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2A revision > Haematology > Flashcards

Haematology Flashcards

1
Q

what is anaemia

A
  • low haemoglobin concentration, may be due either to a low red cell mass or increased plasma volume
  • reduced production or increased loss of RBCs
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2
Q

symptoms of anaemia

A
  • Fatigue
  • Dyspnoea
  • Faintness
  • Palpitations
  • Headache
  • Tinnitus
  • Anorexia
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3
Q

what are the ranges for anaemia in men and women

A

Low Hb is <135g/L for men and <115g/L for women

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4
Q

signs of anaemia

A
  • May be absent
  • Pallor
  • Hyperdynamic circulation e.g. tachycardia, flow murmurs and cardiac enlargement
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5
Q

consequences of anaemia

A

-Reduced O2 transport
-Tissue hypoxia
-Compensatory changes:
>Increase tissue perfusion
>Increase O2 transfer to tissues
>Increase red cell production

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6
Q

pathological consequences of anaemia

A
  • Myocardial fatty change
  • Fatty change in liver
  • Aggravate angina/ claudication
  • Skin and nail atrophic changes
  • CNS cell death (cortex and basal ganglia)
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7
Q

what is MCV and how does it separate the types of anaemia

A
  • mean cell volume
  • normal = 76-96 femtolitres
  • low MCV = microcytic anaemia
  • normal = normocytic
  • high = macrocytic
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8
Q

what are the 3 causes of microcytic anaemia

A
  1. Iron-deficiency anaemia – most common cause
  2. Thalassaemia
  3. Sideroblastic anaemia – very rare
    - anaemia of chronic disease is an example
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9
Q

what are 7 causes of normocytic anaemia

A
  • Acute blood loss
  • Anaemia of chronic disease
  • Bone marrow failure
  • Renal failure
  • Hypothyroidism
  • Haemolysis
  • Pregnancy
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10
Q

examples of normocytic anaemia

A
  • Aplastic anaemia
  • Haemolytic – thalassaemia, sickle cell disease, G6PD
  • Infections – malaria
  • Haemorrhage
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11
Q

8 causes of macrocytic anaemia

A
  1. B12 or folate deficiency
  2. Alcohol excess – or liver disease
  3. Reticulocytosis
  4. Cytotoxics
  5. Myelodysplastic syndromes
  6. Marrow infiltration
  7. Hypothyroidism
  8. Antifolate drugs
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12
Q

examples of macrocytic anaemia

A

Megaloblastic
B12/folate deficiency
Pernicious anaemia

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13
Q

what can haemolytic anaemias be

A
  • normocytic or macrocytic
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14
Q

investigations for anaemia

A
  • FBC and film, reticulocyte count, U&E, LFT, TSH, B12, folate, ferritin
  • B12 deficiency = intrinsic factor Abs, Shilling test, coeliac Abs, B12 replacement
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15
Q

causes of iron deficiency anaemia (IDA)

A
  • blood loss (haemorrhage, GI bleeding, menorrhagia)
  • poor diet or poverty
  • malabsorption ( coeliac disease)
  • hook worm causes GI blood loss in tropics
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16
Q

pathology of IDA

A
  • inadequate iron supply so interrupts the final step in haem synthesis
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17
Q

signs of IDA

A
  • Tiredness
  • Often asymptomatic
  • Rare – koilonychias, angular cheilosis and glossitis
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18
Q

tests for IDA

A
  • Blood film: microcytic, hypochromic anaemia
  • Decreased MCV, MCH and MCHC
  • Low ferritin
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19
Q

treatment for IDA

A
  • treat cause
  • ferrous sulfate = oral iron = can cause nausea and diarrhoea
  • use for at least 3 months
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20
Q

what is anaemia of chronic disease

A
  • microcytic/normocytic

- Reduced Hb related to chronic inflammatory disorders, chronic infections and malignancy

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21
Q

pathology of anaemia of chronic disease

A
  • inflammatory cytokines = reduce sensitivity of bone marrow to erythropoietin and lead to failed incorporation of iron into developing red cells
  • arise from 3 problems = poor use of iron in erythropoiesis, cytokines shorten RBC survival, decreased production and response to erythropoietin)
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22
Q

causes of anaemia of chronic disease

A
  • Chronic infection, chronic inflammatory disease or malignancy
  • Vasculitis
  • Autoimmune disorders - Rheumatoid
  • Renal failure
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23
Q

tests for anaemia of chronic disease

A
  • ferritin normal

- B12, folate, TSH and tests for haemolysis

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24
Q

treatment for anaemia of CD

A

-Treat underlying disease
-Erythropoietin (raise Hb levels)
Side effects: flu-like symptoms, hypertension, mild rise in platelet count
-IV iron (overcome functional iron deficiency)

