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Z MD 2 > haematology > Flashcards

haematology Flashcards

1
Q

3 main groups of non-Hodgkins lymphoma

A

indolent lymphoma

aggressive lymphoma

very aggressive lymphoma

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2
Q

typical iron studies seen in iron deficiency anaemia

A

reduced serum ferritin

reduced serum iron

increased TIBC

reduced transferrin saturation

increased soluble transferrin receptor

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3
Q

3 stages of CML

A

chronic - generally asymptomatic

accelerated

blast

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4
Q

how do you diagnose monoclonal gammopathy of uncrtain significance

A

have a monoclonal immunoglobulin band (

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5
Q

monitoring and target of warfarin

A

INR (2.5-3.5)

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6
Q

which “letters” in the staging system for lymphoma are prognostic indicators

A

B, E, X

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7
Q

key findings for polycythaemia

A

increased RBC

increased haematocrit

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8
Q

what do you see in a peripheral blood film of iron deficiency anaemia

A

hypochromic microcytic RBCs

target cells

pencil forms

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9
Q

principles of treatment of diffuse large B cell lymphoma

A

immunochemotherapy - treat them regardless of whether they have symptoms or not CURABLE

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10
Q

main constituent of cryoprecipitate

A

fibrinogen

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11
Q

what mouth signs do you use in severe Vitamin B12

A

Hunter’s glossitis

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12
Q

what is in the DIC screen and what will this and the FBE show if the patient has DIC

A

Screen - APTT, INR, fibrinogen, D-dimer

high APTT

high INR

low fibrinogen

high D-dimer

low platlets

red cell fragmentation

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13
Q

how do you test heparin activity

A

unfractionated heparin - APTT

LMWH - Xa inhibition

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14
Q

treatment of CML

A

imatinib

dasatinib

nolotinib

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15
Q

which enzyme on biochemistry is a poor prognostic sign for lymphoma

A

high lactate dehydrogenase

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16
Q

what causes the increased infections in those with symptomatic myeloma

A

the M protein causes a reduction in normal Igs –> immunodeficiency –> predisposed to infection

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17
Q

iron studies in thalassemia

A
  • normal serum iron
  • normal TIBC
  • normal serum ferritin
  • increased transferrin receptor
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18
Q

treatment of acute promyelocytic leukaemia

A

high dose vitamin A and arsenic (causes cells to mature fast)

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19
Q

explain the interpretatin of a correction study of an abnormal APTT or PT

A

if factor deficiency = APTT/PT will normalize

if ‘inhibitor’ present = persistent abnormality

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20
Q

what are the end organ damage outcomes to diagnose symptomatic multiple myeloma

A

CRAB

  • Calcium = hypercalcaemia –> confusion, constipation, dehydration, coma
  • renal dysfunction/failure - from light chain deposition
  • anaemia - myeloma produces inflammatory cytokines (IL-6) –> anaemia of chronic disease
  • bone lesions/disease + hyperviscosity, amyloidosis and recurrent infections
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21
Q

acute adverse reactions to transfusion

A
  • allergy/anaphylaxia
  • nebrile non-haemolytic transfusion reactions
  • intravascular haemolysis
  • infection
  • transfusion related lung injury
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22
Q

key result on blood test suggesting polycythaemia rubra vera over secondary polycythaemia

A

EPO is low

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23
Q

6 causes of prolonged prothrombin time

A
  • warfarin (II, VII, IX, X)
  • liver disease
  • vitamin K def
  • DIC
  • factor VII def
  • rivaroxaban
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24
Q

commonest ways to present with myeloma

A

problems with bony disease

renal impairment

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25
Q

what are some indications for fresh frozen plasma

A

coagulopathy and bleeding

massive transfusion

bipass

liver disease

acute DIC

warfarin overdose

TTP

plasma exchange

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26
Q

treatment of Haemophilia A

A
  • purified or recombinant Factor VIII therapy
  • Tranexamic acid
  • Topical thrombin
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27
Q

