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Phase 2a > Haematology COPY > Flashcards

Haematology COPY Flashcards

1
Q

What do the terms haematocrit, anaemia and haemophilia mean?

A
  • Haematocrit = percentage of RBCs in cellular component of blood
  • Anaemia = reduced Hb, often due to iron deficiency. Two types: impaired production + increased haemolysis
  • Haemophilia = inability to make blood clots due to factor VIII deficiency (Haemophilia A) or Factor IX (Haemophilia B), A more common
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2
Q

How is blood divided? What are its different components?

A
  • Plasma component (55%):
  • Plasma (55%) = mostly consists of water, with water, salt, glucose and proteins
  • Cellular component (45%):
  • White blood cells and platelets (1%) = part of the immune system (WBC) and responsible for clotting (platelets)
  • Red blood cells (44%) = responsible for carrying O2 and CO2
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3
Q

What is the structure of erythrocytes? What is their lifespan? Where are they formed and removed? What is a reticulocyte?

A
  • Simple cell, anucleate, discoid, biconcave disc
  • Live for 100-120 days
  • O2/CO2 carrier
  • Contain haemoglobin and glycolytic enzymes
  • Formed: adults = bone marrow of axial skeleton, children = all bones, foetus = liver, spleen and yolk sac
  • Removed in spleen, liver, bone marrow + through blood tests
  • Reticulocyte = immature RBC, not usually found in blood
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4
Q

What is the structure of haemoglobin? What is its role? What are the different types of haemoglobin?

A
  • Tetrameric protein with 4 globin chains, each with haem group (porphyrin with Fe2+) = capable of reversibly binding oxygen
  • Carries oxygen from the tissue to the lungs
  • Several haemoglobin types:
  • Haemoglobin: 2 alpha and 2 beta chains
  • Foetal haemoglobin: 2 alpha and 2 gamma chains, means it has a higher affinity for oxygen
  • HbA2: 2 alpha and 2 sigma chains
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5
Q

What is haemopoeisis? Where does this occur in adults and in an embryo? What do stem cells produce? How are RBCs, WBCs and platelets produced?

A
  • Haemopoeisis = formation of new blood cells and platelets. Adults = precursors of mature cells derived from bone marrow of axial skeleton, but all bones in children. Embryos = in yolk sac, liver, spleen + bone marrow. Stem cells = pluripotent so can differentiate into RBCS, WBCs or platelets
  • RBC production = erythropoeisis
  • WBC production = myelopoeisis
  • Platelet production = thrombopoeisis
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6
Q

What are the hormonal factors in erythropoeisis, myelopoeisis + thrombopoeisis?

A
  • Erythropoeisis = hormonal stimulating factor = erythropoeitin, made in kidneys
  • Myleopoeisis = hormonal factor = granulocyte-macrophage colony stimulating factor, will only stimulate production of myeloblastic WBCs + not lymphoid cells
  • Thromobopoeisis = hormonal factor = thrombopoeitin, leads to production of megakaryocytes, which platelets bud from
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7
Q

What are platelets? Where do they originate from? What is the regulatory hormone? What are the two types of granules?

A
  • Platelets = 2-5um, last 7-10 days, circulate in inactive form + anucleate and discoid but become spiculated with pseudopia once activated, form blood clots (coagulation cascade)
  • Originate from megakaryocytes, 1 megakaryocyte = 4000 platelets = membrane blebbing process
  • Regulatory hormone = thrombopoeitin - produced by liver + kidneys
  • Plasma have 2 types of granules: alpha (coagulation factors, fibrinogen and other clotting mediators) and dense (ADP + platelet-activation mediators)
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8
Q

What are leukocytes? What are the two main groups? What is their role? What are the different types?

A
  • Leukocytes = white blood cells
  • Two main groups: granulocytes + agranuloocytes
  • Both involved in immune response, innate = granulocytes, adaptive = lymphocytes
  • Granulocytes:
  • Neutrophil = most abundant WBC, phagocytic and release chemo- + cytokines to induce inflammation. Multi-lobed nucleus, lasts ~10 hours. Granulocyte colony stimulating factor is the regulating hormone for most leukocytes (all the phils)
  • Basophils = bi-lobed nucleus, very prominent dark blue granules of histamine, lasts 8-12 hours. Mature into mast cells, express IgE + release histamine. Mast cells are almost identical to basophils except are tissue-resident and come from a different cell lineage
  • Eosinophils = bi-lobed nucleus that is ‘lozenge-shaped’, distinct granules, lasts 8-12 hours. Role in fighting parasitic infections but also wide range of regulatory functions
  • Agranulocytes:
  • Monocytes = reniform (kidney bean-shaped) nucleus, mature into macrophages (common macrophages you should know: Kupffer cells, alveolar macrophages, osteoclasts), lasts 8-12 hours
  • Lymphocytes - ‘fried egg appearance’, comprise B and T cells (B cells mature in bone marrow, T cells mature in thymus gland). B lymphocytes = plasma cells/memory cells + produce antibodies, T lymphocytes = T helper, T cytotoxic, T suppressor. Lasts 8-12 hours
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9
Q

What is the role of Natural Killer cells? Where are T cells formed, where do they mature and what are the different types? Where to B cells form and mature, and what is their role?

A
  • Natural Killer cells provide non-specific immunity against foreign proteins. They release perforins, which embed into the plasma membrane, creating channels and an influx of extracellular fluid which results in cell lysis
  • T cells form in the bone marrow, but mature in the thymus. There are cytotoxic T cells, which directly attack infected cells, and helper T cells which activate the B cells and form memory T cells
  • B cells form and mature in the bone marrow. They are involved in antibody secretion. B cells mature into plasma cells and memory B cells
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10
Q

What is plasma? Which proteins does it contain? What is serum?

A
  • Plasma = fluid component of blood (55%)
  • Transport medium containing water, salt, glucose + proteins
  • Proteins:
  • Albumin = produced in liver, determines oncotic pressure of blood, keeps intravascular fluid within that space, lack of albumin leads of oedema
  • Carrier proteins
  • Coagulation proteins. These are all produced by the liver
  • Immunoglobins = produced by plasma cells, key role in immunity + vaccination
  • Serum = plasma without clotting factors
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11
Q

What do all blood cells stem from? Where do these cells live?

A
  • Multipotential haemopoietic stem cell (haemocytoblast)
  • Haemotopoietic stem cells live in the bone marrow
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12
Q

What does the multipotential haemopoietic stem cell divide into? What do these two cells produce?

A
  • Common myeloid progenitor and common lymphoid progenitor
  • Common myeloid progenitor = platelets, erythrocytes, mast cells, basophils, neutrophils, eosinophils, monocytes
  • Common lymphoid progenitor = lymphocytes (NK cells, T cells, B cells)
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13
Q

What is primary and secondary haemostasis?

A
  • Primary haemostasis: initiation and formation of the platelet plug - platelet activation
  • Secondary haemostasis: formation of the fibrin clot - intrinsic and extrinsic coagulation cascade
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14
Q

What happens in the platelet plug formation when endothelial cells lining the blood vessels are damaged? What happens when ADP and fibrinogen are released?

A
  • Endothelial cells lining blood vessels damaged, exposes collagen underneath
  • Healthy endothelial cells release Von Willebrand factors (VWF) that binds to exposed collagen
  • Platelets arrive and have VWF receptor. They bind to VWF. This slows down platelets + causes them to become active
  • Platelet activation causes platelets to change shape (smooth discoid to spiculated + pseudopodia) and causes them to express GbIIb/IIIa
  • Platelets contain 2 types of granules: electron dense granules + alpha granules. Electron dense granules release ATP, ADP, serotonin + calcium and produce energy needed for reactions. Alpha granules release fibrinogen, VWF, platelet derived growth factor + heparin antagonist and are used to create mesh work to capture other platelets
  • ADP released by electron dense granules and acts on P2Y1 + P2Y12 to cause platelet activation + amplification. Platelet activation results in increased expression of GbIIb/IIIa + they crosslink with other GbIIb/IIIa receptors on other platelets by binding to fibrinogen. Enables new platelets to bind to old ones = platelet aggregation = positive feedback
  • Fibrinogen releases by alpha granules + binds to GbIIb/IIIa receptors. Needs to be turned to fibrin.
  • Arachadonic acid can be converted to different products depending on COX enzyme. In presence of COX 1, converted to prostaglandin H2, then to thrombroxane A2. This activates prothrombin into thrombin. Thrombin turns fibrinogen into fibrin. Fibrin creates a network of mesh that captures other platelets
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15
Q

What inhibits:

a) thromboxane formation
b) P2Y12 binding
c) thrombin?

A

a) NSAIDs
b) clopidogrel/ticagrelor
c) dabigatran

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16
Q

What is the coagulation casacade? What are the vitamin K dependent procoagulant factors? Draw it out.

A
  • Process of blood clotting, not be confused with platelet plug formation. Coagulation helps stabilise the plug. First protein = Factor XII, which activates Factor XI, which activates Factor IX etc. Ultimately soluble fibrinogen is converted to fibrin which then forms a stable fibrin clot.
  • Intrinsic pathway = happens inside the blood, independent of the extrinsic pathway
  • Extrinsic pathway = cannot happen without some of the proteins from the intrinsic pathway
  • Vitamin K procoagulant factors: 1972. Factor 10, 9, 7 + 2
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17
Q

What can inhibit:

a) factor 9
b) factor 7
c) factor 10a
d) prothrombin (factor 2)

A

a) warfarin (blocks vitamin K)
b) warfarin (blocks vitamin K)
c) warfarin, rivaroxaban (directly), heparin
d) warfarin, heparin (via antithrombin III)

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18
Q

In a full blood count, what is indicative of neutropenia? How do we perform this? What are the causes of neutropenia?

A
  • Neutropenia (low neutrophils) = < 2.0 x 10^9/L
  • Do a blood film and repeat FBC in 4 weeks
  • Causes:
  • Infection (EBV, HIV, Hep B, Hep C)
  • Drugs (Phenytoin, antipsychotics)
  • Endocrine
  • Malignancy (myeloma)
  • Autoimmune
  • Excess alcohol and liver disease
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19
Q

In a full blood count, what is indicative of neutrophilia? How do we perform this? What are the causes of neutrophilia?

