Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

Haematology > Heam week 3 > Flashcards

Heam week 3 Flashcards

1
Q

what increases lymphocytes

A

viral illness: glandular fever (inc mononucleosis - EBV), CMV, toxoplasmosis, rubella, pertussis, mumps, HIV (seroconversion)

chronic infections: TB, brucellosis, syphillis

thyrotoxicosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

what can increase neutrophils

A

bacterial infection
inflammatory disorders
metabolic (uraemia, gout)
acute haemorrhage

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

what can decrease neutrophils

A

some viral infections (hepatitis, infuenza, HIV

SLE

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

what is the lifecycle and role of eosinophils

A

circulate for 24 hours then enter tissues

functional role= mediation of allergic response through release of leukotrienes and IgE/ direct parasitic killing

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

what can increase eosinophils

A

allergic disease
parasitic infections
drug sensitivity
skin diseases (psoriasis), hodgkins, hypereosinophilic syndromes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

what is the lifecycle and role of monocytes

A

20-40 hours in blood before entering tissues to become macrophages
phagocytic/ antigen presenting function

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

what increases monocytes

A

chronic bacterial infections (TB, bacterial endocarditis, brucellosis)
protozoan infections
hodgkins disease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

what is the role of basophils

A

infrequent in peripheral blood

transit the blood en route to tissue to become mast cells (histamine release)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

what increases basophils

A

hypothryoidism
UC
chicken pox

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

what can happen to clones during expansion of malignancy

A

sub clones may appear containing other genetic damage

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

why do you need to assess clonality

A

to help diagnosis:

  • lymphoproliferative disoders: need to teel reactive increase lymphocytes from malignant causes in lymph node biopsies
  • myelodysplastic syndrome: distinguish from other marrow pathology
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

what malignancy presents with features in predominantly one cell lineage

A

acute myeloid leukaemia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

name a malignancy that presents with features in mature cells

A

polycythaemia rubra vera

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

what are the two subgroups of lymphcytes

A

T cells- cell immunity

B cells- humoral immunity

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

what are the primary lymphoid organs

A

bone marrow

thymus

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

what are the secondary lymphoid organs

A

lymph nodes
spleen
lymphoid tissue of the alimentary & resp tracts + circulating lymphocytes in the blood and tissue space

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

what lymphocytes go to the paracortex in the lymph node

A

T cells

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

what are the types of B cell lymphomas

A 
lymphocytic lymphoma
intermediate differentiation (mantle zone) lymphoma 
CLL 
well differentiated lymphomcytic lymphoma (WDLLL
WDLL with plasmacytic differentiation 
walderstoms macroglobulinaemia
nodular lymphoma large cell lymphomas 
burkitts lymphoma
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

what are malignant lymphoma

A

malignant lymphoproliferative diseases in which there is replacement of normal lymphoid structure by collections of abnormal cells

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

what are the two types of malignant lymphoma

A

hodgkins and NHL

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

what age does malignant lymphoma usually present

A

HL- younger

NHL- elderly

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

what are the common symptoms of lymphoma

A

painless lymphadenopathy
splenomegaly and hepatomegaly
constitutional symptoms: fever, night sweats, weight loss
anaemia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

what are common sites of involvement n lymphoma

A 
mediastinum (more in HL)
bone marrow
skin 
brain
testis
thyroid
CNS
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

how is lymphoma diagnosed and classified

A

diagnosis by excision biopsy of lymph node/ appropriate tissue
if red sternberg cells= hodgkins disease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

how is HL classified

A 
4 types: 
mixed cellularity 
lymphocytic predominant 
nodular sclerosing 
lymphocyte depleted
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

how is NHL classified

A

follicular or diffuse architecture:

  • low grade= follicular or nodular involvement
  • high grade= diffuse involvement

(diverse group of diseases)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

what is the origin of NHL

A

can be B lymphocyte (more common) or T lymphocyte (less common)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
28
Q

what staging system for lymphoma

A

ann arbor system:
Stage I One lymph node area involved
Stage II Two or more lymph node areas on one side of the diaphragm
Stage III Involvement of nodes above and below the
diaphragm
Stage IV Involvement outside the lymph node areas

The Stage number is followed by the letter A or B indicating the absence (A) or presence (B) of constitutional symptoms.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
29
Q

how is lymphoma staging done

A

via: clinical exam, CXR, CT scan, bone marrow trephine

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
30
Q

what is the treatment for HL

A

early stage: radiotherapy

late stage: combination chemo

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
31
Q

what is the treatment for NHL

A

low grade- palliative radio and/or chemo

high grade- combo chemo

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
32
Q

what is the prognosis for lymphoma

A

HL- 60-70% cured (prognosis dependent on stage)

