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Haematology 1 Flashcards

Acute Leukaemias Chronic Myeloid Leukaemia and Myeloproliferative disorders Chronic Lymphocytic Leukaemia Lymphomas Myeloma Myelodysplastic syndromes & Bone Marrow Failure Bone Marrow Transplantation (113 cards)

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1
Q

Leukaemia literal meaning

A

white blood

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2
Q

Pathophysiology of Leukaemia

What types exist

A

Caused by mutations in white blood cells or their precursors
Mutations cause proliferation through a variety of mechanisms
Can be rapidly progressive or indolent – (Acute vs Chronic)

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3
Q

List myeloid cell types

A

Branching off from multipotential haematopoietic stem cell
Common Myeloid Progenitor
Megakaryocyte, Mast Cell, Erythroblast, Myeloblasts: Basophil, Eosinophil, Neutrophil, Monocytes –> Macrophage

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4
Q

List Lymphoid cell types

A

Branching off from multipotential haematopoietic stem cell
Common Lymphoid Progenitor
Natural Killer Cell, Small Lymphocytes: T Lymphocytes, B Lymphocytes –> Plasma Cells

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5
Q

Which cells does acute leukaemia affect?

A

The more acute the leukaemia, the higher up it affects the chain of blood cell production

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6
Q

What cell does AML and CML affect?

A

Common Myeloid Progenitor

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7
Q

What causes CML?

A

BCR-ABL mutation in the myeloid progenitor or prior stem cell line

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8
Q

What is myeloma?

A

Myeloma – Plasma cell dyscrasia – proliferation of PLASMA cells in bone marrow

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9
Q

What cells does CLL affect?

A

Small Lymphocytes: T Lymphocytes and B Lymphocytes

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10
Q

What cell does ALL affect?

A

Common Lymphoid Progenitor

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11
Q

Clinical Presentation of Leukaemia

A

anaemia, thrombocytopenia, leukopenia/neutropenia

Splenomegaly less common than chronic leukaemias

Bone pain common

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12
Q

Pathophysiology of Leukaemia:

A

Rapid proliferation of cells, causing packed bone marrow

Results in bone marrow failure

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13
Q

3 overheading signs of Leukaemia

A

Anaemia, Thrombocytopenia, Leukopenia

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14
Q

3 overheading signs of Acute Leukaemia and associated symptoms

A

Anaemia: shortness of breath, chest pain on exertion, fatigue
Thrombocytopenia: easy bruising, petechial rashes, spontaneous bleeding e.g. epistaxis
Leukopenia: frequent or severe infections including opportunistic infections e.g. fungal infections
Additionally, bone pain

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15
Q

How does chronic leukaemia differ from acute leukaemia?

A

Slower proliferation of malignant cells
Less burden of disease in bone marrow
Clonal cells can pool in lymph nodes or in the spleen
Clinical manifestations – lymphadenopathy, splenomegaly

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16
Q

Key features of ALL in the history

A

Child – typically 2-5 yrs old
Hepatosplenomegaly (usually in chronic L, but children have small organs so occurs in ALL too)
Bone pain / limp (can be this alone - KEY FACT!)
Fevers
CNS symptoms
Testicular swelling (rare but specific - pooling of cells) (bALLs)

Adults – similar to AML, lymphadenopathy

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17
Q

Key features of ALL on blood tests

A

LOW PLATELETS (THROMBOCYTOPENIA)
LOW HB (ANAEMIA)
HIGH WCC
CIRCULATING BLASTS

Usually present with thrombocytopenia and anaemia
High white cell count (if severe, will be normal or suppressed)
Some analysers will indicate presence of blasts but sometimes these will be flagged as lymphocytes.
Circulating blasts are abnormal (should be in bone marrow)

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18
Q

https://imgur.com/2ZABq6m

interpret this blood film

A

High nucleus – cytoplasm ratio

It is not possible to tell ALL from AML on blood film most of the time!

This is Precursor B-cell ALL

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19
Q

What are key Ix for ALL?

A

Flow Cytometry and Bone Marrow Biopsy

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20
Q

What is Flow Cytometry?

A

a technique used to detect and measure physical and chemical characteristics of a population of cells or particles. A sample containing cells or particles is suspended in a fluid and injected into the flow cytometer instrument.