A
  • Neutrophilia (high neutrophils) = > 7.5 x 10^9/L

  • Repeat FBC in 4 weeks
  • Causes:
  • Infection (bacterial, VZV, HSV) = most common cause
  • Drugs (steroids)
  • Malignancy (leukaemia, lymphoma)
  • Rheumatoid arthritis
  • Gout
  • Hypoxia
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20
Q

In a full blood count, what is indicative of lymphopenia? What are its causes? How do we treat it?

A
  • Lymphopenia (low lymphocytes) = < 1.3 x 10^9 L
  • Causes:
  • Drugs (steroids)
  • Infection (post-viral = common)
  • Malignancy
  • Renal/hepatic impairment
  • SLE, RA
  • Anorexia nervosa
  • Treat the underlying cause
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21
Q

In a full blood count, what is indicative of lymphocytosis? What number indicates an emergency? What are the causes of lymphocytosis?

A
  • Lymphocytosis (high lymphocytes) = > 3.5 x 10^9/L
  • If > 20x10^9/L refer urgently – emergency
  • Causes:
  • Infection (EBV, CMV, pertussis)
  • Stress
  • Vigorous exercise
  • Malignancy
  • Post splenectomy
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22
Q

In a full blood count, what is indicative of monocytosis? What are the causes of monocytosis?

A
  • Monocytosis (high monocytes) = > 0.8 x 10^9 L
  • Causes:
  • Malaria
  • Typhoid
  • TB
  • Myelodysplastic syndromes
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23
Q

In a full blood count, what is indicative of eosinophilia? How is the test performed? What are the causes? What should we check for when taking the patient’s history?

A
  • Eosinophilia (high eosinophils) = > 0.44 x 10^9 L
  • Do blood film and repeat FBC in 2 weeks
  • Causes:
  • Asthma
  • PARASITIC INFECTIONS
  • Drugs (penicillin, allopurinol, amitriptyline)
  • Smoking
  • Endocrine (Addison’s)
  • Malignancy
  • Endocarditis
  • Post-splenectomy
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24
Q

In a full blood count, what is indicative of thrombocytopenia? What level indicate an urgent referral? What are the causes of thrombocytopenia?

A
  • Thrombocytopenia (low platelets) = < 150 x 10^9/L
  • If < 20 x 10^9/L refer urgently
  • Causes:
  • Viral infection (EBV, HIV, Malaria, TB)
  • Drugs (NSAIDs, Heparin, Digoxin, PPIs)
  • Alcohol
  • Malignancies
  • Liver and renal disease
  • Aplastic anaemias
  • SLE
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25
Q

In a full blood count, what is indicative of thrombocytosis? What level indicates an urgent referral? What are the causes of thrombocytosis?

A
  • Thrombocytosis (high platelets) = > 400 x 10^9/L
  • If >1000 x 10^9/L refer urgently – emergency
  • Causes:
  • Reactive (infection, inflammation, haemorrhage, post-surgery)
  • Malignancies
  • Splenectomy
  • Iron deficiency
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26
Q

What is the PT/INR test? What is it used for? What are the normal times? What are the times affected by?

A
  • PT/INR test = prothrombin time. The INR measures PT test over PT normal (+ a constant)
  • The prothrombin time is a measure of the time taken for a blood clot to form via the EXTRINSIC pathway. Normal time = 12-13 seconds
  • Normal INR time = 0.8-1.1
  • Affected by liver disease, DIC, vitamin K deficiency and warfarin levels (too high)
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27
Q

What does aPTT measure? What is the normal range? Which conditions are linked with an abnormal aPTT?

A
  • aPTT = partial thromboplastin time (35-45 seconds)
  • It is the measure of the time take for blood to clot via the INTRINSIC pathway
  • aPTT indicated issued with factors VIII, IX and XI
  • The main conditions linked to this are:
  • Haemophilia A (VIII)
  • Haemophilia B (IX)
  • von Willebrand’s disease (vWF pairs with factor VIII)
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28
Q

What does bleeding time assess and measure? What is the normal range? Which platelet specific disorders increase bleeding time?

A
  • Bleeding Time (1-6 minutes) assesses overall platelet function and levels. It is a measure of how long it take a patient to stop bleeding from a wound
  • Platelet specific disorders increase BT:
  • von Willebrand’s disease
  • ITP
  • DIC
  • Thrombocytopaenia
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29
Q

What does thrombin time measure? What is the normal range? What causes a prolonged time?

A
  • Thrombin Time (10-15 seconds) tests how fast fibrinogen is converted to fibrin by thrombin
  • If the time is prolonged, it is caused by either a synthetic issue or a consumption time:
  • DIC
  • Liver failure
  • Malnutrition
  • Abnormal fibrinolysis
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30
Q

What is multiple myeloma? What does it result in? How many excess immunoglobulins are there? What is it associated with? In which individuals is it most commonly found in?

A
  • A malignancy of plasma cells
  • The accumulation of malignant plasma cells in the bone marrow leads to progressive bone marrow failure. 55% excess IgG and 20% excess IgA
  • Multiple myeloma is associated with the production of a characteristic paraprotein, kidney failure and bone disease
  • Most common in adults > 75 and Afro-Caribbean people
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31
Q

In order to make a diagnosis of myeloma, there must be evidence of mono-clonality. What is mono-clonality?

A

Abnormal proliferation of a single clone of plasma cell leading to immunoglobulin secretion and causing organ dysfunction especially to the kidney

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32
Q

Suggest 3 ways in which multiple myeloma can lead to AKI.

A
  1. Deposition of light chain
  2. Hypercalcaemia
  3. Hyperuricaemia
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33
Q

In extreme cases, patients with myeloma can present with blurred vision, gangrene and bleeding. What is the pathology behind this?

A

Paraproteins form aggregates in the blood and change the viscosity

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34
Q

What disease often precedes myeloma? What is this disease?

A
  • MONOCLONAL GAMMOPATHY OF UNDETERMINED SIGNIFICANCE (MGUS)
  • A common disease with paraprotein present in the serum but no myeloma. Often asymptomatic. <10% plasma cells in the bone marrow
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35
Q

In approximately 2/3 of people with myeloma, what might their urine contain?

A

Immunoglobulin light chains with kappa or lamda lineage

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36
Q

Why are patients with myeloma susceptible to recurrent infections?

A

There is a reduction in polyclonal immunoglobulin levels

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37
Q

What are the symptoms and signs of multiple myeloma?

A
  • Symptoms:
  • Fatigue
  • Infection due to neutropenia
  • Signs (OLD CRAB):
  • Old - over 75
  • Calcium over 2.75 mmol/L = AKI
  • Renal impairment
  • Anaemia
  • Bone lesions - pepper pot skull, back pain
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38
Q

Why is calcium elevated in myeloma?

A

There is increased bone resorption and decreased formation meaning there is more calcium in the blood

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39
Q

Why might someone with myeloma have anaemia?

A

The bone marrow is infiltrated with plasma cells. Consequences of this are anaemia, infections and bleeding

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40
Q

Why might someone with myeloma have renal failure?

A

Due to light chain deposition

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41
Q

What are the investigations for suspected myeloma?

A
  • FBC - anaemia, raised ESR
  • Blood film - rouleaux (aggregations of RBCs)
  • Bone marrow biopsy - increased plasma cells
  • Serum and urine electrophoresis - looking for a monoclonal protein band - BENCE JONES protein in urine
  • X-rays - pepper pot skull, fractures
  • CT - bone lesions, cord compression
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42
Q

What is the treatment for MGUS and asymptomatic myeloma?

A

Watch and wait

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43
Q

Describe the treatment for symptomatic myeloma.

A
  • BISPHOSPHONATES for bones, e.g. Zoledronate
  • Analgesia (avoid NSAIDs due to renal issue)
  • Radiotherapy
  • Blood transfusions
  • Infection control (most common death)
  • Chemotherapy: VCD, VTD and MTP
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44
Q

What type of disease is multiple myeloma? What is the aim for clinicians? What will happen near the end of the disease?

A
  • Multiple myeloma is a palliative disease, so everyone will relapse
  • The aim is to control the symptoms and neoplasia as best as possible to get the best quality of life for the patient
  • At the end of the disease, end organ damage will occur:
  • Lytic bone lesions
  • Infection
  • Fractures
  • Renal failure
  • Proteinuria
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45
Q

What is a lymphoma? Which organs may it affect?

A
  • A malignant growth of T and B cells, predominantly in the lymph nodes (MAINLY B LYMPHOCYTES)
  • Although predominantly in the lymph nodes, lymphomas may also affect:
  • Blood
  • Liver
  • Spleen
  • Bone marrow
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46
Q

What are the two classifications of lymphoma?

A
  • Hodgkin lymphoma (aggressive and indolent)
  • Non-Hodgkin lymphoma
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47
Q

How do we stage lymphomas?

A
  • ANN-ARBOR staging is used for lymphoma
  • 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 pruritis (severe itching of skin)
  • Suffix B – Presence of B symptoms, such as fever, night sweats and weight loss
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48
Q

Describe the pathophysiology of lymphoma.

A

There is impaired immunosurveillance and infected B cells escape regulation and proliferate (this is just a theory)

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49
Q

Give 4 risk factors for lymphoma.

A
  1. Primary immunodeficiency
  2. Secondary immunodeficiency, e.g. HIV
  3. Infection, e.g. EBV, HTLV-1
  4. Autoimmune disorders, e.g. RA
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50
Q

What is the epidemiology of Hodgkin lymphoma?

A
  • BIMODAL (peaks twice in life) - EARLY 20S AND 70S
  • Male predominance
  • Associated with EBV, SLE, siblings
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51
Q

What is the difference between Hodgkin and non-Hodgkin lymphoma?

A

Hodgkin lymphoma is marked by the presence of REED-STERNBERG cells. In non-Hodgkin lymphoma, these cells are not present

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52
Q

What are the signs and symptoms of Hodgkin lymphoma?

