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High Yield Path - Haem > Haem: Haematology of Systemic Disease (Laz) > Flashcards

Haem: Haematology of Systemic Disease (Laz) Flashcards

1
Q

What are the principles of haematological disorders

A

Either excess or deficiency of:
-** Soluble proteins **
- Factor VII excess —-> Haemophilia A
- Protein C excess —>Pro-thombotic disease

**- Cellular Components **
- Erythrocytes —> polycythaemia v anaemia
- Leucocytes —> leukaemia
- Lymphocytes -> leukaemia v lymphopenia
- Platelets ——-> essential thrombocytopenia or ITP

Additionally: autoimmune disorders

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2
Q

Differentiate between Primary and Secondary haematological disorders

A

Primary: inherited or acquired (e.g. Haemophilia, Sickle cell, Polycythaemia Vera)

**Secondary: ** normal haematological system responding to environmental changes or other non-haem pathology (e.g. inflammation driven Factor VIII excess, high altitude polycythaemia)

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3
Q

List some ways in which lymphoma can cause jaundice.

A
  • Direct liver involvement
  • Compression of the bile duct
  • Causing autoimmune haemolytic anaemia
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4
Q

Which types of cancer are associated with causing secondary polycythaemia?

A
  • Renal cell carcinoma
  • Liver cancer
  • Due to the production of EPO
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5
Q

Which types of anaemia can be a first presentation of cancer and are caused by cancer?

A
  • Iron deficiency
  • Anaemia of chronic disease
  • Haemolytic anaemia
  • Leucoerythroblastic anaemia
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6
Q

What is the most common cause of iron deficiency anaemia?

A

Occult blood loss (e.g. GI cancers, urinary tract cancers)

*Iron deficiency anaemia is bleeding until proven otherwise! *

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7
Q

What are the typical laboratory findings of iron deficiency anaemia?

A
  • Microcytic hypochromic anaemia
  • Low ferritin
  • Low transferrin saturation
  • High TIBC
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8
Q

What is leucoerythroblastic anaemia?

A

Anaemia characterised by the presence of red and white cell precursors released form bone marrow.

As premature RBCs are less functional = anaemia

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9
Q

What are the morphological features of leucoerythroblastic anaemia seen on blood film?

A
  • Tear drop red blood cells (aniso- and poikilocytosis)
  • Nucleated RBCs (=premature RBCs)
  • Immature myeloid cells
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10
Q

What does leucoerythroblastic anaemia tend to be caused by?

A
  • First manifestation of **bone marrow infiltration commonly caused by malignancy or mets ** (leukaemia, lymphoma, myeloma, solid tumours)
  • Myelofibrosis
  • Severe infection (e.g. miliary TB, fungal infection)
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11
Q

Define haemolytic anaemia.

A

Anaemia caused by reduced red blood cell survival

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12
Q

List some key laboratory findings in haemolytic anaemia.

A
  • Anaemia
  • Raised reticulocytes (not nucleated as seen in leucoerythroblastic anaemia)
  • Raised unconjugated bilirubin - prehepatic
  • Raised LDH (intracellular enzyme)
  • Low haptoglobins (important for binding to free Hb, due to high levels of haemolysis the levels of haptoglobins decrease as its all binding to a lot of free Hb)
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13
Q

What are the two main groups of haemolytic anaemia? List some examples.

A

Inherited (defects with the cell)

  • Hereditary spherocytosis (membrane problem)
  • G6PD deficiency (enzyme problem)
  • Sickle cell disease, thalassemia (haemoglobin problem)

Acquired (defects with the environment)

  • Immune-mediated
  • Non-immune mediated
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14
Q

Which test distinguishes immune-mediated and non-immune mediated haemolytic anaemia?

A

DAT or Coombs’ test

DAT +ve means that the haemolytic anaemia is mediated through immune destruction of red cells

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15
Q

What morphological change is seen on the blood film of patients with autoimmune haemolytic anaemia?

A

Spherocytes
Agglutinatinon
(alongside DAT +ve) = AIHA

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16
Q

Outline the differences between the 2 types of AIHA

A
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17
Q

List some systemic diseases that can cause autoimmune haemolytic anaemia.

A
  • Cancer involving the immune system (e.g. lymphoma)
  • Disease of the immune system (e.g. SLE)
  • Infections (disturbs the immune system)
18
Q

Lab results for Non-immune mediated anaemia

A

DAT -ve
RBC fragments (schistocytes)
Thrombocytopenia
Hereditary spherocytosis

19
Q

List some causes of non-immune haemolytic anaemia.

A
  • Infection (e.g. malaria - parasite enters RBC causing it to die)
  • Paroxysmal nocturnal haemoglobinuria
  • Microangiopathic haemolytic anaemia (MAHA)
20
Q

2 Main causes of Micro-Angiopathic Haemolytic Anaemia (MAHA).

A
  • Adenocarcinoma
    - DIC, Red cell fragmentation, Low platelets
  • Haemolytic Uraemic Syndrome
    - Triad: MAHA + AKI + thrombocytopenia
    - Caused by e.coli toxin O157
21
Q

Outline the how adenocarcinomas can result in MAHA.

A
  • An underlying adenocarcinoma produces procoagulant cytokines that activate the coagulation cascade
  • This leads to DIC and the formation of fibrin strands in various parts of the microvasculature
  • Red cells will be pushed through these fibrin strands and fragment

NOTE: always consider underlying adenocarcinoma in any patient presenting with MAHA

22
Q

List some causes of secondary polycythaemia.

