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Phase 4 > Haematology 2 > Flashcards

Haematology 2 Flashcards

1
Q

What are the three classification systems used for leukaemia?

A

Morphological

Immunological

Cytogenetic (chromosomal analysis)

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2
Q

What symptoms are seen in ALL and what would you expect the Blood work to show?

A 
Anaemia 
Bruising/ petechiae 
Recurrent infections 
Mediastinal mass 
Hepatomegaly 
Splenomegaly Lymphadenopathy 
Orchidomegaly 
Cranial nerve palsies

Bloods:

  • Anamia
  • MVC - normal/ raised
  • Leucocyte count can be high or low!
  • Thrombocytopenia
  • Blood smear - blast cells

Bone marrow
- hypercellular

Special tests:
- LP *all ALL should receive lP

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3
Q

What is the general management for leukemia?

A

Support

  • blood transfusion
  • IV fluids
  • Allopurinol

Infection control
- neutropenic sepsis

Chemotherapy

  • induction
  • consolidation
  • remission

Allogeneic marrow transplantation

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4
Q

What are some signs of AML?

A

Anaemia
Bleeding
Infection
DIC

Hepatomegaly 
Splenomegaly 
Gum hypertrophy 
Skin involvement
CNS involvement

Diagnosis:

  • WCC is usually high but may be low
  • Bone marrow biopsy - blast cells

Auer rods differentiate from ALL

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5
Q

What are the complications of bone marrow transplant?

A

GVHD
Opportunistic infection
Relapse
Infertility

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6
Q

What is the management of CLL?

A

If symptomatic:

  • chemotherapy
  • Radiotherapy (helps lymphadenopathy and splenomegaly)
  • Stem cell transplant
  • Supportive care

1/3 never progress
1/3 progress slowly
1/3 develop richters lymphoma

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7
Q

Who gets hodgkin’s lymphoma?

A

Bimodal

  • young adults 15-24
  • elderly

SLE patients

Post transplantation

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8
Q

What are the symptoms of hodgkin’s lymphoma?

A 
Enlarged rubbery lymph node - changes in size
Fever 
Weight loss 
Night sweats 
Pruritus

Splenomegaly
anaemia
Cachexia
Hepatomegaly

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9
Q

What investigations should be done into hodgkin’s lymphoma?

A

Bloods:

  • FBC - low lymphocytes is poor sign
  • Blood film
  • ESR
  • LFTs - infiltration/ obstruction due to nodes
  • LDH - high during turnover
  • Urate levels - high during turnover
  • U&Es - to ensure normal function prior to treatment

X-ray:
- CXR - for mediastinal mass

  • CT Chest/ Abdo/ Pelvis
  • PET SCAN
  • ECHO - this is because some of the treatment is going to require good cardiovascular function.
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10
Q

What is meant by stage 1A or 1B or 2B etc in lymphoma?

A

Number refers to the Ann arbor classification
Letter refers to absence or presence of B symptoms.
A - asymptomatic
B - symptoms, night sweats, weight loss etc.

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11
Q

What is the treatment of Hodgkin lymphoma?

A

Stages 1-2A
- Radiotherapy + short chemotherapy course

> 2A
- ABVD chemotherapy courses

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12
Q

What are the complications of Hodgkin’s lymphoma treatment?

A

Risk of secondary malignancies

  • breast
  • lung
  • melanoma
  • thyroid cancers

Ischemic heart disease
- radiation

Infertility

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13
Q

What are the risk factors for Non - hodgkin lymphomas?

A

Immunodeficiency

  • Drugs
  • HIV

Infections:

  • EBV
  • H. Pylori

Autoimmune conditions:

  • Sjogrens
  • SLE

Drugs:

  • Ciclosporin
  • Radiation therapy
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14
Q

What are the signs and symptoms of non-hodgkin lymphoma?

A 
Superficial lymphadenopathy
This can cause compression of surrounding structures: 
- Biliary obstruction 
- ureteric obstruction 
- nerve compression
- dysphagia

Symptoms due to local inflammation:

  • Ascites
  • Pleural effusion

B symptoms

Pancytopenia

  • infection
  • anaemia

Extranodal disease:

  1. Gut
    - MALT - H.Pylori related - symptoms of gastric Ca
    - Non - MALT - Symptoms of gastric Ca
    - Enteropathy related T cell lymphoma
  2. Skin
    - Mycosis Fungoides
  3. Oropharynx
    - Waldeyer’s ring

Systemic features:

  • weight loss
  • night sweats
  • fever
  • Pancytopenia
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15
Q

What investigations should be done into Non - Hodgkin’s lymphoma?

