Haem Flashcards

1
Q

what is mcv for microcytic anaemia and what does it suggest

A

MCV < 80
insufficient haemoglobin production

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2
Q

what is mcv for normocytic anaemia and what does it suggest

A

mcv 80-100
decreased blood volume or decreased erythropoiesis

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3
Q

what is mcv for macrocytic anaemia and what does it suggest

A

mcv > 100
defective DNA synthesis and repair

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4
Q

clinical features of anaemia

A

pallor
dyspnoea on exertion
fatigue
tachycardia (bcos need more blood)
koilonychia (iron deficiency)
pica

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5
Q

what would iron studies for iron deficiency anaemia show

A

low ferritin (confirms IDA)
raised transferrin
increased TIBA

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6
Q

what would iron studies show for anaemia of chronic disease

A

high ferritin
low transferrin
reduced TIBC
raised ESR
increased hepcidin

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7
Q

what investigation would you use to diagnose thalassaemia

A

haemoglobin electrophoresis

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8
Q

what investigation would you use to diagnose sickle cell disease

A

haemoglobin electrophoresis

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9
Q

what does increased HbA2 suggest

A

beta thalassemia trait

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10
Q

what does blood film show for iron deficiency anaemia

A

pencil poikilocytes
hypochromic and microcytic red cells
target cells
anisopoikilocytosis

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11
Q

investigations for macrocytic anaemia

A

vit b12 and folate levels
homocysteine and methylmalonic acid levels

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12
Q

what do hypersegmented neutrophils on blood film suggest

A

megaloblastic anaemia (b12/folate deficiency)

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13
Q

test for autoimmune haemolytic anaemia

A

DAT test (aka Coombs test)

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14
Q

lab results for haemolytic anaemia

(and first line treatment)

A

raised unconjugated bilirubin
raised LDH (raised when intracellular contents released)
raised reticulocytes
low haptoglobin
spherocytes on blood film
may be warm (IgG antibodies, SLE, CLL) or cold (IgM antibodies, mycoplasma, mononucleosis, lymphoma)
Tx with steroids (prednisolone)

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15
Q

management for anaemia

A

blood transfusion with RBCs if severe
nutrient replacement
IDA > ferrous sulfate

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16
Q

Ix for microcytic anaemia

A

FBC
blood film
blood chemistry
iron studies

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17
Q

mnemonic for types of microcytic anaemias

A

Find Those Small Cells Last

Fe deficiency
Thalassemia
Sideroblastic
Chronic disease
Lead poisoning

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18
Q

causes of iron deficiency anaemia

A

decreased intake:
-eating disorders
-dietary restrictions
-food insecurity

decreased absorption
-coeliac disease
-surgical resection of GI tract
-bariatric surgery
-excessive dietary calcium
-tannates
-oxalates

increased need and growth
-pregnancy, lactation
-growing children

increased loss of blood

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19
Q

complications of iron deficiency anaemia

A

high output heart failure
angina
cardioresp failure
impaired growth and development

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20
Q

signs and symptoms of iron deficiency anaemia

A

split into decreased oxygen to tissues and effects on epithelium

decreased oxygen to tissues
-pallor
-fatigue
-exertional dyspnoea
-angina
-compensation: palpitations, increased pulse, increased cardiac output, tachypnoea, shunting of blood to vital organs

effects on epthelium
-glossitis
-scaling, fissuring, dryness, lip scaling
-koilonychia
-oesophageal stricture

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21
Q

lab results for IDA

A

low RBC count
low/normal reticulocytes
low hb, haematocrit
hypochromic-microcytic erythrocytes
- low MCV, low MCH, low MCHC

blood film shows rbc with central pallor, anisocytosis, poikilocytosis, target cells

iron studies show:
decreased serum iron
decreased ferritin
high transferrin
increased total iron binding capacity

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22
Q

treatment for IDA

A

PO iron supplements (ferrous sulfate)
parenteral iron for severe persistent anaemia or intolerance or non adherence to PO iron

