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Year 3 > Haematology > Flashcards

Haematology Flashcards

1
Q

Malaria

A

a parasitic infection caused by protozoa of the genus plasmodium, this infection is almost exclusively sen in tropics & subtropics

Notifiable disease in the UK

NB consider malaria in every febrile travel returning from a malaria endemic area in the last year

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2
Q

Protective factors for malaria

A

sickle cell trait
G6PD deficiency
HLA-B53

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3
Q

Species of malaria

A

Plasmodium falciparum

  • commonest type
  • most severe kind

non falciparum (plasmodium vivax/ovale/malarine)

  • plasmodium vivax is most common
  • vivax is usually benign
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4
Q

Presentation of malaria

A

fever

  • often recurring
  • cyclical, occurring every 48-72h

chills, rigors, headache, cough, myalgia, GI upset
jaundice
hepato/splenomegaly

Severe disease (usually P. falciparum)

  • impaired consciousness
  • SOB
  • bleeding
  • fits
  • AKI, ARDS, anaemia
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5
Q

Investigations for malaria

A

Blood smears with Giemsa stain

  • gold standard
  • presence of parasites within RBCs

FBC

  • ↓Hb
  • thrombocytopenia
  • ↑ reticulocytes

Rapid diagnostic tests (RDIs)
-detect parasite antigens

LFTs
-often abnormal

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6
Q

Management of malaria

A

Falciparum:

  • Artemisinin-based combination therapy (ART) or PO quinine if uncomplicated
  • IV quinine in severe disease

Non-falciparum malaria:

  • 1st line = chloroquine
  • 2nd line = ART (1st line if chloroquine resistance)
  • primaquine (as relapse prevention, destroys liver hypnozoites)
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7
Q

Prevention of malaria

A

reduced chance of being bitten

  • misquito nets
  • mosquito spray

Chemoprophylaxis

  • start 1 week befogging entering malarious area
  • chloroquine, proguanil, mefloquine
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8
Q

Hodgkin’s lymphoma

A

malignant tumour of the lymphatic system characterised histologically by the presence of Reed-Sternberg cells (multinucleate giant cells)

Bi-modal age distribution

  • peak age 20-34yrs
  • 2nd peak >70yrs

classical Hodgkin’s lymphoma (most common), especially nodular sclerosing kind

NB lymphocyte deplete kind is rare & carries worst prognosis

Risk factors include immunodeficiency, EBV infection & autoimmune disease

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9
Q

Hodgkin’s lymphoma characteristic histological feature

A

Reed-Sternberg cells (multinucleate giant cells)

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10
Q

Presentation of Hodgkin’s lymphoma

A

painless non tender asymmetrical lymphadenopathy

  • most frequently affects cervical nodes
  • may present as mediastinal mass

B symptoms

  • weight loss, fever, night sweats
  • imply poor prognosis

alcohol induced pain at sites of nodal disease

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11
Q

Investigations for Hodgkin’s lymphoma

A

FBC

  • ↑/↓ WCC
  • ↓Hb
  • eosinophilia

Lymph node biopsy

  • Reed-Sternberg cells
  • Hodgkins cells

CXR

  • mediastinal mass
  • mediastinal lymphadenopathy

ESR (↑), LDH (↑)

CT, Gallium scan, PET-CT

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12
Q

Management of Hodgkin’s lymphoma

A

chemo+radiotherapy
-ABVD or BEACOPP regimes

autologous stem cell transplant

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13
Q

Non-Hodgkin’s lymphoma

A

a heterogenous group of malignancies of the lymphoid system, ~5x more common than Hodgkin’s lymphoma

usually seen in >50y/o pts

Subtypes

  • B-cell lymphomas (~85%)
  • T-cell lymphomas (~15%)

Risk factors include Caucasian origin, EBC infection, FH of lymphoma, immunodeficiency, autoimmune disease

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14
Q

Presentation of Non-Hodgkin’s lymphoma

A

painless lymphadenopathy

  • non tender
  • rubbery
  • asymmetrical

constitutional/B-symptoms
-fever, weight loss, night sweats, lethargy

splenomegaly, hepatomegaly

Extra-nodal disease (more common than in Hodgkins)
-early satiety, GI bleeding, headache, skin lesions, pruritus

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15
Q

Investigations for Non-Hodgkin’s lymphoma

A

excision node biopsy
-investigation of choice

CT chest/abdo/pelvis

FBC (↓Hb), ESR (↑), LDH (↑)
PET-CT

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16
Q

Management of Non-Hodgkin’s lymphoma

A

depends on subtype

generally watch & wait (if low grade), chemo or radiotherapy

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17
Q

Burkitt’s lymphoma

A

high grade non-hodgkins lymphoma, that is rapidly growing & aggressive

usually seen in children, and is strongly associated with EBV infection

characterised by starry-sky appearance on biopsy (lymphocyte sheets interspersed with macrophages containing dead apoptotic tumour cells)

managed with chemo

Strongly associated with tumour lysis syndrome

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18
Q

Acute lymphoblastic leukaemia (ALL)

A

a malignant clonal disease that develops when lymphoid progenitor cells undergo uncontrolled proliferation

most common cancer in children with peak incidence at 2-5yrs
-~80% of leukemias in children)

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19
Q

Presentation of Acute lymphoblastic leukaemia (ALL)

A

sudden onset & rapid progression
fatigue dizziness, palpations, pallor, weakness
bone & joint pain
headache, neck stiffness
fever without obvious infection
bruising, epistaxis, petechiae, ecchymosis
LUQ fullness, early satiety (due to splenomegaly)
testicular enlargement
lymphadenopathy

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20
Q

Investigations for Acute lymphoblastic leukaemia (ALL)

A

FBC

  • ↓ Hb
  • ↓ platelets
  • ↓ neutrophils
  • ↓/↑WCC

Blood smear
-leukaemic lymphoblasts

Bone marrow aspiration &biopsy
-≥20% blast cells

clotting (may show DIC), LDH (↑), uric acid (↑)
LFTs, U&Es,

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21
Q

Management of Acute lymphoblastic leukaemia (ALL)

A

Induction (to restore normal haematopoesis)
-corticosteroids + vincristine/cyclophosphamide/doxorubicin

Maintenance
-mercaptopurine + methotrexate

CNS prophylaxis
-intrathecal methotrexate

Stem cell transplant

NB relapses after treatment have very poor prognosis

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22
Q

Acute myeloid leukaemia (AML)

