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0. Year 5 > Haematology > Flashcards

Haematology Flashcards

1
Q

Follicular lymphoma

  • presentation?
  • what would you find on CT?
  • Genetic analysis?
A
  • fever, night sweats
  • weight loss
  • CT - mediastinal lymphadenopathy
  • Biopsy genetic analysis - translocation between chromosomes 14 and 18

indolent; chromosomal translocation of chr 14 and 18 - overexpression of BCL2; painless adenopathy,

systemic symptoms - fevers, night sweats, weight loss; anaemia, thrombocytopena, lymphocytosis

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2
Q

Burkitt’s lymphoma

  • what gene is over expressed?
  • what chromosomes are involved?
A

c-myc

-translocation between chromosome 8 and 14

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3
Q

AML

  • what age does it affect most often?
  • does it affect men or women more often?
  • risk factors
  • pathophysiology
  • treatment
A
  • acute and rapid progression
  • common in patients over 50
  • M>W

RF

  • previous radiotherapy
  • Down syndrome
  • Bone marrow gets infiltrated by blast cells - patient develops anaemia, thrombocytopenia, and bleeding problems
  • increased risks of infections
  • bone marrow biopsy - at last 30% blast cells

Tx - chemotherapy, sometimes bone marrow transplant

5 year survival 20%

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4
Q

Acute lymphoblastic leukaemia

A
  • clonal proliferation of lymphoid precursors (blasts) -> results in pancytopenia
  • young children (3-7 years)
  • most common pediatric cancer
  • higher incidence in association with trisomy 21
  • anaemia, persistent fever, upper abdo pain - relating to splenomegaly

Ix

  • bone marrow aspirate
  • biopsy - 30% blasts
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5
Q

CLL

A

clonal proliferation of monoclonal B lymphocytes

  • usually affects those over 70 years old

usually asymptomatic

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6
Q

CML

A

chromosomal translocation involving chromosome 9 and 22

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7
Q

Haemophilia

  • inheritance?
  • what is it?
  • coagulation results
  • treatment of minor bleeds
  • treatment of major bleeds
A
  • X linked recessive
  • Factor 8 deficiency
  • increased bleeding with minor injury

Prolonged APTT; normal Pt and bleeding time

Treat minor bleeds with desmopression (stimulates Factor 8 release stored within blood vessel walls) and tranexamic acid to encourage clotting.

Major bleeds require recombinant factor 8

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8
Q

B12 deficiency

  • where is B12 found?
  • how is it absorbed?
  • when does it commonly present?
  • what type of anaemia is it?
  • what type of symptoms?
  • how to diagnose?
  • what is the treatment?
A

mainly in meat and animal products.

Forms a complex with intrinsic factor (secreted by gastric parietal cells) in the stomach. The complex is absorbed in the ileum.

Common in 6th decade.

Macrocytic anaemia

Symptoms

  • fatigue
  • SOB on exertion
  • neurological symptoms - peripheral loss of vibration and proprioception in glove-and-stocking distribution, loss of reflexes, weakness and paraesthesia

if severe - hepatosplenomegaly and heart failure

Ix
- Vit B12 levels
FBC - anaemia, raised MCV
often pancytopenia and

Blood film - hypersegmented neutrophils, megaloblasts, oval microcytes

Tx
- IM B12 (hydroxocobalamin) on alternate days for first 6 doses then every 2 months

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9
Q

Afro-carribean toddler with painful limbs and jaundice

A

Sickle cell disease

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10
Q

2 year old boy with easy bruising and bleeding into knee joint after minor accident

A

Haemophilia A

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11
Q

What degrades fibrin clots?

A

Plasmin

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12
Q

What is needed to convert fibrinogen to fibrin?

A

Prothrombin

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13
Q

What part of the coagulation pathway does hemophilia A affect?

A

Intrinsic

- deficiency in factor 8

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14
Q

What factor is deficient in Haemophilia B?

A

factor 9

  • clinically indistinguishable from hemophilia A
  • Also known as Christmas disease
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15
Q

Does myeloma predispose you to a hyper coagulable state?

A

YES - at greater risk of clots, strokes

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16
Q

25 year old woman

  • recurrent epistaxes
  • menorrhagia
  • easy bruising
A

Immune thrombocytopenia purpura

treatment - steroids or splenectomy

Acute - IV immunoglobulin or platelet transfusions

17
Q

Vitamin B12

- blood results

A

macrocytic anaemia
thrombocytopenia
leucopenia

Hypersegmented neutrophils due to abnormal nuclear synthesis

18
Q

30 year old woman, prolonged hospital bed rest following spinal surgery.

becomes febrile, increasingly confused and drowsy, multiple bruises noted.

  • high urea
  • low platelets
A

Thrombotic thrombocytopenic purpura

Idiopathic response to infection, trauma or heparin therapy.

includes HUS

Microangiopathic anaemia

neurological symptoms are common

May develop bleeding or thrombotic complications.

Tx - supportive, or consider steroids or plasmapheresis

19
Q

Thrombotic thrombocytopenic purpura

A

Idiopathic response to infection, trauma or heparin therapy.

includes HUS

Microangiopathic anaemia

neurological symptoms are common

May develop bleeding or thrombotic complications.