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25
Q

what is sideroblastic anaemia

A
  • bone marrow produces ringed sideroblasts rather than healthy red blood cells
  • ineffective erythropoiesis, leading to increased iron absorption and iron loading in marrow
  • enough iron but can’t incorporate it
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26
Q

causes of sideroblastic anaemia

A
  • Congenital – rare X-linked
  • Chemotherapy
  • Anti-TB drugs
  • Alcohol excess
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27
Q

tests for sideroblastic anaemia

A
  • Increased ferritin
  • Hypochromic blood film
  • Disease-defining sideroblasts in the marrow
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28
Q

treatment for sideroblastic anaemia

A

Remove the cause
Pyridoxine (vit B6)
Repeated transfusions for severe anaemia

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29
Q

megaloblastic causes of macrocytic anaemia

A
  • a megaloblast is a cell in which nuclear maturation is delayed compared with the cytoplasm.
  • B12 and folate deficiency: both are required for DNA synthesis
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30
Q

non-megaloblastic causes of macrocytic anaemia

A

alcohol excess, reticulocytosis, liver disease, hypothyroidism.

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31
Q

other haematological causes of macrocytic anaemia

A

myelodysplasia, myeloma, myeloproliferative disorders, aplastic anaemia

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32
Q

tests for macrocytic anaemia

A
  • B12 and folate deficiency
  • blood film = hyper segmented neutrophils in B12 and serum folate
  • bone marrow biopsy = megaloblastic or normoblastic marrow, abnormal or increase erythropoiesis
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33
Q

what is folate and folate deficiency

A
  • in green vegetables, nuts, yeast, and liver; it is synthesised by gut bacteria.
  • Maternal folate deficiency causes foetal neural tube defects.
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34
Q

causes of folate deficiency

A
  • Poor diet
  • Increased demand e.g. pregnancy or increased cell turnover
  • Malabsorption e.g. coeliac disease
  • Alcohol
  • Drugs: anti-epileptics, methotrexate
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35
Q

tests for folate deficiency

A
  • Blood film – anisocytosis and poikilocytosis with large oval macrocytes
  • Serum and red cell folate assay
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36
Q

treatment for folate deficiency

A
  • underlying cause
  • folic acid 5mg/day for 4 months
  • pregnancy = prophylactic doses of 400mcg/day given until 12 weeks (prevents spina bifida)
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37
Q

what is pernicious anaemia

A
  • autoimmune condition in which atrophic gastritis leads to a lack of IF secretion from the parietal cells of the stomach.
  • Dietary B12 therefore remains unbound and consequently cannot be absorbed by the terminal ileum
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38
Q

causes of B12 deficiency

A
  • Dietary
  • Malabsorption during digestion
  • Congenital metabolic errors
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39
Q

pathology of pernicious anaemia

A
  • Autoimmune gastritis affecting the fundus.
  • Parietal and chief cells are replaced with mucin-secreting cells = no IF secretion = B12 deficiency
  • B12 and folate needed for DNA synthesis so red cell development stops = remain as megaloblasts
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40
Q

differential diagnoses for pernicious anaemia

A
  • other autoimmune diseases
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41
Q

clinical presentation of pernicious anaemia/ B12 deficiency

A

-Symptoms of anaemia
-Mild jaundice due to haemolysis
-B12 deficiency causes:
>Neurological – paraesthesia, peripheral neuropathy, subacute combined degeneration of spinal cord
> irritability, depression, psychosis and dementia

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42
Q

tests for pernicious anaemia/ B12 deficiency

A
  • FBC = decreased Hb, increased MCV, WCC and platelets low
  • reticulocytes may be low
  • megaloblasts in bone marrow
  • parietal cell Abs, IF Abs
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43
Q

treatment for pernicious anaemia/ B12 deficiency

A
  • treat cause
  • malabsorption = hydroxocobalamin to replenish B12 stores
  • oral B12
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44
Q

what is haemolytic anaemia

A
  • due to haemolysis = abnormal breakdown of RBCs
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45
Q

immune mediated acquired haemolytic anaemia

A
  • direct antiglobulin test +ve
  • Drug-induced: binding to RBC membranes or production of immune complexes.
  • Autoimmune haemolytic anaemia: autoantibodies causing extravascular haemolysis and spherocytosis.
  • Paroxysmal cold haemoglobinuria: seen with viruses/syphilis
  • Isoimmune: acute transfusion reaction
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46
Q

direct antiglobulin acquired haemolytic anaemia

A
  • Coombs -ve

- Autoimmune hepatitis; hepatitis B and C; post flu and other vaccinations, drugs