A

the malignant clinical expansion of myeloid blasts in the bone marrow, peripheral blood or extra medullary tissue

may be primary disease or secondary transformation of other lymphoproliferative disorders

Most common acute leukaemia in adults usually seen age 65yrs

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23
Q

Risk factors for Acute myeloid leukaemia (AML)

A

aplastic anaemia
myelofibrosis
paroxysmal nocturnal haemoglobulinaemia
polycythaemia rubera vera

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24
Q

Presentation of Acute myeloid leukaemia (AML)

A 
anaemia (pallor, fatigue, weakness)
fever
petechiae
purpura
ecchymosis 
epistaxis
frequent infections 
hepatosplenomegaly 
leukaemia cutis (nodular skin lesions, gray-blue/purple)
gingival hypertrophy
gingivitis 
bleeding gums
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25
Q

Investigations for Acute myeloid leukaemia (AML)

A

FBC

  • ↓ Hb
  • ↓ platelets
  • ↓ neutrophils

clotting

  • DIC common
  • ↑PT, ↑aPTT, ↓fibrinogen

LDH (↑)

Bone marrow biopsy
-≥20% blast cells

LFTs, U&Es, blood film/smear

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26
Q

Management of Acute myeloid leukaemia (AML)

A

induction
-cytarabine + daunorubicin

consolidation
-cytarabine / daunorubicin / mitoxantrone

Stem cell transplant

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27
Q

Acute promyelotic anaemia (APL)

A

presents younger than other AMLs (~25y/o)

often has DIC/thrombocytopenia at presentation

has Auer-rods on histology

treated with arsenic trioxide (ATO) or all-trans relinoic acid (ATRA)

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28
Q

Epidemiology of leukaemias

A

ALL = most common leukaemia in children

CLL = most common leukaemia in adults
-often has lymphadenopathy

AML = most common acute leukaemia in adults

CML= only ~15% of adult leukaemias

  • rarely has lymphadenopathy
  • associated with Philadelphia chromosome
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29
Q

Chronic lymphocytic anaemia (CLL)

A

malignant monoclonal expansion of B lymphocytes with accumulation of abnormal lymphocytes in blood / bone marrow / spleen / lymphnodes / liver

most common leukaemia in adults, usually diagnosed ~70y/o

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30
Q

Presentation of Chronic lymphocytic anaemia (CLL)

A

variable presentation with insidious onset, may be symptomatic & incidentally found on blood test

symmetrically enlarged painless lymph nodes (NB CML rarely has lymphadenopathy)
susceptibility to infection 
anorexia, weight loss
hepatosplenomegaly, abdo discomfort 
brusing, petichae
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31
Q

Investigations for Chronic lymphocytic anaemia (CLL)

A

FBC

  • ↓ Hb
  • ↑↑ WCC
  • ↓ platelets

Blood film
-smear cells & smudge cells

Immunophenotyping
-key investigations

Bone marrow aspirate / lymph node biopsy

T53 gene test
-done before treatment

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32
Q

Management of Chronic lymphocytic anaemia (CLL)

A

chemotherapy

stem cell transplant

if CD20 +ve
-rituximab

TP53 targeted therapy
-ibrutinib

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33
Q

Chronic myeloid anaemia (CML)

A

a myeloproliferative disorder of pluripotent haemopoietic stem cells affecting one or all cell lines

usually seen in adults age 60-65 yrs

associated with Philadelphia chromosome in ~95% of pts

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34
Q

Presentation of Chronic myeloid anaemia (CML)

A

often asymptomatic & incidentally found on blood test, otherwise symptoms have insidious onset

fatigue, night sweats, weight loss
abdo fullness, abdo distension
LUQ pain
splenomegaly & hepatomegaly
easy bruising

NB CML only rarely has enlarged lymph nodes, but they are common in CLL = key differentiating factor

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35
Q

Investigations for Chronic myeloid anaemia (CML)

A

FBC

  • ↓ Hb
  • ↑ WCC (>100x10^9/L)
  • ↓/↑ platelets

Blood film

  • all stages of maturation seen
  • looks like bone marrow aspire

LDH (↑)
leukocyte alkaline phosphate (↓)

bone marrow aspirate & biopsy
cytogenetics
Fluorescent in situ hybridisation (FISH)
quantitive reverse transcriptase PCR

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36
Q

Myeloma/multiple myeloma

A

a plasma cell dyscrasia characterised by terminally differentiated plasma cells, infiltration of the bone marrow by plasma cells and presence of a monoclonal immunoglobulin in the serum and/or urine

classified by immunoglobulin type (IgG most common)

2nd most common haematological malignancy

median age of presentation is 70yrs

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37
Q

Epidemiology of Myeloma/multiple myeloma

A

classified by immunoglobulin type
-IgG most common type

2nd most common haematological malignancy

median age of presentation is 70yrs

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38
Q

Epidemiology of Myeloma/multiple myeloma

A

classified by immunoglobulin type
-IgG most common type

2nd most common haematological malignancy

median age of presentation is 70yrs

more common in men & afro-carribbeans

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39
Q

Presentation of Myeloma/multiple myeloma

A

bone pain
-particularly back pain

pathological fractures
lethargy, anorexia, night sweats, weight loss
↑ Ca2+ (constipation, nausea, confusion)
dehydration, thirst, pallor
easy bruising / bleeding
↑susceptibility to infection & repeated infections

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40
Q

Presentation of Myeloma/multiple myeloma

A

bone pain
-particularly back pain

pathological fractures
lethargy, anorexia, night sweats, weight loss
↑ Ca2+ (constipation, nausea, confusion)
Renal damage (dehydration, thirst, pallor)
easy bruising / bleeding
↑susceptibility to infection & repeated infections

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41
Q

Investigations for Myeloma/multiple myeloma

A

FBC

  • ↓ Hb
  • ↓ platelets

U&Es

  • ↑ urea
  • ↑ creatinine

serum / urine electrophoresis

  • ↑ monoclonal IgA / IgG
  • Bence-Joyce proteins in urine

Bone marrow aspiration & biopsy
-monoclonal plasma cell infiltration

X-ray

  • rain drop skill
  • randomly placed dark spots due to bone lysis

Ca2+ (↑)
Whole body MRI

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42
Q

Diagnostic criteria for Myeloma/multiple myeloma

A

Symptomatic multiple myeloma is defined at diagnosis by the presence of all 3 of the following:

  • Monoclonal plasma cells in the bone marrow >10%
  • Monoclonal protein within serum / urine (as determined by electrophoresis)
  • Evidence of end-organ damage e.g. hypercalcaemia, elevated creatinine, anaemia or lytic bone lesions/fractures
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43
Q

Management of Myeloma/multiple myeloma

A

currently deemed incurable

elderly pts/not suitable for transplant
-Thalidomide + an Alkylating agent + Dexamethasone

suitable for transplant

  • Bortezomib + dexamthasone
  • followed by stem cell transplant

NB pts should be mounted every 3 months with blood tests & electrophoresis

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44
Q

Polycythaemia

A

refers to an increased number of Red blood cells in the body

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45
Q

Aetiology of Polycythaemia

A

Relative causes:
-dehydration, stress (gaissbock syndrome)

Primary:
-polycythaemia vera

Secondary

  • COPD
  • high altitude
  • OSA
  • uterine fibroids (cause ↑ EPO)
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46
Q

Polycythaemia vera

A

myeloproliferative disorder characterised by erythropoietic independent rise in erythrocyte & platelets count

associated with a mutation in the JAK2 gene

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47
Q

Presentation of Polycythaemia vera

A

maybe be asymptomatic or incidentally found on blood tests

hyper viscosity syndrome
-mucosal bleeding, visual changes, neurological symptoms (dizziness, headache, tinnitus)

Pruritus
-typically after contact with warm water

plethoric appearance

  • flushed face with purple hue
  • cyanotic lips

erythromyalgia
-warmth, pain, erythema, infarction of distal extremities

thrombosis
-e.g. stroke, MI, PE, DVT, thrombophlebitis

haemorrhage
-from gums, easy bruising, GI bleeds

splenomegaly, hypertension

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48
Q

Investigations for Polycythaemia vera

A

FBC

  • ↑ Hb/RBC
  • ↑ haematocrit
  • ↑ platelets
  • ↑ WCC
  • ↓ MCV
Jak2 gene mutation (+ve)
LDH (↑)
erythropoietin (↓)
ferritin (often ↓)
LFTs

NB in secondary causes of polycythaemia there is only an isolated ↑ Hb/RBC and no ↑ platelets

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49
Q

Management of Polycythaemia vera

A

Venesection (1st line)

Myelosuppression (2nd line)

  • e.g. with hydroxyurea or phosphorus-32
  • ↑ risk of secondary leukaemia

JAK2 inhibitors
-e.g. ruxolitinib

Aspirin

  • low dose
  • to decrease risk of thromboembolic events

NB ~5% of pts progress to myelofibrosis or acute leukaemia (↑ risk of this with myelosuppression therapy)

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50
Q

Neutropenia

A

refers ti a low neutrophil count i.e. <1.5x10^9 (normal range (2.0-7.5 x10^9)

important to recognise as it predisposes to infection

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51
Q

Aetiology of neutropenia

A

Viral
-HIV, EBV, hepatitis

Medications:
-cytotoxics/chemotherapy, carbimazole, clozapine

Benign ethnic neutropenia

  • seen in black africans, afro-carribbean individuals
  • no treatment required

Haemtological malignacies

  • aplastic anaemia
  • myelodysplastic malignancy

SLE, RA, haemodialysis

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52
Q

Neutropenic sepsis / Febrile neutropenia

A

oral temp ≥38.5°C / ≥2 consecutive readings ≥38.0°C with a neutrophil count <0.5 x10^9

often associated with cancer therapy

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53
Q

Investigations for Neutropenic sepsis / Febrile neutropenia

A

Only investigate after starting Abx

FBC (↓ neutrophils)
blood cultures
CXR
U&Es
LFTs
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54
Q

Presentation of Neutropenic sepsis / Febrile neutropenia

A

fever
tachycardia
hypotension

recent chemo therapy

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55
Q

Thrombocytopenia

A

a platelets count below the normal range i.e. <150 x10^9/L

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56
Q

Aetiology of thrombocytopenia

A

Most severe

  • immune thrombocytopenia (ITP)
  • Disseminated intravascular coagulation (DIC)
  • thrombotic thrombocytopenic purpura (TTP)
  • haematological malignancies

Others

  • heparin induced thrombocytopenia (HIT)
  • HELLP syndrome
  • Antiphospholipid syndrome
  • Medication (quinine, aspirin, thiazides, sulphonamides)
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57
Q

Pseudo thrombocytopenia

A

can be caused the use of EDTA as an anticoagulant

has low platelet count but without suggestive symptoms

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58
Q

Investigations for thrombocytopenia

A 
FBC
Blood film
coagulation screen
U&Es
LFTs
consider bone marrow biopsy

NB rapidly falling platelet count even if within normal range is worrying

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59
Q

Management of thrombocytopenia

A

treat any significant bleeds
consult haematology
consider replacing platelets
-e.g. if anticipated need for urgent surgery, significant bleeding or neurological symptoms

NB rapidly falling platelet count even if within normal range is worrying

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60
Q

Presentation of thrombocytopenia

A

epistaxis
-usually excessive, prolonged, frequent

bleeding gums
↑ bleeding from tooth extractions 
spontaneous bruising
menorrhagia
PPH
excessive bleeding after surgery
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61
Q

Immune thrombocytopenia (ITP)

A

An autoimmune disorder with autoantibodies to platelets causing thrombocytopenia, which may be triggered by viral infections

NB in children it is usually acute, following a viral infection or vaccination but is generally self limiting (lasts 1-2 weeks)

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62
Q

Presentation of Immune thrombocytopenia (ITP)

A

often asymptomatic & incidentally picked up on FBC

petechiae, bruising, epistaxis
absent splenomegaly
major haemorrhages e.g. intracranial bleeds are uncommon

NB presence of splenomegaly should make you consider other diagnosis

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63
Q

Investigations & Management of Immune thrombocytopenia (ITP)

A

FBC shows platelet count <100 x10^9/L & blood film shows normal platelets

1st line
-oral prednisolone

if active bleed or urgent need to ↑ platelets use IVIG (human immunoglobulin

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64
Q

Investigations & Management of Immune thrombocytopenia (ITP)

A

FBC shows platelet count <100 x10^9/L & blood film shows normal platelets

1st line
-oral prednisolone

if active bleed or urgent need to ↑ platelets use IVIG (human immunoglobulin)

