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Gallbladder disease
https://t.me/med1917
→ Gallbladder stores and concentrates bile
→ Fatty acids / amino acids in duodenum stimulate CCK release
→ CCK stimulates gallbladder contraction & bile release
Investigations
1. History & abdo exam + pregnancy test + urine dip
2. Bloods: FBC, CRP/ESR, LFTs, clotting (± amylase)
3. Imaging:
→ USS (shows GB/duct dilation)
→ MRCP (95% sensitive to detect stone)
Types of bile stone
→ ERCP (Dx & Tx but invasive)
→ CT if concerned about tumour
Chapter 4: Hepato-pancreato-biliary 55
Contents of bile:
• Water (97%)
• Bile salts / pigments
• Cholesterol
• Phospholipids
USS = FIRST-LINE imaging
1. CHOLESTEROL – crystallisation of excess cholesterol
2. PIGMENT – contain calcium bilirubinate
• Black: haemolytic conditions (SCA, malaria)
Risk factors*:
Fat Female
Fair Fertile
Forty FHx
Complications of gallstones
1. In gallbladder: biliary colic (cystic duct obstruction), acute cholecystitis,
perforation*, carcinoma
2. In bile ducts: biliary colic (common duct obstruction) → ascending cholangitis
or acute pancreatitis
3. In intestine: gallstone ileus (stone erodes through gallbladder = fistula to
duodenum → intestinal obstruction)
Management
OBSTRUCTION INFLAMMATION
= temporary duct obstruction
Symptoms:
• Colicky RUQ / diffuse pain
(visceral)
▶ crescendos (stop if stone moves)
▶ radiates to back/shoulder
▶ worse with food (esp. fatty)
• ± N&V
• Obstructive jaundice
if common duct blocked/Mirizzi’s
Investigations:
• Bloods = normal
• USS/MRCP (shows dilation/stones)
Management:
• Bed rest, fluid, analgesia, NBM
• Elective cholecystectomy (by 6w)
4
BILIARY COLIC
Systemically well!
*needs emergency cholecystectomy
AXR shows air in biliary tree
ACUTE CHOLECYSTITIS
= long-term cystic duct obstruction
causes inflammation of GB wall
Systemically unwell!
Symptoms:
Initially: biliary colic
With inflammation: (localised pain)
• Fever & vomiting
• Severe RUQ pain + peritonism
O/E:
Guarding & rigidity + Murphy’s sign
Investigations:
• Bloods: WCC, CRP, ESR
• USS/MRCP (dilation, thick GB wall)
Management: ABCDE
• IV fluid, analgesia + IV ABX
• Elective cholecystectomy (by 1w
but ideally within 72h)
*Crohn’s & ileal resection = additional RFs as bile
salt absorption
most gallstones = asymptomatic, until complications
Mirizzi’s syndrome:
Gallstone impacts in cystic duct / Hartmann’s
pouch
• Extrinsic compression of hepatic duct
• Obstructive jaundice without dilation of CBD
• C. perfringens
• Klebsiella
INFECTION
ASCENDING CHOLANGITIS
= long-term common bile duct
obstruction causes duct infection
Symptoms:
Charcot’s triad:
1. High swinging
fever (chills
+ rigors)
2. RUQ pain
3. Jaundice
Investigations:
• Bloods: WCC, CRP, ESR
• LFTs: obstructive picture
Management: ABCDE + Sepsis 6
• IV fluids, analgesia + IV ABX
(e.g. cefuroxime + metronidazole)
• Emergency ERCP ± stenting
• E. coli
Reynolds’ pentad:
+ 4. Confusion
+ 5. Shock
(ICU admission)
Key DDx: GORD, PUD, acute pancreatitis,
IBD, acute hepatitis, pyelonephritis
4
BMJ Best Practice (2021) Gallstones, acute cholecystitis, acute cholangitis
Murphy’s sign: press over GB – patient has sharp pain during inspiration as
peritoneum hits your hand
Medicine
56 Chapter 4: Hepato-pancreato-biliary
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Courvoisier’s law:
Painless, obstructive jaundice with palpable
gallbladder = unlikely due to gallstones
→ think carcinoma of pancreas
Other gallbladder pathologies
CARCINOMA OF THE GALLBLADDER
→ Uncommon (occurs in elderly)
→ Associated with long-standing gallstones