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47
Q

microangiopathic haemolytic anaemia

A

intravascular haemolysis and schistocytes

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48
Q

infection acquired haemolytic anaemia

A
  • from malaria = RBC lysis and haemoglobinuria
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49
Q

hereditary haemolytic anaemia

A
  1. enzyme defect = G6PD and pyruvate kinase deficiency
  2. membrane defects = spherocytosis, elliptocytosis
  3. haemoglobinopathy = sickle cell, thalassaemia
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50
Q

what is aplastic anaemia

A
  • bone marrow failure
  • defined as pancytopenia with hypoplastic marrow (stops making cells)
  • pancytopenia = reduced numbers of all major cell lines
  • reduction in pluripotent stem cells
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51
Q

causes of aplastic anaemia

A 
Autoimmune 
Drugs 
Viruses – parvovirus, hepatitis 
Irradiation 
Inherited – Fanconi anaemia
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52
Q

symptoms of aplastic anaemia

A
  • bruising, blood blisters in mouth
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53
Q

what is polycythaemia vera

A
  • abnormal increase in the number of circulating RBCs
  • malignant proliferation of a clone (haematopoietic myeloid stem cells) derived from somatic mutation of one pluripotent stem cell
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54
Q

clinical manifestations of polycythaemia vera

A
  • vague symptoms due to hyper viscosity
  • characteristic = Itching after a hot bath, Erythromelalgia, Burning sensation in fingers and toes
  • splenomegaly
  • gout
  • facial plethora
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55
Q

investigations for polycythaemia vera

A
  • increased: RCC, Hb, PCV, WBC, platelets, HCT
  • JAK2 testing (present in most)
  • marrow = hyper cellularity, erythroid hyperplasia
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56
Q

management of polycythaemia vera

A
  • aim to keep haematocrit (HCT) < 0.45 to decrease risk of thrombosis
  • low dose aspirin
  • complications = ay progress to acute myeloid leukaemia
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57
Q

sickle cell disease pathology

A
  • inherited AR
  • abnormal Hb results in vaso-occlusive crises
  • amino acid substitution in gene coding for beta Hb chain = production of HbS instead of HbA
  • HbS is 50x less soluble and under low O2 conditions it polymerises into rods causing sickle shape
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58
Q

genetics of sickle cell disease

A
  • HbS from mutation in beta globin gene on chromosome 4
  • homozygotes (SS) = SICKLE CELL ANAEMIA
  • heterozygotes (AS)= sickle cell trait (protects against malaria)
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59
Q

clinical presentation of sickle cell disease

A
  • Asymptomatic
  • Asthenia (abnormal weakness/ lack of energy)
  • Jaundice
  • Ulcers around the ankles
  • Bone deformities
  • Infections
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60
Q

painful vaso-occlusive crises in sickle cell

A
  • microvascular occlusion
  • ribs, spine, pelvis, tummy, legs and arms and hands/feet (particularly in children).
  • Affects the marrow, causing severe pain, triggered by cold, dehydration, infection or hypoxia
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61
Q

complications of sickle cell disease

A
  • Acute complications (Painful crisis, Sickle chest syndrome, Stroke)
  • Chronic complications (Renal impairment, Pulmonary hypertension, Joint damage)
62
Q

investigations for sickle cell disease

A
  • Neonatal screening
  • Hb electrophoresis – one major single HbS band and no normal HbA.
  • Blood test - Increased reticulocytes, increased bilirubin.
  • Film – sickle cells and target cells.
  • Sickle solubility test - +ve
63
Q

management of sickle cell disease

A

Transfusion
Hydroxycarbamide (increases foetal haemoglobin)
Stem cell transplant

64
Q

what is Thalassaemia

A
  • inherited RBC disorder
  • underproduction of alpha or beta chains
  • accumulation of unpaired chains so developing RBC are destroyed by spleen.
65
Q

