Abdominal Flashcards
Autosomal Dominant Conditions
APKD type 1 (chromo 16) and type 2 (chromo 4)
Hereditary spherocytosis
HHT
Autosomal Recessive Conditions
Haemochromatosis (chromo 6)
Wilson’s disease (chromo 13)
Alpha 1 antitrypsin (chromo 14)
How can you differentiate the spleen from a kidney on palpation?
1) Dull to percussion
2) Not ballotable
3) Palpable splenic notch
4) Cannot get above splenic mass
Outline some causes of Spider Naevi?
= vascular lesion in distribution of SVC
> 5 = pathological
1) Normal in childhood
2) Pregnancy
3) Oestrogen
4) Chronic liver disease
5) Thyrotoxicosis
Outline causes of Acanthosis Nigricans
Insulin-resistant diabetes mellitus
Paraneoplastic
Hypo/hyperthyroidism
Acromegaly
Cushing’s disease
Obesity
Outline causes of gynaecomastia
Idiopathic
Chronic liver disease
Chronic kidney disease
Thyrotoxicosis
Congenital e.g. Klinefelter’s
Drugs e.g.
- Haloperidol
- Spironolactone
- Omeprazole
- Alcohol
- Ketoconazole
- Digoxin
Outline causes of Hepatomegaly
Cs & Is of Hepatomegaly
C - Cirrhosis
C - Carcinoma (HCC)
C - CCF
I - Infectious e.g. Hepatitis, EBV, CMV
I - Immune(Auto) e.g. PBC, PSC
I - Infiltrative e.g. Amyloidosis
I - Iron (Haemochromatosis)
What is pre-hepatic jaundice?
Jaundice = elevated serum bilirubin
Pre-hepatic = disruption in bilirubin pathway occurs prior to hepatic bilirubin conjugation
Increased RBC haemolysis = increase in levels of UNCONJUGATED bilirubin
Causes:
- Haemolytic anaemias
- Gilbert’s (autosomal recessive)
What is post-hepatic jaundice?
Jaundice = elevated serum bilirubin
Post-hepatic = disruption in bilirubin pathway occurs after hepatic bilirubin conjugation, and results in reduced excretion of bilirubin from body
Blockage prevents excretion of CONJUGATED bilirubin
Causes:
- Gallstones
- Pancreatic cancer
- Cholangitis
- Cholangiocarcinoma
Which blood tests can you do to differentiate between types of jaundice?
Liver function tests (ALT>ALP indicates hepatic damage, raised yGT and ALP indicates post hepatic “cholestatic”)
Split bilirubin (conj & unconj bilirubin)
Outline the blood tests involved in a liver screen
AUTOIMMUNE
- ANA, anti-mitochondrial antibody, anti-smooth muscle antibody, liver-kidney microsomal antibody
- Anti-TTG
INFECTIOUS
- Hep B surface antigen, Hep C antibody
- Hep B core antibody
- CMV/EBV (if acute)
Immunoglobulins
METABOLIC
- HbA1c
- Ferritin & iron studies
- Caeruloplasmin
Alpha feto-protein
Paracetamol (if acute)
?alpha 1 AT level
Outline the pathophysiology of Liver Cirrhosis
1) Hepatocellular injury
2) Stimulates stellate cells to become myofibroblasts
3) Myofibroblasts secrete pro-inflammatory cytokines and produce collagen
4) = Tissue fibrosis
5) = Distorts hepatic architechture and vasculature
6) = Portal hypertension and only hepatocellular injury due to reduced perfusion
What are the main causes of liver cirrhosis?
TOP 3
Alcohol
MASLD
Hepatitis C
Other important causes
- Autoimmune e.g. AIH, PBC
- PSC
- Haemochromatosis
- Wilson’s
- CF
- Alpha 1 AT
What is decompensated CLD? How could this present?
= acute deterioration in liver function in patients with cirrhosis
Jaundice
Asterixis
Confusion
Ascites
Peripheral oedema
Outline some risk factors for developing decompensated liver disease
Alcohol
Infection
AKI/Dehydration
Constipation
Medications e.g. sedating agents
Thrombosis e.g. hepatic vein thrombus
Fluid overload
What is Child Pugh grading?
