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Pathology Term 3 > Week 5 - WBC HAEMATOLOGY > Flashcards

Week 5 - WBC HAEMATOLOGY Flashcards

Reactive WBC Disorders, Leukemia, Lymphoma, MPD + MDS

1
Q

What are the 2 routine stains in haematology?

A
  1. Eosin stain
    - taken up by eosinophil granules
    - red/pink
  2. Basic stain
    - taken up by basophil granules
    - blue

*neutrophils take up NEITHER

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2
Q

What is the normal WBC range?

A

4-11 x 10^9/L

  • neutrophils = 2-8 x 10^9/L
  • lymphocytes = 1-4 x 10^9/L
  • others = <1 x 10^9/L
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3
Q

What are the physiologic changes in WBCs?

A

Infancy/Aged:

  • lymphocytosis (incr. lymphocytes
  • in infancy 60-70% of peripheral blood is lymphocytes

Pregnancy:
-leucocytosis (incr. leucocytes)

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4
Q

When will you commonly see increased neutrophils and immature neutrophils?

A

Increased neutrophils
-acute infection/inflammation

Immature neutrophils

  • LEFT SHIFT
  • severe infection/inflammation
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5
Q

What is Left Shift?

A
  • an increase in the no. of immature leucocytes (typically increased neutrophil band cells) in peripheral blood
  • seen in severe infection/inflammation (increase need for neutrophils)
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6
Q

What are the toxic changes to neutrophils in acute infections?

A
  1. toxic granulations
  2. dohle bodies
  3. vacuolation

*suggestion of early marrow release

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7
Q

What are the causes of lymphocytosis?

A
  • viral infections **
  • chronic infections
  • immune reactions
  • pertussis
  • TB
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8
Q

What are the only 2 bacterial conditions which cause a lymphocytosis?

A

pertussis + TB

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9
Q

What is the difference in lymphocyte appearance between lymphocytosis and reactive lymphocytosis?

A

lymphocytosis:
-small, round, large nucleus, scanty cytoplasm

reactive lymphocytosis:

  • activated T cells
  • large, more cytoplasm, flowing between RBCs (v. fragile and large)
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10
Q

When would you see reactive lymphocytosis?

A

viral infections

-e.g. EBV

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11
Q

What are the causes of eosinophilia?

A
  • allergy/hay fever
  • hypersensitivity
  • asthma
  • parasites
  • worminfestations
  • hodgkin’s lymphoma
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12
Q

What should you suspect if you see basophilia on a peripheral blood smear?

A

CML

  • basophilia = v. rare
  • chronic myeloid leukemia
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13
Q

What is a common cause of leucocytopenia?

A

steroid therapy

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14
Q

What is lymphoerythroblastic reaction and when is it seen?

A
  • leucoblasts + erythroblasts in blood
  • irrespective of number
  • release of all (both WBC + RBC) types of immature cells from bone marrow

seen in:
-marrow fibrosis, malignant infiltration, severe hyperplasia or infection (increased stimulation of BM)

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15
Q

What is leukemoid reaction and what are the causes?

A
  • very high WBC counts
  • severe and excessive leucocytosis with immature cells (leukemia-like)

Causes:

  • severe infections (usually children)
  • severe hemolytic anemia/crisis
  • marrow infiltrations/metastasis (leucoerythroblastic reaction)
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16
Q

What is neutropenia and agranulocytosis?

A

Neutropenia

  • decreased neutrophils (<1.5 x 10^9/L)
  • any condition

Agranulocytosis

  • v. severe (<0.5 x 10^9/L)
  • severe infections (life-threatening)
  • decreased production –> marrow failure
  • increased destruction –> immune, splenomegaly, septicemia, viral (HBV)
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17
Q

What is pancytopenia and what are the causes?

A

-decr. ALL cell types (RBCs, WBCs, Plts)

Causes:

  • BM suppression
  • aplastic anemia
  • megaloblastic anemia
  • myelodysplastic syndromes
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18
Q

What is reactive lymphadenitis?

A

-enlarged lymph nodes due to reaction to an infection or inflammation

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19
Q

What are the clinical features of reactive lymphadenitis?

