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Flashcards in Haem1 Deck (417)
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331

In pts with sickle cell anaemia, what viral infection can precipitate aplastic anaemia?

Parvovirus

332

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

333

T/F? PNH makes pts more exposed to VTE

True

334

What's the gold standard investigation for PNH?

CD59 & CD55 on flow cytometry

335

Name 3 features of PNH

- Haemolytic anaemia

336

Is hereditary spherocytosis AD or AR inheritance?

AD

337

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

338

Osmotic fragility test diagnoses what?

Hereditary spherocytosis

339

Hereditary spherocytosis can be diagnosed by what?

Osmotic fragility test

340

What's the inheritance pattern of G6PD deficiency?

Male (X-linked recessive)

341

What is seen on blood films in G6PD deficiency?

Heinz bodies

342

What is seen on blood films in hereditary spherocytosis?

Spherocytes (round, lack of central pallor)

343

What's the diagnostic test for G6PD deficiency?

Measure enzyme activity of G6PD

344

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

345

Name 2 causes of polycythemia

Dehydration

346

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

347

Burkitt's lymphoma is a/w what gene translocation?

c-myc, usually t: 8:14

348

The c-myc gene translocation (t 8:14) is a/w what haematologic malignancy?

Burkitt's lymphoma

349

Unfractionated heparin forms a complex to inhibit what factors? How about LMWH?

UFH - activates antithrombin III. Inhibits thrombin, factors Xa, IXa, XIa, XIIa

350

How is unfractionated heparin monitored (what blood test)? How about LMWH?

UFH - APTT

351

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).

352

What alternative treatment could you use in someone who develops HITs?

Lepirudin

353

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.

354

How many pts (%) with CML have Philadelphia chromosome? (t 9,22)

95%

355

What's the first line management of chronic CML?

Imatinib is FIRST LINE TREATMENT

356

What are the findings on immunophenotyping in CLL?

B-cells (CD 19 positive)

357

A patient has excessive bleeding post a dental procedure. Found to also have a petechial rash, slightly elevated APTT and reduced FVIII activity. ? Dx

vWD

358

What's the most common inherited bleeding disorder?

vWD

359

Is vWD inherited AD, AR or X-linked?

AD

360

What are the 3 types of vWD?

1) Partial reduction in vWF (80% of pts, AD)