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21
Q

What causes ALL?

what is a method of remembering this?

A

BCR-ABL1 t(9;22) associated with 20-30% of ALL in adults

ALL can affect testicles - Balls - B-ALL spells out BALL.
B standing for BCR-ABL1 t(9:22) mutation

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22
Q

How is ALL treated?

A

Chemotherapy - Imatinib or other TKI for BCR-ABL1

Induction –> Consolidation –> Maintenance –> Remission
Possible transplant

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23
Q

Key features of AML in the history

A

Incidence increases with age
Might have had pre-existing MDS (myelodysplastic syndromes can transform into ALL)
Symptoms of cytopenias

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24
Q

Key features of AML on blood tests

A

anaemia (low haemoglobin) (bone marrow suppression)
high WCC
low platelets (bone marrow suppression)
neutropenia (lack of mature white cells due to xs blasts)
high blasts
normal INR - normal for AML
Abnormal INR - possible DIC due to acute promyelocytic leukaemia

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25
https://imgur.com/ERMjP3z what is the feature and dx
Auer Rods AML (one is enough to diagnose AML or MDS)
26
https://imgur.com/0YTUM0h what is the feature and dx
Auer Rods AML (one is enough to diagnose AML or MDS)
27
https://imgur.com/Oe1u3pi what is the feature and dx
Auer Rods AML (one is enough to diagnose AML or MDS)
28
https://imgur.com/HUniyD6 what is the feature and dx
Auer Rods AML (one is enough to diagnose AML or MDS)
29
https://imgur.com/oerYgPF what is the feature and dx
Faggot Cells (stacks of Auer Rods) AML
30
Next steps if suspecting AML but no Auer Rods? | Positive Result?
Flow Cytometry | MPO
31
Important subtype of AML? Significance? Treatment?
T(15;17) Acute Promyelocytic Leukaemia Presents with DIC. Good prognosis Treat with: All-Trans Retinoic Acid (ATRA) aka Vitamin A: Forces cells to differentiate, stops proliferation
32
AML treatment? | Prognosis?
Similar to ALL - Imatinib or similar TKI Possibility of targeted agents in future Poor prognosis particularly in elderly who won’t tolerate stem cell transplant
33
What are myeloproliferative Neoplasms?
These are increased production of the myeloid lineage i.e. anything following the Common Myeloid Progenitor in the chain
34
What is the myeloproliferative neoplasm affecting megakaryocytes? Diagnosis? Associated Mutation? Treatment?
Essential Thrombocytopenia Platelet count consistently >450 JAK2 Mutation in 55% Aspirin to reduce stroke risk Hydroxycarbamide to lower count
35
What is the myeloproliferative neoplasm affecting Erythrocytes? Diagnosis? Other features? Risks? Associated Mutation? Treatment?
Polycythaemia Vera Haematocrit >0.52 / 0.48 (M/F) Often thrombocytothaemia as well High Risk of thrombotic events e.g. stroke, MI, Budd-Chiari Syndrome (very dense, thick blood) JAK2 Mutation 95% Aspirin to reduce stroke risk Venesection (taking blood to lower haematocrit) Hydroxycarbamide to lower count
36
What is Haematocrit?
the ratio of the volume of red blood cells to the total volume of blood.
37
Meaning of 'Polycythaemia'?
Excess production of Red Blood Cells
38
What is caused by occlusion of the hepatic veins that drain the liver. It presents with the classical triad of abdominal pain, ascites, and liver enlargement.
Budd–Chiari syndrome affects 1 in 1 million
39
What causes proliferation of stem cells in the bone marrow & subsequent fibrosis? Pathophysiology? Associated mutation? Ix results? Blood film finding? Pathagnomonic Ix and Result? Treatment?
