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Haematology > Interactive cases: Prof Bain and Prof McDonald > Flashcards

Interactive cases: Prof Bain and Prof McDonald Flashcards

1
Q

How to distinguish between thymoma and ALL?

A

In thymoma you wouldn’t get high WCC unlike in ALL

ALL:

  • high WCC count
  • Blast cells
  • possible anaemia/thrombocytopaenia due to bone marrow infiltration
  • possible enlagment of organs i.e. Testes, thymus

**NB: T-cell ALL can cause a thymoma**

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2
Q

Immunophenotyping vs cytochemistry

A

Immunophenotyping: used to distinguish between AML and ALL. uses flow cytometry.

Cytochemistry: looks for specific things in cells using stains. not used much anymore.

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3
Q

What does high WCC in acute leukamiea signify in terms of prognosis?

A

Bad prognosis that reduces the chances of cure

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4
Q

What does a coagulation screen include?

A

APTT

PT

Bleeding time

*does nOT include platelet count - this is done in FBC*

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5
Q

Coagulation screen in DIC

A
  • APTT:
    • initially SHORT - because there’s so much activation of the clotting factors
    • then later prolonged as clotting factors get used up
  • PT
    • prolonged
  • Bleeding time
    • prolonged
  • Fibrinogen
    • low
  • FDP (D-dimer)
    • elevated
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6
Q

What test confirms DIC?

A

D-dimer

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7
Q

Which leukamiea is associated with DIC?

A

APML

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8
Q

Which ethnicities tend to have lower neutrophil counts?

A

Afrocarribbeans and Africans

ethnic neutropenia whereby Africans and Afro-Caribbeans could have lower neutrophil count and this might be NORMAL.

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9
Q

Name a key haemoatological cause of macrocytic non-megaloblastic anaemia?

A

Myelodysplastic syndrome

Also multiple myeloma and myeloproliferative disorders

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10
Q

What causes megaloblastic anaemia?

A

Vitamin B12 or folate deficiency or cytotoxic drugs

there are no haematological malignancies that cause megaloblastic anaemia

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11
Q

Anti IF vs anti parietal cell antibodies

A

anti-IF: more specific for pernicious anaemia

anti-parietal cell: more sensitive

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12
Q

Platelet count and RBC in essential thrombocythaemia vs PCV

A

PCV:

  • erythrocytosis
  • thrombocytosis

ET:

  • isolated thrombocytosis
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13
Q

PCV treatment

A
  • venesection: lower the Hb
  • hydroxycarbamide: lowers platelet count
  • aspirin to reduce thrombosis risk
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14
Q

Testicular swelling, 3-5 year old

A

ALL

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15
Q
A
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16
Q

Auer rods

A

AML

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17
Q

Philadelphia chromosome

t (9:22)

BCR-ABL-1

Left shift

A

CML

*left shift means that you seei mmature myeloid cells in the bloodstream*

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18
Q

Smear cells/smudge cells

A

CLL

or small cell lymphoma

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19
Q

JAK2+

High haematocrit

flushed appearance

strokes/budd chiari

A

PCV

Budd chiari: Hepatic veins (veins that drain the liver) are blocked or narrowed by a clot (mass of blood cells).

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20
Q

high platelets

strokes

may have jak2 mutation

A

ET

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21
Q

Dry tap, teardorp cells (dacrocytes), massive splenomegaly

A

Myelofibrosis

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22
Q

Painful LNs with alcohol

Reed0sternberg cells

EBV+

A

Hodgkin’s

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23
Q

t(14,18); centroblasts

A

Follicular lymphoma

*low grade B cell NHL

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24
Q

t(11,14)

mantle cells

A

mantle cell lymphoma

*aggressive B cell NHL

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25
Q

t (8,14)

starry sky appearance

EBV+

HIV+

A

Burkitt’s

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26
Q

CRAB+

Bence jones protein\IgG/A > 30

A

Multiple myeloma

**rmb IgM - waldernstrom’s

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27
Q

No CRAB

Paraprotein < 30 (but elevated)

