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Medical Finals 2020 > Haematology > Flashcards

Haematology Flashcards

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USMLE® Step 1 flashcards
1
Q

What is haematopoeisis?

A

The formation of different types of blood cells

Too much (hyperfunction) can result in splenomegaly

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2
Q

What are B symptoms?

In what do they occur?

A
  • Fever
  • Night sweats
  • Weight loss >10% body weight

Occurs in lymphoma and some leukaemias

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3
Q

How can you measure heparin and what reversal agent may be used?

A

APTT - raised (intrinsic pathway)

Protamine can reverse it

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4
Q

Give some causes of an elevated APTT?

A
  • Heparin
  • Haemophilia
  • VWD
  • Antiphospholipid syndrome
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5
Q

What part of the coagulation cascade does heparin act on?

A

Intrinsic pathway - can monitor it by measuring APTT

Heparin activate antithrombin, which activates thrombin and Xa. Unfractionated heparin has a short half-life.
(LMWH is subcut, can monitor through anti Xa level)

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6
Q

What are the four different types of leukaemia?

A
  • Acute myeloid leukaemia
  • Acute lymphoblastic leukaemia
  • Chronic myeloid leukaemia
  • Chronic lymphoblastic leukaemia
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7
Q

What is the most common type of leukaemia in adults and children respectively?

A

Adults - CLL

Children - ALL

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8
Q

Which leukaemia is associated with the Philadelphia chromosome and what is the genetic fault?

A

CML
9;22 translocation

Can occur in ALL in which it indicates a poor prognosis

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9
Q

In the different types of leukaemia, are there any specific cells that are abnormal?

A

AML - auer rods and neutrophils on blood film, increased in blast cells
ALL - lymphocytes
CLL - lymphocytes

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10
Q

What is Richter transformation?

A

When CLL turns into non-Hodgkin’s Lymphoma

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11
Q

What is your drug of choice for CML?

A

Imatinib

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12
Q

What do auer rods indicate on blood film?

A

AML

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13
Q

What symptoms might someone with an acute leukaemia present with?

A
  • Malaise
  • Anorexia
  • Fever
  • Anaemia
  • Pallor
  • Lymphadenopathy
  • Neutropenia
  • Hepatosplenomegaly
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14
Q

What is a Blast Crisis and in what does it occur?

A

It occurs in CML
Is is an increase in lymphoblast cells in the blood or bone marrow resulting in a fever, bone pain, fatigue and severe anaemia

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15
Q

What investigations should you do to investigate a potential leukaemia?

A

Bloods: raised WCC, blood film with blast cells
CXR: mediastinal widening
Bone marrow aspirate

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16
Q

What are the 5 phases of chemotherapy treatment in leukaemias?

A
  • Induction
  • Consolidation and CNS protection
  • Interim maintenance
  • Delayed intensification
  • Continuing maintenance
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17
Q

What are some defining features of chronic lymphocytic leukaemia?

A
  • elderly
  • lymphocytosis (increased WCC)
  • smudge/smear cells on blood film
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18
Q

What are the two main types of thalassaemia?

A

Alpha - 4 types

Beta - 3 types

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19
Q

What are the 4 kinds of alpha thalassaemia?

A

Depends on how many alpha chains are normal.
4 normal = fine
3 normal = asymptomatic, borderline if they have any symptoms
2 normal = slightly anaemic
1 normal = HbH, very anaemic, microchromic, splenomegaly, bone changes
0 normal = HbBarts, incompatible with life, hydrops fetalis

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20
Q

How is thalassaemia treated and the side effects managed?

A
  • regular blood transfusions

- iron chelation (desferrioxamine)

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21
Q

What investigations can you do?

A

Hb electrophoresis

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22
Q

What are your INR targets for AF, VTE?

A

AF - 2.5
VTE 2.5 (3.5 if recurrent)
INR<2

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23
Q

How is warfarin reversed?

A

Stop warfarin
Vitamin K (takes 4-24hrs)
FFP
Human prothrombin complex (beriplex, takes 1hr)

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24
Q

What blood result gives your best indicator of warfarin other than INR?

A

PT - extrinsic pathway

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25
Q

What investigation do you do for hereditary spherocytosis and what do you see?

A

EMA binding assay

spherocytes

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26
Q

What is the name for too many and too few platelets?

