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BPT exams > Haematology > Flashcards

Haematology Flashcards

1
Q

Transient erythroblastic anaemia of childhood can be differentiated from Diamond Blackfan anaemia by:

A

Normal erythrocyte ADA activity (Increased in Diamond Blackfan)

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2
Q

Glanzmann thrombasthenia (GT) is a genetic platelet disorder in which the platelets have qualitative or quantitative deficiencies?

A

Qualitative disorder - deficiencies of the fibrinogen receptor αIIbβ3

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3
Q

__________ must be considered in young males found to have thrombocytopenia with small platelets, particularly if there is a history of eczema and recurrent infection

A

Wiskott-Aldrich syndrome

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4
Q

What is the inheritance pattern of Wiskott Aldrich syndrome?

A

X-Linked; affects WAS gene

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5
Q

What is the role of splenectomy in ITP? (indications)

A

1) Severe and chronic ITP >1yr
OR
2) Life threatening haemorrhage and ITP that does not respond to normal treatment

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6
Q

What percentage of patients with ITP progress to chronic ITP (>1yr)?

A

20%

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7
Q

3 most common bugs associated with ITP?

A

EBV, HIV and H Pylori

but also all the common viral bugs

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8
Q

What are some common drugs that cause ITP?

A

Valproic acid, phenytoin, carbamazepine, sulfonamides, vancomycin, and trimethoprim-sulfamethoxazole

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9
Q

What is the pentad of symptoms seen with TTP?

A

Pentad of fever, microangiopathic hemolytic anemia, thrombocytopenia, abnormal renal function, and central nervous system (CNS)

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10
Q

What is the treatment of TTP?

A

Plasmapheresis

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11
Q

What are the two common causes for TTP?

A

Acquired: Ab to ADAMTS13
Congenital: Lack of ADAST13

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12
Q

What is normally the role of ADAST13?

A

Responsible for cleaving the high-molecular-weight multimers of VWF; thus absence of ADAMST13 leads to clumping of platelets

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13
Q

What is Kasabach-Merritt syndrome?

A

Localized intravascular coagulation causing thrombocytopenia and hypofibrinogenemia associated with a giant haemangioma

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14
Q

What are the two major congenital causes for thrombocytopenia and how do you differentiate them?

A

Congenital amegakaryocytic thrombocytopenia (CAMT) and Thrombocytopenia-Absent radius syndorme

CAMT have normal exam otherwise and are associated with mutation in the stem cell TPO receptor (MPL)

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15
Q

What is due to a severe deficiency or absence of the vWF receptor (GP1b complex) on the platelet membrane?

A

Bernard Soullier syndrome

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16
Q

What is primary haemostasis and what is the pathway?

A

Formation of platelet plug:

Vascular injury -> vasoconstriction -> subendothelial matrix exposed -> vWF changes configuration -> activation and aggregation of platelets => platelet plug

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17
Q

What receptor allows platelets to bind to VonWillebrand factor?

A

GP1B

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18
Q

After vascular injury occurs; what chemical is secreted by the vessels to cause vasoconstriction?

A

Endothelin

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19
Q

Once platelets are activated they release three primary chemicals - what are they and what are their roles?

A

They release:

1) Ca - important for secondary haemostasis
2) ADP
3) Thromboxane A2 - important to aggregate other platelets

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20
Q

What receptor is required for fibrinogen to bind to platelets?

A

GIIB/GIIA

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21
Q

How is Factor X-> Xa activation occur in the extrinsic pathway?

A

Via 7a + TF + Ca

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22
Q

What factors are part of the common pathway?

A

Factor Xa, Factor II (prothrombin), Fibrinogen (Factor I), Factor XIII

1, 2, 10, 13

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23
Q

Coag cascade:

In the common pathway Factor ____ binds with _____ + factor ____= prothrombin activator (needed to make prothrombin into thrombin)

A

Factor Xa binds with TF + factor V = prothrombin activator (needed to make prothrombin into thrombin)

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24
Q

What does antithrombin do?

A

It helps dissolve the clot and prevents more clotting by binding to thrombin and factor X

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25
Q

How does Heparin work?

A

Heparin binds to antithrombin and increases its affinity and make it more potent.

thus you measure Factor Xa levels to see how it is working. High factor Xa levels means heparin has bound to it and it can’t do it’s job. High Xa means more thin blood

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26
Q

When is steroids or IVIG recommended in ITP?

A

Moderate ITP:

Epistaxis >5 mins
Haematuria
Haematochezia
Painful oral purpura
Significant menorrhagia
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27
Q

How is CF related to coagulopathy?