A

Much of the tests for Non-hodgkin’s are the same as hodgkin’s, expect there are few others:

Bloods:

  • FBC
  • U&Es
  • LFTs
  • LDHs

*HIV testing

Node biopsy
Marrow aspiration + Trephine
Immunophenotyping

LP
- if CNS signs

CT Chest/ Abdo/ Pelvis

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16
Q

What is the management of Non- Hodgkin lymphomas?

A

Low grade:

  • no treatment if asymptomatic
  • Radiotherapy
  • Rituximab

High Grade:
- RCHOP

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17
Q

Prior to taking bone marrow aspiration what may need to be done?

A

Coagulation disorders may need to be corrected.

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18
Q

What are the possible causes for pancytopenia?

A

Bone marrow failure:

  • aplastic anaemia
  • Viral - Parvovirus B19

Bone marrow infiltration:

  • Leukaemia
  • Myeloma
  • Lymphoma (NHL)
  • Myelodysplasia

Infective Haematopoiesis:

  • B12 deficiency
  • AIDS

Peripheral pooling:

  • Myelofibrosis
  • SLE
  • Hypersplenism
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19
Q

What diagnostic test is required in Pancytopenia?

A

Bone marrow aspiration
+
Marrow Trephine
- posterior iliac crest

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20
Q

How should pancytopenia be investigated?

A

Look for underlying causes.

Bloods:

  • FBC
  • Blood film
  • B12/ Folate

Bone marrow aspiration and trephine

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21
Q

How is pancytopenia managed?

A

Address underlying cause

Blood products
Platelets
Neutropenia control

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22
Q

What are infections that can be transfused via blood transfusion:

A

Hep C

Hep B

HIV

vCJV

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23
Q

Which factors help to discriminate between High grade and low grade lymphomas?

A

High grades:

  • Diffuse large B cell
  • Burkitt’s
  • Mantle

Fast growing
More active B symptoms
Higher levels of LDH
CRP is high

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24
Q

What are the complications of CLL?

A

Autoimmune Haemolytica

Agammaglobulinemia

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25
Q

What are some differentials to Non- hodgkin lymphoma?

A

Other leukaemias
Sarcoidosis
EBV

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26
Q

Who should undergo further investigations for DVTs?

A

> 55 years which was unprovoked

those with multiple DVTs

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27
Q

What are some of the clinic signs of myelofibrosis?

What investigations?

What is the treatment?

A

Recurrent DVTs in unusual places
Episodes of gout - High urate turnover
High platelet count on FBC

Bone marrow biopsy with trephine needed for diagnosis
- fibrosis within the bone marrow

Treatment:

  • supportive
  • Bone marrow transplant
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28
Q

How does Essential thrombocythemia present?

how is it treated?

A

Abnormal clotting
Abnormal bleeding
Microvascular occlusion

Investigations:

  • FBC - thrombocytosis
  • rule out other causes of thrombocytosis

Treatment:

  • Aspirin
  • Hydroxycarbamide
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29
Q

If there is signs of haemolytic anaemias, what further investigations should be done in the blood work up?

A

LDH
Haptoglobin
LFTs - bilirubin

If haemolysis is proven then:

Direct Coombs test should be performed.

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30
Q

What advice should be given to those with G6PD? and how is it transferred?

A

x-linked

mainly:
- mediatrian
- African

Avoid:

  • Henna tattoos
  • Antimalarials - Primaquine
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31
Q

How is hereditary spherocytosis and elliptocytosis passed on and how are the treated?

A

Autosomal dominant

Treated with splenectomy

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32
Q

As well as looking investigating the blood work up, what else should be investigated in beta thalassemia?

A

MRI of heart
- iron build up

TFTs
- thyroid toxicity to iron build up

Pancreas dysfunction
- same reason

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33
Q

What are some of the complications of beta thalassemia outwith Anaemia and extramedullary hematopoiesis?