Symptoms:
• RUQ pain
• Weight loss
• Obstructive jaundice & palpable mass (late sign)
Management: poor prognosis as late presentation
• Radical cholecystectomy (± liver resection if affected)
CHOLANGIOCARCINOMA (adenocarcinoma of bile ducts / ampulla)
→ Common sites = at ductal confluences
→ Mostly in elderly patients
→ Can be 2° to PSC/IBD
Sx occur late
chronic cholecystitis)
(& then mimic
Symptoms:
• Painless progressive jaundice
• Weight loss
Management: poor prognosis as late presentation
• Whipple’s procedure (if operable)
• Palliative stenting (relieves jaundice & helps gastric emptying)
Sx occur late
PANCREATIC CANCER
& mimic
Medicine
Pancreatic neoplasms
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90% = ductal adenocarcinomas (60% head, 25% body, 15% tail). <2% = islet
cell tumours (insulinoma, glucagonoma, gastrinoma). 95% involve a KRAS2
mutation. Pancreatic neoplasms present late and are aggressive. They have a
poor prognosis: 3% 5y survival, mean survival = 6m
Symptoms
• Painless obstructive jaundice – pale stools, dark urine
• Weight loss, anorexia, cachexia
• Non-specific, dull, abdominal pain (may radiate to back)
• New onset diabetes: weight loss, polydipsia, polyuria (endocrine function)
• Pancreatic enzyme insufficiency: steatorrhoea (exocrine function)
• Thrombophlebitis migrans (Trousseau’s syndrome)
= thrombophlebitis following spontaneous clots
Signs
• Courvoisier’s sign
• Hepatosplenomegaly
• Epigastric mass
• Lymphadenopathy
Investigations
Chapter 4: Hepato-pancreato-biliary 57
Risk factors for pancreatic neoplasms:
• >80y
• Smoking/alcohol
• DM/obesity
• Chronic pancreatitis
Courvoisier’s law:
A palpable gallbladder in the presence of
painless, obstructive jaundice = unlikely due
to gallstones
→ think about a tumour in head of pancreas
(presses on bile duct)
• Bloods: FBC, U&Es, clotting, LFT ( conj. BR)
• Tumour markers: CA19-9* (level correlates with tumour volume)
• Abdo USS: pancreatic mass / dilated common bile duct, ascites
• Endoscopic USS/ERCP & biopsy (+ staging & grading)
• Contrast-enhanced CT: confirm Dx and staging
Management
Depends on tumour stage & patient factors
1. SURGICAL RESECTION = Whipple’s procedure (pancreaticoduodenectomy)
• Resect head of pancreas, first part of duodenum, gallbladder & bile duct
• Complications: delayed gastric emptying, bile leaks, pancreatic fistulae
2. PALLIATIVE = stenting & Sx relief ± chemo
• Stents help jaundice & gastric outlet obstruction
Only 15–25% cure rate
*CA19-9 = non-specific but can monitor progress
DIAGNOSTIC investigations
Surgery only suitable if
no mets and patient is fit
Post-op chemo can slow disease progression
Medicine
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59
https://t.me/med1917
time
coagulation
dehydrogenase
factor
haemoglobinuria
chromatography
cardiovascular overload
unknown significance
purpura
60 Chapter 5: Haematology
e
Macrophage
Megakaryoblast
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Denitions
→ Adults: occurs in red bone marrow (vertebrae, ribs, pelvis)
→ Children: occurs in all bones
→ Fetus: occurs in liver & spleen
→ Stimulated by EPO (produced by JGA in kidney in response to O2)
→ RBC lifespan = 120d
→ side-effect of carbimazole
Neutrophilia (>10) Neutropenia (<1.5)
• Neoplasia/CML
• Myeloproliferative
disorder
• Bleeding/burns
• Inflammation
• Severe sepsis
• Autoimmune (SLE)
• Hypersplenism (Felty’s)
• B12/folate deficiency
• Smoking
• GCSF
Lymphocytosis (>5) Lymphopenia (<1)
Megakaryocyte
Platelets
After division some cells
remain stem cells.