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65
Q

Thrombotic thrombocytopenic purpura (TTP)

A

thrombotic microangiopathic where micro thrombi consisting primarily of platelets occlude microvasculature due to deficiency of ADAMTS13

should be treated as an emergency

most common in adults usually aged ~40 yrs

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66
Q

Investigations for Thrombotic thrombocytopenic purpura (TTP)

A

FBC

  • ↓ platelets
  • ↓ Hb
  • ↑ reticulocytes
  • ↓ haptoglobin

U&Es

  • ↑ urea
  • ↑ creatinine

urinalysis
-proteinuria ± haematuria

Blood film
-schisticytes (erythrocyte fragements)

LDH (↑↑)
direct coombs test (-ve)

pretreatment ADAMTS13 activity levels & ADAMTS13

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67
Q

Presentation fo Thrombotic thrombocytopenic purpura (TTP)

A

Fever

Fluctuating neurological signs

  • delirium, altered mental status
  • stroke, seizures
  • headache, dizziness

Microangiopathic haemolytic anemia

  • fatigue, dyspnoea, pallor
  • jaundice, myalgia, arthralgia

Impaired renal function:
-haematuria, oliguria, proteinuria

Purpura
-non palpable small purpuric spots or petechiae

68
Q

Direct & indirect Coombs test

A

Direct
-checks if antibodies are stuck to RBC surface

In-driect
-checks for free antibodies in serum

help to rule out haemolytic anaemia

69
Q

Management of Thrombotic thrombocytopenic purpura (TTP)

A

Plasmaphoresis
-IV plasma exchange

Steroids
-high dose prednisolone

Rituximab
-if severe

NB is treated as an emergency

70
Q

Features of Thrombotic thrombocytopenic purpura (TTP)

A

Pentad of

  • fever
  • fluctuating neurological signs
  • microangiopathic haemolytic anemia
  • thrombocytopenia
  • renal failure
71
Q

Von Willebrand disease (vWD)

A

The most common hereditary coagulopathy due to the deficiency or abnormal function of von Willebrand factor (vWF)

characteristically behaves like a platelet disorder as vWF promotes platelet adhesion & platelet plug formation + vWF binds factor VIII preventing its clearance

inherited in an autosomal dominant fashion

72
Q

Von Willebrand disease (vWD)

A

The most common hereditary coagulopathy due to the deficiency or abnormal function of von Willebrand factor (vWF)

characteristically behaves like a platelet disorder as vWF promotes platelet adhesion & platelet plug formation + vWF binds factor VIII preventing its clearance

inherited mostly in an autosomal dominant fashion

73
Q

Presentation of Von Willebrand disease (vWD)

A

often asymptomatic

bleeding tendency from mucosa

  • epistaxis
  • menorrhagia (common in women)

prolonged bleeding from minor injuries / post surgery

NB haemoarthrosis are rare

74
Q

Investigations for Von Willebrand disease (vWD)

A

Coagulation profile

  • PT (normal)
  • aPTT (may be ↑)
  • bleeding time (↑)
FBC (normal)
Factor VIII (may be ↓)
vWF antigen (↓)
ristocetin cofactor assay (defective platelet aggregation)
75
Q

Management of Von Willebrand disease (vWD)

A

TXA for mild bleeding

Desmopressin (DDAVP)
-stimulates vWF release

vWF containing factor VIII concentrate
-for severe bleeding or surgical prophylaxis

76
Q

Thrombocytosis

A

a platelets count >450 x10^9/L

Aetiology

  • reactive (platelets = acute phase reactant_
    • e.g. stress, surgery, infection
  • malignancy
    • e.g. CML
  • hyposplenism / post splenectomy
  • essential thrombocytosis
77
Q

Essential thrombocytosis (primary thrombocytosis)

A

most common myeloproliferative neoplasm due to sustained dysregulates megakaryocytic proliferation, usually seen in pts age 50-60yrs

78
Q

Essential thrombocytosis presentation

A

commonly asymptomatic

erythromelalgia
-burning pain & erythema of hands and feet

thrombotic events (stroke/TIA/retinal artery occlusion)

Vasomotor symptoms
-headache, visual disturbance, ocular migraines

splenomegaly/hepatomegaly

bleeding
-primarily GI, simulates duodenal ulcer following duodenal arcade thrombosis

79
Q

Investigations for Essential thrombocytosis

A

FBC
-platelets >600 x10^9

Blood smear
-immature precursor cells e.g. myelocytes

Bone marrow aspirate

  • hypercellularity
  • megakaryocyte hyperplasia

LDH (↑)
CRP/ESR (normal)

80
Q

Management of Essential thrombocytosis

A

Hydroxyurea (hydroxycarbamide)
-to ↓ platelet count

low dose aspirin
-↓ thrombotic risk

NB Interferon-alpha is preferred in young pts & pregnant women over hydroxyurea

81
Q

Haemophilia

A

an X-linked recessive inherited bleeding disorder resulting in the deficiency of coagulation factors

82
Q

Genetics of haemophilia

A

X-linked recessive inheritance

hence only seen in males

83
Q

Types of haemophilia

A

Haemophilia A

  • deficiency in clotting factor VIII
  • accounts for ~80% of cases

Haemophilia B

  • deficiency in clotting factor IX
  • accounts for ~20% of cases

Haemophilia C

  • deficiency of factor XI
  • very rare
  • seen in Ashkenazi jews
84
Q

Presentation of haemophilia

A

repeated haemarthrosis

  • especially of knees
  • can lead to haemophilic arthropathy

frequent brusing & haematomas
oral mucosal bleeding, epistaxis, excessive bleeding after minor procedures
musculoskeletal bleeding/haematomas

NB female carriers may present with mild symptoms

85
Q

Investigations for haemophilia

A

Coagulation screen

  • aPTT ↑
  • PT normal
  • bleeding time normal

Factor VIII/IX assay
- ↓ / absent

joint x-rays
-may show degenerative joint disease

86
Q

Management of haemophilia A

A

prophylactic factor VIII

if acute bleeding
-FFP/recombinant factor VIII

87
Q

Management of haemophilia B

A

if acute bleeding

  • 1st line = recombinant factor IX
  • 2nd line = PCC or plasma derived factor IX
88
Q

Disseminated intravascular coagulation (DIC)

