Bone marrow failure Flashcards
Symptoms of bone marrow failure
Anaemia - fatigue, tachycardia, SOB
Thrombocytopenia - bruising, petechiae, ecchymoses
Neutropenia - infection
Examples of bone marrow failure syndromes with failure of cell production
Agranulocytosis (single line)
Aplastic anaemia (all 3 lines)
Acquired
Causes of acquired bone marrow failure with failure of cell production
Infection - EBV, HIV, Hep C, parvovirus
Drug induced - NSAIDs, methotrexate, carbamazepine
Immune
Radiation
Vitamin deficiency - B12, folate
Infiltration - mets, myelofibrosis, Gaucher’s disease, myeloma
Pathophysiology of aplastic anaemia
Insult to bone marrow stem cell which triggers immune response
Damage to haemopoeitic stem cells
Resulting pancytopenia
Investigations for aplastic anaemia
History - symptoms, assess infection risk FBC Reticulocyte count B12, folate Infection screen LFTs U+Es TFTs
How to diagnose aplastic anaemia
2 bone marrow assessments (at a different time and from a different place)
Aspirate and trephine
Treatment for aplastic anaemia
Reduce infection risk Aggressively treat any infections Transfuse blood products if appropriate Bone marrow transplant if young ATG and ALG immunotherapy
What conditions can aplastic anaemia transform to if untreated
20% Anaemia paroxysmal nocturnal haemoglobinuria
15% AML
Prognosis of untreated aplastic anaemia
<6 months
What are myelodysplastic syndromes
Bone marrow failure syndromes with ineffective cell production
Demographic affected by myelodysplastic syndromes
Incidence increases with increasing age
Primary vs secondary myelodysplastic syndromes
Primary usually idiopathic
Secondary usually due to a bone marrow insult e.g chemotherapy or radiotherapy
WHO classification of myelodysplastic syndromes
Refractory cytopenia with unilineage dysplasia
Refractory cytopenia with multi lineage dysplasia
Refractory anaemia with ring sideroblasts
Refractory anaemia with excess blasts (RAEB)-1
RAEB-2
FAB classification of myelodysplastic syndromes
Refractory anaemia
Refractory anaemia with ring sideroblasts
Refractory anaemia with excess blasts
Refractory anaemia with excess blasts in transformation (to AML)
Diagnosis of myelodysplastic syndromes
FBC U+Es LFTs B12 Folate Infection risk Bone marrow aspirate and trephine