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AANIYA PATH > Aani Haem: MM/MPS/MDS > Flashcards

Aani Haem: MM/MPS/MDS Flashcards

1
Q

What is the normal healthy ratio of kappa: lambda Igs?

A

60:40 Kappa:lambda

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2
Q

What do you get made in MM?

A

Production of monoclonal Ig (paraproteins)

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3
Q

Which test can be done to detect MM?

A

Serum Free Light Chain electrophoresis

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4
Q

What is the Kappa:Lambda ratio in MM?

A

K:L 99:1

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5
Q

What is an immunoglobulin made up of?

A

2 light chains & 2 heavy chains

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6
Q

What is a plasma cell?

A

A differentiated B lymphocyte whose function is to create Abs

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7
Q

What are the clinical features of MM?

A

CRAB

Calcium is high
Renal failure
Anaemia (+pancytopaenia)
Bones - pain, osteoporosis, osteolytic lesions, fractures, pepper pot skull

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8
Q

Why do you get renal failure in MM?

A

because the free light chains are excreted by the kidney but are toxic

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9
Q

What are free light chains?

A

Fragmented Igs

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10
Q

Why do you get bone symptoms in MM?

A

Because plasma cells stimulate osteoclasts

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11
Q

Why do you get bone symptoms in MM?

A

Because plasma cells stimulate osteoclasts and inhibit osteoblasts

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12
Q

Why do you get pancytopaenia in MM?

A

Overcrowing of plasma cells in BM can crowd out normally functioning cells

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13
Q

What do you see on electrophoresis in MM?

A

A dense narrow band (M protein). This is made up of the monoclonal identical Igs

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14
Q

Which staging system is used in MM?

A

Durie-Salmon staging

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15
Q

Which cytokine markers are in MM?

A

CD 138
CD 38
CD 56
CD 58

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16
Q

Which chains do you see in MM?

A

Bence-Jones Protein

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17
Q

How much of the BM needs to be plasma cells to diagnose MM?

A

At least 10% with other symptoms
OR
isolated 60% (smouldering myeloma - >10% plasma cells in BM but no CRAB symptoms)

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18
Q

What do you see on the MM blood film?

A

Rouleaux (Stacking of RBCs)

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19
Q

Will the ESR be high or low in MM?

A

High

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20
Q

Describe what MM plasma cells look like?

A

Their nucleus has been pushed to the side because of the enlarged cytoplasm

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21
Q

Mnemonic to remember treatment of MM?

A

Stay Put In Class
MM is an educational society that wants you to stay put in class

Steroids
Proteosome inhibitors
Immunomodulators
Chemo cytostatic drugs

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22
Q

What is the treatment for MM?

A

SPIC
Steroids e.g. Dexamethasone or Prednisalone

Proteosome Inhibitors e.g. BORTEZOMIB

Immunomodulators e.g. Lenalidomide

Chemo e.g. Melphalan (block DNA replication)

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23
Q

What is it when there is >10% plasma cells in BM but no CRAB symptoms?

A

Smouldering Myeloma

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24
Q

Which chromosomes are affected in MGUS to MM transformation?

