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House Hemat > Anaemia Of Bone Marrow Failure > Flashcards

Anaemia Of Bone Marrow Failure Flashcards

1
Q

Bone marrow failure is defined as ______________________ affecting one or more cell lines due to damage to the bone marrow.

A

reduction or cessation of blood cell production

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2
Q

This bone marrow damage may be due

•to disruption of the _____________

•or disruption of the ____________

A

erythroid precursors pool

structure of the marrow.

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3
Q

BONE MARROW FAILURE

It usually presents with ______ and ______

A

anaemia and reticulopenia

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4
Q

PATHOPHYSIOLOGY OF BONE
MARROW FAILURE

-Destruction of _________
-Disruption of ________
-Decreased production of __________
-Ineffective _______
-Loss of _________ tissue due to infiltration of marrow by _______

A

haemopoietic stem cells (HSC)

bone marrow microenvironment

Haemopoietic growth factors or hormones

haemopoiesis

normal haemopoietic; abnormal cells

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5
Q

AETIOPATHOGENESIS of BMF

Haemopoietic cell damage causing ____________. This may be:

•Congenital with inherited gene mutations
–________ anaemia, ______ anaemia, ____________ syndrome.

A

hypoplastic or aplastic anaemia

fanconi’s; Diamond-Blackfan ; Shwachman- Diamond

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6
Q

AETIOPATHOGENESIS of BMF

Haemopoietic cell damage causing hypoplastic or aplastic Anaemia. This may be:

Acquired –

_________
________
_________

________ agents, ______ (e.g. benzene),
______e.g. alkylating agents-Busulphan

_________

A

viruses
autoimmunity
ionizing radiation

antineoplastic; poisons

Drugs

Paroxysmal Nocturnal haemoglobinaemia

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7
Q

Bone marrow failure can be caused by :

•Marrow structural abnormality e.g. ____

Maturation defects – e.g.______, _______

Differentiation defects – e.g._______.

A

myelofibrosis

Vitamin B12 deficiency; Folate deficiency

Myelodysplasia

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8
Q

Bone marrow failure can be caused by :

Bone marrow infiltration – e.g. ________, ________

Marrow infections- e.g. ______,_____

A

Lymphoma/leukaemia; metastatic carcinoma.

tuberculosis, sarcoidosis

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9
Q

Features of anaemia:-

•____,_____,______,______,______

Features of Thrombocytopenia –
–
easy ______, _______ , bleeding from the ________

A

tiredness, weakness, pallor, breathlessness, tachycardia

bruising; petechiae; nose and/or gums

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10
Q

Features of Neutropenia:

recurrent or severe _________

A

bacterial infections.

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11
Q

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 ———

A

normo; normo; variable cytopenias

reticulopenia

hypo; normo; hyper ; effective erythropoiesis

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12
Q

Pancytopenia is a condition in which a person’s body has _____________

A

too few red blood cells, white blood cells, and platelets.

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13
Q

DIFFERENTIAL DIAGNOSIS

Other causes of pancytopenias, eg
–_________
–_________

A

Hypersplenism

Evans syndrome

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14
Q

The management will depend on the _________ and the ___________

A

underlying cause

degree of bone marrow failure.

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15
Q

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

A

pancytopenia

reticulopenia

haemopoietic tissue in the marrow

involvement of the marrow

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16
Q

Aplastic Anaemia can only be congenital

T/F

A

F

can be acquired or congenital

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17
Q

Causes of aplastic anaemia

Congenital
• __________

A

Fanconi anaemia

18
Q

Causes of aplastic anaemia

•ACQUIRED

  • Idiopathic ( ____ % of cases)

-secondary
•Chemicals: ____,_____,____ ,_____

•Drugs:
– ______ agents
–________

•Ionizing radiation

•Infections:
– _______
– Others: e.g _____

•________________

A

67

benzene, Toulene, glue sniffing, insecticides

alkylating; Idiosyncratic

Viruses ; Tuberculosis

Paroxysmal nocturnal haemoglobinuria

19
Q

alkylating agents eg _______
– Idiosyncratic: ______,______

A

busulphan

chloramphenicol, NSAIDS

20
Q

viruses that can cause aplastic Anaemia

List 5

A

Hepatitis, EBV, HIV , parvovirus , Epstein Barr

21
Q

The most common side effects of alkylating agents include: _______ (____) anemia and _________(____)

A

hair loss (alopecia)

reduced blood cell counts (pancytopenia)

22
Q

PATHOGENESIS of aplastic Anaemia

1)due to a _______________ together with ___________

Or

2)__________ against them so that they are unable to repopulate the bone marrow

A

reduction in the number of pluripotent stem cells

a fault in the ones remaining

an immune reaction

23
Q

Differentiate between NSAA, SAA, and VSAA under the following characteristics

Neutrophils
Platelets
Reticulocyte

A

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

24
Q

NSAA has haemoglobin concentration of _________

A

Less than 10g/dl

25
Q

Full meaning of :

NSAA
SAA
VSAA

A

Non- severe aplastic Anaemia
Severe aplastic Anaemia
Very severe aplastic Anaemia

26
Q

TREATMENT of aplastic Anaemia

Treatment of _______😂, if possible

Removal of _____ (____, ____)

A

underlying causes

Cause; drugs benzene

27
Q

TREATMENT of aplastic anemia

•Supportive:
_________ and _______
Cytokines (_____ and _____)
________ antibiotics: Infections
_______

A

Blood and platelet transfusion

Epo &G-CSF

Broadspectrum

Asepsis

28
Q

TREATMENT of aplastic anemia

_______________ for Severe disease, Patient age ____years, availability of ____
-__________donors

A

Bone marrow transplant

<40

HLA

identical sibling marrow

29
Q

TREATMENT of aplastic anemia

Immunosuppressive therapy: (if for those that ____________)

•___________ with ________

•Poor response/relapse: ____-matched (related or unrelated?) donor

A

Do not meet the criteria for bone marrow transplant

Antithymocyte globulin with cyclosporine A

HLA

Unrelated

30
Q

TREATMENT of aplastic Anaemia

______ therapy

A

Androgen

31
Q

Hyper splenism

If your spleen is overactive, it _____________

A

removes the blood cells too early and too quickly

32
Q

Evans syndrome is ____________ in which _______ attack ______,_______,________

A

an autoimmune disorder

antibodies

red blood cells (RBC), platelets and/or neutrophils

33
Q

You have Anaemia once your bone marrow cellularity is less than ____% of the 100%

A

25

34
Q

Sarcoidosis

The growth of ____________ in different parts of the body.

A

tiny collections of inflammatory cells

35
Q

Which of the following does not cause pancytopenia?
a. Iron deficiency
b. Folate deficiency
c. Aplastic anemia
d. AML
e. Cyclophosphamide

A

A

36
Q

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

A

B

37
Q

In Fanconi anemia, Physical development is always normal

A

True

38
Q

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

A

D

39
Q

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

A

B

40
Q

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

A

3

41
Q

PNH is associated with all of the following, except:
a. Aplastic anemia
b. Increased LAP score
c. Venous thrombosis
d. Iron deficiency

A

B

42
Q

The genetic defect in PNH is ??

All PNH cells are sensitive to complement mediated lysis

A

Somatic mutation

False

House Hemat (29 decks)

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