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MRCP 1 > Haematology > Flashcards

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
pathology of polycythemia rubra vera | what is the mutation?
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
classical symptoms of thrombotic thrombocytopenic purpura
``` pentad: fever renal failure thrombocytopenia neurological symptoms (due to emboli) microangiopathic haemolytic anaemia (anaemia, jaundice, schistocytes on blood film) ```
27
pathology of thrombotic thrombocytopenic purpura
idiopathic or secondary (ca, preg, meds, HIV) inhibition of ADAMTS13 enzyme which breaks down vWF multimers --> increased platelet aggregation and thrombi
28
What are the biochemical derrangements in Antiphospholipid syndrome?
paradoxical rise in APTT | low platelets
29
inheritance of haemophilias?
x-linked recessive
30
What is the pathology of haemophilia A
deficiency in factor VIII (8) --> increased bleeding (prolonged APTT, normal PT)
31
what is the pathology of haemophilia B
deficiency in factor IX (9) --> increased bleeding (prolonged APTT, normal PT)
32
causes of warm autoimmune haemolytic anaemia features of WAIHA Mx
drugs: methyldopa neoplasm: lymphoma, CLL autoimmune dz: SLE ``` extravascular reaction (eg spleen), IgG mediated steroids, immunosuppression, splenectomy ```
33
causes of cold autoimmune haemolytic anaemia
neoplasia: CLL, NHL, Walenstrom's macroglobulinaemia infections: mycoplasma, EBV, CMV, varicella. intravascular, features similar to Raynaud's responds less well to steroids.
34
Treatment of polycythemia ruba Vera
venesection 1st line Hydroxycarbamide to decrease platelet count (JAK2 positive in 95%)
35
What is the pathology behind factor V Leiden mutation?
Increased factor V resistance to activated protein C -> hyper-coagulable state (DVT/PE)
36
Mx of thrombotic thrombocytopenic purpura
``` Plasma exchange (1st choice) Steroids, immunosuppressants, vincristine ```
37
Prognosis marker for myeloma
B2-microglobulin - increased levels = poor prognosis
38
types of Hodgkin's lymphoma: best prognosis worst prognosis most common
best prog: lymphocyte predominant (5%) worst prog: lymphocyte depleted (rare) most common: nodular sclerosing (70%) - associated with lacunar cells
39
Poor prognostic factors in CLL
``` male >70 fast lymphocyte doubling time raised LDH CD58 expression positive ```
40
factors associated with a poor prognosis in Hodgkin's lymphoma
male >45 years stage 4 disease Hb <15
41
name and define the 4 types of crisis seen in sickle cell
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
features of Paroxysmal Nocturnal haematuria | pathology
``` 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
Mx of paroxysmal nocturnal haematuria
replacement blood products anticoagulant (increased risk of VTE) ?stem cell transplantation
44
pathology of Hereditary Spherocytosis | inheritance?
most common hereditary haemolytic anaemia in causcasians | Auto Dom defect in RBC cytoskeleton ->RBC destruction by spleen
45
features of spherocytosis Dx Mx
FTT, jaundice, gallstones, splenomegaly, aplastic crisis secondary to parvovirus infection Dx with osmotic fragility test Mx: folate replacement, splenectomy
46
what is Burkitt's lymphoma | associated gene translocation and disease
high grade B cell lymphoma t(8:14) EBV
47
features of tumour lysis syndrome | Mx
``` 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
features of Waldenstrom's macroglobulinaemia
``` IgM paraproteinaemia weight loss hyperviscosity syndrome hepatosplenomegaly lymphadenopathy cryoglobulinaemia ```
49
thymoma associations
``` MG (30-40% of patients with thymoma) red cell aplasia dermatomyositis SLE SIADH ```
50
Multiple myeloma symptoms
rapidly increasing back pain which doesn't vary with time of day or movement. raised ESR, normal CRP renal failure normocytic anaemia
51
pathology of multiple myeloma
accumulation of malignant plasma cells, generally derived from 1 clone in the bone marrow. production of a monoclonal immunoglobulin
52
Dx of multiple myeloma | Rx
Bence Jones protein in urine. >10% plasma cells in bone marrow aspirate Rx: melphalan chemotherapy. Autologous transplant if <65
53
features of AML
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
Ix of ALL
positive Periodic acid-Schiff stain and non-specific esterase
55
Rx of cold auto-immune haemolytic anaemia
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
Coag changes seen in anti phospholipid syndrome
Prolonged APTT with normal PT Due to lupus antibody Causes pro thrombotic state
57
Lab test features of a factor 7 deficiency
Only factor that is unique to PT so will have prolonged PT with normal APTT. Autosomal recessive, rare
58
Features of iron overload Mx Causes
Grey skin, heart failure, diabetes Iron chelation - deferoxamine Secondary to chronic blood transfusions
59
Features of multiple myeloma
``` CRAB calcium (raised) renal failure anaemia (normocytic normochromic) bone pain (and lytic lesions) ```
60
features of monoclonal gammopathy of unknown significance (MGUS)
low paraprotein band IgG <2 asymptomatic and normal blood count 1% progress to myeloma (therefore MGUS is premalignant)
61
Indications for platelet transfusion
platelets <10 | pts with marrow suppression - chem/radiotherapy, marrow infiltration
62
contraindications for platelet transfusion
thrombotic thrombocytopenic purpura | microangiopathic anaemia, thrombocytopenia, fever, neuro symptoms, RF

MRCP 1 (15 decks)

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