Haem III Flashcards

1
Q

What causes MDS

A

myeloid bone marrow cells not maturing properly and therefore not producing healthy blood cells

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2
Q

Blood findings - MDS

A

causes low levels of blood components that originate from the myeloid cell line:

Anaemia
Neutropenia (low neutrophil count)
Thrombocytopenia (low platelets)

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3
Q

Presentation of MDS

A

anaemia (fatigue, pallor or shortness of breath), neutropenia (frequent or severe infections) or thrombocytopenia (purpura or bleeding).

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4
Q

Diagnosis of MDS

A

Full blood count will be abnormal. There may be blasts on the blood film.

The diagnosis is confirmed by bone marrow aspiration and biopsy.

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5
Q

Mx of MDS

A

Watchful waiting
Supportive treatment with blood transfusions if severely anaemic
Chemotherapy
Stem cell transplantation

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6
Q

What is pernicious anaemia

A

autoimmune condition where antibodies form against the parietal cells or intrinsic factor. A lack of intrinsic factor prevents the absorption of vitamin B12 and the patient becomes vitamin B12 deficient.

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7
Q

Presentation of vitamin B12 deficiency

A

Peripheral neuropathy with numbness or paraesthesia (pins and needles)
Loss of vibration sense or proprioception
Visual changes
Mood or cognitive changes

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8
Q

Diagnosis of pernicious anaemia

A

Intrinsic factor antibody is the first line investigation

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9
Q

Mx of b12 deficiency

A

oral replacement with cyanocobalamin

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10
Q

Mx of pernicious anaemia

A

1mg of intramuscular hydroxycobalamin 3 times weekly for 2 weeks, then every 3 months

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11
Q

Which should be treated first between b12 and folate deficiencies

A

it is important to treat the B12 deficiency first before correcting the folate deficiency.

Treating patients with folic acid when they have a B12 deficiency can lead to subacute combined degeneration of the cord.

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12
Q

Inherited causes of haemolytic anaemia

A
Hereditary Spherocytosis
Hereditary Elliptocytosis
Thalassaemia
Sickle Cell Anaemia
G6PD Deficiency
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13
Q

Acquired causes of haemolytic anaemia

A
Autoimmune haemolytic anaemia
Alloimmune haemolytic anaemia (transfusions reactions and haemolytic disease of newborn)
Paroxysmal nocturnal haemoglobinuria
Microangiopathic haemolytic anaemia
Prosthetic valve related haemolysis
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14
Q

Features of haemolytic anaemia

A

Anaemia due to the reduction in circulating red blood cells

Splenomegaly as the spleen becomes filled with destroyed red blood cells

Jaundice as bilirubin is released during the destruction of red blood cells

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15
Q

IX in haemolytic anaemia

A

FBC - normocytic
Blood film - schistocytes(fragments of RBCs)
Direct Coombs test - positive in autoimmune haemolytic anaemia

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16
Q

What is hereditary spherocytosis

A

Autosomal dominant condition. It causes sphere shaped red blood cells that are fragile and easily break down when passing through the spleen.

17
Q

Presentation of hereditary spherocytosis

A

jaundice, gallstones, splenomegaly and notably aplastic crisis in the presence of the parvovirus

18
Q

Treatment for hereditary spherocytosis

A

folate supplementation and splenectomy. Removal of the gallbladder (cholecystectomy) may be required if gallstones are a problem

19
Q

Inheritance of G6PD def

A

X linked recessive

20
Q

Triggers of G6PD def

A

infections, medications or fava beans

21
Q

Presentation of G6PD def

A

jaundice (usually in the neonatal period), gallstones, anaemia, splenomegaly and Heinz bodies on blood film

22
Q

Diagnosis of G6PD def

A

G6PD enzyme assay

23
Q

Medications which can trigger hemolysis

A

primaquine (an antimalarial), ciprofloxacin, sulfonylureas, sulfasalazine and other sulphonamide drugs.

24
Q

Features of warm type autoimmune haemolytic anaemia

A

Haemolysis occurs at normal or above normal temperatures.

It is usually idiopathic

25
Q

What is cold type AIHA often secondary to

A

lymphoma, leukaemia, systemic lupus erythematosus and

26
Q

Infections associated with AIHA

A

mycoplasma, EBV, CMV and HIV

27
Q

Mx of autoimmune haemolytic anaemia

A

Blood transfusions
Prednisolone (steroids)
Rituximab (a monoclonal antibody against B cells)
Splenectomy

28
Q

Presentation of PNH

A

red urine in the morning containing haemoglobin and haemosiderin

Predisposed to thrombosis(DVT, PE)

Smooth muscle dystonia(oesophageal spasm and erectile dysfunction)

29
Q

Mx of PNH

A

eculizumab or bone marrow transplantation

30
Q

Mx of prosthetic valve haemolytic

A

Monitoring
Oral iron
Blood transfusion if severe
Revision surgery may be required in severe cases