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3rd Year Medicine UoM > Haematology > Flashcards

Haematology Flashcards

1
Q

What is leukaemia?

A

A malignant neoplastic process involving one of the white blood cell lines. It can be lymphocytic or myeloid depending on the line that is affected
Originate and are primarily bone marrow based.
Involve circulating lymphoblasts

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2
Q

How are leukaemia’s classified?

A

Acute = involves excessive number of immature cells (blast cells) which dominate peripheral blood films and overcrowd bone marrow preventing healthy cells being produced

Chronic= involves overproduction of very abnormal mature cells. These are seen in excess in peripheral blood films

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3
Q

How does acute lymphocytic leukaemia present? ALL

A

-mainly in children (75%). Also seen at ages 35 and 80
-signs and symptoms usually present for only a few weeks before diagnosis
-symptoms are mainly due to cytopenias (anaemia-fatigue, dizziness, pallor, thrombocytopenia-bruising, bleeding, menorrhagia and neutropenia -recurrent infection, fever)
- signs include = lymph node involvement (generalised, enlarged, painless and freely moveable), hepatosplenamegaly, renal enlargement,
- CNS infiltration by lymphoblasts can present as papilpoedema , nuchal rigidity, meningismus, focal neurological signs
-painless unilateral testicle enlargement
bone pain

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4
Q

What investigations should be performed for suspected ALL?

A 
Peripheral blood smear - lymphoblasts 
FBC with differentials
Coagulation profile 
Baseline virology = hiv? Hep?
CSF examination 
CXR
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5
Q

What are common blood test results for ALL?

A 
Normocytic normochromic anaemia 
Low reticulocyte count 
Leukocytosis 
Neutropenia 
Thrombocytopenia
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6
Q

How is ALL diagnosed ?

A

1) 20/30% lymphoblasts present on peripheral blood smear or bone marrow aspirate
2) immunophenotyping of BM biopsy shows markers of lymphoid or myeloid leukamia cells. (Differentiated by monoclonal antibodies )

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7
Q

How is ALL managed /treated?

A

1) supportive treatment = manage risk of infection/haemorrhage etc
- quinolone antibiotics will prevent gram -ve bacteria
-fluid therapy
-platelet tranfusion if < 10
-haematopoietic GFs e.g. filgrastim, sargromastim (both accelerate neutrophil recovery after chemo induced BM suppression)
2) chemotherapy
-induction
-consolidation =
-maintenance =mercaptopurine, methotrexate
3) allogenic stem cell transplant SCT
-

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8
Q

What is leukaemia?

A

A malignant neoplastic process involving one of the white blood cell lines. It can be lymphocytic or myeloid depending on the line that is affected
Originate and are primarily bone marrow based.
Involve circulating lymphoblasts

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9
Q

How are leukaemia’s classified?

A

Acute = involves excessive number of immature cells (blast cells) which dominate peripheral blood films and overcrowd bone marrow preventing healthy cells being produced

Chronic= involves overproduction of very abnormal mature cells. These are seen in excess in peripheral blood films

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10
Q

How does acute lymphocytic leukaemia present? ALL

A
  • mainly in children (75%). Also seen at ages 35 and 80
  • signs and symptoms usually present for only a few weeks before diagnosis
  • symptoms are mainly due to cytopenias (anaemia-fatigue, dizziness, pallor, thrombocytopenia-bruising, bleeding, menorrhagia and neutropenia -recurrent infection, fever)
  • signs include = lymph node involvement (generalised, enlarged, painless and freely moveable), hepatosplenamegaly, renal enlargement,
  • CNS infiltration by lymphoblasts can present as papilpoedema , nuchal rigidity, meningismus, focal neurological signs
  • painless unilateral testicle enlargement
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11
Q

What investigations should be performed for suspected ALL?

A 
Peripheral blood smear - lymphoblasts 
FBC with differentials
Coagulation profile 
Baseline virology = hiv? Hep?
CSF examination 
CXR
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12
Q

What are common blood test results for ALL?

A 
Normocytic normochromic anaemia 
Low reticulocyte count 
Leukocytosis 
Neutropenia 
Thrombocytopenia
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13
Q

How is ALL diagnosed ?

