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Pastest - Abdo Flashcards

1
Q

What is the inverted J scar of liver transplantation also called?

A

The makuuchi insicion

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2
Q

What do you need to cover in patients who have liver transplant?

A

Is the transplant functioning adequately?
-Any evidence of decompensated liver disease?
-Note gynaecomastia and/or dupytrens will persist post transplant and does not represent decompensation
-Severe splenomegaly may not fully resolve

Any noticeable evidence of immunosuppression
-Any seborrhoeic warts, aktinic keratoses or evidence of scars from skin malignancies?
-Steroids could be indicated by insulin injection sites or evidence of glucose monitoring

Any evidence of underlying disease?
-Venesection (haemochromatosis), xanthelasma (PBC) etc

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3
Q

What are the conditions that may require liver transplantation?

A

Cirrhosis
-ALD, NAFLD, Chronic autoimmune hepatitis, haemochromatosis,

HCC

Acute fulminant liver failure

Variant syndromes:
-Diuretic resistant ascites
-Chronic hepatic encephalopathy
-Intractable pruritis
-Hepatopulmonary syndrome
-Polycystic liver disease
-Recurrent cholangitis

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4
Q

What is the definition of fulminant liver failure?

A

Multisystem disorder with dysfunction and encephalopathy within 8 weeks of symptom onset and no evidence of underlying liver disease

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5
Q

What is the criteria for elective liver transplantation?

A

Chronic liver disease/ failure and high UK end stage liver disease score (UKELD) 49 or more
-Derived from Na, Creat, Bili, and INR

Patients with portal pulmonary hypertension should have a clinically significant response to longacting prostacyclin analogues or there alternatives like bosentan/ sildenafil.

Patients with severe alcoholic hepatitis may be considered if expected survival with transplantation is more than the expected survival without.
-Maddrey or glasgow hepatitis score can be used to prognosticate

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6
Q

What are the causes of ascites?

A

Portal hypertension secondary to cirrhosis (most common)

Vascular
-Bud chiari, CCF, constrictive pericarditis

Low albumin
-Nephrotic syndrome or protein losing enteropathy

Peritoneal disease
-Meig’s syndrome, infectious peritonitis (TB or fungal disease), malignant disease

Misc
-Pancreatic, chylous, advanced hyperthyroidism

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7
Q

SAAG >/=1.1 causes?

A

Portal hypertension with 97% accuracy

Cirrhosis
CCF
Nephrotic syndrome
Bud chiari
Meig’s

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8
Q

SAAG <1.1 causes?

A

Peritoneal carcinomatosis
TB
Pancreatitis

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9
Q

What is the differential diagnosis of the underlying cause of transplanted kidney (ESRF)?

A

Unknown (26%)

Diabetes (20%)
-Insulin injection sites, visual impairment, diabetic foot, BM sticks/glucometer at bedside

Glomerulonephritis (11%)
-butterfly rash of SLE, purpura of vasculitiides or cryoglobulinaemia, scleroderma, RA, alopecia, vitiligo, cushingoid (in absence of graft), hearing impairment (alports, GPA), Stigmata of infective endocarditis

Renovascular disease (8%)
-signs of extrarenal emboli or advanced atherosclerosis (e.g. focal neurological deficits, previous amputation, ischaemic foot ulcer, dyslipidaemia)

Pyelonephritis (8%) - nephrectomy scar

HTN (6%)

ADPKD (6%)
-Hepatomegaly 50-70% (Usually women and severe renal disease)
-Focal neurological deficit (intracranial aneurysms (8%)

Other (15%)

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10
Q

What are the causes of transplanted kidney graft failure?

A

Hyperacute
-Immediate and antibody related. Complement mediated small vessel thrombosis and graft ischaemia

Acute
-T cell mediated destruction of the graft (mostly IL-2 mediated). Responds to immunosuppression.
-May have multiple episodes

Chronic
-Antibody mediated graft destruction continues insidiously. Arterial endothelium is involved, resulting in ischaemia and fibrosis.

Disease recurrence (5% of graft loss)

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11
Q

Disease recurrence in transplanted kidney can happen in all diseases except…

A

Alport’s disease
Polycystic kidney disease
Hypertension
Chronic pyelonephritis
Chronic interstitial nephritis

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12
Q

What is included in a renal screen?