A

Odd numbers e.g. 3,5,11 etc

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25
How to you manage MGUS?
You don't- just watch for transformation into MM
26
How to you manage smouldering myeloma?
You don't- just watch for transformation into MM
27
What does MGUS stand for?
Monoclonal Gammaglobinopathy of Unknown Significance
28
What is the plasma cell levels in MGUS?
<10% plasma cells in BM. No CRAB symptoms
29
Which race are more affected by MM?
Black
30
What is a paraprotein?
abnormal immunoglobulin fragment or immunoglobulin light chain that is produced in excess by an abnormal monoclonal proliferation of plasma cells
31
Other terms for paraproteins?
M protein, M component, spike protein, myeloma protein
32
What are the 3 conditions in which paraproteins are made? (Paraproteinaemias)
Multiple Myeloma MGUS Waldenstrom;s Macroglobinaemia (or LPL)
33
What condition can you get as a result of paraproteins?
Amyloidosis - deposition of paraproteins in tissue
34
Which stain do you do for amyloidosis?
Congo-Red stain (under polarised light)
35
What will you find after staining in amyloidosis?
Apple Green Bifringents
36
Which demographic are more likely to get Waldentrom's?
Elderly Men (Think: Old Man Waldo)
37
What is Waldenstrom's Macroglobinaemia ?
A low grade Non hodgkin's Lymphoma. | Monoclonal serum igM inflitrates LNs and BM.
38
Complications of paraproteins?
Hyperviscocity Renal failure Amyloidosis Immune Suppression
39
Describe what you would find in myeloma bone disease?
- Pain - Lytic lesions e.g. Salt and pepper skull - Cord compression due to vertebral fractures
40
Describe what you would find in myeloma nephropathy?
- Cast injury (blocked tubules) - Inflammation of prox tubules --> necrosis - Crystals (faconi syndrome, flc crystal deposition in kidneys)
41
treatment for Waldenstrom's Macroglobinaemia?
Cyclophosphamide Plasmapheresis Chlorambucil
42
What is Myelodysplastic Syndrome?
Immature blood cells in the bone marrow do not mature
43
How many blasts cells in MDS bone marrow?
<20%
44
How many blast cells in a normal bone marrow?
<5%
45
How many blast cells in AML bone marrow?
>20%
46
If MDS gets worse it can progress to which kind of leukaemia?
AML
47
Clinical features of MDS?
Bone marrow failure: Anaemia (fatigue) Thrombocytopaenia (bleeding) Neutropaenia (Infection)
48
Which possible defective cells might you see in MDS bone marrow?
RBCs: Ringed Sideroblasts (RBCs with abnormal iron around nucelus) WBS: Hyposegmented neutrophils Platelets: Micromegakaryocytes with hypolobated nuclei
49
What are the different types of MDS?
1. Refractory Anaemia (RA) 2. Refractory Anaemia with Ringed Siderobalsts (RARS) 3. Refractory Cytopaenia with Multilineage Dysplasia (RCMD) 4. Refractory Cytopaenia with Miltilineage Dysplasia and Ringed Sideroblasts (RCMDRS) 5. Myelodysplasia Syndrome with 5q deletion (MDS 5q del) 6. Refractory Anaemia with Excess Blasts 1 (RAEB 1) 7. Refractory Anaemia with Excess Blasts 2 (RAEB 2) They come in 3 pairs plus 1 (5q deletion)
50
Describe the BM features in RA (of MDS)?
Erythroid dysplasia with <5% blasts
51
Describe the BM features in RARS (of MDS)?
Erythroid dysplasia with <5% blasts and >15% Ringed Sideroblasts
52
Describe the BM features in RCMD (of MDS)?
Dysplasia in >10% cells from 2+ different lineages
53
Describe the BM features in RCMDRS (of MDS)?
Dysplasia in >10% cells from 2+ different lineages and >15% Ringed Sideroblasts
54
Describe the BM features in RAEB1 (of MDS)?
Dysplastia 5-9% blasts
55
Describe the BM features in RAEB2 (of MDS)?
Dysplasia 10-19% blasts And Auer Rods (almost AML)
56
Describe the BM features in MDS 5q deletion (of MDS)?
Megakaryocytes with hypolobated nuclei and <5% blasts
57
Treatment of MDS?
Supportive: Transfusions EPO G-CSF (for neutrophils) ABx for infections Immunomodulators e.g. Lenalidomide Chemo Allogenic SCT
58
What is the prognosis of MDS?
Rule of 1/3s. 1/3 will die from infection 1/3 will die from bleeding 1/3 will die from AML
59
How many MDS pts progress to AML?
50%
60