A

MGUS

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28
Q

WBC count normal range

A

Females: 4.5 to 11.0 × 109/L

Males: 4.5 to 11.0 × 109/L

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29
Q

Hb normal range: males and females

A

Females: 12.0 to 15.5 grams per deciliter (120-155 grams/litre)

Males: 13.5 to 17.5 grams per deciliter (135-175 grams/ litre)

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30
Q

MCV normal range

A

Females: 80-96 femtolitres per cell

Males: 80-96 femtolitres per cell

**basc above 100 is marocytosis

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31
Q

Platelet count normal range

A

Males + females: 150-400 x 109/L

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32
Q

How to distingiush between PCV and chronic hypoxia induced polycythaemia (secondary polycthaemia)?

A

PCV:

  • thrombocytosis + erythrocytosis
  • low erythropoietin
  • Jak 2 mutation

In secondary polycythaemia you would get:

  • isolated erythrocytosis
  • high erythropoietin
  • no Jak 2 mutation
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33
Q

Test to confirm PCV

A

FBC:

  • High HB + Hct
  • high platelets
  • high MCV
  • WBC normal (or moderately raised)

JAK2 mutation - DIAGNOSTIC- this is the first one to do; usually this is enough

Bone marrow aspirate + trephine biopsy

  • Increased cellularity- i..e no fat spaces, all being occupied by mature cells
  • moderate reticulin fibrosis

serum EPO - low

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34
Q

Reactive neutrophilia vs CML vs AML

A

Reactive

  • neutrophillia i.e. 15-20)
  • band cells (neutrophils without segmented nucleus)
  • toxic granuloation
  • vacuolation
  • no immature cells

CML

  • neutrophilia i.e. (50-500 x 109/L)
  • basiphilia
  • immature myelocytes BUT NO excess myeloblasts (< 5%) in the bone marrow
  • eosinophilia

AML

  • neutrophilia i.e. > 300)
  • myeloblasts (even more immature than myelocytes)
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35
Q

high WCC, RBC count, Hb is upper limit of normal

neutrophilia

basophilia

eosinophilia

A

CML

**you can have high RBC*

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36
Q

What causes gangrenes?

A

Abnormlaities of the blood vessels - atheroscleorsis

Abnormalities of the circulating blood-

  • polycythaemia:
  • cold agglutinin: which causes the RBCs to stick together in the cold
  • cryoglobulinaemia:protein that precipitates in the cold which blocks blood vessels
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37
Q

Isolated thrombocytopaniea in an elderly man

A

Autoimmune thrombocytopaniea purpura

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38
Q

Differentials for bruising in a child

A
  1. NAI
  2. thrombocytopaenia
    - autoimmune thrombocytopaenia
    - alloimmune thrombocytopaenia
    - ALL
  3. coagulation disorders
    - haemophilias- most likely
    - VWD
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39
Q

What does high RCDW mean?

A

RDW= red cell distribution width

RDW= red cell distribution width (which is high) – this tells you there is anisocytosis: diff sized cells present

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40
Q

What haematological abnormalities can be seen in patients with rheumatoid arthritis?

A
  • anaemia of chronic disease
  • iron deficiency anaemia
    • haemorrhage due to aspirin and NSAIDS
  • neutropaenia or thrombocytopaniea
    • due to drugs
  • Felty syndrome
    • due to the inflammation
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41
Q

What is felty syndrome?

A

Triad of

  • neutropaenia
  • splenomegaly
  • raised ESR
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42
Q
A
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43
Q

Osteomyelitis x-ray changes

A
  • patchy changes in bone

*distinguishes it from septic arthritis

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44
Q

Red herrings in osteomyelitis

A
  • fever may not be present
  • WCC may not be raised

CRP is usually always raised

—> can still be osteomyelitis

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45
Q

DDx of MAHA

A
  1. HUS
  2. TTP
  3. DIC
46
Q

TTP pentad

+ defect

+ treatment

A

Pentad:

  • MAHA
  • thrombocytopaenia
  • renal failure
  • fever
  • neurological symptoms eg altered consciousness

Defect:

deficiency of ADAMSTS13 enzyme: von Willebrand factor cleaving protease

Causes multimers of VWF to form and cause platelet clots

47
Q

How to treat TTP?