Severity of cirrhosis score
A to C
- A = 100% 1 year survival
- C = 50% 1 year survival
5 components to score
1) Serum albumin
2) Ascites
3) Bilirubin
4) INR
5) Hepatic encephalopathy
Outline how you would investigate for ?chronic liver disease
Full history
Medication history
Alcohol history
Routine bloods, including FBC, U&Es, coagulation and LFTs
Non-invasive liver screen blood tests
- See other flashcard
USS Liver
?CTAP/MRCP (depends of type of jaundice)
FIB-4 score (predicts likelihood of advanced fibrosis)
Transient Elastography (fibroscan)
Liver biopsy?
Who are offered Transient Elastography in order to screen for liver cirrhosis?
Patients with Hepatitis C
Men who drink > 50 units of alcohol/week
Women who drink > 35 units of alcohol/week
Patients with known Alcoholic Liver Disease
Which criteria can help you decide which patients with cirrhosis should have OGD surveillance for oesophageal varices?
Baveno Criteria
How often should patients with liver cirrhosis be offered HCC screening? What does this entail?
Liver USS every 6 months +/- aFP levels
Outline the management of ascites
Reduce dietary intake of sodium
Aldosterone antagonist (spironolactone)
Monitor weight
Therapeutic abdominal paracentesis
Transjugular intrahepatic portosystemic shunts
?Prophylactic antibiotics e.g. ciprofloxacin
- If previous SBP or cirrhotic with ascitic protein < 15g/L
Risk of TIPS procedure
Increased risk of hepatic encephalopathy
What would be the main indication for performing a transjugular liver biopsy as opposed to percutaneous?
Ascites
How can you delineate between transudate and exudate causes of ascites?
Serum:Ascites Albumin Gradient
SAAG>11g/L = Transudate
SAAG< 11g/L = Exudate
Causes of Transudate Ascites
Cirrhosis with portal HTN
CCF
Nephrotic syndrome
Budd-Chiari
Portal Vein thrombosis
Malabsorption
Myxoedema
(Failures and Thrombuses)
Causes of Exudate Ascites
Malignancy e.g. metastatic GI cancers, HCC, ovarian, peritoneal
TB peritonitis
Pancreatic ascites
Meig’s Syndrome
= ovarian fibroma, pleural effusion & ascites
Bowel Obstruction
Serositis in CTD
What is hepatorenal syndrome?
Renal hypoperfusion and failure, secondary to severe liver disease
Type 1 = Rapidly progressive, poor prognosis
Type 2 = Slowly progressive
How would you manage a patient with hepatorenal syndrome?
MDT approach - liver and renal specialists
Terlipressin (splanchnic vasoconstrictor)
Cautious monitoring of fluid balance - + HAS 20%
TIPSS
Grading of Hepatic Encephalopathy
West Craven Criteria
0 = clinically normal
1 = mild confusion, irritability, short attention span, disordered sleep
2 = drowsy, disorientated, inappropriate
3 = somnolent but rousable to voice, v confused, amnesia
4 = comatose
Alcoholic Hepatitis
= inflammatory condition related to ongoing alcohol intake
Severe Alcoholic Hepatitis = 30-50% 4 week mortality
Transaminases usually >500
AST:ALT > 2
MANAGEMENT
- Alcohol abstinence + withdrawal support
- MDT approach (nutrition, ASNs)
- Maddrey’s discrimnant function can help determine if will benefit from steroids (>32)
MELD score (Model for End Stage LD) - prognostic predictor
Autoimmune Hepatitis
3 main types
1) Type 1
= positive ANA and anti-smooth muscle antibodies
- Adults & children
2) Type 2
= Anti-liver/kidney microsomal type 1 antibodies (LKM1)
- Mainly children
3) Type 3
= Soluble liver-kidney antigen
- Middle aged adults
Usually young women, with associated autoimmune conditions e.g. coeliac
Raised IgG!!!!
Autoantibodies & liver biopsy
MANAGEMENT
- Steroids or steroid-sparing immunosuppression e.g. azathioprine
- Liver transplant
Primary Biliary Cholangitis
Autoimmune condition - destruction of small intrahepatic ducts
F:M = 9:1, middle aged women
Anti-mitochondrial antibodies & IgM
Associations = RA, Sjogren’s, Systemic sclerosis, coeliac
Classically asymptomatic with isolated raised ALP
Itch/fatigue/RUQ pain/jaundice
Management of Primary Biliary Cholangitis
MDT approach
Alcohol abstinence
Pruitus = cholestyramine
Vitamins ADEK (d2 reduced fat soluble vitamin absorption)
Ursodeoxycholic acid (reduces cholestasis and can improve prognosis)
Liver transplant (esp if bili > 100)
Possible complications of PBC
Liver cirrhosis
HCC (20x increased risk)
Osteoporosis/osteomalacia
Vitamin ADEK deficiency
If have liver transplant, there is a risk of recurrence!
