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Haematology Flashcards

1
Q

sx of anaemia

A 
fatigue
dyspnoea
lightheaded
palpitations
headache
tinnitus
anorexia
(angina if pre-existing coronary disease)
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2
Q

signs of anaemia

A

pallor (+ of conjunc)
tachycardia
flow murmurs (ejection systolic over apex)

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3
Q

causes of microcytic anaemia

A

iron def
thalassaemia
sideroblastic

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4
Q

causes of normocytic anaemia

A 
chronic disease
acute blood loss
bone marrow failure
renal failure (erythropoetin)
hypothyroid
haemolysis
pregnancy
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5
Q

in a patient with normocytic anaemia who also has low WCC an low platelets, what diagnosis would you suspect?

A

marrow failure

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6
Q

causes of high MCV (macrocytic) anaemia

A 
B12/folate def
alcohol excess/ liver disease
reticulocytosis (e.g. with haemolysis)
cytotoxics e.g. hydroxycarbamide
myeloma
marrow infiltration
hypothyroid
antifolate drugs e.g. phenytoin
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7
Q

causes of iron deficiency anaemia

A

blood loss - menorrhagia, GI
poor diet
malabsorption - coeliac
hookworm

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8
Q

signs of iron deficiency anaemia (now rare)

A

koilonychia (spoon nails)
atrophic glossitis
angular stomatitis

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9
Q

tests in iron deficiency

A 
FBC
haematinics
blood film
coeliac serology
gastroscopy + colonoscopy
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10
Q

Tx for iron deficiency anaemia

A

treat the cause
ferrous sulphate
(then IV iron)

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11
Q

Side effects of ferrous sulphate

A

nausea
abdo discomfort
constipation/ diarrhoea
black stools

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12
Q

pathophysiology behind anaemia of chronic disease

A
  1. poor use of iron in erythropoesis
  2. cytokines shorten RBC survival
  3. reduced EPO production + response to it
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13
Q

examples of causes of anaemia of chronic disease

A 
malignancy
RA
chronic infection
vasculitis
renal failure
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14
Q

Mx of anaemia of chronic disease

A

treat underlying cause

EPO

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15
Q

what is sideroblastic anaemia

A

ineffective erythropoesis and iron loading in marrow

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16
Q

what is haemosiderosis in sideroblastic anaemia

A

endocrine, liver and hear damage due to iron deposition

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17
Q

causes of sideroblastic anaemia

A 
congenital
idiopathic
chemo
anti-TB drugs
irradiation
alcohol excess
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18
Q

what test results would you see in sideroblastic anaemia

A 
low Hb
low MCV
high ferritin
hypochromic blood film
sideroblasts in marrow biopsy
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19
Q

Tx of sideroblastic anaemia

A

remove cause
tranfusion
pyridoxine

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20
Q

tests in macrocytic anaemia

A

FBC, haematinics, LFT, TFT
blood film
bone marrow biopsy

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21
Q

in what foods is folate found?

A

liver
green veg
nuts
yeast

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22
Q

maternal folate deficiency causes what problem in fetus

A

neural tube defects

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23
Q

causes of folate deficiency

A
  1. poor diet e.g. elderly, poverty, alcoholics
  2. increased demand e.g. preg, increased cell turnover (CA, haemolysis, inflamm disease, renal dialysis)
  3. malabsorption [coeliac]
  4. alcohol
  5. drugs [antiep, methotrex, trimeth]
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24
Q

