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Haematology AB > Anaemia AB > Flashcards

Anaemia AB Flashcards

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1
Q

What is the main source of iron for haematopoiesis?

A

Recycled iron from reticuloendothelial system

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2
Q

Where is most of the body’s iron stored?

A

Hb in circulating red cells

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3
Q

What regulates iron absorption?

A

1) Oxygen tension
2) Intracellular iron levels
3) Systemic iron needs

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4
Q

What is the role of hypoxia-induced factor?

A

Hypoxia-induced factor (HIF-2a)

  • induced by reduced oxygen tension
  • transcriptional control of DMT-1 and ferroportin
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5
Q

What is the role of iron regulatory proteins?

A

Iron regulatory proteins type 1 and 2 (IRPS1/2)

  • responds to intracellular iron level
  • binds to iron-response elements that impact on mRNA stability and translation
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6
Q

What is the role of hepcidin?

A

Hepcidin: binds to ferroportin and induces its degradation

  • Systemic regulation of iron absorption; liver production
  • Regulated by: HFE, TfR2, HJV, inflammation, hypoxia, EPO
  • Acute phase reactant, largely mediated by IL-6
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7
Q

How do hepcidin levels differentiate between iron overload and iron deficiency?

A

Iron overload: hepcidin increased

Iron deficiency: hepcidin reduced

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8
Q

Where is iron absorbed?

A

Duodenum

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9
Q

Iron deficiency anaemia - what do you see on blood film?

A

Microcytic, hypochromic cells, pencil cells, thrombocytosis, increased RDW

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10
Q

Iron deficiency anaemia - what do you see on iron studies?

A 
Transferrin increased
Transferrin saturation decreased
TIBC increased
Ferritin reduced
Soluble transferrin receptor increased (less specific than low ferritin)
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11
Q

Anaemia of chronic disease - what is the mechanism?

A

IL-6&raquo_space; increased hepcidin&raquo_space; ferroportin degraded

Altered or abnormal iron haemostasis
- reduced absorption or trapping in macrophages

Reduced red cell production by bone marrow
- toxicity/cell death of precursors; CK mediated effect

Blunted response to EPO
- reduced production of EPO, reduced receptors, reduced responsiveness

Shortened red cell survival
- erythrophagocytosis; CK and free radical damage

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12
Q

How does soluble transferrin receptor differentiate iron deficiency from anaemia of chronic disease?

A

Increased in iron deficiency anaemia

Normal in anaemia of chronic disease

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13
Q

What factors affect the level of soluble transferrin factor?

A

Soluble transferrin receptor is derived from bone marrow erythroid precursors

Directly proportional to erythropoietic rate

Inversely proportional to iron stores

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14
Q

Blood film - what is the cause of ovalocytes?

A

Megaloblastic anaemia

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15
Q

Megaloblastic anaemia - which drugs cause this?

A

Antifolate drugs
- MTX, pentamidine, TMP

DNA synthesis
- AZA, hydroxyurea, Zidovudine, chemo

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16
Q

Macrocytic anaemia - what are the causes?

A 
B12/folate deficiency
Drugs (antifolate, DNA synthesis)
Reticulocytosis
BM pathology (MDS, myeloma, aplastic anaemia)
Liver disease, EtOH, phenytoin
Copper deficiency
Arsenic
Down syndrome
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17
Q

Macrocytic anaemia - what are some factitious causes?

A

Cold agglutinins
Old sample
Hyper-osmolar state

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18
Q

B12 - where is it absorbed?

A

Terminal ileum

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19
Q

B12 - how is it absorbed?

A

Initially bound by transcobalamin I or haptocorrin (R protein)

Pancreatic enzymes release cobalamin from these and allow binding to intrinsic factor from gastric parietal cells

B12-IF complex binds to receptor (cubulin) in terminal ileum, taken up by receptor-mediated endocytosis and absorped into portal circulation

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20
Q

Pernicious anaemia - what is the mechanism?