A
  • LYMPHADENOPATHY - painful nodes on drinking alcohol
  • Systemic ‘B’ symptoms, e.g. fever, night sweats, weight loss
  • Enlarged rubbery non-tendon nodes
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53
Q

What are the investigations for Hodgkin lymphoma? The presence of what cell confirms the diagnosis?

A
  • FBC = anaemia, high ESR
  • CXR/CT for staging
  • LYMPH NODE BIOPSY - REED STERNBERG cells
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54
Q

How do we treat Hodgkin’s lymphoma?

A
  • CHEMOTHERAPY ABVD: Adriamycin, Bleomycin, Vinblastine, Dacarbazine
  • Radiotherapy
  • Steroids
  • Stem cell/bone marrow transplant
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55
Q

What is the treatment for stage 1 - 2A Hodgkins lymphoma? What is the treatment for stage 2B - 4 Hodgkins lymphoma?

A
  • Short course combination chemotherapy followed by radiotherapy
  • Combination chemotherapy
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56
Q

What are the possible complications of treatment for Hodgkin’s lymphoma?

A
  1. Secondary malignancies
  2. IHD
  3. Infertility
  4. Nausea
  5. Alopecia
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57
Q

What is the epidemiology of non-Hodgkin lymphoma?

A
  • ADULTS 40+
  • Associated with EBV + Burkitt’s lymphoma
  • Family history increases risk
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58
Q

What are the signs and symptoms of non-Hodgkin lymphoma?

A
  • Painless lymphadenopathy
  • Systemic ‘B’ symptoms, e.g. fever, sweating, weight loss, fever
  • Can get hepatosplenomegaly
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59
Q

What are the investigations for non-Hodgkin lymphoma?

A
  • FBC - anaemia, high ESR
  • Imaging (CT/CXR) for staging
  • LYMPH NODE BIOPSY - NO REED-STERNBERG CELLS
  • Raised lactate dehydrogenase
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60
Q

How do we treat non-Hodgkin’s lymphoma?

A
  • If low grade and symptomless, do nothing
  • RCHOP CHEMOTHERAPY: Rituximab, Cyclophosphamide, Hydroxy-danicorubicin, Vincristine, Prednisolone
  • Steroids
  • Monoclonal antibodies to CD20 - Rituximab
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61
Q

What is leukaemia? Name the four sub-types of leukaemia.

A

A malignant proliferation of haematopoeitic stem cells

4 sub-types:

  1. Acute myeloid leukaemia
  2. Chronic myeloid leukaemia
  3. Acute lymphoblastic leukaemia
  4. Chronic lymphoblastic leukaemia
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62
Q

What is acute myeloid leukaemia? What is its epidemiology? What is it associated with?

A
  • Acute myeloid leukaemia = neoplastic proliferation of MYELOBLASTS (become granulocytes)
  • Seen in elderly (adults aged 40+). Progresses very rapidly; 20% 3 year survival. Medical emergency
  • Associated with DOWN’S SYNDROME, haematological disorders and ionising radiation
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63
Q

What are the signs and symptoms of acute myeloid leukaemia?

A
  • Anaemia - breathlessness, fatigue, pallor
  • Infection
  • Hepatosplenomegaly
  • Bleeding and bruising
  • GUM HYPERTROPHY
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64
Q

Why are anaemia, infection and bleeding symptoms of leukaemia? Why are hepatomegaly and splenomoegaly symptoms of leukaemia?

A
  • Because of bone marrow failure
  • Because of tissue infiltration
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65
Q

What are the investigations for acute myeloid leukaemia? What is diagnostic?

A
  • BONE MARROW BIOPSY - AUER RODS
  • FBC - anaemia, thrombocytopenia, neutropenia
  • Blood film - leukaemic blast cells
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66
Q

Describe the treatment for acute myeloid leukaemia.

A
  • Blood and platelet transfusions
  • Chemotherapy
  • Stem cell/bone marrow transplant
  • Antibiotics
  • Allopurinol to prevent tumour lysis
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67
Q

What is acute lymphoblastic leukaemia? What is its epidemiology? What is it associated with?

A
  • Acute lymphoid leukaemia = uncontrolled proliferation of immature LYMPHOBLASTS (become NK cells and lymphocytes)
  • MOST COMMON LEUKAEMIA IN CHILDREN AGED 0-4 YEARS
  • Associated with DOWN’S SYNDROME and ionising radiation
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68
Q

What are the signs and symptoms of acute lymphoblastic leukaemia?

A
  • CNS INVOLVEMENT - HEADACHES, CRANIAL NERVE PALSIES
  • Anaemia - breathlessness, fatigue, pallor
  • Bleeding/bruising
  • Infection
  • Hepatosplenomegaly
  • Peripheral lymphadenopathy
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69
Q

What are the investigations for acute lymphoblastic leukaemia?

A
  • FBC - anaemia, thrombocytopenia, neutropenia
  • Blood film - leukaemic blast cells
  • Lumbar puncture - CNS involvement
  • CXR and CT - lymphadenopathy
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70
Q

Describe the treatment for acute lymphoblastic leukaemia.

A
  • Blood and platelet transfusion
  • Chemotherapy - METHOTREXATE
  • Steroids
  • Allopurinol to prevent tumour lysis syndrome
  • Acute control of infections with IV antibiotics - neutropenia makes this high risk
  • Stem cell transplant
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71
Q

What is chronic myeloid leukaemia? What is its epidemiology? What are the vast majority of cases associated with?

A
  • Proliferation of myeloid blood cells - affects neutrophils, basophils, eosinophils and macrophages
  • Seen the most in adults 40-60
  • Vast majority associated with the PHILIDELPHIA CHROMOSOME
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72
Q

What are the signs and symptoms of chronic myeloid leukaemia?

A
  • MASSIVE HEPATOSPLENOMEGALY
  • GOUT
  • Loss of weight, fever, sweats, fatigue
  • Bleeding (platelet dysfunction)
  • Abdominal pain (splenic enlargement)
  • Anaemia - breathlessness, fatigue, pallor
  • Infection
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73
Q

What are the investigations for chronic myeloid leukaemia? What would be diagnostic? Which chromosome is seen in 80% of cases?

A
  • FBC - HIGH WBCs, anaemia, raised myeloid cells
  • PHILIDELPHIA CHROMOSOME seen in 80+% of cases - t(9;2), stimulates cell division
  • Increased B12
  • Blood film - left shift (indicates presence of immature neutrophils), basophilia
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74
Q

What is the treatment for chronic myeloid leukaemia?

A
  • Chemotherapy
  • TYROSINE KINASE INHIBITORS, e.g. IMATINIB given orally
  • Stem cell transplant

(CM went to the PHILIDELPHIA centre and got MASSIVE HEPATOSPLENOMEGALY AND GOUT. She got given TYROSINE KINASE INHIBITORS)

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75
Q

What is chronic lymphoblastic leukaemia? What is its epidemiology?

A
  • Chronic lymphoblastic leukaemia = proliferation of the B lymphocytes
  • MOST COMMON LEUKAEMIA IN ADULTS
  • Seen in adults 70+
  • Immunocompromised people = 3x more likely to develop CLL
  • Pneumonia can trigger CLL
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76
Q

What are the signs and symptoms of chronic lymphoblastic leukaemia?

A
  • OFTEN ASYMPTOMATIC
  • Lymphadenopathy
  • Severe cases see weight loss, sweats and anorexia
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77
Q

What are the complications of chronic lymphoblastic leukaemia?

A
  • Richter’s syndrome = transformation to aggressive lymphoma
  • Autoimmune haemolysis
  • Bone marrow failure
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78
Q

What are the investigations for chronic lymphoblastic leukaemia? What is diagnostic?

A
  • FBC - high WBC with high lymphocytes
  • Blood film - small mature lymphocytes, SMUDGE CELLS (remnants of leukocytes)
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79
Q

How do we treat chronic lymphoblastic leukaemia?

A
  • WATCH AND WAIT in early stages
  • Chemotherapy (RITUXIMAB)
  • Targeted therapy, e.g. bruton kinase inhibitors (ibrutinib)
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80
Q

Name the 3 broad categories of red cell disorders.

A
  1. Haemoglobinopathies
  2. Membranopathies
  3. Enzymopathies
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81
Q

What is normal adult haemoglobin made of? What is foetal haemoglobin made of? What is haemoglobin S?

A
  • 2 alpha and 2 beta chains
  • 2 alpha and 2 gamma chains
  • Haemoglobin S is a variant of Hb arising from a point mutation in the beta globin gene. The mutation leads to a single amino acid change, valine -> glutamine
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82
Q

What is sickle cell disease/anaemia? Is it recessive or dominant, and are the carriers heterozygous or homozygous? What is its epidemiology?

A
  • Sickle cell disease = production of abnormal beta-globin chains via a single base mutation of A to T. HbS polymerises - sickle shaped RBC (reduced carrying capacity for O2)
  • Autosomal recessive disorder
  • Sickle cell anaemia is homozygous, sickle cell trait is heterozygous
  • Most common in Africa
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83
Q

What is the advantage of being a carrier of sickle cell disease?

A

Carriage offers protection against falciparum malaria

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84
Q

If both parents are carriers of the sickle trait. What is the chance that their first child will have sickle cell disease?

A

Their offspring have a 1/4 chance of being affected with a sickle cell disease. (50% chance of being a carrier)

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85
Q

How long do sickle cells last for?

A

5-10 days - this explains why sickle cell disease is described as haemolytic

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86
Q

What are the risk factors for sickle cell anaemia?

A
  • Low O2
  • Cold weather
  • Parvovirus B19
  • Exertion
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87
Q

What are the chronic complications of sickle cell anaemia?

A
  • Avascular necrosis of joints
  • Silent CNS infarcts
  • Retinopathy
  • Nephropathy
  • ED
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88
Q

What are is the acute presentation of a crisis for sickle cell anaemia?

A
  • MSK: bone pain, joint pain
  • Infection
  • Resp: dyspnoea, cough, hypoxia
  • CNS: stroke
  • GI: sequestration crisis (blood outflow from the spleen is blocked, blood accumulates - splenomegaly)
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89
Q

What are the investigations for sickle cell anaemia? What is diagnostic?