management of folate deficiency

Ix + Mx

A

assess for poor diet
coeliac serology

folic acid + B12

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25
causes of glossitis
``` B12 deficiency iron def contact derm/ food allergy crohns, coeliac drugs alcoholism ```
26
causes of B12 deficiency
diet [vegan] | malabsorp [PA, crohns, resection]
27
food sources of B12
meat fish dairy products
28
describe how absorption of B12 occurs
intrinsic factor from the stomach binds B12, allowing it to be absorbed in the terminal ileum
29
clinical features of B12 deficiency
lemon tinged skin [pallor + jaundice] glossitis anaemia Sx psych: irritable, dementia, depression, psychosis parasthesia, peripheral neuropathy subacute combined degeneration of the spinal cord
30
classical triad of subacute combined degeneration of the spinal cord - seen in B12 deficiency
extensor plantars absentknee jerks absent ankle jerks
31
describe briefly the patholgy behind pernicious anaemia
autoimmune atophic gastritis leading to reduced secretion of intrinsic factor from the parietal cells unbound B12 therefore not absorbed
32
other diseases associated with pernicious anaemia
autoimmune: thyroid, addisons, vitiligo, hypoparathyroidsim | gastric carcinoma
33
Ix in pernicious anaemia
``` FBC [Hb, MCV, WCC, platelets] haematinics parietal cell autoantibodies intrinsic factor antibodies blood film [hypersegmented neutrophils] marrow ```
34
Mx of B12 deficiency
treat cause | B12 IM/ PO
35
why is there a marked continuing high MCV after initiating B12 treatment for deficiency
reticulocytosis
36
normal lifespan of a RBC
120 days
37
haemolysis can occur intravascularly or extravascularly. Explain extravascular haemolysis
macrophages in liver, spleen, bone marrow (reticuloendothelial system)
38
investigation findings that would support incerased red cell breakdown
1. raised bilirubin 2. low Hb, normal or high MCV 3. high urinary urobilinogen 4. high serum LDH (released from red cells)
39
what blood test finding would suggest increased red cell production?
high MCV [reticulocytosis]
40
an examinatin finding that may suggest extravascular haemolysis
splenomegaly
41
important aspects of the history in suspected haemolytic anaemia
``` FH previous anaemia ethnicity dark urine drugs travel ```
42
important aspects of the examination in suspected haemolytic anaemia
jaundice hepatosplenomegaly gallstones [^bili] leg ulcers
43
Ix in haemolytic anaemia
``` FBC LFT [bili] LDH urinary urobilinogen haptoglobin blood film ``` if travel Hx - malaria thick + thin films extras: osmotic fragility testing direct coombs test Hb electrophoresis
44
what does coombs test identify
immune causes of haemolytic anaemia | identifies RBCs coated with antibody
45
acquired causes of haemolytic anaemia
``` infection autoimmune malignancy CLL, lymphoma TTP, HUS drugs DIC transfusion hep B+C vaccinations pre-eclampsia ```
46
hereditary causes of haemolytic anaemia
enzyme defect: G6PD deficiency, PKD membrane defect: hereditary spherocytosis haemoglobinopathy: sickle cell, thalasaemia
47
how is sickle cell inherited
autosomal recessive
48
pathophysiology of sickle cell
abnormal haemoglobin results in deformed RBCs (sickle cells). these haemolyse -> vaso-occlusive crises
49
investigation findings in sickle cell
FBC - low Hb, ^reticulocytes/MCV blood film - sickle cells + target cells LFT - high bili
50
triggers for a vaso-occlusive 'painful' crisis in sickle cell
cold hypoxia dehydration infection
51
presentation of vaso-occlusive 'painful' crisis
``` severe pain dactylitis in <3 yr olds acute abdomen [mesenteric ischaemia] stroke, seizure, cognitive defects avascular necrosis (e.g. femoral head) leg ulcers priapism ```
52
what is a sequestration crisis in sickle cell and Mx?
pooling of blood in spleen and liver > organomegaly, shock, severe anaemia transfusion
53
what is a aplastic crisis in sickle cell