A

DO NOT GIVE PLATELET TRANSFUSION BECAUSE THIS MAKES THE CONDITION WORSE!!

tx: plasma exchange

48
Q

Blood film features of hyposplenism

A

Howell–Jolly bodies - nuclear remnants

Target cells

Occasional nucleated red blood cell

Lymphocytosis

Macrocytosis

Acanthocytes

49
Q

Normal myeloblast and lymphoblast counts in bone marrow vs myelodysplasia + AML and ALL

A

myeloid cells

<5%: normal

5-20%: myelodysplasia

>20%: AML

lymphoid cells

<5%: normal

>20%: ALL

50
Q

Key marker of lymphoblasts

A

TDT positive

51
Q

What may myeloblasts have in their cytoplasm?

A

Aeur rods

52
Q

Which cell markers are expressed on mature B cells?

A

CD19: receptor for CAR T cells

CD20: receptor for rituximab

53
Q

Cell surface markers of peripheral blood lymphoid T cells

A

CD3

CD4- helper T cells

CD8- cytotoxic T cells

CD5

54
Q

How to distinguish between immature and mature lymphoid cells?

A

Immature: TDT positive

Mature: TDT negative

**true for T and B lymphocytes

55
Q

How to distinguish between mature and immature B cells?

A

Mature B cells and plasma cells express surface Ig

56
Q

Which receptor on B cells to CART cells bind to?

A

CD19

57
Q

If you see myeloblasts in the blood film what does that suggest?

A

1) AML
2) leukoerythroblastic anaemia

58
Q

What single test distinguishes autoimmune from inherited haemolytic anaemia?

A

DAT test

Positive: autoimmune haemolytic anaemia

59
Q

If you see spherocytes what two diagnoses are suggested?

A

1) autoimmune haemolytic anaemia
2) hereditary spherocytosis

60
Q

What type of anaemia is haemolytic anaemia?

A

Can be macrocytic- because of reticulocytes are larger than normal rbc

61
Q

Give causes of non-immune acquired haemolytic anaemia

A
  1. malaria!!!
  2. MAHA- HUS, TTP, DIC
  3. paroxysmal nocturnal haemoglobinuria
62
Q

How can SLE affect the platelets?

A

Can cause idiopathic thrombocytopaenic purpura

63
Q

Causes of autoimmune haemolytic anaemia: key

A

Warm: SLE, drugs, CLL (evan’s syndrome)

Cold: mycoplasma, other infections

64
Q

ACD vs iron deficiency anaemia

A

The key is transferrin!!!

Iron deficiency anaemia: high transferrin

ACD: low/normal transferrin

65
Q

What protein mediates anaemia of chronic disease

A

hepcidin

*this is raised in ACD as it’s an inflammatory protein

it sequesters iron in macrophages so it’s low in the blood

66
Q

Can you get IDA with normal ferritin?

A

Yes!! If you have co-existent ACD

67
Q
A
68
Q

Isolated single lineage cytopaenia: causes

A
69
Q

If you have very high MCV (higher than 120)- what are the top differentials?

A

B12 or folate deficiency

70
Q

What does pancytopaenia with tear drop cells, nucleated RBC and immature granulocytes in blood film suggest?

A

Leukoerythroblastic anaemia- i.e. bone marrow infiltration

  • haematological cancer: leukaemia, lymphoma, myeloma
  • metastatic: prostate, breast, lung
71
Q

Chronic vs acute leukemia: white cell count

A

Chronic always has high white cell count (Acc to Prof McDonald)

72
Q

Classification of pancytopaenia

A
73
Q
  • normal Hb
  • VERY HIGH WCC
  • normal lymphocytes
  • raised neutrophils

Confirmatory test?

A

CML

BCR-ABL1 gene fusion i.e philadelphia chromosome

74
Q

What type of protein is ABL?

A

Tyrosine kinase

75
Q

Treatment for CML

A

Tyrosine kinase inhibitor- imatinib

76
Q

Do you see myeloblasts or myelocytes in CML?