Myeloid stem cell
Proerythroblast
Reticulocyte
Erythrocyte
Basophil Neutrophil Eosinophil Monocyte
Multipotent hematopoietic
stem cell (hemocytoblast)
MonoblastMyeloblast
The remaining cell goes down one of two paths
depending on the chemical signals received.
Lymphoid stem cell
Lymphoblast
Natural killer cell
(Large granular
lymphocyte)
T lymphocyteB lymphocyt
Small
lymphocyte
Fig. 5.1 Haematopoiesis.
Function of blood cells
Red blood cells: transport oxygen around the body (& CO2 for removal)
White blood cells: form part of the immune system to fight infection
Platelets: involved in blood clotting
Types of white cell
→ T cells = regulate all parts of adaptive immune system
→ B cells = become plasma cells & secrete antibodies
→ Natural killer cells = pathogen destruction
Plasma cell
• Chronic infection
• Septic shock / MI /
trauma
• Smoking
• Raised BMI /
metabolic syndrome
Medicine
• CAR-T therapy
• Autoimmune (SLE)
• Renal failure
• HIV
Anaemia is defined as Hb <130g/L in men & <115g/L in women
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Chapter 5: Haematology 61
Key questions to ask:
Causes
1. RBC production e.g. IDA (most common), BM disorders, cytotoxic
drugs / chemotherapy, CKD, aplastic anaemia
2. RBC destruction e.g. SCD, thalassaemias, G6PD deficiency, autoimmune
haemolysis
3. Blood loss (rare in children) e.g. vWD, Meckel diverticulum
Signs/symptoms
• Fatigue/weakness
• Pallor (conjunctiva)
• SOB/tachycardia/dizziness
→ Koilonychia: IDA
→ Jaundice: haemolytic anaemia
→ Leg ulcers: sickle cell disease
→ Tingling fingers/toes: B12 deficiency
Symptoms occur if Hb <60–70g/L
Investigations
• FBC – MCV (size of RBC) & MHC (Hb per RBC), reticulocytes
• Iron studies – serum iron & ferritin, TIBC
• Blood film – size, shape, colour of red cells
• Serum bilirubin – high in haemolysis
• Hb HPLC or Hb electrophoresis – shows amount of each Hb type
(HbS, HbA, HbF)
1. Isolated anaemia or pancytopenia?
(pancytopenia suggests BM failure)
2. What is the MCV?
All patients are screened for anaemia
before surgery
→ If <100g/L check haematinics and replace
if low
→ If <60g/L as above, but transfuse if active
bleeding
DDx microcytic anaemia: TAILS
DDx macrocytic anaemia: ABCDEF
Types of haemoglobin
Adults normally have 2 HbA alleles (HbAA). Patients with haemoglobinopathies such as
thalassaemia or SCD have at least one abnormal allele, causing non-HbA haemoglobins
(e.g. HbSS, HbAS)
DDx normocytic anaemia: CHARMD
Medicine
62 Chapter 5: Haematology
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Dietary advice for IDA
High Fe foods:
• Red meat, liver
• Pulses, beans, peas
• Leafy, green veg
• Oily fish
• Fortified cereals
• Dried fruit / nuts
Foods to avoid:
• Excess cow’s milk (only 10% Fe is absorbed)
• Tannin (tea) (inhibits Fe absorption)
Must r/o GI bleed
inolder patients
Iron-deciency anaemia
• Inadequate intake – Fe-deficient diet e.g. vegetarian
• Malabsorption – coeliac, gastrectomy
• Increased requirements – pregnancy
• Chronic blood loss – menorrhagia / GI bleed
1. FBC: MCV = microcytic
2. Iron studies: serum iron & serum ferritin, TIBC (body tries to Fe uptake)
3. Blood film: abnormally shaped, small, hypochromic (pale) RBCs
1. Determine cause: thorough Hx & exam → r/o serious causes
▶ OGD/colonoscopy, anti-tTG, menorrhagia Hx
2. Treat underlying cause
3. Dietary advice