A

Urine albumin or protein / creatinine ratio

Urinalysis

ANA, ENA, DsDNA, pANCA, cANCA

Complement

Creatine kinase

Infectious screen: ASO titre, thick and thin blood films (malaria), blood cultures (IE)

Cryoglobulinaemia

Chest radiograph: pulmonary renal syndromes

Renal tract ultrasound

Renal biopsy

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13
Q

What are the causes of blood in the urine in the context of renal failure?

A

Papillary nephropathy
-Causes are POSTCARD

Pyelonephritis
Obstruction
Sickle-cell disease
TB
Cirrhosis
Analgesic nephropathy
Renal vein thrombosis
Diabetes

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14
Q

How do you differentiate between pre-renal causes of AKI and ATN?

A

Pre-renal cause
-Urine sodium <10 mmol/L
-Urine osmolality >500 mosmol/L
-Urine: plasma Osmolality >1.1

ATN
-Urine sodium >10 mmol/L
-Urine osmolality <400 mosmol/L
-Urine: plasma osmolality <1.1

Note in the early stages of renal failure due to prerenal causes tubular concentrating power is retained, whereas in ATN it is lost. Prerenal insults will eventually result in ATN, making this distinction impossible.

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15
Q

What are the symptoms and signs of post-transplant lymphoproliferative disease?

A

Weight loss, anaemia, lymphadenopathy, fever, GI symptoms, glandular fever syndrome

The term PTLD incluees many tumours, ranging from B-cell hyperplasia to immunoblastic lymphoma. Overwheling majority are associated with EBV infection.

All cases carry a high mortality. Around 50% of cases occur in the first year after transplantation and have a poorer prognosis.

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16
Q

What are the signs of a failing renal transplant?

A

Signs of fluid overload
HTN
Tenderness over the graft (suggesting chronic rejection)
Evidence of actively used means of vascular access (tunnelled line, fistula and needling)
Uraemia (excoriation, uraemic pericarditis)
Cushingoid features (steroid use)

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17
Q

What malignancies occur with increased frequency after transplantation?

A

Skin and lip squamous carcinomas
Lymphoma
PTLD

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18
Q

What are the clinical signs of ciclosporin taking?

A

Gum hypertrophy (rare)

Sebaceous gland hypertrophy and acne (especially in males)

Alopecia

Tremor

Hirsutism

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19
Q

What are the immunosuppressants used in renal transplants?

A

Ciclosporin
-Polypeptide of 11 amino acids of fungal origin
-Acts through calcineurin inhibition thus preventing production of IL-2 and T-helper cell recruitment and activation

Tacrolimus
-Macrolide antibiotic that also works through calcineurin inhibition

Corticosteroids
-Inhibit all stages of T-cell maturation and activation
-Also prevent IL-1 and IL-6 production by macrophages

Azathioprine
-Anti-metabolite derivative of 6 mercaptopurine
-Reduces DNA and RNA synthesis, thus limiting immune cell turnover

Basiliximab and Dacilzumab
-Humanised monoclonal antibodies that target teh IL-2 receptor.

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20
Q

What are the options for RRT and catheter types?

A

AV fistulae
-The preferred routes of vascular access for haemodialysis

Permanent cuffed tunnelled catheters
-Right internal jugular > left sided
-Look for multiple scars at both exit sites (over the upper chest) and incisions at the base of the neck

Subcutaneous “buttonhole” ports
-These dialysis catheters have terminals lying just beneath the skin. The skin re-grows over the port between sessions, reducing the likelihood of infections

Dacron / PFTE grafts
-These have a more uniform shape and a less distensible texture on palpation compared with AV fistulae.
-They are often sited in the groin if arm sites have been exhausted by multiple previous fistulae

Tenckhoff catheters
-These are usually sited just above the umbilicus in the midline. Used for peritoneal dialysis.

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21
Q

What are the preferred place (in order of preference) for AV fistula for dialysis?

A

Wrist of non-dominant arm
Cubital fossa of non-dominant arm
Wrist of dominant arm
Cubital fossa of dominant arm

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22
Q

What are the complications of previous dialysis methods?

