Haematology Flashcards

1
Q

Splenectomy

A

Causes: trauma, rupture, AIHA, ITP, HS, hypersplenism
Complications: Redistributive thrombocytosis (early VTE),
Gastric dilation, Left LL atelectasis, susception to infection (haemophilus, pneumo, menigo)
Mx: Immunisations (pnemococcal, HiB, Men C, Flu)
Daily Abx (Pen V or erythromycin)
Warning/alert card
Film: Howell-Jolly bodies, Pappenheimer bodies, target cells

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2
Q

Hyposplenism

A
Causes:
•	Splenectomy
•	SCD
•	Coeliac disease
•	IBD
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3
Q

Microcytic anaemia

A
Haem defect
•	IDA
•	Sideroblastic/lead poisoning
•	Anaemia of chronic disease
Globin defect
•	Thalasaemia
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4
Q

Normocytic anaemia

A
  • Recent blood loss
  • Bone marrow failure
  • Renal failure
  • Early anaemia of chronic disease
  • Pregnancy (Increased plasma volume)
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5
Q

Macrocytic anaemia

A
Megaloblastic
•	Vit B12 or folate def
•	Anti-folate drugs (phenytoin, methotrexate)
•	Cytotoxics
Non-megaloblastic
•	Reticulocytosis
•	Alcohol or liver disease
•	Hypothyroidism 
•	Myelodysplasia
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6
Q

Haemolytic anaemia

A

Acquired
• Immune mediate
o AIHA (warm, cold or PCH)
o Drugs (penicillin, quinine, methyldopa)
o Allo-immune (acute transfusion reaction)
• Paroxysmal nocturnal haemaglobinuria
• Mechanical
o MAHA (DIC, HUS, TTP)
o Heart valve
• Infection (malaria)
• Burns
Hereditary
• Enzyme (G6PD def and pyruvate kinase def)
• Membrane (Hereditary spherocytosis and eliptocytosis)
• Haemaglobinopathy (SCD and thalasthaemia)

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7
Q

Iron deficiency anaemia

A

Signs: koilonychias, angular stomatitis/glossitis/cheilosis, Plummer-Vinson syndrome.
Causes: Increased loss, decreased intake, malabsorption
Ix: Decreased ferritin, increased TIBC, decreased transferrin. Blood film (anisocytosis, poikilocytosis, pencil cells)
Imaging: Upper and lower endoscopy
Mx: ferrous sulphate 200mg PO TDS (SE: GI upset)

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8
Q

Sideroblastic anaemia

A

Ineffective erythropoiesis – red cells fail to form haem causes deposition of iron in mitochondria which rings around nucleus. Causes endo, liver and cardio damage
Congenital: delta-aminolevulinate synthase-2 def
Acquired: Myelodysplasia, alcohol, lead, anti-TB meds
Ix: Hypochromic microcytic anaemia, raised ferritin
BM biopsy: sideroblasts and increased iron stores
Mx: supportive + treat underlying cause
?pyridoxine

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9
Q

Hereditary spherocytosis

A

AD defect in RBC membrane, spherocytes get trapped in spleen – extravascular haemolysis
Features: splenomegaly, jaundice, pigmented gallstones
Complications: Aplastic crisis, megaloblastic crisis
Ix: ↑osmotic fragility, spherocytes, DAT -ve
Mx: folate and splenectomy

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10
Q

G6PD deficiency

A

X-linked disorder of pentose phosphate shunt, ↓NADPH production – RBC oxidative damage
Mediterranean and far east populations
Haemolysis triggers: fava bean, naphthalene (moth balls), infection, drugs (antimalarias, henna, dapsone, sulphonamides)
Ix: Film (irregularly contracted cells, Bite cells, ghost cells, blister cells, Heinz bodies) G6PD assay
Mx: treat infection, stop precipitant, transfusions

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11
Q

Coagulopathy

Vascular disorders

A
Vascular disorders
•	Congenital
o	HHT
o	Ehler’s Danlos syndrome 
o	Pseudoxantoma elasticum
•	Acquired
o	Senile purpura
o	Vit C def
o	Infection (meningococcus)
o	Steroids
o	Vasculitis
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12
Q

Coagulopathy

Platelet disorders

A
Thrombocytopenia
•	Decreased production
o	BM failure (aplastic, inflitraion, drugs)
o	Megaloblastic anaemia
•	Increased destruction 
o	Immune: ITP, SLE, CLL, heparin, viruses
o	Non-immune: DIC, TTP, HUS, PNH, APLS
•	Splenic pooling
o	Portal HTN
o	SCD
Functional defects
Drugs (aspirin, clopidogrel)
Secondary (paraproteinaemias, uraemia)
Hereditary
•	Bernard-Soulier GpIb def
•	Glanzmann’s: GpIIb/IIIa def
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13
Q

Coagulopathy

Coagulation disorders

A
Acquired:
•	Severe liver disease
•	Anticoagulants
•	Vit K def
Congenital:
•	Haemophilia A: F8 def
•	Haemophilia B: F9 def
•	VWD
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14
Q

Immediate blood transfusion reactions

A
Immediate:
•	Haemolytic
•	Bacterial contamination
•	Febrile non-haemolytic 
•	Allergic 
•	TRALI
•	Fluid overload
•	Massive transfusion
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15
Q

Delayed blood transfusion reactions

A
Delayed:
•	Delayed haemolytic 
•	Iron overload
•	Post-transfusion purpura 
•	GvHD
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16
Q