A
  • Blood film - sickled erythrocytes seen
  • Blood count - low Hb (range of 60-80 g/L), low MCV
  • POSITIVE sickle solubility test - does not distinguish trait from disease SO NOT DIAGNOSTIC
  • Haemoglobin electrophoresis - CONFIRMS DIAGNOSIS as detects HbS band
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90
Q

What is the treatment for sickle cell anaemia?

A
  • Acute attacks: IV fluids, morphine, oxygen, blood transfusion
  • Chronic: oral HYDROXYCARBAMIDE to reduce frequency of crises, stem cell transplant, folic acid
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91
Q

What is the significance of parvovirus for someone with sickle cell disease?

A

Parvovirus is a common infection in children. It leads to decreased RBC production and can cause a dramatic drop in Hb in patients who already have a reduced RBC lifespan. This can be dangerous for someone with sickle cell

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92
Q

What is thalassaemia? What are the different types?

A
  • There is reduced synthesis of one or more globin chains leading to a reduction in Hb -> anaemia
  • Alpha and beta thalassaemia
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93
Q

What is alpha thalassaemia? What are the different types?

A
  • Gene deletion on one or both of alpha chains (chromosome 16)
  • 4 gene deletion (Hydrops foetalis)
  • 3 gene deletion (Hb H disease)
  • 2 gene deletion (trait)
  • 1 gene deletion (silent carrier)
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94
Q

What is the presentation for the different types of alpha thalassaemia?

A
  • Alpha (trait) [-a/-a or –/aa] - asymptomatic or mild anaemia
  • Alpha (Hb H disease) [-a/–] - mild microcytic anaemia, haemolytic anaemia
  • Alpha (Hydrops foetalis) [–/–] - incompatible with life
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95
Q

What are the investigations for alpha thalassaemia? What is diagnostic?

A
  • FBC showing microcytic anaemia
  • Elevated serum iron
  • Haemoglobin electrophoresis
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96
Q

Describe the treatment for alpha thalassaemia.

A
  • Treat with transfusions during crises
  • Desferrioxamine to aid iron excretion
  • Folic acid
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97
Q

What is beta thalassaemia? What are the different types?

A
  • Excess alpha chains, few beta chains. Point mutations in the gene (chromosome 11)
  • Minor
  • Intermedia
  • Major
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98
Q

What are the presentations for the different types of beta thalassaemia?

A
  • Minor (heterozygous) - asymptomatic, hypochromic RBC’s
  • Intermedia - anaemia, frequent infections
  • Major - failure to thrive, transfusion dependant (signs: bossing of skull, overgrowth of maxilla)
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99
Q

Where would you normally take a bone marrow biopsy from?

A

Posterior iliac crest

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100
Q

What are the investigations for beta thalassaemia? What is diagnostic?

A

Blood film - will see reticulocytosis, microcytic anaemia, and nucleated red blood cells in circulation

  • Hb electrophoresis
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101
Q

Which clinical classification of thalassaemia relies on regular transfusions?

A

Thalassaemia major

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102
Q

Why is it important to monitor iron levels in someone with beta thalassaemia major?

A

There is a risk of IRON OVERLOAD from the regular transfusions. Excess iron will be deposited in various organs, e.g. the liver and spleen and cause fibrosis

103
Q

What are enzymopathies? Name a common enzymopathy.

A
  • Enzyme deficiencies that lead to a shortened RBC lifespan
  • Glucose-6-Phosphate Dehydrogenase (G6PD) Deficiency
104
Q

What is glucose-6-phosphate dehydrogenase (G6PD) deficiency? What is its inheritance pattern? What is its epidemiology?

A
  • G6PD is protective against oxidative damage. In G6PD, RBCs are exposed to more oxidative damage = haemolysis
  • It is X-linked
  • More common in Africa, Middle East and SE Asia
  • More common in males
105
Q

What are the signs and symptoms of G6PD deficiency? What is it exacerbated by?

A
  • Most are asymptomatic
  • Haemolytic anaemia, e.g. fatigue, breathlessness
  • Splenomegaly
  • Exacerbated by ingesting fava beans
106
Q

What are the investigations for G6PD deficiency? What is diagnostic?

A
  • Blood film: HEINZ BODIES
  • Bloods: anaemia, increased LDH, increased reticulocytosis
  • G6PD enzyme levels low (note: can be normal)
107
Q

How do we treat G6PD deficiency? What are the drug interactions?

A
  • Blood transfusions
  • Stop any exacerbating drugs
  • Drug interactions:
  • Primaquine – malaria
  • Nitrofurantoin – UTIs
  • Sulphonamides – bacterial infections
108
Q

Describe the inheritance pattern for membranopathies. Name 2 common membranopathies.

A
  • Autosomal dominant
  • Spherocytosis and elliptocytosis
109
Q

Briefly describe the physiology of membranopathies.

A

Deficiency of red blood cell membrane proteins caused by genetic lesions

110
Q

What is hereditary spherocytosis? What is its epidemiology? What are the signs and symptoms?

A
  • There is a problem with the red blood cells. Instead of being shaped like a disc, the cells are round like a sphere. These red blood cells (called spherocytes) are more fragile than disc-shaped RBCs
  • The most common form of inherited haemolytic anaemia in the US and northern Europe
  • May be asymptomatic
  • Pallor
  • Jaundice
  • Splenomegaly
111
Q

What are the investigations for spherocytosis?

A
  • FBC
  • Reticulocyte count - elevated
  • Blood smear - presence of spherocytes
112
Q

How do we treat spherocytosis?

A
  • Blood transfusions
  • Folic acid (helps make RBCs)
  • Splenectomy with severe spherocytosis
113
Q

What is elliptocytosis?

A

RBCs assume elliptical shape

114
Q

What is malaria? What are the different types of plasmodium? What is its epidemiology?

A
  • Malaris is a NOTIFIABLE DISEASE
  • Malaria is transmitted by the bite of a female anopheles mosquito. The mosquito carries plasmodium (5 types):
    1. Plasmodium falciparum (most important)
    2. Plasmodium ovale
    3. Plasmodium vivax
    4. Plasmodium malariae
    5. Plasmodium knowlesi
  • Most common in Africa
115
Q

What are the signs and symptoms of malaria? How long after infection do symptoms occur?

A
  • Non-specific symptoms:
  • Fever, chills
  • Headache
  • Cough
  • Splenomegaly
  • Severe disease in P. falciparum:
  • Shortness of breath
  • Fits and hypovolaemia
  • AKI and nephrotic syndrome
  • Symptoms occur from 6 days post infection
  • P. vivax and P. ovale commonly present 6 months post infection BUT they can relapse as they lay dormant in the liver for years
116
Q

What are the investigations for malaria?

A
  • Travel history
  • THICK AND THIN BLOOD SMEARS - negative film can be seen. If negative, 2 more films should be sent over the next 48 hours
  • Rapid diagnostic tests - detect parasitic antigens
  • PCR
  • FBC, LFTs, U&Es, blood gases, blood culture
  • CXR, lumbar puncture
117
Q

Describe the treatment for malaria. Which individuals should not be treated?

A
  • Uncomplicated: ORAL CHLOROQUINE
  • Complicated: IV ARTESUNATE
  • DO NOT TREAT THOSE WITH G6PD DEFICIENCY - need specialist help. Primaquine is contra-indicated in G6PD deficiency and pregnancy
118
Q

What are the stages of the plasmodia life cycle in the human called?

A

Exo-erythrocytic and endo-erythrocytic stages

119
Q

Describe the life cycle of malaria.

A
  1. Infected mosquito injects parasite (sporozoite) when it bites human
  2. Sporozoites travel to liver via blood and take up residence in hepatocytes
  3. In the liver, the sporozoites multiply and become merozoites. The hepatocytes then burst, releasing it into the blood
  4. In the blood, these merozoites invade erythrocytes and multiply again until the cell bursts
  5. The cycle repeats itself, the merozoites invade RBCs and multiply and then burst out. This causes chills, fever and sweating
  6. After several asexual cycles, merozoites can invade RBCs + instead of replicating, they develop into sexual form of the parasite (gametocytes)
  7. Mosquito bites the infected human. It digests the gametocytes which will allows it to develop into mature sex cells (gametes)
  8. Male and female gametes enter sporogenic cycle producing more pathogenic sporozoites
  9. Mosquito (female) can infect another human causing malaria
120
Q

What organs are responsible for removal of RBC’s?

A
  1. Spleen
  2. Liver
  3. Bone marrow
  4. Blood loss
121
Q

What is anaemia?

A

A decrease in the amount of Hb or RED BLOOD CELLS (Hb <130 in men, <120 in women)

122
Q

Using the full blood count, what measurement do we take and therefore how do we categorise anaemia?

A
  • There will be decreased Hb, and we look at the MCV (MEAN CORPUSCULAR VOLUME = average size of the red blood cells):
  • Microcytic anaemia (<80fL)
  • Normocytic anaemia (80-100fL)
  • Macrocytic anaemia (>100fL)
123
Q

What is the general presentation of anaemia?

A
  • Symptoms: fatigue, headache, dizziness, dyspnoea (especially on exertion)
  • Signs: tachycardia, skin pallor, conjunctiva pallor, intermittent claudication
124
Q

What are the investigations for anaemia?

A
  • Blood tests - FBC = look at white blood cells and platelets. Also blood film
  • Reticulocyte count - rate of red blood cell production in the bone marrow
  • Serum iron and ferritin test
  • Peripheral blood smear - assesses shape of red blood cells
  • Haemoglobin electrophoresis - evaluates abnormal haemoglobin. Present in thalassemia and sickle cell disease
  • Osmotic fragility test - assesses fragility of red blood cells
  • Endoscopy/colonoscopy - only if anaemia is suspected due to a bleed
125
Q

What types of anaemia do these signs belong to:

a) koilonychia (spoon-shaped nails)
b) angular stomatitis
c) lemon-yellow skin
d) jaundice/dark urine

A

a) iron deficiency
b) iron deficiency, B12 deficiency
c) B12 deficiency
d) haemolytic anaemia

126
Q

What are the causes of microcytic anaemia?