Primary Sclerosing Cholangitis
= Inflammatory condition of intra and extrahepatic ducts = progressive scarring and obstruction
Associations = UC (80% of PSC pts have UC), Crohn’s, HIV
Male > Female
pANCA positive
ANA and anti-smooth muscle antibodies
MCRP –> ERCP (beaded appearance)
?Liver biopsy
Management of Primary Sclerosing Cholangitis
MDT approach
Alcohol abstinence
Vitamins (ADEK)
Ursodeoxycholic acid
?Liver transplant
SCREENING
- If have IBD = annual colonoscopies
- Screening for cholangiocarcinoma
Possible complications of Primary Sclerosing Cholangitis
Chronic liver disease & cirrhosis
Cholangiocarcinoma (10% increased risk!)
Increased risk of colorectal cancer
Haemochromatosis
Autosomal recessive disorder of iron metabolism
HFE gene on chromosome 6 (variable penetrance)
Fatigue, arthralgia, sexual dysfunction (anterior pituitary function impaired in 2/3)
Multisystem
- Bronze skin
- Hypopituitarism = hypothyroid, sexual dysfunction, amenorrhoea
- Dilated cardiomyopathy
- T1DM
- CLD/cirrhosis
- Arthritis, pseudogout
+++ increased risk of HCC
How would you investigate for haemochromatosis?
Full history & examination etc
Ferritin & Iron studies
- Raised ferritin
- Raised transferrin saturation (>55% in men, > 50% in women)
- Raised iron levels
- Low TIBC
HbA1c
Liver biopsy (Perl’s Stain)
Joint xrays (chondrocalcinosis)
Echocardiogram
Genetic testing (HFE gene most common) and offer family screening
Management of Haemochromatosis
MDT approach - liver, endocrine, cardiology
Alcohol abstinence (increases iron accumulation)
Genetic counselling
Venesection (weekly to start) - according to transferrin saturation & ferritin levels
Desferrioxamine = Iron chelator
?Liver transplant
MONITORING
- Regular iron studies
- 6 monthly USS & aFP
- Monitor HbA1c
Wilson’s Disease
Autosomal recessive condition resulting in excess copper deposition in tissues
ATP7B gene on chromosome 13
Onset = 10-25 years old
Multisystem
- Asterixis, chorea, dementia/neuropsych
- Kayser-Fleischer rings
- Haemolytic anaemia
- Hepatitis –> cirrhosis
- Renal tubular acidosis
Low levels of caeruloplasmin (= copper transport in serum) = less copper in serum, more deposited in tissues
Increased urinary copper excretion
Rx = pencillamine (chelator)
Alpha 1 Antitrypsin Deficiency
Autosomal Recessive
SERPINA1 gene on chromo 14
COPD like respiratory disease (young pts with no smoking history)
Liver cirrhosis
MANAGEMENT
- Alcohol abstinence
- Stop smoking
- MDT approach
- ?recombinant alpha 1 AT
Hepatocellular Carcinoma
3rd most common cancer worldwide
CAUSES
- Chronic Hepatitis B
- Chronic Hepatitis C
- Alcohol
- Haemochromatosis
- PBC
SCREENING = 6 monthly USS +/- aFP
Offered for high risk groups e.g.
- Cirrhosis secondary to Hep B/C
- Cirrhosis secondary to haemochromatosis
- Male pt with alcoholic cirrhosis
Which cancer commonly metastasize to the liver?