Myelofibrosis clonal proliferation of stem cells (first cell in the chain) results in cytokine release and fibrosis of bone marrow The fibrosis causes reduced production of all cell lineages JAK2 Mutation in 50% Pancytopenia Splenomegaly (can be massive splenomegaly) Tear Drop Cells Bone Marrow aspiration - Dry Tap (no aspirate) Stem cell transplant likely only cure Ruloxitnib - JAK inhibitor
40
What is a key type of MPN?
A key type of myeloproliferative neoplasm is CML Increased production of the Myeloid Lineage
41
What are key features of CML in the history?
Typically onset age 35-55 in EMQs LUQ pain - Splenomegaly Mostly ASYMPTOMATIC if diagnosed in chronic phase May present with symptoms of acute leukaemia if in accelerated / blast phase (~10%)
42
Typical age of onset of CML?
35-55 | Ahmed - Saeid
43
Key features of CML on blood tests
Haemoglobin - normal (unlikely v anaemic) WCC - 30 (v. v. high - Leukocytosis) Platelets 450 (high - if chronic may have dropped, 50% have elevated platelet count) Neutrophils 15 (neutrophilia) Basophils (might be elevated blood count) Monocytes (
44
Clinical Presentation of Chronic Leukaemia (4 points)
Slower proliferation of malignant cells Less burden of disease in bone marrow Clonal cells can pool in lymph nodes or in the spleen Clinical manifestations – lymphadenopathy, splenomegaly
45
CELLS WITH HIGH NUCLEUS - CYTOPLASM RATIO ON BLOOD FILM
ALL (maybe AML - can't tell apart)
46
Ix for ALL
Bone Marry Biopsy & Flow Cytometry
47
BCR-ABL1 t(9;22) associated with?
20-30% of ALL in adults | may indicate CML too
48
Mx for ALL?
Imatinib or other tyrosine kinase inhibitor for bCR-ABL1 possibly transplant
49
Key features of AML on blood tests
``` low hb high WCC low platelets low neutrophils raised blasts INR normal (AML) INR high (DIC due to acute proteomyelocytic leukaemia) ```
50
Auer Rods?
AML | possibly MDS
51
what are faggot cells?
stacks of auer rods - indicate AML
52
Ix for AML if no auer rods seen?
Flow Cytometry - (MPO)
53
What is acute promyelocytic leukaemia?
type of AML with good prognosis and different mx (w/ ATRA) that presents with DIC (clotting and procoagulant depletion - clots & difficulty forming more clots - low PLT and fibrinogen)
54
AML treatment
Imatinib similar to ALL - (chemotherapy) poor prognosis, particularly in elderly who can't tolderate stem cell transplant bone marrow transplant if treatment not working
55
What are myeloproliferative neoplasms?
Excess production of red cells, white cells or megakaryocytes or any other myeloid cell.
56
What are myeloproliferative neoplasms?
INCREASED PRODUCTION OF MYELOID LINEAGE CELLS
57
PLATELET COUNT >450 CONSISTENTLY (no hx of recent infection where it can be raised) KEY COMPLICATION MX
ESSENTIAL THROMBOCYTHAEMIA STROKE HYDROXYCARBAMIDE & ASPIRIN (to reduce stroke risk)
58
What are the 4 main myeloproliferative disorders?
Polycythaemia Vera (RBCs) Essential Thrombocythaemia (PLTs) CML (granulocytes) Myelofibrosis (stem cells fibrose bone marrow - all cell lineages REDUCED)
59
What medication lowers myeloid cell count?
Hydroxycarbamide
60
Haemocrit >0.5 ±0.2 High PLT dx mutation % important complication mx
POLYCYTHAEMIA VERA JAK2 MUTATION 95% MX ASPIRIN to reduce stroke risk VENESECTION to lower haematocrit HYDROXYCARBAMIDE to lower count
61
Low RBC Low WCC Low PLT Dx & Ix Mutation & % features Mx ASSOCIATION ON BLOOD FILM
Myelofibrosis - clonal proliferation of stem cells in bone marrow --> cytokine release and fibrosis of bone marrow --> reduced prod. all myeloid cell lineages Ix: BONE MARROW BIOPSY - DRY TAP!! JAK2 MUTATION in 50% Pancytopenia SPLENOMEGALY - can be MASSIVE SPLENOMEGALY Mx: STEM CELL TRANSPLANT likely only cure RULOXITINIB - JAK inhibitor! TEARDROP CELLS
62
age range of CML? common presenting complaints?
35-55 LUQ pain & splenomegaly
63
``` mildly low Hb v high WCC high PLT high NEUT possibly high basophils low monocytes ```
CML
64
Left Shift on blood film
CML
65
CML blood film features