A

Myelocytes - in chornic phase

if you see >20% blasts –> blast crisis

77
Q

Which cell surface marker is aberrently expressed in CLL?

A

CD5 and CD23 POSITIVE markers

  • NOTE: CD5 and CD23 should never be seen in a normal B cell
78
Q

Main prognostic factor for CLL

A

TP53 mutation status

79
Q

Link between CLL and anaemia

A

CLL can cause haemolytic anaemia

as it is a disease of the immune system

80
Q

Can you exclude myeloma if you have normal plasma imuunoglobulin?

A

No- can have light chain only myeloma

Need to check serum free light chains or urine bence jones protein

81
Q

What is the cause of renal failure in myeloma?

A

Cast nephropathy

  • deposition of light chains in the basement membrane precipitating renal failure
82
Q

Name a JAK1 inhibitor

A

Ruxolitinib

83
Q

What causes raised ESR?

A

raised acute phase proteins such as fibrinogen

**promotes rouleaux formation

84
Q

Causes of isolated raised ESR and normal CRP

A

SLE

Multiple myeloma

lymphoma

pregnancy

anaemia

85
Q
A
86
Q

translocation : t(11,14) associated to which malignancny

A

mantle cell lymphoma- agressive high grade NHL

87
Q
  • A 5y/o boy of Indian ethnic origin presented with lymphadenopathy and a mediastinal mass on chest radiology
  • WBC: 180 x 109/L
  • Hb: 93g/L
  • Platelet count: 43 x 109/L

what is the diagnosis

A

ALL

  • there are blast cells present on blood film
  • there was a VERY HIGH WCC
  • The low Hb and platelet count are the result of bone marrow infiltration
  • mediastinal mass is the thymus, which is infiltrated by T lymphoblasts

The VERY HIGH WCC in a child means a diagnosis of leukaemia is almost CERTAIN

88
Q

48y/o male- railway engineer

  • 2 week history of bleeding gums
  • Attended dentist- severe bleeding
  • 1 episode of haematuria
  • Minor bruising
  • Attended A&E

O/E

  • Left subconjunctival haemorrhage
  • Small bruises over the abdomen
  • No enlarged lymph nodes
  • No hepatosplenomegaly

QUESTION 3: What test is most likely to reveal the cause of the problem?

A
  • Blood count, film and coagulation screen

would also do LFTs and creatinine as this sounds like a very ill patient

89
Q

How could you explain a SHORT APTT and a low fibrinogen?

A
  • DIC
  • The APTT is short is because there are activated coagulation factors in the circulation which is making it short
  • will also have severe thrombocytopaenia and low fibrinogen
90
Q

what does this blood film show

A

granules AND Auer rods

would suspect these cells are MYELOID

91
Q
  • Granules strongly suggest which lineage
A

myeloid

92
Q

What diagnosis do you suspect on a background of abnormal clotting

A

hyper-granular (very granular) promyelocytes (blast looking)

Acute promyelocytic leukaemia

93
Q

Which tests would be the most useful to confirm the APML

A
  • Cytogenetic analysis/ FISH/ molecular genetic analysis
  • Do NOT need cytochemistry as you can see the brightly stained granules.
  • Immunophenotyping can help but it is less specific than cytogenetic analysis to show the translocation or FISH to show the 15;17 fusion gene

Fish: FISH showed PML-RARA fusion

94
Q

what is treatment for APML + prognosis

A
  • Platelets
  • Chemotherapy
  • All-trans-retinoic acid (ATRA)

Complete remission can be achieved

95
Q
  • 68 y/o woman, retired secretary
  • Gradual onset of fatigue, lethargy and exertional dyspnoea
  • Non-smoker, not much alcohol, good diet

O/E:

  • Pallor (conjunctival and nail bed)
  • Mild ankle oedema

which one test should you do next :

  • Blood film
  • Bone marrow aspirate
  • Liver function tests
  • Thyroid function tests
  • Serum Vitamin B12 and red cell folate
A