A

Thrombocytosis - too many

Thrombocytopenia - too many

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27
Q

What are your management options for myeloma?

A
  • bone marrow transplant
  • bisphosphonates
  • blood transfusion
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28
Q

What investigations would be abnormal in myeloma? What other investigations would you want to do?

A
  • IgG, IgA - raised
  • serum electrophoresis + urine (Bence-Jones Protein)
  • proteinaemia
  • bone marrow biopsy
  • U&E check kidney function due to light chain depositions
  • calcium - raised in myeloma
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29
Q

What investigations are used to diagnose Beta-thalassaemia?

A

FBC - microcytic hypochromic anaemia
Haemoglobin electrophoresis
DNA testing

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30
Q

What is your management of beta thalassaemia major?

What is a problem with this?

A

Regular blood transfusions

Iron induced oxidative stress of liver, heart, brain, requires iron chelation therapy

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31
Q

EMA binding assay is for…?

A

Hereditary spherocytosis

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32
Q

What is the only thing the indirect Coombs test is used for?

A

Rhesus haemolytic disease of the newborn

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33
Q

How might a myeloma present?

A

Back pain
Bone pain, bone destruction
Haematuria, hypercalcaemia
Systemic - malaise, anaemia, fever, recurrent infections

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34
Q

What is a myeloma a malignancy of?

A

Bone marrow plasma cells

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35
Q

What is CRAB an acronym for and which condition is it applicable?

A

Calcium
Renal (creatinine raised)
Anaemia
Bone (lytic lesions)

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36
Q

What will you see on the investigations performed in a myeloma patient?

A

Raised calcium
Raised urea
Urine protein electrophoresis - immunoglobulin light chains (Bence-Jones protein)
24-hour urine immunofixation - paraproteinemia, raised IgG, IgA
Blood film - rouleaux formation
‘Pepperpot skull’ on CXR/PET

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37
Q

How are myelomas managed?

A

Thalidomide (NOT IN PREGNANCY)
Transplantation
Chemo + Radiotherapy

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38
Q

What supportive treatment is given in myelomas?

A

Bisphosphonates
Blood transfusions for anaemia
Prophylactic antibiotics

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39
Q

What are the three types of beta thalassaemia?

A

Minor - asymptomatic carriers, the trait is present
Intermedia - less severe anaemia
Major (Cooley’s anaemia) transfusion dependent

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40
Q

In thalassaemia patients on regular transfusions what is a major complication and a way to combat this?

A

Iron deposition and overload

Treat with chelation (desferrioxamine)

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41
Q

What are some inherited and acquired causes of thrombophilia?

A

Inherited: Factor V Leiden
Antithrombin deficiency

Acquired: antiphospholipid syndrome, cancer, Behcet’s disease, liver disease or nehrotic syndrome

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42
Q

What are some risk factors for Lymphoma?

A

Hodgkin’s Lymphoma - EBV

NHL - Toxins, Family History

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43
Q

What indicates a worse prognosis in Hodgkin’s Lymphoma?

A

Lymphocyte depleted

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44
Q

What is your main medical management of sickle cell disease?

A

Hydroxycarbamide

Definitive treatment is stem cell transplant

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45
Q

What are some potential complications of polycythaemia?

A

Gout

Thrombosis

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46
Q

What can cause polycythaemia?

A

Dehydration

COPD and sleep apnoea

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47
Q

How do you manage polycythaemia?

A

Venesection

Aspirin

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48
Q

What are the main causes of microcytic anaemia?

A

Iron deficiency

Chronic disease

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49
Q

What are the main causes of normocytic anaemia?

A

Blood loss
Chronic disease
Malignancy

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50
Q

What are the main causes of macrocytic anaemia?

A

B12 & Folate deficiency
Alcohol
Hypothyroidism

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51
Q

What is the genetic issue in haemophilia?

A

A - factor VIII deficiency

B - factor IX deficiency

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52
Q

How is G6PD inherited?

A

X-linked inheritance

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53
Q

How is von willebrands disease inherited?

A

Autosomal dominant

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54
Q

How do you treat Von Willebrand’s disease?

A

Transexamic acid
Desmopressin

AVOID aspirin and NSAIDs due to bleeding risk

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55
Q

What can trigger discovery of G6PD?

A

Antimalarials

Infections

56
Q

What should be done in patients with a raised INR/PT on warfarin?