what are the 2 most common lymphomas in the community

A

diffuse large B cell lymphoma

follicular lymphoma

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28
Q

treatment of DIC

A
  • treat underlying cause
  • give fresh frozen plasma (aim for APTT to be more than 1.5)
  • replace fibrinogen (give cryoprecipitate) aiming for a fibrinogen level over 1.5
  • give the patient platelets - aiming for above 50
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29
Q

what is “bulky” lymphoma

A

any lymph node mass that is more than 10cm in diameter or more than 1/3 of the diameter of the chest if the node is mediastinal

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30
Q

what is the staging system for lymphoma

A

Ann-Arbor staging system

  • by CT scan
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31
Q

which factor is also known as prothrombin

A

factor 2

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32
Q

what does onycholysis (distal nail separation) suggest

A

trauma

psoriasis

thyrotoxicosis

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33
Q

How do you differentiate between Hodgkins and non-Hodgkins lymphoma

A

Hodgkins lymphoma has Reed-Sternberg Cells - don’t express the CD20 antigen

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34
Q

CLL is the same disease as

A

small lymphocytic lymphoma

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35
Q

what are the lineage associated antigens on myeloid and lymphoid cells (when doing flow cytometry)

A

myeloid - myeloperoxidase

lymphoid - tdt

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36
Q

what is the normal number of plasma cells in the BM

A

less than 5%

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37
Q

3 groups of AML

A
  • AML with recurrent cytogenetic features
  • Secondary AML arising from MDS or arising from previous chemotherapy
  • AML not otherwise classified
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38
Q

What is the special thing about apixaban compared to the other NOACs

A

Not as metabolized through the kidney as the others

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39
Q

most common cause of persistent APTT abnormality after correction study

A

lupus anticoagulant

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40
Q

classification of micro/normo/macrocytic

A

micro MCV less than 80

normo MCV 80-100

macro MCV more than 100

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41
Q

what are the extra “letters” in the staging system for lymphoma and what do they mean

A

A = no B symptoms

B = B symptoms present

S = spleen involved

E = extranodal disease

X = bulky disease

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42
Q

What is DIC and what is the pathobiology

A

Disseminated intravascular coagulation

trigger activates coagulation cascade –> get little clots formed within BVs –> as the coagulation cascade continues –> depletion of the coagulation factors –> bleed

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43
Q

what is the time frame for going from smouldering to symptomatic myeloma

A

18 months to 3 years

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44
Q

how many drinks per week does it take for you to be at risk of macrocytic anaemia

A

more than 14

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45
Q

principles of treatment for Hodgkins lymphoma

A

one of the most curable lymphomas - use combination ABVD chemotherapy or Esculated beacopp

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46
Q

clinical manifestations of haemophilia A

A
  • haemarthrosis
  • subcutaneous and intramuscular haematomas
  • psoas and retroperitoneal haematomas
  • Traumatic bleeding
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47
Q

explain the role of tissue factor

A

initiates thrombin generation

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48
Q

key blood results in haemolytic anaemia

A

normocytic (usually - may be macro if % reticulocytes are high)

high LDH

high unconjugated bilirubin

low haptoglobin

high reticulocytes

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49
Q

explain the staging system for lymphoma

A

Stage 1 = one focal area

Stage 2 = 2 focal areas but both on the same side of the diaphragm

Stage 3 = focal disease on each side of the diaphragm

Stage 4 = systemic disease

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50
Q

what is the difference between smouldering and symptomatic multiple myeloma

A

symptomatic multiple myeloma has evidence of end organ damage, while smouldering does not