A

Horner’s syndrome: multiple line insertions

Dilated superfical veins: distinctive superficial varicosities over the chest wall from multiple central venous stenoses or thromboses

Digital ischaemia: radio-brachiochephalic steal syndromes. Particularly in patients with diabetes

Abdominal hernias: About 15% of patients on long term peritoneal dialysis.

B2-microgobulin amyloidosis
-earliest manifestation is carpal tunnel syndrome

Malnutrition and protein loss

Bacterial peritonitis

Abdominal Hernia

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23
Q

What are the advantages and disadvantages of continuous ambulatory peritoneal dialysis?

A

Advantages
-Simple to learn
-Ambulatory
-Haemodynamic tolerance
-Fer dietary restrictions

Disadvantages
-Time consuming exchanges
-Peritonitis
-Protein loss
-Excessive glucose load
-Sterile technique needed
-Peritoneum vulnerable to injury.

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24
Q

How can you differentiate kidney from liver/ spleen?

A

Can get above it
Ballotable
Not notched
Irregular surface
Minimal movement inferiorly with respiration
Resonant to percussion due to overlying bowel (i.e. retroperitoneal)

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25
Q

What are the differentials for polycystic kidney disease?

A

Renal cell carcinoma
-Lymphadenopathy and cachexia

Hydronephrosis
-Smooth enlargement only in severe cases
-Associated palpable bladder suggests BOO

Adrenal mass
-Phaeochromocytoma and adrenal carcinoma

Retroperitoneal soft-tissue tumours

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26
Q

What are the causes of renal cysts?

A

Tuberous sclerosis
-20% develop cysts but less than 5% severe disease with HTN and ESKD

Von hippel-lindau disease
-Inherited mutation of the VHL tumour suppressor gene gives rise to cyst formation in the kidney, pancreas, liver and epididymis
-70% of patients develop RCC
-The oncogenesis of RCC in genotypically normal patients involves mutation of VHL gene in 50% of patients

ADPKD:

Autosomal recessive polycystic disease

Simple cysts (benign) and medullary cystic disease (which leads to progressive renal failure)

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27
Q

What is the pathogenesis of ADPKD

A

Defects in PKD1 and PKD2 genes give rise to faulty polycystin proteins but a “second hit” acquired mutation is required in the normal allele to give rise to cyst formation (hence the onset in adulthood)

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28
Q

What is the pathogenesis of autosomal recessive polycystic disease?

A

The gene for fibrocytin (PKHD1) is defective, resulting in faulty embryogenesis of the collecting tubule (and biliary defects invariably resulting in liver disease).

Neonatal presentation is with pulmonary hypoplasia secondary to oligohydramnios.

Childhood presentation is similar to adult polycystic disease, with hypertension and renal insufficiency.

29
Q

What disorders are associated with autosomal dominant polycystic kidney disease?

A

Liver cysts occur in up to 70% of cases but significant involvement with hepatomegaly are almost entirely restricted to female patients with severe renal disease

Intracerebral aneurysms (15% prevalence)
-Relative incidence of SAH is 4x greater than general population (no evidence for screening)

HTN
-Almost universal when kidneys are palpable
-Detectable in 70% of adults prior to cysts being detectable on USS

Mitral valve prolapse (25% prevalance)
-Always say to examiner you would like to examine precordium)

30
Q

What is included in liver screen?

A

Viral hepatitis serology
-Hep B surface antigen (HbsAg), Hep C antibody (HCV IgG)

Metabolic disorder screen
-Haemachromatosis
-Ceruloplasmin
-a1 antitrypsin level

Autoimmune liver disease
-PBC - AMA, ANA
-AIH - ANA, ASMA, ALKMA, ASLA, RF

Liver biopsy

31
Q

Explain the child pugh score

A

Bilirubin
-<34 = 1, 34-50 = 2, >50 = 3

Albumin
->35 = 1, 28-35 = 2, <28 = 3

PT
<4 = 1, 4-6 = 2, >6 = 3

Encephalopathy
-Non = 1, Grade 1 = 2, Grade 2/3 = 3

Ascites
-None = 1, Moderate = 2, Severe/ diuretic resistant = 3

Class A patients have a >90% 6 month survival rate, whereas >12 have a 40% 6 month survival rate

32
Q

What is the management of ascites?