A

Hypogonadism

Hypothyroidism

Pancreatic dysfunction

Heart disease

Hypocalcaemia

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34
Q

Which group of people are most likely to get Beta thalassaemia?

A

Mediatrerian

Far East

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35
Q

How is Beta thalassaemia managed?

A
  • Folate supplements
  • Regular blood transfusions
  • Iron Chelating agents
  • Splenectomy
  • Hormone replacement - due to secondary damage
  • Bone marrow transplant - in the young

Prevention:
- antenatal diagnosis - fetal blood or DNA

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36
Q

What are the signs and symptoms of polycythemia ruba vera? How is it investigated and diagnosed?

A 
>60 year olds 
Hyperviscosity symptoms: 
- headache 
- amourosis fugax 
- lethargy
- visual changes "looking through watery car windscreen"

tinnitus

Itch after warm bath

Facial Plethora

Investigations:

  • FBC
  • Haematinics
  • B12 - increased

Bone marrow aspiration
- hypercellular with erythroid hyperplasia

  • EPO levels (low)
  • JAK2 investigations

Treatment:

  • Aspirin
  • Venesection
  • Hydroxycarbamide
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37
Q

In Beta thalassemia what would you see on electrophoresis of the serum?

A

Increased HbF

Increased HbA2

Lack of HbA

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38
Q

Name some differentials for Hodgkin’s lymphoma:

A
  • CLL
    • Abscess from infection
    • Metastasis from oesophagus
    • Non- Hodgkin’s lymphoma

Lipoma

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39
Q

What is another type of NHL which affects the gastrointestinal tract?

A

MALT
- associated with H.Pylori

Treated with:

  • antibiotics - remove source
  • Chemotherapy
  • radiotherapy
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40
Q

What are the causes of Burkitt’s lymphoma?

A

Endemic - EBV
HIV related
Sporadic - most likely to affect ileocaecal valve

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41
Q

How can fibrogen be measured?

A

Thrombin time

42
Q

What will the PaO2 be in anaemia?

A

Normal, PaO2 is the diffusion of O2 in blood

43
Q

What is the MCH?

A

Mean corpuscle Haemoglobin

- will be low in Anemia

44
Q

What are the typical x-ray appearances seen on beta thalassemia?

A

Hair on end appearance of skull
Bossing of the skull
Thinning of the cortical bone

45
Q

What is the reticulocyte count in iron deficiency anemia?

A

Low

- not enough iron to create enough RBCs

46
Q

What is the mutation defect in sickle cell?

A

Non conservative point mutation.
glutamine to valine

Making the beta chain non water soluble.

47
Q

What are some triggers to cause a sickle cell to sickle?

A

Low oxygen
Infection
Acidosis
Dehydration

48
Q

What are the crisis that occur in sickle cell disease?

A

Aplastic crisis

Splenic sequestration

Acute chest syndrome

49
Q

If someone has a prolonged APTT what test should be done next?

A

Corrected APTT

50
Q

How is haemophilia A and B inherited?

A

X linked recessive

51
Q

What are the INR levels wanted for various conditions of warfarin?

A

P.E
Post DVT
AF
= 2.5

Mechanical heart valve
antiphospholipid syndrome
coronary artery bypass
= 3.5

52
Q

What management should be given in a major bleed with increased INR?

A

Stop warfarin

Give vitamin K

FFP
+/-

Prothrombin complex concentrate

53
Q

What are the different types of von willebrand disease?

A

Type 1: Quantitative deficiency

Type 2: Qualitative deficiency

Type 3: Complete lack of Von Willebrand
- inherited recessively

54
Q

What are some causes of thrombocytosis?

A

Splenectomy

Malignant disease

Post surgery

Post bleeding
- afterwards

Acute phase reactant - so anything that stimulates

Iron deficiency

55
Q

What would cause a raised thrombin time?

A

Fibrinogen deficiency

Heparin

56
Q

What is needed for there to be a graft vs host disease?

A
  1. Graft tissue must contain immune cells
  2. Host must be immune suppressed
  3. The host must be immunologically different
57
Q

What are the symptoms of GVHD? and the order in which they occur

A

1st Skin

  • painful itchy rash
  • lacrimal glands

2nd Liver

  • Jaundice
  • Raise in LFTs

G.I

  • diarrhoea
  • Intestinal disease
58
Q

What can help differentiate between the microcytic anaemias?