beta thalassaemia

A
  • mutations in β-globin genes on chromosome 11, leading to decreased β chain production, or its absence
  • minor = carrier state, usually asymptomatic
  • intermedia = moderate anaemia, splenomegaly but no need for regular transfusions
  • major = homozygous, severe anaemia, lifelong transfusions
66
Q

clinical presentation of beta thalassaemia major

A
  • Failure to feed
  • Listless (lacking energy)
  • Crying
  • Pale
  • Extra-medullary haematopoiesis = head shape (skull bossing) and hepatosplenomegaly.
67
Q

investigations for beta thalassaemia

A
  • FBC – 40-70g/L Hb, MCV and MCH very low
  • Film – large and small very pale red cells, nucleated RBCs
  • Hb electrophoresis
68
Q

treatment for beta thalassaemia

A
  • Regular transfusions
  • Iron chelation (removal of excess iron)
  • Endocrine supplementation
  • A histocompatible marrow transplant can offer chance of cure
  • need regular monitoring of ferritin, cardiac +liver MRI, endocrine testing
69
Q

iron overload in thalassaemia

A
  • life long blood transfucions increases body iron load, no way to excrete
  • iron deposited in liver and spleen ( also endocrine glands and heart) = fibrosis and cirrhosis
70
Q

alpha thalassaemia

A
  • two separate α-globin genes on each chromosome 16 – four genes termed αα/αα.
  • 4 genes are deleted, death is in utero – Bart’s hydrops.
  • 3 genes are deleted (–/-α), HbH disease occurs – moderate anaemia and features of haemolysis (hepatosplenomegaly, leg ulcers, jaundice).
  • 2 genes are deleted (–/αα) – asymptomatic carrier state
  • one gene is deleted – normal clinical state
71
Q

what are membranopathies

A
  • AD conditions
  • abnormally shaped RBC
  • Deficiency of red cell membrane proteins caused by a variety of genetic lesions
  • spherocytosis, elliptocytosis
  • need folate, splenectomy may help
72
Q

Glucose-6-phosphate dehydrogenase (G6PD) deficiency

A
  • x linked recessive
  • lack of enzyme that protects against oxidative stress
  • decreased glutathione production
  • oxidative crises (rapid anaemia and jaundice) caused by drugs, broad beans, illness, henna
  • enzyme assay and blood film for bite and blister cells
  • transfusions if severe
73
Q

pyruvate kinase deficiency

A
  • AR
  • decreased ATP causes decreased RBC survival
  • enzyme assay
  • splenectomy may help and transfuse during crisis
74
Q

platelet physiology

A
  • Produced in bone marrow
  • Megakaryocyte fragments
  • Regulated by thrombopoietin – produced in the liver
  • Low platelets = reduced bound TPO = increased free TPO = increased megakaryocyte stimulation = increased platelets
75
Q

clinical presentation of platelet dysfunction

A
  • Mucosal bleeding
  • Easy bruising
  • Petechiae, purpura (red/purple discoloured skin)
  • Traumatic haematomas
76
Q

what causes low platelets

A
  • decreased marrow production
  • excess destruction
    > immune thrombocytopenia = IgG Abs against platelet surface glycoproteins and opsonize platelets, primary = following viral infection/ vaccination, secondary = associated with malignancy and infection
    > thrombotic thrombocytopenia = widespread adhesion and aggregation causes microvascular thrombosis
77
Q

impaired platelets

A
  • Poorly functioning platelets: myeloproliferative disease
  • Congenital – Von Willebrand disease
  • Acquired – uraemia, drugs
78
Q

dysfunctional production of platelets in bone marrow

A
  • myelodysplasia
79
Q

investigations for platelet abnormalities

A
  • FBC – isolated thrombocytopenia
  • Raised lactate dehydrogenase levels (from ischaemic or necrotic cells)
  • Physical examination – signs of bleeding or splenomegaly
80
Q

management of platelet abnormalities

A
  • immunosuppression
  • treat cause
  • clopidogrel/ aspirin inhibit platelets
  • if bleeding give platelets - disappear quickly
  • tranexamic acid = inhibits breakdown of fibrin to stop mucosal bleeding
  • splenectomy
81
Q

causes of DVT

A
  • Circumstantial (Surgery, Immobilisation, Long haul flights)
  • Genetic (Factor V Leiden, Antithrombin deficiency)
  • Acquired (Anti-phospholipid syndrome, Lupus anticoagulant)
82
Q

