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Flashcards in Clinical Haematology Deck (201)
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1
Q

Is iron deficiency anaemia common?

A

Yes - seen in 14% of menstruating women

2
Q

Signs of iron deficiency anaemia (name 4)?

A

koilinychia, angular stomatitis, atrophic glossitis, pallor

3
Q

What happens to ferritin and iron in iron deficient anaemia?

A

reduced

4
Q

Give 3 ways in which causes of iron deficiency anaemia should be investigated.

A

Endoscopy
Stool microscopy
Barium enema

5
Q

How should iron deficient anaemia be managed?

A

treat cause

give ferrous sulphate

6
Q

What is the cause of anaemia of chronic disease?

A

There is a poor use of iron in erythropoiesis and shortening of the red blood cell
Decreased response to erythropoetin

7
Q

What type of anaemia is found in chronic disease?

A

mild, normocytic

8
Q

What happens to ferritin in anaemia of chronic disease?

A

normal or raised

9
Q

How should anaemia of chronic disease be treated?

A

underlying cause
erythropoietin can be given to raise haemoglobin level
Iron can be given parentally

10
Q

How does alpha thalassaemia trait present?

A

asymptomatic

11
Q

How does HbH disease present?

A
Wide variation, from asymptomatic to transfusion dependant, but may have:
splenomegaly
jaundice
growth retardation
gallstones
12
Q

How does Hb Bart’s hydrops fetalis syndrome present?

A
pallor
oedema
cardiac failure
severe hepatosplenomegaly
Most die in utero
13
Q

Treatment for alpha thalassaemia trait?

A

none needed

14
Q

Treatment of mild HbH disease?

A

transfusion only needed at times of intercurrent illness

15
Q

Treatment of severe HbH disease?

A

Transfusion dependant, splenectomy may be useful

Folic acid supplements

16
Q

What may be seen on blood film in alpha thalassaemia triat?

A

red cell inclusions (HbH)

17
Q

What may be seen on blood film in HbH disease?

A

target cells

Anisopoikilocytosis

18
Q

Skeletal changes and hair on end sign, presenting aged 6-24 months?

A

Beta thalassaemia major

19
Q

Symptoms of beta thallasaemia intermedia (B+/B+ or B0/B+)?

A

moderate anaemia

20
Q

Symptoms of beta thallassaemia major (B0/B0)?

A

severe anaemia, hepatosplenomegaly, organ damage,

21
Q

What is diagnostic of beta thalassaemia trait?

A

raised HbA2

22
Q

What is seen is beta thalassaemia major in investigation?

A

very low MCV/MCH
Reticulocytosis
Ansiopoikilocytosis and target cells

23
Q

In beta thalassaemia major, HbA2 and HbA are sometimes raised, but what is the main Hb present?

A

HbF

24
Q

What should be sued for diagnosis of lead poisoning

A

Blood lead level

25
Q

What will be seen on investigation of lead poisoning?

A

microcytic anaemia

basophilic stippling and clover morphology

26
Q

What are some signs of lead poisoning?

A

Peripheral neuropathy, blurred lines on gum margin, constipation and abdo pain

27
Q

Treatment for lead poisoning?

A

chelating agents, DMSA, D-penicillamine, EDTA, demicercaprol

28
Q

What inheritance is sickle cell anaemia?

A

autosomal recessive

29
Q

What is the treatment for the vasoocclusive painful sickle cell crisis?

A

Opiate analgesia, hydration, rest, oxygen, red cell exchange if severe

30
Q

What may trigger a sickle cell vasoocclusive crisis?

A

cold

31
Q

What are the 3 types of sickle cell crisis?

A

vaso-occlusive
aplastic
sequestrian crisis

32
Q

What causes an aplastic crisis?

A

parvovirus B19

33
Q

Who does sequestration crisis mainly affect?

A

children

34
Q

What is seen in an aplastic crisis?

A

a sudden fall in haemoglobin

35
Q

In which sickle cell crisis type is there severe pain with hands and feet affected, an acute abdomen, leg ulcers etc?

A

Vaso-occlusive painful crisis

36
Q

In which crisis type is there shock, worsening of anaemia, acute chest syndrome?

A

Sequestration crisis

37
Q

Who are usually more frequently and severely affected by hereditary haemochromatosis?

A

middle aged men

38
Q

Why will haemochromatosis present later in women?

A

because menstrual loss is protective

39
Q

Name the inherited disorder of iron metabolism in which increased intestinal iron absorption leads to deposition of iron in organs, joints and skin?