NHL- low grade: incurable (except early stage), indolent course, 7-10 year survival, 60-80 progress to high grade
high grade rapidly progressing, 30-40% cured
(prognosis dependent on histology: follicular or diffuse)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
33
Q

what are the functions of the spleen

A

filtration (pitting- removal of cell debris and culling- removal of old or abnormal red cells)
storage (platelet, granulocytes and red cells, iron)
haematopoiesis
immunological

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
34
Q

does the spleen usually do haematopoiesis

A

pathological sign

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
35
Q

what can cause hyposplenism

A

congenital absence

developmental: neonatal, old age
surgical: trauma
infarction: sickle cell disease, essential thrombocytopenia
infiltration: lymphoma, amyloid
drugs: methyl dopa, irradiation/ chemo
- immune: SLE, RA, coeliac disease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
36
Q

what is seen on blood film in hyposplenism

A

peripheral blood red cells show distortion, acanthocytes (spikes on RBCs), and target cells, together with inclusion of nuclear remnants (Howell-Jolly bodies) reflecting the absence of splenic “pitting” function

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
37
Q

what organisms become high risk in hyposplenism

A

strep pneumonia and haemophilis influenzae

blood borne polysaccharide encapsulated bacteria

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
38
Q

what infection prophylaxis is given in hyposplenism

A

pneumococcal immunisation
haemophilus influenza B vaccine (before splenectomy)

long term oral antibiotic prophylaxis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
39
Q

what is hypersplenism

A

enhanced cellular filtration, pathological pooling and increased plasma volume
modest shortening of blood cell lifespan

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
40
Q

what are the effects seen in hypersplenism

A

enlarged spleen
deficit in one/ more blood cell lines
normal/ increased cellularity of bone

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
41
Q

what can cause splenomegaly

A

work hypertrophy: haemolytic disease, acute infections, subacute bacterial endocarditis, chronic infections (TB, brucellosis, syphilis), parasitic infections

immune relates: RA, SLE

portal hypertension: congestive splenomegaly

infiltrative

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
42
Q

what are the infiltrative causes of splenomegaly

A

extramedullary haematopoiesis:

  • myeloproliferative diseases (CGL, myelofibrosis, polycythaemia)
  • acute leukaemia
  • haemoglobinopathies (thalassaemia)

lymphoproliferative:
- chronic lymphocytic leukaemia (lymphoma)

amyloidosis
gauchers disease
iron overload

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
43
Q

what are the indications for a splenectomy

A

pain: due to enlargement/ infarction
hypersplenism: severe cytopenias

autoimmune cytopenias: immune thrombocytopenias, autoimmune haemolysis

intrinsic red cell abnormalities: cell membrane (HS, elliptocytosis), enzyme defects (pyruvate deficiency), haemoglobinopathies (thalassaemia, sickle cell)

diagnosis

traumatic rupture

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
44
Q

what sites can be used for bone marrow examination

A

posterior iliac crest
sternum
anterior iliac crest

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
45
Q

what does a bone marrow aspirate allow

A

cellular morphology
flow cytometry/ immunostaining (cellular antigenic profile)
cytogenetics

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
46
Q

what does trephine allow

A

analysis of bone marrow architecture

immunohistochemistry (B cell markers in lymphoma)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
47
Q

how is bone marrow processed for morphological diagnosis

A

aspirate (liquid): smeared onto microscopy slide, stained with haematoxylin/ eosin stains
specific stains for acute leukaemia (peri-iodoc acid schiff/ sudan black) and iron status (perls stain)

trephine (core of bone): formalin fixed and parafin embedded, stained with haematoxylin/ eosin (silver stain for reticulin fibrosis)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
48
Q

what is pancytopenia

A

rediced Hb, white cell and platelets

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
49
Q

what are the clinical complications of pancytopenia

A

anaemia: fatigue, cadiovascular compromise, pallor
thrombocytopenia: increased risk of bleeding, skin purpura, epistaxis, oral, GI, internal (fundus, cerebral)
neutropenia: sepsis, infections: soft tissue/ skin, oral/ perianal, indwelling central line (hickman)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
50
Q

what are myeloproliferative disorders

A

clonal malignant disorders characterised by overgrowth of one or more haematopoietic lineages

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
51
Q

myeloproliferative disorder of red cells=?

