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Haematology > Haematology P2 > Flashcards

Haematology P2 Flashcards

1
Q

Transfusion thresholds and targets after transfusion red blood cells:

A 
transfusion threshold:
-without ACS: 70g/L
-with ACS: 80g/L
target after transfusion:
-without ACS: 70-90g/L
-with ACS: 80-100g/L
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2
Q

How should RBC be stored before infusion and how quickly transfused?

A
  • stored at 4 degrees

- non urgent scenario, RBC transfused over 90-120 minutes

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3
Q

What is cryoglobulinaemia:

A
  • immunoglobulins which undergo reversible precipitation at 4 degrees
  • dissolve at 37 degrees
  • 1/3 idiopathic
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4
Q

Type I cryoglobulinaemia:

A
  • monoclonal - IgG or IgM

- associations: multiple myeloma, Waldenstrom macroglobulinaemia

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5
Q

Type II cryoglobulinaemia:

A
  • mixed monoclonal and polyclonal, usually with rheumatoid factor
  • associations: hep C, rheumatoid arthritis, Sjogren’s, lymphoma
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6
Q

Type III cryoglobulinaemia:

A
  • polyclonal: usually rheumatoid factor

- associations: rheumatoid arthritis, Sjogren’s

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7
Q

Symptoms cryoglobulinaemia:

A
  • Raynaud’s only type I
  • cutaneous: vascular purpura, distal ulceration, ulceration
  • arthralgia
  • renal involvement (diffuse glomerulonephritis)
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8
Q

Cryoglobulinaemia tests:

A
  • low complement (esp C4)

- high ESR

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9
Q

Treatment of cryoglobulinaemia:

A
  • immunosuppression

- plasmapheresis

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10
Q

What is cryoprecipitate?

A
  • blood product made from plasma (factor VIII and fibrinogen)
  • usually transfuse as 6 unit pool
  • indications: massive haemorrhage and uncontrolled bleeding due to haemophilia
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11
Q

DIC

A
  • dysregulated coagulation and fibrinolysis
  • widespread clotting and bleeding
  • mediated by release of TF which triggers extrinsic pathway and subsequently intrinsic pathway
  • caused by sepsis, trauma, obstetric complications (HELLP), malignancy
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12
Q

What is HELLP syndrome?

A

haemolysis, elevated liver function tests, low platelets

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13
Q

Typical picture of DIC:

A
  • low platelets
  • prolonged APTT, prothrombin and bleeding time
  • fibrin degradation products raised
  • shistocytes due to microangiopathic haemolytic anaemia
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14
Q

Factor V Leiden

A
  • activated protein C resistance
  • most common inherited thrombophilia
  • mis-sense mutation activated by factor V is inactivated 10 times more slowly by activated protein C than normal
  • hetero: 4-5 fold risk VTE
  • homo: 10 fold risk VTE
  • screening not recommended
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15
Q

Fanconi anaemia:

A
  • autosomal recessive
  • haematological: aplastic anaemia, increased risk acute myeloid leukaemia
  • neurological
  • skeletal abnormalities: short, thumb/radius abnormalities
  • cafe au lait spots
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16
Q

G6PD deficiency

A
  • commonest RBC enzyme defect
  • Mediterranean, Africa
  • x-linked recessive
  • reduces NADPH and glutathione which increases red cell susceptibility to oxidative stress
  • diagnose by checking levels 3 months after acute episode of haemolysis when RBCs with most severe reduced G6PD will have been haemolysed
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17
Q

Features of G6PD deficiency:

A
  • neonatal jaundice
  • intravascular haemolysis
  • gallstone
  • splenomegaly
  • Heinz bodies on blood film, bit and blister cells
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18
Q

Drug causes of haemolysis:

A
  • antimalarials: primaquine
  • ciprofloxacin
  • sulph- group drugs: sulphonamides, sulphasalazine, sulfonylureas
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19
Q

What is GVHD?

