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Haematology > Myeloproliferative, myelodysplastic and aplastic anaemia > Flashcards

Myeloproliferative, myelodysplastic and aplastic anaemia Flashcards

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1
Q

Haemopoesis Definition

A

The production of blood cells

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2
Q

Where does haemopoesis occur in the foetus?

A

0-2 months - yolk sac
2-7 months - liver, spleen
5-9 months - bone marrow

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3
Q

Where does haemopoesis occur in infants

A

Bone marrow - all bones

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4
Q

Where does haemopoesis occur in adults

A

Bone marrow

vertebrae, ribs, sternum, skull, sacrum, pelvis, ends of femur

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5
Q

Haemopoesis hierarchy

A

DRAW

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6
Q

Pluriopotent stem cell

A

Numbers diminish with age, committed to divide

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7
Q

Colony forming unit

A

commited to divide

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8
Q

Where are haemopoetic stem cells found?

A

BM
Peripheral blood after treatment with G-CSF
Umbillical cord blood

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9
Q

Haempoetic stem cell characteristics

A
  • self renewal
  • unspecialised
  • ability to differentiate (mature)
  • quescent (non in cell cycle)
  • rare cell
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10
Q

Control of stem cell fate

A

HSC can

a. undergo self-renewal (identical copy)
b. Apoptosis (programmed cell death)
c. Differentations (Matureation and specialisation)

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11
Q

Types of stem cell division

A

Types of division

Symmetrical - both daughter cells mature leading to a contraction in stem cell numbers

Assymetrical division- one new daughter cell (maintenance of numbers), one differentiations

Symmetrical stem cell - Expansion
of stem cell number

Balance is influencced by complex interplay of micro-environment (the niche) and internal ques

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12
Q

Stroma definition

A

BM microenviroment that supports the developing haemopoetic stem cell

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13
Q

Stroma/ bone marrow microenvironment features

A

Stromal cells + ECM

Stromal cells - macrophages, fibroblasts, endothelial cells, fat cells, reticulum cells

Surrounded by ECM
-laminin, fibronectin, collagen, Proteoglycans

Sinusoids: network of BVs lined by a singel layer of endothelial cells, support haemopoetic cells, allow passage of newly formed cells into the circulation

Haemopoetic cords/islands
- site of haemopoesis

Macrophages

  • found within haemopoetic cords
  • contain stored iron (haemosiderrin + ferritin)
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14
Q

Pre-malignant and malignant conditions are termed clonal because..?

A

They arise from a single ancestral cells

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15
Q

Examples

A
  1. over-production = Myeloproliferative disorder
  2. Under production = Aplastic anaemia (e.g fanconis)
  3. Abnormal cells produced
    = myelodysplasia, leukaemia
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16
Q

Myeloproliferative disorders

A

Polycythaemia rubra vera
Essential thrombocytosis
Myelofibrosis

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17
Q

Myeloproliferative disorders definition

A

Uncontrolled proliferation of one or more of the myeloid stem cell line. While the cells proliferate the also retain the ability to differntiate into RBCs, WBCs or platelets

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18
Q

Myelofibrosis definition

A

Marrow scarring due to hyperplasia of megakarocytes which causes increased production of PDGF

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19
Q

Causes of myelofibrosis

Study These Flashcards
A

50% JAK2, 50% calreticulin

Increased PDGF leads to myeloid metaplasia

Massive hepatomegaly

20
Q

Presentation of myelofibrosis

Study These Flashcards
A

constitutional symptoms

Abdominal discomfort - massive spleenn

BM failure (Anaemia, Infection, bleeding)

21
Q

Investigations of myelofibrosis

Study These Flashcards
A

BM

  • aspirate- dry
  • trephone - shows fibrosis

FBC

  • pts in high then thrombocytopenia
  • decreased RBC
  • WCC increased or decreased

Blood fillm

  • tear drops RBCs
  • leucoerythroblastic cells
22
Q

Treatment of myelofibrosis

Study These Flashcards
A

Under 50
- myeloblative SCT

50-65
- non-myeloblative BMT

23
Q

Prognosis of myelofibrosis

Study These Flashcards
A

can be curative with transplant

24
Q

Essential thrombocytosis definition

Study These Flashcards
A

Clonal proliferation of megakaryocytes leads to increased platelets (thrombocytosis) with impaired function

Pt >600x 10 9 peristently

25
Cause of Essential thrombocytosis
50% calreticulin mutation | 50% JAK2
26
Clinical features of essential thrombocytosis
microvascular occlusion - headaches - dizziness - erythomegaly - splenomegaly - thombosis - bleeding
27
Investigations of essential thrombocytosis
exclude other causes (bleeding, infection, malignancy) FBC (+ repeat) Pt >600 x109 persistently ESR, CRP, Fibrinogen
28
Treatment of essential thrombocytosis
Stratification into risk groups Low risk - (60 - one or more high risk features plt >1500 x109/l previous thrombosis or thrombotic risk factors e.g diabetes or hypertension
29
Polycythaemia rubra vera definition
increase in RBC volume due to a clonal malignancy of a marrow stem cell
30
Causes of Polycythaemia rubra vera
Jak2 mutation is present in more than 90%
31
Clinical features of Polycythaemia rubra vera
due to hyperviscosity and hypervolaemia - headaches - dizziness - visual disturbances - tinnitus - pruritis (after hot bath) - erythromyalgia - gout (increased urate) - plerothic complexion - splenomegaly - thrombosis - haemorrhage
32
Investigations of Polycythaemia rubra vera
``` bloods -increased RCC -increased packed cell volume - increased Hb Blood film JAK2 mut Serum ferritin ```
33
Management of Polycythaemia rubra vera
``` no cure venesection low dose aspirin -cytotoxic drugs e.g hydroxycarbamine if symptomatic -splenomegaly - radioactive phosphorus ```
34
Prognosis of Polycythaemia rubra vera
Most remain well | -thrombosis/ haemorrhage main complocation
35
Classification of myeloproliferative disorders based on the cell type
RBC - polycythaemia rubra vera WBC - chronic myeloid leukaemia Platelets - Essential thrombocytopenia Fibroblasts - myelofibrosis
36
High risk ET treatment
First line = apsirin + hydroxycarbamide (ribonucelotide reductase inhibitor resulting in reduced production of deoxyribonucleotides Second line therapy = anagrelide + aspiring - inhibits megakaryocyte differentiation Inf-a useful in management of ET in pregnancy JAK2 inhbitrs e.g ruxoltinib
37
JAK 2 inhibitors mechanism of action
JACK2 mutations result in continuous activatio of JAK receptor regardless of ligand binding Specifically ruxotilinib Inhibits JAK1 and 2 -70% of pts reduuced splenomegaly and functional improvement
38
Myelodysplastic syndrome (MDS) definition
Dysplasia and ineffective haemooesis in >1 o the myeloid series (RBC, Pts, Neutrophils)
39
Features of Myelodysplastic syndrome (MDS)
Common in elderly 25% transform to AML de novo or secondary to previous chemo/rx associated with acquired cytogenetic abnormalities
40
CFs Myelodysplastic syndrome (MDS)
20% present with infections | 70-80% present with fatigue due to anaemia
41
Management of Myelodysplastic syndrome (MDS)
Depends on age of patient - supportive care- blood and plt transfusion - Growth factors - erythropoetin + granucolcyte colony stimulating factor (not much success as stimulating cells produces more dysplastic cells) - Low dose chemo e.g hydroxycarbamide, low dose cytarabine - immunosuppression - high dose chemo - only in selected patients (AML-type) - Allogenic stem cell transplantation only in selevted patients
42
Types of Myelodysplastic syndromes (MDS)
Multiple sub-types based on morphology and % blasts 1. refractory anaemia or 2. refractory anaemia with sideroblasts (perinuclear ring of iron granules) 3. refractory anaemia with excessive blasts
43
Fanconis anaemia features
10-20% of aplastic anaemia cases Autosomal recessive inheritance BONE MARROW FAILURE
44
Fanconis anaemia characteristics
- somatic abnormalites - bone marrow failure - short telomeres - malignancy - chromosome instability
45
Types of Fanconis anaemia
7 genetic FANCA to FANC-G
46
Clinical features of Fanconis anaemia
``` micropthalmia GU malformations GI malformations mental retardation Hearing loss CNS e.g hydrocephalus ```
47
Treatment of Fanconis anaemia
``` Gold standard- allogenc stem cell transplant Other options are: -supportive care -corticosteroids -androgens ``` Life time surveillance for secondary tumours

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