EFA/ Haematology Tutorial

Question 1

What is this? What causes this?

Answer 1

Nutmeg liver
HF: pressure increases in central vein
Pattern of blood going from middle of the lobule to the 6 corners

Question 2

A 67M presents to A+E disoriented. He is unkempt + smells of alcohol. OE he is jaundiced, has an enlarged abdomen + spier naevi on his chest. He demonstrates a liver flap.
What is the most likely cause of his condition?
A. Hypercalcaemia
B. Hyperammonaemia
C. Hyperkalaemia
D. Hyponatraemia

Answer 2

B. Hyperammonaemia
High level of ammonia poisons you, causing encephalopathy + liver flap

Question 3

Hb 80
MCV 102
Reticulocyte count 257 (50-100)
BR 33 (<17)
ALT 50 (5-42)
ALP 178 (100-300)
LDH 657 (200-450)
Creatinine 72 (60-125)
Hb 80
MCV 102
What is the most likely explanation for these lab results?
A. Acquired haemolytic anaemia
B. Transfusion associated GvHD
C. Obstrutive jaundice caused by pigment Gallstones
D. Transfusion transmitted Hepatitis E
E. Pernicious anaemia

Answer 3

A. Acquired haemolytic anaemia
LDH + BR are high

Incorrect options because:
B: severely immunocompromised state- pancytopenia
C. BR would be conjugated
D. Hepatic jaundice picture: raised transaminases + BR
E. MCV would be much higher

Question 4

Summarise pre-hepatic jaundice

Answer 4

Excessive red cell breakdown- overwhelms Liver’s ability to conjugate BR
Unconjugated hyperbilirubinaemia
Any BR that is conjugated will be excreted normally

Question 5

Give 2 differential diagnoses for pre-hepatic jaundice

Answer 5

Haemolytic anaemia

Question 6

Summarise hepatic jaundice

Answer 6

Dysfunction of hepatic cells
Lose ability to conjugate BR/ cirrhotic liver compresses biliary tree causing obstruction
Unconjugated + conjugated BR in blood

Question 7

Give 6 differential diagnoses for hepatic jaundice

Answer 7

Alcoholic liver disease
Viral hepatitis
Iatrogenic e.g. drugs
AI hepatitis
PSC/ PBC
Hepatocellular carcinoma

Question 8

Summarise post hepatic jaundice

Answer 8

Obstruction of biliary drainage.
Conjugated hyperbilirubinaemia

Question 9

Give 3 types of differential diagnosis for post hepatic jaundice

Answer 9

Intra-luminal: Gallstones
Mural: cholangiocarcinoma, strictures, drug induced cholestasis
Extra-mural: pancreatic cancer/ abdo masses e.g. lymphoma

Question 10

In which disease states do you see coca cola dark urine?

Answer 10

Conjugated hyperbilirubinaemia
Mixed hyperbilirubinaemia
(as conjugated hyperbilirubinaemia is water soluble + can be excreted in urine)

Question 11

In which disease states do you see pale stools? Why is this?

Answer 11

Post hepatic, Obstructive jaundice
Reduced levels of stercobilin entering the GIT (which usually gives colour to stool)

Question 12

In a peripheral film spherocytes are seen in which scenario? What causes this?
A. Microangiopathic haemolytic anaemia
B. Folate deficiency anaemia
C. DAT positive hereditary spherocytosis
D. G6PD associated acute haemolysis
E. DAT positive AI haemolytic anaemia

Answer 12

E. DAT positive AI haemolytic anaemia

Immunoglobulin bind RBC as passes through spleen, spleen recognises Ig bound to antigen- damages RBC membrane
RBC seals itself again, but with loss of membrane for same intracellular vol constricting it into a small spherocytic shape

Question 13

What is the mechanism causing spherocyte formation in hereditary spherocytosis?

Answer 13

Mutation in one of the proteins involved in red cell membrane construction: Ankyrin, Spectrin
NO immune mediated damage

Question 14

What does the DAT test determine?

Answer 14

Whether normal RBCs (with no Ig bound to them)
OR
Whether RBCs are bound to by immunoglobulin in patients producing antibodies against the red cell membrane

Question 15

In which condition are ghost cells seen?

Answer 15

G6PD associated acute haemolysis

Question 16

What is seen on the blood film in a patient with folate deficiency anaemia?

