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HAEM - Abbie > 2. Haematology of systemic disease > Flashcards

2. Haematology of systemic disease Flashcards

1
Q

Case; A patient with lymphoma is referred by the GP with new onset jaundice, anaemia and raised LDH. What are the possible scenarios?

A
  1. Lymphoma stage 4 with bone marrow and liver involved (hepatic)
  2. Lymphoma with nodes compressing the bile duct plus anaemia of inflammation (post-hepatic)
  3. Lymphoma with acquired autoimmune haemolytic anaemia (AIHA) (pre-hepatic)
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2
Q

Many causes of cancer are associated with anaemia. Anaemia may be the first presentation of cancer. What types of anaemia might this be?

A

Iron deficiency, anaemia of chronic disease, leucoerythroblastic anaemia, haemolytic anaemia. Cancer could also cause secondary polycythaemia - renal cell cancers and liver cancer could inappropriately secrete EPO resulting in polycythaemia.

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3
Q

What is the most common cause of IDA?

A

Occult blood loss is the MOST COMMON cause

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4
Q

What are causes of occult blood loss?

A

GI cancers (e.g. gastric, colon); urinary tract cancers (e.g. renal cell carcinoma, bladder cancer)

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5
Q

What are laboratory findings of IDA?

A

o Low ferritin
o Low transferrin saturation
o High TIBC

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6
Q

IMPORTANT: iron deficiency is BLEEDING until proven otherwise. True or false?

A

True

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7
Q

What is leucoerythroblastic anaemia in simple terms?

A

In simple terms: ‘red cell and white cell precursor anaemia’. (This causes a variable degree of anaemia)

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8
Q

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

A

Teardrop red blood cells (aniso and poikilocytosis);

nucleated RBCs; immature myeloid cells

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9
Q

Why are nucleated red blood cells an abnormal finding?

A

RBCs don’t normally have a nucleus

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10
Q

Leucoerythroblastic anaemia tends to occur as a result of bone marrow infiltration. What are causes of bone marrow infiltration?

A
  1. Cancer: haematopoietic (leukaemia, lymphoma, myeloma), non haematopoietic (breast, bronchus, prostate).
  2. Myelofibrosis: massive splenomegaly, dry tap on BM aspirate
  3. Severe infection: miliary TB and severe fungal infection
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11
Q

What is often the first manifestation of some kind of malignancy involving the bone marrow?

A

Leucoerythroblastic anaemia

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12
Q

What is haemolytic anaemia defined as?

A

Defined as shortened red cell survival

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13
Q

What are common distinguishing features of haemolytic anaemia?

A

Anaemia; reticulocytosis; raised unconjugated bilirubin; raised LDH; reduced haptoglobins

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14
Q

What is going on in the bone marrow in haemolytic anaemia?

A

in haemolytic anaemia, the blood cells are being broken down in the peripheral circulation so the bone marrow itself is perfectly healthy and is able to compensate for blood loss by releasing more red cells, including reticulocytes

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15
Q

What intracellular enzyme is released when red cells break down?

A

LDH

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16
Q

What are the two main groups of haemolytic anaemia?

A

INHERITED (defects of red cell) and ACQUIRED (defects of the environment)

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17
Q

Where is the inherited defect in hereditary spherocytosis, resulting in haemolytic anaemia?

A

RBC membrane

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18
Q

Where is the inherited defect in G6PD deficiency, resulting in haemolytic anaemia?

A

RBC cytoplasm/enzymes

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19
Q

Where is the inherited defect in sickle cell disease and thalassemia, resulting in haemolytic anaemia?

A

RBC haemoglobin

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20
Q

What are the two types of acquired haemolytic anaemia?

A

Immune mediated and non-immune mediated

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21
Q

What test can be used to distinguish between immune mediated and non-immune mediated haemolytic anaemia?

A

DAT or Coombs’ test

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22
Q

What does DAT +ve mean in acquired haemolytic anaemia?

A

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

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23
Q

Autoimmune haemolytic anaemia results in the formation of what shape RBCs?

A

Spherocytes

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24
Q

Autoimmune haemolytic anaemia is associated with several systemic disorders such as:

A

Cancer of the immune system (e.g. lymphoma); disease of the immune system (e.g. SLE); infection (disturbs the immune system)

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25
Q

Summarise the features of AIHA

A

Spherocytes, anaemia, reticulocytosis, raised bilirubin unconjugated, raised LDH, positive DAT

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26
Q

What are possible causes of positive DAT?

A

Idiopathic, underlying lymphoma, chronic lymphocytic leukaemia, systemic auto-immune disease e.g. SLE

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27
Q

What result will DAT have in non-immune haemolytic anaemia?

A

Negative. The red cell breakdown is NOT caused by the immune system

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28
Q

What are causes of non-immune haemolytic anaemia?

A

Infection (such as malaria) can cause red cell haemolysis. This can also be caused by Microangiopathic Haemolytic Anaemia (MAHA)

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29
Q

What are key features of MAHA?

A

Usually caused by underlying adenocarcinoma; red cell fragments; low platelets; DIC/bleeding

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30
Q

What is the mechanism of MAHA?