What are myeloproliferative disorders?
Conditions that cause the BM to produce excess cells i.e. proliferation of mature cells. This is of one or more cell line.
61
How do you categorise MPD?
Philadelphia Cr + | Philadelphia Cr -
62
What are the Philadelphia Cr + MPDs?
CML
63
What are the Philadelphia Cr - MPDs?
PRV (Polycythaemia Rubra Vera) ET (Essential Thrombocytosis) MF (myelofibrosis)
64
Which mutation is associated with Philadelphia Cr Negative MPDs?
JAK2 mutations
65
Which MPD is most associated with JAK2?
PRV - 1-00% of PRV pts have JAK2 mutation. JAK2 v617F has worse prognosis
66
What does the JAK2 mutation do?
The JAK2 mutation eliminates the BM's need for growth factors in order to proliferate i.e. uncontrolled proliferation
67
What are the causes of polycythaemia?
Primary: PRV Familial polycythaemia Secondary: Renal Cancer High altitude Chronic hypoxia
68
What is pseudopolycythaemia?
When the red cell mass is normal but plasma volume has dropped so it appears as though the red cell mass is increased. (relative)
69
What causes pseudopolycythaemia?
``` Alcohol Dehydration Burns Smoking Obesity Diuretics ```
70
Which specific point mutation is associated with PRV?
JAK2 V612F
71
Clinical features of PRV?
``` Blurred vision Headache Stroke Red nose Gout (due to increased red cell turnover) Erythromelagia (painful extremities) Splenomegaly ```
72
Investigations for PRV?
Hb wil be raised Hct will be raised Low serum EPO (due to neg feedback) Sometimes high WCC
73
Treatment of PRV?
Venesection Reduce viscocity - aspirin (reduce platelets) Hydroxycarbamide
74
Describe BM aspirate of PRV?
No fat spaces, taken up with cells esp platelets and RBCs
75
What is the target platelet range in PRV treatment?
400 x 10^9
76
Causes of appropriate polycythaemia?
High altitude Hypoxic lung disease e.g. CF Cyanotic Heart Disease High affinity Hb
77
Causes of INappropriate polycythaemia?
Renal Cell Carcinoma Doping Uterine Myoma Ectopic EPO excretion from other tumours
78
What is the prognosis of polycythaemia?
Risk of progression to CML | Many pts die of thombosis
79
What is Myelofibrosis (MF)?
A MPD where there is also fibrosis of the BM or collagenous replaces BM tissue
80
Which mutation is associated with MF?
50% have JAK2 (unlike PRV where 100% have JAK2)
81
Describe the course of Myelofibrosis
Pre-fibrotic phase - hypocellular | Fibrotic phase - splenomegaly
82
Diagnostic test for myelofibrosis?
BM aspirate - Dry Tap
83
What blood changes do you get in myelofibrosis?
Initially high WCC then later pancytopaenia
84
Clinical features of myelofibrosis?
Massive splenomegaly Can present with Budd-Chiari syndrome Pancytopaenia symptoms
85
Blood film of myelofibrosis?
Tear drop Poikilocytes | Leukoerythrobloasts
86
Treatment of Myelofibrosis?
``` Ruxolitinib (JAK2 inhibitor) Treat symptoms e.g. aneamia with transfusions Thalidomide Steroids SCT Hydrocarbamide ```
87
What is essential thrombocythaemia?
An MPD where megakaryocytes dominate the BM
88
Which mutations are ass. with Essential Thrombocythaemia?
50% JAK2 | EXON 12 too
89
What do you see on BM aspirate for essential thrombocythaemia?
Clustering of megakaryocytes
90
What is an unusual clinical symptom of essential thrombocythaemia?
Bleeding! Some people's platelets are hyporeactive and can cause bleeding
91
Clinical features of essential thrombocythaemia?
- VTE - Stoke - MI - Splenomegaly - Dizziness - Visual disturbances
92
Definitive investigation for Essential Thrombocythaemia?
Platelets will consistently be >600 x 10^9
93
What does hydroxycarbamide do?
It is an antimetabolite. It suppresses cells
94
Treatment of Essential Thrombocythaemia?
Aspirin (obv, reduces platelets) Anagrelide - reduces formation of platelets by megakaryocytes Hydroxycarbamide - cell metabolsim inhibitor
95
What will you see on Essential Thrombocythaemia Blood film?
Large platelets, megakaryocyte fragments
96
What does anagrelide do?
It reduces protuction of platelets from megakaryocytes

AANIYA PATH (35 decks)

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