Week 2 - Interpreting Blood results - GI Malignancy, Pernicious anaemia, DKA, Hereditary Spherocytosis, Microangiopathic Hameolytic Anaemia

Question 1

Q How would you classify this anaemia? Q What further test would be best to confirm the cause of the anaemia?

Answer 1

The 62 year old has a hypochromic microcytic anaemia The next best test would be to measure the serum ferritin to rule out iron deficiency anaemia

Question 2

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Answer 2

There is thrombocytosis on the first film - the platelet count is doubled on FBC There is lymphopenia on the blood film also - cannot see any typical or atypical lymphocytes These can both be summed up by C - reactive changes

Question 3

Case 1 - Iron deficiency with weight loss, 62 years of age, pale, fatigued, high WBC Q What is the likely underlying cause of the iron deficiency * a dietary due to the anorexia * b inflammatory bowel disease * c malignancy of the gastrointestinal tract * d coeliac disease * e myeloproliferative disorder

Answer 3

C- malignancy of the GI tract is the likely cause of the iron deficiency anaemia in this patient - upper and lower GI endoscopy should be carried out

Question 4

Describe the blood results Q How would you classify this anaemia? * a normocytic normochromic * b macrocytic normochromic * c hypochromic normochromic * d hypochromic microcytic

Answer 4

Low haemgolobin - anaemic Erythropenic High MCV - macrocytic Thrombocytopenic Leucopenic The patient is pancytopenic Would classify her anaemia as b - macrocytic normochromic (although MCH is high, no such thing as a hyperhromic cell)

Question 5

How would you describe this blood film? Also, what is Anisopoikilocyotosis- seen on the blood film (anisopoikilocytes are seen?

Answer 5

This blood film shows hypersegmented neutrophils and macrooavalocytes Anisopoikolocytosis - variance in size and shape (macro and oval) Anisocytosis - this is the variance in size of a RBC Poikilocytosis - this is the variance in shape of a RBC

Question 6

What test would you do next to establish the diagnosis? a bone marrow aspirate b reticulocyte count c liver function tests d serum B12./serum folate e intrinsic factor autoantibodies What type of anaemia is it likely that the patient has?

Answer 6

Measure the d - serum B12/serum folate It is likely that the patient has pernicious anaemia

Question 7

Which antibodies would be measured to confirm the pernicious anaemia? What is pernicious anaemia due to? She is pancytopenic. If you did a bone marrow would it be and why? * a hypocellular * b normocellular * c hypercellular

Answer 7

Anti-gastric parietal cell antibodies - this is sensitive but not specific to this disease Anti-intrinsic factor antibodies - this is specific to this disease but only 50% sensitive If a bone marrow was carried out - would reveal hypercellular The bone marrow is trying to produce cells but because the cells are abnormal even in the primitive stages - there is intramedullary haemolysis resulting in the anaemia

Question 8

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Answer 8

E - B12 deficiency causes premature red cell destruction in the marrow - due to intramedullary haemolysis because of ineffective erythropoiesis This results in excess bilirubin production Haemoglobin and lactate dehydrogenase (LDH) are released from dead red cells Haemoglobin converted to bilirubin

Question 9

Describe the full blood count and what the striking abnormality is on the count?

Answer 9

Normal Hb Normal MCV - high end High RBC count The striking abnormality is the neutrophilia on the blood count - almost 4 times the norm

Question 10

What does this blood film show?

Answer 10

This blood film shows neutrophilia - multiple neutrophils seen here The blood films also shows echinocytes - these are usually caused by renal or liver failure

Question 11

What might be the causes of this patients neutrophilia? a sepsis b alcohol excess c leukaemia d drug abuse e smoking How do steroids cause neutrophilia? WHat are alternative causes for the neutrophilia?

Answer 11

A -sepsis Alternative causes for neutrophilia * Leukamia * Drug abuse - including steroid use - causes the cells to dermarginate increasing the neutrophil count (also increase the half life of neutrophils) * Smoking * Acute haemorrhage or malignancy also may cause neutrophilia

Question 12

What abnormality on the patients FBC may show that the patient was dehydrated?

