Haematology Flashcards by Harriet Guthrie

What is the normal level of Hb for women?

120-160 g/L

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What is the normal level of Hb for men?

135-175 g/L

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What are the subunits in fetal haemoglobin?

2x alpha

2x gamma

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What are the subunits in adult haemoglobin?

2x alpha

2x beta

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What causes alpha thalassaemia?

A deletion of one of the genes that codes for alpha haemoglobin

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How does HbF affect sickle cell disease?

Fetal haemoglobin is protective, so if there is more HbF, symptoms will be less severe.

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What cell type do erythrocytes evolve from?

Haemopoietic stem cells which then evolve into myeloid stem cells.
Reticulocytes

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What are the four types of leukaemia?

Acute myeloid leukaemia
Acute lymphoblastic leukaemia
Chronic myeloid leukaemia
Chronic lymphocytic leukaemia

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What is myelodysplasia?

Very low levels of white cells, red cells and myeloid precursors.

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How would you treat MGUS/ smouldering myeloma?

You dont!= Watch and wait

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How does myeloma lead to bone lytic lesions?

There are too many plasma cells, which stimulate too many osteoclasts, and inhibit osteoblasts (Treated with bisphosphonates)

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What things are measured in a FBC?

Haemoglobin
MCV
Haemocrit percentage
Platelet count
WCC
Lymphocyte count
Monocyte count
Basophil count
Eosinophil count

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What is haematopoiesis?

Synthesis of blood cells

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Where does haematopoiesis?

Normally in the bone marrow of the axial skeleton

- Can have extramedullary haematopoesis especially in the spleen

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What stimulates production of erythrocytes?

EPO

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What causes myeloproliferative neoplasms?

Gene mutations in the signalling pathway for blood cell proliferation- leading to an excess of mature cells in the blood

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Give some examples of myeloproliferative neoplasms

Chronic myeloid leukaemia (CML)
Polycythaemia vera
Essential thrombocythaemia
Primary myelofibrosis

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What is JAK-STAT pathway?

The signaling pathway for cell formation

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What is thrombocytosis?

Elevated platelet count

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How would you investigate a haematological malignancy?

History
Examination
FBC
Blood count
Immunophenotyping
Bone marrow biopsy
Cytogenetics
Molecular studies
Imaging

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What is the commonest cancer of childhood?

Acute lymphoblastic leukaemia

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What causes lymphoma?

Idiopathic
Primary immunodeficiency= CVID
Secondary immunodeficiency= HIV
Infection= EBV
Autoimmune disease

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What are the “B” symptoms?

Fever
Weight loss
Night sweats

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How is lymphoma diagnosed?

Clinical history and examination
Lymph node biopsy
Blood film/ Bone marrow biopsy
Immunophenotyping
Cytogenetics
CT chest/abdomen

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What sites are common to bleeds in haemophilia?

Soft tissue- iliopsoas, thigh, calf, buttock, deltoid/forearm, neck

What is the main treatment of haemophilia?

Factor replacement - NovoSeven in children if there are inhibitors= Recombinant VIIA - Or immune tolerance therapy if inhibitors

What are haemophilia inhibitors?

Inhibitors to the factor

What is the role of vWF?

- It causes platelet aggregation | - It is bound to factor VIII - and mediates it

What are the roles of thrombin?

It converts fibrinogen to fibrin - It converts factor XIII to XIIIa - It acts as a negative feedback loop to prothrombin

Which coagulation factors are vitamin K dependent?

10, 9, 7, 2

What is haemophilia A?

A deficiency of factor VIII

What is haemophilia B?

A deficiency of factor IX

What is vWD?

A deficiency of von Willebrand Factor= Causes spontaneous bleeding

Where are platelets produced and via what cell?

Produced in the bone marrow Megakaryocyte fragments Triggered by TPO

What are platelets?

Anucleate cells with a lifespan of 7-10 days - They adhere and aggregate to form a platelet plug (primary haematopoesis) - Removed by spleen

By what pathway are blood clots broken down?

- Fibrinolytic pathway | - Plasminogen forms plasmin which leads to fibrin breakdown

What causes the platelet plug to remain still?

- Prostaglandins inhibit platelet aggregation | - NO inhibits platelet aggregation

What are the causes of low platelets?

- Production failure - Increased removal - Artefactual

What are the components of Virchow's triad?

- Stasis of venous circulation - Hypercoagulability - Endothelial injury

How may a pulmonary embolism appear on x ray?