A

Not bleeding – withhold warfarin until INR <5
Bleeding – medical emergency – seek senior help, discuss with haematologist, vitamin K 5mg STAT IV, consider prothrombin complex concentrate (Beriplex), withhold warfarin until INR <5.

57
Q

How might haemophilia present?

A

Haemarthrosis – bleeding joints

Usually in childhood

58
Q

What are some causes of increased INR/PT? (increased bleeding risk)

A

Overcoagulation with warfarin
DIC
Acute liver failure

59
Q

A patient presents with shortness of breath, tachycardia and isolated anaemia on bloods - suggest some differentials?

What are some important questions to ask about?

A
  • PE
  • HF
  • COPD
  • Anaemia
  • Chest infection
  • Pulmonary fibrosis

Ask about: DH (methotrexate will cause anaemia), menorrhagia, bleeding (GI, stool)
Investigations: Fe deficiency, B12/Folate

60
Q

What is the difference between haematinics and a full iron profile?

A

Haematinics will only give ferretin which is an acute stage protein. A full iron profile will give you iron binding capacity etc.

61
Q

What are some differentials for splenomegaly?

A
  • malaria
  • CML (myeloproliferative disorder)
  • myelofibrosis
  • lymphoproliferative disorders - CLL, lymphoma
  • [sickle cell crisis only in sequestration, usually the spleen decreases in size]
  • haemolytic disorders - anaemia, hereditary spherocytosis
    EBV (infectious mononucleosis, glandular fever, monospot test)
  • HIV, CMV, endocarditis
  • RA/SLE
  • Liver disorders - portal hypertension
62
Q

What associated symptoms may occur in a patient presenting with splenomegaly?

A
  • weight loss
  • night sweats
  • travel history
  • fever
  • sore throats
  • FB
  • bruising
  • bleeding
  • myalgia
63
Q

What investigations would you want to do on someone with splenomegaly?

A
  • blood film
  • FBC
  • monospot test
  • CT abdomen
  • LFT
  • BBV screen
64
Q

Acquired haemophilia is a deficiency of what?

A

Factor VIII

- presents with PR bleed, sheek bleeding (abdominal bleeding)

65
Q

What do you do with an abnormal clotting result?

A
  • REPEAT sample
  • check chronicity
  • check anticoagulation
  • liver disease/DIC
  • request lupus anticoagulant screen - this is an autoantibody that interferes with the test
  • Long APTT - request factor levels, but discuss before doing this with a haematologist
66
Q

What factors are part of the extrinsic pathway?

A

PT

VII

67
Q

What factors are part of the intrinsic pathway?

A

VIII, IX, XI, XII

APTT

68
Q

What type of anaemia is iron deficiency anaemia?

A

Microcytic hypochromic

69
Q

What does hypochromic mean?

A

pale, low mean cell haemoglobin

70
Q

What is HbF?

A

Foetal haemoglobin, two alphas, two gammas

71
Q

How is Sickle Cell Disease inherited?

A

Autosomal recessive

72
Q

What chromosome is it on and what is the mutation?

A

Single amino acid, chromosome 11

73
Q

What is the lifespan of a sickle cell?

A

20-30 days

74
Q

What is seen on blood film for SCD?

A

Reticulocytes (due to increased production, RBC precursors with nuclear components)

75
Q

What is a risk for sickle cell trait?

A

Sudden death in extreme exertion or severe hypoxia (anaesthetic, deep sea diving)

76
Q

What are some classic presentation features of sickle cell disease?

A

Splenomegaly (multiple splenic infarcts and autosplenectomy), anaemia, increased infection risk (mainly pneumococcal)

77
Q

How is a vaso-occlusive crisis managed?

A

Analgesia, O2 high flow, hydration, blood flow

78
Q

What are some causes of iron deficiency anaemia?

A

Malnutrition, poor absorption, poor diet, menstruation, GI bleed, coeliac disease

79
Q

What is a sickle cell crisis?

A

Vaso-occlusive (acute chest syndrome), aplastic crisis

80
Q

What is a vaso-occlusive crisis?

A

Hb becomes deoxygenated at once. Cold, dehydration, hypoxia, lots of sickling and occlusion of vessels. There is pain due to oxygen deprivation. Swollen joints, dactylitis, acute abdomen (if mesenteric ischaemia or renal papillary necrosis).

