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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
Q

How to you manage MGUS?

A

You don’t- just watch for transformation into MM

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26
Q

How to you manage smouldering myeloma?

A

You don’t- just watch for transformation into MM

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27
Q

What does MGUS stand for?

A

Monoclonal Gammaglobinopathy of Unknown Significance

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28
Q

What is the plasma cell levels in MGUS?

A

<10% plasma cells in BM. No CRAB symptoms

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29
Q

Which race are more affected by MM?

A

Black

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30
Q

What is a paraprotein?

A

abnormal immunoglobulin fragment or immunoglobulin light chain that is produced in excess by an abnormal monoclonal proliferation of plasma cells

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31
Q

Other terms for paraproteins?

A

M protein, M component, spike protein, myeloma protein

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32
Q

What are the 3 conditions in which paraproteins are made? (Paraproteinaemias)

A

Multiple Myeloma
MGUS
Waldenstrom;s Macroglobinaemia (or LPL)

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33
Q

What condition can you get as a result of paraproteins?

A

Amyloidosis - deposition of paraproteins in tissue

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34
Q

Which stain do you do for amyloidosis?

A

Congo-Red stain (under polarised light)

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35
Q

What will you find after staining in amyloidosis?

A

Apple Green Bifringents

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36
Q

Which demographic are more likely to get Waldentrom’s?

A

Elderly Men (Think: Old Man Waldo)

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37
Q

What is Waldenstrom’s Macroglobinaemia ?

A

A low grade Non hodgkin’s Lymphoma.

Monoclonal serum igM inflitrates LNs and BM.

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38
Q

Complications of paraproteins?

A

Hyperviscocity
Renal failure
Amyloidosis
Immune Suppression

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39
Q

Describe what you would find in myeloma bone disease?

A
  • Pain
  • Lytic lesions e.g. Salt and pepper skull
  • Cord compression due to vertebral fractures
40
Q

Describe what you would find in myeloma nephropathy?

A
  • Cast injury (blocked tubules)
  • Inflammation of prox tubules –> necrosis
  • Crystals (faconi syndrome, flc crystal deposition in kidneys)
41
Q

treatment for Waldenstrom’s Macroglobinaemia?

A

Cyclophosphamide
Plasmapheresis
Chlorambucil

42
Q

What is Myelodysplastic Syndrome?

A

Immature blood cells in the bone marrow do not mature

43
Q

How many blasts cells in MDS bone marrow?

A

<20%

44
Q

How many blast cells in a normal bone marrow?

A

<5%

45
Q

How many blast cells in AML bone marrow?

A

> 20%

46
Q

If MDS gets worse it can progress to which kind of leukaemia?

A

AML

47
Q

Clinical features of MDS?

A

Bone marrow failure:
Anaemia (fatigue)
Thrombocytopaenia (bleeding)
Neutropaenia (Infection)

48
Q

Which possible defective cells might you see in MDS bone marrow?

A

RBCs: Ringed Sideroblasts (RBCs with abnormal iron around nucelus)
WBS: Hyposegmented neutrophils
Platelets: Micromegakaryocytes with hypolobated nuclei

49
Q

What are the different types of MDS?

A
  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
Q

Describe the BM features in RA (of MDS)?

A

Erythroid dysplasia with <5% blasts

51
Q

Describe the BM features in RARS (of MDS)?

A

Erythroid dysplasia with <5% blasts and >15% Ringed Sideroblasts

52
Q

Describe the BM features in RCMD (of MDS)?

A

Dysplasia in >10% cells from 2+ different lineages

53
Q

Describe the BM features in RCMDRS (of MDS)?

A

Dysplasia in >10% cells from 2+ different lineages and >15% Ringed Sideroblasts

54
Q

Describe the BM features in RAEB1 (of MDS)?

A

Dysplastia 5-9% blasts

55
Q

Describe the BM features in RAEB2 (of MDS)?

A

Dysplasia 10-19% blasts And Auer Rods (almost AML)

56
Q

Describe the BM features in MDS 5q deletion (of MDS)?

A

Megakaryocytes with hypolobated nuclei and <5% blasts

57
Q

Treatment of MDS?

A

Supportive: Transfusions
EPO
G-CSF (for neutrophils)
ABx for infections

Immunomodulators e.g. Lenalidomide
Chemo
Allogenic SCT

58
Q

What is the prognosis of MDS?

A

Rule of 1/3s.
1/3 will die from infection
1/3 will die from bleeding
1/3 will die from AML

59
Q

How many MDS pts progress to AML?

A

50%

60
Q

What are myeloproliferative disorders?

A

Conditions that cause the BM to produce excess cells i.e. proliferation of mature cells. This is of one or more cell line.

61
Q

How do you categorise MPD?