A
  • IRON DEFICIENCY: blood loss, poor diet, malabsorption, hookworm
  • Anaemia of chronic disease - cancer, HF, CKD
  • Sickle cell
  • Thalassaemia
  • Sideroblastic anaemia
127
Q

What is iron deficiency anaemia? What is the effect of iron deficiency anaemia on iron binding capacity? Why might measuring serum ferritin be inaccurate for looking at iron levels?

A
  • Iron deficiency anaemia is where the body does not produce enough RBCs because the blood iron levels are too low
  • Iron binding capacity will be raised
  • Ferritin is an acute phase protein and so its concentration will increase in response to inflammation
128
Q

What are the causes of iron deficiency anaemia?

A
  • REDUCED ABSORPTION (low intake/malabsorption at small intestine/drugs like PPI’s and tetracyclines)
  • INCREASED UTILISATION (pregnancy)
  • BLOOD LOSS (stools/urine/trauma/surgery/menorrhagia)
129
Q

What are the investigations for iron deficiency anaemia? What are the results?

A
  • FBC: low Hb, Low MCV <95fl
  • Iron studies:

Low ferratin (unless active inflammation)

Low serum iron

Low transferrin saturation

Raised transferrin (protein mediating iron transport in the blood. Raised because the body tries to compensate for reduced iron levels, by producing more transferrin. The saturation still remains low)

  • Blood film: small, hypochromic cells
130
Q

What are the causes of anaemia of chronic disease? What are the investigations?

A
  • Cause: chronic infection, chronic inflammation (connective tissue diseases), neoplasia
  • FBC: low Hb, low or normal MCV, high ESR
  • Iron studies:

Normal/raised ferratin

Low serum iron

Low transferrin saturation/transferrin (release is the problem, not the iron stores)

131
Q

What can be found on a blood film in a patient with sideroblastic anaemia?

A

RINGED ‘SIDEROBLASTS’ ON BLOOD FILM

132
Q

How do we treat microcytic anaemia? What are the side effects of one of the drugs?

A
  • Iron deficiency anaemia:
  • Treat underlying cause
  • Oral iron – ferrous sulphate
  • IV iron – emergencies
  • Ferrous sulphate side effects:
  • Nausea
  • Abdominal discomfort
  • Diarrhoea/constipation
  • BLACK STOOLS
  • Alternative is ferrous gluconate
  • Anaemia of chronic disease:
  • Control chronic disease
133
Q

What are the causes of normocytic anaemia?

A
  • If reticulocyte count high: HAEMOLYTIC anaemia, acute blood loss
  • If reticulocyte count low: bone marrow disorders (APLASTIC anaemia)
  • Anaemia of chronic disease
  • Pregnancy
  • Renal failure
  • Combined haematinic deficiency
134
Q

What are the causes of haemolytic anaemia? What is the presentation of haemolytic anaemia? What are the investigations for haemolytic anaemia?

A
  • RBCs destroyed faster than they can be made:
  • Causes:

Autoimmune haemolytic anaemia

Sepsis/disseminated intravascular coagulopathy (DIC)

G6PD deficiency

Sickle cell, thalassemia

Presentation: jaundice, dark urine

Investigations: raised reticulocytes (as breakdown is the cause), raised UNCONJUGATED BILIRUBIN, raised urobilinogen, schistocytes on blood film

135
Q

What is aplastic anaemia?

A

When bone marrow stem cells are damaged -> pancytopenia

136
Q

How do we treat normocytic anaemia?

A
  • Treat underlying cause
  • Improve diet with plenty of vitamins
  • Erythropoietin injections
137
Q

What are the causes of macrocytic anaemia? What is the key investigation? What are we looking for?

A
  • B12/folate deficiency (PERNICIOUS ANAEMIA)
  • Excess alcohol/liver disease
  • Hypothyroidism
  • Alcohol - chronic consumption affects bone marrow
  • Blood film - MEGALOBLASTIC OR NON-MEGALOBLASTIC
138
Q

Give 4 causes of vitamin B12 deficiency.

A
  • PERNICIOUS ANAEMIA - lack of intrinsic factor, usually produced by parietal cells in the stomach. Allows B12 absorption in the terminal ileum
  • Malabsorption (coeliac / IBD / bowel resection / ileostomy)
  • Decreased dietary intake (found in meat, fish, eggs, milk)
  • Chronic nitrous oxide use
139
Q

Give 4 causes of folate deficiency.

A
  1. Dietary
  2. Malabsorption
  3. Increased requirement, e.g. in pregnancy
  4. Folate antagonists, e.g. methotrexate
140
Q

What is pernicious anaemia? What are the symptoms? What are the investigations?

A
  • Pernicious anaemia = intrinsic factor ANTIBODIES are produced. These attack any intrinsic factor produced by the parietal cells (parietal cells produce intrinsic factor and hydrochloric acid. Intrinsic factor is essential for vitamin B12 absorption in the terminal ileum, whilst HCl allows B12 to be released from food). You need adequate vitamin B12 to make healthy RBCs, so we have less RBCs in pernicious anaemia
  • General anaemia presentation and a range of neurological features, i.e. symmetrical, paraesthesia, ataxia, delusions
  • Bloods: raised MCV, low Hb, low B12
  • ‘MEGALOBLASTIC’ anaemia - defined by cell changes on blood smear
141
Q

What kind of anaemia could methotrexate cause?

A

Macrocytic due to folate deficiency

142
Q

Explain how pernicious anaemia leads to B12 deficiency.

A

Pernicious anaemia leads to a loss of parietal cells -> reduced intrinsic factor production -> vitamin B12 malabsorption

143
Q

Give 5 causes of iron deficiency.

A
  1. Blood loss
  2. Poor absorption
  3. Decreased intake in diet
  4. Hook worm!
  5. Breastfeeding, low iron in breast milk
144
Q

How do we treat macrocytic anaemia?

A
  • Dietary - oral B12
  • Folic acid deficiency:
  • Folic acid tablets
  • Daily for 4 months
  • Pernicious anaemia:
  • B12 injections - IM hydroxocobalamin
  • Oral B12
145
Q

What is polycythaemia? What are the two types of polycythaemia? What are they due to? What is its pathophysiology?

A
  • Polycythaemia = HIGH CONCENTRATION OF ERYTHROCYTES IN THE BLOOD. It is a MYELOPROLIFERATIVE NEOPLASM
  • RELATIVE and ABSOLUTE (primary and secondary) polycythaemia:
  • RELATIVE = normal number of erythrocytes, but reduction in plasma. Causes include obesity, dehydration, and excessive alcohol consumption
  • ABSOLUTE:
  • Primary polycythaemia - increase in RBC mass due to mutations in bone marrow = POLYCYTHAEMIA VERA
  • Secondary polycythaemia - due to hypoxia (high altitiude, chronic lung disease, COPD) and increased erythropoietin secretion (renal carcinoma)
  • More RBCs = thicker blood = less able to travel through blood vessels and organs. Many of the symptoms of polycythaemia are caused by this sluggish flow of blood
146
Q

Which mutation do 95% on polycythaemia patients have?

A

JAK2 mutation

147
Q

What are the signs and symptoms of polycythaemia? How can we distinguish between primary and secondary polycythaemia?

A
  • Headaches and dizziness
  • Fatigue
  • Blurred vision
  • Tinnitus
  • Erythromelalgia – burning sensation in fingers and toes
  • Hypertension
  • Hepatosplenomegaly and itching, especially after contact with warm water (distinguishes polycythaemia vera from secondary)
148
Q

What are the investigations for polycythaemia? What is diagnostic?

A
  • FBC - raised WBC, platelets, and haemoglobin
  • JAK2 mutation on genetic screen
  • Low serum erythropoetin
  • Bone marrow biopsy showing proliferation of granulocytes and megakaryocytes
149
Q

Describe the treatment for polycythaemia.

A
  • No cure - treatment aim is to maintain normal blood count:
  • Venesection
  • Low aspirin daily
  • Hydroxycarbamide for those at high risk of thrombus
  • Radioactive phosphorus in those over 70
150
Q

What is the definition of febrile neutropenia?

A

Temperature >38°C in a patient with neutrophil count <1x10^9/L

151
Q

Give 4 risk factors for febrile neutropenia.

A
  1. If the patient had chemotherapy <6 weeks ago
  2. Any patient who has had a stem cell transplant <1 year ago
  3. Any haematological condition causing neutropenia
  4. Bone marrow infiltration
152
Q

What is the presentation of febrile neutropenia?

A
  1. Pyrexia, 38°C
  2. Generally unwell
  3. Confusion
  4. Hypotensive
  5. Tachycardic
153
Q

Describe the management of febrile neutropenia.

A
  1. Thorough history and examination
  2. Bloods
  3. Antibiotics within 1 hour!
154
Q

What is Haemophilia A? What is it split into? What is its epidemiology?

A
  • Haemophilia is a condition that affects the blood’s ability to clot. Haemophilia A is caused by factor VIII deficiency due to a mutation
  • Split into severe (<1% factor VIII activity), moderate (1-5%) and mid (>5%)
  • Massive male predominance (X-linked recessive). 5 times more common than Haemophilia B. Commoner in Afro-Caribbeans
155
Q

What are the signs and symptoms of Haemophilia A?

A

Soft tissue bleeding pattern - into muscles, joints, haematoma formation

156
Q

What are the investigations for Haemophilia A? What is diagnostic?

A
  • FBC - low haematocrit, low Hb
  • Normal prothrombin and bleeding time (PT), PROLONGED activated partial thromboplastin time (aPTT)
  • Plasma assay - heavily reduced factor VIII
  • CT head - look for haemorrhages
157
Q

Describe the treatment for Haemophilia A.

A
  • Prophylactic factor VIII
  • Fresh frozen plasma containing factor VIII to be given for acute bleeds
  • Desmopressin to boost factor VIII activity
158
Q

What is Haemophilia B? What are the different types? What is the epidemiology?

A
  • Caused by Factor IX deficiency
  • Mild disease has factor IX levels of 6-30%
  • Moderate disease has levels of 1-5%
  • Severe disease has levels of <1% - this accounts for 50% of cases
  • Male predominance (X-linked recessive)
159
Q

What are the signs and symptoms of Haemophilia B?

