Anaemia Of Bone Marrow Failure

Question 1

What is bone marrow failure❓

What then is anaemia of bone marrow failure❓

Answer 1

🚫blood cell production affecting cell lines due to damage to the bone marrow

Anaemia secondary to bone marrow failure

Question 2

What are the causes of bone marrow failure❓

Answer 2

Destruction of HSC due to injury (drugs, chemicals, radiation, viruses etc)

Premature senescence and apoptosis of HSC due to mutations

Ineffective haemopoesis due to stem cell mutation, B12/folate deficiency

Destruction of HIM that supports haemopoiesis

⬇️production of haemopoietic growth factors or hormones

Loss of haemopoietic tissue due to infiltration of marrow by abnormal cells

Question 3

Anaemia of bone marrow failure will usually present with❓

Answer 3

Anaemia

Reticulopenia

Question 4

The aetiopathogenesis of bone marrow failure may include❓

Answer 4

1. HC damage causing hypoplastic or aplastic anemia:

Congenital:
Fanconi’s anaemia
Diamond-Blackfan anemia
Shwachman-Diamond Syndrome

Acquired: 
Virus (HBV, EBV, parvovirus B19, HIV)
Autoimmune dx 
Radiation 
Antineoplastic agents/poisons (benzene)
Drugs (alkylating agents- Busulphan)
Paroxysmal Noctural Haemoglobinuria 
Pregnancy 

2. BM structural abnormality:
   Myelofibrosis
3. Maturation defects:
   B12 and B9 deficiency
4. Differentiation defects:
   Myelodysplasia
5. BM infiltration:
   Lymphoma/leukemia
   Bony metastasis
6. BM infections:
   TB/ sarcoidosis

Question 5

What classes of drugs are associated with BM damage❓

List a few drugs you know under each class

Answer 5

Antibiotics: Chloramphenicol
Penicillin
Cephalosporins
Sulfonamide

Antidepressants:
Lithium
Tricyclics

Anti-epileptics:
Dilantin
Carbamazepine
Felbamate

Anti-inflammatory:
Phenylbutazone
Gold salts

Anti-arrhythmias:
Lidocaine
Quinidine
Procainamide

Diuretics:
Acetazolamide

Antimalarias:
Quinacrine

Question 6

What are the clinical features you’re likely to observe in a patient with anemia of bone marrow failure❓

Answer 6

Anemia:
Tiredness 
Weakness 
Pallor 
Tachycardia 

Thrombocytopenia:
Easy bruising
Petechiae
Bleeding from nose/gums

Neutropenia:
⬇️immunity, recurrent or severe infections

Question 7

What tests would you use to confirm anemia of bone marrow failure❓

Answer 7

Peripheral blood film: Normocytic normochromic anemia
Cytopenias

Reticulocyte count:
Reticulopenia

BM aspirate and biopsy:
Usually hypoplastic
Can be normoplastic/hyperplastic in effective erythropoiesis

Question 8

Polychromasia will be observed in moderate bone marrow failure but not severe BM failure

True or false

Answer 8

True

Question 9

If you suspect a case of anemia due to bone marrow failure b/c of pancytopenia, what could be a differential❓

Answer 9

Hypersplenism

Evans Syndrome

Question 10

What is aplastic anemia❓

Answer 10

Disorder in which BM decreases or 🚫 production of blood cells

Question 11

What what test results would you confirm aplastic anemia❓

Answer 11

Blood film:
Normocytic/macrocyclic RBC
⬇️platelets <100*10^9/L
Neutropenia/Monocytopenia

Pancytopenia  
Hypocellularity (aplasia), ⬆️fat spaces 
Reticulopenia 
⬇️Haemopoietic tissue 
🚫cancerous or abnormal cells in BM

Question 12

Aplastic anemia could be primary or secondary.

Shed some light on this

Answer 12

Primary:
Congenital eg Fanconi’s anemia
Idiopathic acquired (67%)

Secondary:
Chemicals ie benzene, bulsulphan 
Drugs eg chloramphenicol, NSAIDs
Insecticides 
Radiation 
Infections: EBV, HBV, HIV, erythrovirus, TB 
Paroxysmal nocturnal haemoglobinuria

Question 13

What is the pathogenesis of aplastic anemia❓

Answer 13

⬇️no of pluripotent stem cells + fault in those remaining

OR

Immune rxn against them

Question 14

What would a patient with aplastic anemia present in the hospital with❓

Answer 14

Anemia:
Weakness
Shortness of breath

Thrombocytopenia:
Epistaxix 
Menorrhagia 
Bleeding from gum 
Petechiae 
Echymosis 

Neutropenia:
⬇️immunity
Fever
Recurrent infections

Question 15

In non-severe aplastic anemia, what is the..

1. Bone cellularity
2. Neutrophil count
3. Platelets count
4. Hb, reticulocyte count❓

Answer 15

NSAA
Bone cellularity: <25%

Neutrophil count: 0.5-1.5 * 10^9/L

Platelet count: 20-50 * 10^9/L

Hb: <10g/dL

Reticulocytes: <30 * 10^9/L

Question 16

In severe aplastic anemia, what is the..

1. Bone cellularity
2. Neutrophil count
3. Platelets count
4. Reticulocyte count❓

Answer 16

SAA
Bone cellularity: <25%

Neutrophil count: 0.2-0.5 * 10^9/L

Platelet count: <20 * 10^9/L

Reticulocytes: <20 * 10^9/L

Question 17

In very severe aplastic anemia, what is the..

1. Bone cellularity
2. Neutrophil count
3. Platelets count
4. Reticulocyte count❓

Answer 17

VSAA
Bone cellularity: <25%

Neutrophil count: <0.2 * 10^9/L

Platelet count: <20 * 10^9/L

Reticulocytes: <20 * 10^9/L

Question 18

How would you treat a patient with aplastic anemia❓

Answer 18

Treat underlying cause:
Broadspectrum antibiotics

Removal of cause (drugs, benzene)

Supportive:
Blood/platelet transfusion
Cytokines

Curative:
Bone marrow transplant, <40yrs if severe

Immunosuppressive therapy:
Cyclosporine

Androgen therapy