Colorectal (adenocarcinoma)
Lung
Breast
Pancreatic
Gastric
Oesophageal
Most common conditions for Liver Transplant
Commonest in UK = ALD
ALD
Paracetamol OD
MASLD
Autoimmune disease e.g. PBC
Haemochromatosis
HCC
Criteria for Liver Transplant (Paracetamol)
= King’s Criteria
ABG pH < 7.3
Encephalopathy grade 3-4
PT > 100 seconds
Criteria for Liver Transplant (Non paracetamol)
All 3 of :
1) PT>100 seconds
2) Grade 3-4 encephalopathy
3) Cr >300
OR any 3 of …
- PT > 50 seconds
- Jaundice/hepatic encephalopathy for >7 days
- >10 or <40 years old
- Bilirubin >300
- Unfavorable aetiology
Complications of Liver Transplant
Surgical Complications
- Bleeding
- Post op infection
- Biliary leak
- Hepatic/portal vein thrombosis
Transplant Dysfunction
- Rejection/graft failure
- Recurrence of previous disease
Complications of immunosuppression
- Infections
- Malignancy
- CVD
Complications of Immunosuppression
Infections - Bacterial, viral, fungal
Malignancy, esp skin
Nephrotoxicity
HTN
Increased CVD risk
Causes of Pancreatitis
GET SMASHED
Gallstones
Ethanol
Trauma
Steroids
Mumps
Autoimmune
Smoking
Hypercalcaemia/Hypertriglyceridaemia
ERCP
Drugs e.g. azathioprine
Genetic causes
- Cystic fibrosis
Complications of Pancreatitis
ACUTE
- Multisystem SIRs
- Resp failure/ARDS
CHRONIC
- Pain
- T3DM
- Portal/splenic vein thrombosis
- Biliary/duodenal obstruction
- Malignancy
- Pseudocysts
Management of Chronic Pancreatitis
CONSERVATIVE
- Alcohol abstinence
- Stop smoking
- Nutrition input
MEDICAL
- Creon if evidence of malabsorption (faecal elastase) + PPI
- Analgesia, pain team
SURGICAL
- Endoscopic USS drainage of pseudocysts or AXIOS stent 6 week after initial presentation
Indication for Combined Pancreas Kidney Transplant
T1DM +/- ESRF
Pros & Cons of Combined Pancreas Kidney Transplant
PROs
+ Potentially curative of T1DM
+ Free from haemodialysis and insulin therapy
+ Reduced risk of vascular complications of diabetes (unclear if affects retinopathy yet)
CONS
- Lifelong immunosuppression (with risks of toxicity/infection/cancer)
- Risk of rejection/failure (5-7%)
- Risk of graft thrombosis
Why are transplanted pancreas now attached to the duodenum?
Previously, transplant pancreas was anastomosed to bladder
- Could monitor pancreatic activity/risk of pancreatitis through urinary amylase
However - risk of urological complications and reflux pancreatitis!
Now, most transplanted pancreas are attached to the duodenum
Complications of Chronic Kidney Disease
1) HTN & IHD
2) Anaemia (due to reduced EPO production)
3) Bone Mineral Disease
- May require parathyroidectomy for secondary hyperparathyroidism
4) Fluid retention
5) Uraemic complications e.g. pericarditis, encephalopathy
6) Calciphylaxis- painful necrotic lesions
Causes of Enlarged Kidneys
Autosomal Dominant PKD
Hydronephrosis
Renal tumour(s)
Congenital renal anomalies e.g. horseshoe kidney
Amyloidosis
Commonest causes of ESRF
Diabetes nephropathy
Hypertensive nephropathy
ADPKD
Chronic Glomerulonephritis
Obstructive uropathy
Indications for Renal Replacement Therapy
RRT should be considered once eGFR <15
1) Hyperkalaemia (if refractory to Rx)
2) Metabolic acidosis (if refractory to Rx)
3) Complications of uraemia e.g. pericarditis
4) Severe pulmonary oedema/intractable fluid overload
Contraindications to Living Donor Kidney Transplant
Uncontrolled HTN
Active Malignancy
Chronic infection
Bilateral renal artery atherosclerosis
Sickle Cell Disease
Why are Kidney Transplants placed in the pelvis?
Good blood supply
Sufficient space
Proximity to bladder for ureteric anastomosis
Easy access for biopsies/nephrostomies
Complications of AV Fistulae
Infection
- of AVF site
- Endocarditis
Thrombosis
Stenosis
- Often present with acute limb pain
Steal Syndrome
- Reduction/reversal of arterial blood flow distal to AVF = hand ischaemia
Complications of Haemodialysis
DURING USE
- Hypotension
- Disequilibrium (blood urea reduced too rapidly = cerebral oedema)
- First-Use Syndrome = reaction to dialyser membrane (mild to anaphylactic)
- Haemolysis
LONG TERM
- CVD (20X more likely to die from CVD)
- Malnutrition
- Cardiomyopathy
Burden on quality of life - hospital appointments, restricted travel
Contraindications to Peritoneal Dialysis
IBD
End-stage liver disease with ascites (SBP!)
