Clinical Haematology Flashcards
(201 cards)
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
What will be seen on investigation of lead poisoning?
microcytic anaemia
| basophilic stippling and clover morphology
26
What are some signs of lead poisoning?
Peripheral neuropathy, blurred lines on gum margin, constipation and abdo pain
27
Treatment for lead poisoning?
chelating agents, DMSA, D-penicillamine, EDTA, demicercaprol
28
What inheritance is sickle cell anaemia?
autosomal recessive
29
What is the treatment for the vasoocclusive painful sickle cell crisis?
Opiate analgesia, hydration, rest, oxygen, red cell exchange if severe
30
What may trigger a sickle cell vasoocclusive crisis?
cold
31
What are the 3 types of sickle cell crisis?
vaso-occlusive
aplastic
sequestrian crisis
32
What causes an aplastic crisis?
parvovirus B19
33
Who does sequestration crisis mainly affect?
children
34
What is seen in an aplastic crisis?
a sudden fall in haemoglobin
35
In which sickle cell crisis type is there severe pain with hands and feet affected, an acute abdomen, leg ulcers etc?
Vaso-occlusive painful crisis
36
In which crisis type is there shock, worsening of anaemia, acute chest syndrome?
Sequestration crisis
37
Who are usually more frequently and severely affected by hereditary haemochromatosis?
middle aged men
38
Why will haemochromatosis present later in women?
because menstrual loss is protective
39
Name the inherited disorder of iron metabolism in which increased intestinal iron absorption leads to deposition of iron in organs, joints and skin?
hereditary haemochromatosis
40
Early symptoms of hereditary haemochromatosis?
asymptomatic, tiredness of arthralgia (2nd and 3rd MCP joints, and knee pseudogout)
41
Late symptoms of hereditary haemochromatosis?
slate grey skin pigmentation, signs of chronic liver disease, hepatomegaly, cirrhosis, dilated cardiomyopathy, osteoporosis, endocrine problems
42
Investigations for hereditary haemochromatosis?
Bloods - will find raised LFTs, and ferritin
Imaging will find chrondrocalcinosis
Liver MRI may show iron overload
43
What can assess severity of disease of hereditary haemochromatosis?
Perl's stain on liver biopsy - will quantify iron loading
44
What are 4 sources of iron overloading?
- repeated red cell transfusions
- excessive iron absorption related to overactive erythropoiesis
- ineffective erythropoiesis eg thalassaemia
- refractory hypoplastic anaemias
45
Treatment of iron overloading?
iron chelating agents (desferrioxamine, deferipone, deferasirox) all begin with D
46
Describe what happens in ABO incompatible blood transfusion.
Intravascular haemolysis
47
What is seen in urine in ABO incompatible blood transfusion, and in what other situations may this be seen?
Haemoglobinuria (PINK urine, turns BLACK on standing)
| This may also be seen in G6PD deficiency and Falciparum malaria (Black water fever)
48
Describe G6PD deficiency
X linked intravascular haemolysis, oxidative stress may occur
49
What can the oxidative stress in G6PD deficiency cause?
rapid anaemia
| jaundice
50
Where does G6PD deficiency tend to occur?
Meditteranean, Africa or far/middle east
51
What type of cells are seen on blood film in G6PD deficiency?
Bite and blister cells
52
Exposure to what can predispose G6PD deficiency?
Vica fava (broad beans)
53
When does falciparum malaria/blackwater fever present?
mostly within 1 month of mosquito bite
54
Symptoms of falciparum malaria/blackwater fever?
prodromal headache, malaise, myalgia, anaemia, jaundice, hepatosplenomegaly
55
What sort of drugs may cause drug induced haemolytic anaemia?
penicillin - causes formation of RBC autoantibodies
| Quinine - production of immune complexes
56
What causes spherocytosis?
autoantibodies in Autoimmune haemolytic anaemia
57
What are the two types of Autoimmune haemolytic anaemia?
Warm AIHA - IgG mediated
| Cold AIHA - IgM mediated
58
Which type of AIHA binds at body temperate?
Warm AIHA
59
What type of AIHA binds at cold temperates?
