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Year 5: Pathology > Haem: Systemic Disease > Flashcards

Haem: Systemic Disease Flashcards

1
Q

What are the 4 common anaemia types relevant to haematology?

A

Iron deficiency anaemia
Leucoerythroblastic anaemia
microangiopathic anaemia
Heamolytic aneamia

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2
Q

What are the causes and lab findings of Iron deficiency aneamia?

A

Most common -blood loss (occult or not)
-GI, Gastric, Lung, Ovarian, Urinary tract cancers (renal/bladder)
Mennoraghia

IDA is caused by bleeding until proven otherwise -always act on low FE

Lab findings:
FBC- low heam, low RBC, Low MCV
heamtinics -Low ferritin, transferring. Increased TIBC
Blood film - microcytic, Hypochromic aneamia

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3
Q

What are the blood film findings of an iron deficiency anaemia?

A

Microcytic, hypochromic aneamia

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4
Q

Why is anemia relevant to heamatological cancers

A

Common first manifestation of heam disease-
as a result of low FE,
as a result of impaired heamopoesis
as a result of systemic inflammation

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5
Q

What is leucoerythroblastic anaemia? what is it often a sign of?

A

Aneamia where both RED and WHITE cells are low
“red and white cell PRECURSOR anaemia”

usually the 1st manifestation of bone marrow infiltration -
3 main BM infiltration causes:
cancer, myelofibrosis and infections

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6
Q

what are the 3 main causes of leucoerythroblastic anaemia?

A

usually the 1st manifestation of bone marrow infiltration -
3 main BM infiltration causes:

1) cancer -
heamatopoetic (leukemia/lumphoma/myeloma)
Non-heamatopoetic (secondary tumours -breast, prostate, bronchus)

2) Non malignant /Myelofibrosis

3) Severe infection -
military TB, severe Fungal infections

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7
Q

what are the blood film features of leucoerythroblastic anaemia?

A

Tear drop cells - poikocytosis

Nucleated RBC (immature) in Peripheral blood
Myelocytes (neutrophil precursors)
These are a result of the destruction of the barrier preventing precursor exit from the BM
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8
Q

What is haemolytic anaemia? Recall the 2 groups of aetiology and a few of their components

A

Heamolytic aneamia - shortnened red cell survival

aetiologies are either :

1) Inherited (defects of the RBC):
a) Membrane: Hereditary sphrocytosis
b) Cytoplasm/enzyme: G6PD deficiency
c) Haemoglobin: sickle cell, thalassemia

2) Aquired (increased breakdown due to environement)
a) Immune mediated
b) Non-immune mediated

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9
Q

What are laboratory features of all haemolytic anemias?

A

Not all might be present but these tend to be seen in haemolytic anaemias:

Anaemia
Reticulocytosis (BM is healthy so trying to replace)
Raised unconjugated Bilirubin
Raised LDH
Reduced haptoglobins
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10
Q

What are the lab features of an auto-immune mediated anaemia

A

Coombs /DAT test POSITIVE
Raised LDH
Reticulocytosis
AIHA :spherocytes on a film

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11
Q

what are the causes of autoimmune mediated haemolytic aneamia

A

Can often be idiopathic

Associated with systemic immune disease:
e.g.: SLE, CLL, Drug allergies, Mycoplasma infections

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12
Q

What are the lab features of non-immune mediated haemolytic anaemia?

A

DAT/Coombs negative
Thrombocytopenia
film: Shistocytes (RBC fragments)

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13
Q

What are causes of non-immune mediated haemolytic anaemia?

A

Most common worldwide is Malaria

Paroxysmal nocturnal haemoglobinuria (Ham’s test +ve)

Microangiopathic haemolytic anaemia (MAHA)
Adenocarninoma release toxic granules
HUS (e.coli)-triad of MAHA, Thrombocytopenia, AKI)
TTP (autoimmune)- pentad of MAHA, Thrombocytopenia, AKI, neuro issues and fever

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14
Q

What are 5 causes of neutrophillia?

A

1) corticosteroids
2) Underlying neoplasia
3) tissue inflammation (e.g. pancreatitis)
4) Myeloproliferative or leukaemic disorder
5) Pyogenic infection (MOST COMMON)
a few (viral, brucella, typhoid) dont

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15
Q

How can you use a blood film to assess a neutrophillia?

A

Neutrophillia can either be reactive (normal) or malignant

reactive -Neutrophilia
no immature cells, toxic granulation,

Malignant -
incredibly raised neutrophil count
Immature myelocytes, basophilia = AML

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16
Q

What are the main causes of easonophilia

A

1) Reactive-
allergies, parasites, underlying neoplasms

2) Primary (very rare)-
eosinophilic leukemia

17
Q

What are the main causes of raised basophils?