increase dietary iron
vit C increases absorption
calcium DECREASES absorption

blood transfusion

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23
Q

components of HbF

A

2 alpha globin + 2 gamma globin

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24
Q

components of HbA

A

2 alpha globin + 2 beta globin

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25
components of HbA2
2 alpha globin + 2 delta globin
26
ethnic group beta thalassaemia most commonly seen
Mediterranean descent
27
ethnic group associated with alpha thalassaemia
asian and african
28
what types of beta thalassaemia are there
beta globin chains are coded by two alleles so two forms of the disease beta thalassaemia minor (one defective allele) beta thalassaemia major (two defective alleles)
29
what are the types of alpha thalassaemia
alpha globin chains are coded by four alleles so four forms of the disease silent carrier (one defective) alpha thalassaemia trait (two defective) haemoglobin H disease (three defective) haemoglobin bart disease (four defective alleles)
30
clinical features of beta thalassaemia
minor - mild microcytic hypochromic anaemia, symptomatic major- severe haemolytic anaemia, hepatosplenomegaly, skeletal deformities
31
clinical features of alpha thalassaemia
silent carrier - asymptomatic alpha thalassaemia trait - microcytic hypochromic red cells, no anaemia haemoglobin H (HbH) disease - microcytic hypochromic anaemia with anaemia haemoglobin bart disease - intrauterine death
32
Ix for thalassaemia
Hb - electrophoresis to check HbA2 levels and HbH FBC (microcytic hypochromic anaemia) raised reticulocytes
33
interpretation of Hb electrophoresis for thalassemia
minor alpha thalassemia: normal or low MCV/MCH, normal or low HbA2, normal HbF HbH disease: low MCV/MCH, normal or low HbA2, normal or high HbF, HbH present beta thalassemia: low MCV/MCH, high HbA2, high HbF, absent HbH
34
management for thalassaemia
thalassaemia major - lifelong transfusion therapy - may lead to iron overload and organ failure so iron chelation therapy with desferrioxamine (deferoxamine) genetic counselling and screening tests for relatives folic acid supplements to support erythropoiesis consultation with cardiology, other specialties ongoing monitoring
35
complications of beta thalassaemia
haemolytic, microcytic, hypochromic anaemia: - chronic leg tissue hypoxia - leg ulcers - high output heart failure - hypermetabolic state > nutritional deficiency extramedullary haematopoiesis: - bone marrow hyperplasia which can lead to structural malformations haemolysis - increased bilirubin leads to gallstones iron overload - myocardium > arrythmia, restrictive cardiomyopathy, heart failure - endocrinopathies - liver cirrhosis and hepatocellular cancer - renal insufficiency treatment related complications
36
signs and symptoms of thalassemia
hypoxia: -systemic: pallor, fatigue, activity intolerance -cardiac: low bp, tachycardia, arrythmias chronic haemolysis: - jaundice, dark urine, hepatosplenomegaly
37
lab results thalassaemia
reduced serum Hb hypochromic microcytic erythrocytes blood smear (poikilocytosis, anisocytosis, erythroblasts, target cells) increased LDH increased unconjugated bilirubin decreased haptoglobin increased serum iron and ferritin
38
how does chronic disease cause anaemia
infective organism wants to utilise body's iron cytokines switch off iron transport via hepcidin in response increases iron storage and decreases its presence in serum
39
is anaemia of chronic disease normocytic or microcytic
usually normocytic but eventually become microcytic when iron is depleted
40
point mutation for sickle cell disease
beta globin gene, Chr 11 autosomal recessive
41
sickling of cells is predisposed by
hypoxia dehydration acidosis infection
42
lab results for sickle cell disease
blood film: Howell-Jolly Bodies Sickled cells vaso-occlusion, haemolysis, splenic sequestration > decreased haemoglobin, haematocrit, RBC count reticulocytosis increased WBC count
43
what happens during sickle cell crisis
acute painful crisis stroke sequestration crisis chronic cholecystitis
44
management of sickle cell crisis
mnemonic - Sickle Acute Painful Crisis Supportive oxygen Antibiotics (if needed) Pain relief Cannula (IV fluids) and Crizanlizumab for prevention Exchange blood transfusion Splenectomy Cholecystectomy
45
Diagnostic tests for sickle cell anaemia
Hb electrophoresis blood film
46
complications of sickle cell anaemia
dactylitis acute chest syndrome haemolytic anaemia priapism aplastic crisis
47
management of sickle anaemia