A

Initial paracentesis followed by
-Salt restriction <2g/day
-Spironolactone (urine sodium > urine potassium demonstrates adequate natiuresis)
-If adequate natiuresis is achieved and ascites persists then add furosemide
-Fluid restriction unnecessary unless serum sodium <125mmol/L
-Patients should be considered for liver transplantation if appropriate

Diuretic resistant ascites occurs when renal impairment prohibits increasing diurestics or ascites simply does not resolve despite maximum therapy.

Serial paracentesis and TIPSS are potential therapeutic options while liver transplantation is expedited.

Surgically fitted pumps can divert ascitic fluid into the bladder at a programmable rate and may provide significant symptom relief in patients ineligible for transplantation.

33
Q

What is the treatment for variceal haemorrhage?

A

Terlipressin, IV antibiotics, large bore intravenous access and resuscitation

Urgent endoscopy with banding

Serial variceal banding until obliterated, with regular surveillance ehterafter

If endoscopy fails to control bleeding, consider TIPSS

Regular B blocker aiming for HR 55-60

Consider transplantation if appropriate

34
Q

What are the symptoms and signs of PBC?

A

Those of chronic liver disease but also:
-Middle aged woman
-Xanthelasma
-Jaundice
-Excoriations
-Sicca syndrome (xerophthalmia, xerostomia)

35
Q

What are the investigations of PBC?

A

AMA (for diagnosis) and AMA subtype for prognosis)
-Liver biopsy is not necessary if above are positive because their specificity and sensitivity are >95%

Anti-gastric parietal cell antibodies are positive in 60% of cases, ANAs in 20% and ASMAs in 50%

It is also worth mentioning you would have a low thresshold for testing other associated autoimmune conditions, including
-Hashimotos, systemic sclerosis, sjogrens, coeliac

36
Q

What is the management of PBC?

A

Cholestasis is treated with ursodeoxycholic acid
-Delays progression to cirrhosis
-Improves biochemical markers of cholestasis
-Improves symptoms of lethargy and pruritis

Pruritis is initially treated with cholestyramine (bile acid sequestrant) and antihistamines but for many pts this is inadequate
-Oral naltrexone (second line),
-Rifampicin helps but causes decline in liver function
-Plasmapheresis and molecular absorbent recycling system have been used to varying efficacy in severe cases
-Severe uncontrollable pruritis is an indication for transplantation

Hypercholesterolaemia can be treated with statins

Osteoporosis occurs independently to vitamin D malabsorption. Calcium supplements and bisphosphonates are mainstays of treatment.

Fat soluble vitamin deficiency treated with replacement

37
Q

What indicates a poorer prognosis in PBC?

A

Patients with high bilirubin level

Females compared with males

High anti-M2 and anti-M4 AMA subtype concentrations.

(patients with only anti-M9 AMA subtype tend to run a benign, non progressive course)

38
Q

What features point towards a diagnosis of hereditary spherocytosis?

A

Middle-aged (or older) men - twice as common in men as woman

Slate-grey skin appearance

Evidence of arthritis

Evidence of diabetes (look for insulin injection sites)

Evidence of cardiomegaly (displaced apex, signs of congestive cardiac failure)

Venesection marks in the cubital fossae

39
Q

What are the differential diagnoses for hereditary haemochromatosis (HH)?

A

Autosomal dominant: ferroportin deficiency

Autosomal recessive:
-HH due to HFE mutations (most common)
-HH due to transferrin receptor 2 mutations
-Juvenile haemochromatosis due to hepcidin mutations
-Juvenile haemochromatosis due to haemojuvelin mutations
-Iron overload secondary to transferrinaemia
-Iron overload secondary to ceruloplasminaemia

Acquired:
-Thalassaemia
-Myelodysplasias
-Dyserythropoietic anaemias
-Sickle cell disease
-Red cell enzyme deficiencies
-Multiple blood transfusions

40
Q

What initial tests do you use to diagnose hereditary haemochromotosis?

A

State that you would initially request indirect serological markers of iron stores:
-Fasting serum free iron
-TIBC
-Ferritin level

The fasting free iron and TIBC determine the transferrin saturation; a value of >50% has a sensitivity of 92% and specificity of 93%. If the cut off is lowered to 45%, the sensitivity increases to 100%, but this also detects liver disease associated with secondary iron overload such as alcohol related liver disease, hep C and NASH.