parvovirus B19 infection leads to sudden reduction in marrow production of RBCs
54
complications of sickle cell
``` splenic infarction poor growth CKD gallstones retinal disease iron overload lung hypoxia -> fibrosis -> pULM HTN ```
55
Mx of chronic sickle cell
``` analgesia for crises hydroxycarbamide Abx prophylaxis + immunisations [splenic infarct] transfusions bone marrow transplant ```
56
Mx of sickle cell crisis
``` analgesia septic screen fluids O2 Abx if pyrexial transfusion/ exchange transfusion ```
57
Sx of acute chest syndrome in sickle cell
``` wheeze dyspnoea pain fever cough ```
58
what causes the infiltrates in acute chest syndrome in sickle cell
infection | fat embolism from bone marrow
59
Mx of acute chest syndrome in sickle cell
``` O2 analgesia Abx [cephalosporin + macrolide] bronchodilators in wheeze] transfusion/ exchange ```
60
examples of macrolide Abx
azithromycin clarithromycin erythromycin
61
examples of cephalosporin Abx
cefuroxime | ceftriaxone
62
what is thalasaemia
reduced production of one globin chain | leads to haemolysis
63
where in the world is thalassaemia common?
med to far east
64
Ix in beta thalasaaemia
``` FBC haematinics film Hb electrophoresis MRI [for myocardial siderosis] ```
65
clinical features of patient with beta thalassaemia
``` presents in 1st yr of life severe anaemia failure to thrive hepatosplenomeg skull deformity osteopenia ```
66
mx of beta thalassaemia
life long regular transfusions folate iron chelators [deferipone + desferrioxamine] ascorbic acid -> iron excretion splenectomy Mx of endrocrine complications [DM, thyroid] marrow transplant
67
side effect / risk of regular transfusions in beta thalassaemia, and the consequences of this
iron overload endocrine: pituitary, thyroid, pancreatic failure [DM]. liver disease cardiac toxicity
68
what are the 3 processes that halt bleeding after injury? (and therefore the 3 types of bleeding disorder)
vasoconstriuction platelet plugging coag cascade
69
bleeding disorders - platelet/vascular vs coagulation disorder. What is the pattern of bleeding in platelet/vascular disorders?
1. prolonged bleeding from cuts 2. bleeding into skin (bruising, purpura) 3. Mucous membranes (epistaxis, gums, menorrhagia)
70
bleeding disorders - platelet/vascular vs coagulation disorder. What is the pattern of bleeding in coagulation disorders?
into joionts and muscles
71
bleeding disorders: give 4 examples of platelet disorders
``` decreased production: leukaemia, myeloma aplastic anaemia (marrow failure) cytotoxic drugs radiotherapy ``` destruction: ITP (anti-platelet antibodies) SLE drugs e.g. heparin non-immune: DIC TTP HUS
72
bleeding disorders: congenital coagulation disorders
haemophilia | VWd
73
bleeding disorders: acquired coagulation disorders
anticoagulants liver disease DIC vit K deficiency [diet, absorption]
74
what are haemophilia A + B
A =factor 8 deficiency | B= factor 9 def
75
what is the pattern of inheritance in haemophilia A + B
X linked recessive
76
how does haemophilia A present
early life or post-surg/trauma bleeds into joints - arthropathy and into muscles - haematomas (+nerve palsies, compartment syndrome)
77
how is haemophilia A diagnosed
^APTT | low factor VIII assay
78
Mx of haemophilia A
``` desmopressin [^s factor VIII] compression and elevation in minor bleeds avoid IM injection, NSAIDs recombinant factor VIII genetic councelling ```
79
Mx of haemophilia B
recombinant factor IX
80
what is acquired haemophilia and how is it managed
factor VIII autoantibodies suddenly appear and cause big mucosal bleeds steroids
81
why does liver disease produce a bleeding disorder
1. reduced synthesis of clotting factors 2. reduced absorption of vitamin K 3. abnormalities in platelet function
82
why does malabsorption lead to bleeding disorder?
reduced uptake of vit K (needed for factor synth)
83
what is the final product of the clotting cascade?