COLD AIHA
60
What type of AIHA may follow infection?
cold
61
What is ass. with Cold AIHA?
chronic anaemia, made worse by the cold
| often ass with Raynauds/acrocyanosis
62
Treatment of cold AIHA?
keep warm
| chlorambucil may help
63
Treatment of warm AIHA?
steroids
| immunosuppressant/splenectomy
64
What inheritance is hereditary spherocytosis?
autosomal dominant
65
What type of haemolysis occurs in hereditary spherocytosis?
extravascular haemolysis
66
Why do gallstones occur in hereditary spherocytosis?
increased bilirubin levels
67
What can be seen in blood film in hereditary spherocytosis?
increased fragility of red blood cells in hypotonic solutions
68
What type of macrocytic anaemias are B12/folate?
megaloblastic
69
Where is folate normally found?
leafy veg, yeast
70
What is more likely to be caused by inadequate intake, B12 or folate deficiency?
folate
71
In which type of anaemia can there be dorsal column abnormalities?
folate deficiency
72
What type of cells are destroyed in pernicious anaemia?
gastric parietal cells
73
What does blood film show in pernicious anaemia?
Macrovalocytes and hypersegmented neutrophils
74
What two autoantibodies should be checked for in pernicious anaemia?
anti GPC and anti IF
75
What is treatment for pernicious anaemia?
B12 (hydroxycobalamin injections) for life
| folic acid tablets orally
76
Causes of non megaloblastic macrocytic anaemia?
alcohol, liver disease, hypothyroidism, marrow failures
77
Myeloma normal age of diagnosis?
65
78
Risk factors for myeloma?
pernicious anaemia, exposure to benzene/agent orange, radiation exposure
79
Most common type of antibody produced in myeloma?
IgG
80
What is myeloma?
malignant neoplasm of PLASMA cells
81
What type of anaemia in myeloma?
normocytic, normochromic
82
Blood film of myeloma?
Rouleux formation
83
Urine in myeloma?
bence jones proteins
84
Imaging shows what in myeloma?
pepper pot skull, lytic lesions
85
Symptoms of myeloma?
backache, increased calcium, bone pain, anaemia, renal impairment
86
What happens to ESR and PV in myeloma?
persistently raised
87
Treatment of myeloma?
```
Dexamethasone/Prednisolone
Alkylating agents (cyclophosphamide or mephalan)
```
88
What monitors response in myeloma to treatment?
paraprotein
89
What can correct hypercalcaemia and bone pain in myeloma?
bisphosphonate
90
Describe monoclonal gammopathy of uncertain significance (MGUC)
LOW paraprotein, no evidence of myeloma/organ damage
V common
BUT can progress to myeloma
91
What is the name for a group of disorders characterized by extracellular deposits of a protein in abdnormal fibrillary form, resistant to degradation?
amyloidosis
92
What are the 2 types of amyloidosis
```
AL amyloidosis (primary)
AA amyloidosis (secondary)
```
93
Which type of amyloidosis causes macroglossia?
AL amyloidosis
94
Which type of amyloidosis only affects kidneys, liver and spleen?
AA amuloid
95
What is used to confirm AL amyloid deposition?
Organ biopsy
96
What will a congo red stain show in AL amyloidosis?
apple green birefreingence under polarized light
97
Treatment for AL amyloidosis?
optimize nutrition
| oral mephalan and prednisolone extends life by 3 months
98
What is the name given to a lymphoplasmacytoid lymphoma producing a monoclonal IgM paraprotein?
Waldenstrom's macroglobulinaemia
99
What is acute lymphoblastic leukemia a malignancy of?
lymphoid cells, affecting B or T cell lines
| Uncontrolled proliferation of immature blast cells with marrow failure and tissue infiltration
100
Associations/risk factors for ALL?
X rays during prengnayc
Downs
Commonest cause of cancer in childhood
101
What type of cells are seen in ALL?
Abnormal cells with high nuclea:cytoplasmic ratio
102
What is required for a definitive diagnosis of ALL?
Immunophenotyping
103
What is used in ALL to look for mediastinal and abdominal lymphadenopathy>
CXR and CT
104
What prevents tumour lysis syndrome in ALL?
allopurinol
105
Why are infections dangerous in ALL?
due to neutropenia
106
What is acute myeloid leukemia?
neoplastic proliferation of blast cells derived from marrow myeloid elements
107
Who gets AML?
elderly
108
What can AML be associated with?
complication of chemo
Myelodysplastic states
Radiation
Downs
109
What symptoms separate AML from ALL?