A
  • enlarged LNs
  • tender/painful
  • mobile (not fixed)
  • inflammation in the area
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20
Q

What are the microscopic features of reactive lymphadenitis?

A
  • follicle hyperplasia
  • sinus histiocytosis
  • granuloma in TB
  • abscess in bacterial/cat-scratch disease
  • tingible body macrophages –> eating away unwanted lymphocytes
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21
Q

What are the types of myeloid neoplasms?

A

Acute myeloid leukemia (AML)
-neoplastic blast cells WITHOUT maturation

Myeloproliferative disorders (+CML)
-neoplastic blasts mature along one or more cell lines

Myelodysplastic syndromes

  • blasts mature in a dysplastic manner –> destruction
  • increased abnormal cells in BM/decreased in blood
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22
Q

What are the types of lymphatic/lymphoid neoplasms?

A

Lymphatic leukemia

  • ALL
  • CLL
  • Myeloma

Lymphoma

  • non-hodgkins lymphoma
  • hodgkins lymphoma
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23
Q

What is leukemia?

A
  • malignant WBCs in blood
  • malignancy of blast cells in BM –> spread to blood + blood-related organs (liver, spleen, LNs)
  • unkown etiology (radiation, viral ?)
  • oncogenesis –> incr. division, decr. differentiation
  • many subtypes –> gene-specific, Dx. + Tx.
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24
Q

What are the common oncogenes in CML, AML, ALL, MPD?

A

CML –> BCR-ABL
AML –> RARA
ALL –> BCL2
MPD –> JAK2

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25
Q

What is the most common subtype of AML?

A

M3 –> promyelocytic leukemia

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26
Q

What is the most common subtype of ALL?

A

L1 –> small monomorphic
-CD10+ (pre-B lymphocytes)

N.B. FAB classification (L1, L2, L3)

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27
Q

What chronic leukemia is the most common clinically?

A

CLL

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28
Q

What are the clinical features of leukemia?

A

Decreased hemopoiesis:

  • anemia (low RBCs)
  • fever/infections (low WBCs)
  • bleeding tendency (low plts)

Organ infiltration:

  • bone/back pain
  • hepatosplenomegaly
  • lymphadenopathy
  • BM, spleen, liver, LNs
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29
Q

What is CD10+?

A
  • immature B cell marker

- seen in L1 subtype of ALL (small monomorphic)

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30
Q

What age is ALL increased in and what are the clinical features?

A
  • any age, common in CHILDREN
  • growth failure, fever, anemia, PROMINENT lymphadenopathy, MINIMAL hepatosplenomegaly, bleeding

*LYMPHADENOPATHY marked

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31
Q

Why is there more prominent lymphadenopathy in ALL?

A

-malignancy of LYMPHOBLASTS –> increased spread to lymph nodes

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32
Q

What is the microscopy of ALL?

A

Lymphoblasts

-plenty of large, round blast cells with little blue cytoplasm and NO granules

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33
Q

How do you differentiate between a normal lymphocyte and a lymphoblast (i.e. in ALL)?

A
  • compare size to surrounding RBCs
  • lymphoblasts > size than RBCs
  • lymphocytes ~ size of RBCs
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34
Q

What age group is AML increased in and what are the clinical features?

A
  • adults (40-60yrs common)
  • anemia, fever, bleeding (same as ALL)
  • moderate/prominent hepatosplenomegaly
  • NO significant lymphadenopathy
  • gum hypertrophy*
  • increased infections –> candidiasis (oral thrush), gingivitis + hyperplasia, bruising –> purpura
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35
Q

What is the microscopy of AML?

A

Myeloblasts

-plenty of large, round blast cells with more clear cytoplasm with granules and auer rods

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36
Q

What are Auer rods?

A
  • seen on microscopy of AML
  • granules join to form crystal-like structures within cytoplasm –> AUER RODS
  • Dx. of AML
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37
Q

What is AML-M4 known as?

A

Myelomonocytic

-cells present with pink cytoplasm (myeloblasts) and blue cytoplasm (monoblasts)

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38
Q

What is AML-M7?