prevention of DVT

A
  • hydration, mobilisation, compression stockings, foot pumps

- LMW heparin

83
Q

clinical manifestations of DVT

A
  • pain swelling

- tenderness, warmth, decolourisation

84
Q

investigations for DVT

A
  • D-dimer - normal excludes diagnosis but positive doesn’t confirm
  • US compression test proximal veins
  • venogram for calf
85
Q

treatment for DVT

A
  • Oral warfarin
  • LMWH
  • DOAC (direct oral anticoagulant)
  • Compression stockings
  • Treat underlying cause
86
Q

what is a PE

A
  • blockage of a pulmonary artery

- often from blood clots from DVTs

87
Q

pathology of PE

A

-large PE is haemodynamically significant = block inflow of blood to lungs = can’t oxygenate the blood = hypotension, cyanosis, severe dyspnoea and right heart strain/failure

88
Q

clinical manifestations of PE

A
  • pleuritic chest pain, SOB, cyanosis

- tachycardia, tachypnoea, pleural

89
Q

investigations for PE

A
  • CXR usually normal
  • ECG – sinus tachycardia
  • Blood gases: type 1 respiratory failure, decreased O2 and CO2
  • D-dimer: normal excludes diagnosis
  • Ventilation/perfusion scan: mismatch defects
90
Q

management of PE

A

LMW heparin
DOAC
Treat the cause

91
Q

Von Willebrand Disease

A
  • inherited coagulation disorder
  • lack of vWF leads to failure of platelet adhesion and factor VIII deficiency
  • mucosal bleeding (nose) and severe bleeding after injury
92
Q

Haemophilia

A
  • inherited disorder of haemostasis characterised by bleeding tendency due to a deficiency of either factor VIII (haemophilia A) or factor IX (haemophilia B)
  • x linked recessive
  • lack of VIII-factor IX complex so no activation of factor X so impairs clotting
93
Q

haemophilia A

A
  • Factor VIII deficiency
  • haematomas, bleeding into joints = crippling arthropathy
  • increased APTT and decreased factor VIII assay
  • avoid NSAIDs and im injections
  • desmopressin raises factor VIII levels
94
Q

haemophilia B

A
  • factor IX deficiency

- treat with recombinant factor IX

95
Q

liver disease and bleeding disorders

A
  • complicated bleeding disorder

- decreased synthesis of clotting factors, decreased absorption of vitamin K, abnormalities of platelet function.

96
Q

malabsorption and bleeding disorders

A
  • less uptake of vit K

- IV vit K

97
Q

what is leukaemia

A

Malignant proliferation of haemopoietic cells

98
Q

what is acute lymphoblastic leukaemia (ALL)

A
  • malignancy of lymphoid cells
  • affecting B/T lymphocyte cell linages
  • arresting maturation and promoting uncontrolled proliferation of immature blast cells, with marrow failure and tissue infiltration
99
Q

signs and symptoms of ALL

A
  • marrow failure = anaemia
  • infections = tonsillitis and fevers
  • bleeding = thrombocytopenia
  • hepato/ splenomegaly, lymphadenopathy
100
Q

tests for ALL

A
  • blast cells of blood film and bone marrow
  • WCC usually high
  • CXR and CT = look for lymphadenopathy
  • lumbar puncture
101
Q

treatment for ALL

A
  • support = transfusions, IV fluids, Hickman line for IV access
  • infections = IV antibiotics + prophylactic treatment
  • chemo
102
Q

what is acute myeloid leukaemia (AML)

A

Neoplastic proliferation of blast cells derived from marrow myeloid elements

103
Q

signs and symptoms of AML

A
  • marrow failure

- infiltration = hepatosplenomegaly, gum hypertrophy, skin involvement

104
Q

diagnosis of AML

A
  • WCC increased

- bone marrow biopsy = Auer rods

105
Q

treatment for AML

A
  • chemo (Daunorubicin and cytarabine)
  • supportive measures (transfusions, IV fluids etc)
  • bone marrow transplant (Pluripotent haematopoietic stem cells)
106
Q

what is chronic myeloid leukaemia (CML)

A

uncontrolled clonal proliferation of myeloid (blood-forming tissue in the bone marrow) cells
- Philadelphia chromosome (chromosome 9 and 22 translocation)