A

polycythaemia rubra vera

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
52
Q

myeloproliferative disorder of platelets=?

A

essential thrombocythaemia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
53
Q

myeloproliferative disorder of white cells=?

A

chronic myeloid leukaemia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
54
Q

myeloproliferative disorder of reactive fibroblasts=?

A

myelofibrosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
55
Q

what is the age of presentation and survival of CML

A

40-50

18 months untreated, 5 years treated

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
56
Q

what is the age of presentation and survival of polycythaemia rubra vera

A

55-60

8-15 years treated

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
57
Q

what is the age of presentation and survival of myelofibrosis

A

60-70

1.4-5 years (treated)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
58
Q

what is the age of presentation and survival of essential thrombocythaemia

A

females 30-40
both sexes 50-70
>10 years treated

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
59
Q

what precursor is affected in CML

A

granulocyte precursor

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
60
Q

what is an overlapping myeloproliferative syndrome

A

overproduction predominating in one lineage, often accompanied by an increase in cell numbers (to a lesser degree) in others e.g. chronic myeloid leukaemia often accompanied by a high platelet count

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
61
Q

what is a myeloproliferative transformation

A

when a MP disorder transforms either into another MP state (PRV -> myelofibrosis) or to acute leukaemia (common in CML)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
62
Q

what is the clinical presentation of myeloproliferative disorders

A

all have: gout (increased nucleoprotein turnover) and (hypermetabolism) fatigue, weight loss, night sweats

myelofibrosis: splenomegaly, bone marrow failure (infections, anaemia, bleeding)

CML: splenomegaly, hepatomegaly

PRV: hyperviscosity: HAs, dizziness, tinnitus, visual disturbance, itching after hot bath, burning sensation in fingers and toes, facial plethora, splenomehagly

ET: bleeding (abnormal platelet function) or arterial venous thrombosis: HAs, atypical chest pain, light headedness, erythromelalgia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
63
Q

how are myeloproliferative disorders diagnosed

A

Polycythaemia rubra vera

  • Red cell mass increased
  • Bone marrow trephine

Chronic myeloid leukaemia

  • Morphology
  • Philadelphia chromosome

Essential thrombocythaemia

  • Persistent raised platelet count >600 x 109/l with no other explanation
  • Bone marrow trephine shows clumps of megakaryocytes

Myelofibrosis
-Bone marrow fibrosis ++ (trephine biopsy)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
64
Q

what is the treatment for ET

A

No treatment if no previous thrombosis and platelets <1500 x 109/l

Oral cytotoxics to control myelo-proliferation e.g. Hydroxyurea

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
65
Q

what is the treatment for CML

A

Cytotoxics e.g. Hydroxyurea or α-Interferon

Allogenic bone marrow transplant (may be curative)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
66
Q

what is the treatment for myelofibrosis

A

supportive: blood product transfusion

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
67
Q

what is polycythaemia

A

high Hb concentration, red cell count and haemtocrit

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
68
Q

in polycythaemia why must you measure red cell mass and plasma volume

A

to distinguish between:

low plasma volume Polycythaemia (low plasma volume and normal red cell mass) (aka reactive polycythaemia)

true polycythaemia (normal plasma volume, increased red cell mass)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
69
Q

what can cause reactive polycythaemia

A

acute: dehydration, diuretics, burns
chronic: obesity, HPTx, high alcohol and tobacco intake

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
70
Q

what can cause true polycythaemia

A

primary: polycythaemia rubra vera
secondary: hypoxia (high altitudes, chronic lung disease, cyanotic congenital heart disease, heavy smoking) or inappropriately high erythropoietin secretion (renal or hepatocellular carcinoma)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
71
Q

what test for polcythaemia

A

primary: platelet and white cell counts high
bone marrow aspirate/ trephine
spleen USS/ electrophoresis

secondary: arterial O2 sats
CXR
renal USS
Hb/ erythropoietin assay

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
72
Q

what can cause excess erythropoietin production with tissue hypoxia

A

chronic lung disease
congenital heart disease
altitude
high affinity Hb variant

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
73
Q

what can cause excess erythropoietin production with no tissue hypoxia

A

tumour (e,g, renal)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
74
Q

what can cause autonomous bone marrow proliferation causing polycythaemia

A

myeloproliferative polycythaemia

haemopoietic (stem cell disorder)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
75
Q

what are the possible complications of polcythaemia

A

thrombosis (risk greatest in PRV)
transformation into acute leukaemia
gout

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
76
Q

how do you stop malignant overproliferation and its consequences in polycythaemia