conservative: -trigger avoidance -vaccination medical: -vaccinations -hydroxyurea (increases cell deformability, decreases RBC endothelial adhesion, reduces sickling) -hydroxycarbamide -prophylactic Abx - L-glutamate (reduced sickling) - pain management surgical: -bone marrow transplant (curative)
48
what type of anaemia is sickle cell disease
normocytic haemolytic anaemia
49
causes of megaloblastic anaemia
cobalamin and/or folate deficiency b12 deficiency impaired DNA synthesis during erythropoiesis insufficient diet or increased requirements malabsorption
50
what causes pernicious anaemia
vitamin b12 deficiency secondary to intrinsic factor (IF) deficiency
51
signs and symptoms of megaloblastic anaemia
fatigue, activity intolerance, pallor compensatory mechanisms: increased heart rate, bounding pulse jaundice, splenomegaly from neuronal demyelination: numbness, glove and stocking paraesthesia, weakness hyporeflexia rombergs +ve
52
lab results megaloblastic anaemia
increased MCV and MCH hypersegmented neutrophils anisocytosis and poikilocytosis decreased serum hb and haematocrit decreased serum b12 or folate levels increased homocysteine and methylmalonic acid
53
causes of non-megaloblastic macrocytic anaemia
Myelodysplasia Hypothyroidism Liver disease Alcohol
54
features of haemolytic anaemia
scleral icterus pale conjunctivae and skin
55
blood test results haemolytic anaemia
Hb low Haptoglobin low Unconjugated bilirubin raised LDH raised
56
Causes of hereditary haemolytic anaemias
membrane - hereditary spherocytosis enzymes- G-6-P-D deficiency haemoglobin - sickle cell, thalassaemia
57
causes of g6pd deficiency
genetic - x linked recessive certain drugs, fava beans infection oxidative stress > build up of free radicals
58
signs and symptoms of g6pd deficiency
asymptomatic until exposed to oxidative stress acute haemolysis: -pallor, jaundice, dark urine -abdo/back pain due to increased splenic activity favism
59
investigations and results for g6pd deficiency
decreased hb heinz bodies bite cells elevated bilirubin elevated LDH decreased haptoglobin reticulocytosis screening g6pd assay
60
what could parvovirus b19 cause
aplastic crisis
61
signs and symptoms of hereditary spherocytosis
jaundice anaemic signs and symptoms splenomegaly
62
ix for hereditary spherocytosis
osmotic fragility test + coombs test negative low hb reticulocytosis spherocytosis schistocytes elevated bilirubin, elevated ldh, decreased haptoglobih
63
treatment for hereditary spherocytosis
splenectomy blood transfusions phototherapy folic acid supplements
64
what do you find on blood film for microangiopathic haemolytic anaemia
sheared RBCs - schistocytes
64
what do you find on blood film for microangiopathic haemolytic anaemia
sheared RBCs - schistocytes
65
haemolytic uraemic syndrome triad
thrombocytopenia microangiopathic haemolytic anaemia acute kidney injury
66
typical patient profile for haemolytic uraemic syndrome
child infected with shiga toxin-producing e.coli with bloody diarrhoea
67
what is atypical haemolytic uremic syndrome
no preceding diarrhoea
68
lab results for haemolytic uremic syndrome
requires triad proteinuria, haematuria schistocytes on blood film
69
differential diagnosis for haemolytic uraemic syndrome
TTP - measure ADAMTS13 activity in plasma DIC - DIC panel (pTT, INR, d-dimer, fibrinogen)
70
abx for haemolytic uraemic syndrome
none! shiga-like toxin clears in days to week, abx not recommended as dead bacteria potentially release more toxins
71
what is disseminated intravascular coagulation
severe concurrent clotting and bleeding
72
causes of DIC
pancreatitis sepsis obstetric complications cancers trauma ABO reaction
73
features of DIC
bleeding features: - petechiae - ecchymoses - haematuria clotting features: - prolonged APPT - prolonged PT haemolytic features: - jaundice - conjunctival pallor + features of underlying pathology
74
lab results for DIC
low platelets low Hb low fibrinogen low clotting factors increased PT increased aPPT increased FDPs increased d-dimer schistocytes due to MAHA
75
cause thrombotic thrombocytopaenic purpura
defunct ADAMTS-13 enzyme
76
clinical signs for TTP
ADAMTS: Antiglobulin negative Decreased platelets AKI MAHA Temperature Swinging CNS signs
77
DIC management
treat underlying disease platelet + coagulation factors transfusion anticoagulation - heparin
78
complication for DIC
acute renal failure life-threatening haemorrhage haemothorax gangrene
79
blood cancer classification
leukaemia: - myeloid - lymphoid lymphoma: - Hodgkin - non-Hodgkin other: - myelodysplasia - myeloma - polycythaemia vera, myelofibrosis, etc
