Haematology

Question 1

B lymphocytes can become

Answer 1

plasma cells or memory B cells

Question 2

T lymphocytes can become

Answer 2

CD4 (T helper cells)
CD8 (cytotoxic T cells)
natural killer cells

Question 3

define anisocytosis and when it is seen

Answer 3

variation in size of red blood cells
…myelodysplastic syndrome

Question 4

when are target cellls seen on a blood film?

Answer 4

iron defiency anaemia
post splenectomy

Question 5

what are heinz bodies and when are they seen?

Answer 5

individual blobs seen inside red blood cells caused by denatured globin
in G6PD and alpha thalassaemia

Question 6

what are howell jolly bodies and when are they seen?

Answer 6

individual blobs of DNA inside red cells which is normally removed by spleen
in post splenectomy and severe anaemia

Question 7

what are reticulocytes and when are they seen?

Answer 7

immatured red cells, slightly larger than standard erythrocytes and still have RNA material
seen in haemolytic anaemia

Question 8

what are schistocytes and when are they seen?

Answer 8

fragments of red cells
caused by haemolytic uraemic syndrome, DIC

Question 9

what are sideroblasts and when are they seen?

Answer 9

red cells that contain blobs of iron, in myelodysplastic syndrome

Question 10

what are smudge cells and when are they seen?

Answer 10

ruptured white cells
in chronic lymphocytic leukaemia

Question 11

what are spherocytes and when are they seen?

Answer 11

spherical red cells
autoimmune haemolytic anaemia

Question 12

what are the causes of microcytic anaemias?

Answer 12

T – Thalassaemia
A – Anaemia of chronic disease
I – Iron deficiency anaemia
L – Lead poisoning
S – Sideroblastic anaemia

Question 13

what are the causes of normocytic anaemia?

Answer 13

A – Acute blood loss
A – Anaemia of Chronic Disease
A – Aplastic Anaemia
H – Haemolytic Anaemia
H – Hypothyroidism

Question 14

what are the causes of macrocytic anaemias?

Answer 14

megaloblastic (impaired DNA synthesis) - B12 defiency, folate deficiency
normoblastic macrocytic - alcohol, reticulocytosis, hypothyroidism, liver disease, azathioprine

Question 15

what are the sx and signs of anaemia?

Answer 15

Tiredness
Shortness of breath
Headaches
Dizziness
Palpitations
Worsening of other conditions such as angina, heart failure or peripheral vascular disease

Pale skin
Conjunctival pallor
Tachycardia
Raised respiratory rate

Question 16

what are two specific signs of iron def anaemia?

Answer 16

Pica describes dietary cravings for abnormal things such as dirt and can signify iron deficiency
Hair loss can indicate iron deficiency anaemia
koilonychia
angular chelitis
atrophic glossitis

Question 17

what is a specific sign seen in haemolytic anaemia?

Answer 17

jaundice

Question 18

what is a specific sign seen in thalassaemia?

Answer 18

bone deformities

Question 19

how is anaemia investigated?

Answer 19

Haemoglobin
Mean Cell Volume (MCV)
B12
Folate
Ferritin
Blood film
OGD
bone marrow biopsy

Question 20

what is the physiology behind iron absorption?

Answer 20

iron is mainly absorbed in duodenum and jerjenum in soluble form, Fe2+ (this requires acid to keep in this form - so PPI can interfere)

Question 21

what are some causes of iron deficiency anaemia?

Answer 21

Blood loss is the most common cause in adults - menorrhagia, GI cancer, oesophagitis, gastritis, IBD
Dietary Insufficiency is the most common cause in growing children
Poor iron absorption
Increased requirements during pregnancy

Question 22

how do blood results change in iron def anaemia?

Answer 22

Total iron binding capacity can be used as a marker for how much transferrin is in the blood. It is an easier test to perform than measuring transferrin. Both TIBC and transferrin levels increase in iron deficiency and decrease in iron overload

Question 23

how is iron def anaemia treated?

Answer 23

Blood transfusion. This will immediately correct the anaemia but not the underlying iron deficiency and also carries risks.
Iron infusion e.g. “cosmofer”. There is a very small risk of anaphylaxis but it quickly corrects the iron deficiency. It should be avoided during sepsis as iron “feeds” bacteria.
Oral iron e.g. ferrous sulfate 200mg three times daily. This slowly corrects the iron deficiency. Oral iron causes constipation and black coloured stools. It is unsuitable where malabsorption is the cause of the anaemia.

Question 24

B12 defiency can be caused by

Answer 24

insufficient dietary intake of vit B12
pernicious anaemia

Question 25

what is the pathology behind pernicious anaemia?

Answer 25

Paritetal cells of stomach produce intrinsic factor - essential for absorption of vit B12 il ileum. Pernicious anaemia is an autoimmune condition where antibodies form against the parietal cells or intrinsic factor. A lack of intrinsic factor prevents the absorption of vitamin B12

Question 26

what are the sx of pernicious anaemia?

Answer 26

vit b 12 defiency causes neurological sx - numbness, paraesthesia loss of vibration sense or proprioception visual changes mood changes

Question 27

how is pernicious anaemia diagnsoed?

Answer 27

test for intrinsic factor antibody

Question 28

how is pernicious anaemia managed?

Answer 28

dietary (lack of b12) - oral cyanocobalamin if severe (pernicious anaemia is cause)- IM hydroxycobalamin

Question 29

what are the inherited haemolytic anaemias?