A

Soft tissue bleeding pattern – into muscles, joints, haematoma formation

160
Q

What are the investigations for Haemophilia B? What is diagnostic?

A
  • Normal prothrombin time
  • Prolonged activated partial thromboplastin time
  • Low factor IX levels
  • Endoscopy if GI bleed suspected
161
Q

Decribe the treatment for Haemophilia B.

A
  • TREATMENT MUST BE STARTED BEFORE A CONFIRMED DIAGNOSIS
  • Recombinant factor IX
  • Vaccination against Hep A and B
  • Patients should wear a medical emergency bracelet
162
Q

What is von Willebrand disease? What is it caused by? What is its inheritance pattern? What is its epidemiology?

A
  • Caused by defect in the QUALITY or QUANTITY of vWF - vWF assists platelet plug formation. It binds to factor VIII preventing clearance from plasma - FVIII deficiency can therefore occur
  • Type 1 (most common) is AUTOSOMAL DOMINANT. Primarily a PRIMARY HAEMOSTASIS DISORDER
  • More common in females. Poorer prognosis in blood type O
163
Q

What are the signs and symptoms of von Willebrand’s disease?

A
  • Bleeding from mucosa:
  • Epistaxis (nose bleeds)
  • Menorrhagia (heavy periods)
  • GI bleeds
  • Spontaneous bleeding
164
Q

What are the different types of von Willebrand disease?

A
  • Type 1, 2, 3 (severe bleeding), platelet type
  • Type 1 is the most common (60-80%)
  • Platelet type is the most rare (70 cases)
165
Q

What are the investigations for von Willebrand’s disease?

A
  • Plasma vWF decreased
  • Factor VIII levels - can be decreased as vWF is not present to protect it. aPTT can be prolonged if FVIII is decreased
  • FBC
166
Q

Describe the treatment for von Willebrand’s disease.

A
  • Type 1 disease responds to DESMOPRESSIN
  • Tranexamic acid - used for minor bleeds
167
Q

What is disseminated intravascular coagulation (DIC)? What is it associated with?

A
  • Widespread activation of the coagulation cascade and platelet activation due to cytokine release after sepsis, for example -> fibrin in vessel walls
  • Results in microvascular thrombosis and consumption of clotting factors
  • Associated with previous sepsis, malignancies, crush syndrome, transplant rejection, drugs
168
Q

Give 3 causes of disseminated intravascular coagulation (DIC).

A
  1. Sepsis
  2. Crush syndrome
  3. Malignancy
169
Q

What are the signs and symptoms of disseminated intravascular coagulation?

A
  • Bleeding (bruising, spontaneous bleeding at venepuncture sites, or after minor trauma, epistaxis (nosebleeds), GI bleeds)
  • Adult respiratory distress syndrome (ARDS)
170
Q

What are the investigations for disseminated intravascular coagulation?

A
  • Bloods: prolonged PTT and APTT, low fibrinogen, high d-dimers, low platelets
  • Blood smear: schistocytes
171
Q

What is the effect on TT, PTT and APTT in someone with disseminated intravascular coagulation (DIC)? What is the effect on fibrinogen in someone with disseminated intravascular coagulation (DIC)?

A
  • All increased
  • Decreased
172
Q

Describe the treatment for disseminated intravascular coagulation.

A
  • Treat underlying cause, e.g. sepsis
  • Platelet transfusion in those who present with BLEEDING ONLY
  • Low fibrinogen = cryoprecipitate (contains fibrinogen and some clotting factors)
  • FFP: contains clotting factors
173
Q

What is tumour lysis syndrome? What can it cause?

A

Break down of neoplastic cells -> content release -> metabolic disturbances; can cause hyperuricaemia, hyperkalaemia, hyperphosphatemia, hypocalcaemia, and AKI

174
Q

Give 3 risk factors for tumour lysis syndrome.

A
  1. High tumour burden
  2. Pre-existing renal failure
  3. Increasing age
175
Q

What are the signs and symptoms of tumour lysis syndrome?

A

Following chemotherapy, the characteristic syndrome is rapid development of:

  • Hyperuricaemia, hyperkalaemia, hyperphosphatemia
  • Hypocalcaemia
  • AKI
  • Occurs more frequently with aggressive treatment of haem malignancies like non-Hodgkin lymphoma, acute leukaemia
176
Q

What are the investigations for tumour lysis syndrome?

A
  • Serum uric acid
  • Serum phosphate
  • Serum potassium
  • Serum calcium
177
Q

Describe the treatment of tumour lysis syndrome.

A
  1. Aggressive hydration
  2. Monitor electrolytes
  3. Drugs to reduce uric acid production, e.g. allopurinol

Important as it is the reason Allopurinol is given alongside some leukaemia treatments

178
Q

What does rituximab target?

A

Targets CD20 on the surface of B cells

179
Q

What stimulates the production of platelets? Where is this produced? What would damage to this area cause?

A
  • Thrombopoietin (TPO) is produced in the liver
  • This stimulates the production of platelets by megakaryocytes
  • Liver damage = decreased platelets
180
Q

What do these do:

a) Thromboxane A2 (TXA2)
b) P2Y12
c) GP IIb/IIIa

A
  • Thromboxane A2 (TXA2):
  • Synthesised in platelets via COX-1
  • Induces platelet aggregation and vasoconstriction
  • P2Y12:
  • Receptor on platelets
  • Amplifies platelets activation
  • Important for clopidogrel
  • GP IIb/IIIa:
  • Receptor for von Willebrand factor
  • vWF is a clotting factor essential for platelet adherence to vessels
181
Q

What are the causes of platelet dysfunction?

A
  • Decreased production: leukaemia, lymphoma, myeloma, low B12/folate, liver disease - reduced TPO, methotrexate
  • Increased destruction: ITP, DIC, TTP
  • Reduced platelet function: congenital abnormality, aspirin, von Willebrand disease, uraemia (raised urea levels in blood)
182
Q

What is immune thrombocytopenia purpura (ITP)? What are the different types? What is its epidemiology?

A
  • Autoimmune disease, IgG destruction of Gpiib/iiia -> platelets can’t activate -> problem with PRIMARY HAEMOSTASIS
  • Primary: often post-viral infection, self-limiting
  • Secondary: malignancies, HIV
  • Mainly seen in children aged 2-6 POST-VIRAL
183
Q

What are the signs and symptoms of ITP?

A
  • Easy bruising and bleeding
  • Fatigue
  • Fever
  • Jaundice
  • Petechiae
  • Purpura
  • Neurological deficit
184
Q

What are the investigations for ITP?

A
  • FBC - raised WCC, low Hb, thrombocytopenia
  • PT and aPTT abnormal
  • Platelet autoantibodies present in 70% of cases
185
Q

Describe the treatment for ITP.

A
  • Prednisolone
  • IV IgG
186
Q

What is thrombotic thrombocytopenic purpura (TTP)? What is its epidemiology? What is it associated with?

A
  • Rare condition caused by a deficiency of von Willebrand factor cleaving protein (ADAM TS13) protein deficiency. It causes microvascular clots. Problem with PRIMARY HAEMOSTASIS
  • More common in adults
  • More common in females (3:2)
  • Associated with HIV, cancer and SLE
187
Q

What are the signs and symptoms of TTP?

A
  • Fatigue
  • Fever
  • Jaundice
  • Petechiae
  • Purpura
  • Neurological deficit
188
Q

What are the investigations for TTP?

A
  • FBC: raised WCC, low Hb, low platelets
  • Other: raised bilirubin, raised creatinine
  • Blood smear - FRAGMENTED ERYTHROCYTES (SCHISTOCYTES)
  • Normal PT and aPTT
189
Q

Describe the treatment for TTP. What is contra-indicated?

A
  • IV plasma exchange
  • IV methylprednisolone
  • Rituximab (monoclonal antibody)
  • Platelet transfusions are CONTRA-INDICATED
190
Q

What is hyperviscosity syndrome?

A

Increase in blood viscosity usually due to high levels of immunoglobulins

191
Q

Give 2 consequences of hyperviscosity syndrome.

A
  1. Vascular stasis
  2. Hypoperfusion
192
Q

Describe the presentation of hyperviscosity syndrome.

A
  1. Mucosal bleeding
  2. Visual change
  3. Neurological disturbances
  4. Breathlessness
  5. Fatigue
193
Q

What investigations might you do in someone who you suspect has hyperviscosity syndrome?

A
  1. FBC and blood film; look for rouleaux formation
  2. U&E
  3. Immunoglobulins
194
Q

What is the treatment for hyperviscosity syndrome?

A
  1. Keep hydrated
  2. Avoid blood transfusion
  3. Treat the underlying cause
195
Q

How does warfarin work?

A

It antagonises vitamin K and so you get a reduction in clotting factors 2, 7, 9 and 10

196
Q

How does heparin work?

A

It activates antithrombin which then inhibits thrombin and factor Xa

197
Q

What is the difference between Raynaud’s disease and Raynaud’s phenomenon?

A

Raynaud’s disease is idiopathic.
Raynaud’s phenomenon can be due to SLE, scleroderma, RA, drugs, e.g. beta blockers

198
Q

Describe the pathophysiological mechanism behind Raynaud’s disease.

A

Peripheral digital ischaemia due to intermittent spasm in arteries that supply the fingers/toes. Precipitated by cold/stress

199
Q

Describe the colour changes that are seen in Raynaud’s.

A
  • Pale - due to vasoconstriction
  • Cyanotic - due to deoxygenation
  • Red - due to hyperaemia
200
Q

Describe the treatment for Raynaud’s disease.

A
  1. Physical protection
  2. Vasodilators
  3. Nifedipine (CCB)
  4. Stop smoking
201
Q

Give a risk factor for spinal cord compression.

A

Any malignancy that can cause compression, e.g. bone metastasis

202
Q

Describe the presentation of spinal cord compression.

A
  1. Back pain
  2. Weakness in legs
  3. Inability to control bladder
  4. Spastic paresis
  5. Sensory level
203
Q

Describe the management of spinal cord compression.

