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SBOMS > Haematology > Flashcards

Haematology Flashcards

1
Q

Four factors that natural haemostasis relies on?

A

Vascular endothelium

Platelets

Coagulation factors

Fibrinolysis

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2
Q

What platelets formed from, what protein stimulates that?

A

Formed from megakaryocytes. Stimulated by thrombopoietin.

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3
Q

What organ mostly produces thrombopoeitin?

A

The Liver

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4
Q

Lifecycle of a platelet?

A

Circulate for 6-8days and are then destroyed in the spleen.

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5
Q

What will a blood film and a blood count tell you about platelets?

A

Number and shape of platelets

Can reveal different disorders such as leukaemia

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6
Q

Examples of coagulation tests?

A

PT (prothrombin time)
APTT (Activated Partial Thromboplastin Time)
PTTK
Thrombin Time

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7
Q

Prothrombin time is elongated in what disorders?

A
  • Abnormal factor II, V, VII, X
  • Liver disease
  • Warfarin
  • Vit K deficiency
  • DIC (disseminated intravascular coagulation)
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8
Q

What is Disseminated intravascular coagulation? (DIC)

A

Pathological condition in which there is abnormal activation of the clotting cascade and blood clots form in small vessels around the body.

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9
Q

What is INR?

A

Special Prothrombin time - used for monitoring warfarin.

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10
Q

Activated Partial Thromboplastin Time (APTT) is prolonged in what?

A
  • Deficiency/ inhibition of II, V, VIII, IX, X, XI, XII (not VII) – NB XII deficiency can be asymptomatic
  • Unfractionated heparin therapy (not LMW heparins)
  • Liver disease
  • Vit K deficiency
  • DIC
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11
Q

How does a correction test work?

A

Adds normal plasma to patients plasma and if that corrects the clotting time there is a deficiency. If not it is likely there is an inhibitor present.

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12
Q

Different types of purpura?

A

Senile - associated with old age and small trauma (benign)

Henoch-schonlein - associated with children following infection, mostly harmless, can lead to CKD

Steroid induced

Infection associated purpura

Thrombotic thrombocytopenic purpura

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13
Q

What is purpura?

A

A rash of purple spots on the skin caused by internal bleeding from small blood vessels.

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14
Q

Causes of thrombocytopenia?

A

Drugs, chemicals and viral infections - lead to megakaryocyte depression

Leukaemia, lymphoma, aplastic anaemia. HIV - bone marrow failure

DIC, Thrombotic thrombocytopenic purpura (TTP), Heparin - increased platelet production leads to splenomegaly.

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15
Q

Congenital causes of platelet disorders?

A

Bernard-soulier

Glazmanns

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16
Q

Acquired causes of platelet disorders?

A

NSAIDs
Renal Failure
Cardiopulmonary bypass (the pump in surgery)
Myeloproliferative disorders

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17
Q

Examples of coagulation disorders?

A

Congenital:

  • Haemophilia (A and B)
  • Von-willibrand disease (deficiency in von-willibrand factor)
  • Factor XI deficiency

Acquired:

  • Vit K deficiency
  • Liver disease
  • DIC
  • Anticoagulant drugs
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18
Q

What is the diagnosis?

  • 4 year old boy with increased bleeding tendency
  • No FHx
  • Prolonged APTT, other clotting times are normal
    Corrects with normal plasma
  • Factor VIII assay is low
A

Haemophilia A

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19
Q

Types of Haemophilia, what are they lacking in each?

A

A - lacking factor VIII (8)

B - lacking factor IX (9)

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20
Q

Management of haemophilia?

A

Refer to a haemophilia centre - screen mother and siblings

Mild haemophilia treated with tranexamic acid and desmopressin

Severe will require factor VIII replacement

Avoid use of NSAIDS and injections if at all possible

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21
Q

Symptoms of anaemia?

A

Lethargy

SOB

Angina

headache

22
Q

Causes of anaemia?

A

Bleeding

Iron, B12, Folate deficiency

Malabsorption

Systemic illness

Haemolysis

Inherited RBC disorders

Bone marrow failure

23
Q

Causes of microcytic anaemia?

A

iron deficiency, haemoglobinopathies (including thalassaemias)

24
Q

Causes of normocytic anaemia

A

Anaemia of chronic disease

25
Q

causes of macrocytic anaemia

A

B12/Folate deficiency

26
Q

Features of B12 deficiency anaemias?