A

hereditary haemochromatosis

40
Q

Early symptoms of hereditary haemochromatosis?

A

asymptomatic, tiredness of arthralgia (2nd and 3rd MCP joints, and knee pseudogout)

41
Q

Late symptoms of hereditary haemochromatosis?

A

slate grey skin pigmentation, signs of chronic liver disease, hepatomegaly, cirrhosis, dilated cardiomyopathy, osteoporosis, endocrine problems

42
Q

Investigations for hereditary haemochromatosis?

A

Bloods - will find raised LFTs, and ferritin
Imaging will find chrondrocalcinosis
Liver MRI may show iron overload

43
Q

What can assess severity of disease of hereditary haemochromatosis?

A

Perl’s stain on liver biopsy - will quantify iron loading

44
Q

What are 4 sources of iron overloading?

A
  • repeated red cell transfusions
  • excessive iron absorption related to overactive erythropoiesis
  • ineffective erythropoiesis eg thalassaemia
  • refractory hypoplastic anaemias
45
Q

Treatment of iron overloading?

A

iron chelating agents (desferrioxamine, deferipone, deferasirox) all begin with D

46
Q

Describe what happens in ABO incompatible blood transfusion.

A

Intravascular haemolysis

47
Q

What is seen in urine in ABO incompatible blood transfusion, and in what other situations may this be seen?

A

Haemoglobinuria (PINK urine, turns BLACK on standing)

This may also be seen in G6PD deficiency and Falciparum malaria (Black water fever)

48
Q

Describe G6PD deficiency

A

X linked intravascular haemolysis, oxidative stress may occur

49
Q

What can the oxidative stress in G6PD deficiency cause?

A

rapid anaemia

jaundice

50
Q

Where does G6PD deficiency tend to occur?

A

Meditteranean, Africa or far/middle east

51
Q

What type of cells are seen on blood film in G6PD deficiency?

A

Bite and blister cells

52
Q

Exposure to what can predispose G6PD deficiency?

A

Vica fava (broad beans)

53
Q

When does falciparum malaria/blackwater fever present?

A

mostly within 1 month of mosquito bite

54
Q

Symptoms of falciparum malaria/blackwater fever?

A

prodromal headache, malaise, myalgia, anaemia, jaundice, hepatosplenomegaly

55
Q

What sort of drugs may cause drug induced haemolytic anaemia?

A

penicillin - causes formation of RBC autoantibodies

Quinine - production of immune complexes

56
Q

What causes spherocytosis?

A

autoantibodies in Autoimmune haemolytic anaemia

57
Q

What are the two types of Autoimmune haemolytic anaemia?

A

Warm AIHA - IgG mediated

Cold AIHA - IgM mediated

58
Q

Which type of AIHA binds at body temperate?

A

Warm AIHA

59
Q

What type of AIHA binds at cold temperates?

A

COLD AIHA

60
Q

What type of AIHA may follow infection?

A

cold

61
Q

What is ass. with Cold AIHA?

A

chronic anaemia, made worse by the cold

often ass with Raynauds/acrocyanosis

62
Q

Treatment of cold AIHA?

A

keep warm

chlorambucil may help

63
Q

Treatment of warm AIHA?

A

steroids

immunosuppressant/splenectomy

64
Q

What inheritance is hereditary spherocytosis?

A

autosomal dominant

65
Q

What type of haemolysis occurs in hereditary spherocytosis?

A

extravascular haemolysis

66
Q

Why do gallstones occur in hereditary spherocytosis?

A

increased bilirubin levels

67
Q

What can be seen in blood film in hereditary spherocytosis?

A

increased fragility of red blood cells in hypotonic solutions

68
Q

What type of macrocytic anaemias are B12/folate?

A

megaloblastic

69
Q

Where is folate normally found?

A

leafy veg, yeast

70
Q

What is more likely to be caused by inadequate intake, B12 or folate deficiency?

A

folate

71
Q

In which type of anaemia can there be dorsal column abnormalities?

A

folate deficiency

72
Q

What type of cells are destroyed in pernicious anaemia?

A

gastric parietal cells

73
Q

What does blood film show in pernicious anaemia?

A

Macrovalocytes and hypersegmented neutrophils

74
Q

What two autoantibodies should be checked for in pernicious anaemia?

A

anti GPC and anti IF

75
Q

What is treatment for pernicious anaemia?