Unrepaired abdominal hernias
Previous complex abdo surgeries
Polycystic Kidney Disease
Complications of Peritoneal Dialysis
Peritonitis
- PD fluid WBC >100
- Can be recurrent/refractory
- Staph aureus most common cause
Hyperglycaemia (glucose used as dialysate)
Catheter blockage
Fluid retention
Constipation
Hernias
Sclerosing Encapsulating Peritonitis –> risk of bowel obstruction
Outline example regime of immunosuppression for renal transplant
INITIAL
- Ciclosporin/tacrolimus with monoclonal antibody
MAINTENANCE
- Ciclosporin/tacrolimus with mycophenolate mofetil
+ regular steroids if >1 steroid responsive acute rejection episode
Side Effects of Calcineurin Inhibitors
= Ciclosporin and Tacrolimus
Nephrotoxicity (C>T)
Tremor
Hyperkalaemia
HTN & hyperlipidaemia (CVD!!)
CICLOSPORIN
- Gingival hyperplasia
- Hypertrichosis
TACROLIMUS
- PRES
- New Onset Diabetes After Transplant (NODAT)
- Alopecia
Examples of renal disease which can recur post renal transplant
IgA Nephropathy
Membranoproliferative GN
- lipodystrophy
Focal Segmental Glomerulonephritis
Membranous GN
Amyloidosis
Opportunistic Infections in Renal Transplant pts
CMV
EBV
Pneumocystis jiroveic
BK virus
- usually dormant in renal tract
- causes ureteric stenosis and interstitial nephritis
JC virus
- PML!
Autosomal Dominant Polycystic Kidney Disease
Most common cause of inherited kidney disease
Autosomal dominant
2 main types
- Type 1 = PKD1 - polycystin 1 on chromo 16
- Type 2 = PKD 2 - polycystin 2 on chromo 4
EXTRA-RENAL FEATURES
- Berry aneurysms (SAH)
- Mitral valve prolapse/regurg
- Aortic root dilatation / dissection
- Liver cysts (70%)!!!
- Diverticulitis –> perforation
- Abdominal herniae
Management of ADPKD
BP control - ACEIs
Tolvaptan = vasopressin receptor antagonist
- Can slow rate of renal function decline & cyst progression
- Offered if CKD 2 or 3 or rapidly progressive
?Nephrectomy
Plan for RRT (monitor renal function)
Indications for Nephrectomy in patients with ADPKD
1) Make room for kidney transplant
2) Chronic pain
3) Chronic renal cyst infections
4) Progression to RCC (rare)
Alport’s Syndrome
X-linked dominant condition (COL45 gene)
- abnormal type IV collagen
More severe in males
Progressive renal failure
Bilateral sensorineural deafness
Lenticonus
Retinitis pigmentosa
Rx = ACEIs and renal transplant
HOWEVER - can develop anti-GBM antibodies to transplanted kidney = Goodpasture’s syndrome
Ulcerative Colitis
Limited to colon
Continuous but superficial mucosal inflammation
Crypt abscesses on histology
Smoking improves symptoms/stopping smoking can unmask condition
80% of PSC pts have UC
Colectomy = curative
Crohn’s Disease
Mouth to anus. Perianal disease particularly key feature of crohn’s disease
Skip lesions with transmural inflammation
Granulomas on histology
Smoking exacerbates symptoms
Diagnostic criteria for Acute Colitis
= Truelove & Witts Criteria
MILD
<4 stools/day
No systemic features
Normal ESR
MODERATE
>4 stools/day
Minimal systemic features
SEVERE
>6 stools/day with blood
Systemic features e.g. fever, tachycardia
ESR >30
Management of Acute Colitis
IV fluids
IV steroids (100mg hydrocortisone QDS)
VTE prophylaxis
Avoid antispasmodics/antimotility drugs
Discuss with gastro +/- surgical team
eCXR and AXR important but if acutely unwell, consider CT AP
Indications for urgent surgical intervention in patients with severe UC
Toxic megacolon (caecum>9cm, colon > 5.5cm)
Still having >8 stools/day or CRP>45 after 3 days of IV steroids
Failure to respond to treatment after 10 days
Management of UC (mild-moderate)
Inducing Remission
- Topical +/- oral aminosalicylate
- PO corticosteroid if not responding
Maintaining Remission
- Topical and/or oral aminosalicylate
- Azathioprine if
1) Severe relapse
2) >2 exacs in past year
Factors which increase UC patient’s risk of colorectal cancer
Have had UC > 10 years
Pancolitis
Onset before age of 15
Unremitting
Poor compliance to treatment
Inducing and maintaining remission in Crohn’s Disease
Inducing Remission
- Aminosalicylates and/or steroids
- Isolated perianal disease = metronidazole
- Refractory/fistulating/severe = Infliximab
Maintaining Remission
- STOP SMOKING!