A

CF leads to reduction in Fat soluble vitamins which are DEKA:

Thus Vitamin K is affected and it affects factors 2, 7 , 9, 10; which is involved in the Extrinsic pathway (affecting PT)

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28
Q

What are the two primary roles for VonWillebrand factor?

A

1) vWF adheres to the subendothelial matrix after vascular damage, where its conformation is changed so platelets adhere to it
2) vWF also serves as the carrier protein for factor VIII in plasma

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29
Q

There are three forms of VonWillebrand disease? What are they and how do you differentiate them with Ristocetin testing?

A

Type 1 - Reduced amount of factor
Type 2 - Qualitative deficiency in factor
Type 3 - Absence or minimal factor

Ristocetin activity:
>0.7-1: Factor present, but limited type I
<0.7: Bad factor (Type II)
0 : Absent factor. (type III)

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30
Q

When is DDAVP helpful in VonWillebrand disease?

A

It is helpful for type I; not necessarily for Type II and III

31
Q

How does DDAVP work?

A

It promotes release of vWF from endothelial cells

32
Q

A young child has an acquired cause of intravscular haemolysis with features of pancytopenia presenting with symptoms commonly on anaemia or thrombi. What is the diagnosis?

With the use of anti-CD59 for RBCs and anti-CD55 and anti-CD59 for granulocytes, flow cytometry is more sensitive than the classic RBC lysis (Ham or sucrose) tests in detecting these reduced glycolipid-bound membrane proteins

A

he has paroxysmal nocturnal haemoglobinuria

This is a defect in the CD59 membrane of the RBC which makes it more susceptible to haemolysis from complement cascade

33
Q

Treatment for paroxysmal nocturnal haemoglobinuria?

A

eculizumab therapy - monoclonal antibody against complement component C5

34
Q

Which clinical feature do you nearly always get in hereditary spherocytosis?

Can help you differentiate a clinical case from G6PD?

A

Splenomegaly!

Although HS leads to intravascular haemolysis, this happens in the spleen and thus big spleen

35
Q

What are the most common abnormalities or defects seen in HS?

A

Abnormalities of ankyrin or spectrin are the most common molecular defects

36
Q

What is the inheritance pattern of HS?

A

75% Autosomal dominant

25% due to a De Novo mutation with no fam history

37
Q

What is a common complication in HS?

A

Gallstones

Superimposed anaemia with Parovirus B19 infection

38
Q

What is the normal role of G6PD

A

G6PD catalyzes the conversion of glucose 6-phosphate to 6-phosphogluconic acid. This reaction produces NADPH, which maintains GSH (glutathione in its reduced, functional state).

GSH provides protection against oxidant threats from certain drugs and infections that would otherwise cause precipitation of hemoglobin (Heinz bodies) or damage the RBC membrane.

39
Q

is Wiskott Aldrich associated with big or small platelets?

A

Small!

40
Q

Newborn with petechiae. A blood film shows large platelets which appear pale (Grey) and hypogranulated (key!)

A

Gray platelet syndrome

41
Q

What is the mechanism of action of Hydroxyurea and what are the side effects?

A

Inhibition of ribonucleotide reductase

Also causes increased production of HbF

Can be myelosuppressive and cause pancytopenia

42
Q

How is Fanconi anaemia diagnosed?

A

Through chromosome breakage studies

43
Q

AML and MDS lie along a disease continuum. The distinction between the two relies on …

A

AML and MDS lie along a disease continuum. The distinction between the two relies on blast percentage, with >20% blast forms diagnostic of AML.

44
Q

What is the pathophysiology for anaemia of chronic disease?

A

Anaemia of chronic disease is caused by increased hepcidin that causes iron to be retained within cells of the reticuloendothelial system, reducing availability of iron for erythroid progenitor cells in the bone marrow. This leads to low circulating serum iron, reduced transferrin/transferrin saturation and normal-high ferritin levels.

45
Q

What is the triad of symptoms associated with Dyskeratosis congenita?

A

Dysplastic nails, lacy reticular pigmentation of the upper chest and/or neck, and oral leukoplakia

46
Q

Hoyeraal-Hreidarsson syndrome is a multisystem disorder that presents in early childhood, which requires the features of DC along with ________ to establish the diagnosis

A

Cerebellar hypoplasia

Can also have immune deficiency

47
Q

___________ is caused by compound heterozygous mutations in the CTC1 gene and has overlapping features with DC, including sparse and graying hair, dystrophic nails, and anemia. Telomeres are very short.

Also characterized by retinal telangiectasia and exudates

A

Coats plus syndrome

48
Q

How do you treat Dyskeratosis congeinta?

A

Androgens

HSCT is curative though

49
Q

Name atleast 5 common side effects of Cyclosporin?