A
  1. Bed rest
  2. High dose steroids
  3. Analgesia
  4. Urgent MRI of the whole spine
204
Q

What is glandular fever? What is it commonly caused by? What are its signs and symptoms?

A
  • Glandular fever (infectious mononucleosis) is an infection spread through spit
  • It is commonly caused by EBV
  • Very high temperature or feeling hot and shivery
  • Severe sore throat
  • Swollen glands
  • Extreme tiredness
  • Tonsillitis that is not getting better
205
Q

How do we treat glandular fever?

A
  • DO NOT give antibiotics - it is caused by a virus
  • There is no cure, it gets better by itself
206
Q

Briefly describe these diseases:

a) Protein C/S deficiency
b) Factor V Leiden
c) Antithrombin III deficiency
d) Antiphospholipid syndrome

A

a) Protein C/S Deficiency: less inhibition of factor VIII and V, autosomal dominant
b) Factor V Leiden: factor V is resistant to liver protein C, most common inherited thrombophilia (autosomal dominant)
c) Antithrombin III deficiency: II, IX, X are no longer inhibited
d) Antiphospholipid syndrome (Hughes Syndrome) – probably most likely to be seen in an exam: AP antibodies bind to cell surfaces, risks: SLE, female, diabetes, HTN, obesity, presentation: unexplained thrombosis (DVT/PE), recurrent miscarriage, Livedo reticularis

207
Q

What is heparin-induced thrombocytopenia? What is its presentation? Describe its treatment.

A
  • Following heparin administration, rarely (5%), antibodies bind to the drug - prothrombotic state. Problem with primary haemostasis
  • Presentation:

Onset of emboli (PE/DVT/CSVT) following heparin administration

Suspect in any patient on heparin who’s platelets fall >50%

Bloods: platelets low, D-dimer high

  • Treatment:

Stop heparin

Anticoagulate

208
Q

What is haemolytic uraemic syndrome? What is its presentation? What are its investigations?

A
  • Paediatric condition, following infection with SHIGA-TOXIN producing bacteria like e.coli/shigella (typically 5d after gastroenteritis). Microvascular clot formation, deposition of platelets and fibrin in small vessels
  • Presentation:

Classic triad - haemolytic anaemia, AKI (oliguria, haematuria, hypertension), thrombocytopenia

  • Investigations:

Bloods: thrombocytopenia, anaemia, high reticulocytes, high LDH, high bilirubin

Blood smear: schistocytes

Differentiate from TTP with ADAMTS13 testing

209
Q

What is myelodysplasia? What is its presentation? What are its investigations?

A
  • Heterogenous group of conditions, defined by cell dysplasia. Often manifests as anaemia and bone marrow failure - haemopoietic tissue is displaced by the dysplastic cells
  • Presentation:

General S&S of anaemia

Neutropenia (frequent infections)

  • Investigations:

Histological diagnosis. 10% of 500 cells must show dysplasia

Wide variety of abnormalities

210
Q

What is essential thrombocythaemia? What mutation is present in 50%? What is its presentation? Describe its investigations?

A
  • Elevated platelet count. 50% have JAK-2 mutation, meaning TPO is constantly stimulating platelet production
  • Presentation: increased thrombus risk (PE, DVT, CSVT)
  • Investigations:

FBC - thrombocytosis

Genetic testing for JAK mutation

Bone marrow biopsy - MEGAKARYOCYTES

211
Q

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

a) Iron deficiency anaemia
b) Thalassaemia
c) Anaemia of chronic disease
d) Pernicious anaemia
e) Bone marrow failure

A

D) Pernicious anaemia

212
Q

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?

a) AML
b) ALL
c) CML
d) CLL
e) Multiple myeloma

A

A) AML

213
Q

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?

a) Monocytosis
b) Thrombocytopenia
c) Eosinophilia
d) Neutrophilia
e) Lymphocytosis

A

C) Eosinophilia

  • Eosinophilia is seen in parasitic disease.
  • Monocytosis is seen in malaria, typhoid and TB. Thrombocytopenia is seen in viral infection, malignancies, SLE and liver disease. Neutrophilia is seen in malignancy, hypoxia, bacterial infection and rheumatoid arthritis. Lymphocytosis is seen in infection, stress, malignancy and after vigorous exercise.
214
Q

You are a junior doctor working on a paediatric ward. Your patient is aged 3 and has anaemia, hepatosplenomegaly, headaches and bone pain. You perform a number of tests and start treating them for cancer.

Which cancer is the most likely diagnosis?

a) AML
b) ALL
c) CML
d) CLL
e) Multiple Myeloma

A

B) ALL. Acute lymphoblastic leukaemia is the most common paediatric cancer. AML, CML and CLL are all more common in adults

215
Q

What do these cells indicate?

a) Thalassaemia
b) Hodgkin lymphoma
c) Multiple myeloma
d) DVT
e) G6PD deficiency

A

E) G6PD deficiency. These are bite cells

216
Q

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?

a) Platelet transfusion
b) Apixaban
c) Heparin
d) Prednisolone
e) You would not give medication

A

D) Prednisolone

  • In ITP, steroids are the first-line treatment. Platelet transfusions are also appropriate, but they would not be given initially
217
Q

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?

a) ECG
b) CTPA
c) CXR
d) Doppler ultrasound
e) Arterial blood gas

A

B) CPTA

  • This is most likely a pulmonary embolism. He has 5 risk factors and a text book presentation. He would score more than 4 if a Wells Score was carried out, therefore the most appropriate investigation would be a CTPA
218
Q

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?

a) FBC
b) Blood culture
c) Thick and thin blood smears
d) Haemoglobin electrophoresis
e) Bone marrow biopsy

A

C) Thick and thin blood smears

This is most likely Malaria. She has the symptoms and corresponding travel history. To confirm a diagnosis of malaria you need a positive thick and thin blood smear

219
Q

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?

A

Name 2 causes of neutropenia.

  • Infection
  • Drugs (phenytoin, antipsychotics)
  • Malignancy
  • Excess alcohol and liver disease

When would you want to repeat the patient’s bloods?

  • 4 weeks’ time
220
Q

You are a GP who has recently seen a patient complaining of fatigue, difficulty concentrating, breathlessness and headaches. You do a full blood count and diagnose iron deficiency anaemia.

What type of anaemia causes iron deficiency anaemia?

What are 2 other causes of this type of anaemia?

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

A

What type of anaemia causes iron deficiency anaemia?

  • Microcytic

What are 2 other causes of this type of anaemia?

  • Chronic disease (cancer, HF, CKD), and thalassaemia

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
221
Q

You are a junior doctor on you oncology rotation. You see a patient who presents with fever, sweating, enlarged, rubbery nodes that are painful when he drinks vodka and he says he feels tired all of the time. You do a blood smear and see Reed Sternberg cells. You also organise imaging to be done, and see the disease both above and below his diaphragm, there is no organ involvement.

How would you treat this patient?

Describe the disease using the Ann Arbour classification.

A

How would you treat this patient?

  • Chemotherapy ABVD:
  • Adriamycin
  • Bleomycin
  • Vinblastine
  • Dacarbazine

Describe the disease using the Ann Arbour classification.

  • Stage III B
  • 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
222
Q

What is the characteristic genetic abnormality in Chronic Myeloid Leukaemia?

A. t(15;17) ATRA gene

B. t(9;22) Philadelphia chromosome

C. t(8;21) AML/ETO gene

D. t(8;14) cMYC oncogene

A

B. t(9;22) Philadelphia chromosome

223
Q

What class of drug best describes Rituximab?

A. Cytotoxic chemotherapy

B. Disease-modifying therapy

C. Monoclonal antibody

D. Antibiotic

A

C. Monoclonal antibody

224
Q

Which age group is characteristically affected by Hodgkin’s lymphoma?

A. Children

B. Teenagers and young adults

C. Middle aged (40-60 yrs)

D. Older aged (>60yrs)

A

B. Teenagers and young adults

225
Q

How is myeloma bone disease usually assessed?

A. Plain X-ray

B. Clinical assessment

C. Isotope bone scan

D. PET scan

A

A. Plain X-ray

226
Q

What is the correct mechanism of action for the anti-emetic drug Ondansetron?

A. Peripheral D2 antagonist

B. Central D2 antagonist

C. Anti-cholinergic

D. 5HT3 antagonist

A

D. 5HT3 antagonist

227
Q

What is the commonest cause of microcytic anaemia?

A. B12 deficiency

B. Iron deficiency

C. Haematologic malignancy

D. Hereditary spherocytosis

A

B. Iron deficiency

228
Q

In sickle cell anaemia what would you expect to see the reticulocyte count?

A. Absent

B. Low

C. Normal

D. Raised

A

D. Raised

229
Q

Bacterial infection usually causes:

A. Low lymphocytes

B. Low neutrophils

C. High lymphocytes

D. High neutrophils

A

D. High neutrophils

230
Q

Which best outlines the approach to the management of a patient with suspected febrile neutropaenia?

A. Encourage fluids and paracetamol

B. Perform cultures and wait for results before starting antibiotics

C. Perform cultures and start oral antibiotics

D. Perform cultures and start broad spectrum IV antibiotics

A

D. Perform cultures and start broad spectrum IV antibiotics

231
Q

Malignant spinal cord compression usually presents with?

A. Back pain, ataxia and sensory neuropathy

B. Back pain, spastic paresis and a sensory level

C. Perianal numbness and urinary incontinence

D. Weak legs impaired joint position sense

A

B. Back pain, spastic paresis and a sensory level

232
Q

How does Aspirin exert its antiplatelet effect?

A. ADP receptor antagonist

B. Inhibition of Cyclooxygenase enzyme

C. Inhibition of Glycoprotein IIb-IIIa

D. Inhibition of PAR4 receptor

A

B. Inhibition of Cyclooxygenase enzyme

233
Q

What are the different types of blood product? What are their uses?

A
234
Q

A 70-year-old man is in A&E after losing a significant amount of blood. He takes warfarin. An INR test shows a result of 6.8 (target on warfarin = 2.0-3.0).