A
  • Cancer (renal, hepatocellular, bronchial)
  • High altitude
  • Hypoxic lung disease
  • Congenital cyanotic heart disease
23
Q

How would you interpret an abnormal WBC count

A
  1. Look at the FBC - any changes in Hb, Platelets as well? Any changes in lineages in WBC (myeloid e.g. neutrophils) v lymphocytes)
  2. Look at blood film - normal or abnormal morphology (chronic vs acute leukaemia)
24
Q

What is the main difference seen in the blood film of patients with acute and chronic leukaemia?

A
  • Chronic - mature white cells are raised
  • Acute - immature blast cells are raised
25
Q

List the 2 main causes of neutrophilia.

A

Reactant Neutrophilia
- Infection (most likely pyogenic infection)
- Corticosteroids
- Underlying neoplasia
- Tissue inflammation (e.g. colitis, pancreatitis)

Malignant
- Myeloproliferative
- Leukaemic disorders

26
Q

List some infections that characteristically do not cause neutrophilia.

A
  • Brucella
  • Typhoid
  • Many viral diseases
27
Q

List some key features of a reactive neutrophilia on a blood film.

A
  • Band cells (presence of immature neutrophils (band cells) show that the bone marrow has been signalled to release more WBCs)
  • Toxic granulation
  • Clinical signs of infection/inflammation
28
Q

What are some key blood film and clinical features suggestive of a myeloproliferative disorder?

A
  • Neutrophilia
  • Basophilia
  • Immature myelocytes
  • Splenomegaly

NOTE: you may see raised Hb and raised platelets in CML if it affects those lineages

29
Q

What are some key blood film features suggestive of AML vs CML?

A

AML
* Neutropenia
* Myeloblasts

CML
* Neutrophilia/basophilia
* Splenomegaly

30
Q

List some causes of monocytosis.

A
  • Bacteria: TB, Brucella, typhoid
  • Viral: CMV, VZV
  • Sarcoidosis
  • Chronic myelomonocytic leukaemia
31
Q

List some causes of reactive eosinophilia.

A
  • Parasitic infection
  • Allergy (e.g. asthma, rheumatoid arthritis)
  • Underlying neoplasms (e.g. Hodgkin’s lymphoma, T cell lymphoma, NHL)
  • Drug reaction (e.g. erythema multiforme)
32
Q

Which gene mutation causes chronic eosinophilic leukaemia?

A

FIP1L1-PDGFRa fusion gene

33
Q

Which type of virus typically causes basophilia?

A

Pox viruses

34
Q

What investigations are typically used when investigating lymphocytosis?

A
  • Clinical examination
  • FBC
  • Light microscopy
  • Flow cytometry (identify lineage and stage of differentiation)
  • Molecular genetics (TCR or Ig gene)
35
Q

List some causes of reactive lymphocytosis.

A
  • Infection (EBV, CMV, toxoplasmosis, rubella, HSV)
  • Autoimmune diseases (NOTE: these are more likely to cause lymphopaenia)
  • Sarcoidosis
36
Q

How would the lymphocytes seen on a blood film due to a viral infection be different from leukaemia/lymphoma?

A
  • Viral infection: reactive lymphocytes (more abundant cytoplasm - lower N:C ratio) or atypical lymphocytes (EBV)
  • CLL or NHL: small lymphocytes (large N:C ratio) and smear cells
37
Q

Outline how flow cytometry is used to identify cell types.

A
  • Fluorescently labelled monoclonal antibodies targeted at different antigens are washed over the cells
  • Cells are passed through the flow cytometer and the fluorescence is recorded
  • Dependent on the antigens present on the cells, you can identify the stage of maturation
38
Q

What are the CD markers that differentiate between B and T Cells

A

B cells —> CD19, CD22
T cells —> CD2

39
Q

What is light chain restriction?

A
  • An individual B cell will either express kappa or lambda light chains (not both)
  • In response to an infection, you will get polyclonal B cell response so there will be a roughly even mixture of kappa and lambda light chains
  • In lymphoproliferative disorders, monoclonal proliferation of a B cell expressing only one type of light chain (e.g. kappa) will mean that the proportion of kappa relative to lambda will increase (e.g. showing an overwhelming majority of kappa)
40
Q

Causes of Lymphopenia

A
  • HIV
  • Autoimmune disorders
  • Inherited immune deficiency syndromes
  • Drugs (chemotherapy)
41
Q

Female aged 39 treated for breast cancer 4 years previously presents with recent onset jaundice and hepatomegaly. The following are her blood results:
Hb - 87g/l
Reticulocyte 15x10^9 (20-92)
Bilirubin 50mm/L
DAT -ve
Blood film in picture

What is the diagnosis:
A) Iron deficiency anaemia
B) Anaemia of chronic disease
C) BM mets from breast cancer
D) MAHA
E) AIHA

A

B) BM mets from breast cancer (accounts for low reticulocyte count - as BM is infiltrated, nucleated RBCs seen in film)

  • Not IDA as would not expect jaundice, would not give nucleated red blood cells in PB
  • Not MAHA as would not get leucoerythroblastic problems in blood as BM is healthy
  • Not AIHA as DAT is -ve

High Yield Path - Haem (7 decks)

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