- Westermark sign - Palla's sign - Hampton's hump - Melting sign

What is hampton hump?

A classic wedge-shaped triangular opacity displaying a pulmonary embolus

What are the symptoms and signs of a DVT?

Symptoms: Pain, swelling Signs: Tenderness, swelling, warmth, discolouration

What are the clinical features of a massive pulmonary embolism?

- Hypotension - Cyanosis - Severe dyspnoea - Right heart strain// failure

What are the signs and symptoms of pulmonary embolism?

Symptoms: breathlessness, pleuritic chest pain, may have signs/ symptoms of DVT Signs: tachycardia, tachypnoea, pleural rub

How is pulmonary embolism prevented?

- Early mobilsation and hydration - Compression stockings - Thrombophylaxis

What are the arteries of the leg

- Common iliac - Femoral - Popliteal - Tibial

What are the three major branches of the abdominal aorta?

- Coeliac trunk - Superior mesenteric - Inferior mesenteric

At what point does the coeliac trunk come off the aorta?

T12

At what point does the superior mesenteric come off the aorta?

At what point does the inferior mesenteric come off the aorta?

At what point do the renal arteries come off the abdominal aorta?

What are the branches of the common hepatic artery?

- Proper hepatic - Gastroduodenal - Right gastric

What are the three branches that come off the loop of the aorta?

- Brachiocephalic - Left common carotid - Left subclavian

What is intermittent claudication?

Cramping pain that is induced by exercise and relieved by rest. Pain is distal to the site of atheroma, and is due to lactic acid production as a result of reduced blood supply.

What is critical ischaemia?

Tissue is dying and suffering at rest (rest pain= typically nocturnal). Blood supply is inadequate to allow basal metabolism.

What are the 5 main types of WBC?

- Neutrophils - Monocytes - Eosinophils - Basophils - Lymphocytes

What are the causes of neutropenia?

- Infection (EBV, HIV, Hep B, Hep C - Drugs (Phenytoin, antipsychotics) - Endocrine - Malignancy (myeloma) - Autoimmune - Excess alcohol and liver disease

What are the causes of neutrophilia?

- Infection (bacterial, VZV, HSV) - Drugs (steroids) - Malignancy (leukaemia, lymphoma) - Rheumatoid arthritis - Gout - Hypoxia

What are the causes of lymphopenia?

- Drugs (steroids) - Infection (post-viral = common) - Malignancy - Renal/hepatic impairment - SLE, RA - Anorexia nervosa

What are the causes of lymphocytosis?

- Infection (EBV, CMV, Pertussis) - Stress - Vigorous exercise - Malignancy - Post splenectomy

What are the causes of monocytosis?

- Malaria - Typhoid - TB - Myelodysplastic Syndromes

What are the causes of eosinophilia?

- Asthma - Parasitic infections - Addisons - Malignancy - Smoking

What are the causes of thrombocytopenia?

- Viral infections - Drugs - Alcohol - Malignancy - Liver and renal disease - Aplastic anaemias - SLE

What are the causes of thrombocytosis?

- Reactive - Malignancy - Splenectomy - Iron deficiency

What are the causes of platelet plug formation?

1. von Willebrand’s Factor (vWF) adheres to the vascular injury. 2. vWF then binds to GpIb receptors on surrounding platelets. 3. After binding, the ADP (P2Y12) receptor on the platelet is activated leads to increased expression of GpIIb/IIIa. 4. These newly expressed receptors then finally bind to fibrinogen, which leads to further platelet aggregation.

What are the 4 coagulation tests/

- PT - APTT - Bleeding time - Thrombin time

What types of malaria can lay dormant for years?

- P Ovale | - P Vivax

Angular stomatitis, lemon-yellow skin, ataxia and delusions are hallmarks of which type of anaemia?

Pernicious anaemia

You are a junior doctor on your haematology rotation. You have just seen a 50 year old woman who presented with anaemia, gum hypertrophy and bone pain. You do a full blood count which shows thrombocytopaenia and neutropoenia. A bone marrow biopsy shows Auer rods. What would you treat this woman for?

Acute Myeloid leukaemia

You are an F2 on a tropic medicine rotation. You are seeing a patient who has recently been on holiday to Asia and is now complaining of nausea, loss of appetite, abdominal pain and some weight loss. Her symptoms started 6 weeks after returning to the UK. You perform some tests and diagnose tapeworm. Which of the following would you expect to see?