A

(to reduce thrombotic risk)

  • venesection
  • cytoxic chemo (oral hydroxyurea)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
77
Q

what chromosome in CML

A

philadelphia chromosome: t(9:22)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
78
Q

what results from monosomy 7

A

myelodysplasia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
79
Q

what results from trisomy 12

A

CLL

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
80
Q

what is immunoglobulin light chain restriction

A

κ or λ light chain ratio on surface of B-lymphocytes skewed if clonal population of B- cells expressing only one of these e.g. in lymphoma/ chronic lymphocytic leukaemia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
81
Q

how many immunolgobulin gene arrangements are seen in malignant lymphoid cells

A

one = clonality

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
82
Q

what crisis would require increased white cell production

A

Infection, inflammatory diseases, malignancy, injury or trauma (including surgery)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
83
Q

what are the functions of each type of white cell

A

Neutrophils: phagocytosis (non-specific) – release lysozyme
Lymphocytes: B – antibody production; T – cell-mediated
Monocytes: big – differentiate into macrophages – phagocytose
Eosinophils: secrete granules to attack parasites
Basophils: release histamine in inflammatory response

84
Q

what kind of leucocytosis should you worry about

A

non reactive: malignancy- clonal, intrinsic

85
Q

give some examples of leucocytosis that is not reactive?

A

Acute leukaemias – immature cells in blood AML if myeloid precursor (and different types), B or T ALL – maturation defect
Chronic leukaemia – mature cells in the blood, but too many – overproduction CML, CLL

86
Q

what is clonality

A

A single precursor cell multiplying rapidly and becoming the predominant cell

87
Q

are reactive disorder and leukaemias poly or monoclonal

A

reactive disorders= polyclonal

leukaemias= monoclonal

88
Q

what is pancytopenia

A

global reduction in all cells in the blood - reduced red and white cell and platelets

89
Q

what is aplastic anaemia

A

pancytopenia with hypocellular bone marrow but no infiltration/ marrow fibrosis
most idiopathic but can also be:
-congenital (fanconi anaemia)
-acquired (idiopathic, infection, EBV, HIV, parovirus, toxic exposure, drugs, pregnancy, transfusional graft vs host disease, sickle cell anaemia (aplastic crisis with parovirus))

environmental factors (damage induced by chemicals, drugs, viruses) that alter the antigen profile of haemopoietic stem/progenitor cells. This could lead to lymphocyte activation and an autoimmune reaction against haemopoietic stem/progenitor cells. Consequently, there is bone marrow failure affecting production of red cells, neutrophils and platelets.

90
Q

what can cause pancytopenia

A

increased destruction: hypersplenism

marrow infiltration: cancer, sarcoidosis

ineffective erythropoiesis: megaloblastic anaemia (B12/folate def)
myelodysplasia

aplastic anaemia

91
Q

what is MCH

A

mean corpuscular Hb

92
Q

what is MCHC

A

mean corpuscular Hb concentration

93
Q

what is RDW

A

recticulocyte count

94
Q

what can high haematocrit count mean

A

increased red cells

reduced plasma- alcohol, sudden stress, siuretics

95
Q

what happens to plasma volume in pregnancy

A

increases

96
Q

what can cause polycythaemia

A

-1st true polycythaemia – related to bone marrow defect- myeloproliferative polycytheamia (clonal abnormality)

-2nd polycythaemia – physiological response to hypoxia etc
- smoking can produce polycythaemia because carbon monoxide combines with haemoglobin
Lung disease, cardiac disease i.e low oxygen stimulation→ ↑ erythropoietin →↑ rbc production, can see similar effect with some epo secreting tumours – renal Ca
Iatrogenic epo use, testosterone supplements (compare male and female hb/rbc/hct indices)

97
Q

how does myeloproliferative polycythaemia present clincally

A

Global symptoms: fatigue, poor concentration(sluggish blood flow); headache; itching; redness; enlarged liver and /or spleen; or asymptomatic
hypermetabolic consequences sweats wt loss gout
thrombotic consequences eg portal vein thrombosis

98
Q

what is the risk in polycythaemia

A
  • increased viscosity of blood → stroke
  • increased risk of thrombosis esp in 1o true polycythaemia because all cells can be increased including platelets
  • gout – due to increased uric acid due to increased cell turnover
99
Q

what two mechanisms cause high platelets

A

Reactive response to infection, inflammation, malignancy

Primary clonal marrow disorder

100
Q

Two years later she returns complaining of blurred vision and painful finger tips. One of her toes has turned ‘blue’ overnight and she is concerned. This time blood count demonstrates a normal haemoglobin and white cell count, but her platelet count is 900 x 109/l.
Blood film examination reveals numerous giant platelets. All inflammatory markers are normal and she is otherwise well.