A
  • multi-system complication of allogenic bone marrow transplant or solid organ transplant or transfusion in immunocompromised
  • donor T cells mount immune response to host cells
  • not same as transplant rejection
  • poor prognosis
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20
Q

Criteria for diagnosis of GVHD:

A
  • transplanted tissue has immunologically functioning cells
  • recipient and donor immunologically different
  • recipient immunocompromised
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21
Q

Acute GVHD:

A
  • within 100 days of transplant
  • usually affects skin, liver and GI tract
  • multi-organ involvement - worse prognosis
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22
Q

Chronic GVHD:

A
  • following acute disease or de novo
  • after 100 days post tranplante
  • more varied picture: lung, eye, skin, GI
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23
Q

Signs/symptoms of acute GVHD:

A
  • painful maculopapular rash (often neck, palms and soles) which can progress to erythoderma or toxic epidermal necrolysis
  • jaundice
  • watery and blood diarrhoea
  • persistent n&v
  • culture negative fever
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24
Q

Signs/symptoms of chronic GVHD:

A
  • skin: poikiloderma, scleroderma, vitiligo, lichen planus
  • eye: keratoconjunctivitis sicca, corneal ulcers, scleritis
  • GI: dysphagia, odynophagia, ulceration, ileus
  • lung
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25
Q

Management of GVHD:

A
  • immunosuppression (IV steroids)
  • supportive
  • anti-TNF, mTOR inhibitors and extracorpeal photopheresis
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26
Q

Granulocyte colony stimulant factors:

A
  • used to increase neutrophil count in neutropenic patients
  • secondary to chemotherapy
  • e.g. filgrastim, perfilgrastim
27
Q

Causes of hereditary haemolytic anaemia:

A
  • membrane: hereditary spherocytosis/elliptocytosis
  • metabolism: G6PD deficiency
  • haemoglobinopathies: sickle cell, thalassaemia
28
Q

Causes of acquired haemolytic anaemia:

A 
immune causes:
-automminue: warm/cold Ab
-alloimune: transfusion, haemolytic disease newborn
-drugs: methyldopa, penicillin
non-immune:
-microangiopathic haemolytic anaemia: TTP/HUS, DIC, malignancy, pre-eclampsia
-prosthetic valves
-paroxysmal nocturnal haemoglobinuria
-malaria
-dapsone
29
Q

Haemophilia

A
  • x-linked recessive disorder of coagulation
  • A - factor VIII def
  • B - factor IX def
  • causes haemoarthroses, haematomas, prolonged bleeding
  • prolonged APTT, normal bleeding time, thrombin time and PT
30
Q

What is hereditary angioedema?

A
  • autosomal dominant
  • low plasma C1 inhibitor protein
  • C1INH is serine protease inhibitor
  • uncontrolled release of bradykinin resulting in oedema of tissue
31
Q

Investigations in hereditary angioedema:

A
  • C1-INH low during attack

- low C2 and C4 (C4 most reliable and screening tool)

32
Q

Symptoms of hereditary angioedema:

A
  • attacks may be proceeded by painful macular rash
  • painless, non-pruritic swelling of subcutaneous/submucosal tissue
  • upper airways, skin or abdominal organs (occasionally abdominal pain due to visceral oedema)
  • urticaria not usually feature
33
Q

Management of hereditary angioedema:

A
  • acute: HAE does not respond to adrenaline, antihistamines or glucocorticoids
  • IV C1 inhibitor concentrate, FFP if not available
  • prophylaxis: anabolic steroid Danazol
34
Q

What is hereditary spherocytosis?

A
  • most common hereditary haemolytic anaemia in northern european
  • autosomal dominant defect of RBC cytoskeleton
  • sphere shaped
  • survival reduced as destroyed by spleen
35
Q

Presentation of hereditary spherocytosis:

A
  • failure to thrive
  • jaundice, gallstones
  • splenomegaly
  • aplastic crisis precipitated by parvovirus infection
  • degree of haemolysis variable
  • MCHC elevated
36
Q

Diagnosis of hereditary spherocytosis:

A
  • EMA binding test and cryohaemolysis test

- atypical presentations electrophoresis analysis of erythrocyte membranes

37
Q

Management of acute haemolytic crisis:

A
  • generally supportive

- transfusion if necessary

38
Q

Long term treatment of hereditary spherocytosis:

A
  • folate replacement

- splenectomy

39
Q

Causes and features of hyposplenism:

A
  • splenectomy
  • sickle cell
  • coeliac disease, dermatitis herpetiformis
  • Graves’
  • SLE
  • amyloid

features: Howel-Jolly bodies, siderocytes

40
Q

What is ITP?

A
  • immune mediated reduction in platelet count
  • AB against GPIIb/IIIa or Ib-V-IX complex
  • children have acute thrombocytopenia that may follow infection or vaccination
  • adults more chronic condition
  • more common in older females
41
Q

Presentation of ITP:

A
  • incidentally following routine bloods
  • petechiae, purpura
  • bleeding
  • catastrophic bleeding uncommon
42
Q

Management of ITP:

A
  • first line treatment for ITP is oral prednisone

- pooled normal human Ig (IVIG) may be used

43
Q

What is Evan’s syndrome?