Answer 29

Hereditary Spherocytosis Hereditary Elliptocytosis Thalassaemia Sickle Cell Anaemia G6PD Deficiency

Question 30

what are the acquired haemolytic anaemias?

Answer 30

Autoimmune haemolytic anaemia Alloimmune haemolytic anaemia (transfusions reactions and haemolytic disease of newborn) Paroxysmal nocturnal haemoglobinuria Microangiopathic haemolytic anaemia Prosthetic valve related haemolysis

Question 31

what are some clincial fx of sickle cell anaemia?

Answer 31

good periods, and crisis crisis include thrombotic, 'painful crises' (vaso occlusive) sequestration acute chest syndrome aplastic haemolytic ...provide supportive treatment

Question 32

what are the clinical fx of polycythaemia vera?

Answer 32

The presence of increased haemoglobin, a plethoric appearance (red appearance), splenomegaly, and pruritus suggest a diagnosis of PV, a myeloproliferative disorder.

Question 33

how is suspected haemotological malignancy managed in young people?

Answer 33

Any of the following features in a person aged 0-24 years should prompt a very urgent full blood count (within 48 hours) to investigate for leukaemia: Pallor Persistent fatigue Unexplained fever Unexplained persistent infections Generalised lymphadenopathy Persistent or unexplained bone pain Unexplained bruising Unexplained bleeding

Question 34

what type of hodgkin lymphomas had the best prognosis?

Answer 34

Hodgkin's lymphoma - best prognosis = lymphocyte predominant

Question 35

haemolysis blood results

Answer 35

Anaemia with reticulocytosis is suggestive of haemolysis or bleeding. The markedly raised bilirubin with otherwise normal LFTs is more in keeping with haemolysis, however, it is important to remember that haemorrhage can also result in increased bilirubin levels. Haemolysis results in the release of free haemoglobin. Haptoglobin is produced in the liver, and its primary function is to bind to free haemoglobin. Therefore low haptoglobins is a key feature of haemolysis

Question 36

chronic myeloid leukaemia

Answer 36

This patient has chronic myeloid leukaemia (CML), a haematological malignancy that affects older adults (60-70 years) and presents with progressive anaemia, weight loss and night sweats. Splenomegaly is common on examination and laboratory tests demonstrate an increase in granulocyte levels at different stages of maturation. Leucocyte alkaline phosphatase is decreased in CML. This patient is presenting with similar symptoms of lethargy, weight loss, night sweats and splenomegaly. His blood tests also demonstrate a leucocytosis that is predominantly of the myeloid lineage. The treatment of choice for CML is imatinib, a tyrosine kinase inhibitor, preventing further downstream cell division.

Question 37

hyposplenism

Answer 37

Target cells and Howell-Jolly bodies may be seen in coeliac disease → hyposplenism

Question 38

haemophilia

Answer 38

Haemophilia is an X-linked recessive disorder of coagulation. Up to 30% of patients have no family history of the condition. Haemophilia A is due to a deficiency of factor VIII whilst in haemophilia B (Christmas disease) there is a lack of factor IX - prolonged APTT

Question 39

define myelodysplastic syndrome

Answer 39

acquired neoplastic disorder of hematopoietic stem cells pre-leukaemia, may progress to AML more common with age presents with bone marrow failure (low Hb, neutropaenia, thrombocytopenia)

Question 40

define myelofibrosis

Answer 40

a myeloproliferative disorder - hyperplasia of abnormal megakaryocytes Features e.g. elderly person with symptoms of anaemia e.g. fatigue (the most common presenting symptom) massive splenomegaly hypermetabolic symptoms: weight loss, night sweats etc Laboratory findings- anaemia high WBC and platelet count early in the disease

Question 41

define polcythaemia vera

Answer 41

myeloproliferative disorder caused by clonal proliferation of a marrow stem cell leading to an increase in red cell volume, often accompanied by overproduction of neutrophils and platelets. related to mutation in JAK2 is present in approximately 95% of patients Features- pruritus, typically after a hot bath splenomegaly hypertension hyperviscosity arterial thrombosis venous thrombosis haemorrhage (secondary to abnormal platelet function) low ESR

Question 42

what is the management for a chest crisis experienced by a sickle cell patient?

Answer 42

Exchange transfusions are a way of reducing the number of sickle red cells and increasing the number of normal red cells, in order to improve oxygenation

Question 43

what are the clinical fx of chronic myeloid leukaemia and the tx?

Answer 43

chronic myeloid leukaemia (CML), indicated by the high white cell count which is due to high numbers of myeloid cells and the chronic nature of the presentation. The absence of blast cells indicates this is not acute leukaemia. The presence of anaemia and thrombocytopenia is due to bone marrow dysfunction, usually caused by myelofibrosis, indicating a need to start treatment. The first-line treatment for CML is the tyrosine kinase inhibitor imatinib. It is taken as a daily tablet and has excellent results in CML.

Question 44

how are lymphomas staged?

Answer 44

ann arbor

Question 45

thalassaemia trait

Answer 45

The key finding here is a severe microcytosis that is disproportionately lower than the haemoglobin level; this is suggestive of either beta- or alpha thalassaemia trait

Question 46

how do you manage a sickle cell crisis?

Answer 46

managing sickle cell crisis should be analgesia, oxygen, and IV fluids. You can also consider antibiotics if you suspect an infection, and transfusion if the Hb is low

Question 47

hodgkin lymphoma

Answer 47

Reed-Sternberg cells are diagnostic of Hodgkin's lymphoma