107
Q

symptoms of CML

A
  • Mostly chronic and insidious
  • Weight loss
  • Tiredness
  • Fever
  • Sweats
  • May be features of gout
  • Bleeding
  • Abdominal discomfort
108
Q

signs of CML

A
  • splenomegaly
  • Hepatomegaly
  • Anaemia
  • Bruising
109
Q

tests for CML

A
  • WBC – increased neutrophils, monocytes, basophils, eosinophils
  • Film: left shift + basophilia
  • Philadelphia chromosome
  • Cytogenetic analysis of blood or bone marrow for Ph
  • Increased urate
110
Q

treatment of CML

A
  • Target molecular therapy – tyrosine kinase inhibitors: Imatinib
  • hydroxycarbamide
111
Q

what is chronic lymphocytic leukaemia (CLL)

A
  • most common leukaemia.
  • progressive accumulation of a malignant clone of functionally incompetent beta cells.
  • Mutations, trisomy’s and deletions influence risk.
112
Q

signs of CLL

A
  • Enlarged, rubbery, non-tender nodes
  • Splenomegaly
  • Hepatomegaly
113
Q

tests for CLL

A
  • Increased lymphocytes
  • Autoimmune haemolysis
  • Marrow infiltration; decreased Hb, neutrophils, platelets
114
Q

treatment of CLL

A
  • supportive (transfusions, IV immunoglobulin)
  • stem cell transplant
  • radiotherapy (lymphadenopathy and splenomegaly)
  • chemo (rituximab and fludarabine)
115
Q

what is a lymphoma

A

malignant growth of white blood cells – predominantly the lymph nodes but also found in the blood, bone marrow, liver and spleen

116
Q

pathology of lymphoma

A
  • malignant proliferation of lymphocytes = accumulate in lymph nodes = lymphadenopathy
  • histologically divided into Hodgkin’s and non-Hodgkin’s lymphoma
117
Q

what is Hodgkin’s lymphoma

A

Characteristic cells with mirror-image nuclei are found, called Reed-Sternberg

118
Q

symptoms of Hodgkin’s lymphoma

A
  • Enlarged, non-tender, rubbery superficial lymph nodes.
  • Node size will fluctuate and become matted
  • Constitutional upset – fever, weight loss, night sweats, pruritus, lethargy.
  • Alcohol-induced lymph pain
119
Q

signs of Hodgkin’s lymphoma

A
  • lymphadenopathy
  • cachexia ( wasting of body)
  • anaemia
  • spleno/hepatomegaly
120
Q

investigations for Hodgkin’s lymphoma

A
  • lymph node excision biopsy, image guided biopsy

- CXR, CT/PET

121
Q

Ann-Arbor staging of Hodgkin’s lymphoma

A

I.Confined to single lymph node region
II.Involvement of two or more nodal areas on the same side of the diaphragm
III.Involvement of nodes on both sides of the diaphragm
IV.Spread beyond the lymph nodes on both sides of the diaphragm
- A = no systemic symptoms
- B = systemic symptoms = worse disease

122
Q

management of Hodgkin’s lymphoma

A
  • Stages IA-IIA: radiotherapy + short courses of chemotherapy
  • Stages IIA-IVB (with >3 areas involved): longer courses of chemotherapy
  • good long term survival
123
Q

non-Hodgkin’s lymphoma

A
  • all without Reed-Sternberg cells
124
Q

causes of non-H lymphoma

A
  • immunodeficiency

- infection ( EBV transform cells, Helicobacter pylori)

125
Q

symptoms and signs of non-H lymphoma

A
  • superficial lymphadenopathy
  • extra-nodal disease
  • gut = dyspepsia, weight loss, dysphagia
  • small bowel lymphoma = diarrhoea, vomit, abdo pain
  • oropharynx = obstructed breathing
  • systemic features
  • pancytopenia from marrow involvement
126
Q

investigations for non-H lymphoma

A
  • bloods
  • marrow and node biopsy
  • CT/PET
127
Q

management of non-H lymphoma

A
  • low grade = indolent, incurable, radio and chemo , rituximab maintains remission
  • high grade= more aggressive, often curable (Burkitt’s lymphoma), R-CHOP chemo regimen
128
Q

what is myeloma

A

malignant tumour of the bone marrow involving myeloma cells (a type of WBC)