81
Q

What is acute chest syndrome?

A

vaso-occlusive crisis of lungs, new pulmonary infiltrate on CXR with dyspnoea, fever, pain, cough. Give O2, chest fever, NIV (if severe)

82
Q

How is sickle cell disease managed?

A

regular management – avoid triggers (cold, dehydration, hypoxia, exhaustion, alcohol, smoking), folic acid supplements, oral pen V, full vaccinations

83
Q

Give some complications of Sickle Cell Disease

A

Complications of SCD: chronic pain, PH, stroke, infection, gallstones, retinopathy, avascular necrosis of femoral or humoral heads

84
Q

What are the 3 main causes of MASSIVE splenomagaly?

A

CML
Myelofibrosis
Malaria

85
Q

What factor does rivaroxaban work on?

A

RivaroXaban - Xa

Same as ApiXaban

86
Q

What does dabigatran work on?

A

Direct thrombin inhibitor

87
Q

What might you see on a blood film for hyposplenism (or post-splenectomy)?

A

Howell-Jolly bodies

Target cells

88
Q

Poikilocytes are seen in the blood films and may indicate what?

A

Iron deficiency anaemia
B12 deficiency
Myelofibrosis

89
Q

Give some non-pathological causes of decreased Hb.

A

Pregnancy
Dehydration
IV fluid

90
Q

What is the difference between thrombocytosis and thrombocytopaenia?

A 
Thrombocytosis = increased platelets
Thrombocytopaenia = decreased platelets
91
Q

What are classic symptoms of anaemia?

A 
Fatigue
Palpitations
Anorexia
Sypnoea
Pallor (conjunctival)
Tachycardia
92
Q

What are the three main pathophysiologies behind anaemia?

A

Impaired production
Increased red cell destruction
Blood loss

93
Q

What are the different causes of microcytic anaemia?

A

Iron deficiency anaemia
Chronic disease
Thalassaemia

94
Q

What are the different causes of normocytic anaemia?

A

Acute blood loss
Anaemia of chronic disease
Malignancy

95
Q

What are the different causes of macrocytic anaemia?

A

B12/Folate deficiency
Alcohol
Hypothyroidism

96
Q

What are some signs of iron deficiency anaemia?

A

Glossitis - inflammation of the tongue
Koilonychia - spoon shaped nails
Angular cheilitis - ulceration at the corner of the mouth
Post-cricoid webs - Plummer Vinson syndrome, characterised by difficulty swallowing and iron deficiency

97
Q

What will be seen on a coagulation/iron screen in iron deficiency anaemia?

A 
Low ferritin (transferrin, TIBC)
Low serum iron
98
Q

What are your investigations of choice for iron deficiency anaemia?

A

Bloods: FBC, U&E, full iron

Colonoscopy/OGD

99
Q

What are some side effects of PO iron?

A

Black stools
Constipation
Upset stomach
Staining of teeth

100
Q

What is Von Willebrands’s disease?

A

A common inherited bleeding disorder, most are autosomal dominant
Results in prolonged bleeding time, prolonged APTT, reduced factor VIII

101
Q

How do you manage Von Willebrand’s Disease?

A

Transexamic acid - mild bleeding
Desmopressin - raises levels of vWF from Weibel-Palade bodies in endothelial cells
Factor VIII concentrate

102
Q

What is the main factor in cryoprecipitate and what are indications for transfusion?

A

Factor VIII
Massive haemorrhage
Uncontrolled bleeding due to haemophilia
Low fibrinogen level

103
Q

What forms of anticoagulation can be used during pregnancy?

A

WARFARIN IS CONTRAINDICATED

S/C LMWH - preferred to IV

104
Q

When should a patient have an urgent OCG/colonoscopy?

A

Men with Hb under 110g/L

105
Q

Haemarthrosis is a typical feature of what?

A 
Haemophilia A and B
A tense, swollen joint, often knee
No history of trauma
No sign of injury
No fever
No fracture or arthritis on x-ray
106
Q

What is the most common type of HL?

A

Nodular sclerosing

107
Q

What is a lymphoma?

A

Malignant growth of WBC, occurs in lymph glands

108
Q

What are the different types of HL?

A

Nodular sclerosing
Lymphocyte rich HL - high infiltrate
Mixed - worst prognosis, drenching sweats, fever, weight loss
Lymphocyte depleted - poor prognosis, Reed Sternberg cells

109
Q

What are the two common types of NHL?