A

B12 (hydroxycobalamin injections) for life

folic acid tablets orally

76
Q

Causes of non megaloblastic macrocytic anaemia?

A

alcohol, liver disease, hypothyroidism, marrow failures

77
Q

Myeloma normal age of diagnosis?

A

65

78
Q

Risk factors for myeloma?

A

pernicious anaemia, exposure to benzene/agent orange, radiation exposure

79
Q

Most common type of antibody produced in myeloma?

A

IgG

80
Q

What is myeloma?

A

malignant neoplasm of PLASMA cells

81
Q

What type of anaemia in myeloma?

A

normocytic, normochromic

82
Q

Blood film of myeloma?

A

Rouleux formation

83
Q

Urine in myeloma?

A

bence jones proteins

84
Q

Imaging shows what in myeloma?

A

pepper pot skull, lytic lesions

85
Q

Symptoms of myeloma?

A

backache, increased calcium, bone pain, anaemia, renal impairment

86
Q

What happens to ESR and PV in myeloma?

A

persistently raised

87
Q

Treatment of myeloma?

A
Dexamethasone/Prednisolone
Alkylating agents (cyclophosphamide or mephalan)
88
Q

What monitors response in myeloma to treatment?

A

paraprotein

89
Q

What can correct hypercalcaemia and bone pain in myeloma?

A

bisphosphonate

90
Q

Describe monoclonal gammopathy of uncertain significance (MGUC)

A

LOW paraprotein, no evidence of myeloma/organ damage
V common
BUT can progress to myeloma

91
Q

What is the name for a group of disorders characterized by extracellular deposits of a protein in abdnormal fibrillary form, resistant to degradation?

A

amyloidosis

92
Q

What are the 2 types of amyloidosis

A
AL amyloidosis (primary)
AA amyloidosis (secondary)
93
Q

Which type of amyloidosis causes macroglossia?

A

AL amyloidosis

94
Q

Which type of amyloidosis only affects kidneys, liver and spleen?

A

AA amuloid

95
Q

What is used to confirm AL amyloid deposition?

A

Organ biopsy

96
Q

What will a congo red stain show in AL amyloidosis?

A

apple green birefreingence under polarized light

97
Q

Treatment for AL amyloidosis?

A

optimize nutrition

oral mephalan and prednisolone extends life by 3 months

98
Q

What is the name given to a lymphoplasmacytoid lymphoma producing a monoclonal IgM paraprotein?

A

Waldenstrom’s macroglobulinaemia

99
Q

What is acute lymphoblastic leukemia a malignancy of?

A

lymphoid cells, affecting B or T cell lines

Uncontrolled proliferation of immature blast cells with marrow failure and tissue infiltration

100
Q

Associations/risk factors for ALL?

A

X rays during prengnayc
Downs
Commonest cause of cancer in childhood

101
Q

What type of cells are seen in ALL?

A

Abnormal cells with high nuclea:cytoplasmic ratio

102
Q

What is required for a definitive diagnosis of ALL?

A

Immunophenotyping

103
Q

What is used in ALL to look for mediastinal and abdominal lymphadenopathy>

A

CXR and CT

104
Q

What prevents tumour lysis syndrome in ALL?

A

allopurinol

105
Q

Why are infections dangerous in ALL?

A

due to neutropenia

106
Q

What is acute myeloid leukemia?

A

neoplastic proliferation of blast cells derived from marrow myeloid elements

107
Q

Who gets AML?

A

elderly

108
Q

What can AML be associated with?

A

complication of chemo
Myelodysplastic states
Radiation
Downs

109
Q

What symptoms separate AML from ALL?

A

ALL - meningism, cranial palsies etc

AML - gum hypertrophy, skin involvement

110
Q

What symptoms are common to both AML and ALL?

A

Anaemia, infection, bleeding, hepato/splenomegaly

111
Q

What is seen on blood film in AML?

A

Auer rods in white cells

112
Q

Treatment of AML?

A

chemotherapy, intensive

113
Q

Hodgkin’s lymphoma - what is it?

A

disorders caused by the malignant proliferations of lymphocytes

114
Q

Is Hodgkin’s lymphoma more common in males or females?

A

males

115
Q

Who gets Hodgkin’s lymphoma?

A

young adults and elderly

116
Q

What is Hodgkin’s lymphoma ass with?

A

Western
Obesity
SLE
Post transplant

117
Q

Symptoms of Hodgkin’s?