41
Q

When do you test for HFE gene status?

A

A raised (>50%) transferrin saturation and/or raised ferritin (>1000 ng/mL) warrant HFE gene status testing.

Of all patients with iron overload
-90% will be homozygous for the C282Y mutation
-5-6% will be compound gererozygotes for the C282Y and H63D mutations
-The remainder will have ‘non-HFE’ associated iron overload.

Note that half of cirrhotic patients with HH will have normal LFTs. Therefore liver biopsy was previously recommended in patients >40 years or those with raised transaminases in order to rule out other causes of liver disease however now MRI assesses both both fibrosis and iron content.

42
Q

What tests in hereditary haemochromatosis look for extrahepatic disease?

A

Fasting glucose
LH, FSH, testosterone level
AP radiography of both hands (arthritis)

43
Q

What is the management of haemochoromatosis?

A

Regular phlebotomy aiming for ferritin level of 50ng/ml.
-Hypogonadiam and arthopathy tend not to improve with phlebotomy, whereas insulin requirements may fall and cardiac function improve.

Fibrosis does not reverse after phlebotomy and the risk of HCC remains. HCC accounts for 30% all deaths so regular USS and a-fetoprotein is necessary.

Decompensated liver disease and early HCC are indications for transplantation.

anti-arrhythmics and ICD in cardiac dysrhythmias

44
Q

What are the extrahepatic manifestations in hereditary haemochromatosis?

A

Skin hyperpigmentation (70%)
Hypogonadism (50%)
Amenorrhoea (15%)
Diabetes Mellitus (50%)
Arthopathy (70%)
cardiomyopathy (15-30%)
ECG abnormalities/ arrhythmias (35%)

45
Q

What are the physical signs of alcoholic liver disease?

A

As for chronic liver disease and:
-parotid swelling and squaring of the jaw
-Dupuytren’s contractures
-Peripheral neuropathy (tell the examiner you would like to exclude this)
-Facial flushing

3-4 times for likely to be a man than a woman.

46
Q

What is marchiafava-bignami disease?

A

Necrosis of the corpus callosum

The key sign is apraxia of the non-dominant hand indicating interhemispherical disconnection. It is associated with alcohol use.

47
Q

What are the physical signs of chronic viral hepatitis?

A

As for chronic liver disease but also:
-Signs of current injecting drug use
-Tattoos/ piercings
-Age and nationality
—-e.g. Hep b - south east asia, china, sub saharan africa, amazon basin, canada, greenland.

48
Q

What are the extrahepatic manifestations of hepatitis B?

A

Polyarteritis nodosa

Glomerulonephritis and nephrotic syndrome

Arthritis dermatitis syndrome

Palpable purpura

49
Q

What are the extrahepatic manifestations of hepatitis C?

A

Essential mixed cryoglobulinaemia
Lymphoma
Glomerulonephritis
Porphyria cutanea tarda
Diabetes mellitus
Autoimmune phenomena
Peripheral neuropathy

50
Q

What are the physical signs of autoimmune hepatitis?

A

As for chronic liver disease but look for:
-Usually a middle aged female
-Signs of thyroid disease
-Signs of rheumatoid arthritis
-Signs of scleroderma

51
Q

What are the investigations for autoimmune hepatitis?

A

Liver biopsy
-Most important test when AIH is suspected.
-There is a high degree of overlap with other conditions (PBC, PSC, autoimmune cholangitis), which can be differentiated only histologically. It also assesses severity and is used after treatment to determine whether remission has been achieved.

Autoantibodies
-Type 1- ANA, Anti-smooth muscle antibody
-Type 2- Anti liver kidney microsomal
-Type 3- Anti-soluble liver antigen

Serum electrophoresis and immunoglobulin

Transaminases to monitor response to therapy

Exclude other autoimmune conditions.

52
Q

How does the epidemiology of the 3 types of AIH differ?

A

Type 2 is rare in adults

Type 1 and 3 have similar responsiveness to steroids, female predominance (80%), rates of other autoimmune disease (40%) and raised polyclonal gamma-globulin

Type 3 is however more likely to progress to cirrhosis

53
Q

What other autoimmune diseases can occur with AIH?