Tx - supportive, or consider steroids or plasmapheresis

20
Q

10 year old boy with epistaxis and bruising two weeks after a sore throat.

A

ITP = immune thrombocytopenia purpura

  • usually acute
  • post-infective
  • self-limiting

Tx = supportive but rarely required

21
Q

Van Willebrand disease

- coagulation result

A

Normal prothrombin time

Prolonged APTT and bleeding

22
Q

Hereditary haemochromatosis

A

excessive circulating iron and deposition into tissue causing tissue damage.

AR inheritance

caused by mutations in haemachromatosis gene (HFE)
- mutation leads to excessive absorption in intestinal tract, iron deposition in organs/tissues

3/4 patients are asymptomatic

  • skin discoloration
  • diabetes mellitus
  • systemic symptoms - weakness, lethargy, arthropathy, cardiomyopathy

Men - gonadal atrophy, erectile dysfunction

Ix

  • raised serum iron
  • high transferrin saturation
  • decreased total iron binding capacity
  • raised serum ferritin level
  • abnormal LFTs

Tx

  • venesection
  • iron chelation therapy
23
Q

Which anaemia is megaloblastic?

A

Vitamin B12 deficiency

24
Q

Causes of fragment cells?

A

damaged RBCs

  • prosthetic heart valves
  • renal dialysis
  • microangiopathic haemolytic anaemias
25
Q

23 year old - previous admission for recurrent chest pains, and now presents with priapism

A

sickle cells

26
Q

Iron deficiency anaemia

A

hypochromia
anisocytosis - RBCs unequal size
poikilocytosis - RBCs - RBCs of varying shapes

27
Q

sickle cell treatment?

A

Hydroxycarbamide - increases amount of circulating fetal haemoglobin

28
Q

DIC

- coagulation results?

A

Triad of thrombocytopenia =

High prothrombin time
(excessive bleeding)
High APTT
High TT

Low fibrinogen levels
BUT
High D-dimer (fibrin degradation product - high due to intense fibrinolytic activity within blood)

Paradoxical state in which patient is prone to clotting because the body has used up all the clotting factors. BUT also prone to bleeding.

If untreated - may progress to multi organ failure and widespread bleeding

29
Q

Haemophilia

- coagulation results

A

Prolonged APTT

Normal prothrombin

30
Q

23 year old, Afro-carribean woman

  • tender swelling of hands and feet
  • scleral icterus
  • high temp, low O2 saturation
  • right lower lobe consolidation
  • sinus tachycardia
  • abnormally shaped erythrocytes, Howell-Jolly bodies
A

Sickle cell disease - acute chest syndrome

Insult (e.g. infection, PE) to body which results in reduced blood oxygenation - hypoxia

Hypoxia results in increased sickling of RBCs - blocks blood vessels traveling through lungs (and spleen), causing further reduced oxygenation.

Painful swelling of hands/feet = dactylitis
- seen in painful occlusive crises (precursors of acute chest syndrome)

Signs of splenic disruption - Howell-Jolly bodies and nucleated red cells)

31
Q

Previously fit and well, 23 year old Nigerian man presents to A and E with a 1 week history of flu-like illness and dry cough. Clinically anaemic and pyrexial and has coarse crackles on auscultation of base of right lung.
FBC - low Hb, high MCV, normal platelets

A

hemolytic anaemia following mycoplasma pneumoniae infection

32
Q

Cause of low Hb in someone with sickle cell anaemia.

  • has low reticulocytes
A

Parvovirus infection

33
Q

What characteristic features would you find on blood film of hyposplenism - e.g. post-splenectomy?

A 
Hyposplenism e.g. post-splenectomy
target cells
Howell-Jolly bodies
Pappenheimer bodies
siderotic granules
acanthocytes
34
Q

what features would you see on blood film of iron deficiency anaemia?

A

target cells
‘pencil’ poikilocytes
if combined with B12/folate deficiency a ‘dimorphic’ film occurs with mixed microcytic and macrocytic cells

35
Q

Thrombocytopenia

  • name some causes of severe/ moderate thrombocytopenia?
A 
Causes of severe thrombocytopenia
ITP
DIC
TTP
haematological malignancy
Causes of moderate thrombocytopenia
heparin induced thrombocytopenia (HIT)
drug-induced (e.g. quinine, diuretics, sulphonamides, aspirin, thiazides)
alcohol
liver disease
hypersplenism
viral infection (EBV, HIV, hepatitis)
pregnancy
SLE/antiphospholipid syndrome
vitamin B12 deficiency
36
Q

Is thrombocytopenia a common finding in alcoholic liver disease?

A

YES

Recent alcohol intake would make patients have megaloblastic anaemia.

37
Q

Is SLE a cause of haemolytic anaemia?

A

YES

38
Q

What condition would you find ‘tear drop’ poikilocytes?

A

Myelofibrosis

39
Q

Coeliac disease

- what could you find on the bloodstream?

A

hyposplenism - Howell-Jolly bodies and siderocytes

0. Year 5 (9 decks)

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