Anaemia Of Bone Marrow Failure Flashcards
Bone marrow failure is defined as ______________________ affecting one or more cell lines due to damage to the bone marrow.
reduction or cessation of blood cell production
This bone marrow damage may be due
•to disruption of the _____________
•or disruption of the ____________
erythroid precursors pool
structure of the marrow.
BONE MARROW FAILURE
It usually presents with ______ and ______
anaemia and reticulopenia
PATHOPHYSIOLOGY OF BONE
MARROW FAILURE
-Destruction of _________
-Disruption of ________
-Decreased production of __________
-Ineffective _______
-Loss of _________ tissue due to infiltration of marrow by _______
haemopoietic stem cells (HSC)
bone marrow microenvironment
Haemopoietic growth factors or hormones
haemopoiesis
normal haemopoietic; abnormal cells
AETIOPATHOGENESIS of BMF
Haemopoietic cell damage causing ____________. This may be:
•Congenital with inherited gene mutations
–________ anaemia, ______ anaemia, ____________ syndrome.
hypoplastic or aplastic anaemia
fanconi’s; Diamond-Blackfan ; Shwachman- Diamond
AETIOPATHOGENESIS of BMF
Haemopoietic cell damage causing hypoplastic or aplastic Anaemia. This may be:
Acquired –
_________
________
_________
________ agents, ______ (e.g. benzene),
______e.g. alkylating agents-Busulphan
_________
viruses
autoimmunity
ionizing radiation
antineoplastic; poisons
Drugs
Paroxysmal Nocturnal haemoglobinaemia
Bone marrow failure can be caused by :
•Marrow structural abnormality e.g. ____
Maturation defects – e.g.______, _______
Differentiation defects – e.g._______.
myelofibrosis
Vitamin B12 deficiency; Folate deficiency
Myelodysplasia
Bone marrow failure can be caused by :
Bone marrow infiltration – e.g. ________, ________
Marrow infections- e.g. ______,_____
Lymphoma/leukaemia; metastatic carcinoma.
tuberculosis, sarcoidosis
Features of anaemia:-
•____,_____,______,______,______
Features of Thrombocytopenia –
–
easy ______, _______ , bleeding from the ________
tiredness, weakness, pallor, breathlessness, tachycardia
bruising; petechiae; nose and/or gums
Features of Neutropenia:
recurrent or severe _________
bacterial infections.
LABORATORY INVESTIGATIONS
•FBC/peripheral blood film:
– Usually a ___cytic ___chromic anaemia with ____________
•Reticulocyte count: ___________
•Bone marrow aspirate and biopsy:
– Usually ___plastic but can be _____plastic or ____plastic as in ———
normo; normo; variable cytopenias
reticulopenia
hypo; normo; hyper ; effective erythropoiesis
Pancytopenia is a condition in which a person’s body has _____________
too few red blood cells, white blood cells, and platelets.
DIFFERENTIAL DIAGNOSIS
Other causes of pancytopenias, eg
–_________
–_________
Hypersplenism
Evans syndrome
The management will depend on the _________ and the ___________
underlying cause
degree of bone marrow failure.