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51
Q

what is the only indolent lymphoma that you can cure

A

stage 1 follicular lymphoma - treat by radiation

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52
Q

iron studiesin anaemia of chronic disease

A
  • decreased serum iron
  • decreased TIBC
  • decreased transferrin saturation
  • normal/increased serum ferritin
  • normal soluble transferrin receptor
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53
Q

what is the commonest leukaemia

A

CLL

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54
Q

difference between the actions of unfractionated heparin compared to LMW heparin

A

unfractionated = inactivates Xa and IIa in approximately 1:1 efficiency

LMW = inactivates Xa and IIa in approximately 5:1 ratio

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55
Q

what causes polycythaemia rubra vera

A

mutationn in JAK2 leading to abnormality in RBC progenitors

56
Q

Signs and Sx of polycythaemia rubra vera

A

hyperviscosity

hypertension

splenomegaly

57
Q

5 major causes of macrocytic anaemia

A

ABCDEF

Alcohol

B12

compensatory reticulocytosis (myelodysplasia)

drugs

endocrine - hypothyroidism

Folate deficiency

58
Q

what are the new anticoagulants (NOACs) prescribed for in general

A
  • prevention of stroke/embolism in AF
  • apixiban also used for VTE prevention
  • rivaroxaban also used for VTE treatment
59
Q

when should you suspect a patient has myeloma

A
  • patient presents with a fracture or bone pain without any precipitating event and XR shows lytic lesions, severe OP or crush fractures
  • unexplained anaemia and high ESR
  • high total protein despite a normal or low albumin
  • unexplained hypercalcaemia
  • unexplained renal failure
60
Q

what is the normal amount of blasts in the BM

A

less than 5%

61
Q

what does nail thickening suggest

A

psoriasis

fungal infection

62
Q

what are the stages of differentiation of myeloma

A

monoclonal gammopathy of uncertain significance –> smouldering multiple myeloma –> symptomatic multiple myeloma

63
Q

what causes leukostasis in acute leukaemia

A

so many blasts in the blood –> blood very thick

64
Q

what is the definition of acute leukaemia

A

more than 20% blasts in the bone marroq

65
Q

characteristics of acute leukaemia

A

blasts

acute time frame

die quickly

generally curable

lots of B symptoms

66
Q

What are the 5 causes of microcytic hypochromic anaemia and what are the main 3

A

TAILS

Thalassaemia

Anaemia of chronic disease

Iron deficiency - reduced iron stores

Lead poisoning

Sideroblastic anaemia

67
Q

what is pseduo-von Willebrand disease

A

mutation in platelet integrin GPIb so that it has decreased binding with vWF

68
Q

major management of B-major thalassaemia

A

blood transfusion

iron chelation therapy

folic acid supplementation

splenomegaly

allogenic BMT

69
Q

what symptoms point to lymphoma

A

palpable non-tender, rubbery lymphadenopathy

dyspnoea and cough (with mediastinal mass)

PUO and night sweats

70
Q

what is an alloantibody

A

an antibody that develops in a patient’s plasma after exposure to red blood cell antigens that are not present on their own red blood cells

71
Q

how do you monitor response to therapy in CML

A

by PCR - the amount of the abnormal bcr-abl transcript in the peripheral blood each 3 months

72
Q

what are the medical emergencies associated with acute leukaemia

A

DIC

leukostasis

febrile neutropaenia

73
Q

what is myelofibrosis

A

normal bone marrow tissue is gradually replaced with fibrous scar like tissue causing progressive BM failure

74
Q

which enzyme breaks down a thrombus

A

plasmin

75
Q

what is febrile neutropaenia

A

fever of over 38 degrees in a presence of neutorphils less than 1

76
Q

what is the difference between cure and complete remission

A

cure = the disease goes away and never comes back for the rest of the life

complete remission = disappearance of the disease based on the most sensitive test we have

77
Q

principles of treatment of someone with very aggressive lymphoma

A

treat IMMEDIATELY

78
Q

which factors are involved in the intrinsic coagulation pathway

A

factors 12, 11, 9 –> 10

79
Q

presentation of leukostasis

A

white out of the lungs –> SOB

blurry vision

stroke

confusion

80
Q

treatment of leukostasis

A

plasma exchange, then give chemotherapy to bring WCC down very quickly

81
Q

functions of vWF

A
  • mediates platelet adhesion at site of injury
  • stabilizes factor VIII in circulation
82
Q

causes of NON-megaloblastic anaemia

A

alcohol

drug induced

liver disease

endocrine (hypo/hyperthyroidism)

pregnancy

83
Q

what is Haemophilia B

A

X linked recessive disorder that causes factor IX deficiency (less common than A)

84
Q

basic principles of treatment of acute leukaemia

A

chemotherapy!

