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Haematology > Haemolytic Anaemias 2 > Flashcards

Haemolytic Anaemias 2 Flashcards

1
Q

Hereditary spherocytosis inheritance pattern?

A

Autosomal dominant

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2
Q

Hereditary spherocytosis pathophysiology?

A

Spectrin or ankyrin deficiency (membrane proteins)

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3
Q

Diagnosis of Hereditary spherocytosis?

A

spherocytes, osmotic fagility (lyse in hypotonic solution), flow cytometry

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4
Q

Treatment of Hereditary spherocytosis?

A

Splenectomy

Folic Acid

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5
Q

Inheritance pattern of Hereditary elliptocytosis?

A

Autosomal dominant - spectrin mutation

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6
Q

Inheritance pattern of G6PD Deficiency?

A

X-Linked Commonest RBC enzyme defect

Prevalent in malaria zones

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7
Q

Diagnosis of G6PD Deficiency?

A

Enzyme assay 2-3 months after crisis

Crisis - rapid anaemia and jaundice, Bite cells and Heinz bodies

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8
Q

Precipitants of G6PD Deficiency?

A

Oxidants: Drugs, broad beans, acute stressors, moth balls, infection

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9
Q

Treatment of G6PD Deficiency?

A

Avoid precipitants
Transfuse if severe
Genetic Screening

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10
Q

Inheritance pattern of Pyruvate Kinase Deficiency?

A

Autosomal recessive

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11
Q

Clinical Features of Pyruvate Kinase Deficiency?

A

Severe neonatal jaundice
Splenomegaly
Haemoytic Anaemia

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12
Q

Treatment of Pyruvate Kinase Deficiency?

A

Most untreated
Blood Transfusion
Splenectomy

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13
Q

Inheritance pattern of Sickle Cell Disease?

A

Autosomal Recessive

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14
Q

Mutation of Sickle Cell Disease

A 
Single base
GAG => GTG
Glu => Val
codon 6 of Beta Chain
HbS instead of HbA
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15
Q

4 Types of Sickle Cell and severity

A

Hb SS - Severe
HbAS - Trait - usually asymptomatic
HbSC - One HbS one HbC (Defective B chain)
HbS/Beta - One HbS one Thalassaemia

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16
Q

What triggers the sickling?

A

Lowered O2 Tension - HbS polymerisation - sickling

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17
Q

Features of Haemolysis in Sickle Cell Disease?

A 
Anaemia 6-8g/dL
Splenomegaly
Folate Deficiency
Gallstones
Aplastic Crisis
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18
Q

Features of Vaso-Occlusion in Sickle Cell Disease?

A 
SICKLED
Stroke
Infarctions (Hyposlenism, CKD)
Crises (Splenic, sequestration, chest and pain)
Kidney (Papillary necrosis, nephrotic syndrome)
Liver (gallstones)
Eyes (Retinopathy)
Dactilitis (Impaired growth)
Mesenteric Ischaemia
Priapism
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19
Q

Diagnosis of Sickle Cell Disease?

A

Sickle and target cells on blood film
Sickle solubility test
Hb Electrophoresis
Guthrie test - pneumococcal prophylaxis

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20
Q

Treatment of Sickle Cell Disease?

A 
Analgesia
Folic Acid
Penicillin V
Pneumovax
HiB vaccine
Hydroxycarbamide
carotid Doppler monitoring
Prophylactic exchange transfusion if turbulent
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21
Q

Pathophysiology of Thalassaemia?

A

Unbalanced Hb synthesis - unmatched chains precipitate - haemolysis and ineffective erythropoiesis

22
Q

Signs in Beta THalassaemia?

A

Skull Bossing
Maxillary Hypertrophy
Hairs on end Skull x-ray
Hepatosplenomegaly

23
Q

Diagnosis of Beta Thalassaemia

A

Hb Electrophoresis

Guthrie at birth

24
Q

Treatment of Beta THalassaemia

A

Blood transfusions
Desferrioxamine to stop iron overload
Folic acid

25
Q

How many alpha chains are there and what happens to them in alpha thalassaemia?

A 
4 chains
Deleted
2/4 - trait - asymptomatic, mild anaemia
3/4 - Moderate anaemia, splenomegaly
4/4 - Hydrops fetalis - incompatible with life
26
Q

Immunoglobulin in WAIHA

A

IgG

27
Q

Blood film findings in WAIHA

A

Spherocytes

28
Q

Causes of WAIHA

A

Primary Idiopathic

Lymphoma, CLL, SLE, Methyldopa

29
Q

Management of WAIHA

A

Steroids
Splenectomy
Immunosupperssion

30
Q

Immunoglobulin in Cold Agglutinin Disease

A

IgM

31
Q

Condition associated in Cold Agglutinin Disease

A

Raynaud’s Phenomenon

32
Q

Diagnostic test for Autoimmune Haemolytic Anaemia

A

Direct Antiglobulin Test (DAT/Coombs Test)

33
Q

Causes of Cold Agglutinin Disease

A

Primary idiopathic
Lymphoma
Infections: EBV, mycoplasma

34
Q

Management in Cold Agglutinin Disease

A

Treat underlying condition
Avoid the cold
Chlorambucil (chemo)

35
Q

Cause and symptoms of PCH

A

Viral Infection

Haemoglobin in Urine: measles, syphilis, VZV

36
Q

Pathophysiology of PCH

A

Donath-Landsteiner Antibodies - stick to RBCs in cold, complement-mediated haemolysis on rewarming

37
Q

Immunoglobulin in PCH

A

IgG

Self-limiting - dissociate at higher temp than IgM

38
Q

Name 3 Auto Immune Haemolytic Anaemias

A

WAIHA
Cold-Agglutinin
PCH

39
Q

Name 2 Non-immune Haemolytic Anaemias

A

Paroxysmal Nocturnal Haemoglobinuria

Microangiopathic Haemolytic Anaemia (MAHA)

40
Q

Pathophysiology of PNH

A

Acquired loss of surface GPI markers on RBCs - complement mediated lysis - chronic IV haemolysis

41
Q

Features of PNH

A

Morning haemoglobinuria

Thrombosis (Budd-Chiari)

42
Q

Diagnosis of PNH

A

Immunophenotype - altrered GPI

Ham’s Test - in-vitro acid induced lysis

43
Q

Treatment of PNH

A

Iron/Folate supplements
Prophylactic vaccines/ Abx
Monoclonal antibodies (eculizumab) - stops complement from binding to RBCs

44
Q

Pathophysiology of MAHA

A

Mechanical RBC destruction - forced through fibrin/plt mesh

Schistocytes

45
Q

Causes of MAHA

A

Haemolytic Uraemic Syndrome
Throbotic THrombocytopenic Purpura
DIC
Pre-Eclampsia

46
Q

Treatment of MAHA

A

Plasma Exchange

47
Q

Pentad of TTP

A

MAHA, Fever, Renal Impairment, Neuro abnormalities, Thrombocytopenia

48
Q

Cause of TTP

A

Autoimmune platelet activation

49
Q

Cause of HUS

A

E. Coli - Toxin damage to endothelial cells - Fibrin mesh and RBC damage - Impaired renal function - MAHA

50
Q

Symptoms of HUS

A

Diarrhea, Renal failure, no neuro problems

Children and Elderly

Haematology (17 decks)

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