of waldenstrom’s macroglobulinaemia

A

bone marrow biopsy - infiltration of the bone marrow with lymphoplasmacytoid lymphoma cells

rituximab

107
Q

features of wiskott aldrich syndrome

A

X linked recessive cause of primary immunodeficiency due to a combined B- and T-cell dysfunction

Features
recurrent bacterial infections (e.g. Chest)
eczema
thrombocytopaenia
low IgM levels

Finals (27 decks)

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