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Pathology > Haematology > Flashcards

Haematology Flashcards

1
Q

What are the typical features of thrombotic thrombocytopenia purpura?

A

Pentad:

  • Microangiopathic haemolytic anaemia
  • Thrombocytopenia
  • Fever
  • Neurological abnormalities
  • Renal impairment
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2
Q

What is the risk of giving children aspirin?

A

Reye syndrome - rapidly progressing encephalopathy

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3
Q

Name of the condition whereby pancytopenia occurs in rheumatoid arthritis

A

Felty syndrome (enlarged spleen)

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4
Q

What is the pathomechanism of thrombotic thrombocytopenic purpura, and what is the first line treatment?

A
  • Defect in vWF cleaving protease ADAMTS13
  • Autoimmune
  • Large multimers of vWF –> thrombi
  • Plasma exchange to remove antibodies and replace protease
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5
Q

Risks of hyposplenism

A
  • encapsulated bacteria: meningococcus, pneumococcus, haemophilus influenzae
  • malaria
  • capnocytophaga canimorsus
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6
Q

How to manage hyposplenism

A
  • Vaccinate against pneumococcus, meningococcus, haemophilia influenzae, and influenza virus
  • Lifelong penicillin
  • Advise on dog bites/malaria zones/prompt treatment of infection
  • Issue splenectomy card and information sheet
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7
Q

Patholophysiology of anaemia of chronic disease

A
  • Reduced RC lifespan
  • Reduced proliferation of RC precursors
  • Suppressed EPO production
  • Impaired iron utilization
  • Cytokine release
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8
Q

What are the haematinics in anaemia of chronic disease?

A

Normochromic, normocytic anaemia
Ferritin high/normal
Fe and transferrin low

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9
Q

Which rhesus blood type is a universal donor?

A

RhD negative

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10
Q

What type of antibodies are those formed against ABO antigens?

A

IgM

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11
Q

What type of antibodies are those formed against RhD antigen?

A

IgG

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12
Q

Which type of antibody is responsible for delayed and immediate transfusion reactions?

A

Delayed: IgG
Immediate: IgM

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13
Q

Why might blood be irradiated prior to transfusion, and what patient group would be the recipients?

A

Prevent transfusion associated graft versus host disease in immunocompromised patients

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14
Q

In which 2 conditions is platelet transfusion contraindicated?

A

heparin-induced thrombocytopenia, and thrombotic thrombocytopenic prupura

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15
Q

What causes febrile non haemolytic transfusion reactions, and how are they managed?

A

Cytokines released by white blood cells while in storage. Paracetamol.

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16
Q

Which blood transfusion component is most likely to be contaminated by bacteria?

A

Platelets

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17
Q

What causes immediate collapse during transfusion of bacterial contaminated blood?

A

Endotoxin

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18
Q

In what condition may allergic reaction to transfusion be life threatening?

A

IgA deficiency

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19
Q

What is the pathomechanism of transfusion related acute lung injury (TRALI), and how can it be prevented?

A

Anti WBC antibodies from donor bind to antigens on recipients WBCs. Aggregates collect in pulmonary capillaries.
Prevent by using male donors for plasma and platelets (no HLA antibodies).

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20
Q

Which patients are affected by post-transfusion purpura?

A

HPA-1a negative patients who have previously been immunised by pregnancy to transfusion to develop HLA-1a antibodies

21
Q

Which type of antibodies cross the placenta to cause haemolytic disease of the foetus/newborn?

A

IgG

22
Q

What are the consequences of haemolytic disease of the newborn to the fetus?

A

Hydrops fetalis, kernicterus

23
Q

How to manage a pregnancy where the body has anti-RBC antibodies?

A
  • Monitor fetus for anaemia using MCA doppler USS
  • Intrauterine transfusion
  • At delivery monitor baby’s Hb and bilirubin
  • Exchange transfusion (reduce bilirubin, raise Hb)
  • Phototherapy to reduce bilirubin
24
Q

Which is the most important antibody causing haemolytic disease of the newborn?