How do you explain Mrs Matthews blood results ?

A

primary marrow overproduction – clonal myeloproliferative disorder
(no positive inflammatory markers)

101
Q

what treatment to prevent thrombotic complications in thrombocytosis

A

antiplatelets- aspirin

102
Q

what happens in autoimmune pancytopenia e.g. SLE

A

autoimmune destruction of cell lineages through the recognition of multiple antigens on MATURE cells

103
Q

what causes lymphcytosis

A

infections acute and chronic

leukaemias and lymphomas (esp CLL)

104
Q

what causes atypical lymphocytes

A

(clear cytoplasm with blue rim)
EBV

CMV, HIV, parovirus, denguev

105
Q

what are risk factors for hodgkins lymphoma

A

EBV, affected sibling, SLE, post transplantation

106
Q

what is the peak age for HL

A 
young adults (15-24)
elderly
107
Q

what are the symptoms of HL

A

rubbery non tender nodes
systemc symptoms: sweats, weight loss, itch, fever, lethargy
alcohol induce node pain

108
Q

how is HL staged

A

Ann arbour system

109
Q

which has quicker onset heparin injections or warfarin tablets

A

heparin

110
Q

what does prothrombin time depend on

A

thromboplastin

111
Q

name a drig that impaired warfarin metabolism

A

alcohol

112
Q

what is lymphoplasmacytic lymphoma

A

aka waldenstroms macroglobulinaemia= slow growing low grade NHL

113
Q

what can cause hyperiscosity syndrome

A 
polycythaemia (PV)
leukocytosis (leukaemia) 
myeloma
wladenstroms
drugs: COCP, diuretics, chemo
114
Q

what increases plasma viscosity

A

any inflammation, anaemia or macrocytosis

115
Q

what paraproteins are made in waldenstroms

A

IgM paraprotein

116
Q

ciculating blasts/ lymphoblasts suggests what

A

lymphoblastic leukaemia

117
Q

which type of leukaemia commonly has CNS involvement

A

acute lymphblastic (cranial nerve palsies, meningism)

118
Q

pain where is common in myeloma

A

back pain- osteolytic bone lesions

119
Q

what electrolyte abnormality is common in myeloma

A

hypercalcaemia

120
Q

how does HUS present in biochem and blood tests

A 
microangiopathic haemolytic anaemia
low Hb
increased LDH
decreased haptoglobin
fragments on film 
decreased platelets
AKI (thrombosis of glomerular capillaries)
121
Q

what is the diagnostic traid for HUS

A

Microangiopathic haemolytic anaemia (Coombs’ test negative).
Thrombocytopenia.
Acute kidney injury (acute renal failure)

122
Q

what are neurological complications of HUS

A

altered mental state
CVA
seizures

123
Q

what happens to neutrophils post chemo

A

drop 7-14 days after

go back to normal in 3-4 weeks

124
Q 
20year old student complains of one week of severe sore throat, malaise, and lethargy and sweats. He is also complaining of painful and swollen neck glands.
On examination: looks unwell and has significantly enlarged 
Cervical and tonsillar glands.
Hb		158g/dL
WBC	20.0x109/L (raised)
Platelets	100x109/L (slightly low) 
slightly low neutrophils 
high lymphocytes
numerous aytpical lymphocytes

diagnosis?

A

EBV

125
Q

what are atypical lymphocytes

A

activated forms- are responding to something
are bigger, cytoplasm scallops around red cells, blue in colour (active proteins: T cells= enzymes, B cells= immunoglobulins)

126
Q

what activates lymphocytes

A

viral infections:
EBV (glandular fever)
HIV (always do HIV test where activated lymphocytes)
URTI (RSV, influenza, parainfluenza)

127
Q

what does infective mononucleosis cause (EBV)

A

infects throat epithelial and B lymphocytes
activates T cells in response

results in glandular fever= tonsilar exudate and lymphadenopathy

128
Q

how is EBV diagnosed

A

EBV serology (IgM)

129
Q

what might you get if you give amoxicillin in EBV

A

amoxycillin induced rash

130
Q

what can cause lymphadenopathy

A

regional:
- bacterial abscess
- mets

generalised:

  • viral
  • CTD/RA/sarcoidosis
  • vasculitis
  • lymphoid malignancy (lymphoma)
  • metastatic cancer
131
Q

what nodes are commonly affected in sarcoidosis

A

hilar
cervical
axillary

132
Q

what are nodes like in viral infection

A

Painful/tender
Hard
Regular
Not associated with overlying inflammation.
Not tethered.- not invading surrounding tissues

133
Q

what happens to neutrophils when there is infection/ tissue damage

A

toxic granulation and vacuolation (response to tissue damage: fight bacterial infection, granules release enzymes that fight bacterial/ repair tissues)

134
Q

what is the differential cell count

A

breaks it down into constituent cell

135
Q

what can cause a neutrophilia

A 
bacterial infection 
inflammation e.g. RA
trauma/ post op 
corticosteroids (decrease neutrophil margination in circulation) 
myeloproliferative diseases (rare)
136
Q

what is the most common cause of leukocytosis

A

neutrophilia

137
Q

what causes a lymphcytosis

A

viral infection esp pertussis (often associated with a mild neutropenia/ thrombocytopenia)
childhood response to infection (immune system not fully formed)
chronic lymphocytic leukaemia (smear cells)

138
Q

what is the main role of lymphocytes

A

fight infection- viral

139
Q

what constitutes a pancytopenia

A

low Hb
low WBC
low platelets

140
Q

when should you suspect HL over NHL

A

if young female
disease more common ABOVE the diaphragm
if B symptoms and itch, alcohol induce pain present

141
Q

how do you differentiate HL from NHL and the other causes of lymphadenopathy

A

node biopsy

142
Q

what is the histology of HL

A

nodular sclerosing

reed sternberg cells

143
Q

what is the histology of high grade NHL

A

immature lymphoid cells (blasts- big prominent nucleoli)

144
Q

what is the histology of low grade NHL

A

mature lymphcytes are the malignant clone (normal size mature lymphocytes)

145
Q

what does leucoerthroblastic mean

A

immature red (nucleated) and white cells (myelo blast)

146
Q

what can cause a leucoerythroblast blood picture

A

marrow infiltration

  • lymphoma
  • non haem mets
  • fibrosis (myelofibrosis- when scar tissue forms in bone marrow)- these push out immature cells
marrow stress: 
-sepsis
-bleeding
-shock 
(hypercellular marrow needed to make more blood)
147
Q

what does survival of HL depend on

A

stage (1= 90%, 4= 50%)

148
Q

how is HL staged

A

CT and PET scans

149
Q

what cell type are you most worried about dropping in pancytopenia

A

neutrophils- sepsis

150
Q

is marroe stroma derived from haemopoietic stem cell

A

no

151
Q

what are the immediate effects of cytotoxic drugs

A

Bone marrow suppression
Gut mucosal damage (mucositis)
Hair loss (alopecia)

152
Q

what are the consequences of bone marrow failure

A

anaemia
neutropenia (infections: gram _ve sepsis, hickman line cellulitis, viral herpes simplex, fungal - chronic neutropenia, spergilloma (ball of fungus due to aspergillus)
bleeding

153
Q

what bugs are you most worried about in neutropenia

A

gram -ve (esp from bowel in chemo- get mucositis- gut leaky, bug from here get into bloodstream)

154
Q

what is the treatment for neutropenic fever

A 
immediate Abx (esp against gram -ves)
tamozin and gentamicin

if worried its a resp infection add in clarithromycin

155
Q

what is given to prevent fungal infections in neutropenic patients

A

prophylatic anti fungal (spirgilloma can be fatal- forms cysts which has bleed as comp)

156
Q

what can cause pancytopenia

A

increased destruction:

  • immune
  • sepsis (commonest cause)

sequestration:
- hypersplenism (liver disease)

decreased production:

  • infiltration
  • B12 def
  • aplastic anaemia
  • drugs (chemo, immunosuppressants)
  • viruses (EBV)
  • radiation
157
Q

how do you find the cause of pancytopenia

A

Hx
exam- enlarged spleen, sepsis
Ix- reticulocyte count (shows if marrow working), B12/folate
abdo USS (spleen)

bone marrow exam:

  • hypocellular
  • hypercellular (normal cells increased + increased destruction/ replacement of normal cells with malignancy)
158
Q

what causes hypocellular bone marroe

A

drug induced aplasia
some viruses
aplastic anaemia

159
Q

what causes a hypercellular marrow

A 
infiltration 
peripheral destruction (hypersplenism)
160
Q

what supportive treatment for people on chemo with pancytopenia

A

prompt ABx if fever

red cell and platelet transfusions

161
Q

what is the role of the spleen

A

immune response
removal of effete (old) red cells
mediator of autoimmune disease (reticuloendothelial system- v vascular, senses antigens on cells as they circulate)
needed for immunity against encapsulated organisms