4. Oral iron supplements e.g. ferrous sulphate/fumarate
▶ TDS until Hb normal then minimum 3m OD
▶ Can use IV iron if oral not tolerated/absorbed
NB: if no response to Tx consider Ix for other causes (esp. malabsorption)
NB. Ferritin will be raised in
inflammation
e.g. GI malignancy
Intrinsic factor binds B12 in stomach to
enable absorption in the terminal ileum
B12 = coenzyme needed for folate conversion
Folate = needed for RBC synthesis
B12 deficiency can cause peripheral neuropathy,
subacute spinal cord degeneration & angular
cheilitis
If B12 AND folate deficient, must replace
B12 first to avoid subacute combined
degeneration of the spinal cord
B12 & folate deciency
• Low dietary intake – vegan/vegetarian
• Malabsorption (in terminal ileum) – e.g. Crohn’s, gastrectomy
• Low intrinsic factor – e.g. autoimmune (pernicious anaemia)
• Low dietary intake
• Malabsorption (in duodenum/jejunum) – e.g. coeliac, jejunal resection
• Increased requirements – pregnancy, haemolytic anaemia
1. FBC: MCV = macrocytic – often >125
2. Blood film: hypersegmented neutrophils (>5 lobules) & tear-drop cells
3. Iron & B12 studies: B12, serum folate, cobalamin
4. Intrinsic factor antibodies
B12
= eggs, fortified cereals, dairy
Folate
= broccoli, peas, brown rice
• If not pernicious anaemia, dietary advice
• IM B12 & folic acid (5mg OD) supplements (4m)
• If pernicious anaemia: lifelong IM B12 replacement (hydroxocobalamin)
RBC aplasia
reticulocytes, normal BR, Coombs test –ve
Medicine
• Diamond–Black anaemia = rare, congenital → raised MCV ± short stature,
abnormal thumbs
• Transient erythroblastopenia = triggered by viral infection in children
• Parvovirus B19 – infects young RBCs – only causes RBC aplasia in children/
adults with inherited haemolytic anaemia
1
BNF Treatment Summary – Anaemia, Iron Deciency
2
BNF Treatment Summary – Anaemia, Megaloblastic
Chapter 5: Haematology 63
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Clinical features
• Anaemia (normo-/macrocytic)
• Mild splenomegaly
• Jaundice
Further investigations
• Hb electrophoresis
• Direct Coombs test
Investigations
• FBC: Hb
• Blood film: reticulocytes* → lilac
(polychromasia), strange shape
• Bilirubin: unconjugated BR
• Lactate dehydrogenase: LDH
(freed from inside RBCs)
• Haptoglobin: (as binds to
bilirubin)
Extrinsic causes
extravascular haemolysis & spherocytosis
→ Primary
→ Secondary: to another autoimmune condition,
lymphoproliferative disorder or infection
Warm AHA (direct Coombs IgA-positive) Cold AHA (direct Coombs IgM-positive)
Optimal binding 37°C <4°C
RBC destruction Extravascular (spleen/liver) Intravascular (IgM binds complement)
Management Folate plus:
1st line: steroids ± mycophenolate
2nd line: rituximab or splenectomy
Associated with Raynaud’s
Can cause abdo pain and
acrocyanosis
1st line: folate and cold avoidance
2nd line: rituximab ± chemotherapy
*destruction of RBCs results in increased RBC
production
Overview of causes
Intrinsic
• Hb alpha or beta chain abnormality
(thalassaemias, SCD)
• RBC membrane abnormality (hereditary
spherocytosis)
• Enzyme defects (G6PD deficiency, pyruvate
kinase deficiency)
Extrinsic
Immune-mediated → Coombs positive
→ Autoimmune (warm AHA & cold AHA)
→ Drugs (nitrofurantoin, penicillin, quinine)
Non-immune mediated → Coombs negative
→ Infection (malaria, CMV, E. coli, Strep.)