Eosinophilia

You are an F1 who sees a 5 year-old patient with diagnosed ITP. His mother tells you that he has recently had a lot of nosebleeds and feels tired all the time. How would you treat this patient?

ITP= Prednisolone

Mr. Jones has been in hospital for a week recovering from a knee replacement. He is 75, has hypertension and is a smoker. He hasn’t been able to move very much since his surgery and refuses to wear compression stockings. Suddenly, Mr Jones becomes very short of breath and sits up to breath. His heart rate increases to 108 and he is breathing much faster than usual. What investigation do you want to order?

CTPA

Camilla is a 25 year old lady who has just returned home from visiting Ghana. She presents with very generic flu-like symptoms (fever, headache, cough) and is concerned that something is wrong. Whilst taking her history, you learn that she suffered from many mosquito bites during her trip. What investigation would you organise to confirm your diagnosis?

Thick and thin blood smears

Last week you saw a patient in GP who was complaining of tiredness and recurrent infection. You ordered a full blood count and the results have shown neutropenia. Name 2 causes of neutropenia. When would you want to repeat the patient’s bloods?

- Infection - Drugs (phenytoin, antipsychotics) - Malignancy - Excess alcohol and liver disease Repeat in 4 weeks’ time

What type of anaemia causes iron deficiency anaemia?

Microcytic

You decide to treat your patient with ferrous sulphate. What side effects would you want to alert the patient of?

Nausea, abdominal discomfort, GI upset, black stools

What is Ann Arbour classification?

A way to stage lymphoma

What are the stages of Ann Arbour classification?

I – Confined to a single lymph node region II – Involvement of 2 or more nodal areas on the same side of the diaphragm III – Involvement of 2 or more nodal areas on both sides of the diaphragm IV – Spread beyond the lymph nodes, e.g. bone marrow or liver Suffix A – No systemic symptoms other than puruitis (severe itching of skin) Suffix B – Presence of B symptoms, such as fever, night sweats and weight loss

Define Anaemia (no values needed)

Low Hb Concentration due to reduced cell mass or increased plasma volume

Give 3 causes of Macrocytic Anaemia

``` - Megaloblastic= B12/Folate Deficiency Non-Megaloblastic - Alcohol excess/liver disease - Hypothyroidism - Immunosuppressive (e.g azathioprine) - Myelodyplasia - Reticulocytosis ```

What is the cause of Chronic Myeloid Leukaemia?

Translocation of a gene between chromosome 9 and 22 leading to Philadelphia chromosome

What is the main distinguishing feature between Hodgkin’s and Non-Hodgkin’s lymphoma?

In Hodgkin’s (1) Presence of Reed-Sternberg Cells OR In Non-Hodgkin’s an Absence of Reed-Sternberg Cells

Give the name of the staging used in Hodgkin’s lymphoma and describe a patient who is diagnosed with Stage IIIB

- Ann Arbor Staging - Multiple lymph nodes affected - Above and below the diaphragm - Presence of B symptoms E.g Weight loss, Loss of appetite, night sweats

John, a 56 year-old male, is recovering chemotherapy treatment for his Acute Myeloid Leukaemia. You perform a blood test which reveals hyperkalaemia, hyperuricemia, hyperphosphatemia, hypocalcaemia. What condition has john developed?

Tumour Lysis Syndrome

How might hyperkalaemia present itself on ECG?

- Loss of P waves - Broad/ Wide QRS complex - Tall tented T waves

How is tumour lysis syndrome treated?

Aggressive Hydration/ IV Fluids, Medication to decrease uric acid, Replacement of Electrolyte Balance (e.g via dialysis)

Tom, a 6-year old boy, comes into the children’s hospital. His mum tells you that tom has missed the past 3-4 days of school with a chesty cough which you see in his notes is as a result of a recent chest infection. She has brought him in because of having 4 spontaneous node bleeds that afternoon. Tom appears tired and on examination has swollen lymph nodes. What is your suspected diagnosis?

Acute Lymphoblastic leukaemia

What would an ALL blood film show?

High conc of blast cells

What are the 2 features required for a diagnosis of febrile neutropenia?

- Temperature > 38o c | - Absolute Neutrophil Count < 1

Why do patients with myeloma experience bone loss?

Myeloma leads to cytokines stimulating osteoclasts and inhibiting osteoblasts

Rituximab is can be used in the treatment of certain leukaemias. What type of drug is Rituximab and what specifically does it target?

Monoclonal Antibody CD20 protein on the surface of B cells

What is the most common cancer of childhood?

ALL

Where is Philadelphia chromosome and what condition is it associated with?

Translocation of a gene between chromosome 9 and 22 | Chronic Myeloid Leukaemia

What are the causes of normocytic anaemia?

- Chronic disease - Acute Blood loss - Renal failure - Pregnancy - Hypothyroidism, hypopituitism

What are the causes of microcytic anaemia?

- Chronic disease - Iron deficiency - Thalassemia

What is the most common leukaemia?

Chronic lymphocytic