A 
Gingival hyperplasia
Hirsutism
nephrotoxicity
HT
hypercholesterolaemia
neurotoxicity (tremor, headache, paraesthesiae)
deranged LFTs
hypomagnesaemia
hyperkalaemia
diarrhoea
50
Q

Definition of first order kinetics: XX

Zero order absorption = XX

A

Definition of first order kinetics: a constant percentage (or fraction) of cells is killed

Zero order absorption = when rate of absorption is independent of amount of drug remaining in gut

51
Q

Southern blot identifies XX

Northern blot identifies XX

Western blot identifies XX

A

Southern blot identifies DNA

Northern blot identifies RNA

Western blot identifies protein

52
Q

Revesz syndrome is basically Dyskeratosis congenita with ________

A

Retinal pathology

53
Q

Factor XIII deficiency presents with early or delayed bleeding?

A

Delayed (presents 12-24 hours later)

54
Q

What is the target of Transexamic acid?

A

Binds to plasminogen

This decreases the conversion of plasminogen to plasmin, preventing fibrin degradation and preserving the framework of fibrin’s matrix structure.

55
Q

Does haemoglobinopathy give you a high or a low RDC?

A

High RCC

56
Q

Goat’s milk contains no __________

A

Folic acid

57
Q

What are the coag abnormalities assoc with Factor XIII deficiency?

A

Commonly normal coags!

58
Q

Differentiating between Heparin and Lupus Anticoagulant?

A

Heparin would have elevated thrombin time.

PT time may remain normal, but elevated APTT.

59
Q

cafe au lait spot, one kidney, normocytic anaemia, mild thrombocytopenia, and mild neutropenia

A

Fanconi anaemia

60
Q

TIBC elevated or low in:

  • Thalassaemia
  • Iron Def
A
  • Thalassemia; low TIBC (or normal)

- Iron def: high TIBC (or normal)

61
Q

Lifespan of platelets?

A

10 days

62
Q

What factors does Cryoprecipitate have?

A

factor XIII, von Willebrand factor (vWF), and factor VIII

63
Q

What substance can most rapidly correct a high INR secondary to warfarin?

A

Prothrombin concentrate complex

64
Q

What is HbH made of ?

A

4 beta chains - seen in Alpha thal major (3 genes)

65
Q

Warm immune haemolysis and cold immune haemolysis:

  • IgG positive?
  • IgM positive?
A
  • Warm - IgG positive

- Cold - IgM positve

66
Q

In PNH, the mutation causes cell membranes to be deficient (either partially or completely) in proteins that impede cell destruction through which pathway?

A

Complement-mediated lysis via the constitutively active alternative pathway

This include decay-accelerating factor (DFA, CD55), the membrane inhibitor of reactive lysis (CD59), and the C8-binding protein

67
Q

Apart from haemolysis, thrombocytopenia and leukopenia, PNH can present with:

A

1) Thrombosis with abdominal pain - Budd-Chiari syndrome (hepatic veins), or splenomegaly (splenic vein)
2) Hemoglobinuria - uncommon in children

68
Q

What is the diagnostic test of choice for PNH and why?

A

Flow cytometry

With the use of anti-CD59 for RBCs and anti-CD55 and anti-CD59 for granulocytes, flow cytometry is more sensitive than the classic RBC lysis (Ham or sucrose) tests in detecting these reduced glycolipid-bound membrane proteins

69
Q

Eculizumab is a monoclonal antibody against _____

A

C5

Used to treat PNH

70
Q

How long does it take to reach steady levels of a drug/medication?

A

3-5 half lives

71
Q

What does salicylate poisoning initially show on a blood gas?

A

Respiratory alkalosis initially

1) Phase I is characterized by hyperventilation resulting from direct respiratory center stimulation, leading to respiratory alkalosis and compensatory alkaluria. Potassium and sodium bicarbonate are excreted in the urine.
2) Phase II is characterized by paradoxic aciduria in the presence of continued respiratory alkalosis occurs when sufficient potassium has been lost from the kidneys
3) Phase III is characterized by dehydration, hypokalemia, and progressive metabolic acidosis. This phase may begin 4–6 hours after ingestion in a young infant

72
Q

Dinutuximab is an anti-___

A

anti-GD2+

73
Q

Hallmark cytopenia of Evan Syndrome is ______ and ________.

Evan syndrome is also known as ___________ and thus Flow cytometry also shows ________

A

Autoimmune thrombocytopenia and anaemia

Evan sydnrome is also known as autoimune lymphoproliferative disorder and thus with flow cytometry you get double negative T cells.

Germline mutation in the FAS gene leading to abnormal lymphocyte haemostasis and apoptosis

74
Q

Burr cells (echinocytes) are seen in presence of exposure to ___________

A

High levels of urea

BPT exams (24 decks)

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