Myelodysplastic syndrome Flashcards
(37 cards)
What is myelodysplastic syndrome
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)
Risk factors for primary MDS
Male sex
Caucasia
>60 years
Seconadry MDS causes
Therapy related - 2-10 years after chemo
Radiotherapy related
What cancer treatments increase the risk of MDS
Breast cancer
Leukaemias
Lymphmoas
Heaad and neck
GI
lung
Prostate cancer
Types of MDS WHO
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
Clinical features of MDS why difficult to diagnose
20% asymptomatic
Non specific, recurrent infections
Depends on cell line effected
Clinical features MDS - which bloodline causes what
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
Bloods in MDS
Low RBC, WCC and/or platelets
Macrocytic anaemia
Sev neutropenia
What see on peripheral blood film in MDS
Nucleated RBC
Ringed sideroblasts
Howell-Jolly bodies
Basophilic stippling
Hypoglobulated or unilobulated neutrophil nuclei
Large agranular platelets and megakaryocytes
Initial investigations MDS
Blood test
Blood film
Further investigations MDS
Bone marrow biopsy
Cytogenetic studies
What see in MDS on bone marrow biopsy
Hypercellular dysplastic bone marrow w numerous cells of all three lines
Can also have hypocellular bone marrow
Blasts, magakaryocytes ringed sideroblasts etc
What results are common in cytogenetics in MDS
5q deletion, monosomy 7 - -7 or 7q, trisomty 8 - +8
What cytogenetics have a better prognosis in MDS
Single genetic abnormalities
5q deletion - respons to one medicaiton
How differentiate Aplastic anameia w MDS
Aplastic anaemia - marrow hypoplasia without dysplasia
MDS - always dysplasia
Aplastic anaemia often AI or toxic exposure history
What is different in the smear of CML vs MDS
CML - increased monocytes
CML bone marrow - Proliferative bone marrow picture with both dysplasia and hypercellularity
Supportive care MDS
Blood transfusions especially RBC
Iron chelation therapy in transfusion overload eg desferrioxamine, deferasirox
Erythroid timulating agents ESA
Antibiotics prophylaxis
What is used to treat 5q del MDS
Lenalidomide
What med can be used for intermediate and high risk MDS
Azacitidine - hypomethylating agent
What can be used as immunosupression in younger patients with normal karyotype
ATG
Only cure for DS
Allogeneic HSCT
Complications of MDS
Bleeding - IC, pulmonary, GI
Infections - atypical, neurtopenic sepsis
Progression to AML - 30% of MDS
What forms of MDS are more likely to progress to AML
MDS-EB, MDS-RS, MDS-RS-MLD, MDS-MLD
What side effects can azacitidine cause
Fever, N+V, diarrhoea or constipation, fatigue and weakness
