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MRCP 1 > Clinical Haematology > Flashcards

Clinical Haematology Flashcards

1
Q

What happens in CLL

A

Monoclonal proliferation of well differentiated lymphocytes (B cells in 99%)

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2
Q

Features of CLL

A

Anorexia, weight loss
Infections, bleeding
Lymphadenopathy (more marked than CML)

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3
Q

Complications of CLL

A

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

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4
Q

What is Meig’s syndrome

A

Ovarian fibroma with pleural effusion and ascites

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5
Q

Von Willibrand disease inheritance

A

Autosomal dominant in 80%

Most common inherited bleeding disorder

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6
Q 
Tumour antigens - association with cancers
PSA
alpha-feto protein
CEA (carcinoembryonic antigen)
S-100
Bombesin
A 
Prostate cancer
Hepatocellular carcinoma, teratoma
Colorectal carcinoma
Melanoma, schwannoma
Small cell lung carcinoma, gastric cancer, neuroblastoma
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7
Q

Features of Von Willibrand disease

A

Epistaxis and menorrhagia - behaves like a platelet disorder
haemoarthroses and muscle haematomas are rare

Most common inherited bleeding disorder

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8
Q

Role of Von Willebrand factor

A

Promotes platelet aggregation to damaged epithelium

Carrier molecule for factor VIII

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9
Q

Ix for Von Willebrand disease and results

A

APTT - prolonged
Moderately reduced factor VIII levels
Defective platelet aggregation with ristocetin

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10
Q

Drugs cleared by haemodialysis

A 
BLAST
barbiturates
Lithium
Alcohol (inc methanol)
Salicylates
Theophyllines
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11
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

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12
Q

When do you monitor LFTs for statins and at what level do you stop them?

A

Baseline, 3months and 1 yr

Stop treatment if ALT rises and persists >3x upper limit of normal

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13
Q

Who should receive a statin?

A

Pts with established CV disease (IHD, stroke, PVD, TIA)
10 yr CV risk >20%
T2DM pts with other CV RISK

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14
Q

What is the Philadelphia chromosome

what is it’s result

A

translocation 9-22
in >95% of patients with chronic myeloid leukaemia (CML)
codes for a protein with increased tyrosine kinase activity

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15
Q

presentation of chronic myeloid leukaemia

A

aged 40-50
anaemia, weight loss, abdominal pain
splenomegaly may be marked

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16
Q

Mx of chronic myeloid leukaemia

A

imatinib 1st choice (tyrosine kinase inhibitor associated with BCR-ABL gene defect)
hydroxyurea
interferon-alpha
allogenic bone marrow transplant

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17
Q

tear drop poikilocytes on a blood film

A

myelofibrosis

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18
Q

causes of target cells (codocytes) on a blood film?

A

sickle cell (leads to autosplenectomy)
Hyposplenism (unable to remove damaged cells)
thalassaemia
iron def anaemia (decreasing haem content)
liver disease (defunct enzyme increasing cell surface membrane)

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19
Q

most common presenting features of myelofibrosis?

blood film findings?

A

lethargy

tear drop poikilocytes

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20
Q

Chronic lymphocytic leukaemia (CLL) Ix of choice

A

Blood film - smudge cells
Immunophenotyping - B cells (CD19 positive)
CD5 and CD23 normally positive in CLL

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21
Q

what is a Howell-Jolly body on a blood film?

what are the causes?

A

RBCs with remaining fragments of DNA, usually removed by spleen.
hyposplenism, asplenia, autosplenism from Sickle cell

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22
Q

what is the enzyme affected in Acute Intermittent Porphyria (AIP)?
Sx AIP

A

auto dom defective porphobilinogen deAminase
abdominal pain, neuropsychiatric Sx, hypertension and tachycardia
urine turns purple on prolonged exposure to sunlight

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23
Q

which enzyme is affected in porphyria cutanea tarda (PCT)

A

uroporphyrinogen deCarboxylase

photosensitive rash with bullae

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24
Q

features of Wiskott-Aldrich syndrome

inheritance

A 
primary immunodeficiency B and T cells
recurrent bacterial infections
eczema
thrombocytopenia
x linked recessive - WASP gene
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25
Q

pathology of polycythemia rubra vera

what is the mutation?

A

myeloproliferative disorder of bone marrow stem cells –> increased RBC, neutrophils and platelets
JAK2 mutation is seen in 95% of pts

5-15% progress to myelofibrosis and AML

26
Q

classical symptoms of thrombotic thrombocytopenic purpura

A 
pentad:
fever
renal failure
thrombocytopenia
neurological symptoms (due to emboli)
microangiopathic haemolytic anaemia (anaemia, jaundice, schistocytes on blood film)
27
Q

pathology of thrombotic thrombocytopenic purpura

A

idiopathic or secondary (ca, preg, meds, HIV) inhibition of ADAMTS13 enzyme which breaks down vWF multimers –> increased platelet aggregation and thrombi

28
Q

What are the biochemical derrangements in Antiphospholipid syndrome?

A

paradoxical rise in APTT

low platelets

29
Q

inheritance of haemophilias?

A

x-linked recessive

30
Q

What is the pathology of haemophilia A

A

deficiency in factor VIII (8) –> increased bleeding (prolonged APTT, normal PT)

31
Q

what is the pathology of haemophilia B

A

deficiency in factor IX (9) –> increased bleeding (prolonged APTT, normal PT)

32
Q

causes of warm autoimmune haemolytic anaemia
features of WAIHA
Mx

A

drugs: methyldopa
neoplasm: lymphoma, CLL
autoimmune dz: SLE

extravascular reaction (eg spleen), IgG mediated
steroids, immunosuppression, splenectomy
33
Q

causes of cold autoimmune haemolytic anaemia

A

neoplasia: CLL, NHL, Walenstrom’s macroglobulinaemia
infections: mycoplasma, EBV, CMV, varicella.

intravascular, features similar to Raynaud’s
responds less well to steroids.