``` Features: “Left shift” Leukocytosis Eosinophilia Basophilia ``` Hypolobated megakaryocytes in bone marrow
66
The bigger the cells the more _____ they are
immatrure
67
mutation in CML which Ix used?
PHILADELPHIA CHROMOSOME aka BCR-ABL1 fusion gene translocation t(9;22) FISH (fluo in situ hybridisation)
68
What are the phases of CML?
3 phases Chronic (85-90%) Accelerated (gradually more blasts in bone marrow) Blast Phase (20% blasts in bone marrow - behaves like AML)
69
Management of CML Prognosis worst case scenario?
Tyrosine Kinase Inhibitors: 1st gen: Imatinib 2nd gen: Dasatinib, Niltinib, Bosutinib 3rd gen: Pontaninib >90% 10 yr survival Small percentage of patients will fail treatment and need transplants
70
Signs and Symptoms of CLL
``` ASYMPTOMATIC dx on bloods AGE >50 - incidence increases w age Men:Women 2:1 can present with lymphadenopathy/splenomegaly, ITP/haemolytic anaemias ```
71
``` Normal Hb (occasionally low) EXTREMELY high WCC (100) PLT normal NEUT normal Lymphocytes 95 (WCC made up of mature lymphocytes) ```
CLL
72
Smear cells (blood film)
CLL
73
Smudge Cells (blood film)
CLL
74
Ix with CLL?
Flow cytometry Usually B-cell but can get T-cell CLL Same pathology as small lymphocytic lymphoma but different distribution: blood/marrow vs lymph nodes - i.e.more lymph nodes than blood
75
Mx w CLL
STAGE DEPENDENT: A - no cytopenia, <3 areas of lymphoid involvement B – no cytopenia, 3+ areas of lymphoid involvement C – cytopenias A – watch and wait B – consider treatment C – treat Richters syndrome: transformation of CLL to aggressive disease (ALL / high grade lymphoma) Treat with either: IBRUTINIB (Brutons TK Inhibtor) FCR (fludarabine, cyclophosphamide, rituximab) Stem Cell Transplant
76
2 other ways of describing the philadelphia chromosome? how is it detected?
BCR-ABL1 fusion gene t(9;22) by FISH (fluorescene in situ hybridisation)
77
CML Treatment? if fails?
Imatinib Transplants
78
how does CML present?
RUQ pain and splenomegaly
79
how does CLL present?
asymptomatic - picked up on routine blood tests
80
What is pathagnomonic for CLL?
Smear Cells / Smudge Cells
81
Blood film of CLL
Lymphocytosis | SMEAR/SMUDGE CELLS
82
Treatment of CLL?
(if stage C) Ibrutinib or FCR (fludarabine, cyclophosphamide, rituximab) if failing, Stem cell transplant
83
Which leukaemia is associated with very high lymphocytes?
CLL | lymphoid lineage
84
Which leukaemias are indicated by: Auer Rods Smear Cells Left Shift
Auer rods - AML Smear/Smudge Cells - CLL Left Shift (more immature cells, less mature cells) -CML
85
Way of remembering what smear/smudge cells indicate?
They are chronic and they are LL lymphoblastic leukaemia because they are S/S i.e. double letter
86
Which leukaemia is associated with high basophils?
CML | myeloid lineage
87
Which leukaemias are associated with higher levels of blasts in the marrow?
Acute Leukaemias have over 20% blasts in the bone marrow
88
Which leukaemia *can* have a high platelet count?
CML (myeloid lineage)
89
Which leukaemia is associated with high eosinophils?
CML | myeloid lineage
90
Which leukaemias have blast cells in the blood?
I think all of them can have them, however blasts are more characterisitc of acute leukaemias
91
What can go on to form AML?
Myelodysplastic Syndromes?
92
What are Myelodysplastic Syndromes?
Dysplastic changes - abnormal cells 1 or more myeloid cell lines (erythroids, megakaryocyte, granulocyte) Usually asymptomatic - however there is risk of progression to AML Present with incidental cytopenia
93
Pelger Huet Cells =
Hyposegmented Neutrophils Pseudo-Pelger Anomally causes: Congenital (lamin B Receptor mutation) Acquired (myelogenous leukaemia and myelodysplastic syndromes (granulocytic lineage))
94
How does MDS present?