fibrin
84
how does chronic ITP present (immune thrombocytopenia)
``` bleeding purpura (esp. pressure areas) menorrhagia epistaxis usually women ```
85
Mx of ITP
``` none if mild pred IV Ig for temporary e.g. surgery splenectomy ritux ```
86
when would a D-dimer be raised
``` fibrin degradation product so... DVT PE DIC inflammation e.g. infection, malignancy ```
87
pre-op patient - what questions mioght you ask to assess their risk of excessive bleeding?
previous unexplained big/prolonged bleed liver disease SLE drugs e.g. anticoags
88
complications of massive blood transfusion
``` thrombocytopenia hypocalc low clotting factors hyperkal hypothermia ```
89
how would you transfuse a HF patient without overloading?
give with furosemide
90
what is an acute haemolytic reaction in blood transfsuion and what Sx
ABO/Rh incompatibility ``` pyrexia agitation low BP abdo/chest pain flushing oozing venepuncture sites DIC ```
91
why do you need to be wary using heparin in renal failure patinet and how would you combat this?
accumulates lower dose
92
contraindictations to giving heparin e.g. for DVT prophylaxis
``` bleeding disorders low platelets on bloods peptic ulcer cerebral haemoprrhage severe HTN neurosurg ```
93
contraindictations to giving warfarin
peptic ulcer bleeding disorders severe HTN pregnancy [terat]
94
contraindications to NOACs
renal/ liver failure active bleeding lesion at risk of bleeding low clotting factors
95
management of patinet on warfarin with minor or major e.g. intracranial bleed
minor - vit K | major - prothromin complex concentrate and vit K (or FFP if PCC unavailable)
96
what is tumour lysis syndrome and what are the dangerous consequences
chemo -> cell death -> ^urate, ^K+, ^phosphate, low calcium arrhythmia, renal failure
97
how do you prevent tumour lysis syndrome complications
hydration | allopurinol
98
causes of DIC
malignancy trauma sepsis obstetric emergencies
99
DIC Mx
treat cause platelets cryo [fibrinogen] FFP [coag factors]
100
pathology behind ALL
malignancy of lymphoid cells in the bone marrow leading to proliferation of immature blasts ∴ marrow failure
101
ALL and AML, more common in adult or child?
ALL childhood | AML adult
102
clinical features in ALL
``` anaemia Sx e.g. pallor SOB bleeding (brusing/epistaxis) infection lymphadenopathy hepatosplenomegaly orchidomegaly CNS involvment - cranial nerve palsy/meningism ```
103
how is acute leukaemia diagnosed
blood film - blast cells | bone marrow biopsy - blast cells
104
auer rods within blast cells are pathognomic of which leukaemia
AML
105
Ix in acute leukaemias
``` FBC [anaemia, thrombocytopenia, WCC↑] blood film bone marrow biopsy LP fever - CXR, cultures CXR/CT lymphadenopathy ```
106
Mx of ALL
1. chemo +/- intrathecal 2. transfusion for anaemia 3. platelets for thrombocytopenia 4. IV Abx for infection (+prophylaxis) 5. fluids + allopurinol to prevent tumour lysis syndrome 6. bone marrow transplant
107
ALL has an increased risk of CSF involvement. How would you combat this?
give chemo into csf
108
common infection site in ALL
chest mouth perianal skin
109
clinical features in AML
``` anaemia bleeding infections hepatosplenomegaly gum hypertrophy skin involvement ```
110
tests in AML
bone marrow biopsy | FBC [WCC↑/↓/↔]
111
complications of AML and its treatment
infections, sepsis tumour lysis syndrome from chemo leukostasis
112
Mx of AML
1. chemo 2. transfusion for anaemia 3. platelets for thrombocytopenia 4. IV Abx for infection (+prophylaxis) 5. fluids + allopurinol to prevent tumour lysis syndrome 6. bone marrow transplant
113
CML chromosome
philadelphia
114
CML Sx
``` weight loss tired fever sweats gout bleeding abdo discomfort [splenomeg] ```
115
signs in CML
splenomegaly hepatomeg anaemia bruising
116
Ix in CML
``` FBC [^WCC] urate B12 bone marrow biopsy genetic for Phili ```
117
Mx for CML
imatinib hydroxycarbamide chemo marrow transplant
118