Aplastic anaemia

A

Rare stem cell disorder, 15-24 yrs and >60yrs
Features: pancytopenia, hypocellular marrow
Inherited:
• Fanconi anaemia (Ashkenazi, short pigmented)
• Dyskeratosis congenital (premature ageing)
• Swachman-Diamond syn (+ exocrine pancreatic failure)
Acquired:
• Drugs
• Viruses (parvivirus, hepatitis)
• Autoimmune (SLE
Ix: BM biopsy
Mx: supportive – transfusion
Immunosuppression
Allogenic BMT (may be curative)

17
Q

Bone Marrow failure causes

A
Congenital: Fanconi anaemia
Acquired:
•	Idiopathic aplastic anaemia
•	BM infiltration
•	Haematological (Leukaemia, lymphoma, myelofibrosis, myelodysplasia, megaloblastic anaemia)
•	Infection: HIV
•	Radiation
•	Drugs: Cytotoxic, Abx (chloramphenicol, sulphonamides), diuretics (thiazides), anti-thyroid (carbimazole), Anti-psychotic (clozapine), Anti-epileptic (phenytoin)
18
Q

Drugs causing bone marrow failure

A
Cytotoxic (cyclophosphamide, azathioprine, methotrexate) 
Abx (chloramphenicol, sulphonamides)
diuretics (thiazides)
Anti-thyroid (carbimazole)
Anti-psychotic (clozapine)
Anti-epileptic (phenytoin)
19
Q

Myeloproliferative conditions

A
  1. Polycythaemia vera
  2. Essential thrombocythaemia
  3. Primary myelofibrosis
  4. CML
20
Q

Acute lymphoblastic leukaemia

A

Children 2-5 yrs, rare in adults (worse prognosis)
Arrested lymphoblast development and proliferation
80% B lineage, 20% T lineage
RF: Genetic, radiation, Down’s
Features: BM failure, lymphadenopathy, orchidomegaly, thymic enlargement, hepatosplenomegaly, bone pain
CNS: palsies, meningism
Ix: ↑WCC↓RBC,PMN, plats. Bone marrow aspirate
>20% blasts
Mx: supportive (blood productions, allopurinol), Infection prophylaxis.
Chemo: 1. Remission 2. Consolidation 3. Maintenance
BMT – best option for younger patients

21
Q

Chronic lymphocytic leukaemia

A

Features: asymptomatic, anaorexia, weight loss, bleeding, infections, lymphadenopathy
Complications: hypogammaglobulinaemia (causing infections), warm AIHA, Richter’s transformation
Ix: film – smudge cells, immunophenotyping

22
Q

Chronic myeloid leukaemia

A

Philadelphia chromosome t(9;22) BCR-ABL gene
Features: middle age, anaemia, weight loss, abdo pain, splenomegaly

Mx: Imatinib (Inhibits tyrosine kinase associated with BCR-ABL defect), hydroxyurea, Interferon-alpha, allogenic BMT

23
Q

Hodgkin’s lymphoma

A

Malignancy proliferation of lymphocytes with Reed-Sternberg cells. Bimodal age distribution (3rd and 7th decades)
Types:
1. Modular sclerosing
2. Mixed cellularity
3. Lymphocyte predominant
4. Lymphocyte depleted
Features: Weight loss, fever, night sweats

24
Q

Non-Hodgkin’s lymphoma

A

Burkitts lymphoma t(8;14)

Mantle cell lymphoma t(11;14)

25
Q

Massive splenomegaly

A
>20 cm 
Causes:
•	CML
•	Myelofibrosis
•	Malaria
•	Leishmaniasis
•	Gauchers, (AR, glucocerebrosidase def)
26
Q

Splenomegaly

A
Haematological
•	Haemolysis (hereditary spherocytosis)
•	Myeloproliferative disorders
•	Leukaemia, lymphoma
Infection
•	EBV, CMV, hepatitis, HIV
•	TB, infective endocarditis
•	Malaria, leishmaniasis, hydatid disease
Portal HTN – cirrhosis, Budd-Chiari
Connective tissue: SLE, RA, Sjogrens
Other:
•	Sarcoid
•	Amyloidosis
•	Gauchers
•	Primary antibody deficiency
27
Q

Polycythaemia rubra vera

A

Overproduction of RBC (often accompanied by increase of neutrophils and platelets) JAK2 Mutation
Features: hyperviscosity, pruritus (post hot bath), splenomegaly, haemorrhage, plethoric appearance, HTN (1/3)
Ix: JAK2, FBC, serum ferratin, U&Es, LFTs

28
Q

Thrombophilia

A
Gain of function polymorphisms: 
•	Factor V leiden (activated protein C resistance)
•	Prothrombin gene mutation
Deficiencies of naturally occurring anticoagnulants:
•	Antithrombin III deficiency 
•	Protein C deficiency 
•	Protein S deficiency 
Acquired
•	Antiphospholipid syndrome
Drugs: cOCP
29
Q

Waldenstrom’s macroglobulinaemia

A

Older men, lymphoplasmacytoid malignancy - secretion of IgM paraprotein
Features:
Weight loss, lethargy, hyperviscosity (visual disturbance), hepatosplenomegaly, lymphadenopathy, Cryoglobulinaemia (Raynauds)
Ix: monoclonal IgM paraproteinaemia

30
Q

Trephine Biopsy

A

Bone marrow biopsy – sample of tissue taken for investigation

Complication: bleeding, infection, require transfusion, damage to local structures