80
which cells are affected acute myeloid leukaemia
rapid proliferation of MYELOBLASTS these crowd out bone marrow so you don't get downstream production of neutrophils and other granulocytes
81
risk factors for AML
incidence increases with age down's syndrome irradiation anti-cancer drugs
82
signs and symptoms AML
bone marrow failure: pallor, bleeding, infections tissue infiltration: swollen gums, mild splenomegaly Auer rods on cytology signs of neutropenia, anaemia, thrombocytopenia
83
what is acute promyelocytic leukaemia
hyper-aggressive subtype of AML genetic translocation - fuses RAR alpha gene with PML gene Faggot cells on cytology- lots of Auer rods
84
what cells are proliferated in ALL
lymphoblasts
85
most common childhood blood cancer
ALL
86
risk factors ALL
childhood genetics radiation influenza
87
signs and symptoms of ALL
bone marrow failure: pallor, bleeding, infections tissue infiltration: lymphadenopathy, hepatosplenomegaly, swollen testes, tender bones
88
how do signs and symptoms of ALL differ from AML
lymph node swelling common in ALL unlike AML B symptoms often present in ALL (fever, night sweats, weight loss)
89
hyperproliferation of which cell types in CML
myeloid stem cells (granulocyte precursor)
90
3 stages of CML
chronic phase accelerated phase blast crisis
91
risk factors for CML
male Philadelphia chromosome (BCR-ABL1 fusion gene)
92
signs and symptoms of CML
up to 50% asymptomatic massive splenomegaly hypermetabolic symptoms: - weight loss, malaise, sweating bone marrow failure: - pallor, bleeding, infections hyperviscosity symptoms: (too many rbcs) - thrombotic events, headaches
93
pathophysiology of CLL
progressive accumulation of functionally incompetent lymphocytes caused by failure of apoptosis
94
ALL bone marrow biopsy result
>20% lymphoblasts on bone marrow biopsy
95
risk factors CLL
male genetics
96
signs and symptoms of CLL
50% asymptomatic occasionally non-tender lymphadenopathy occasional bone marrow failure symptoms
97
how is CLL usually diagnosed
routine blood test - lymphocytosis smear/smudge cells on blood film
98
leukaemia investigations to order
FBC - unusual cell counts LDH - elevated in cancer Blood smear - to look at cells Bone marrow aspirate - enables conclusive diagnoses CXR - to look for mediastinal lymphadenopathy (ALL) Immunophenotyping - looking at cell markers and antigens to enable leukaemia classification
99
how do patients with lymphoma present
lump + systemic symptoms (FLAWS)
100
hodgkins lymphoma risk factors
bimodal age distribution (peaks between 20-30 and >50) 50% association with EBV (warning: EBV presence non specific)
101
hodgkins lymphoma sign and symptoms
painless enlarging neck mass which may become painful after alcohol consumed B symptoms non tender rubbery lymphadenopathy with splenomegaly +/- hepatomegaly neutrophilia
102
diagnosis of Hodgkins lymphoma
Reed-Stenberg cells on lymph node biopsy these are bi-nucleate lymphocytes
103
Who's going to ace their exams
chelsea, hannah, maz and wese
104
risk factors for non Hodgkins lymphoma
EBV, HIV, SLE, Sjorgens incidence increases with age
105
signs and symptoms of non-Hodgkins lymphoma
painless enlarging mass in neck, axilla or groin B symptoms (tho less common than HL) organ involvement - skin rashes, headache, hepatosplenomegaly neutropenia
106
diagnosis of non hodgkins lymphoma
no reed-sternberg cells on lymph node biopsy
107
Burkitt lymphoma usually cancer of what cells
b lymphocytes
108
risk factors of Burkitt lymphoma
strong association with EBV infection chronic malaria reduces resistance to EBV HIV
109
presentation of burkitt lymphoma
rapidly enlarging lymph node in jaw
110
what does microscopy show in burkitt lymphoma
starry sky appearance
111
how is lymphoma staged
Ann Arbor staging system stage 1: single lymph node region or single organ stage 2: two or more lymph node regions same side of diaphragm stage 3: two or more lymph node regions above or below the diaphragm stage 4: widespread disease, multiple organs with or without lymph node involvement
112
cause of tumour lysis syndrome
metabolic abnormalities that arise as result of cancer treatment release of contents from lysed cells causes a constellation of symptoms
113
symptoms of tumour lysis syndrome and how they arise
released phosphate: - forms calcium phosphate crystals - leads to kidney failure and hypocalcaemia released potassium: - causes hyperkalaemia - leads to arrythmia released uric acid: - forms urate crystals - leads to gout