``` "An accumulation of mature B cells that have escaped apoptosis and undergone cell cycle arrest" A) CLL B) CML C) ALL D) AML ```

``` "Blood count would show low/normal Hb, very high WCC with high lymphocytes, smudge cells in vitro" A) CLL B) CML C) ALL D) AML ```

``` "Uncontrolled clonal proliferation of myeloid cells due to Philladelphia chromosome" A) CLL B) CML C) ALL D) AML ```

``` "Blood count would show low Hb, very high WCC with high neutrophils, basophils and eosinophils." A) CLL B) CML C) ALL D) AML ```

``` "Treated using Imatinib (a tyrosine kinase inhibitor) or stem cell transplant" A) CLL B) CML C) ALL D) AML ```

``` "A cancer of childhood" A) CLL B) CML C) ALL D) AML ```

``` "Malignancy of immature lymphoid cells= Usually B cell precursors if in children" A) CLL B) CML C) ALL D) AML ```

``` "Blood count would show high WCC, with blast cells on the film." A) CLL B) CML C) ALL D) AML ```

C or D | Need to differentiate using microscopy, immunophenotyping and molecular methods

``` "Neoplastic proliferation of blast cells derived from marrow myeloid" A) CLL B) CML C) ALL D) AML ```

What is allopurinol used for?

Prevention of tumour lysis syndrome

How do ALL and AML present?

- Anaemia= Breathlessness, fatigue, angina, pallor, murmur - Infecton= Fever, mouth ulcers - Bleeding due to low platelets= Bleeding, bruising, hepatosplenomegaly

How are ALL and AML diagnosed?

- Blood count= High WCC (may not be in AML), high blast cells - Bone marrow biopsy - Differentiation between the two is based on microscopy, immunophenotyping and molecular methods

How are ALL and AML treated?

- Blood and platelet transfusion - Treat infections quickly - Allopurinol to prevent TLS - IV fluids via hickman line - Chemotherapy - Marrow transplantation

What are "Popcorn cells" associated with?

Nodular lymphocyte predominant Hodgkin's lymphoma

What are "Reed-Sternberg cells" associated with?

Classical Hodgkin's lymphoma

What are "smudge cells" associated with?

May be seen in CLL in vitro

What is ABVD chemotherapy and what is it used to treat?

Hodgkin's Lymphoma - A= Adriamycin - B= Bleomycin - V= Vinblastine - D= Dacarbazine

What is "A cancer of the plasma cells leading to an excess of one type of immunoglobulins, usually IgG"?

Myeloma

What is Bence jones protein in urine associated with?

Myeloma

A patient has a monoclonal protein band in their urine, increased plasma cells on bone marrow biopsy, anaemia, high calcium and bone lesions on x ray. What is likely?

Myeloma

A patient has a monoclonal protein band in their urine, increased plasma cells on bone marrow biopsy, anaemia, high calcium and bone lesions on x ray. What is likely?

Myeloma

What would be a classic chemotherapy treatment for myeloma?

bortezomib (Biological therapy) , thalidomide (Biological therapy) and dexamethasone (Steroid or bortezomib (Biological therapy), cyclophosphamide (Chemotherapy) and vincristine (Chemotherapy)

``` A 39 year old man presents with a three month history of lethargy, fatigue and night sweats. On examination you note enlarged, painless cervical lymph nodes and splenomegaly. Blood tests show Hb 103 g/l (normal 135-180g/l), WCC 12.0 (normal 3.4-11), ESR 70 (<12). Histologically multinucleated giant cells (Reed-Sternberg cells) are noted. What is the most likely cause of this patient’s symptoms? A) Burkitt’s Lymphoma B) Hodgkin’s Lymphoma C) Chronic Myeloid Leukemia D) Non-Hodgkin’s Lymphoma E) Tuberculosis ```

A 58 year old man has been admitted to the general medical ward with abdominal pain and fluctuating consciousness. On examination he has shifting dullness and yellowing of sclera. A social history has been taken and he has been started on a reducing regime of diazepam. Blood tests have been undertaken, which show: Hb 92 g/l (normal 135-180), MCV 130fl (80-102fl), platelet count 145 (140-450). What is the most likely cause for this patient’s anemia? A) Autoimmune pernicious anaemia B) Malabsorption causing folate deficiency C) Iron deficiency anaemia D) Anaemia of chronic disease E) Chronic excess alcohol