A

Fever, weight loss, night sweats, PRURITIS, alcohol induced lymph node pain

118
Q

What is lymphadenopathy like in Hodgkin’s lymphoma?

A

enlarged, painless, non tender, rubbery lymph nodes

119
Q

What indicates a worse prognosis in Hodgkin’s lymphoma?

A

increased ESR or decreased haemoglobin

120
Q

What is non-Hodgkin’s lymphoma?

A

disorders caused by malignant proliferation proliferation of lymphocytes but with NO REED_STERNBERG cells

121
Q

What cells are present in Hodgkin’s lymphoma?

A

reed Sternberg cells

122
Q

Where are most Non-Hodgkin’s lymphomas from in terms of cell lines?

A

B cell lines

123
Q

Causes of Non-Hodgkin’s?

A

Immunodeficiency, HTLV-1, HIV, H pylori, toxins

124
Q

How many patients have nodal disease at presentation (superficial lymphadenopathy)?

A

75%

125
Q

What are other symptoms of Non-Hodgkin’s?

A

oropharynx lymphoma can cause sore throat, obstructed breathing
Gut - symptoms of gastric cancer
Systemic e.g. night sweats, fever, weight loss

126
Q

How is fanconis anaemia inherited?

A

Autosomal recessive

127
Q

What is fanconis anaemia?

A

Defective stem cell repair/chromosomal fragility leads to aplastic anaemia

128
Q

What does fanconis anaemia lead to an increased risk of?

A

AML
Breast cancer
Bone marrow failure

129
Q

Café au lait spots, short stature, absent radii, microcephaly, syndactyly?

A

fanconis anaemia

130
Q

What is seen in fanconis anaemia in bloods?

A

Macrocytosis followed by thrombocytopenia, then neutropenia

131
Q

What is the treatment for fanconis anaemia?

A

stem cell transplant

132
Q

Name the rare stem cell disorder leading to pancytopenia and hypoplastic marrow.

A

Aplastic anaemia

133
Q

What is needed for diagnosis of aplastic anaemia?

A

marrow examination

134
Q

What is chronic myeloid leukemia?

A

Uncontrolled clonal proliferation of myeloid cells

135
Q

Who gets CML?

A

40-60yr olds (rare in childhood)

136
Q

Philadelphia chromosome?

A

CML

137
Q

Translocation of what occurs in CML?

A

between long arm of chromosome 9 and 22 (forms a fusion gene)

138
Q

What are some symptoms of CML?

A

general weight loss, tirderness, fever etc
Features of GOUT
bleeding
Abdominal discomfort

139
Q

What happens to B12 in CML?

A

increased

140
Q

What happens to bone marrow in CML?

A

hypercellular

141
Q

Does Philadelphia chromosome increase or worsen prognosis in CML?

A

increases

142
Q

Treatment of CML?

A

BCR-ABL tyrosine kinase inhibitor

143
Q

How should CML be treated once it enters blast phase?

A

like ALL

144
Q

WHat is the cure for CML?

A

stem cell transplant

145
Q

What is polycythemia rubra vera?

A

malignant proliferation of a clone dervided from one pluripotent stem cell

146
Q

Mutation of what occurs in polycythemia rubra vera?

A

JAK2

147
Q

Who is polycythemia rubra vera common in?

A

over 60s

148
Q

Key symptoms of polycythemia rubra vera?

A
dizziness/tinnitus
headaches
visual disturbance
ITCH AFTER A HOT BATH
Ertyrhomelalgia (burning sensation in fingers and toes)
Facial plethora (reddish complextion)
GOUT
149
Q

What happens to alkaline phosphate score in polycythemia rubra vera?

A

raised

150
Q

In what is alkaline phosphate score decreased?

A

CML

151
Q

Treatment for Polycythemia rubra vera?

A

Venesection in young patients

Hydroxycarbamide

152
Q

Main complcations of polycythemia rubra vera?

A

thrombosis and haemorrhage

153
Q

Causes of secondary polycythemia?

A

chronic hypoxia, smoking, erythropoietin secreting tumour

154
Q

What happens to RBCs in polycythemia?

A

increased

155
Q

What causes pseudopolycythemia?

A

dehydration, diuretic therapy, obesity

156
Q

What happens to red cell mass in pseydopolycythaemia?

A

red cell mass stays the same, but plasma volume decreases (leading to high haematocrit)

157
Q

Treatment of essential thrombocythaemia?

A

low dose aspirin/hydroxycarbamide

158
Q

What is myelofibrosis?