A

reactive-Poxvirus

malignant -CML

18
Q

What are the main causes of raised monocytes/monocytosis?

A

quite rare but:

Chronic infection such as TB, brucella
some viral: CMV
Sarcoidosis
Chronic myelomonocytic leukemia

19
Q

What are the causes of Lymphocytosis? and lymphopenia

A

Lymphocytosis -
Secondary (reactive): infection (EBV, CMV, Hepatitis, rubella, etc)
Autoimmune disorder
Sarcoidosis

Primary -Monoclonal proliferation (CLL, NHL, MM, ALL)

Lymphopenia -
HIV, autoimmune, Drugs (chemo), inherited

20
Q

How can a blood film help identify causes of lymphopenia

A

Want to take a film of peripheral blood in any lymphocytosis -

identify if cells are MATURE or IMMATURE

mature can be caused by:
Reactive/atypical -normal
Small and smear cells -CML

or IMMATURE - lymphoblast-probably ALL

21
Q

How do antibodies change depending on the cause of the lymphocytosis

A

Only valid for B-cells
But reactive lymphocytosis will be POLYclonal -kappa and lambda chains present

malignant - only kappa or lambda chains present

22
Q

What are some ways to classify leukemias?

A

Can be complicated because a long chain of cells can go cancerous .
this flash card TRIES to summarise it a bit maybe HELP

Can either be ; MYELOID or LYMPHOID
And if Lymphoid-either B or T cell related

then an important split is - is a precursor growing (e.g.: ALL) out of control or a mature cell (Multiple myeloma)

usually chronic and acute mean
Chronic - increased proliferation but NORMAL differentiation - gives many mature cells

Acute -proliferation increased and differentiation broken -many immature cells

23
Q

what methods are available to identify leukemias? Why bother?

A

From a biopsy, a lot can be found:

1) Morphology -
malignant/reactive, mature/immature,
2) Immunophenotyping
(use Cluster of differentiation of cells to identify what they are) -
Lymphoid/myeloid, T or B cell, mature/immature, stage of maturation
3)cytogenetics - look at DNA
identify translocations (Philadelphia Chr in CML
4) Molecular genetics-
identify individual mutations (e.g.: JAK2)

why?
can identify the actual disease
can help treatment -eg Philadelphia chr,
Help prognosis

24
Q

What is true polycythemia? and relative?

what are some causes of relative polycythemia

A

Polycythemia is defined as abnormally high heamatocrit -

relative - normal amount of RBC but lower plasma volume- raise the ration
causes: Diuretics, alcohol, obesity

true-normal amount of plasma but increased RBC -also raising the ration

25
Q

What is the difference between primary and secondary true polycythemia ? give a few causes of secondary true polycythemia

A

Primary - caused by an increased production of RBC directly -cancer

secondary -caused by an increase in EPO driving a rise in RBC

appropriate - in high altitude, hypoxia, cyanotic heart disease, COPD

inapropriate: Renal disease (cysts, cancers, inflammations

26
Q

what is a primary true polycythemia?

A

Polycythemia vera - haematological malignancy caused by excessive proliferation BUT NORMAL DIFFERENTIATION of RBC-called a myeloproliferative disorder

27
Q

What are 3 forms of myeloproliferative disorders? How can they be described

A

defined as Haematological malignancy caused by excessive proliferation BUT NORMAL DIFFERENTIATION of RBC

Polycythemia vera, Essential thrombocytosis and myelofibrosis

can be Philadelphia car positive -Polycythemia vera

or can be Philadelphia car negative- Essential thrombocytosis and myelofibrosis

all have mutations of the JAK2 gene -and constant tyrosine kinase activation causing proliferation

28
Q

What is myelofibrosis? What are some symptoms and diagnostic features?

A

Fibrosis of the bone marrow because of excessive proliferation of myelofibroblasts -depositing fibrin in BM until it cannot function

classically will cause anaemia, thrombocytopenia, leukopenia etc -and Sx associated with those

classic diagnostic -DRY tap when trying to biopsy
leucoerythroblastic anaemia
tear drop poikocytes

it has a bad prognosis

29
Q

what is essential thrombocytopenia? How does it present? how is it treated? what is is prognosis/complication?

A

A disorder of excessive proliferation of platelets leading to hypercoagulatable states

often asymptomatic it can present 1st time as a stroke/CVA/TIA/ etc

or other extreme-excessive bleeding as platelets stop working

treat with aspirin to thin the blood
and hydroxycarbamide to kill extra cells

it can become ALL after a few years

30
Q

How does polycythemia vera present? how is it treated?

A

Usually asymptomatic but the hyperviscous blood can cause:
headaches, light headedness, visual changes, fatigue, pruritus after bath
and stroke/tia/cva

treat with - venesection to remove some RBC
and hyxdroxycarabamide-kills off some RBC

Year 5: Pathology (2 decks)

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