``` A 4 year old boy came to clinic to have bloods taken for a check-up following a recent stay in hospital with a chest infection. However, following venipuncture, his mother has noticed that he bled for quite some time. On examination you also notice multiple petechiae on his legs, without any history of significant trauma. Mum remembers that her brother had the same problem when he was young. Blood tests show PT 12 seconds (normal 11-14), APTT 55 seconds (normal 29-39) & fibrinogen 2.2g/l (normal 1.9-4.3). Von Willebrand factor is normal. Factor VIII assay is reduced, whilst factor IX is normal. What is the most likely cause of the above presentation? A) Non Accidental Injury B) Ehlers-Danlos syndrome C) Von Willebrand Disease D)Hemophilia A E) Hemophilia B ```

A 68 year old man has presented with progressive shortness of breath, fatigue and worsening pain in his left leg and back. He has lost 2 stone of weight in the past year. On his last appointment it was noted that his renal function was deranged. Routine bloods show Hb 104g/l (normal 135-180), MCV 93fl (normal 80-102fl), WCC 2.8 (normal 3.4-11.0). Which of the following investigations would be most useful initially in finding the source of his symptoms? A) Abdominal ultrasound B) Lumbar spine X ray C) Blood film D) Urine electrophoresis (Bence Jones protein) E) DEXA Scan

``` A 7 year old girl has presented with petechiae on her knees and shins as well as frequent nosebleeds. She had a viral infection 2 weeks ago from which she apparently recovered. She appears clinically well in herself, with no temperature. She has no joint pain. PT, APTT, vW factor, factor VIII and factor IX are all normal. Her platelets are 100 (140-450). What is the most likely cause of these findings? A) Meningococcal septicaemia B) Henoch-Schonlein purpura C) Idiopathic thrombocytopenic purpura D) Von Willebrand’s disease E) Non-accidental injury ```

A 19-year-old female presents to the GP with a one-week history of fever, numbness, and difficulty speaking. She has never experienced anything like this before, and takes no medication. On examination, splenomegaly is noted as well as several purpura on her extremities. Bloods reveals normocytic anemia, decreased platelets, and elevated creatinine. What is the most likely diagnosis? A) Disseminated intravascular coagulation B) Drug-induced thrombocytopenia C) Haemolytic uraemic syndrome D) Immune thrombocytopenic purpura E) Thrombotic thrombocytopenia purpura

``` A four-year-old boy presents to the GP surgery with his mother. He has been feeling unwell for the past 3 weeks, with fevers, headaches and tiredness. His mother is concerned that he has been getting worse over time instead of better. He looks very pale and has many bruises and petechiae on his arms and legs, which have appeared spontaneously over the past week. Abdominal examination reveals an enlarged liver and spleen. An urgent full blood count shows: haemoglobin 68g/L (normal 120-180), platelets 25 (normal 150-450), white cell count 83 (normal 3.4-11.0). What is the most likely diagnosis in this child? A) Acute lymphoblastic leukaemia B) Aplastic anaemia C) Epstein-Barr virus D) Idiopathic thrombocytopenic purpura E) Lymphoma ```

``` A 6-month-old baby boy born to Pakistani parents presents with failure to thrive. He is found to have pallor and hepatosplenomegaly. His FBC shows Hb 78 g/l (99–141), MCV 65 fl (71–84) and MCH 18 pg (24–34). His blood film shows hypochromia, microcytosis and some nucleated red blood cells. Serum ferritin is 25 μg/l (14–200). What is the most likely diagnosis A) β thalassaemia B) Congenital dyserythropoietic anaemia C) Congenital sideroblastic anaemia D) Iron deficiency anaemia E) Lymphoma ```