A

Hypersegmented neutrophils

Macrocytic and oval red cells

Mild Jaundice

Peripheral neuropathy

27
Q

What is pancytopaenia?

A

deficiency of white cells red cells and platelets in the blood

28
Q

Causes of pancytopaenia?

A
  • Bone marrow failure/infiltration (leukaemia, lymphoma, carcinomas, myelofibrosis)
  • Septicaemia
  • Splenomegaly
  • Immune-mediated destruction
29
Q

Types of leukaemia?

A

Acute - myeloid (AML) or lymphoblastic (ALL)

Chronic - myeloid (CML) or lymphocytic (CLL)

30
Q

What is acute leukaemia defined as?

A

> 20% of nucleated bone marrow cells are blasts

31
Q

Causes of leukaemia?

A
  • Ionising radiation
  • Viruses
  • Chemicals
  • Congenital factors
    • Acquired haematological disorders
32
Q

Most common type of cancer in pts below 15 yrs

A

ALL

33
Q

Most common type of cancer in pts above 50 yrs

A

AML

34
Q

Clinical features of leukaemia (ALL and AML)?

A

Anaemia
Neutropenia
Thrombocytopenia

35
Q

Clinical features of CML?

A
  • Fatigue
  • Malaise
  • Anorexia
  • Abdominal discomfort (splenomegaly/ splenic infarction)
  • Excessive sweating from hypermetabolism
  • Pallor
  • XS WBC breakdown = gout
  • Priapism (painful persistent erection from hypercoagulation)
36
Q

Most common type of leukaemia overall?

A

CLL

37
Q

Triphasic course of CML?

A

1) Chronic

2) Accelerated course

38
Q

Features of CLL?

A
  • Insidious onset
  • Proliferation and accumulation of immune incompetent B-lymphocytes
  • Associated with infiltration of lymph nodes, spleen and liver
  • Infection is common due to neutropenia, hypogammaglobulinaemia, impaired immunity
  • Can lead to diffuse lymphoma/Richter’s syndrome or PLL
39
Q

Role of von willibrands factor?

A

Helps Platelets stick to the endothelium

40
Q

Basic coagulation cascade?

A

Prothrombin — Thrombin

Fibrinogen — fibrin

Fibrin goes on to form a fibrin polymer and then with factor XIIIa forms stable fibrin.

41
Q

The anti coagulation cascade basic steps?

A

Plasminogen — plasmin

Plasmin converts fibrin to soluble products

42
Q

What is idiopathic acquired aplastic anaemia?

A

Intrinsic stem cell defect, where your bone marrow no longer produces cells, due to:

  • Failure of stromal microenvironment
  • Growth factor defect or deficiency
  • Immune suppression of the marrow
43
Q

Symptoms of idiopathic acquired aplastic anaemia?

A

Fatigue
Bleeding
Infections

44
Q

Lab investigations for anaemia?

A

Blood count and film

Haematinics

B12, folate, ferritin

Inflammatory indices

Reticulocytes

Special tests:

  • Bone marrow biopsy
  • Special tests for haemolysis
45
Q

Lab features of iron deficiency?

A

Low haemoglobin

Low ferritin (low iron stores)

Blood film appearances:

  • Hypochromic, microcytic cells,
  • Pencil shaped cells
  • Target cells
  • Increased variation in red cell shape
46
Q

Lab features of folate deficiency?

A

Hypersegmented neutrophils (same as b12)

Macrocytic and oval red cells (same as b12)

Low serum folate

Low red cell folate

47
Q

Lab findings for anaemia of chronic disease?

A

Most are normal

48
Q

Lab findings in haemolytic anaemia?

A

Decreased Hb

Raised reticulocyte (immature red cell without a nucleus) count

Red cells show altered red cell shape and colour

(depending on underlying cause of haemolysis)

Serum bilirubin raised
Serum haptoglobins low or absent

49
Q

Tests to diagnose leukaemia?

A

Examination of blood and bone marrow

Cell morphology

Flow cytometry to detect antigens and cell surface molecules

Cytogenetic studies

50
Q

Lab findings for idiopathic acquired aplastic anaemia?

A

Pancytopenia
Low reticulocyte count; red cells may be macrocytic
Marrow markedly low in cells
Neutrophil count low
No abnormal cells in the peripheral blood
Normal cytogenetics

SBOMS (18 decks)

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