A

a syndrome characterised by systemic activation of the coagulation cascade resulting in the formation of intravascular thrombi & the depletion of platelets + coagulation factors

89
Q

Aetiology of Disseminated intravascular coagulation (DIC)

A

-Sepsis
-trauma (trauma induced coagulopathy)
-malignancy
-obstetric complications (e.g. HELLP syndrome, amniotic fluid embolus)
-organ failure (e.g. pancreatitis, fulminant hepatitis)
transfusion reaction

90
Q

Presentation of Disseminated intravascular coagulation (DIC)

A 
oliguria, hypotension, tachycardia
large bruises 
spontaneous bleeding at venipuncture sites / soft palate / site of trauma 
massive haemorrhage 
organ failure
-ARDS
-PE
-purpura fulminans (DIC + extensive skin necrosis)
-Waterhouse-Friedirchsen syndrome
91
Q

Investigations for Disseminated intravascular coagulation (DIC)

A

Coagulation screen

  • PT ↑
  • aPTT ↑
  • bleeding time ↑
  • Fibrinogen ↓

FBC
-platelets ↓

D-Dimer ↑

92
Q

Management of Disseminated intravascular coagulation (DIC)

A

treat underlying cause
blood products as needed

anticoagulation

  • if hypercoaguability is the main problem
  • therapeutic dose LMWH/UFH

do not use antifibrinolytics e.g. TXA in pts with DIC

93
Q

Thalassaemia

A

a group of hereditary haemoglobin disorders characterised by mutations in the alpha or beta global chains

inherited in autosomal recessive pattern

94
Q

Alpha-Thalassaemia

A

deficient production of α-globin

2 α genes present on each chromosome 16

95
Q

Types of Alpha-Thalassaemia

A

Normal (α,α / α,α)

a+ heterozygous i.e. silent carrier (α,- / α,α)

  • i.e. one allele affected
  • borderline Hb & MCV
  • pt asymptomatic

a+ homozygous (α,- / α,- or α,α / -,-)

  • i.e. two alleles affected
  • slightly low Hb, slightly low MCV & MCH
  • pt asymptomatic

HbH disease (α,- / -,-)

  • i.e. three alleles affected
  • microcytic hypo chromic anaemia
  • splenomegaly & skeletal deformities

α Thalassaemia major / Hb Barts (-,- / -,-)

  • i.e. all four alleles affected
  • hydrops fettles & intrauterine death
  • incompatible with life
  • Hb Barts = γ chain tetramere
96
Q

Investigations for Alpha-Thalassaemia

A

FBC

  • Hb ↓
  • MCV ↓
  • MCH ↓
  • reticulocytes ↑
  • RBC count ↑

Iron studies

  • iron ↑
  • ferritin ↑

Blood film + supra vital stain
-Heinz bodies (HbH inclusion bodies of β-chain tetrameres)

97
Q

Investigations for Alpha-Thalassaemia

A

FBC

  • Hb ↓
  • MCV ↓
  • MCH ↓
  • reticulocytes ↑
  • RBC count ↑

Iron studies

  • iron ↑
  • ferritin ↑

Blood film + supra vital stain
-Heinz bodies (HbH inclusion bodies of β-chain tetrameres)

98
Q

Management of Alpha-Thalassaemia

A

no management for silent carriers or asymptomatic pts

folic acid supplements for most pts

Blood transfusions
-especially in HbH disease

Splenectomy if hyposplenism

Iron chelation
-e.g. desferrioxamine to prevent iron overload

99
Q

Beta-Thalassaemia

A

deficient production of β globes

one β global gene on each chromosome 11

100
Q

Types of Beta-Thalassaemia

A

Normal (β / β)

β Thalassaemia trait (β / -)

  • one functional allele, one non functional
  • HbA2 >4%
  • slight anaemia, ↓MCV, ↓MCH
  • asymptomatic

β Thalassaemia major (- / -)

  • absence of β chains
  • HbF >90%
  • severe haemolytic anemia ↓↓MCV, ↓↓MCH
  • hepatosplenomegaly & skeletal deformities
  • chronic diffusion dependency
  • presents in 1st year of life with failure to thrive
101
Q

Investigations for Beta-Thalassaemia

A

FBC

  • Hb ↓
  • MCV ↓
  • MCH ↓
  • reticulocytes ↑

Blood film + supra vital stain

  • Target cells
  • Teardrop cells

Haemoglobin analysis

  • minimal/no HbA
  • HbF ↑
  • HbA2 ↑

LDH (↑)

102
Q

Management of Beta-Thalassaemia

A

Repeated transfusions
-risk of iron overload

iron chelation
-e.g. with IV desferrioxamine or PO deferrasirox

Folic acid supplements as required

consider splenectomy

103
Q

Sickle cell anaemia

A

Autosomal recessive single gene defect in the β chain of haemoglobin leading to production of sickle haemoglobin (HbS)

more common in people of african descent

most common form of intrinsic haemolytic anaemia

104
Q

Sickle cell trait

A

i.e. heterozygous individuals carrying HBSA

people with sickle cell trait are generally asymptomatic but may present with gross haematuria due to renal papillary necrosis

protective against malaria

105
Q

Presentation of sickle cell disease

A

symptoms begin age 3-6 months as HbF levels drop

pallor, jaundice, lethargy, growth restriction, weakness
failure to thrive
splenomegaly

further presentations with crisis

106
Q

Vaso-occlusive crisis

A

Most common type of sickle cell crisis
due to obstruction of microcirculation by sickle RBCs leading to ischaemia

precipitated by cold, infection, dehydration, hypoxia etc.

causes severe pain, swollen joints, dactylitis, priapism (in men)
may affect major organs e.g. stroke if brain, bowel ischaemia if mesentery, renal papillary necrosis (loin pain &haematuria)

107
Q

Acute chest syndrome

A

a type of sickle cell crisis
essentially a vast-occlusive crisis of the lung vasculature

presents with dyspnoea, hypoxia, chest pain, tachypnoea & new infiltrates on CXR

symptoms + new infiltrates = diagnostic

108
Q

Aplastic crisis

A

temporary cessation of erythropoiesis leading to severe anaemia in sickle cell pts

usually due to parvovirus B19 infections

leads to rapid ↓Hb (~1 week), due to bone marrow suppression the reticulocyte count is ↓