Blood film

In reality, with a macrocytosis you would also do Vitamin B12 and Cell folate and LFTs

96
Q

what does this show

A
  • There are macrocytes but NO oval macrocytes nor hypersegmentation of neutrophils
  • There are hypochromic microcytes
  • There is also an elliptocyte
  • This is a dimorphic film
  • This is NOT associated with B12/ folate deficiency, hypothyroidism or liver disease
97
Q

what does this show of bone marrow aspirate

A
  • shows increased blast cells (12%)- the normal is < 5%
  • ring sideroblasts (can see the ring of iron-containing granules around the nucleus)

​

DIAGNOSIS: myelodysplastic syndrome with excess blasts

98
Q
  • 72y/o Indian woman
  • Vegetarian, teetotal, non-smoker
  • SOBOE
  • Fatigue
  • Painful gums and tongue
  • Unable to eat spicy food

O/E: pallor only

FBC:

  • low Hb
  • normal platelets
  • high MCV
  • normal WCC

what does this show + What is the most important test

A

hyper-segmented neutrophils

macrocytes and oval macrocytes too

Vitamin B12 and folate assays

99
Q

what is the next investigation to do if there is unexplained macrocytes (normal B12 + folate) and elevated LDH

A

Bone marrow aspirate

100
Q
A
101
Q

if anaemia was caused by vitamin B12 and folate what would you see in the blood film

A

oval macrocytes

macrocytosis

hypersegmented nuclei

102
Q

what is the diagnosis of this blood film

A
  • Giant metamyelocytes are present
  • Megaloblasts are also present
  • Hyper-segmented neutrophils can also be seen

This is CLASSICAL for a vitamin B12 and folate deficiency

103
Q

+ What test would you do next to try and confirm the diagnosis?

A
  • Polycythaemia vera
  • With polycythaemia vera, you also often have a:
    • High platelet count
    • High WCC

Note: Essential Thrombocythemia, there is an isolated high platelet count

  • Molecular analysis for JAK2 mutation
104
Q

what does this show

A
  • The RBC, Hb and Hct are HIGH
  • The patient appears to be polycythaemic

This can be true or pseudopolycythaemia- canno tell from these results

105
Q

identify which is normal/abnormal + most likely diagnosis given this is seen on a background of low Hb and MCV

A
  • The left film= normal, right film= abnormal

hypochromia (increased pallor in the centre of the RBCs)- confirms anaemia

some poikilocytes and anisocytosis

  • Iron deficiency anaemia
106
Q
A
107
Q

what is the likley diagnosis

A
  • Osteomyelitis
  • The abnormality in the bone (patchy changes) points you to osteomyelitis more so than septic arthritis

Note can get osteomyleitis in the absecne of fever, raised WCC. but CRP would almost always be high

108
Q
  • 21-year-old woman presented with abdominal pain, bruising and altered level of consciousness
  • She had a low-grade fever
  • Her platelet count was 15 x 109/L
  • Her bilirubin was increased and LDH was gently increased

Her creatinine was marginally increased

QUESTION 8: What is the most likely diagnosis?

A
  • There are RBC fragments - indicative of MAHA
  • Microspherocytes are present
  • Thrombotic thrombocytopaenic purpura
109
Q

most common complication of infectious mononucleiosis

A
  • splenomegaly occurs in 50% of patients with infectious mononucleosis
  • Splenic rupture occurs in 0.1-0.5% of patients
110
Q

risks of hyposplenism

A
  • Overwhelming bacterial sepsis (particularly pneumococcal or H. influenzae
  • Fatal malaria
  • Fatal Capnocytophaga canimorsus infection (typically occurs from dog bites)
  • Babesiosis (parasitic infection
111
Q

follow up for hyposplenism

A
  • Vaccinate for:
    • Pneumococcus
    • Meningococcus
    • Haemophilus influenzae
  • Vaccinate against influenza
  • Prescribe life-long penicillin
  • Advise the patient on:
    • Dog bites
    • Travel to malaria zones
    • Prompt treatment of infection
  • Issue a splenectomy card and information sheet

Haematology (16 decks)

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