162
Q

what can splenectomy treat

A

ITP and AIHA

163
Q

what do you need to vaccinate against if doing a splenectomy

A

meningococcus, pneumococcus and haemophilus influenzae type b

164
Q

what does left shift mean on a blood film

A

when immature neutrophils are released e.g. in infection

165
Q

what causes malaria

A

P. falciparum parasite (seen inside RBCs)

166
Q

what can happen to the spleen in coeliac disease

A

hyposplenism

167
Q

what do monocytes look like

A

horse-shaped nucleus and steel-grey cytoplasm with vacuoles

168
Q

A 28 year old woman attending the antenatal clinic has a blood count performed with the results as follows: Haemoglobin low, MCV low, MCH low, white cell count normal, neutrophil count normal and platelet count normal. The serum ferritin is within the normal range.

what test next

A

Haemoglobin analysis by high performance liquid chromatography (HPLC)
The mild anaemia in the presence of a normal serum ferritin reflects the dilutional (physiological) anaemia of pregnancy. The normal serum ferritin makes iron deficiency unlikely. The disproportionate reduction in MCV and MCH compared to the haemoglobin indicates the possibility of a haemoglobinopathy trait (heterozygous) which is best investigated by haemoglobin analysis.

169
Q

what does a high Hb suggest

A

myeloproliferative polcythaemia vera

170
Q

what test for polycythaemia vera

A

JAK2 gene

171
Q

cytopenias and excess blasts suggest what

A

acute leukaemias

172
Q

what test for leukaemia

A

immunophenotyping (will clarify myeloid or lymphoid lineages)

173
Q

A 58 year old lady with a family history of hypothyroidism and atrophic gastritis presents with fatigue, macrocytosis and pancytopenia.
most likely diagnosis?

A

pernicious anaemia

B12 deficiency in patients with pernicious anaemia (autoimmune destruction of gastric parietal cells) can present with pancytopenia and not just an isolated macrocytic anaemia. It is not uncommon to elicit a personal or family history of other autoimmune disorders

174
Q

A 25 year old male has recurrent admissions to hospitals with pain in his legs and chest wall. On one occasion, he became extremely breathless and required a red cell exchange transfusion.
most likely diagnosis

A

sickle cell anaemia

Patients with sickle cell disease can have uncomplicated vaso-occlusive crisis in their musculoskeletal system. Sickle cell crisis in the pulomonary vasculature is a life-threatening emergency that required prompt therapy with exchange transfusion

175
Q

A 21 year old girl has a history of heavy periods and investigations indicate a defect in primary haemostasis. Her blood count is normal.
most likely diagnosis

A

von willebrands disease

disorder of primary haemostasis due to deficiency of von Willebrand factor which bridges platelets to sub-endothelial collagen following endothelial injury. This affects platelet adhesion at the site of injury.

176
Q

how does rituximab work

A

humanised monoclonal antibody directed against CD20, expressed on B cells and B cell lymphomas

177
Q

how does imatinibwork

A

tyrosine kinase inhibitor that is inhibits BCR-ABL-1 protein unique to chronic myeloid leukaemia

178
Q

how does aspirin work

A

irreversible inactivator of cyclooxygenase that is required for the production of prostaglandins and thromboxanes for platelet aggregation

179
Q

which drug:

It is an ADP antagonist

A

clopidogrel

180
Q

how does clopidogrel work

A

selectively inhibits the binding of adenosine diphosphate (ADP) to its platelet receptor and the subsequent ADP-mediated activation of the glycoprotein GPIIb/IIIa complex, thereby inhibiting platelet aggregation. This action is irreversible.

181
Q

which drug:

It irreversibly inactivates cyclooxygenase 1

A

aspirin

182
Q

which drug:

It is a highly selective direct inhibitor of activated factor X

A

Riveroxiban

183
Q

how does riveroxiban work

A

competitively inhibits factor Xa. Factor Xa along with factor Va for the prothrombinase complex which converts prothrombin to thrombin

184
Q

most likely cause:

Normal prothrombin time, prolonged partial thromboplastin time, normal platelet count, normal fibrinogen.