A 
Serum Iron 
TIBC 
Serum ferritin 
Iron in marrow 
Distribution of width **Only deficiencies will cause this

Mentzer test** for IDA vs Thalassemia

*thalassaemias tend to have normal levels across all

59
Q

What is the test that can be done into pernicious anaemia?

A

Schilling test
- radioactive B12 is given

Urine is collected. Normally 10% is excreted. if this is abnormal then oral intrinsic factor is given. and the above is repeated.
- if normal excretion then diagnosis of pernicious anaemia

60
Q

List some cause so folate deficiency:

A 
Diet 
Alcohol 
Coeliac disease
Antifolate drugs - methotrexate 
Inflammatory bowel disease
61
Q

What is the two types of electrophresis done on multiple myeloma and how do they differ?

A

Plasma electrophoresis - shows there is a spike

Immunoelectrophoresis - differentiates the spike

62
Q

How does myeloma cause kidney failure?

A

Amyloidosis build up

Renal tubular obstruction
- due to bence Jone’s proteins

Excessive use of NSAIDs due to bone pain

Direct toxicity of the paraproteins

63
Q

Whats the pneumonic for Myeloma?

A

CRABBI

  • calcium
  • Renal
  • Anaemia
  • Bone pain
  • Bleeding
  • Infection
64
Q

What is a poor prognostic factor in myeloma?

A

Low Albumin

65
Q

What is the colour of urine in pre-hepatic jaundice?

A

Coco cola like urine

66
Q

How is G6PD deficiency inherited and thus who won’t pass it on?

A

X - linked. therefore if father has it - they won’t pass it on.

67
Q

Break down of the platelet bleeding disorders

A

Idiopathic thrombocytopaenic purpura (ITP) - Isolated thrombocytopaenia in a relatively well person

Thrombotoic thrombocytopaenic purpura (TTP) - Thrombocytopaenia in a very unwell person

Haemolytic uraemic syndrome (HUS) - Thrombocytopaenia, schistocytes, renal failure, post-dysentry

Henoch Schonlein Purpura (HSP) - Abdo pain, joint pain, haematuria, purpura. Kids.

Haemorrhagic Haemolytic Telangiectasis (HHT) - Epistaxis, GI bleeds, telangiectasia

68
Q

How should tranexamic acid be administered in a major bleed?

A

IV slow injection

69
Q

What type of transplant is giving to those with lymphoma?

A

Autologous Stem cell transplant
- use of GSF to draw out stem cells which can be collected and re-introduced following chemotherapy to shut down the patients bone marrow

70
Q

When carrying out cytogenetic testing on leukemia, what type of molecular testing can be done?

A

FISH

71
Q

When planning therapy for lymphoma/ leukemia etc what tests should be done?

A

Biochemistry
- U&Es etc

Cardiac function

  • ECG
  • Echocardiogram
72
Q

What are the classic signs of Iron deficiency anaemia?

A 
Fatigue 
Breathlessness 
Headaches 
Tinnitus 
Palpitation

Signs:

  • Pallor
  • Hyperdynamic circulation
  • Flow murmur
  • Koilonychia
  • Esophageal webs
  • Angular stomatitis
73
Q

Why do you get a lemon tinge in B12 deficiency?

A

Hypercellularity with breakdown in the marrow

- haemolytic anaemia

74
Q

What test is used to diagnose Hereditary spherocytosis? and what is the treatment?

A

Osmotic fragility test

Splenectomy and folate

75
Q

What is the long term management of sickle cell?

A

Penicillin V
Pneumococcal vaccine
Folate

Hydroxycarbamide

Bone marrow transplant - controversial

76
Q

Treatment for haemophilia A:

A

Desmopressin

Recombinant factor 8

  • for severe.
  • Can be reduced by autoantibodies against it

Tranexamic acid if severe bleeding

77
Q

What are the clinical signs of antiphospholipid syndrome?

A

CLOT

  • Coagulation defect - Increased APTT
  • Livedo reticularis
  • Obstetric complications
  • Thrombocytopenia
78
Q

What investigations should be done into suspected thrombophilia?
What is the treatment?