APLASTIC ANAEMIA
This is a rare disorder characterised by
–_______
–________
– a marked decrease in the amount of ____________ (hypocellularity/aplasia)
– absence of ________ by disease eg leukaemia, cancers, myelodysplasia
pancytopenia
reticulopenia
haemopoietic tissue in the marrow
involvement of the marrow
Aplastic Anaemia can only be congenital
T/F
F
can be acquired or congenital
Causes of aplastic anaemia
Congenital
• __________
Fanconi anaemia
Causes of aplastic anaemia
•ACQUIRED
- Idiopathic ( ____ % of cases)
-secondary
•Chemicals: ____,_____,____ ,_____
•Drugs:
– ______ agents
–________
•Ionizing radiation
•Infections:
– _______
– Others: e.g _____
•________________
67
benzene, Toulene, glue sniffing, insecticides
alkylating; Idiosyncratic
Viruses ; Tuberculosis
Paroxysmal nocturnal haemoglobinuria
alkylating agents eg _______
– Idiosyncratic: ______,______
busulphan
chloramphenicol, NSAIDS
viruses that can cause aplastic Anaemia
List 5
Hepatitis, EBV, HIV , parvovirus , Epstein Barr
The most common side effects of alkylating agents include: _______ (____) anemia and _________(____)
hair loss (alopecia)
reduced blood cell counts (pancytopenia)
PATHOGENESIS of aplastic Anaemia
1)due to a _______________ together with ___________
Or
2)__________ against them so that they are unable to repopulate the bone marrow
reduction in the number of pluripotent stem cells
a fault in the ones remaining
an immune reaction
Differentiate between NSAA, SAA, and VSAA under the following characteristics
Neutrophils
Platelets
Reticulocyte
NSAA: 0.5-1.5 ; 20-50; less than 30
SAA: 0.2-0.5; less than 20; less than 20 or less than 15
VSAA: less than 0.2; same as SAA; same as SAA
NSAA has haemoglobin concentration of _________
Less than 10g/dl
Full meaning of :
NSAA
SAA
VSAA
Non- severe aplastic Anaemia
Severe aplastic Anaemia
Very severe aplastic Anaemia
TREATMENT of aplastic Anaemia
Treatment of _______😂, if possible
Removal of _____ (____, ____)
underlying causes
Cause; drugs benzene
TREATMENT of aplastic anemia
•Supportive:
_________ and _______
Cytokines (_____ and _____)
________ antibiotics: Infections
_______
Blood and platelet transfusion
Epo &G-CSF
Broadspectrum
Asepsis
TREATMENT of aplastic anemia
_______________ for Severe disease, Patient age ____years, availability of ____
-__________donors
Bone marrow transplant
<40
HLA
identical sibling marrow
TREATMENT of aplastic anemia
Immunosuppressive therapy: (if for those that ____________)
•___________ with ________
•Poor response/relapse: ____-matched (related or unrelated?) donor
Do not meet the criteria for bone marrow transplant
Antithymocyte globulin with cyclosporine A
HLA
Unrelated
TREATMENT of aplastic Anaemia
______ therapy
Androgen
Hyper splenism
If your spleen is overactive, it _____________
removes the blood cells too early and too quickly
Evans syndrome is ____________ in which _______ attack ______,_______,________
an autoimmune disorder
antibodies
red blood cells (RBC), platelets and/or neutrophils
You have Anaemia once your bone marrow cellularity is less than ____% of the 100%
25
Sarcoidosis
The growth of ____________ in different parts of the body.
tiny collections of inflammatory cells
Which of the following does not cause pancytopenia?
a. Iron deficiency
b. Folate deficiency
c. Aplastic anemia
d. AML
e. Cyclophosphamide
A
A male child presented with neutropenia, exocrine pancreatic insufficiency and short stature. Serum biochemistry showed deranged LFT. The disease manifests as autosomal recessive trait and has a propensity to transform to MDS or AML. Which of the following best suites the diagnosis?
a. Fanconi anemia
b. Shwachman–Diamond syndrome
c. Blackfan–Diamond syndrome
d. Cysticfibrosis
B
In Fanconi anemia, Physical development is always normal
True
Bone marrow biopsy is superior to aspirate in diagnosing all of the following, except:
a. Aplastic anemia
b. Granuloma involving bone marrow
c. Myelofibrosis
d. Iron stores
D
Which of the following is not the etiological agent for aplastic anemia?
a. Hepatitis B virus
b. Hepatitis C virus
c. Non A non B virus
d. Hepatitis A virus
B
Which is not a feature of PNH?
1. Occurs due to PIG – A gene mutation
2. Cells are deficient in GPI linked proteins
3. Never associated with MDS
4. Patients can present with thrombosis
3
PNH is associated with all of the following, except:
a. Aplastic anemia
b. Increased LAP score
c. Venous thrombosis
d. Iron deficiency
B
The genetic defect in PNH is ??
All PNH cells are sensitive to complement mediated lysis
Somatic mutation
False