A

Philadelphia Cr +

Philadelphia Cr -

62
Q

What are the Philadelphia Cr + MPDs?

A

CML

63
Q

What are the Philadelphia Cr - MPDs?

A

PRV (Polycythaemia Rubra Vera)
ET (Essential Thrombocytosis)
MF (myelofibrosis)

64
Q

Which mutation is associated with Philadelphia Cr Negative MPDs?

A

JAK2 mutations

65
Q

Which MPD is most associated with JAK2?

A

PRV - 1-00% of PRV pts have JAK2 mutation. JAK2 v617F has worse prognosis

66
Q

What does the JAK2 mutation do?

A

The JAK2 mutation eliminates the BM’s need for growth factors in order to proliferate i.e. uncontrolled proliferation

67
Q

What are the causes of polycythaemia?

A

Primary:
PRV
Familial polycythaemia

Secondary:
Renal Cancer
High altitude
Chronic hypoxia

68
Q

What is pseudopolycythaemia?

A

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
Q

What causes pseudopolycythaemia?

A 
Alcohol
Dehydration
Burns
Smoking
Obesity
Diuretics
70
Q

Which specific point mutation is associated with PRV?

A

JAK2 V612F

71
Q

Clinical features of PRV?

A 
Blurred vision
Headache
Stroke
Red nose
Gout (due to increased red cell turnover)
Erythromelagia (painful extremities)
Splenomegaly
72
Q

Investigations for PRV?

A

Hb wil be raised
Hct will be raised
Low serum EPO (due to neg feedback)
Sometimes high WCC

73
Q

Treatment of PRV?

A

Venesection
Reduce viscocity - aspirin (reduce platelets)
Hydroxycarbamide

74
Q

Describe BM aspirate of PRV?

A

No fat spaces, taken up with cells esp platelets and RBCs

75
Q

What is the target platelet range in PRV treatment?

A

400 x 10^9

76
Q

Causes of appropriate polycythaemia?

A

High altitude
Hypoxic lung disease e.g. CF
Cyanotic Heart Disease
High affinity Hb

77
Q

Causes of INappropriate polycythaemia?

A

Renal Cell Carcinoma
Doping
Uterine Myoma
Ectopic EPO excretion from other tumours

78
Q

What is the prognosis of polycythaemia?

A

Risk of progression to CML

Many pts die of thombosis

79
Q

What is Myelofibrosis (MF)?

A

A MPD where there is also fibrosis of the BM or collagenous replaces BM tissue

80
Q

Which mutation is associated with MF?

A

50% have JAK2 (unlike PRV where 100% have JAK2)

81
Q

Describe the course of Myelofibrosis

A

Pre-fibrotic phase - hypocellular

Fibrotic phase - splenomegaly

82
Q

Diagnostic test for myelofibrosis?

A

BM aspirate - Dry Tap

83
Q

What blood changes do you get in myelofibrosis?

A

Initially high WCC then later pancytopaenia

84
Q

Clinical features of myelofibrosis?

A

Massive splenomegaly
Can present with Budd-Chiari syndrome
Pancytopaenia symptoms

85
Q

Blood film of myelofibrosis?

A

Tear drop Poikilocytes

Leukoerythrobloasts

86
Q

Treatment of Myelofibrosis?

A 
Ruxolitinib (JAK2 inhibitor) 
Treat symptoms e.g. aneamia with transfusions
Thalidomide
Steroids
SCT
Hydrocarbamide
87
Q

What is essential thrombocythaemia?

A

An MPD where megakaryocytes dominate the BM

88
Q

Which mutations are ass. with Essential Thrombocythaemia?

A

50% JAK2

EXON 12 too

89
Q

What do you see on BM aspirate for essential thrombocythaemia?

A

Clustering of megakaryocytes

90
Q

What is an unusual clinical symptom of essential thrombocythaemia?

A

Bleeding! Some people’s platelets are hyporeactive and can cause bleeding

91
Q

Clinical features of essential thrombocythaemia?

A
  • VTE
  • Stoke
  • MI
  • Splenomegaly
  • Dizziness
  • Visual disturbances
92
Q

Definitive investigation for Essential Thrombocythaemia?

A

Platelets will consistently be >600 x 10^9

93
Q

What does hydroxycarbamide do?

A

It is an antimetabolite. It suppresses cells

94
Q

Treatment of Essential Thrombocythaemia?

A

Aspirin (obv, reduces platelets)
Anagrelide - reduces formation of platelets by megakaryocytes
Hydroxycarbamide - cell metabolsim inhibitor

95
Q

What will you see on Essential Thrombocythaemia Blood film?

A

Large platelets, megakaryocyte fragments

96
Q

What does anagrelide do?

A

It reduces protuction of platelets from megakaryocytes

AANIYA PATH (35 decks)

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