34
Q

Treatment of polycythemia ruba Vera

A

venesection 1st line
Hydroxycarbamide to decrease platelet count
(JAK2 positive in 95%)

35
Q

What is the pathology behind factor V Leiden mutation?

A

Increased factor V resistance to activated protein C -> hyper-coagulable state (DVT/PE)

36
Q

Mx of thrombotic thrombocytopenic purpura

A 
Plasma exchange (1st choice)
Steroids, immunosuppressants, vincristine
37
Q

Prognosis marker for myeloma

A

B2-microglobulin - increased levels = poor prognosis

38
Q

types of Hodgkin’s lymphoma:
best prognosis
worst prognosis
most common

A

best prog: lymphocyte predominant (5%)
worst prog: lymphocyte depleted (rare)
most common: nodular sclerosing (70%) - associated with lacunar cells

39
Q

Poor prognostic factors in CLL

A 
male
>70
fast lymphocyte doubling time
raised LDH
CD58 expression positive
40
Q

factors associated with a poor prognosis in Hodgkin’s lymphoma

A

male
>45 years
stage 4 disease
Hb <15

41
Q

name and define the 4 types of crisis seen in sickle cell

A

thrombosis crisis - painful, precipitated by infection/deoxygenation/dehydration
sequestration - sickling in organs -> pooling of blood
aplastic - secondary to parvovirus infection. Sudden fall in Hb
Haemolytic - rare. Hb drop due to haemolysis

42
Q

features of Paroxysmal Nocturnal haematuria

pathology

A 
haemolytic anaemia
any/all blood lines may be affected 
haemoglobinuria - classically dark morning urine
Thrombosis eg Budd-Chiari
Aplastic anaemia may develop
low CD55 and 59
43
Q

Mx of paroxysmal nocturnal haematuria

A

replacement blood products
anticoagulant (increased risk of VTE)
?stem cell transplantation

44
Q

pathology of Hereditary Spherocytosis

inheritance?

A

most common hereditary haemolytic anaemia in causcasians

Auto Dom defect in RBC cytoskeleton ->RBC destruction by spleen

45
Q

features of spherocytosis
Dx
Mx

A

FTT, jaundice, gallstones, splenomegaly,
aplastic crisis secondary to parvovirus infection
Dx with osmotic fragility test
Mx: folate replacement, splenectomy

46
Q

what is Burkitt’s lymphoma

associated gene translocation and disease

A

high grade B cell lymphoma
t(8:14)
EBV

47
Q

features of tumour lysis syndrome

Mx

A 
increased K, Phos, Urate
low calcium, 
ARF
Rasburicase (urate oxidase recombinant) given prior to chemo. converts uric acid to allantoin, better renally excreted.
High risk in Burkitt's lymphoma
48
Q

features of Waldenstrom’s macroglobulinaemia

A 
IgM paraproteinaemia
weight loss
hyperviscosity syndrome
hepatosplenomegaly
lymphadenopathy
cryoglobulinaemia
49
Q

thymoma associations

A 
MG (30-40% of patients with thymoma)
red cell aplasia
dermatomyositis
SLE
SIADH
50
Q

Multiple myeloma symptoms

A

rapidly increasing back pain which doesn’t vary with time of day or movement.
raised ESR, normal CRP
renal failure
normocytic anaemia

51
Q

pathology of multiple myeloma

A

accumulation of malignant plasma cells, generally derived from 1 clone in the bone marrow.
production of a monoclonal immunoglobulin

52
Q

Dx of multiple myeloma

Rx

A

Bence Jones protein in urine.
>10% plasma cells in bone marrow aspirate
Rx: melphalan chemotherapy. Autologous transplant if <65

53
Q

features of AML

A

hyperviscosity symptoms (headache, fits, confusion, focal neurology, coma)
gum hypertrophy (deposits of leukaemic cells)
Auer rods present in bone marrow
positive stain for Sudan Black and myeloperoxidase

54
Q

Ix of ALL

A

positive Periodic acid-Schiff stain and non-specific esterase

55
Q

Rx of cold auto-immune haemolytic anaemia

A

keep warm
folic acid supplements (for all haemolytic anaemias)
avoid FFP (well thawed!)
chlorambucil and rituximab (anti CD20) - removes B cells producing the IgG

56
Q

Coag changes seen in anti phospholipid syndrome

A

Prolonged APTT with normal PT
Due to lupus antibody
Causes pro thrombotic state

57
Q

Lab test features of a factor 7 deficiency

A

Only factor that is unique to PT so will have prolonged PT with normal APTT.
Autosomal recessive, rare

58
Q

Features of iron overload
Mx
Causes

A

Grey skin, heart failure, diabetes
Iron chelation - deferoxamine
Secondary to chronic blood transfusions

59
Q

Features of multiple myeloma

A 
CRAB
calcium (raised)
renal failure
anaemia (normocytic normochromic)
bone pain (and lytic lesions)
60
Q

features of monoclonal gammopathy of unknown significance (MGUS)

A

low paraprotein band IgG <2
asymptomatic and normal blood count
1% progress to myeloma (therefore MGUS is premalignant)

61
Q

Indications for platelet transfusion

A

platelets <10

pts with marrow suppression - chem/radiotherapy, marrow infiltration

62
Q

contraindications for platelet transfusion

A

thrombotic thrombocytopenic purpura

microangiopathic anaemia, thrombocytopenia, fever, neuro symptoms, RF

MRCP 1 (15 decks)

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