``` A 63-year-old man with rheumatoid arthritis has come in complaining about tiredness and fatigue. His blood tests show an Hb of 98 g/l (low) and an MCV of 78 fl ( 80-100). His serum iron and TIBC are low and his serum ferritin is 115 μg/l (15–200). The most likely diagnosis is: A)Iron deficiency Anemia B) Acquired sideroblastic anemia C) Anemia of chronic disease D) Aplastic Anemia E) β thalassemia trait ```

A 59-year-old woman presents with fatigue. Her FBC shows WBC 4.8 × 109 /l(normal), Hb 93 g/l (low), MCV 115 fl(80-100). Her blood film shows macrocytes and hypersegmented neutrophils. She has a past history of Hashimoto thyroiditis. What is the most appropriate test to confirm the diagnosis you suspect? A) Beta cell antibodies B) Intrinsic factor antibodies and Parietal cell antibodies C) Adrenal antibodies D) Plasma homocysteine E) Serum iron levels

A 1-year-old boy presents with sudden-onset fever and vomiting. Findings include irritability, tachycardia, pallor, cold extremities, diffuse skin rash with abdominal petechiae, signs of meningeal irritation and is hypotensive. He is bleeding from the IV sites. Blood tests show leukocytosis, markedly decreased platelet count, increased prothrombin time (PT) and partial thromboplastin time (APTT), decreased fibrinogen, elevated fibrin degradation products, elevated urea, and metabolic acidosis. What is the most likely A) Hemolytic Uremic Syndrome B) Thrombotic Thrombocytopenic Purpura C) Disseminated Intravascular Coagulation D) ITP E) Von Willebrand Disease diagnosis?

A 26 year old female presents to her GP with fatigue, palpitations and increasing breathlessness on exertion. She has a history of heavy periods. What are the most likely finding on iron studies?

Decreased ferritin, decreased serum iron, increased TIBC

A 20-year-old female student is referred by her GP to your specialist clinic because she has felt unwell for the past two weeks. She has had a sore throat and felt very fatigued. On examination she seems pale, her throat is inflamed, and she has swollen cervical lymph nodes. You examine her abdomen and find a palpable spleen just below the left costal margin. Her blood tests show low hemoglobin, elevated LDH and bilirubin and a positive Direct Coombs Test for complement on the surface of cells. What is the most likely diagnosis A) Autoimmune Hemolytic Anemia B) Hemolytic uremic syndrome C) Disseminated intravascular coagulation D) Malaria E) Paroxysmal nocturnal hemoglobinuria

A 16-year-old boy presents to his GP complaining of nosebleeds and bleeding after brushing his teeth. He is unsure of how long this has been occurring but decided to seek advice after having to continually excuse himself from lessons. On examination you notice he has some skin bruises. A blood test shows a prolonged bleeding time and activated partial thromboplastin time (APTT), while platelet count and prothrombin times are all normal. The most likely diagnosis is: A) Von Willebrand disease B) Liver disease C) Disseminated intravascular coagulation D) Congenital afibrinogenaemia E) Glanzmann’s thrombasthenia

A 33-year-old man travels to South Africa to take part in a safari. On arriving, the patient takes his antimalarial tablets. A few days into his course he becomes ill complaining of shortness of breath, pallor and bloody urine. Blood tests reveal anemia and reduced hematocrit, while a blood smear shows the presence of Heinz bodies. The most likely diagnosis is: A) Hereditary elliptocytosis B) Glucose-6-phosphate dehydrogenase deficiency C) Hereditary spherocytosis D) Autoimmune hemolytic anemia E) Microangiopathic hemolytic anemia

A 65-year-old man presents to you reporting he has become increasingly worried about his lack of energy in the last 2 weeks. He mentions he has been increasingly tired, sleeping for long periods and has suffered from fevers unresponsive to paracetamol. He became increasingly worried when he noticed bleeding originating from his gums. A blood film shows rod like inclusion bodies in granular blast cells. The most likely diagnosis is: A) Acute lymphoblastic leukemia B) DiGeorge syndrome C) Disseminated intravascular coagulation D) Acute myeloid leukemia E) Afibrinogenemia

``` A 47-year-old woman presents complaining of dark stools and painful fingers on both hands. She appears plethoric and complains of severe itching, often when she takes a hot shower. A large liver and spleen is palpable. You measure red cell mass which is raised but EPO levels were decreased. Which of the following is likely to be the diagnosis? A) Anemia of chronic disease B) AML C) Polycythemia Rubra Vera D) ALL E) Pyruvate-Kinase deficiency ```