A

ITP associated with AIHA

44
Q

Symptoms of iron deficiency anaemia:

A
  • fatigue, SOB on exertion
  • palpitations
  • pallor
  • nail changes (koilonychia)
  • hair loss
  • atrophic glossitis
  • post-cricoid webs
  • angular stomatitis
45
Q

Investigations for iron deficiency anaemia:

A
  • FBC: hypochromic microcytic anaemia
  • low serum ferritin
  • TIBC high
  • transferrin sats low
  • blodo film: anisopoikilocytosis (RBC different sizes and shapes), target cells, pencil poikilocytes
46
Q

Management of iron deficiency anaemia:

A
  • oral ferrous sulphate: take for 3 months after correction to replenish stores
  • iron rich diet
47
Q

ADR of iron supplementation:

A
  • nausea
  • abdo pain
  • constipation
  • diarrhoea
48
Q

Iron def anaemia vs anaemia of chronic disease:

A 
iron def anaemia:
-serum iron <8
-high TIBC
-low transferrin saturation
-low ferritin 
anaemia of chronic disease:
-serum iron <15
-low TIBC
-low transferrin saturation
-high ferritin
49
Q

What does a decrease in haptoglobin suggest?

A

intravascular haemolysis

50
Q

What does an increase in MCHC suggest?

A
  • hereditary spherocytosis

- autoimmune haemolytic anaemia

51
Q

What does a decrease in MCHC suggest?

A

mircrocytic anaemia e.g. iron deficiency

52
Q

What does lead poisoning cause and what are the features?

A 
-defective ferrochelastase
 and ALA dehydrates function
-absominal pain
-peripheral neuropathy
-fatigue
-constipation
-blue lines on gum margin
53
Q

Investigations with lead poisoning:

A
  • blood lead >10mcg/dL
  • microcytic anaemia
  • basophilic stippling and clover leaf morphology
  • sometimes raised serum and uric delta aminolaevulinic acid (distinguish from AIP)
  • increased urinary coprophorphyrin
  • in children, lead can accumulate in metaphysis of bones
54
Q

Management of lead poisoning:

A
  • dimercaptosuccinic acid (DMSA)
  • D-penicillamine
  • EDTA
  • dimercaprol
55
Q

Macrocytic anaemia causes:

A 
megaloblastic causes:
-vit B12 def
-folate def
normoblastic causes:
-alcohol
-liver disease
-hypothyroidism
-pregnancy
-reticulocytosis
-myelodysplasia
-drugs: cytotoxics
56
Q

What is MGUS?

A

-monoclonal gammopathy of undetermined significance
(benign paraproteinaemia and monoclonal gammopathy)
-eventually develop myeloma

57
Q

Features of MGUS:

A
  • usually asymptomatic
  • no bone pain or increased risk of infections
  • 10-30% demyelinating neuropathy
58
Q

Differentiating features of MGUS from myeloma:

A
  • normal immune function
  • normal beta 2 microglobulin levels
  • lower level of paraproteinaemia than myeloma
  • stable level of paraproteinaemia
  • no clinical features of myeloma e.g. lytic lesions or renal disease
59
Q

Causes of microcytic anaemia:

A
  • iron def anaemia
  • thalassaemia
  • congenital sideroblastic anaemia
  • anaemia of chronic disease
  • lead poisoning
60
Q

What is myelodysplastic syndrome and what are the features:

A
  • acquired neoplastic disorder of haematopoietic stem cells
  • pre-leukaemia may progress to AML
  • more common with age
  • presents with bone marrow failure (anaemia, neutropenia, thrombocytopenia)
61
Q

Myelofibrosis:

A
  • myeloproliferative disorder
  • hyperplasia of abnormal megakaryocytes
  • resultant disease of platelet derived growth factor though to stimulate fibroblasts
  • haematopoiesis in liver and spleen
62
Q

Features of myelofibrosis:

A
  • elderly with anaemia symptoms
  • massive splenomegaly
  • hypermetabolic symptoms: weight loss, night sweats
63
Q

Lab findings of myelofibrosis:

A
  • anaemia
  • high WBC and platelet count early in disease
  • tear drop poikilocytes on blood film
  • unobtainable bone marrow biopsy (dry tap) so trephine biopsy needed
  • high urate and LDH (reflect increased cell turnover)
64
Q

What do bite and blister cells indicate:

A

G6PD deficiency

Haematology (4 decks)

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