A

Megakaryoblastic leukemia

  • blasts
  • abnormal megakaryocytes
  • plenty of abnormal giant platelets
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39
Q

Briefly compare AML vs. ALL

A

ALL

  • children increased
  • increased lymphadenopathy/minimal hepatosplenomegaly
  • scanty cytoplasm (lymphoblasts)
  • NO granules

AML

  • adults increased
  • increased hepatosplenomegaly/NO lymphadenopathy
  • more clear cytoplasm (myeloblasts)
  • granules + AUER rods

*BOTH –> anemia, fever, bleeding, infections

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40
Q

Gum hypertrophy is typical of what leukemia?

A

AML

-leukemic infiltration

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41
Q

What are some features of chronic leukemias?

A

CML + CLL

  • later age
  • chronic
  • asymptomatic for many years
  • slow + gradual progression
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42
Q

What is CLL (chronic lymphocytic leukemia) similar to?

A

Small lymphocytic lymphoma (SLL)

-NHL

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43
Q

What are the features of CLL?

A
  • similar to SLL (NHL)
  • anemia, fever, bleeding –> slow over many yrs
  • lymphocytosis + lymphadenopathy
  • MASSIVE splenomegaly (over yrs) + mild hepatomegaly
  • commonest = B cell (CD5+ –> T cell Ag); high BCL2 expression
  • hypogammaglobulinemia –> non-functioning lymphocytes (despite increased no.)
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44
Q

What are the clinical phases of CLL?

A

Chronic (*commonest):
-slow progress (yrs)

Accelerated phase:

  • high grade lymphoma/leukemia
  • terminal phase after many yrs
  • incr. mortality :(
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45
Q

What are the features on blood film of CLL?

A
  • plenty of normal looking lymphocytes

- SMUDGE CELLS –> smeared/crushed cells from smearing blood as cancer cells are fragile (v. characteristic of CLL)

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46
Q

When would you see smudge cells?

A

CLL peripheral blood smear

47
Q

What chromosome is characteristically affected in CML?

A

Philadelphia chromosome

-translocation of 22 onto 9

48
Q

What is the difference between CLL + SLL (NHL)?

A

CLL
-increased leukemic lymphocytes in BLOOD

SLL/NHL
-increased leukemic lymphocytes in LYMPH NODES

49
Q

What is the commonest type of CLL?

A

B CELL:

  • CD5+ (T cell Ag)
  • high BCL2 gene expression
50
Q

Why is there hypogammaglobulinemia in CLL depite an increased no. of lymphocytes?

A

They are NON-FUNCTIONING

-produce monoclonal Abs which are of NO use physiologically

51
Q

What oncogene is mutated in CML commonly?

A

BCR-ABL fusion gene activation

-Breakpoint Cluster Region - Ableson Leukemia gene –> carcinogenesis (uncontrolled blast cell proliferation)

52
Q

True or False?

There is marked left shift in CML

A

True

53
Q

What are the key features of CML?

A

Marked leucocytosis (>50 x 10^9/L)

  • ALL cells
  • not just blast cells –> marked left shift

Marked hepatosplenomegaly

Basophilia –> v. characteristic

54
Q

What are the 3 clinical phases of CML?

A

Chronic
-slow progress (yrs)

Accelerated
-rapid progress (mths)

Blast Crisis
-acute leukemia (wks)

55
Q

What is Blast Crisis?

A
  • clinical phase of CML
  • pt. deteriorates within weeks
  • blast cells over proliferate and take over from previous variety of immature/mature cells (Left Shift)
  • acute leukemia (decr. mature/incr. immature)
56
Q

What is multiple myeloma?

A
  • leukemia of PLASMA CELLS (B lymphocytes)
  • HYPERGAMMAGLOBULINEMIA*
  • old age, males increased
57
Q

What condition would you see a perinuclear halo and what is it resulted from?

A

Multiple myeloma

  • perinuclear halo is at region of golgi apparatus
  • B cells loaded with immunoglobulins (monoclonal Ab –> not useful)
58
Q

Why is there an increased risk of infarctions, visual difficulties and even blindness in multiple myeloma?