A 
FBC 
Coagulation studies 
Factor V leiden 
Anti-cardiolipin/ Lupus Anticoagulant test 
Immunoassays for Protein C and S 
PCR for thrombin gene defect

Life long anticoagulation
- warfarin

79
Q

What is the staging for CLL?

A

Binet staging

80
Q

What are the major symptom of bone marrow failure and what treatment can be used in primary causes?

A

Major symptoms are all those in keeping with pancytopenia but bruising and bleeding are big problems.

  • young person
  • Bone marrow transplant
  • > 40
  • Anti - thymocyte globulin (anti T cell globulin to prevent more destruction in the bone marrow)
  • ciclosporin
81
Q

What are the most common thrombophilia diseases:

A

Factor V leiden
- APC resistance

Prothrombin gene mutation
- higher levels of prothrombin

Protein S and Protein C deficiency

Antithrombin III deficiency

Acquired
- phospholipid syndrome

82
Q

Name some drugs that can cause bone marrow suppression:

A

Sulfonamides

Chloramphenicol

Carbimazole

Chemotherapy

Phenytoin

83
Q

What is the immunosuppressive drugs used in treatment of bone marrow failure?

A

Anti - thymocyte globulin

Ciclosporin

84
Q

What is it called when there is painful extremities associated with polycythemia vera?

A

Erythromelalgia

- due to abnormal dilation of vessels

85
Q

What would you expect to see on urine dipstick of someone with myeloma?

A

Increased specific gravity

- bence jones proteins don’t show up

86
Q

What are some of the complications of myeloma?

A

Hypercalcemia

Cord Compression

AKI

Hyperviscosity
- may require plasmapheresis

87
Q

What are the poor prognostic indicators in myeloma?

A

Low albumin

Beta 2 microglobulins

88
Q

What defines monoclonal gammopathy of uncertain significance?

A

<30g/L of paraprotein
<10% blast cell
Lack of CRAB symptoms

89
Q

What is Waldenstrom’s macroglobulinemia defined by:

A

IgM
- only agglutinates when cold

Peripheral neuropathy symptoms

90
Q

What are some complications of splenectomy?

A

Super infection
Early retribution of platelets - VTE
Left lower lobe atelectasis

91
Q

List some causes of very high ESR:

A 
Myeloma 
SLE 
AAA
Prostate cancer 
Ulcerative colitis
92
Q

What are some causes of Basophilia:

A

CML

Parasitic infection

93
Q

What are some raised lab features associated with Pernicious anemia?

A

Increased homocysteine
Increased gastrin levels
Achlorhydria

94
Q

Compare and contrast macrocytic anaemia vs megaloblastic anemia:

A

Megaloblastic:

  • Hypersegmented neutrophils
  • Neurological deficits
  • Pancytopenia
  • Enlarged glossitis

*macrocytic anaemia does not have these

95
Q

Why might there be a high level of haptoglobin? making it difficulty to interrupt results?

A

Haptoglobin is an acute phase reactant protein

- thus may raise inflammation and mask the true effects of the haemolysis

96
Q

List some complications of sickle cell:

A

Acute chest syndrome

Sequestration crisis

Aplastic crisis

Priapism

Ulceration

Kidney damage
- haematuria can be seen

Gallstone formation

97
Q

What investigations should be done into haemolytic anaemia?

A 
FBC 
Blood film 
LDH 
Haptoglobin 
Reticulocyte count 
Hemoglobin level 
Plasma electrophoresis - sickle cell

Orifices:
- urine analysis

Special tests:
- Coomb’s test

98
Q

What are some differentials for Myeloma?

A

MGUS

Amyloidosis

Waldenstrom’s macroglobulinemia

Lymphoma

99
Q

What is the mechanisms of Leiden V mutation?

A

Factor V resistant against protein C

100
Q

In renal failure what is a good anti-thrombin medication to be put on?

A

Apixaban

101
Q

What are the mechanisms that lead to kidney damage in myeloma? ad how does it present?

A

Bence Jones accumulation

  • direct toxicity
  • accumulation within renal tubules

NSAID use

Amyloidosis

Stones.
Renal failure
Casts
Pyelonephritis

Phase 4 (49 decks)

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