In pts with sickle cell anaemia, what viral infection can precipitate aplastic anaemia?
Is paroxysmal nocturnal haemoglobinuria inherited or acquired?
Acquired disorder leading to haemolysis of haematologic cells, thought to be due to increased sensitivity of cell membranes to complement due to lack of glycoprotein glycosyl-phosphatidylinositol
T/F? PNH makes pts more exposed to VTE
What's the gold standard investigation for PNH?
CD59 & CD55 on flow cytometry
Name 3 features of PNH
- Haemolytic anaemia
Is hereditary spherocytosis AD or AR inheritance?
What's the underlying cause of hereditary spherocytosis? ie) what bit is abnormal, and how?
RC cytoskeleton is deformed; no longer biconcave disc shape but rather sphere-shaped. This reduces RC survival --> destroyed by spleen
Osmotic fragility test diagnoses what?
Hereditary spherocytosis can be diagnosed by what?
Osmotic fragility test
What's the inheritance pattern of G6PD deficiency?
Male (X-linked recessive)
What is seen on blood films in G6PD deficiency?
What is seen on blood films in hereditary spherocytosis?
Spherocytes (round, lack of central pallor)
What's the diagnostic test for G6PD deficiency?
Measure enzyme activity of G6PD
T/F? Haemochromatosis is a/w polycythemia
False. Not a/w polycythemia. Usually blood tests show raised ferritin & iron, a/w transferrin saturation > 60% and a low total iron binding capacity
Name 2 causes of polycythemia
Having the Philadelphia chromosome (t 9:22) is a good prognostic marker in one disease, and a bad prognostic marker in another. Can you name them?
Philadelphia chromosome GOOD in CML, but BAD in AML & ALL
Burkitt's lymphoma is a/w what gene translocation?
c-myc, usually t: 8:14
The c-myc gene translocation (t 8:14) is a/w what haematologic malignancy?
Unfractionated heparin forms a complex to inhibit what factors? How about LMWH?
UFH - activates antithrombin III. Inhibits thrombin, factors Xa, IXa, XIa, XIIa
How is unfractionated heparin monitored (what blood test)? How about LMWH?
UFH - APTT
HITs is caused by what?
Immune mediated-antibodies, which activate platelets. This results in low platelet count (>50% reduction), (however HITs paradoxically is a prothrombotic condition).
What alternative treatment could you use in someone who develops HITs?
What is imatinib? When is it used?
Imatinib = tyrosine kinase inhibitor. Used in CML. Specifically inhibits the tyrosine kinase associated with the BCR-ABL defect.
How many pts (%) with CML have Philadelphia chromosome? (t 9,22)
What's the first line management of chronic CML?
Imatinib is FIRST LINE TREATMENT
What are the findings on immunophenotyping in CLL?
B-cells (CD 19 positive)
A patient has excessive bleeding post a dental procedure. Found to also have a petechial rash, slightly elevated APTT and reduced FVIII activity. ? Dx
What's the most common inherited bleeding disorder?
Is vWD inherited AD, AR or X-linked?
What are the 3 types of vWD?
1) Partial reduction in vWF (80% of pts, AD)