ALL - meningism, cranial palsies etc
| AML - gum hypertrophy, skin involvement
110
What symptoms are common to both AML and ALL?
Anaemia, infection, bleeding, hepato/splenomegaly
111
What is seen on blood film in AML?
Auer rods in white cells
112
Treatment of AML?
chemotherapy, intensive
113
Hodgkin's lymphoma - what is it?
disorders caused by the malignant proliferations of lymphocytes
114
Is Hodgkin's lymphoma more common in males or females?
males
115
Who gets Hodgkin's lymphoma?
young adults and elderly
116
What is Hodgkin's lymphoma ass with?
Western
Obesity
SLE
Post transplant
117
Symptoms of Hodgkin's?
Fever, weight loss, night sweats, PRURITIS, alcohol induced lymph node pain
118
What is lymphadenopathy like in Hodgkin's lymphoma?
enlarged, painless, non tender, rubbery lymph nodes
119
What indicates a worse prognosis in Hodgkin's lymphoma?
increased ESR or decreased haemoglobin
120
What is non-Hodgkin's lymphoma?
disorders caused by malignant proliferation proliferation of lymphocytes but with NO REED_STERNBERG cells
121
What cells are present in Hodgkin's lymphoma?
reed Sternberg cells
122
Where are most Non-Hodgkin's lymphomas from in terms of cell lines?
B cell lines
123
Causes of Non-Hodgkin's?
Immunodeficiency, HTLV-1, HIV, H pylori, toxins
124
How many patients have nodal disease at presentation (superficial lymphadenopathy)?
75%
125
What are other symptoms of Non-Hodgkin's?
oropharynx lymphoma can cause sore throat, obstructed breathing
Gut - symptoms of gastric cancer
Systemic e.g. night sweats, fever, weight loss
126
How is fanconis anaemia inherited?
Autosomal recessive
127
What is fanconis anaemia?
Defective stem cell repair/chromosomal fragility leads to aplastic anaemia
128
What does fanconis anaemia lead to an increased risk of?
AML
Breast cancer
Bone marrow failure
129
Café au lait spots, short stature, absent radii, microcephaly, syndactyly?
fanconis anaemia
130
What is seen in fanconis anaemia in bloods?
Macrocytosis followed by thrombocytopenia, then neutropenia
131
What is the treatment for fanconis anaemia?
stem cell transplant
132
Name the rare stem cell disorder leading to pancytopenia and hypoplastic marrow.
Aplastic anaemia
133
What is needed for diagnosis of aplastic anaemia?
marrow examination
134
What is chronic myeloid leukemia?
Uncontrolled clonal proliferation of myeloid cells
135
Who gets CML?
40-60yr olds (rare in childhood)
136
Philadelphia chromosome?
CML
137
Translocation of what occurs in CML?
between long arm of chromosome 9 and 22 (forms a fusion gene)
138
What are some symptoms of CML?
general weight loss, tirderness, fever etc
Features of GOUT
bleeding
Abdominal discomfort
139
What happens to B12 in CML?
increased
140
What happens to bone marrow in CML?
hypercellular
141
Does Philadelphia chromosome increase or worsen prognosis in CML?
increases
142
Treatment of CML?
BCR-ABL tyrosine kinase inhibitor
143
How should CML be treated once it enters blast phase?
like ALL
144
WHat is the cure for CML?
stem cell transplant
145
What is polycythemia rubra vera?
malignant proliferation of a clone dervided from one pluripotent stem cell
146
Mutation of what occurs in polycythemia rubra vera?
JAK2
147
Who is polycythemia rubra vera common in?
over 60s
148
Key symptoms of polycythemia rubra vera?
```
dizziness/tinnitus
headaches
visual disturbance
ITCH AFTER A HOT BATH
Ertyrhomelalgia (burning sensation in fingers and toes)
Facial plethora (reddish complextion)
GOUT
```
149
What happens to alkaline phosphate score in polycythemia rubra vera?
raised
150
In what is alkaline phosphate score decreased?
CML
151
Treatment for Polycythemia rubra vera?