114
which cells are proliferated in multiple myeloma
do not be fooled - not myeloid lineage proliferation of plasma cells
115
what do cancerous plasma cells produce in multiple myeloma
monoclonal immunoglobulin (IgG or IgA)
116
risk factors for multiple myeloma
age > 70 Afro-Caribbean ionising radiation agricultural work (benzene, herbicides) HIV
117
presentation of multiple myeloma (hint: cancer star sign symbol)
CRAB Calcium high: bones, stones, abdo groans Renal impairment: worse prognosis Anaemia: due to bone marrow crowding Bone lesions: increased osteoclast activation (back/rib pain) monoclonal Ig also crowds out production of normal polyclonal Ig causing infections
118
multiple myeloma investigations
bloods: -raised ESR, CRP, urea, Cr, Ca - normal ALP (distinguish form bone malignancy where ALP high) blood film: - Rouleaux (rbc stacked like coins) formation serum/urine electrophoresis: - Bence Jones proteins bone marrow aspirate: - increased plasma cells > 10% x-ray: - to assess osteolytic lesions
119
what is monoclonal gammopathy of unknown significance
pre-malignant condition with accumulation of some monoclonal plasma cells absent CRAB features
120
what is myelodysplasia
group of syndromes where the immature blood cells do not mature normally
121
difference between primary and secondary myelodysplasia
primary: intrinsic bone marrow problem secondary: previous chemotherapy/radiotherapy
122
myelodysplasia signs and symptoms
may initially be asymptomatic chronic pancytopaenia (anaemia, neutropenia, thrombocytopaenia)
123
presentation of haemophilia
usually presents early in life or after surgery/trauma deep bleeding and bruising (secondary haemostasis) - haemarthrosis - haematoma - excessive bruising and haematuria x-linked recessive (male)
124
which clotting factor deficient in haemophilia A
factor 8
125
which clotting factor deficient in haemophilia B
factor 9
126
diagnosis of haemophilia
prolonged APTT (factor 8 + 9 in intrinsic pathway) factor assay to confirm diagnosis
127
which clotting factor does vWF stabilise
factor 8
128
what is type 1 von willebrand syndrome
reduced levels of normal vWF autosomal dominant
129
what is type 2 von willebrand syndrome
adequate levels of abnormal vWF autosomal dominant
130
what is type 3 von willebrand syndrome
complete lack of vWF and reduced factor 8 autosomal recessive
131
presentation von willebrand syndrome
superficial bleeding bruising, epistaxis, menorrhagia prolonged gum bleeding after dental procedures prolonged bleeding from minor wounds type 3 more severe and causes deep bleeding into joints and soft tissues
132
diagnosis of von willebrand syndrome
depends on type prolonged bleeding time prolonged APTT and normal PT (factor 8 reduction) reduced vWF (except in type 2) normal platelets
133
difference between acute overt DIC and chronic non-overt DIC
Acute overt: emergency and life-threatening significant platelet and clotting factor depletion > bleeding Chronic non-overt: slower rate, time for compensatory responses hypercoagulable state but less bleeding
134
what is haemochromatosis
autosomal recessive disorder of iron absorption and metabolism resulting in iron accumulation
135
risk factors for haemochromatosis
genetics male > 40 yrs
136
types of haemochromatosis and how they are caused
primary: - hereditary (autosomal recessive) - mutations on the HFE gene (hint: H(igh) Fe) - most common form secondary: - caused by iron overload - transfusion related
137
clinical features of haemochromatosis
most asymptomatic triad (fatigue, arthralgia, erectile dysfunction) signs of secondary diabetes tanning (bronze pigmentation) liver - cirrhosis, hepatomegaly cardiac failure loss of libido due to hypogonadism
138
Ix for haemochromatosis
kinda opposite to IDA results high serum iron serum transferrin saturation >45% transferrin low raised serum ferritin low TIBC genetic tests
139
management of haemochromatosis
dietary change (low iron diet) therapeutic phlebotomy: (1st line) - regular venesection weekly - target ferritin and transferrin levels drug-induced iron chelation with deferoxamine (2nd line)
140
complication of haemochromatosis
cirrhosis diabetes mellitus heart failure hepatocellular carcinoma (may need USS) hypogonadism
141
what is Wilson's disease
autosomal recessive disorder leading to excess copper deposition in tissues hence LOW COPPER IN SERUM
142
presentation of Wilson's disease
onset usually between 10-25 yrs old combination of liver and neurological disease liver - hepatitis, cirrhosis neurological - basal ganglia degeneration, asterixis, chorea, dementia Kayser-Fleischer rings