CLL Sx
often none | anaemia, infection prone, weight loss, sweats, anorexia if seevre
119
signs in CLL
tender rubbery lymhadenopathy | hepatosplenomeg
120
Ix in CLL
FBC [^lymphocytes] | later marrow infiltration causes anaemia, neutropenia, throbocytopaenia
121
complications of CLL
autoimmune haemolysis infections [hypogammaglobulinaemia] marrow failure
122
Mx of CLL
``` chemo if symptomatic steroids for autoimmune haemolysis radiotherapy [lymphadenopathy/splenomeg] stem cell transplant transfusions IVIg for recurrent infections ```
123
what is lymphoma
malignant proliferation of lymphocytes in lymph nodes | also in peripheral blood and infiltrate organs
124
characteristic cell of hodgkins l;ympohoma
reed-sternberg
125
risk factors for hodgkins lymphoma
affected sibling EBV SLE post transplant
126
what symptoms might mediastinal lymph nodes in hodgkins lymphoma cause by mass effect
bronchial obstruction | SVC obstruction
127
complications of hodgkins Tx
radio: lung/breast/stomach/thyroid CA, melanoma, sarcoma, IHD, hypothyroid, lung fibrosis. chemo: myelosuppression, infection, nausea, alopecia both: AML, non-hodgkins, infertility
128
what is non-hodgkins lymphoma
all lymphomas [malignant proliferation of lymphocytes] without reed-sternberg cells
129
causes of non-hodgkins lymphoma
``` HIV EBV immunosuppressive drugs H.pylori congenital ```
130
sites for non-hodgkins lymphoma
1. lymphadenopathy 2. gastric MALT [h.pylori] 3. non-MALT gastric 4. Small bowel 5. skin 6. oropharynx 7. other: bone, CNS, lung
131
Sx of non-hodgkins lymphoma
1. lymphadenopathy 2. gastric: dyspepsia, dysphagia, vomiting 3. bowel: diarrhoea, vomiting, abdo pain 4. oropharynx: sore throat, obstructed breathing all: fever, night sweats, weight loss marrow involvement: anaemia, infection, bleeding
132
tests in non-hodgkins lymphoma
``` FBC, U+E, LFT, LDH node biopsy marrow CT/PET for staging effusion - cytology LP > csf cytology ```
133
Tx non-hodgkins lymphoma
radio chemo - R-CHOP ritux
134
the myeloproliferative disorders. Name the disorders caused by proliferation of each of the following cell types: RBC, WBC, platelets, fibroblasts
RBC - polycythaemia vera WBC - CML platelets - essential thrombocythaemia fibroblasts - myelofibrosis
135
what is relative polycythaemia
normal RBC mass, low plasma vol e.g. acute - dehydration or chronic - obesity, HTN, alc/smoking
136
what is absolute polycythaemia and its causes
^RBC mass primary [PRV] or secondary [smoking, CHD, lung disease, high altitude] OR inappropriately ^EPO production [RCC, HCC]
137
what is the mutation present in >95% of polycythaemia vera?
JAK2
138
presentation of polycyth RV
asymp hyperviscosity > headache, dizzy, tinitus, visual disturbance itching after hot bath, erythromelalgia gout, arterial + venous thrombosis
139
what age is polycythaemia vera commomer
>60
140
why might polycythaemia patient get gout?
^urate from ^RBC turnover
141
signs in polycythaemia
splenomeg | flushed face
142
Ix in polycythaemia vera
FBC haematinics marrow EPO [low]
143
Mx of polycythaemia vera
reduce haematocrit by: venesection hydroxycarbamide alpha interferon aspirin
144
complications of polycythaemia vera
thrombosis haemorrhage [defective platelets] myelofibrosis acute leukaemia
145
what is essential thrombocythaemia, and what problems does it cause
proliferation in bone marrow -> ^platelets, with abnormal fn. bleeding, thrombosis [arterial and venous], microvascular occlusion
146
Tx of essential thrombocythaemia
aspirin | hydroxycarbamide
147
Sx of essential thrombocythaemia
headache CP light-headed erthromelalgia
148
what is myelofibrosis
marrow fibrosis leading to haematopoiesis in spleen + liver -> massive hepatosplenomeg
149
presentation of myelofibrosis
hypermetabolic Sx: night sweats, fever, weight loss abdo discomfort from hepatosplenomeg marrow failure: anaemia, infections, bleeding
150
Mx of myelofibrosis