A

Autoimmune destruction of gastric mucosa/parietal cells

Leads to reduced acid production and reduced IF

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21
Q

Pernicious anaemia - what are the associations?

A 
Blue eyes, fair hair, northern european
Family history
Blood group A
Vitiligo, thyroid disease, Addison's, hypoparathyroid
Hypogammaglobulinaemia
Gastric carcinoma
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22
Q

Pernicious anaemia - what investigations can you do?

A

Intrinsic factor antibodies - very specific but only 50% sensitive

Parietal cell antibodies - sensitive but not specific (positive in 15% of normal females)

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23
Q

B12 deficiency - causes?

A 
Pernicious anaemia
Nutritional (strict vegans)
Ileal pathology
- Crohn's
- Ileal resection
- Tropical sprue/tapeworm
- Mutation/deficiency of receptor for IF
Gastrectomy
Other
- NO poisoning
- Congenital abnormalities
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24
Q

B12 deficiency - what are the features on blood film?

A 
Macrocytic anaemia 
Hypersegmented neutrophils
- May develop before anaemia develops
Oval macrocytes
Low reticulocyte count
Pancytopaenia may occur
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25
Folate - what is the source?
Dietary (only)
26
Folate - where is it absobed?
Small bowel
27
Folate - how long do the stores last?
Months
28
Folate - where is it stored?
>95% in RBC
29
Decreased haptoglobin - what are the causes
``` Haemolysis Liver failure Megaloblastic anaemia Anaemia of chronic disease Congenital ```
30
Microangiopathic haemolytic anaemia - what are the causes?
``` TTP/HUS Atypical HUS Pre-eclampsia HELLP Malignant hypertension Renal allograft rejection ```
31
Atypical HUS - what is the cause?
Inherited dysregulation of complement system
32
Atypical HUS - what is the treatment?
Eculizumab
33
TTP - what is the classic pentad?
``` Haemolysis with red cell fragmentation Thrombocytopaenia Fever Neurological signs Renal impairment ```
34
TTP - what is the pathogenesis?
1. ADAMTS13 deficiency (hereditary or acquired); protease that cleaves vWF 2. Increased high molecular weight vWF 3. Abnormal platelet aggregation 4. Microvascular thrombosis 5. Tissue ischaemia
35
TTP - what are the causes?
``` Idiopathic Pregnancy Drugs (CsA, chemotherapy) Bone marrow transplant Malignancy HIV Familial ```
36
TTP - what is the mortality if untreated?
>90% - haematologic emergency
37
TTP - what is the treatment?
PLEX Avoid platelet transfusions Immunosuppression
38
PNH - what is the genetic basis?
Rare, acquired clonal disease of haematopoiesis | Somatic mutation in pig-A gene
39
PNH - what is the mechanism?
Defective production of phosphatidylinositol glycan A (PIG-A) which is essential for the formation of the GPI anchor Loss of several surface proteins that protect the cell from complement-mediated lysis: CD55, CD59
40
PNH - what is the diagnosis?
Flow cytometry (gold standard) - CD55 and CD59 on RBC + neutrophils - FLAER
41
PNH - what is the treatment?
Transfusions; SCT Thrombosis management - lifelong after 1st thrombosis Eculizumab: anti-C5 (targets terminal component of C' cascade)
42
Paroxysmal cold haemoglobinuria - causes?
Idiopathic Syphilis Viral infections
43
Paroxysmal cold haemoglobinuria - mechanism?
Biphasic IgG anti-P antibody (Donath-Landsteiner Antibody) binds RBC at low temperatures Upon warming, C' mediated lysis occurs
44
Paroxysmal cold haemoglobinuria - blood film?
Red cell agglutination
45
Paroxysmal cold haemoglobinuria - management?
Cold avoidance | Splenectomy NOT useful
46
Spherocytes - what conditions are they found in?
AIHA | Hereditary spherocytosis
47
Warm AIHA - what are the 6 causes?