Clinical Features • BM failure and cytopenias – infection, bleeding, fatigue • Hypercellular BM • Defective cells: o RBCs e.g. ring sideroblasts (abn nucleated blast surrounded by iron granule ring) o WBCs – hypogranulation, Pseudo-Pelger-huet anomaly (hyposegmented neutro) o Platelets – micromegakaryocytes, hypolobated nuclei N.B. In the exam – use an ‘investigative approach’ to pick out clues that lead to classification
95
Key difference between MDS and Leukaemia?
<20% blasts in MDS, >20% blasts acute leukaemia
96
What are Myelodysplastic Syndromes?
Heterogeneous group of progressive disorders featuring ineffective proliferation and differentiation of abnormally maturing myeloid stem cells. • Characterised by: peripheral cytopenia; qualitative abnormalities of cell maturation; risk of AML transformation. • Typically seen in the elderly; symptoms usually develop over weeks/months (incidental) • By definition all patients have <20% blasts (>20% blasts = acute leukaemia)
97
Treatment of MDS?
Treatment • Supportive – transfusions, EPO, G-CSF, ABx • Biological modifiers – immunosuppressive drugs, lenalidomide, azacytidine • Chemotherapy – similar to AML • Allogeneic SCT
98
Where are the different types of MDS found and what are the types?
``` BLOOD RBCs: Refractory Anaemia (RA) +/ ring sideroblasts ( RARS ) PLATELETS: MDS w/ 5q deletion MDS unclassified WBCs: Refractory Cytopenia with Multilineage Dysplasia (RCMD) +/ ring sideroblasts ( RCMD RS ) ``` BONE MARROW Refractory anaemia with excess blasts (RAEB I) <5% Blasts (RAEB II) 5-19% Blasts
99
Explain staging of both Hodgkin's and Non-Hodgkin's Lymphoma
Ann-Arbor Staging Stage I - IV Stage I - One Group of Lymph Nodes (e.g. axillary, cervical, mediastinal) Stage II - More Than One Group of Lymph Nodes That Are Above The Diaphgragm Stage III - More Than One Group of Lymph Nodes That Are Above and Below the Diaphragm Stage IV - Bone Marrow / Spleen Involement
100
How are Lymphomas classified?
Hodgkin's vs Non-Hodgkin's Hodgkin's has 4 types Non-Hodgkin's is B-cell vs T-Cell (ATLL) Very High vs High vs Low Grade
101
What is pathagnomonic for Hodgkin's Lymphoma?
Reed-Sternburg Cells | owl eye cells
102
Hodgkin's Lymphoma - profile and typical presentation prognosis
affects young pc: lymphadenopathy (often mediastinal) or B symptoms Reed-Sternburg Cells are diagnostic usually good prognosis, stem cell transplant used if fails
103
What disease is associated with Hodgkin's Lymphoma?
EBV | Ebstein Barr Virus infection
104
What is the most common subtype of Hodgkin's Lymphoma?
Nodular Sclerosing
105
What is the most common slow growing (indolent) lymphoma?
Follicular
106
Lymph node biopsy shows large numbers of centroblasts
Follicular Lymphoma
107
t(14;18) ?
causes fusion of BCL2 gene follicular lymphoma
108
What does centroblast look like on biopsy?
perfect circle of many cells
109
What are the 4 types of Hodgkin's Lymphoma?
nodular sclerosis classical Hodgkin lymphoma mixed cellularity classical Hodgkin lymphoma lymphocyte-rich classical Hodgkin lymphoma lymphocyte-depleted classical Hodgkin lymphoma
110
Features of Follicular Lymphoma How does it present? Risk?
Small lymphocytic lymphoma similar to CLL but disease with disease in NODES Presents with lymphadenopathy, usually few very large nodes Risk of transformation to high grade lymphoma
111
Treating Follicular Lymphoma
Is Non-Hodgkin's Lymphoma subtype Expectant management unless high burden of disease
112
Key features of Non-Hodgkin's Lymphoma
Many sub-types – mostly B-cell origin, can be T-cell (ATLL) Present with B-symptoms or lymphadenopathy Incidence increases with age
113
Treatment of Hodgkin's Lymphoma
Treatment is with ABVD chemotherapy usually + radiotherapy Most patients have a good chance of cure Stem cell transplants for rare cases who fail treatment

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