Answer 12

The patient’s haemtocrit level is higher A raised HCT shows that there is an increase in the total red cell volume to total blood volume - ie when the patient is dehydrated

Question 13

You get some more information. He has not been drinking, and is carrying a medic alert bracelet. What diagnostic test would you now do next to establish why he is confused? * a blood cultures * b CT head * c U and Es * d blood gas * e near patient blood glucose * f CRP g urinalysis

Answer 13

e) near patients blood glucose The patient is likely to be going through a DKA (smells like alcohol)

Question 14

What features are seen in the blood film in severe marrow stress? a circulating blast cells b leucoerythroblastic change c rouleaux d reticulocytosis e red cell fragments

Answer 14

Circulating blast cells and reticulocytosis may be seen Can be summed up by B) Leucoerythroblastic change

Question 15

What is seen on the film? What further test would help confirm your suspicions that he is haemolysing : a Coombs’ test (direct antiglobulin test) b Reticulocyte count c osmotic fragility d Hb electrophoresis e red cell labelling study

Answer 15

On the film, can see polychromasia - likely to be due to an increased reticulocyte count as the marrow is trying to compensate for the extramedullary haemolysis Can also see spherecoytes, these are spherical in shape and do not have the biconcave strucutre so no white centre on film To confirm the suspicion of hameolyisis, would order a b - reticuloycte count

Question 16

Reticulocyte count is 513 x10^15/l (normal range 35-85) What is the likely diagnosis? * a warm type haemolyic anaemia * b sickle cell disease * c thalasaemia trait * d Hb Koln * e hereditary spherocytosis

Answer 16

e - Hereditary spherocytosis

Question 17

What further investigations are necessary to confirm the diagnosis? * a no further tests required * b genetic testing to confirm defect * c EMA screening * d red cell osmotic fragility testing * e SDS PAGE of red cell membrane proteins to identify defective protein

Answer 17

D - red cell osmotic fragility testing to confirm the diagnosis of hereditary spherocytosis Incubate red cells in increasingly hypotonic solutions and measure the release of haemgolobin from the lysed cells - sphereoctyes are far more cytolytic than normal red blood cells Direct Coombs test would be -ve as no antibodies present

Question 18

What treatment should you consider for hereditary erthrocytosis? a folic acid b splenectomy c bone marrow transplant d immunosupression e epo and iron

Answer 18

The bone marrow will be hypercellular as it is trying to produce more red cells to combat the extramedullary (also is extravscular in this disease case) haemolysis Therefore will be using more B12 and folate A) folic acid will be needed as the patient will be using it all up - need 100mg per day and stores only last for 4 months

Question 19

Why is this man of short stature? he has hereditary spherocytosis and cholecystectomy at 12 years * a part of the genetic phenotype of the disorder * b chronic anaemia * c damage to hypothalamic/pituitary axis due to iron deposition * d chronic marrow hyperplasia * e chronic liver disease * f chronic folate deficiency

Answer 19

C - damage to hypothalamic/pituitary axis due to iron deposition - i think

Question 20

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Answer 20

Haemoglobin is low RBC count is low MCV is normal MCH is normal A - normochromic normocytic anaemia

Question 21

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Answer 21

c there are both large and small cells and the mean is normal as a result There has been a reticulocytosis in response to haemolysis resulting in the MCV remaining normal - the reticulocytes are being combined to the normal erythrocyte count to make the volume appear normal although there are less RBCs The large cells seen here are c- reticulocytes

Question 22

What are the small cells? * a red cell fragments * b spherocytes * c sickle cells * d burr cells * e tear drop cells

Answer 22

The cells seen in the diagram are A) - red cell fragments - due to lysis of the cells fragments appear These fragments are known as schistocytes,c an also see polychromasia due to retciulocytes A schistocyte or schizocyte is a fragmented part of a red blood cell. Schistocytes are typically irregularly shaped, jagged, and have two pointed ends.

Question 23

What is the pathophysiological process going on here? a Acute folate deficiency precipitated by antimetabolite antibiotics b thrombotic thrombocytopenic purpura (TTP) c autoimmune haemolytic anaemia d disseminated intravascular coagulopathy (DIC) e combine iron deficiency and folate deficiency f microangiopathic haemolytic anaemia (MAHA)

Answer 23

F - Microangiopathic haemolytic anaemia This is where there is mechanical disrutption of the Red blood cells - usually can occur due to haemolytic uraemic syndrome, thombotic thrombocytotic purpura, DIC, leaky heart valves, infections and burns In this case it is due to leaky heart valves

Question 24

What urine test would confirm the intravascular nature of the problem?

Answer 24

Could carry out a urine haemoglobinuria (haemosderinuria also increased in intravascular haemolysis)