A

Anti-D

25
Q

What is the mechanism underlying giving IM anti-D Ig to RhD negative mothers with RhD positive babies? What is the time frame for this therapy?

A

So RhD positive red cells from fetus are removed by mother’s spleen before mother has chance to sensitise and produce antiD antibodies. Give within 72h of sensitising event.

26
Q

What are the consequences of anti-kell antibodies produced by a pregnant mother, on the foetus?

A

Reticulocytopenia and anaemia

27
Q

What are your top differentials for a microcytic anaemia?

A

Iron deficiency, thalassaemia trait, anaemia of chronic disease

28
Q

Which biochemical measurement is raised in iron deficiency anaemia?

A

TIBC or transferrin

29
Q

Which biochemical measurements can be low in anaemia of chronic disease?

A

Hb, transferrin/TIBC, serum iron

30
Q

What are some features in the blood film of iron deficiency anaemia?

A

Pencil cells, tear drop cells, anisopoililocytosis, oval macrocytes

31
Q

What conditions may present with basophilic stippling of RBCs on the blood film?

A

Beta thalassaemia trait
Lead poisoning
Alcoholism
Sideroblastic anaemia

32
Q

When do you see hypersegmented neutrophils?

A

B12/folate deficiency, drugs

33
Q

What are target cells also known as, and in what conditions are they found?

A

Codocytes

Iron deficiency, thalassaemia, hyposplenism, liver disease

34
Q

What are howell-jolly bodies, and in what condition are they found?

A

Nuclear remnants visible in RCs

Hyposplenism

35
Q

What are the peripheral blood film features of megaloblastic anaemia?

A

Hypersegmented neutrophils

Large cells

36
Q

What are blood film features of hyposplenism?

A

Howell Jolly bodies

Target cells

37
Q

In what conditions might someone have a poorly functioning spleen?

A

IBD, coeliac disease, sickle cell disease, SLE

38
Q

What are some causes of pancytopenia?

A 
o	Aplastic anaemia
o	Leukaemia
o	Infiltration e.g. lymphoma, carcinoma
o	Drugs e.g. chemotherapy
o	B12/folate deficiency
39
Q

How can you differentiate APML and AML by the blood film?

A

APML has a lot of auer rods

40
Q

What are some causes of thrombocytopenia?

A

Not making platelets
o Drugs e.g. chemotherapy, thiazides
o Bone marrow disorders e.g. leukaemia, aplastic anaemia, myelodysplasia, myeloma, infiltration with carcinoma

Premature destruction
o ITP (autoimmune)
o DIC
o Heparin

41
Q

Features of multiple myeloma

A

Monoclonal plasma cells
Paraprotein (immunoglobulin) deposition
Bence jones protein (Ig) in urine
CRAB: calcium (raised), renal failure, anaemia, bone (lytic lesions)

42
Q

What is the premalignant condition that precedes multiple myeloma?

A

monoclonal gammopathy of undetermined significance

43
Q

What is a characteristic feature of mature plasma cells?

A

Extensive golgi and reticular endothelium (for Ig production)

44
Q

What is light chain restriction?

A

Only kappa or lambda light chains on Ig is expressed (indicates monoclonal population of plasma cells)

45
Q

Which is the plasma cell surface marker?

A

CD138

46
Q

What is the mechanism of action of proteasome inhibitors for the treatment of multiple myeloma?

A
  • cause build-up of misfolded proteins Intracellularly which cause fatal ER stress and intracellular amino acid starvation
  • Preferentially kills myeloma cells due to unique function of producing enormous amounts of protein
47
Q

What is the cell surface marker CD30 associated with?

A

Anaplastic large cell lymphoma, hodgkin lymphoma

48
Q

What is Ki67 a marker of?

A

Cell proliferation

Pathology (5 decks)

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