Myelodysplastic syndrome Flashcards

1
Q

What is myelodysplastic syndrome

A

Group of haematological disorders - DYSPLASTIC = dysfunctional blood cells. bone marrow functionas abnormally, insufficient growth of pluripotnet stem cells in bone marrow -> insufficient mature blood cells
Can affect severeal lines or one line (erythrocytic, granulocytic and megakaryocytic)

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2
Q

Risk factors for primary MDS

A

Male sex
Caucasia
>60 years

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3
Q

Seconadry MDS causes

A

Therapy related - 2-10 years after chemo
Radiotherapy related

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4
Q

What cancer treatments increase the risk of MDS

A

Breast cancer
Leukaemias
Lymphmoas
Heaad and neck
GI
lung
Prostate cancer

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5
Q

Types of MDS WHO

A

Single lineage dysplasia - MDS-SLD
Multi lineage - MDS-MLD
with ring sideroblasts = MDS-RS
with excess blasts - MDS-EB
with isolated 5q deletion
Unclassifiable - MDS-U

FAB and IPSS classifications are different

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6
Q

Clinical features of MDS why difficult to diagnose

A

20% asymptomatic
Non specific, recurrent infections
Depends on cell line effected

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7
Q

Clinical features MDS - which bloodline causes what

A

Anaemia - classic
Leukocytopenia - recurrent infections esp bacterial skin
THrombocytopenia - easy bruising eg petechoiae
Recurrent bleeding eg recurrent nosebleeds
Hepatmegaly may occur if extramedullary haematopoiesis occrs

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8
Q

Bloods in MDS

A

Low RBC, WCC and/or platelets
Macrocytic anaemia
Sev neutropenia

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9
Q

What see on peripheral blood film in MDS

A

Nucleated RBC
Ringed sideroblasts
Howell-Jolly bodies
Basophilic stippling
Hypoglobulated or unilobulated neutrophil nuclei
Large agranular platelets and megakaryocytes

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10
Q

Initial investigations MDS

A

Blood test
Blood film

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11
Q

Further investigations MDS

A

Bone marrow biopsy
Cytogenetic studies

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12
Q

What see in MDS on bone marrow biopsy

A

Hypercellular dysplastic bone marrow w numerous cells of all three lines
Can also have hypocellular bone marrow
Blasts, magakaryocytes ringed sideroblasts etc

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13
Q

What results are common in cytogenetics in MDS

A

5q deletion, monosomy 7 - -7 or 7q, trisomty 8 - +8

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14
Q

What cytogenetics have a better prognosis in MDS

A

Single genetic abnormalities
5q deletion - respons to one medicaiton

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15
Q

How differentiate Aplastic anameia w MDS

A

Aplastic anaemia - marrow hypoplasia without dysplasia
MDS - always dysplasia
Aplastic anaemia often AI or toxic exposure history

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16
Q

What is different in the smear of CML vs MDS

A

CML - increased monocytes
CML bone marrow - Proliferative bone marrow picture with both dysplasia and hypercellularity

17
Q

Supportive care MDS

A

Blood transfusions especially RBC
Iron chelation therapy in transfusion overload eg desferrioxamine, deferasirox
Erythroid timulating agents ESA
Antibiotics prophylaxis

18
Q

What is used to treat 5q del MDS

A

Lenalidomide

19
Q

What med can be used for intermediate and high risk MDS

A

Azacitidine - hypomethylating agent

20
Q

What can be used as immunosupression in younger patients with normal karyotype

A

ATG

21
Q

Only cure for DS

A

Allogeneic HSCT

22
Q

Complications of MDS

A

Bleeding - IC, pulmonary, GI
Infections - atypical, neurtopenic sepsis
Progression to AML - 30% of MDS

23
Q

What forms of MDS are more likely to progress to AML

A

MDS-EB, MDS-RS, MDS-RS-MLD, MDS-MLD

24
Q

What side effects can azacitidine cause

A

Fever, N+V, diarrhoea or constipation, fatigue and weakness

25
Q

What side effects can lenalidonmide cause

A

Leukocytopenia
Thrombocytopenia
Diarrhoea
Constiation
Fatigue, weakness

26
Q

Side effects of cytarabine

A

Hair loss
Mouth sores
loss of appetitie
N+V
Pancytopenia

27
Q

What is cytarabine used for

A

Patients progressed from MDS to AML

28
Q

What are the early side effects of bone marrow transplant

A

Bleeding, infection, pancytopenia

29
Q

Late side effects of bone marrow transplant

A

GvHD, recurrence 20-50%

30
Q

Risk factors for AML progression

A

Certain subtypes
Ringed sideroblasts are protective against progression
Presence of excess blasts = higher risk progression to AML

31
Q

Who gets MDS

A

Elderly

32
Q

What is refractory anaemia

A

Mildly low count in one cell line from Haeatopoiesis

33
Q

What is refractory cytopenia w multi lineage dysplasia

A

Low counts in multiple cell lines

34
Q

What is refractory anaemia w excess blasts

A

MDS includes production of leukaemia cells -> AML leukaemia

35
Q

What anaemia see in MDS

A

Macrocytic

36
Q

Why is bone marrow transplant rarae in MDS

A

Elderly pop

37
Q
A