A

hyperplasia of megakaryocytes, which produce platelet derived growth factor, leading to intense marrow fibrosis and myeloid metaplasia

159
Q

What shape of RBCs are seen in myelofibrosis?

A

tear drop shaped

160
Q

What causes immune thrombocytopenic purpura?

A

antiplatelet antibodies

161
Q

What are the types of immune thrombocytopenic purpura?

A

acute: usually in children a few days after an infection
Chronic: usually women

162
Q

In immune thrombocytopenic purpura, what can be seen to be invreased in the bone marrow?

A

megakaryocytes

163
Q

Treatment for immune thrombocytopenic purpura?

A

no treatment if mild

If symptoms, prednisolone

164
Q

What is DIC?

A

Disseminated Intravascular Coagulation

Activation of coagulation, consumption of clotting factors/platelets - leads to increased risk of bleeding

165
Q

What can cause DIC?

A

malignancy, sepsis, trauma, obstretic events

166
Q

What kind of blood cells can be seen in DIC?

A

schistocytes (broken blood cells)

167
Q

What reduces DIC risk in acute promyelocytic leukemia?

A

transretoic acid

168
Q

What reduces morality in DIC?

A

activated protein X

169
Q

There are 3 types of Von Willebrand’s disease - which is recessive?

A

type 3 (1 and 2 are dominant)

170
Q

Which is the most common type of VWD?

A

Type 1

171
Q

In which type are there severe symptoms?

A

Type 3

172
Q

What are signs of all types of VWD?

A

bruising, epistaxis, menorrhagia, increased bleed post tooth extraction

173
Q

What is used to treat mild bleeding in VW disease?

A

desmopressin

174
Q

WHat should be avoided in VWD?

A

NSAIDs

175
Q

What causes haemorrhagic disease of the newborn?

A

Vit K deficiency

176
Q

How should haemorrhagic disease of the newborn be treated?

A

Routine IM vit K

177
Q

What is Haemophilia A a deficiency of?

A

factor VIII

178
Q

What is Haemophilia B a deficiency of?

A

Factor IX

179
Q

What is Haemophilia B also known as?

A

Christmas Disease

180
Q

What symptoms may occur in haemophilia?

A

bleeding into joints leads to crippling arthopathy
haematomas
increased pressure can cause nerve palsies/compartment syndrome

181
Q

What should be avoided in Haemophilia?

A

NSAIds and IM injections

182
Q

Which type of haemophilia is most common?

A

A

183
Q

Risk factors in DVT?

A

oestrogen, prev DVT, immobility, surgery, obesity, thrombophilia, age, pregnancy etc

184
Q

Signs of DVT?

A

calf warmth, tenderness, erythema, mild fever, PITTING OEDEMA

185
Q

Test for DVT?

A

D dimer

Ultrasound

186
Q

What is the chief cause of inherited thrombophilia?

A

Factor V Leiden

187
Q

How many people have Factor V Leiden ?

A

5% of population

188
Q

Do most people with FActor V Leiden develop thrombosis?

A

NO

189
Q

What can anti-phospholipid syndrome asso with?

A

SLE

190
Q

Signs of anti-phospholipid syndrome?

A

Coagulation defect
Livedo reticularis
Obstetric complication eg recurrent miscarraige
Thrombocytopenia

191
Q

What happens to platelets in anti-phospholipid syndrome?

A

decreased platelets

192
Q

How is Anti-phospholipid syndrome checked for?

A

Antiicardiolipin

Lupus anticoagulant

193
Q

How is anti-phos syndrome treated?

A

low dose aspirin or warfarin if recurrent thrombosis

194
Q

What to antibodies in anti-phos syndrome lead to a conformational change of?

A

2 glycoprotein 1, leading to activation of both prim and secondary haemostasis and vessel wall abnormalities

195
Q

How is haemorrhagic disease of the new born treated?

A

routine IM vit K

196
Q

What may be used to replace fibrinogen in DIC?

A

cryoprecipitate

197
Q

What may be used to replace coagulation factors in DIC?

A

FFP

198
Q

What need to be replaced in DIC?

A

platelets

199
Q

If patients with immune thrombocytopenia purpura have a relapse, what treatment may cure 90% of patients?

A

splenectomy

200
Q

What treatment may be given in immune thrombocytopenia purpura if symptomatic or if platelets are low?

A

prednisolone

201
Q

What is used to diagnose myelofibrosis?

A

bone marrow trephine