marrow support - transfusions, neutropenic regimen | marrow transplant
151
causes of thrombocytosis
``` essential thrombocythaemia bleeding infection malignancy trauma post-surg iron deficiency chronic inflamm e.g. collagen disorders ```
152
what is myeloma
abnormal proliferation of plasma cells -> secretion of Ig
153
what effect does the excess Ig secreted in myeloma have ?
organ dysfunction e.g. kidney
154
what are the symtoms/ presentation of myeloma
1. bone lesions: backache, fractures 2. hypercalc: many Sx e.g. constipation, abdo pain, confusion, stones 3. anaemia Sx 4. bleeding/ bruising 5. infection! 6. renal impairment [Ig + hypercalc]
155
why do myeloma patients get anaemia and thrombocytopenia
bone marrow infiltration by plasma cells
156
why do myeloma patients get ^infections
1. immunoparesis 2. bone marrow infiltration 3. chemo
157
why do myeloma patients get hypercalcaemia
myeloma cells signal osteoclasts -> increased osteoclast activation
158
what is immunoparesis in myeloma
the Ig secreted by the myeloma cells is very high e.g. IgG. But the rest of the immunoglobulins are very low.
159
Ix in myeloma
``` FBC film ESR U+E marrow serum/urine electrophoresis ->monoclonal band XR [CT/MRI] ECG - hyperkal/hypercalc ```
160
what would be seen on bone marrow biopsy in myeloma
many plasma cells with abnormal forms
161
what might be seen on an XR in myeloma
punched out lytic lesions pepper pot skull fractures, vertebral collapse osteoporosis
162
what aspects are required for diagnosis of myeloma
1. monoclonal band [serum/urine] 2. ^plasma cells on marrow 3. end organ damage [hypercalc/renal/anaemia] 4. bone lesions
163
Mx for myeloma
``` supportive: analgesia bisphos local radio orthopedics transfusion [anaemia] fluids 3L/day (dialysis) Abx for infections IVIg [recurrent inf] ``` chemo stem cell transplant
164
usual cause of death in myeloma
renal failure | infection
165
acute complications of myeloma
``` infection hypercalc spinal cord compression hyperviscosity [light chains] AKI ```
166
casues of hyperviscosity
polycythaemia leukaemia myeloma drugs: OCP, diuretics, IVIG, EPO, chemo, contrast
167
how do you combat hyperviscosity in each of the following: polycythaemia leukaemia myeloma
polycythaemia - venesection leukaemia - leukapheresis myeloma - plasmapheresis [removes Ig]
168
give 4 causes of splenomegaly
``` infection [malaria, EBV, TB] CML myelofibrosis portal HTN/cirrhosis haemolytic anaemia lymphoma ```
169
Ix in splenomegaly
FBC LFT ESR (liver biopsy, marrow biopsy, lymph node biopsy)
170
the most common type of inherited thrombophilia
Factor V Leiden
171
acquired casues of thrombophilia
antiphospholipid syndrome OCP/HRT polycthaemia thrombocytosis
172
risk factors for arterial thrombosis
smoking HTN hyperlipidaemia DM
173
risk factors for venous thrombosis
``` surgery trauma immobility preg OCP/HRT age obesity varicose veins HF malignancy IBD nephrotic syndrome inherited ```
174
why must long term steroids not be stopped suddenly?
addisonian crisis
175
how can you combat steroid side effect osteoporosis
give calcium and vit D consider bisphos exercise and smoking cessation
176
why should steroid patient avoid NSAIDs?
^risk of duodenal ulcer
177
name 5 side effects of steroids
GI: pancreatitis, candidiasis, oesoph ulcer, peptic ulcer MSK: myopathy, osteoP, fractures, growth suppression endocrine: adrenal suppression, cushings CNS: triggers epilepsy, depression, psychosis eye: glaucoma, cataracts, papilloedema ^INFECTIONS
178
in a patient with iron deficiency, with travel Hx, what other Ix would you do and why?
travel Hx - stool microscopy for ova [hookworm]
179
Bleeding hx includes...
Obs (PPH) & surgical hx Vaccinations e.g. haematomas
180
APTT vs PT abnormalities
APTT: (intrinsic) vWf, factor 8,9,10,11 PT: (extrinsic) 7 Both: 5, 2, 10, Anticoagulant, DIC, liver disease

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