FBC (↓Hb, ↓reticulocytes)

requires transfusions

109
Q

Sequestration crisis

A

mainly seen in babies/young children, sickling in organs e.g. spleen/lungs cause blood pooling and worsening of anaemia

presents with splenomegaly, LUQ pain, shock (tachycardia, hypotension)

FBC (↓Hb, ↑reticulocytes)

NB recurrent spleen sequestration is an indication for splenectomy

110
Q

Hyperhaemolytic crisis

A

rare sickle cell crisis

↓Hb due to ↑ rate of haemolysis

111
Q

Investigations for sickle cell disease

A

FBC

  • Hb ↓
  • reticulocytes ↑ (if ↓ indicates aplastic crisis)

Blood smear

  • presence of sickle cells
  • Howell-Jolly bodies
  • nucleated RBCs
  • target cells

Haemoglobin electrophoresis

  • gold standard
  • no HbA
  • ~90% HbSS
  • ~10% HbF

U&Es, Lung function tests, LFTs

112
Q

Screening for sickle cell disease

A

heel prick blood spot test on day 3-10 after birth
OR
pre-conceptual screening in high risk groups

113
Q

Management of sickle cell disease

A

Crisis

  • high dose analgesia e.g. opiates
  • O2, IV fluids
  • blood transfusions / exchange transfusion
  • Abx if signs of infection

folic acid supplements
oral penicillin prophylaxis from diagnosis
unconjugated pneumococcal vaccine from age 2yrs
-repeat every 5 years

exchange transfusions

  • as required
  • should be done if a stroke occurs

hydroxycarbamide (hydroxyurea)

  • to ↑ HbF levels
  • helps ↓ frequency of crisis

NB most pts have crisis plan which includes analgesic regiments, this should be followed, these pts are often deemed to be opiate seeking and left in pain

114
Q

Glucose-6-phosphate dehydrogenase (G6PD) deficiency

A

most common RBC enzyme defect leading to ↑ susceptibility to oxidative stress

X-linked recessive inheritance i.e. male only

usually seen in mediterranean & african people

generally managed by avoiding triggers for crisis & blood transfusion if necessary

115
Q

Presentation of Glucose-6-phosphate dehydrogenase (G6PD) deficiency

A

often asymptomatic

recurring haemolytic crisis
-sudden onset back/abdo pain, jaundice, dark urine, transient splenomegaly, pallor

precipitants include fava beans. chlorquine, isoniazid, NSAIDs, sylph-group drugs

116
Q

Investigations for Glucose-6-phosphate dehydrogenase (G6PD) deficiency

A

FBC

  • Hb ↓
  • reticulocytes ↑

Urinalysis
-haemoglobinuria

Blood smear
-Heinz bodies & bite cells

G6PD enzyme assay
-3 month after acute episodes

Indirect bilirubin (↑)
LDH (↑)
117
Q

Hereditary spherocytosis

A

hereditary abnormality of RBCs caused by defects in structural membrane proteins leading to ↓ lifespan of RBCs

inherited in autosomal dominant fashion

most common cause of hereditary haemolytic anaemia in people of northern european descent

118
Q

Presentation of Hereditary spherocytosis

A 
failure to thrive
jaundice 
splenomegaly
LUQ pain
black pigmented gallstones
pallor 
anaemia
119
Q

Investigations for Hereditary spherocytosis

A

FBC

  • Hb ↓
  • reticulocytes ↑

Blood smear
-spherocytes

EMA binding test
-↓ binding of dye to RBC membranes

Unconjugated bilirubin (↑)
LDH (↑)
direct antiglobulin test (-ve)

NB direct antiglobulin test is +ve in other haemolytic anaemias

120
Q

Management of Hereditary spherocytosis

A

folate replacement
splenectomy
transfusions if necessary

121
Q

Haemolytic anemia

A

a group of disorders that result in premature destruction of RBCs leading to a normocytic anaemia

Types:

  • Intrinsic: due to abnormalities in the RBCs
  • Extrinsic: due to external causes e.g. mechanical damage or immune mediated

Mechanism

  • Intravascular: due to complement fixation, trauma or other extrinsic factors e.g. G6PD/TTP/DIC/ABO mismatch
  • Extravascular: most common type, RBCs removed by phagocytic system due to intrinsic defects e.g. spherocytosis
122
Q

Aetiology of Haemolytic anemia

A

Hereditary
-G6PD, spherocytosis, sickle cell anaemia

Acquired immune:
-autoimmune haemolytic anaemia, transfusion reactions, haemolytic disease of the new born, drugs e.g. penicillin

Acquired non immune:
-DIC, TTP, HUS, malignancy, pre-eclampsia, prosthetic heart valves, paroxysmal nocturnal haemoglobinuria

123
Q

Presentation of Haemolytic anemia

A

may be sudden with tachycardia, dyspnoea, angina, weakness and mild jaundice

gallstones (due to ↑ bilirubin)

haematuria (if intravascular)

124
Q

Investigations for Haemolytic anemia

A

FBC

  • Hb ↓
  • Reticulocytes ↑
  • MCV/MCH normal

direct antiglobulin test (direct coombs test)

  • if +ve suggests immune aetiology
  • if -ve non immune aetiology

blood smear

  • abnormal forms
  • fragments

LDH (↑)
haptoglobin (↓)
bilirubin (↑)
urinalysis (haematuria)

125
Q

Haemolytic uraemia syndrome (HUS)

A

Triad of AKI, microangiopathic haemolytic anaemia (combs -ve) and thrombocytopenia

generally seen in young children, ~90% of cases are due to shiga-toxin producing E.coli (0157:H7)

presents with diarrhoea illness preceding onset of thrombocytopenia, microangiopathic haemolytic anaemia and AKI

Investigations include Hb ↓, haptoglobin ↓, bilirubin ↑, LDH ↑, reticulocytes ↑, platelets ↓, urea ↑, creatinine ↑ & ↑ schistocytes

management is supportive (IV fluids, blood transfusions), with consideration for plasma exchange or eculizumab

126
Q

Aplastic anaemia

A

a condition defined by pancytopenia with hypo cellular bone marrow and absence of abnormal cells

diagnosis requires ≥2 of

  • Hb <100g/L
  • platelets <50x10^9 /L
  • neutrophil count <1.5x10^9 /L
127
Q

Aetiology of Aplastic anaemia

A

~75% of cases are idiopathic

congenital e.g. Flaconi syndrome, drugs e.g. cytotoxics/phenytoin/sulphonamides, radiation, parovirus