85
Q

what do you do if you get an abnormal APTT or PTT

A

go onto do a correction study/mixing study - mix half of the patients serum with half of a control and perform the APTT / PT again

86
Q

common causes of secondary polycythaemia

A

Smoking

COPD

PAH

OSA

R-L shunt

EPO producing tumour

87
Q

iron studies in iron deficiency anaemia

A
  • reduced serum iron
  • reduced transferrin saturation
  • increased TIBC

- decreased serum ferritin

  • increased soluble transferrin receptor
88
Q

when is the platelet numer low enough to have a risk of spontaneous bleeding

A

10-20 x 10^9

89
Q

what is contained in fresh frozen plasma

A

mostly coagulation factors

90
Q

what causes tumour lysis syndrome

A

when large numbers of neoplastic cells are killed rapidly, leading to the release of IC ions and metabolic byproducts into the systemic circulation –> hyperuricaemia, hyperkalaemia, hyperphosphataemia, hypocalcaemia and acute renal failure

91
Q

why do you get hypercalcaemia with multiple myeloma

A

the myeloma produces cytokines that bind to RANKL –> activating OC –> Ca release into serum

92
Q

presentation of someone with diffuse large B cell lymphoma

A

lymphadenopathy and B symptoms

93
Q

typical characteristic look of cells in acute promyelocytic leukaemia

A

have LOTS of Auer Rods

94
Q

common symptoms of acute leukaemia

A

anaemia –> tachycardia, pallor, tachypnoeic, SOB, fatigue

bleeding, bruising, petichiae, thrombocytopaenia

infection - fever bone pain B symptoms

95
Q

3 ways to diagnose acute leukaemia

A

BM biopsy

confirmatory tests: flow cytometry and cytogentic molecular studies

96
Q

4 major causes of normocytic anaemia

A

ABCD

Acute blood loss

bone marrow failure

chronic disease

destruction (aplastic, autoimmune, haemolytic, Sickle cell)

97
Q

what other than enzymes are proteins are essential for the coagulation pathway

A

phospholipids

calcium

98
Q

causes of reticulocytosis with normocytic normochromic anaemia

A

haemolysis

blood loss

99
Q

what is myeloma

A

cancer of the plasma cells

100
Q

types of very aggressive lymphoma

A

Burkitts lymphoma

acute lymphoblastic lymphoma - like ALL

101
Q

types of aggressive lymphoma

A

diffuse large B cell lymphoma

diffuse large T cell lymphoma

102
Q

what are the medical emergencies of multiple myeloma

A

hypercalcaemia

spinal cord compression

renal failure

hyperviscosity

infections

103
Q

what types of bleeding do you get with thrombocytopaenia

A
  • skin and mucous membranes (petechiae, ecchymosis, haemorrhagic vesicles, gingival bleeding and epistaxis)
  • menorrhagia
  • GI bleeding
  • intracranial bleeding
104
Q

what are the screening tests for haemostatic defects

A

platelet count (150-400)

APTT (24-32 sec)

PTT/INR (10-12 seconds)

Thrombin time

105
Q

cause of CML

A

t(9;22) of BCR and ABL that causes constitutively activated tyrosine kinase –> cell growth

106
Q

what skin and nail signs do you see in someone with severe iron deficiency

A

dry skin

koilonychia

brittle hair and nails

hair loss

itching

paleness

107
Q

APTT and INR test which pathways

A

APTT = intrinsic (factors VIII, IX, XI, XII)