``` A 55 year old man presents with fatigue. He has had several episodes of haematemesis in the past 48 hours. He has a history of alcoholic cirrhosis and is being treated for ascites and encephalopathy. OE he has a HR of 85bpm and BP 84/62. What is the most likely cause of his fatigue? A) Excess alcohol intake B) Azathioprine use C) Acute blood loss D) Lead poisoning E) Haemolysis ```

``` A 45 year old woman presents with symptoms of fatigue, depression and weight gain. OE she has a heart rate of 58bpm, dry skin and bilateral eyelid oedema. FBC reveals a macrocytic anemia. What is the likely cause of the anemia? A) Iron deficiency B) B12 deficiency C) Alcohol D) Hypothyroidism E) Lead poisoning ```

``` A 62 year old male presents to his GP for his annual review. He denies any weight loss, fevers or fatigue. Bloods are taken and show an elevated WBC, predominantly lymphocytes. On blood smear, ruptured white blood cells are seen. What is the most likely diagnosis? A) CLL B) AML C) Hodgkin’s lymphoma D) ALL E) Myeloma ```

``` Which of the following drugs works by inhibiting CD20? A) Doxorubicin B) Bleomycin C) Vincristine D) Rituximab E) Cyclophosphamide ```

What are heinz bodies associated with?

Oxidative damage to the hemoglobin in red blood cells. Conditions associated include thalassemia or hemolytic anemia

What are bite cells associated with?

Glucose-6-phosphate dehydrogenase deficiency

Name four risk factors for developing acute leukemia

- Radioation - Benzene and smoking - Prior blood disorders - Genetic conditions such as Down's syndrome - Previous chemotherapy or radiotherapy

What is the major function of bone marrow?

Production of blood cells and non-blood components of bone marrow including fat, cartilage, and fibrous connective tissue

What signs/symptoms are common in patients with lymphoma?

- Painless enlargement of lymph nodes (cervical lymphadenopathy) - Fatigue - Fever - Night sweats - Weight loss - Shortness of breath and cough - Perisistant back of bone pain - Headaches

What investigations are used to obtain a definitive diagnosis of leukaemia or lymphoma?

- Blood film - Bone marrow biopsy - Lymph node biopsy - Immunohistochemistry - Genetic testing

What is the typical age of onset of ALL?

3-7

What are the three main categories used to determine the cause of anaemia?

- Microcytic - Normocytic - Macrocytic

List the two most common inherited haemoglobinopathies

- Thalassemias (Alpha is more common) | - Sickle cell disease

What is the typical age of presentation of alpha-Thalassemia?

In utero

What is the typical age of presentation of beta-Thalassemia?

In infancy

What is the main function of the platelet or thrombocyte?

Haemostasis= Initiate blood clotting

What physical findings would you expect to find with someone with low platelets?

- Mucosal bleeding, easy bruising - Petechiae - Purpura - Traumatic haematomas

What condition is JAK2 mutation associated with?

Polycythaemia Vera Present in half of myelofibrosis

How would you treat overdose of warfarin?

Phytomenadione (Vitamin K)

How would you treat overdose of heparin?

Protamine Sulfate

What could a high reticulocyte count show?

Haemorrhage, haemolysis or after treatment with non-specific haematinics

What would a positive Schumm's test be indicative of?

Intravascular haemolysis

What are blister cells associated with?

Blood enzyme defects such as G6PD deficiency or decreased pyruvate kinase

What are fragmented red blood cells associated with?

- Haemolytic anaemia such as eclampsia, haemolytic uraemic syndrome, Thrombotic thrombycytopenia purpura or DIC

What is a positive direct Coombs' test associated with?

- Autoimmune haemolytic anaemia | - Drugs e.g. methyldopa, quinine, NSAIDs

If a blood smear shows spherocytes, what test should be done next?

direct Coombs' test

What would spherocytes and a negative direct Coombs' test be indicative of?

Hereditary spherocytosis

What deficiency causes hereditary spherocytosis?

The red cell structural membrane protein spectrin

Is the alpha or the beta subunit implicated in sickle cell disease?

Beta

Why does sickle cell anaemia not usually manifest until over 6 months

HbF is protective

What are some causes of relative polycythemia?

Apparent polycythemia due to hypertension, obesity or high alcohol Dehydration

What are "teardrop" red blood cells associated with?

Myelofibrosis

What is the most common blood type in the UK?

O +ve