128
Q

Presentation of Aplastic anaemia

A

fatigue, malaise, pallor, dyspnoea
ankle oedema
purpura, petechiae
mucosal bleeding, retinal haemorrhage

no lymphadenopathy
no hepatosplenomegaly

129
Q

Investigations for Aplastic anaemia

A

FBC

  • Hb <100g/L
  • platelets <50x10^9 /L
  • neutrophil count <1.5x10^9 /L
  • reticulocytes ↓
  • MCV normal

Bone marrow biopsy

  • hypocellular bone marrow
  • no abnormal cells
130
Q

Management of Aplastic anaemia

A

removal of underlying causes e.g. if drug induced

haemopoietic stem cell transplant (HSCT)

immunsuppression e.g. ATG + ciclosporin

supportive care including blood/platelet transfusion

131
Q

Normocytic anaemia

A

a low level of haemoglobin with a normal MCV

Mechanism includes ↓blood volume and/or ↓erythropoiesis

132
Q

Aetiology of Normocytic anaemia

A

Haemolytic anaemias
-sickle cell, HUS, G6PD, hereditary spherocytosis, pyruvate kinase deficiency, TTP, DIC, malaria, mechanical destruction

Aplastic anaemia
-idiopathic, Falconi syndomre

Acute blood loss

anaemia of chronic disease

anaemia of CKD (falls under anaemia of chronic disease)

133
Q

Aetiology of Normocytic anaemia

A

Haemolytic anaemias
-sickle cell, HUS, G6PD, hereditary spherocytosis, pyruvate kinase deficiency, TTP, DIC, malaria, mechanical destruction

Aplastic anaemia
-idiopathic, Falconi syndomre

Acute blood loss

anaemia of chronic disease (most common normocytic anaemia)

anaemia of CKD (falls under anaemia of chronic disease)

134
Q

Anaemia of chronic disease

A

anaemia secondary to chronic inflammation, 2nd most common anaemia overall

aetiology include chronic infections e.g. TB, malignancy, inflammatory conditions e.g. RA/SLE

Investigations include FBC (normocytic, normochromic anaemia), serum iron (↓), total iron binding capacity (↓), ferritin (↑), reticulocyte count (↓), ESR/CRP (↑)

management include treatment of underlying cause, blood transfusion or use of recombinant human erythropoietin/NESP e.g. darbepoetin e.g. in CKD

135
Q

Microcytic anaemia

A

a condition with ↓Hb & ↓MCV due to insufficient Hb production

Aetiology

  • iron deficiency anaemia (most common)
  • lead poisoning
  • late phase anaemia of chronic disease
  • Thalassaemia
  • sideroblastic anaemia
136
Q

Iron deficiency anaemia (IDA)

A

most common type of anaemia

due to a deficiency in iron

137
Q

Aetiology of Iron deficiency anaemia (IDA)

A

excessive blood loss e.g. menorrhagia, GI bleeding
dietary inadequacy (e.g. vegans/vegetarians)
malabsorption (e.g. coeliac disease, IBD)
increased demand (e.g., pregnancy)

NB GI bleeding is the most common cause in men & post menopausal women and should prompt consideration of colon cancer

138
Q

Presentation of Iron deficiency anaemia (IDA)

A

often found incidentally

fatigue, SOB on exertion, pallor, palpitations
koilonychia (spoon nails)
atrophic glossitis
angular stomatitis 
changes in hair / hair loss
brittle nails 
angular chelitis

dysphagia due to oesophageal webs (Plummer-Vinson syndrome)

139
Q

Investigations for Iron deficiency anaemia (IDA)

A

FBC

  • microcytic hypo chromic anaemia
  • reticulocytes ↓

iron studies

  • serum iron ↓
  • total iron binding capacity ↑
  • transferrin ↑
  • transferrin saturation ↓
  • ferritin ↓

Blood film

  • anisopoikilocytosis (red blood cells of different sizes and shapes)
  • target cells (hypo chromic RBCs)
  • ‘pencil’ poikilocytes
140
Q

Management of Iron deficiency anaemia (IDA)

A

Treat underlying cause

PO ferrous sulfate/ferrous salts
-side effects include constipation, dark stools, nausea, diarrhoea, abdo pain

Parenteral iron supplements are reserved for severe cases

Iron rich diet
-e.g. dark green leafy veg, meat, iron fortified bread

NB consider urgent referral if pt with new IDA age >60yrs or <50yrs presenting with rectal bleeding (?colon cancer)

141
Q

Macrocytic anaemia

A

a condition with ↓Hb & ↑MCV due to insufficent nucleus maturation reactive to cytoplasmic expansion due ti defective DNA synthesis or defective DNA repair

Aetiology

  • Megaloblastic anaemia (characterised by hyperhsegmented neutrophils)
    • Vit B12 deficiency
    • Folate deficiency
  • normoblastic anaemia
    • alcohol abuse, liver disease, pregnancy, myelodysplasia, hypothyroidism
142
Q

Pernicious anaemia

A

autoimmune disorder causing Vit B12 deficiency due to autoantibodies against intrinsic factor* ± gastric parietal cells

accounts for ~80% of megaloblastic anaemias

NB ↑ risk of gastric cancer

143
Q

Presentation of Pernicious anaemia

A

fatigue, lethargy, palpitations, faintness, dyspnoea
pallor + jaundice (gives lemon twinge)

peripheral neuropathy
-typical symmetrical affecting arms & legs

subacute combined degeneration of the spinal cord

  • progressive weakness, ataxia, parasthesia
  • can progress to spasticity, paraplegia

neuropsychiatric features
-memory loss, poor concentration, confusion, depression, irritability

144
Q

Investigations for Pernicious anaemia

A

FBC

  • Hb↓
  • MCV↑

Blood smear

  • hypersegmented neutrophils
  • megakaryocytes

Serum Vit B12 (↓)
Folate (normal or ↓)
anti intrinsic factor antibodies (+ve)
anti parietal cell antibodies (may be +ve)

145
Q

transfusion thresholds

A

Hb levels <70g/L
Hb levels <80g/L in ACS

ongoing haemorrhage & chronic anaemia requiring transfusions do not have to meet these thresholds

146
Q

Non haemolytic febrile transfusion reaction

A

thought to be due to leaked cytokines that accumulate during storage

presents with fever, chills and flushing

managed with temporarily stopping infusion, paracetamol, restarting transfusion at slower rate with increased monitoring