→ Microangiopathic
by physical trauma to RBCs in circulation by abnormal microcirculation with
deposition of fibrin strands
• Malignant HTN / pre-eclampsia
• HUS, DIC, vasculitis, TTP, post-BM
transplant
• Prosthetic heart valve
• FBC: Hb, platelets
• Blood film: fragments
• ADAMSTS13 level (cleaving
enzyme): low levels suggest TTP
Intrinsic causes
mutations of RBC membrane proteins (1 in 5000)
spleen → RBC destroyed in the spleen
• Jaundice – may be intermittent
• Anaemia – mild (90g/L – normal) = tired, headaches
• Splenomegaly – due to RBC destruction in spleen
• Gallstones – persistent high bilirubin precipitates into stones
• APLASTIC CRISIS* – new RBCs not made fast enough
1. FBC & iron studies – normocytic anaemia
2. Blood film – spherical RBCs with no central pallor + some microspherocytes
*usually children;
follows parvovirus
B19 & lasts 2–4w
HUS/TTP: normal coagulation
DIC: prolonged coagulation + fibrinogen
ankyrin or spectrin
Fig. 5.2 Spherical RBCs .
Medicine
64 Chapter 5: Haematology
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Management of HS and PK3:
• Oral folic acid – need as RBC synthesis
• Tx for aplastic crisis due to parvovirus:
transfusions until resolves (if chronic
give IVIg)
• Splenectomy – if severe anaemia when
young / poor growth / troublesome Sx
→ will need daily prophylactic ABX afterwards
deficiency of PK enzyme
RBCs haemolysed prematurely
• Chronic haemolytic anaemia – varies greatly in severity
1. FBC & iron studies – normocytic anaemia
2. PK activity levels – reduced (may not be if acute crisis)
3. Direct Coombs test – negative
Risk factors for G6PD deficiency:
African, Mediterranean, Middle-Eastern ethnicity
Management of G6PD deficiency:
1. Safety-net for signs of acute haemolysis
2. Advise on foods/drugs to avoid
(quinine, sulphonamides, nitrofurantoin,
high dose aspirin, fava beans)
3. ± Blood transfusion during haemolytic
crisis
*only seen during haemolytic crisis;
ghost cells
Bone marrow transplant = only cure for PNH
(reserved for most severe/life-threatening cases)
preventing oxidative damage to RBCs)
• Neonatal jaundice – usually within first 3d
• Chronic haemolysis
• Acute intravascular haemolysis – fever, pallor, malaise, dark urine
→ precipitated by infection, certain drugs & fava beans
→ Hb drops <50g/L within 24–48h
• FBC: Hb, reticulocytes
• Unconjugated bilirubin: raised
• LDH: raised
• Blood film: Heinz body inclusions* (lumps of damaged Hb)
• G6PD activity: reduced (may not be during an acute crisis – only check when
stable)
• Direct Coombs test – negative
• A rare, acquired disorder of haematopoietic stem cells, which causes
production of defective RBCs
• Defective cells are haemolysed by the body’s complement system
Medicine
• Episodic haemoglobinuria (dark urine)
• Anaemia (SOB, chest pain, fatigue)
• Thromboses: increased risk of blood clots (can result in PE, Budd–Chiari
syndrome, organ ischaemia)
• Pancytopenia: increased risk of infection & bleeding + anaemia
1. Symptomatic e.g. iron replacement for anaemia
2. Folate replacement for ongoing haemolysis
3. ± Anticoagulation: thrombosis prophylaxis
4. ± Monoclonal antibodies which block complement – very successful
3
British Society for Haematology (2011) Guidelines for the diagnosis and management of hereditary
spherocytosis
4
NORD (2016) The physician's guide to paroxysmal nocturnal haemoglobinuria (PNH)
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