Answer 16

Oval macrocytes

Question 17

In a 25F who is symptomatic from AI haemolytic anaemia which is the most appropriate first line treatment?
A. Prednisolone
B. Transfusion of Group O RhD -ve blood
C. Folic acid supplements
D. Cyclosporin A
E. Ecluzimab (anti-complement) monoclonal antibody

Answer 17

A. Prednisolone
Fast acting, will suppress immune response + allow reduction of haemolysis

NO transfusion- even universal cells will be subject to same pathogenic mechanism
Cyclosporin A could be option longer term but is not rapidly acting

Question 18

DAT positive acquired haemolytic anaemia may be seen in association with which other haematological disorders?
A. CML
B. Polycythaemia Vera
C. Hereditary spherocytosis
D. CLL
E. Hereditary spherocytosis

Answer 18

D. CLL
Prone to forming autoantibodies to RBCs

CML + PV: myeloid disorders, no a/w immune mediated haemolysis

Question 19

In a 29M with bilateral cervical lymphadenopathy which disorder would NOT be in the differential diagnosis list?
A. Acute EBV infection
B. Follicular non-Hodgkin lymphoma
C. Multiple myeloma
D. Acute HIV infection
E. TB

Answer 19

C. Multiple myeloma
NEVER has lymphadenopathy
MM is a disease of BM with abnormal protein affecting kidneys, + with proliferation of BM itself affecting bones + spinal cord

Question 19

In a 29M with bilateral cervical lymphadenopathy which disorder would NOT be in the differential diagnosis list?
A. Acute EBV infection
B. Follicular non-Hodgkin lymphoma
C. Multiple myeloma
D. Acute HIV infection
E. TB

Answer 19

C. Multiple myeloma
NEVER has lymphadenopathy
MM is a disease of BM with abnormal protein affecting kidneys, + with proliferation of BM itself affecting bones + spinal cord

Question 20

Which of the following conditions is NOT associated with a widened mediastinum?
A. Teratoma
B. Thymoma
C. Hodgkin lymphoma
D. B cell ALL
E. B cell non-hodgkin lymphoma

Answer 20

D. B cell acute ALL
Lymphoblasts fill BM, eventually spill into peripheral blood
Become severely unwell, without tx, die of sepsis/ bleeding
Don’t survive long enough to build up a bulky lymphoid infiltration in the LN, Spleen or LN in mediastinum

Question 21

What phrase can be used to remember the causes of a large mediastinal mass?

Answer 21

3 Ts: Teratoma, Thymoma + Terrible lymphomas
(Hodgkins, T cell lymphoblastic, T cell lymphomas involving thymus, B cell non-hodgkin)

Question 22

In a 28F with classical hodgkin lymphoma, nodular sclerosing subtype, ann arbor stage 1A with disease confined to the left cervical nodes which of the following statements is true:
A. Does not require treatment
B. Chemotherapy and radiotherapy will achieve a cure rate >80%
C. Treatment will make her infertile
D. PET CT scan is not required after beginning treatment
E. Chemotherapy only will achieve a cure rate >80%

Answer 22

Chemotherapy only will achieve a cure rate >80%
(limited disease)

Standard tx will not affect fertility
PET CT is required to determine whether complete remission or whether radiotherapy required

Question 23

Which lymphomas DON’T require urgent treatment?

Answer 23

Indolent lymphomas e.g. Follicular lymphoma or even CLL

Question 24

Which therapy is no longer used first line in patients with hodgkins lymphoma?

Answer 24

Radiotherapy
Although combo has higher remission rates, there are higher rates of secondary cancers due to receivign 2 DNA damaging therapies

Question 25

In a 40F splenomegaly + generalised lymphadenopathy which is the most likely haematological dx?
FBC:
WBC: 120.3 x 10^9
Hb: 120 g/l.
Platelets: 130 x 10^9

A. B cell non-hodgkin lymphoma
B. Acute lymphoblastic leukaemia
C. Chronic myeloid leukaemia
D. Chronic lymphocytic leukaemia
E. Hodkins lymphoma

Answer 25

D. Chronic lymphocytic leukaemia

WCC 120 much more likely leukaemia than lymphoma
A: involves nodes, but not usually BM + blood
B: Present acutely- die or get tx- don’t survive long enough to build up splenomegaly + gen lymphadennopathy
C. Myeloid- hepatomegaly + splenomegaly. NO lymphadenopathy
E. disease of LN, rarely involves blood

Question 26

Massive hepatosplernomegaly
Lymphadeopathy
Abnormal blood count: v high WCC, smear cells
Over years Hb and platelets start to fall
What is the likely diagnosis?

Answer 26

Chronic lymphocytic leukaemia

Question 27

Massive hepatosplenomegaly
Abnormal blood count
Hb + platelets preserved
V high WCC: neutrophils, myelocytes, basophils
No lymphadenopathy
What is the likely diagnosis?

Answer 27

Chronic myeloid leukaemia