``` Idiopathic SLE/autoimmune Lymphoproliferative: CLL/lymphoma Infection: HCV, CMV Drugs: methyldopa, antibiotics Evan's syndrome: combination of autoimmune haemolysis with ITP ```
48
Warm AIHA - what is the mechanism?
Antibodies that react with red cells at 37 degrees- IgG*** +/- complement - RBC taken up by macrophages in the RE system via Fc receptors
49
Warm AIHA - what are the investigations?
Direct antiglobulin test (Coombs test) | - Demonstration of autoantibodies attached to the patient's red cells
50
DAT - what are the causes of false positive and false negatives?
False positives: 10% of hospitalised patients - Recent transfusion - delayed haemolytic transfusion reaction False negatives:- IgA or IgM mediated
51
Warm AIHA - what is the treatment?
Prednisolone 1mg/kg then taper- First line IVIG Folate supplement Immunosuppression: AZA/6MP Splenectomy and vaccination - Best second line after steroids Rituximab
52
Warm AIHA - what are the characteristic findings?
Anaemia Haemolysis Spherocytes Splenomegaly
53
Cold AIHA - what is the mechanism?
Antibodies that react with RBCs
54
Cold agglutinin disease - what are the causes?
Primary - Associated with MGUS or asymptomatic LPD Secondary (majority) - LPD - Mycoplasma - EBV - Autoimmune
55
Cold agglutinin disease - treatment?
Cold avoidance Chlorambucil if underlying LPD Rituximab Does NOT respond to steroids or splenectomy
56
Hereditary spherocytosis - what is the pathology?
Loss of VERTICAL interactions Ankyrin 50% Spectrin 30% Band 3 20%
57
Hereditary elliptocytosis - what is the pathology?
Loss of HORIZONTAL interactions Alpha or beta spectrin Protein 4.1 Band 3
58
South-East Asian Ovalocytosis - what is the pathology?
Band 3 abnormality
59
Hereditary spherocytosis - what is the genetic inheritance pattern?
Autosomal dominant - Family history in 75%
60
Hereditary spherocytosis - what is the typical presentation?
Haemolysis of varying intensity, exacerbated by intercurrent illness Jaundice Cholelithiasis Splenomegaly
61
Hereditary spherocytosis - what is on the blood film?
Polychromasia Prominent spherocytes Note - typical film and FHx sufficient to establish diagnosis
62
Hereditary spherocytosis - DAT positive or negative?
Negative
63
Hereditary spherocytosis - flow cytometry findings?
Eosin-5-maleimide (EMA) binding | - Reacts covalently with band 3 protein
64
Hereditary spherocytosis - management?
Folate supplementation | Splenectomy
65
Bite cells - what condition are they found in?
G6PD deficiency | The 'bites' result from removal of denatured Hb by macrophages in the spleen
66
Prickle cells - what condition are they found in?
Pyruvate Kinase deficiency
67
G6PD - what is the pathway involved?
Hexose-monophosphate pathway: pentose phosphate pathway
68
G6PD deficiency - how does the haemolysis present?
Acute haemolytic crisis | Blood film: bite cells, blister cells
69
G6PD deficiency - what is the genetic inheritance and prevalence?
X linked | Common
70
G6PD deficiency - what is the mechanism of RBC damage?
Susceptibility to oxidative stress
71
Pyruvate kinase deficiency - what is the pathway involved?
Glycolytic pathway
72
Pyruvate kinase deficiency - how does the haemolysis present?
Chronic haemolysis
73
Pyruvate kinase deficiency - what is the mechanism of RBC damage?
Reduced ATP formation >> RBC rigidity
74
Pyruvate kinase deficiency - what is the genetic inheritance and prevalence?
Autosomal recessive | Rare
75
G6PD deficiency - how to you test for this and what may cause false negative results?
Enzyme assays | False negative if reticulocytosis (have higher G6PD levels)
76
G6PD deficiency - what are the precipitants?
``` Acute illness/infection Antimalaria drugs - primaquine Sulphur containing drugs - bactrim, dapsone Aspirin Vitamin K analogues Fava beans Probenecid ```