A

An isolated prolonged partial thromboplastin time reflects deficiency of factors involved in the ‘intrinsic’ pathway of coagulation (Factor VIII/IX) or the presence of an anti-phospholipid antibody (lupus anticoagulant) that is not associated with a bleeding phenotype

185
Q

most likely cause:

Prolonged prothrombin time, normal partial thromboplastin time, normal platelet count, normal fibrinogen

A

An isolated prolonged prothrombin time reflects deficiency of factors involved in the ‘extrinsic’ (but not ‘common’) pathway of coagulation and the only coagulation factor involved is therefore Factor VII. Deficiencies of common pathway factors (II,V and X) will cause prolongation of both the prothrombin and activated partial thromboplastin time

186
Q

Prolonged prothrombin time, prolonged partial thromboplastin time, low platelet count, low fibrinogen
most likely cause?

A

Disseminated intravascular coagulation

187
Q

what is the treatmen for AIHA

A

steroid (prednisolone) and folic acid

188
Q 
which of these is false: 
vit k is: 
-bsorbed in upper intestine 
-responsible for bile salt absorption 
-carboxylates specific preformed clotting factors 
-is antagonised by warfarin 
-is water soluble
A

vit k is fat soluble

189
Q

what usually causes febrile non haemolytic transfusion reactions

A

antibodies against donor leukocytes and HLA antigens

190
Q

who is most at risk of TACO

A

those with chronic anaemia and a compensatory high cardiac output

191
Q

what causes transfusion related acute lung disease

A

anti- leucocyte antibodies present in the donation that bind to the patients white cells and cause acute lung injury by degranulation of the affected neutrophils in the lungs. Pulmonary infiltrates are seen on CXR.

192
Q

A 68 year old woman with fatigue has a blood count performed and the results are as follows: Haemoglobin 87g/L (low), MCV 110 fl (high), white cell count 2.4 x 109/l (low), neutrophil count 1 x 109/l (low) and platelet count 100 x 109/l (low). The blood film shows macroovalocytes and hypersegmented neutrophils

A

Pernicious anaemia that causes B12 deficiency will result in impaired nuclear maturation affecting the development of all three haemopoietic lineages, so pancytopenia with macrocytic red cell development can occur in deficiency states. The blood film features are strikingly sensitive for B12/folate deficiency

193
Q

low Hb, high WBC, low neutrophils, low platelets
excess blasts and auer rods on film
58y/o man with fatigue

A

acute myeloid leukaemia

Marrow failure (resulting in the low Hb, Neutrophil and Platelet count) in acute myeloid leukaemia is caused due to the excessive proliferation of primitive cells (myeloblasts). Abnormalities of granulation in these blasts can be visualised in the form of Auer rods.

194
Q

A 52 year old man with a stroke has a blood count performed and the results are as follows: platelet count 930 x 109/l (v high) . The blood film shows an excess of platelets with some giant forms.

A

Essential thrombocythaemia

195
Q

what is essential thrombocythaemia

A

chronic myeloproliferative disorder characterised by excess production of platelets in the bone marrow and an increased risk of thrombosis.

196
Q

what is thrombocytopenia

A

reduced platelets

197
Q

does thrombophilia mean high platelets

A

NO it means increased thrombotic tendency

198
Q

what is HbS

A

glutamic acid to valine substitution in the beta chain of haemoglobin
indicates sickle cell anaemia (homozygous)

199
Q

red cells with loss of central pallor =?

A

spherocytes

200
Q

what causes sickle cell disease

A

a point mutation in the beta chain and this predisposes the haemoglobin to polymerisation resulting in sickled cells and reduced red cell survival

201
Q

what causes myelodysplasia

A

Acquired DNA mutations in haematopoietic stem cells= ineffective haematopoeisis

202
Q

what causes hereditary spherocytosis

A

Congenital mutation in structural red cell proteins= reduced cell deformity= membrane removed in spleen = spherocytes + reduced cell survival

203
Q

77 year old asymptomatic man is found to have a lymphocytosis.

A

chonic lymphocytic leukaemia

204
Q

67 year old man admitted to hospital with back pain, hypercalcemia and renal failure is found to have significant Bence Jones proteinuria

A

multiple myeloma

205
Q

what does bence jones protein in urine mean

A

the excretion of (clonal ie kappa or lambda only)immunoglobulin light chains- seen in myeloma

206
Q 
which of these is not a cause of hereditary thrombophilia:
antithrombin deficiency
protein C deficiency
protein S deficiency
Factor V Leiden
antiphospholipid syndrome
A

antiphospholipid syndrome

Haematology (27 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions