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Haematology > Myeloproliferative, myelodysplastic and aplastic anaemia > Flashcards

Myeloproliferative, myelodysplastic and aplastic anaemia Flashcards

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

A

50% JAK2, 50% calreticulin

Increased PDGF leads to myeloid metaplasia

Massive hepatomegaly

20
Q

Presentation of myelofibrosis

A

constitutional symptoms

Abdominal discomfort - massive spleenn

BM failure (Anaemia, Infection, bleeding)

21
Q

Investigations of myelofibrosis

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

A

Under 50
- myeloblative SCT

50-65
- non-myeloblative BMT

23
Q

Prognosis of myelofibrosis

A

can be curative with transplant

24
Q

Essential thrombocytosis definition

A

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

Pt >600x 10 9 peristently

25
Q

Cause of Essential thrombocytosis

A

50% calreticulin mutation

50% JAK2

26
Q

Clinical features of essential thrombocytosis

A

microvascular occlusion

  • headaches
  • dizziness
  • erythomegaly
  • splenomegaly
  • thombosis
  • bleeding
27
Q

Investigations of essential thrombocytosis

A

exclude other causes (bleeding, infection, malignancy)
FBC (+ repeat)
Pt >600 x109 persistently
ESR, CRP, Fibrinogen

28
Q

Treatment of essential thrombocytosis

A

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
Q

Polycythaemia rubra vera definition

A

increase in RBC volume due to a clonal malignancy of a marrow stem cell

30
Q

Causes of Polycythaemia rubra vera

A

Jak2 mutation is present in more than 90%

31
Q

Clinical features of Polycythaemia rubra vera

A

due to hyperviscosity and hypervolaemia

  • headaches
  • dizziness
  • visual disturbances
  • tinnitus
  • pruritis (after hot bath)
  • erythromyalgia
  • gout (increased urate)
  • plerothic complexion
  • splenomegaly
  • thrombosis
  • haemorrhage
32
Q

Investigations of Polycythaemia rubra vera

A 
bloods 
-increased RCC
-increased packed cell volume 
- increased Hb 
Blood film 
JAK2 mut
Serum ferritin
33
Q

Management of Polycythaemia rubra vera

A 
no cure 
venesection 
low dose aspirin 
-cytotoxic drugs e.g hydroxycarbamine if symptomatic 
-splenomegaly 
- radioactive phosphorus
34
Q

Prognosis of Polycythaemia rubra vera

A

Most remain well

-thrombosis/ haemorrhage main complocation

35
Q

Classification of myeloproliferative disorders based on the cell type

A

RBC - polycythaemia rubra vera
WBC - chronic myeloid leukaemia
Platelets - Essential thrombocytopenia
Fibroblasts - myelofibrosis

36
Q

High risk ET treatment

A

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
Q

JAK 2 inhibitors mechanism of action

A

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
Q

Myelodysplastic syndrome (MDS) definition

A

Dysplasia and ineffective haemooesis in >1 o the myeloid series (RBC, Pts, Neutrophils)

39
Q

Features of Myelodysplastic syndrome (MDS)

A

Common in elderly
25% transform to AML
de novo or secondary to previous chemo/rx
associated with acquired cytogenetic abnormalities

40
Q

CFs Myelodysplastic syndrome (MDS)

A

20% present with infections

70-80% present with fatigue due to anaemia

41
Q

Management of Myelodysplastic syndrome (MDS)

A

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
Q

Types of Myelodysplastic syndromes (MDS)

A

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
Q

Fanconis anaemia features

A

10-20% of aplastic anaemia cases
Autosomal recessive inheritance
BONE MARROW FAILURE

44
Q

Fanconis anaemia characteristics

A
  • somatic abnormalites
  • bone marrow failure
  • short telomeres
  • malignancy
  • chromosome instability
45
Q

Types of Fanconis anaemia

A

7 genetic FANCA to FANC-G

46
Q

Clinical features of Fanconis anaemia

A 
micropthalmia 
GU malformations 
GI malformations 
mental retardation 
Hearing loss 
CNS e.g hydrocephalus
47
Q

Treatment of Fanconis anaemia

A 
Gold standard- allogenc stem cell transplant 
Other options are: 
-supportive care
-corticosteroids 
-androgens

Life time surveillance for secondary tumours

Haematology (11 decks)

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