A

1) 20/30% lymphoblasts present on peripheral blood smear or bone marrow aspirate
2) immunophenotyping of BM biopsy shows markers of lymphoid or myeloid leukamia cells. (Differentiated by monoclonal antibodies )

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14
Q

How is ALL managed /treated?

A

1) supportive treatment = manage risk of infection/haemorrhage etc
- quinolone antibiotics will prevent gram -ve bacteria
- fluid therapy
- platelet tranfusion if < 10
- haematopoietic GFs e.g. filgrastim, sargromastim (both accelerate neutrophil recovery after chemo induced BM suppression)
2) chemotherapy
- induction = prednisolone, vincristine, doxorubicin, anthracyclines, cristantapase, rituximab
- consolidation =cytarabine
- maintenance =mercaptopurine, methotrexate
3) allogenic stem cell transplant SCT

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15
Q

what is the most common malignancy in childhood?

A

ALL

accounts for 80% of childhood leukaemias

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16
Q

what is Acute Myeloid Leukaemia?

A

malignancy arising from myeloblasts (which usually produce the granulocytes and monocytes)
common leukaemia in adults, occurring most at age 65/70
- occurs as primary disease or secondary transformation of a myeloproliferative disease

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17
Q

how does AML present?

A

clinically indistinguishable from ALL
- signs/symptoms from myeloblast infiltration of bone marrow and organs: anaemia, thrombocytopenia, neutropenia (very high WBC and very low neutrophils), hepatosplenomegaly, bone pain
raised WBC count >100000 - bad!
leukostasis = WBC thrombi in vessels supplying brain/heart./lung - hypoperfusion

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18
Q

what investigations should be performed for AML?

A 
full blood tests with differentials
peripheral blood smear
BM biopsy 
CSF examined-CNS involved?
CXR - infection? masses?
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19
Q

how is a diagnosis of AML made?

A

1) presence of myeloblasts in peripheral blood smears
characterised by Phi bodies or Aer Rods
2) BM aspirate and trephine biopsy - immunophenotyping/immunohistochemistry used to differentiate between AML/ALL and classify the type of AML. level of infiltration is also analysed

> 20% myeloblasts in blood smear and BM aspirate = diagnosis

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20
Q

which leukaemia are phi bodies and Aer rods associated with?

A

AML

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21
Q

how is AML treated?

A

intensive chemotherapy.
main drugs = cytarabine and duanorubicin
Bone Marrow Transplant in refractory or relapsing disease

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22
Q

what is the process of bone marrow transplant/stem cell transplant?

A
  • an HLA matched donor is found and bone marrow harvested from iliac crests and sternum
  • the patients immune system is wiped out with radiation and chemo (cyclophosphamide)
    and then patient is repopulated with donor BM
  • ciclosporine and methotrexate (immunosuppressants) used to prevent patient attacking donor BM
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23
Q

what are complications of bone marrow transplant?

A

Graft Vs Host disease (GVHD)
relapse
infertility
opportunistic infection

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24
Q

what are characteristics of chronic myeloid leukaemia?

A
  • occurs in adults 40-60 YO
  • uncontrolled proliferation of myeloid cells
  • may undergo blast transformation (80% to AML and 20% ALL)
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25
Q

how does CML present?

A
  • 20-40% asymptomatic and found incidentally
  • chronic and insidious symptoms e.g. fatigue, weight loss, sweating, fever
  • gout features due to purine breakdown (increased cell turnover)
  • abdo discomfort - splenamegaly
  • bleeding, bruising, anaemia
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26
Q

how is CML diagnosed?

A
  • very raised WBC (all myeloid cells raised e.g. neutrophils, monocytes, basophils etc)
  • hypercellular, large spec of myeloid cells seen in peripheral blood
  • cytogenic analysis of blood/BM for Philadelphia chromosome (present in 95% cases)
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27
Q

which leukaemia is the phildelphia chromosome associated with?

A

chronic myeloid leukaemia

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28
Q

how is CML treated?

A

1st line = Imatinib
hydroxyurea (aka hydroxycarbamide)
interferon alpha
HSCT - considered in patient who have failed TKI therapy

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29
Q

what is the MoA of Imatinib?