A

AIH should be regarded as a multisystem disorder associated with a plethora of autoimmune diseases. These can be divided into intrahepatic (‘overlap’) and extrahepatic.

Intrahepatic
-PBC (7%)
-PSC (6%)
-Autoimmune cholangitis
-Antibody negative AIH

Extrahepatic
-Rheumatological (RA, Sjogren’s, systemic sclerosis)
-Endocrine (Graves’, autoimmune thyroiditis, Juvenile diabetes melitus)
-Gastrointestinal (Coeliac, IBD)
-Haematological (haemolytic anaemia, pernicious anaemia)
-Cardiological (pericarditis, myocarditis
-Renal (glomerulonephritis)

54
Q

What are the indications for treatment in AIH?

A

AST more than 10 fold upper limit of normal or more than 5 fold with an IgG level more than 2 fold normal.

Bridging or multi-acinar necrosis on liver biopsy

Raised AST below criteria but symptomatic (fatigue, arthralgia, jaundice)

55
Q

What is the treatment for AIH?

A

Corticosteroids and azathioprine

Reducing dose steroid used to initiate therapy, followed by maintanence low dose steroid with azathioprine or monotherapy depending on tolerance and side effects.

Approximately 65% of patients respond to initial therapy and enter histological remission. 80% will relapse within 3 years if treatment withdrawn so some advocate for lifelong treatment.

56
Q

What are the physical signs to detect in isolated hepatomegaly without stigmata of chronic disease?

A

Face
-Flushing: carcinoid
-Pale conjunctivae: anaemia of chronic disease (amyloid, sarcoid) or malignancy

Hands:
-Clubbing

Neck
-JVP raised/giant V waves
-Lymphadenopathy, Virchow’s node

Precordium
-S3 or murmur of tricuspid regurgitation

Abdomen
-Pulsatile hepatomegaly (tricuspid regurg)
-Smooth hepatomegaly (sarcoid, amyloid)
-Irregular (malignant infiltration, polycystic, hydatid cysts)
-Tender (acute hepatitis, Budd-Chiari syndrome)
Lymphadenopathy: (malignancy)
-Ascites: (malignant, cardiac failure, Budd-Chiari syndrome)
-Sister Mary Joseph Nodule

Other:
-Cachexia: malignancy
-Fever: amoebiasis
-Ankle oedema: CCF

57
Q

What are the differentials of isolated hepatomegaly without stigmata of chronic liver disease (i.e. cirrhosis)?

A

Most common:
-CCF (+/- tricuspid regurg)
-Metastatic malignancy

Other rarer
-Budd-chiari
-Hydatid cyst
-Riedel’s lobe
-Polycystic liver disease (usually with accompanying renal enlargement)
-Sarcoidosis
-Amyloidosis
-Carcinoid

58
Q

What is the differential diagnosis for ascites without stigmata of chronic liver disease?

A

Non cirrhotic ascites is usually due to cardiac failure or intra-abdominal malignancy.
In younger patients consider nephrotic syndrome

Rare causes include
-Budd-chiari syndrome - look for jaundice and hepatomegaly
-Tuberculous peritonitis - look for lung signs
-Chylous ascites due to lymphatic obstruction

59
Q

What are the most common primary tumours leading to malignant ascites?

A

Ovarian (most common)
Endometrial
Breast
Colon
Gastric
Pancreatic

60
Q

What are the investigations for ascites without stigmata of chronic liver disease?

A

Serum-ascites albumin gradient (SAAG)
- >1.1 g/dL indicate portal hypertensive ascites while values <1.1 indicate non-portal hypertensive causes (most often infection or malignancy). This has almost 100% accuracy in discriminating between these two aetiological categories

Cell count: lymphocytic infiltrate suggest TB or carcinomatous peritoneal disease, whereas neutrophils suggest bacterial peritonitis

Culture/ gram stain

Cytology: if adequate (approx 500ml) fluid taken cytology has up to 75% sensitivity in detecting malignant cells

Other useful investigations:
-ECHO
-CT for malignant disease
-Tumour markers
-Breast Exam +/- mammography
-Diagnostic laparoscopy

61
Q

What are the physical signs to look for in someone with hepatosplenomegaly/ splenomegaly without stigmata of chronic liver disease?