143
classification of haemophilia based on severity
mild: - haematomas following severe trauma - factor 8/9 activity between 5-50% moderate: - haematomas following mild trauma - factor 8/9 activity between 1-5% severe: - spontaneous haematomas - factor 8/9 activity <1%
144
management of haemophilia
substitution of clotting factors (factor concentrates) desmopressin - may be given for mild haemophilia A. triggers release of vWF leading to increase of factor 8 antifibrinolytic therapy (aminocaproic acid or tranexamic acid)
145
management of von willebrand disease
tranexamic acid for mild bleeding desmopressin factor 8 concentrate
146
causes of hyposplenism
operative splenectomy sickle cell anaemia coeliac disease CLL IBD bone marrow transplant congenital asplenia
147
indications for splenectomy
trauma spontaneous rupture hypersplenism neoplasia
148
most common organisms associated with severe infection in hyposplenism
NHS or No Happy Spleen N.meningitidis H.influenzae type b S.pneumoniae (pneumococcus)
149
presentation of hyposplenism
sepsis following splenectomy due to infection (pyrexia, hypotension, tachycardia)
150
Ix for hyposplenism
blood film: howell jolly bodies, target cells, siderocytes imaging: USS, CT, MRI following splenectomy - thrombocytosis (may need prophylactic aspirin)
151
Mx for hyposplenism
immunisations prophylactic antibiotics (penicillin or amoxicillin) - recommended in patients at high risk of pneumococcal infections
152
what are the myeloproliferative disorders
CML polycythaemia vera essential thrombocytosis primary myelofibrosis
153
what is essential thrombocytosis
aka primary thrombocythaemia chronic myeloproliferative disorder associated with sustained dysregulated megakaryote proliferation in the bone marrow increasing the number of circulating platelets causing thrombocytosis
154
risk factors for essential thrombocytosis
50-70 years JAK2 mutation
155
main conditions with massive splenomegaly
CML and myelofibrosis
156
signs and symptoms of myelofibrosis
massive splenomegaly pancytopenia dry tap - failure of bone marrow aspirate tear drop poikiloctes (dactrocytes) on blood film
157
clinical features of myleproliferative disorders
erythromelalgia - burning pain and dusky congestion of extremities, pain worse with heat splenomegaly arterial and venous thrombosis bleeding
158
Ix for myeloproliferative disorders
FBC - platelet count > 450 x 10^9/L JAK2 mutation testing
159
Mx of myeloproliferative disorders
hydroxycarbamide - used to reduce platelet count antiplatelet therapy - aspirin
160
causes of pancytopenia
aplastic anaemia myelofibrosis multiple myeloma methotrexate leukaemia chemotherapy radiotherapy b12 or folate deficiency lymphoma
161
what is polycythaemia
increased hb conc
162
difference between relative and absolute (true) polycythaemia
relative: normal cell mass but low plasma volume caused by dehydration or stress absolute: increased cell mass
163
difference between polycythaemia vera (primary) and secondary polycythaemia
primary is EPO independent secondary driven by excess EPO
164
risk factor for polycythaemia vera
Budd-Chiari syndrome
165
what can polycythaemia vera lead to
AML or myelofibrosis
166
causes on increase in EPO
appropriate: chronic hypoxia inappropriate: hepatocellular carcinoma, renal carcinoma, EPO abuse by athletes
167
clinical features of polycythaemia
constitutional symptoms - weight loss, fatigue, sweating hyperviscosity syndrome - mucosal bleeding + neurological symptoms + visual changes facial redness tenderness or painful burning and redness of fingers palm, heels or toes pruritis - worse when skin touches water splenomegaly
168
Ix and results for polycythaemia
hb elevated hct elevated JAK2 gene mutation screen elevated platelets decrease EPO in polycythaemia vera increased EPO in secondary polycythaemia (check for EPO secreting tumours)
169
Mx for polycythaemia
phlebotomy (venesection) antiplatelet prophylaxis (aspirin) cytoreductive therapy JAK2 inhibition (Ruxolitinib)
170
common anticoagulants
oral: vit K antagonist (warfarin) DOACs (apixaban) parenteral: heparin
171
how does warfarin work
vit K antagonist vik K responsible for production of factors 2,7,9,10 has biggest effect on factor 7 hence prolonged PT can be reversed by replacement of vit K
172
warm autoimmune haemolytic anaemia is associated with which immunoglobulin
IgG warm weather makes me Glad
173
cold autoimmune haemolytic anaemia is associated with which immunoglobulin
IgM cold weather makes me Mad