A

An inhibitor of the tyrosine kinase associated with BCR-ABL defect
2nd gen of the same class = dasatinib, nilotinib

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30
Q

how is blast transformation treated in CML?

A

1st or 2nd gen TKI therapy followed by allogenic HSCT asap!

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31
Q

what are characteristics of chronic lymphocytic leukaemia?

A
  • most common leukaemia
  • median age 70
  • monoclonal proliferation of well-differentiated B cells (almost always)
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32
Q

how does CLL present?

A
  • often none; discovered incidentally
  • 10% will present with typical B symptoms = night sweats, chills, fever, weight loss, fatigue
  • bleeding, infection
  • more marked lymphadenopathy than CML
  • hepatosplenomegaly
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33
Q

what are complications of CLL?

A
  • hypogammaglobulminaemia causing recurrent infections
  • warm autoimmune haemolytic anaemia
  • transformation to high-grade lymphoma (Richters transformation)
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34
Q

how is CLL diagnosed ?

A 
immunophenotyping 
smear cells (smudged B cells) on peripheral blood film
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35
Q

how is CLL treated?

A
  • it is an indolent disease which is incurable
  • patients with advanced or symptomatic disease should receive chemo - but will relapse
  • chemo = FCR (fludarabine, cyclophosphamide, rituximab)
  • steroids treat autoimmune haemolysis
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36
Q

what is prognosis of CLL patients?

A

1/3 never progress
1/3 progress slowly
1/3 progress rapidly
death often due to infection or richters transformation to aggressive lymphoma

37
Q

what are lymphomas?

A

malignant proliferation of lymphocytes, accumulating in lymph nodes causing lymphadenopathy
may be found in peripheral blood and infiltrating organs

38
Q

what are characteristics of hodgkins lymphoma?

A
  • characterised by cells with mirror image nuclei called Reed-Sternberg cells
  • most common malignancy in age range 15-24
  • bimodal age distribution: around age of 25 and 55
39
Q

how does hodgkins lymphoma present?

A
  • lymphadenopathy - painless, asymmetrical
  • B symptoms: night sweats, lethargy, fever, weight loss >10%
  • generalised pruritis
  • alcohol pain
  • normocytic anaemia, eosinophilia
  • LDH raised (cell turnover)
  • cough, pel epstein fever, haemoptysis, dyspnoea
40
Q

how is Hodgkin’s lymphoma diagnosed?

A
  1. complete lymph node excision - submitted for histological analysis
  2. full blood tests
  3. CT scanning of chest, abdo and pelvis (for staging)
  4. CXR
41
Q

how is Hodgkin’s lymphoma staged?

A

with the Ann Arbor System:
1) single lymph node region
2) 2 + lymph node regions on the same side of the diaphragm
3) involvement of LN regions on both sides of the diaphragm
4) extra nodal involvement e.g. liver or BM
(each stage can then be A (no systemic systems), B (presence of B symptoms) or E (localised extra nodal extension)

42
Q

which infection is associated with Hodgkin’s lymphoma ?

A

Ebstein Barr virus EBV

43
Q

what are the 4 subtypes of hodgkins lymphoma?

A
  1. nodular sclerosing (lacunar cells) 70%
  2. mixed cellularity 20-25%
  3. lymphocyte rich 5%
  4. lymphocyte depleted <1%
44
Q

how is hodgkin’s lymphoma treated?

A

early stage disease = radio and short chemo course
later stage disease = radio and longer course of chemo
chemo regime = ABDV = adriamycin (doxorubicin), Bleomycin, dacarbazine, vinblastine
in relapsed disease = high dose chemo and SCT

45
Q

which hodgkins lymphoma subtype has the best prognosis?

A

lymphocyte rich

46
Q

what factors are associated with worse prognosis in hodgkins?

A

male
older age
lymphocyte depleted
stage 4

47
Q

what are non -hodgkins lymphoma?

A
  • a diverse group of lymphomas without Reed-sternberg cells.
  • median age = 55-60
  • most are b cell
48
Q

what are the b symptoms?