129
Q

multiple myeloma

A
  • neoplastic proliferation of bone marrow plasma cells
  • Monoclonal protein in serum/urine
  • Lytic bone lesions/ CRAB end organ damage
  • Excess plasma cells in bone marrow
130
Q

pathology of myeloma

A
  • plasma cell dyscrasias = secretion of IgG/IgA= dysfunction of many organs
  • Clonal expansion -> monoclonal gammopathy of undetermined significance (MGUS) -> early myeloma -> late myeloma -> plasma cell leukaemia
  • increases osteoclast activity
131
Q

what can myeloma cause in the kidneys

A

AKI from light chain deposition and amyloid deposition, hypercalcaemia and hyperuricaemia

132
Q

signs of myeloma

A
  • back pain and fractures
  • anaemia
  • Abnormal FBC
  • Osteolytic lesions
  • Renal failure
  • Hypercalcaemia
  • Raised globulins
  • Raised ESR
133
Q

investigations for myeloma

A
  • protein in blood
  • more plasma cells in bone marrow
  • CRAB (calcium, renal, anaemia, bone)
134
Q

management of myeloma

A
  • analgesia
  • bisphosphonate to reduce fractures
  • radio therapy
  • chemo with stem cell transplant
  • transfusions
  • broad spec Abx for infections
135
Q

what is malaria

A
  • infectious disease caused by protozoan parasites from the Plasmodium family – transmitted by the bite of the Anopheles mosquito or by a contaminated needle or transfusion.
  • Most commonly P. falciparum.
  • P. vivax, P. ovale, P. malariae
136
Q

pathology of malaria

A
  • can be transmitted vertically (pregnancy)
  • main cause = bite of an infected female Anopheles mosquito
  • once infected, travels to liver to mature (produces Schizonts), ruptures then asexual reproduction in RBCs, rupture of erythrocytic schizonts
137
Q

clinical manifestations of malaria

A
  • Fever – consider everyone to have malaria with fever who has visited a malarial area
  • Headache
  • Malaise
  • Myalgia
  • Diarrhoea
  • Cough
  • Delayed diagnosis – jaundice, confusion, seizures
138
Q

investigations for malaria

A
  • immediate blood testing = Microscopy of thick and thin blood smear with giemsa stain = repeated after 12h and 24h
  • travel history
  • rapid diagnostic test detection of parasite antigen
139
Q

management of malaria

A

Artemisinin combination therapy (ACT) achieve rapid clearance of parasites by combined action at different stages of the parasite cycle

140
Q

what is disseminated intravascular coagulation

A

acquired syndrome characterised by activation of coagulation pathways, resulting in formation of intravascular thrombi and depletion of platelets and coagulation factors.

141
Q

signs and symptoms of DIC

A

bleeding, cough, dyspnoea, fever, delirium, petechiae, hypoxia, hypotension

142
Q

management of DIC

A
  • aggressive treatment of cause
  • fresh frozen plasma
  • platelet concentrate and other clotting factors
  • may need heparin
143
Q

causes of DIC

A
  • major trauma
  • sepsis, severe infection
  • obstetric disorders
  • malignancy
  • vascular disorders
  • severe toxic or immune reactions
144
Q

febrile neutropenia

A
  • Can be a complication of chemotherapy
  • Low WBC with a high fever
  • Neutropenia means immune system is weaker and infections can take hold rapidly so need prompt treatment
145
Q

haemochromatosis

A
  • too much iron
  • causes fatigue, liver problems and joint problems, bronzed skin, sexual and psychological disturbance
  • need regular bleeding and low iron diet
146
Q

immune thrombocytopenic purpura

A
  • abs against platelets
  • non-blanching purpura
  • immune suppression - pred, IV immunoglobulins, rituximab (monoclonal ab against B cells)
147
Q

thrombotic thrombocytopenic purpura

A
  • tiny thrombi in vessels use up platelets - microangiopathy
  • thrombocytopenia, purpura, tissue ischaemia/organ damage
  • reduction in protein ADAMTS13 (normally reduces clotting)
148
Q

heparin induced thrombocytopenia

A
  • ab’s against platelets in response to heparin (target platelet factor 4 protein)
  • presents 5-10 days after with clots and low platelets
  • test for HIT abs
  • stop heparin and use other anticoagulant
149
Q

acute transfusion reactions

A
  • within 24h
  • acute haemolytic, febrile non-haemolytic, allergic, and transfusion-related acute lung injury (TRALI).
150
Q

delayed transfusion reaction

A
  • days to weeks after
  • delayed haemolytic transfusion reactions, transfusion-associated graft-versus-host disease, and post-transfusion purpura.
151
Q
A

2A revision (10 decks)

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