How would you reverse the warfarin?

a) Oral Vitamin K
b) Prothrombin Complex Concentrate and Vitamin K
c) Omit warfarin
d) IV Vitamin K
e) Whole Blood

A

B) Prothrombin Complex Concentrate and Vitamin K

235
Q

A 38-year-old woman is being treated DIC spontaneously haemorrhages while you are the FY1 working on the ward. Which blood product would be best to obtain and transfuse her with?

a) Erythropoietin
b) Packed red cells
c) Platelets
d) Prothrombin complex concentrate
e) Cryoprecipitate

A

C) Platelets

236
Q

You are a junior doctor working in haematology. A 62-year-old woman complains of severe fatigue and dizziness that came on over the past few weeks. On examination, you notice lots of bruises and gum hypertrophy. Her FBC shows anaemia and thrombocytopenia, and a bone marrow biopsy showed auer rods. What is the diagnosis?

A. ALL

B. AML

C. CLL

D. CML

E. Myeloma

A

B. AML

237
Q

You are a doctor working in paediatrics. A 3-year-old boy comes in with his parents who are worried about him as he has become very pale, he wants to sleep more often, and they have noticed bruises on his arms and legs. On examination, he has splenomegaly. His FBC shows anaemia, thrombocytopenia, and neutropenia. What is the most likely diagnosis?

A. ALL

B. AML

C. CLL

D. CML

E. Hodgkin lymphoma

A

A. ALL

238
Q

A 75-year-old man presents with fatigue, bone pain, and excessive thirst. His FBC shows anaemia and his ESR is raised

a. What is the most likely diagnosis?
b. What characteristic protein would you find in his urine?

A

a. Myeloma. CRAB - excessive thirst is a symptom of hypercalcaemia, bone pain is caused by bone lesions, and anaemia presents with symptoms such as fatigue.
b. Bence Jones protein

239
Q

A 19-year-old man presents to you after noticing a small lump in his neck. He says it became painful after a night out where he drank alcohol. He has also noticed some weight loss. A biopsy was done which showed Reed Sternberg cells.

a. What is the most likely diagnosis?

Upon imaging, there were enlarged lymph nodes in his neck and axilla region.

b. What stage is his disease?

A

a. Hodgkin lymphoma. Pain upon drinking alcohol and Reed Sternberg cells confirm the diagnosis of Hodgkin lymphoma
b. Stage 2

The disease in 2 or more areas on the same side of the diaphragm

240
Q

You are a medical student on placement in haematology. You are asked to assess a 66 year-old man who has come in to receive routine treatment for Haemophilia A.

Which of the following drugs is the man most likely to be receiving?

a) Recombinant FIX
b) Cryoprecipitate
c) Desmopressin
d) Recombinant FVIII

A

D. Recombinant FVIII. Cryoprecipitate is a mix of clotting factors extracted from plasma. It contains a lot of factor VIII, however it is unlikely to be given as routine haemophilia therapy

241
Q

A 17 year-old boy presents to your A&E department with a 2-day history of severe, deep pain in both knees and his upper legs with increasing fatigue and weakness.

He appears pale and clammy on examination, an abdominal exam notes massive splenomegaly.

Vitals: HR 115, RR 19, BP 110/63, SpO2 94% on air, Temp 36.9

FBC: MCV low, Hb low, reticulocytes mildly raised, haematocrit low

Given the likely diagnosis, what would be the most appropriate therapy?

a) O2 + IV methylprednisolone
b) O2 + IV fluids
c) O2 + IV morphine + IV fluids
d) NSAID’s

A

C. O2 + IV morphine + IV fluids. Acute sickle cell crisis with splenic sequestration

242
Q

An 75 year old women presents to A&E with severe community acquired pneumonia. She is displaying markers of red-flag sepsis, and develops a petechial rash over her entire body.

Blood tests show thrombocytopenia, long PT and APTT, low fibrinogen and a raised D-dimer

What is the name for the complication this patient has developed:

a) Thrombotic thrombocytopenia purpura
b) Disseminated intravascular coagulation
c) Haemolytic uraemic syndrome
d) Tumour lysis syndrome

A

B. Disseminated intravascular coagulation

243
Q

Shane is 34yr old male with Down’s syndrome. He has recently been complaining of increasing breathlessness and fatigue over the last 3 or 4 weeks. Upon looking at his medical record, you also notice he has been getting infections much more commonly than normal.

On examination you note bruising on his legs but otherwise normal.

Which of the following are you most likely to see on a peripheral blood film?

A. Bite cells

B. Smudge cells

C. Auer rods

D. Spherocytes

E. Tear drop cells

A

C. Auer rods

Bite cells seen in G6PD, smudge cells seen in CLL, Auer rods seen in AML, spherocytes seen in hereditary spherocytosis, tear drop cells seen in myelofibrosis

244
Q

Katie, a 25-year old student has been struggling with tiredness for a few months. She presented to the GP having developed weakness and pins and needles in her hands and feet, and difficulty with balance and walking. She has been following a vegan diet for the past 7 years.

What blood tests would you carry out and what would you expect the results to be? (3 marks)

A

• FBC

– ↓Hb

– ↑MCV (macrocytic anaemia)

• Blood film

– Hypersegmented neutrophils (>5 lobes) and presence of oval macrocytes

• Serum cobalamin/vitamin B12

245
Q

Katie is found to have anaemia caused by vitamin B12 deficiency.

1) What is the most common cause of this?
2) What features in a history would point towards it?
3) What tests are required to identify it?

A

1) Most common cause of B12 deficiency anaemia is pernicious anaemia. Vitamin B12 binds to intrinsic factor
2) It is an autoimmune disease - personal or family history of T1DM, autoimmune thyroid disease, Addison’s, vitiligo
3) Test for antibodies: parietal cell and intrinsic factor (specific) antibodies

246
Q

A 56-year-old female presents with a painless right neck lump that has been slowly enlarging for the last 2 years. She denies fevers but states she has been waking up sweaty at night more often. On direct questioning she believes she may have lost some weight.

Physical examination reveals bilateral cervical and axillary adenopathy and a palpable spleen.

Subsequent node biopsy is negative for Reed-Sternberg cells but a diagnosis of non-hodgkin’s lymphoma is made.

What is the most common cell of origin for this malignancy?

A. T lymphocytes

B. B lymphocytes

C. Neutrophils

D. Th lymphocytes

E. Myeloblast

A

B. B lymphocytes

247
Q

William is an 80 year old gentleman who takes warfarin for his metallic heart valve. His daughter called an ambulance after he had a nosebleed which would not stop. A&E blood tests showed his INR was 8.0 (target 2.5)

What should the F2 give to help stop this?

A) Vitamin A

B) Vitamin B

C) Vitamin K

D) Vitamin D

E) Vitamin E

A

C) Vitamin K

248
Q

Janet, (77F) attends A&E after a fall at home. An X-ray shows a fractured femur in the mid-shaft. You note this is a strange fracture to sustain considering her latest DEXA scan did not show osteoporosis.

During her admission Janet notes that she has also been suffering with a few other symptoms that she wonders whether you would be able to help her with. This include:

● A strange tingling/numb sensation around her mouth and on her fingers and toes.

● A worsening back pain for 2-3 months

● She has recently become quite constipated

You take U&E’s as part of routine bloods which shows a high serum urea and creatinine.

Which of the following symptoms/signs points AWAY from a diagnosis of myeloma?

A. Back pain

B. Numbness around the mouth

C. Pathological fracture

D. Constipation

E. High serum urea

A

B. Numbness around the mouth

249
Q

George presents with jaundice, fatigue, dizziness and palpitations. Blood tests show an autoimmune haemolytic anaemia.

Which of these statements regarding haemolytic anaemias is false?

A) The direct antiglobulin test (Coombs’) is used to distinguish autoimmune between non-immune aetiologies

B) Warm AIHA involves the IgG autoantibody

C) Bite cells and Heinz bodies are features of a PBS in G6PD deficiency

D) Signs of sickle cell usually manifest within 1-2 weeks of birth

E) Decreased haptoglobin is a feature of haemolytic anaemia

A

D) Signs of sickle cell usually manifest within 1-2 weeks of birth

250
Q
A

E. G6PD

251
Q

Sam, a 3-year old has had bloody diarrhoea for the past 5 days after eating some dodgy cottage pie at nursery. Their parents are now getting worried as they have a purpuric rash, are very fatigued and are not producing much urine (oliguria).

What is the most likely diagnosis?

A) Immune thrombocytopenic purpura

B) Haemolytic uraemic syndrome

C) Von Willebrands disease

D) Thrombotic thrombocytopenic purpura

E) Kawasaki disease

A

B) Haemolytic uraemic syndrome

252
Q

Adi, a 28M presents with a 5 day history of fever, chills and diarrhoea. He had been volunteering in Sub-Saharan Africa for 3 months, returning 8 weeks ago, and had been bitten by mosquitoes on multiple occasions. He recounts forgetting to take any prophylaxis medications.

On examination, temperature of 38.8C and a mild jaundice can be noted in the sclera. The rest of the examination is normal.

Which of the following is the most appropriate treatment option?

A. IV artesunate

B. Oral amoxicillin

C. IV Co-amoxiclav

D. Oral hydroxychloroquine

E. Oral Benzylpenicillin

A

D. Oral hydroxychloroquine

253
Q

A 17 year old presents to her GP with a 3-month history of tiredness. Further conversation reveals a history of heavy periods and a vegetarian diet. Blood tests show a decreased Hb of 95g/L (>120g/L), decreased MCV of 70 (80-100) and decreased ferritin of 20mcg/L (>30mcg/L). This suggests an iron deficiency anaemia.

1) What changes would you expect to see on a blood film?
2) Name 3 other causes of microcytic anaemia
3) What are 3 signs of iron deficiency anaemia?

A

1) Microcytic hypochromic RBCs, anisocytosis (variation in size of RBCs), poikilocytosis (irregular shaped RBCs)
2) Sideroblastic anaemia, anaemia of chronic disease, thalassaemia
3) Koilonychia (spoon shaped nails), angular stomatitis, atrophic glossitis, pallor

Phase 2a (25 decks)

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