A

fever >38
weight loss >10%
night sweats

49
Q

what are the top 5 most common non-hodgkins lymphomas?

A

1) diffuse large b cell lymphoma (40% cases)
2) follicular lymphoma (20%)
3) mantle cell lymphoma 5%
4) marginal zone lymphoma 5%

50
Q

how are lymphomas classified?

A

low grade = indolent, often incurable, widely disseminated

high grade = aggressive, curable

51
Q

how do non-hodgkins lymphomas present?

A 
painless widespread lymphadenopathy 
hepatosplenamegaly 
B symptoms
LN presence can cause problems in pulmonary tissues, CNS etc
bone pain /back pain

low grade = asymptomatic

52
Q

non-hodgkins lymphoma investigations?

A

FBC, LDH
CT TAP = staging with ann arbur
lymph node excision and evaluation

53
Q

how are NHLs treated?

A

depends on subtype

  • rituximab or interferon alpha for remission maintenance
  • chlorambucil for diffuse disease
  • R-CHOP regime for large b cell lymphoma (Rituximab, cyclophosphamid, hydroxydaunorubicin (doxorubicin), Oncvin (vincristine), Prednisolone
54
Q

what is multiple myeloma?

A
  • malignancy arising from plasma cells (antibody producing B lymphocytes)
  • proliferating plasma cells form deposits in bone - causing osteolytic lesions, seen on xray
55
Q

how does multiple myeloma present?

A
  • bone disease = pain, osteoporosis, pathalogical fractures, osteolytic lesions
  • lethargy, infection
  • hypercalcaemia (mainly due to increase osteoclatic bone reabsorption)
  • renal disease
56
Q

how is multiple myeloma diagnosed?

A

1) Bence Jones Proteins = monoclonal antibodies found in serum and urine
2) increased plasma cells in BM
3) whole body MRI
presence of M protein in urine/blood (virulence factor from streptococcus

57
Q

persistent back pain, hypercalcaemia, anaemia in an older patient raises suspicion of?

A

multiple myeloma

58
Q

when does a lymph node become clinically relevant?

A

adult >1/1.5 cm
child >2 cm
any size but causing neck/head symptoms

59
Q

what are inflammatory causes of lymphadenopathy?

A
  • streptococcus (tonsilitis)
  • strep pneumonia
  • staph aureus
  • adenovirus, rhinovirus, EBV
  • TB
  • HIV
60
Q

how is febrile neutropenia /neutropenic sepsis defined?

A

patient having cancer therapy with neutrophills <0.5x10^9

and either temp >38 or other signs/symptoms of sepsis

61
Q

what antibiotic is given prophylactically to neutropenic cancer patients (<0.5x10^9)

A

fluoroquinolone

62
Q

how is neutropenic sepsis treated?

A

immediate piptaz

vancomycin later

63
Q

in which situations is normochromic, normocytic anaemia seen?

A
  • blood loss
  • anaemia of chronic disease
  • renal failure - deficiency of EPO
64
Q

what causes hypochromic microcytic anaemia?

A

(low MCH, low MCV, low Hb)
this is seen in any condition which decreases production of Hb:
- iron deficiency (diet, malabsorption, blood loss)
- severe protein deficiency
- chronic inflammation (intefers w/ iron metabolism)
- sideroblastic anaemia
- Thalassaemias

65
Q

what are symptoms of iron deficiency?

A 
headache 
craving for non food items
sore or smooth tongue 
brittle nails/hair loss
koilonychia
66
Q

what is thalassemia?

A

an inherited blood disorder in which abnormal Hb is made - due to decrease in synthesis of 1 or more Hb chains. this is due to a defect/absence of 1 or more genes coding for synthesis of alpha or beta chains

67
Q

which haemoglobin types are present in a normal adult?

A

HbA - made from 2 alpha chains and 2 beta chains (97%)

Hb A2 = 2 alpha chains, 2 delta chains

68
Q

what Hb is present in a fetus?

A

HbF = 2 alpha and 2 gamma chains

69
Q

what are features of Beta thalassemia ?