A

Hands: Rheumatoid arthritis (Felty’s syndrome)

Head and neck:
-Lymphadenopathy
-Alopecia (recent chemo)
-Pale conjunctivae (anaemia due to bone marrow failure)
-Tunnelled catheter (for chemotherapy)
-Jaundice (haemolysis)
-Butterfly rash (SLE)

Abdomen
-Smooth enlargement of liver and spleen
-Lymphadenopathy

Other:
-Petechial rash
-shingles
-Bruises
-Evidence of bone marrow biopsy over iliac crest

62
Q

What are the causes of hepatosplenomegaly/ splenomegaly?

A

HAEMATOLOGICAL
-Myeloproliferative (CML, AML, Myelofibrosis)
-Essential thrombocytosis
-Polycythaemia rubra vera
-Lymphoproliferative (CLL, ALL) - marked lymphadenopathy
-Lymphoma

INFECTIVE
Worldwide:
-Malaria
-Kala-azar (abdominal leishmaniasis)
-Schistosomiasis
-Tuberculosis

UK
-Brucellosis (agricultural worker, neurological signs, skin rash)
-Leptospirosis with Weil’s syndrome (jaundice, fever, muscle tenderness, positive Murphy’s)
-Toxoplasmosis (cervical lymphadenopathy)
-Infectious mononucleosis (lymphadenopathy)

STORAGE DISORDERS
-Gaucher’s disease (bony deformity, previous fractures)
-Glucogen storage disease (small build, hypotonia, muscle atrophy)

INFLAMMATORY
-Felty’s syndrome (look for rheumatoid features)
-Amyloid (look for evidence of chronic inflammatory disease)

63
Q

How can you divide causes of hepatosplenomegaly/ splenomegaly depending on size of organomegaly?

A

Mild (spleen just palpable)
-Infectious mononucleosis
-Infective endocarditis

Moderate (up to four finger-breadths)
-Myeloproliferative disorders
-Lymphoproliferative disorders
-Cirrhosis and portal hypertension

Massive
-CML
-Myelofibrosis
-Malaria
-Kala-azar

64
Q

What are the physical signs of polycythaemia rubra vera?

A

Middle aged/ elderly patient
Splenomegaly/ hepatosplenomegaly
Facial plethora
Scratch mark
Hypertension
Enlarged conjunctival vessels
(dilated retinal veins on fundoscopy)
Evidence of venesection
Gout

65
Q

What are the diagnostic criteria for polycythaemia rubra vera?

A

WHO criteria: 2 major and 1 minor OR first major and 2 minor

Major criteria
-Hb >18.5 g/dL (men), >16.5 g/dL (women) or >25% normal predicted red cell mass
-Janus kinase 2 (JAK2) mutation

Minor criteria
-Bone marrow biopsy showing hypercellularity with prominent erythroid, granulocytic and megakaryocytic proliferation
-Serum erythropoietin level below normal range
-Endogenous erythroid colony formation in vitro

Measuring red cell mass with Cr and plasma volume with iodine labelled albumin confirms true erythrocytosis and remains tenet of PRV. This will exclude relative erythrocytosis (caused by states of reduced plasma volume, e.g. diarrhoea) but does not exclude other secondary causes of erythrocytosis

66
Q

What are the physical signs of hereditary spherocytosis?

A

Look for the triad of: splenomegaly, jaundice, pallor (anaemia)

Patient usually of north European origin

Other typical features:
-Cholecystectomy scar
-Splenectomy scar
-Leg ulcers

67
Q

What are the 3 phenotypes of hereditary spherocytosis?

A

Mild:
-Mild HS occurs in 20% of cases. Rarely have signs. Usually picked up by family screening

Moderate:
-Accounts for 75% of all HS cases
-Classic triad of splenomegaly, jaundice and anaemia
-Autosomal dominant

Severe:
-Occurs in 5% of patients
-Usually have undergone splenectomy but response will be incomplete
-Recessive inheritance

68
Q

What is the underlying aetiology of hereditary spherocytosis?

A

a number of red cell membrane proteins may be deficient or inadequately integrated. The most common defect in autosomal dominant HS is B-septrin deficiency. This results in a characteristic spherical structure of erythrocytes, which are rapidly cleared in the spleen leading to (extravascular) haemolysis and splenic hypertrophy.

69
Q
A

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