Venesection in young patients
| Hydroxycarbamide
152
Main complcations of polycythemia rubra vera?
thrombosis and haemorrhage
153
Causes of secondary polycythemia?
chronic hypoxia, smoking, erythropoietin secreting tumour
154
What happens to RBCs in polycythemia?
increased
155
What causes pseudopolycythemia?
dehydration, diuretic therapy, obesity
156
What happens to red cell mass in pseydopolycythaemia?
red cell mass stays the same, but plasma volume decreases (leading to high haematocrit)
157
Treatment of essential thrombocythaemia?
low dose aspirin/hydroxycarbamide
158
What is myelofibrosis?
hyperplasia of megakaryocytes, which produce platelet derived growth factor, leading to intense marrow fibrosis and myeloid metaplasia
159
What shape of RBCs are seen in myelofibrosis?
tear drop shaped
160
What causes immune thrombocytopenic purpura?
antiplatelet antibodies
161
What are the types of immune thrombocytopenic purpura?
acute: usually in children a few days after an infection
Chronic: usually women
162
In immune thrombocytopenic purpura, what can be seen to be invreased in the bone marrow?
megakaryocytes
163
Treatment for immune thrombocytopenic purpura?
no treatment if mild
| If symptoms, prednisolone
164
What is DIC?
Disseminated Intravascular Coagulation
| Activation of coagulation, consumption of clotting factors/platelets - leads to increased risk of bleeding
165
What can cause DIC?
malignancy, sepsis, trauma, obstretic events
166
What kind of blood cells can be seen in DIC?
schistocytes (broken blood cells)
167
What reduces DIC risk in acute promyelocytic leukemia?
transretoic acid
168
What reduces morality in DIC?
activated protein X
169
There are 3 types of Von Willebrand's disease - which is recessive?
type 3 (1 and 2 are dominant)
170
Which is the most common type of VWD?
Type 1
171
In which type are there severe symptoms?
Type 3
172
What are signs of all types of VWD?
bruising, epistaxis, menorrhagia, increased bleed post tooth extraction
173
What is used to treat mild bleeding in VW disease?
desmopressin
174
WHat should be avoided in VWD?
NSAIDs
175
What causes haemorrhagic disease of the newborn?
Vit K deficiency
176
How should haemorrhagic disease of the newborn be treated?
Routine IM vit K
177
What is Haemophilia A a deficiency of?
factor VIII
178
What is Haemophilia B a deficiency of?
Factor IX
179
What is Haemophilia B also known as?
Christmas Disease
180
What symptoms may occur in haemophilia?
bleeding into joints leads to crippling arthopathy
haematomas
increased pressure can cause nerve palsies/compartment syndrome
181
What should be avoided in Haemophilia?
NSAIds and IM injections
182
Which type of haemophilia is most common?
A
183
Risk factors in DVT?
oestrogen, prev DVT, immobility, surgery, obesity, thrombophilia, age, pregnancy etc
184
Signs of DVT?
calf warmth, tenderness, erythema, mild fever, PITTING OEDEMA
185
Test for DVT?
D dimer
| Ultrasound
186
What is the chief cause of inherited thrombophilia?
Factor V Leiden
187
How many people have Factor V Leiden ?
5% of population
188
Do most people with FActor V Leiden develop thrombosis?
NO
189
What can anti-phospholipid syndrome asso with?
SLE
190
Signs of anti-phospholipid syndrome?
Coagulation defect
Livedo reticularis
Obstetric complication eg recurrent miscarraige
Thrombocytopenia
191
What happens to platelets in anti-phospholipid syndrome?
decreased platelets
192
How is Anti-phospholipid syndrome checked for?
Antiicardiolipin
| Lupus anticoagulant
193
How is anti-phos syndrome treated?
low dose aspirin or warfarin if recurrent thrombosis
194
What to antibodies in anti-phos syndrome lead to a conformational change of?
2 glycoprotein 1, leading to activation of both prim and secondary haemostasis and vessel wall abnormalities
195
How is haemorrhagic disease of the new born treated?
routine IM vit K
196
What may be used to replace fibrinogen in DIC?
cryoprecipitate
197
What may be used to replace coagulation factors in DIC?
FFP
198
What need to be replaced in DIC?
platelets
199
If patients with immune thrombocytopenia purpura have a relapse, what treatment may cure 90% of patients?
splenectomy
200
What treatment may be given in immune thrombocytopenia purpura if symptomatic or if platelets are low?
prednisolone
201
What is used to diagnose myelofibrosis?
bone marrow trephine