A
  • can be minor (only 1 beta chain gene affected) or major (both genes affected - so no beta chains produced)
  • major presents in childhood with severe aneamia, failure to grow, skeletal abnormalities
  • spleen enlargement and gallstones are associated
70
Q

how is beta thalassemia major treated?

A
  • regular lifelong blood transfusions
  • BM transplant can be curative
  • oral iron chelators needed to prevent iron overload = deferoxamine or deferiprone
  • splenectomy is common
71
Q

what is the main cause of death in beta thalassaemia?

A

iron overload

72
Q

what are features of alpha thalassemia?

A
  • there are 4 alleles for the alpha chains
  • 2 alleles affected = alpha thalassemia trait
  • 3 alleles affected = Hb H disease
  • 4 alleles affected = alpha thalassemia major
73
Q

how is alpha thalassemia treated?

A

same as beta
with regular blood transfusions
stem cell transplants
splenectomy

74
Q

how does alpha thalassemia present?

A 
jaundice 
fatigue 
pronounced forhead
 hypertension 
splenomegaly 
gallstones
75
Q

what are causes for macrocytic anaemias?

A

can be divided in megaloblastic/non-megaloblastic

> megaloblastic: immature nuclei. caused by b12 and folate deficiency (both needed for DNA synthesis).also cytotoxic drugs
non-megaloblastic: reticulocytosis, liver disease, alcohol excess, hypothyroidism, pregnancy,

associated with rapid cell turnover and reticulocytosis

76
Q

causes for b12 deficiency?

A

lack in diet
malabsorption due to lack of intrinsic factor (autoimmune pernicious anaemia, gastrectomy) or lack of terminal ileum (ileal resection)
crohns disease

77
Q

how does b12 deficiency present?

A

anaemia, mild jaundice, neurological (parasthesia, peripheral neuropathy), neuropsychiatric (depression, psychosis, dementia, irritability)
insidious onset

78
Q

how is b12 deficiency treated?

A

hydroxocoalbumin (B12a) - 1mg IM alternate days for 2 weeks and then 3 monthly for life

79
Q

what are causes for folate deficiency?

A

lack in diet
increased demand - pregnancy/increased cell turnover
alcohol
drugs - antiepileptic, methotrexate,trimethoprim

80
Q

what are congenital haemolytic anaemia conditions?

A

RBC membrane disorder: elliptocytosis, spherocytosis
RBC enzyme deficiencies: G6PD, pyruvate kinase
RBC Hb disorders: thalassaemias, sickle cell

81
Q

what are features of sickle cell disease?

A
  • autosomal recessive disease
  • mutation of the gene coding Beta chain, results in the production of HbS (not HbA)
  • seen mostly in african origin, 1 in 700
  • Hb SS = sickle cell anaemia
  • Hb AS = sickle cell trait
82
Q

what is the pathophysiology of sickel cell anaemia?

A

the HbS produced by mutation polymerases when deoxygenated. this causes the RBCs to deform, producing sickle cells which are fragile and haemolyse. = blocking small vessels

83
Q

how is sickle cell diagnosis made?

A

blood film = sickle cells

electrophoresis = determine hbSS or HbAS and confirms diagnosis

84
Q

how does a sickle cell crisis present?

A

a vaso occlusive crisis is due to microvascular occlusion - causing severe pain.
triggered by cold, infection, dehydration, hypoxia,

85
Q

what is hydroxycarbamide/ hydroxyurea?

A

can be used in chronic sickle cell anaemia if frequent crises occuring
its a chemo drug also used for CML

86
Q

how is a sickle cell crisis managed?

A
  • IV opiates
  • rehydrate
  • oxygen
  • antibiotics if neede
  • blood transfusion if Hb or reticulocytes fall fast
87
Q

how can haemolysis be recognised ?

A

anaemia, raised reticulocyte count and jaundice (unconj.)

88
Q

what is the coombs test?

A

the anti-globulin test
looks for antibodies against RBCs
indirect anti-globulin test is used to cross-match blood for suitable transfusion
a positive test means donor is compatible

89
Q

what are features of hereditary spherocytosis?

A
  • anaemia, macrocytic, raised bilirubin
  • gallstones = frequent complication
  • autosomal dominant (75% cases)

3rd Year Medicine UoM (9 decks)

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