Anaemia Of Bone Marrow Failure Flashcards by Iseoluwa Ojo

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

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This bone marrow damage may be due

•to disruption of the _____________

•or disruption of the ____________

erythroid precursors pool

structure of the marrow.

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BONE MARROW FAILURE

It usually presents with ______ and ______

anaemia and reticulopenia

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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

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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

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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

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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

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Bone marrow failure can be caused by :

Bone marrow infiltration – e.g. ________, ________

Marrow infections- e.g. ______,_____

Lymphoma/leukaemia; metastatic carcinoma.

tuberculosis, sarcoidosis

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Features of anaemia:-

•____,_____,______,______,______

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

tiredness, weakness, pallor, breathlessness, tachycardia

bruising; petechiae; nose and/or gums

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Features of Neutropenia:

recurrent or severe _________

bacterial infections.

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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

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

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

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DIFFERENTIAL DIAGNOSIS

Other causes of pancytopenias, eg
–_________
–_________

Hypersplenism

Evans syndrome

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The management will depend on the _________ and the ___________

underlying cause

degree of bone marrow failure.

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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

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Aplastic Anaemia can only be congenital

T/F

can be acquired or congenital

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Causes of aplastic anaemia

Congenital
• __________

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Fanconi anaemia

Causes of aplastic anaemia

•ACQUIRED

Idiopathic ( ____ % of cases)

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

•Drugs:
– ______ agents
–________

•Ionizing radiation

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

•________________

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benzene, Toulene, glue sniffing, insecticides

alkylating; Idiosyncratic

Viruses ; Tuberculosis

Paroxysmal nocturnal haemoglobinuria

alkylating agents eg _______
– Idiosyncratic: ______,______

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busulphan

chloramphenicol, NSAIDS

viruses that can cause aplastic Anaemia

List 5

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Hepatitis, EBV, HIV , parvovirus , Epstein Barr

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

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hair loss (alopecia)

reduced blood cell counts (pancytopenia)

PATHOGENESIS of aplastic Anaemia

1)due to a _______________ together with ___________

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

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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

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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 _________

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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%

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 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

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

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

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

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

The genetic defect in PNH is ?? All PNH cells are sensitive to complement mediated lysis

Somatic mutation False

Anaemia Of Bone Marrow Failure Flashcards

(42 cards)