A
  • hypergammaglobulinemia from increased monoclonal Ab
  • increased gamma globulin –> STICKY substance
  • blood becomes thick + viscous –> infarctions, visual disturbance, blindness (obstruction to small vessels)
59
Q

What is the pathogenesis of punched out lytic bone lesions + fractures in multiple myeloma?

A
  • leukemia in BM
  • production of osteolytic enzymes
  • increased osteolysis
60
Q

True or False?

CML is a type of MPD

A

True

61
Q

What is the commonest pathogenesis of lymphoid neoplasms?

A
  • commonly related to EBV infection
  • overexpression of BCL2 (ANTIAPOPTOTIC GENE)
  • t(14;18) –> BCL2 + IgM –> B cell neoplasia
  • continuous proliferation of unneeded B lymphocytes
62
Q

What are the general clinical features of lymphoid neoplasms?

A
  • fever, anemia, infections
  • lymphadenopathy, splenomegaly
  • BM infiltration + bleeding ONLY in high-grade neoplasms (unlike leukemias)
63
Q

How are lymphoid neoplasms diagnosed?

A

Immunophenotyping

  • T/B cell Ag commonest –> to differentiate between T cell or B cell lymphomas
  • CD5 –> CLL

Genetic

  • BCL2
  • TdT (ALL)
64
Q

Where do the commonest lymphomas arise from (commonest starting cell)?

A 
Germinal Centre (GC) B cells:
=overproliferation due to BCL2 mutation (antiapoptotic gene)
65
Q

Clinically, what is the commonest type of lymphoma?

A

Non-Hodgkin’s lymphoma

  • 62.4%
  • out of NHL –> Diffuse B cell Lymphoma = commonest
66
Q

What is the common infection implicated in the development of lymphoid neoplasms?

A

EBV

67
Q

What is Hodgkin’s lymphoma and what is the characteristic microscopic feature?

A
  • malignancy of germinal centre B cells
  • double peak (young + late age)
  • REED STERNBERG (RS) CELLS**
68
Q

What is significant about the fever in Hodgkin’s lymphoma?

A

Cyclic Pel-Ebstein Fever (like malaria)

  • 2-3 days
  • only in 30% of cases
69
Q

What are the clinical Sx of Hodgkin’s lymphoma?

A
  • wt. loss
  • sweating
  • fever (cyclical –> Pel-Ebstein)
  • weakness
  • anorexia
70
Q

What are the 2 types of symptom classification for hodgkin’s lymphoma?

A

A –> absence of Sx.

B –> fever, night sweats, wt. loss, etc.

71
Q

Why is staging important in Hodgkin’s lymphoma and outline what it is?

A

*as it is CONTINUOUSLY/CONTIGUOUSLY SPREADING from LN –> LN

stage I –> single or one region LN
stage II –> 1 group of LNs (ABOVE diaphragm)
stage III –> multiple groups of LNs (incl. BELOW diaphragm)
stage IV –> involvement of liver, spleen or BM (high-grade)

72
Q

Which cells are malignant in hodgkin’s lymphoma?

A

Reed-Sternberg Cells

-rest of the cells are reactive inflammatory cells –> lymphocytes, neutrophils, macrophages, eosinophils

73
Q

What region lymphadenopathy is typical in Hodgkin’s lymphoma?

A

Mediastinal lymphadenopathy

-compresses trachea –> cough

74
Q

What is the relationship between alcohol + hodgkin’s lymphoma?

A

Alcohol-induced pain (LN pain)

  • rare
  • but if present –> v. characteristic
75
Q

What is the pathogenesis of hodgkin’s lymphoma?

A
  • RS cells = malignant
  • reactive inflammatory cells = non-malignant (lymphocytes, neutrophils - inflammation, macrophages, eosinophils –> fibroblasts –> fibrosis)
  • spread via nearby LNs (no metastasis unlike NHL)
76
Q

What is the commonest type of hodgkin’s lymphoma?

A

Nodular Sclerosis
-characterised by malignant RS cells, inflammation + FIBROSIS –> extensive fibrosis forming nodules which form fibrous septa

77
Q

Outline classification of hodgkin’s lymphoma

A
  1. nodular sclerosis –> most common*
  2. mixed cellularity
  3. lymphocyte-rich
  4. lymphocyte depletion
  5. nodular lymphocytic predominance
(1-->4 = classical HL - EBV + Ig Tran. mutation)
(5 = NO EBV or Ig Tr. mutation; popcorn cells)
78
Q

What do RS cells look like and how are they related to prognosis?