147
Q

Minor allergic reaction to transfusions

A

due to foreign plasma proteins

presents with urticaria, pruritus (i.e. only cutaneous symptoms)

managed by temporarily stopping infusion, antihistamines, restarting transfusion at slower rate with increased monitoring

148
Q

Anaphylactic transfusion reaction

A

pt presents with SOB, dyspnoea, wheezing, angioedema, hypotension and respiratory distress

managed by stopping transfusion, 500mcg adrenaline IM (0.5ml 1:1000) & supportive measures

149
Q

Acute haemolytic transfusion reaction

A

due to ABO incompatibility (this is a never event)

presents within minutes of starting transfusion with fever, abdo pain, hypotension, tachycardia, dyspnoea, chills & flushing

management
-STOP transfusion, supportive care, fluid resuscitation

NB blood should be returned to lab for confirmation i.e. direct coombs test & repeat crossmatch

150
Q

Acute haemolytic transfusion reaction

A

due to ABO incompatibility (this is a never event)

presents within minutes of starting transfusion with fever, abdo pain, hypotension, tachycardia, dyspnoea, chills & flushing

can lead to DIC & renal failure

management
-STOP transfusion, supportive care, fluid resuscitation

NB blood should be returned to lab for confirmation i.e. direct coombs test & repeat crossmatch

151
Q

Transfusion associated circulatory overload (TACO)

A

fluid overload secondary to excessive rate of transfusion or pre-existing HF

presents with hypertension, extended JVP S4 heart sound, peripheral oedema, pulmonary oedema (on CXR) and dyspnoea

managed by stopping/slowing transfusion & IV diuretics e.g. furosemide

152
Q

Transfusion related acute lung injury (TRALI)

A

non cardiogenic pulmonary oedema due ↑ vascular permeability secondary to neutrophil activation

presents with hypoxia, dyspnoea, hypotension, fever, no signs of fluid overload, SOB & pulmonary infiltrates on CXR

managed by stopping transfusion, giving O2
-consider mechanical ventilation if required

153
Q

Delayed transfusion reaction

A

24h - 28 days post infusion

generally mild symptoms including mild fever, jaundice, anaemia, chest/abdo/back pain

generally self limiting and do not need treatment

154
Q

Fluid resuscitation

A

500ml bolus of crystalloids (usually NaCl 0.9%) over 15 min
-consider 250ml bolus if elderly or known HF

if no response then can repeat boluses to a maximum of 2000ml

  • juniors should get senior help at 1000ml
  • ITU support is needed at 2000ml
155
Q

Routine maintenance fluid requirements

A

Daily requirements over 24h

  • 25-30ml/kg of fluid
  • 1mmol/kg of K+/Na+/Cl-
  • 50-100g of glucose

Nb weight based K+ should be rounded to the nearest common fluid available so in 67kg person give 60mmol (one 40mmol & one 20mmol)

K+ replacement rate should not exceed 10mmol/h

156
Q

Maximum rate of K+ infusion outside ITU

A

K+ replacement rate should not exceed 10mmol/h

157
Q

Calculating fluid rate

A

flow rate = drip factor X (volume (ml)/minutes)

drip factor is found on the giving set packaging
flow rate is in drops/min`

158
Q

Tumour lysis syndrome

A

an oncological emergency resulting from the rapid destruction of tumour cells leading to a massive release of intracellular components generally associated with the introduction of combination chemotherapy

159
Q

Tumour lysis syndrome

A

an oncological emergency resulting from the rapid destruction of tumour cells leading to a massive release of intracellular components generally associated with the introduction of combination chemotherapy

associated with high grade lymphomas & leukemias especially Burkitts lymphoma

160
Q

Characteristic features of Tumour lysis syndrome

A

Hyperkalaemia (K+ ↑)
hyperuricaemia (uric acid ↑)
hyperphosphataemia (Phosphate ↑)
hypocalcaemia (Ca2+ ↓)

161
Q

Presentation of Tumour lysis syndrome

A

typically within 1-5 days of staring chemo

Renal failure
-oedema, lethargy, oliguria

Hyperkalaemia
-arrhythmias (syncope, palpitations, chest pain) nausea, vomiting

Hypocalcaemia
-seizures, tetany, muscle cramps, perioral paraesthesia

162
Q

Investigations of Tumour lysis syndrome

A 
uric acid > 475umol/l or 25% ↑
potassium > 6 mmol/l or 25% ↑
phosphate > 1.125mmol/l or 25% ↑
calcium < 1.75mmol/l or 25% ↓
serum creatinine ≥1.5x upper limit of normal
LDH ↑
FBC
U&Es
163
Q

Management of Tumour lysis syndrome

A

Prevention is key

  • High risk pt = IV allopurinol or IV rasburicase + ↑ hydration
  • low risk pts = PO allopurinol
  • meds to be given when chemo commences

Treatment:

  • IV fluids & hydration
  • IV Rasburicase (do not combine with allopurinol as ↓ activity of Rasburicase)
  • cardiac monitoring
  • IV calcium gluconate (if symptomatic ↓ Ca2+)
164
Q

Heparin induced thrombocytopenia (HIT)

A

an immune mediated prothrombotic disorder characterised by a sudden drop in platelet count in a pt receiving heparin containing products

due to antibody formation against heparin platelet factor 4 (PF4)

usually occurs within 5-10days of initiating heparin

NB although platelet levels are low its a prothrombotic condition (platelet levels are low as they are activated & used up)

165
Q

Presentation of Heparin induced thrombocytopenia (HIT)

A

Thrombotic manifestations

  • mainly DVT, PE
  • less commonly acute limb ischaemia, MI, stroke

localised skin necrosis at heparin injection sites

NB venous thrombosis is more common than retail thrombosis
NB bleeding is very uncommon

166
Q

Investigations and management of Heparin induced thrombocytopenia (HIT)

A

Investigations
-FBC (↓platelets >50%)

Mangement

  • stop heparin
  • address need for ongoing coagulation with argabotran/fondaparinux/danaparoid

NB there should be lifetime avoidance of heparin

167
Q

Factor V Leiden

A

i.e. activated protein C resistance =i most common inherited thrombophilia,

affects ~5% of UK population

leads to ↑ risk of VTEs, but usually not screened for

Year 3 (6 decks)

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