A
  • Underlying adenocarcinoma can release lots of procoagulant cytokines that activate the coagulation cascade
  • Rather than causing focused coagulation at sites of vessel damage, this erratic cytokine release will cause disseminated intravascular coagulation (DIC)
  • Activation of the coagulation cascade in various parts of the microvasculature will result in the production of fibrin strands
  • Red cells are then pushed through these fibrin strands by the blood pressure and they will fragment
  • This presentation should raise suspicion of an underlying adenocarcinoma
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31
Q

What is true polycythaemia?

A

Raised red cell mass

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32
Q

What is the difference between primary and secondary polycythaemia?

A

Secondary polycythemia means that some other condition is causing your body to produce too many red blood cells. Primary polycythemia is genetic. It’s most commonly caused by a mutation in the bone marrow cells, which produce your red blood cells.

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33
Q

What are the EPO levels in primary and secondary polycythaemia?

A

In secondary polycythemia, your EPO level will be high and you’ll have a high red blood cell count. In primary polycythemia, your red blood cell count will be high, but you’ll have a low level of EPO(??).

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34
Q

Causes of secondary polycythaemia?

A

Hepatocellular cancer, bronchial cancer, renal cancer, high altitude, hypoxic lung disease, cyanotic heart disease

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35
Q

Cause of primary polycythaemia and the mutation?

A

Polycythaemia vera (PV), clonal myeloproliferative disorder acquired mutations in JAK2 (Janus kinase)

36
Q

Case: Female aged 39 treated breast cancer 4 years previously. Recent onset of jaundice and hepatomegaly. GP bloods: Hb = 87g/L; bilirubin 50 micromol/L conjugated; DAT -ve; blood film shows nucleated red blood cells (see notes). What is the likely explanation for this anaemia?

A

Leucoerythroblastic anaemia(?). This is likely to be caused by bone metastases. DAT is negative showing that it is NOT caused by immune-mediated destruction of red blood cells. She probably also has liver metastases that have led to obstructive jaundice.

37
Q

What are normal (mature) white blood cells?

A

Phagocytes: granulocytes (neuts, eos, basos), monocytes. Immunocytes: T lymphocytes, B lymphocytes, NK cells.

Granulocytes are polymorphonuclear. It is normal to see lymphoblasts and myeloblasts in the bone marrow but they should be at a relatively low percentage. Blast cells should not be seen in peripheral blood.

38
Q

Where do you normally find blast cells?

A

It is normal to see lymphoblasts and myeloblasts in the bone marrow but they should be at a relatively low percentage. Blast cells should not be seen in peripheral blood.

39
Q

What immature cells are normal in bone marrow in appropriate % but not normal in peripheral blood?

A

Blasts (myelo- or lympho-), promyelocytes, myelocytes

40
Q

What should you do if WBCs are raised?

A

Request a blood film

41
Q

What is the difference between chronic lymphocytic anaemia and acute myeloid leukaemia?

A

If white blood cell counts are abnormal, request a blood film. Mature white cells will be grossly raised in CHRONIC leukaemia. Immature blast cells will be raised in ACUTE leukaemia

42
Q

What are causes of neutrophilia?

A

Corticosteroids (due to demargination); underlying neoplasia; tissue inflammation (e.g. colitis, pancreatitis); myeloproliferative/leukaemia disorders; infection (various acute bacterial, fungal and certain viral infections can cause neutrophilia)

43
Q

Neutrophilia can occur in localised and systemic infections, true or false?

A

True

44
Q

What diseases characteristically DO NOT cause neutrophilia?

A

Brucella, typhoid, many viral infections

45
Q

How does reactive neutrophilia present?

A

Band cells (stage in the maturation of neutrophils) - the presence of increased numbers of band cells (known as a ‘left shift’) shows that the bone marrow has been signalled to release more WBCs; toxic granulation; signs of infection/inflammation

46
Q

How does malignant neutrophilia present? (How do you differentiate between AML and CML)?

A

Neutrophilia + basophilia + immature myelocytes + splenomegaly is suggestive of a myeloproliferative disorder (CML). NOTE: as CML is a bone marrow disease, you may also see raised Hb and raised platelets if all lineages are part of the proliferative process

Neutrophilia + myeloblasts suggests AML.

47
Q

How prevalent is monocytosis?

A

RARE

48
Q

When might monocytosis be seen?

A

May be seen in certain chronic infections and primary haematological disorders:

o TB, brucella, typhoid
o Viral: CMV, VZV
o Sarcoidosis
o Chronic myelomonocytic leukaemia (MDS)

49
Q

What are causes of reactive eosinophilia?

A

Parasitic infection; allergic diseases (e.g. asthma, rheumatoid arthritis, pulmonary eosinophilia); underlying neoplasms (e.g. Hodgkin’s, T cell lymphoma, NHL); drug reaction (e.g. erythema multiforme)

50
Q

What causes chronic eosinophilic leukaemia?

A

Eosinophils are part of the clone; FIP1L1-PDGFRa Fusion gene

51
Q

What is an infective cause of raised neutrophils?