A
  • binucleate, large, ‘owl-eye’ shaped nuclei –> scary looking
  • increased RS cells (malignant cells) = poor prognosis
79
Q

What is the microscopy of nodular sclerosis HL?

A
  • nodular, fibrous septa
  • reactive inflammatory cells –> eosinophils, lymphocytes, neutrophils, plasma cells, etc.
  • RS cells**
80
Q

How can you differentiate between NHL + HL on microscopy?

A

NHL –> uniform cells; NO RS cells

HL –> mixed RS cells + inflammatory cells

81
Q

What are popcorn cells?

A
  • RS cell variant
  • significantly clear cytoplasm, uninucleated
  • seen in Nodular Lymphocytic Predominance (NLP) Hodgkin’s (non-classical hodgkins)
82
Q

What are the clinical features of NHL?

A
  • fever, anemia, infections, lymphadenopathy, splenomegaly (+/-)
  • NO RS cells/eosinophilia **
83
Q

How does the spread of NHL differ to HL?

A
  • NHL has a random spread between LNs (can spread to distal nodes randomly)
  • HL has a contiguous pattern of spread between LNs

*NHL staging is thus DIFFICULT

84
Q

Which cell type is commonest in NHL?

A

B cell

-T cell + histiocytic also possible, unlike HL (ALL B cells)

85
Q

What is small lymphocytic lymphoma + large lymphocytic lymphoma?

A

SLL –> low grade –> CLL
LLL –> high grade –> ALL

  • 2 main types of NHL
  • bigger cell size = higher grade = worse prognosis
86
Q

True or False?

-NHL can be diffuse or follicular on microscopic appearance

A

True

87
Q

What would low, intermediate and high grade NHL appear like on microscopy?

A

Low
-uniform SMALL cells

Intermediate
-MIXED small + large cleaved cells

High
-LARGE cells

88
Q

What is the difference between diffuse NHL + follicular NHL with regards to grading?

A 
Follicular = low-grade (well differentiated)
Diffuse = high-grade
  • BUT –> cell size still matters (i.e. diffuse vs. follicular is NOT the only determining factor for grading NHL)
  • small cells = low grade
  • large cells = high grade
89
Q

What is Burkitt’s lymphoma?

A
  • B cell lymphoma
  • children/young
  • large cell –> high grade
  • endemic in Africa (sporadic elsewhere)
  • MYC-IgH translocation - 14 - EBV*
  • surface B cell marker + IgM
  • common on facio-maxillary region
90
Q

What is microscopy of Burkitt’s lymphoma?

A
  • larger, dark blast cells
  • lipid vacuoles (same as ALL type L3)
  • plenty of scattered macrophages

**STARRY SKY –> characteristic appearance

91
Q

What is the most common type of plasma cell neoplasm?

A

Monoclonal Gammopathy of Undetermined Significance (MGUS)

  • chronic plasma cell neoplasm
  • v. slow growing (benign)
  • <3gm/dL of monoclonal gammaglobulins = diagnostic
  • may develop into myeloma; plasma cytic lymphoma
  • amyloidosis due to increased immunoglobulin production
92
Q

Compare HL vs. NHL

A

HL:

  • avg age 28; double peak at 25 + 60yrs
  • <10% of all lymphomas
  • cervical, chest + mediatinal LNs (85%); extranodal (4%), contiguous spread; mesenteric + waldeyer ring involvement uncommon
  • B cells affected with RS cells present
  • more likely to have systemic (B) Sx.
  • early Dx, predictable, better prognosis

NHL:

  • avg age 67yrs
  • > 60% of all lymphomas
  • multiple sites, chest (40%), extranodal (23%) +BM; non-contiguous spread; mesenteric + wladeyer ring involvement common
  • B cells commonly affected, but T cells, histiocytes + NK cells can also be affected
  • less likely to have systemic (B) Sx.
  • less predictable, early spread, good –> worse prognosis
93
Q

True or False?