A

Follicular - Low grade

Diffuse large B-cells - High grade

110
Q

What is the classical presentation of lymphoma?

A

Painless lymphadenopathy (asymmetrical)
Weight loss, sweating, fever
Splenomegaly/Hepatomegaly

111
Q

What may be seen on X-ray in Lymphoma?

A

Mediastinal widening or mass

112
Q

What is a hallmark finding on biopsy of HL?

A

Reed-Sternberg cells, ‘owl eyes’/popcorn cells

113
Q

What is the staging criteria used for HL?

A

Ann Arbor

114
Q

What are the drugs used in HL chemotherapy treatment?

A 
ABVD
Adriamycin (doxorubicin)
Bleomycin
Vinblastine
Dacarbazine
115
Q

What is myelofibrosis?

A

A myeloproliferative disorder
Anaemia symptoms, massive splenomegaly
Pokilocytes

116
Q

What is an acute haemolytic transfusion reaction and what should be done?

A

Reaction to transfusion
Fever, rigors, decreasing BP, chest pain, dizziness
Terminate the transfusion, give fluid resuscitation with saline and inform the lab

117
Q

What is seen on a biopsy in Burkitt’s Lymphoma?

A

‘Starry sky’ densely packed basophilic cells interspersed with white areas containing cellular debris

118
Q

What are symptoms of polycythaemia rubra vera?

A

Itching
Increased risk of thrombosis
Increased gout risk
Rosacea

119
Q

How do you treat polycythaemia?

A

Aspirin
Venesection
Bone marrow suppressive drugs

120
Q

What are some secondary causes of polycythaemia?

A

Hypoxia - COPD, altitude, obstructive sleep apnoea

121
Q

What does a rouleaux formation on a blood film indicate?

A

Myeloma

122
Q

Give five differentials of bone pain tenderness?

A 
Myeloma
Sickle cell anaemia
Paget's disease of the bone
Hyperparathyroidism
Metastatic lung cancer
123
Q

What kind of inheritance is Glucose-6-phosphate dehydrogenase deficiency?

A

X-linked

124
Q

How can G6PD present?

A

Oxidative stress if infection, acidosis, drugs (nitrofurantoin), dietary requirements (fava beans)
Haemolytic anaemia - red blood cell breakdown - jaundice, red cell fragments on blood film, Heinz bodies on blood film

125
Q

What does the clotting screen look like in DIC?

A

Low platelets
High PT
High APTT
Low fibrinogen

126
Q

What medication can be used in sickle cell disease?

A

Hydroxycarbamide - increased foetal haemoglobin

Can offer stem cell transplant if frequent crises

127
Q

What is the genetic defect in haemophilia?

A

X-linked recessive
Occurs only in boys
A - Factor VIII deficiency
B - Factor IX deficiency

128
Q

What symptoms occur in haemophilia?

A

Prolonged bleeding, muscle bleeds and joint bleeds

Easy bruising, mouth bleeds

129
Q

What kind of inheritance is von Willebrand Disease?

A

Autosomal Dominant

130
Q

What is ITP?

A

Immune Thrombocytopenic Purpura
Immunological thrombocytopaenia caused by antiplatelet autoantibodies resulting in immune destruction of platelets
Occurs acutely post-illness, commonly in children

131
Q

What is tumour lysis syndrome?

A

Breakdown of malignant cells leading to a high uric acid, potassium, phosphate levels and low calcium.
Causes arrythmias, seizures or sudden death

132
Q

What is amyloidosis?

A

Systemic amyloid deposits around the body, can occur primary or secondary to another disease

133
Q

What are features of amyloidosis?

A

Racoon eyes
Unexplained HF
Unexplained weight loss
Renal symptoms

134
Q

What are some acute and chronic reactions to blood transfusions?

A

Febrile reaction
Acute haemolytic reaction – ABO incompatibility, stop transfusion, tell haematologist and saline
Anaphylaxis – bronchospasm, cyanosis – stop transfusion and maintain airway
Bacterial contamination – low BP and rigors
TRALI
Non-haemolytic febrile transfusion reaction

135
Q

Features of pancytopenia?

A

Infection, bleeding, bruising, pallor, fatigue, SOB

Medical Finals 2020 (25 decks)

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