A

bacterial infections

52
Q

What is an inflammatory cause of raised neutrophils?

A

Auto-immune tissue necrosis

53
Q

Neutrophils are raised in which types of neoplasia? ***

A

All types? (An elevated neutrophil-to-lymphocyte ratio is considered a prognostic indicator for patients with cancer.)

54
Q

What is a myeloproliferative cause of raised neutrophils?

A

CML

55
Q

What is an infective cause of raised eosinophils?

A

Parasitic infections

56
Q

What is an inflammatory cause of raised eosinophils?

A

Allergic (asthma, atopy, drug reactions)

57
Q

What is a neoplastic cause of raised eosinophils?

A

Hodgkin’s NHL

58
Q

What is an infective cause of raised basophils?

A

Pox viruses

59
Q

What is a myeloproliferative cause of raised basophils?

A

CML

60
Q

What is an infective cause of raised monocytes?

A

Chronic (TB, Brucella)

61
Q

What is a myeloproliferative cause of raised monocytes?

A

CML

62
Q

Which type of lymphocytosis is more common?

A

Secondary (reactive) more common than primary

63
Q

Which type of lymphocytosis is a polyclonal response to infection and results in chronic inflammation?

A

Secondary

64
Q

Which type of lymphocytosis is a monoclonal lymphoid profileration (e.g. CLL, NHL)?

A

Primary

65
Q

What investigations can you do in a step-by-step interpretation of lymphocytosis?

A

Clinical, FBC, light microscopy/morphology, flow cytometry, molecular genetics. (imaging might be useful for visualising enlarged lymph nodes)

66
Q

How can we interpret lymphocytosis from a clinical exam?

A

Symptoms suggestive of infection or lymphoma, lymphadenopathy/splenomegaly

67
Q

How can we interpret lymphocytosis from FBC?

A

Degree of lymphocytosis

68
Q

How can we interpret lymphocytosis from light microscopy/morphology?

A

Mature cells or primitive lymphoblasts

69
Q

How can we interpret lymphocytosis from flow cytometry?

A

Lineage - B or T cells. Stage of differentiation.

70
Q

How can we interpret molecular genetics?

A

Rearranged: T cell receptor or immunoglobulin gene

71
Q

What are causes of reactive lymphocytosis?

A
  • Infection: EBV, CMV, Toxoplasmosis, infectious hepatitis, rubella, Herpes,
  • Autoimmune disorders (more likely to cause lymphopaenia)
  • Sarcoidosis
72
Q

In morphology of infectious mononucleosis, what kind of lymphocytes can be seen?

A

Mature lymphocytes, could be reactive or atypical lymphocytes

73
Q

In morphology suggesting CLL or NHL, what kind of lymphocytes can be seen?

A

Mature lymphocytes - Small lymphocytes and smear cells

74
Q

In morphology suggesting ALL, what kind of lymphocytes can be seen?

A

Immature lymphocytes - lymphoblasts

75
Q

How does immunophenotyping using flow cytometry work?

A
  • Monoclonal antibody targeted at different antigens will be washed over the cells.
  • The monoclonal antibodies are fluorescently labelled.
  • Cells are passed through a flow cytometer and the fluorescence is recorded.
  • Depending on which antigens are present on the cells, you can determine the stage of maturation.
76
Q

An individual B cell will express both Kappa OR Lambda light chains, true or false?

A

False, an individual B cell will express either Kappa OR Lambda light chains (not both)

77
Q

What happens to the kappa and lambda light chains in response to an infection?

A

In response to an infection, you will get a polyclonal B cell response so immunophenotyping will reveal a mix of kappa and lambda light chains. The mix is roughly even

78
Q

Usually what % of B cells are kappa and lambda?

A

usually 50-60% kappa and 40-50% lambda

79
Q

What is a raised lymphocyte count and a roughly even mix of kappa and lambda suggestive of?

A

Reactive lymphocytosis

80
Q

What will lymphoproliferative disorders shown in immunophenotyping?

A

In lymphoproliferative disorders, if a kappa expressing B cell becomes malignant and excessively proliferates, the proportion of kappa light chains will greatly increase. So, immunophenotyping in lymphoproliferative disorders will show an overwhelming majority of EITHER kappa OR lambda.

81
Q

What is the difference in immunophenotyping results in an infection vs lymphoproliferative disorder?

A

A raised lymphocyte count with a roughly even mix of kappa and lambda is suggestive of a reactive lymphocytosis. Lymphoproliferative disorders will show an overwhelming majority of EITHER kappa OR lambda

82
Q

Summarise anaemia due to decreased production:*

A

BM infiltration, leucoerythroblastic film

83
Q

Summarise anaemia due to increased destruction: *

A
  • Lab markers of haemolysis (retics, LDH, Bilirubin)
  • Immune (spherocytes and DAT) lymphoid tumours
  • Microangiopathic (red cell fragments) adenocarcinoma
84
Q

Summarise polycythaemia: *

A 
o	Primary (MPD)
o	Secondary (renal tumours)
85
Q

What Ix can help diagnose lymphocytosis?*

A

Immunophenotyping - Flow cytometry

HAEM - Abbie (16 decks)

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