Mesenteric + waldeyer ring involvement is common in HL

A

False

-common in NHL

94
Q

What is the commonest mutation seen in MPD and explain its role in the pathogenesis of MPD? What is the exception?

A

JAK2 activation on 9p
-exception = CML –> BCR-ABL mutation

*JAK2 activation causes increased tyrosine kinase activity, PDGF mutations –> growth factor hypersensitivity

95
Q

What are the 4 conditions that constitute MPDs?

A
  1. polycythemia vera (PV) –> RBC maturation
  2. CML –> WBC maturation
  3. essential thrombocythemia (ET) –> Plt maturation
  4. myelofibrosis (MF) –> fibroblast* maturation
    - as a result (secondary to) one of the other 3 conditions

*N.B. mixed = common

96
Q

True or False?

MPD can transform to acute leukemia

A

True

97
Q

What are the outcomes of MPD?

A
  1. acute leukemia (AML)
  2. myelofibrosis (after yrs) –> scarring of BM
    (3. death by MPD) :(
98
Q

True or False?

-in PV, EPO is required for RBC maturation

A

False

  • PV = neoplastic due to JAK2 mutation
  • growth factor (EPO)-independent proliferation
99
Q

What happens to serum EPO in PV?

A

Decreases

-due to increased serum RBCs from growth factor-independent proliferation

100
Q

What are the clinical features of PV?

A
  • red skin/flushing
  • hypercellular marrow
  • hepatosplenomegaly
  • high viscosity blood –> vascular stenosis –> infarctions
  • bleeding - BV damage; plt. abnormality
101
Q

Why is primary myelobrosis common in ET?

A

-plts. release PDGF which stimulates fibroblasts and causes fibrosis/scarring of marrow

102
Q

Why is there increased bleeding in ET?

A
  • JAK2 tyrosine kinase activation

- increased plts –> large + abnormal –> therefore non-functioning –> bleeding

103
Q

What is primary myelofibrosis?

A
  • end result of PV, ET and other MPDs
  • JAK2 tyrosine kinase activation
  • increased marrow fibrosis due to PDGF + TGF-beta from neoplastic stem cells + megakaryocytes

**fibroblasts = non-neoplastic

104
Q

Why is there massive hepatosplenomegaly in primary myelofibrosis?

A

-increased extramedullary hemopoiesis as the whole BM is scarring

105
Q

What characterisitic RBC feature is seen in myelofibrosis?

A

Teardrop RBCs

106
Q

What are Myelodysplastic syndromes (MDSs) AKA?

A
  • refractory anemia

- pre-leukemia

107
Q

What is the common mutation seen in MDS and what does it result in?

A
  • deletions of 5q

- excess proliferation of functional + structurally abnormal DYSPLASTIC cells (MDS)

108
Q

What is the main difference between MPD + MDS?

A

MPD
-functionally abnormal but structurally normal cells

MDS

  • functionally + structurally abnormal dysplastic cells
  • majority cells destroyed in marrow
  • ineffective myelopoiesis
  • peripheral pancytopenia –> anemia, infections, bleeding
109
Q

What is the commonest cell line affected in MDS?

A

RBCs = most common
-anemia –> v. common in pts. with MDS

Sx. = fever, anemia, bleeding (like leukemia)

110
Q

What is the FAB classification of MDS?

A
  1. RA: - refractory anemia; only RBCs affected (<1% BM blasts)
  2. RARS: - RA with ring sideroblasts - iron deposits around nucleus of erythroblasts (<1% blasts)
  3. RAEB: - RA with excess blasts (in BM); faster developing/higher grade disease (1-5% blasts)
  4. RAEB-T: - RAEB in transformation (5-30% blasts)
    * >30% blasts = leukemia
111
Q

What % of blasts is diagnostic for leukemia?

A

> 30%

112
Q

What are the 2 known pathogenetic mechanisms for lymphomas?

A

EBV + BCL2 mutation

113
Q

True or False?

transformation to AML is more common in MDS than MPD

A

True

114
Q

True or False?

peripheral pancytopenia is typical of MPD

A

False

-typical of MDS

Pathology Term 3 (6 decks)

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