77
Immune thrombocytopaenia - what are the associations?
``` AIHA CLL Autoimmune disease (RA, SLE) H pylori Hep C ```
78
Immune thrombocytopaenia - what is the management?
Observation if Plt >30 First line: Prednisolone 1-2mg/kg IVIG Splenectomy with vaccination (most effective) New drugs: Romiplostim: TPO receptor antagonist Eltrombopag: TPO mimetic Other options: Immunosuppressive: AZA etc Rituximab Danazole
79
DIC - what is the prognostic significance in sepsis and severe trauma?
Independent predictor of mortality
80
DIC - what are the causes?
``` Sepsis Trauma Malignancy Pancreatitis Obstetric (amniotic fluid embolus, abruption, HELLP) Liver failure Snake venom ```
81
DIC - what is the management?
Platelet transfusion FFP (coagulation factors) Cryoprecipitate (fibrinogen)
82
HIT - what is the mechanism?
1. IgG Ab recognises heparin-PF4 complexes 2. PF4-Heparin-IgG complex bind to platelet surface 3. Platelet activation and consumption via Fc receptor
83
HIT - at what time in heparin therapy does it typically develop?
5-14 days after commencing heparin
84
HIT - what are the features?
Plt fall by >50% but severe thrombocytopaenia uncommon
85
HIT - what is the management?
Cease Heparin | Anticoagulate with direct thrombin inhibitor (Bivalirudin, Argatroban)
86
HIT - how do you diagnose?
HIT pre-test probability score (4T score) - Thrombocytopaenia, Timing, Thrombosis, oTher causes Immunoassay to detect HIT Ab that binds to PF4 - High Sn, low Sp Functional assay: - Serotonin release assay (SRA) - Heparin-induced platelet aggregation (HIPA)
87
Target cells - causes?
Hb disorders - Thalassaemia Iron deficiency Liver disease
88
What chromosome is Hb alpha on?
Chromosome 16
89
What chromosome is Hb beta on?
Chromosome 11
90
Alpha 0 thal - what is the genetic abnormality?
Deletion or inactivation of both alleles on a single chromosome
91
Alpha + thal - what is the genetic abnormality?
One allele inactivated on the same chromosome
92
Beta 0 thal - what is the genetic abnormality?
Abnormal gene is not expressed
93
Beta + thal - what is the genetic abnormality?
Reduced expression of abnormal gene
94
What is in Hb barts?
4 gamma chains
95
What is in HbF?
2 alpha, 2 gamma chains
96
What is in HbA?
2 alpha, 2 beta chains
97
What is in HbH?
4 beta chains- In alpha thalassaemia (a - / - - )
98
What is the clinical significance of HbH?
High oxygen affinity Inclusion bodies Unstable tetrameres Haemolysis
99
What is in HbA2?
2 alpha, 2 delta chains- Increased in beta thalassaemia
100
What are findings on HPLC in beta thalassaemia trait/minor?
Increased HbA2 *Increrased HbF* Iron deficiency can reduce HbA2 so must assess iron status
101
Beta thalassaemia major - what is the management?
Transfusion support: aiming Hb 9-10 to suppress extramedullary haematopoiesisIron chelation therapy - Desferrioxamine = survival benefit - Ferritin >2500 associated with a higher cardiac risk - Aim ferritin
102
Desferrioxamine - what are the ADR?
``` Local reactions (subcut injection) Deafness Retinal toxicity Growth retardation Infections ```
103
Sickle Hb - what is the abnormality?
CAG to GTG; B-globin gene Substitutes valine for glutamic acid (HbS) HbS polymerizes into long fibres on deoxygenation RBC: distorted, rigid, damaged membranes
104
Sickle cell disease - what is the Hb configutaion?
Hb S/S Hb C/S Hb beta/S
105
Sickle cell disease - what is the genetics and prevalence?
Autosomal recessive | Among most common AR disorders
106
Hyposplenism - what are the features on blood film?
Howell-Jolly bodies Target cells Occasional acanthocytes Lymphocytosis

Haematology AB (7 decks)

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