- 1st line = Azathioprine (test TMPT level first - risk of bone marrow toxicity)
- 2nd line = MTX
Hereditary Haemorrhagic Telangiectasia
A.k.a Osler-Weber-Rendu syndrome
Autosomal dominant
Diagnostic Criteria (4)
- 2 criteria = possible
- 3 criteria = definite
1) Spontaneous/Recurrent Epistaxis
2) Telangiectasias
- Typical sites = lips, oral cavity, fingers & nose
3) Visceral lesions e.g. AVMS
4) FHx (1st degree relative)
Peutz Jeghers Syndrome
= numerous benign hamartomas in GI tract and hyperpigmented macules over hands/lips/feet
Autosomal dominant
Increased risk of malignancy
- Breast
- Ovarian
- Cervical
- Pancreas
- Testicular
50% mortality from GI tract cancer by age of 50
Rx = Pan-intestinal colonoscopy every 2-3 years
Causes of Splenomegaly
1) INFILTRATIVE
- MPD, lymphoma
- Sarcoidosis, amyloidosis
- Gaucher type 1
2) INCREASE IN FUNCTION
= removal of defective of RBCs
- Hereditary Spherocytosis
- Sickle Cell disease
- Thalassaemia
OR
= immune system disordered/overactive
- Malaria
- EBV, CMV
- Visceral leishmaniasis
- Endocarditis
- Felty’s syndrome
- SLE
3) PORTAL HYPERTENSION
Causes of MASSIVE Splenomegaly
cML
Myelofibrosis
Malaria
Visceral leishManiasis
Outline the investigations you would request for work up of a patient with splenomegaly
Abdominal exam +/- rheum/thyroid exam
Bloods
- FBC, U&Es, LFTs
- TFTs
- Blood film
- Haemolysis screen e.g. LDH, haptoglobin, reticulocytes
- HIV screen
Thin and thick films x 3 if suspect malaria
USS abdomen
CT TAP (?malignancy)
Bone marrow aspirate& trephine
Hereditary Spherocytosis
Most common hereditary haemolytic anaemia in pts of Northern European descent
Autosomal dominant
- Defect in RBC cytoskeleton = spherical RBCs = destroyed by spleen sooner
Failure to thrive
Jaundice (prehepatic)
Splenomegaly
Aplastic Crisis (parvovirus infection)
Investigations for Herediary Spherocytosis
Bloods
- FBC, B12, folate, MCV, MCHC
- Blood film (spherocytes!)
- If diagnosis unclear = Cryohaemolysis test & EMA binding
(Used to do osmotic fragility test)
USS abdomen (size of spleen)
Management of Hereditary Spherocytosis
MDT approach
?Genetic counselling
Folate replacement
Splenectomy
- Indicated if:
1) Recurrent anaemia
2) Massive splenomegaly
If get splenectomy =
Need vaccinations against encapsulated organisms (pneum & meningo) prior to surgery and then prophy abx after
Chronic Myeloid Leukaemia
CML = 15% of all leukaemias
= uncontrolled myeloid proliferation
Philadelphia chromosome in 95% = translocation between 9 & 22 (BCR-ABL gene)
Anaemia
B symptoms - fever, weight loss, night sweats
Massive splenomegaly
Rx = Imatinib (inhibits BCR-ABL tyrosine kinase)
?Bone marrow transplant
Chronic Lymphoblastic Leukaemia
Commonest haematological malignancy in adults
Accumulation of mature B cells
“MATURE B cells in MATURE adults”
B symptoms
Enlarged rubbery non-tender LNs
Hepato/splenomegaly
RX
= monitoring of FBC if asymptomatic/no BM failure/splenomegaly <10cm
otherwise
= FCR (fludarabine/cyclophosphamide/rituximab)
Lymphoma
Hodgkins (presence of Reed Sternburg Cells) or Non Hodgkins
Painless lymphadenopathy
- Alcohol induced pain in NHL
B symptoms
Hepato-splenomegaly
Ann-Arbor Staging
Risk of
- Bone marrow failure (anaemia/ neutropaenia/thrombocytopaenia)
- AML transformation
- Compressive effects e.g. SVCO