INR/PTT = extrinsic (factor VII)

108
Q

how can myeloma be found by urine sample

A

can see Bence Jones protein in the urine

109
Q

treatment options for VWB disease

A

desmopressin

Replacement therapy

Tranexamic acid (antifibrinolytics)

fibrin glue

110
Q

which factors are involved in the extrinsic coagulation pathway

A

factor 7 –> 10

111
Q

types of indolent lymphoma

A

follicular lymphoma

CLL/SLL

mantle cell lymphoma

marginal cell lymphoma

112
Q

1 unit of red cells increases the Hb by

A

10g/L

113
Q

what is the difference between a group and hold and a cross match

A

group and hold - match ABO and Rh

cross match - check alloantibodies

114
Q

indication to treat CLL/SLL

A

B symptoms

progressive lymphadenopathy

pancytopaenia

doubling white cell count in less than 6 months

115
Q

characteristics of chronic leukaemia

A

mature cells

occurs over years

die at end stage

generally not curable

B symptoms only at end stage

116
Q

what causes the acute renal failure in symptomatic myeloma

A

light chains get deposited in the kidneys increased infections hypercalcaemia

117
Q

name 5 acquired bleeding disorders

A
  • liver disease
  • Vit K deficiency
  • DIC
  • Excessive anticoagulation
  • idiopathic thrombocytopaenia
118
Q

what does the direct Coombs’ test do

A

tests for immune haemolysis

119
Q

what are the typical iron studies seen in anaemic of chronic disease

A

Fe low

TIBC low

% saturation normal

normal/increased ferritin

120
Q

typical peripheral blood film of macrocytic anaemia

A

hypersegmented neutrophils

macrocytic RBCs

121
Q

what does gingiva hypertrophy suggest

A

leukaemia

122
Q

what is induction thearpy

A

causes an acute reduction of the burden of disease

123
Q

7 causes of prolonged APTT

A
  • liver disease
  • heparin anticoagulation therapy/dabigatran
  • lupus anticoagulant
  • DIC
  • VWBd
  • Haemophilia
  • Factor XII, XI def
124
Q

what is important to remember about acute promyelocytic leukaemia?

A

extremely curable! but deadly if you dont act - die from DIC

125
Q

difference in treatment between “cold” and “warm” haemolytic anaemia

A

cold (C3) - extremely difficult to treat

warm (IgG) - treat like ITP (prednisolone, splenectomy, rituximab, IS)

126
Q

what is haemophilia A

A

X linked recessive disorder that causes factor VIII deficiency

127
Q

cause of corrected correction study with prolonged APTT and PT

A
  • isolated deficiency in common pathway: factors V, X, II and fibrinogen
  • multiple factor deficiences: liver disease, Vit K def, warfarin, DIC
128
Q

principles of treatment for myeloma

A

MGUS - monitor only

smouldering myeloma - observe only

symptomatic myeloma - treat it! - young - induction therapy + autologous SCT - between 65-75 - depends on how fit they are - over 75 - induction therapy only

129
Q

treatment of Haemophilia B

A
  • prothrombin complex
  • purified or recombinant factor IX
130
Q

action of dabigatran and Rivaroxaban

A

dabigatran - IIa antagonist

rivaroxaban - Xa antagonist

131
Q

broad causes of lymphadenopathy

A

infection

malignant disorder

autoimmune disease

storage disease

endocrine disease

132
Q

how do you diagnose multiple myeloma

A

BM shows >10% of plasma cells

133
Q

which particular drugs can cause macrocytic anaemia

A

antipurines

antipyrimidines

AZT

134
Q

what are B symptoms

A

weight loss (more than 10% of baseline weight in 6 month period)

temperature (more than 38)

drenching sweats

135
Q

typical look of blasts in AML and ALL

A

AML - large blasts with Auer rods

ALL - small blasts with no cytoplasmic granules

Z MD 2 (15 decks)

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