Haematology Flashcards
1
Q
Define iron-deficiency anaemia
A
Lack of iron.
2
Q
Define pernicious anaemia ‘B12 deficiency’
A
Not enough RBC due to a lack of B12.
3
Q
Define folate-deficiency
A
Lack of folate.
4
Q
Define haemolytic anaemia
A
Due to increased destruction of red blood cells.
5
Q
How does iron-deficiency anaemia clinically present?
A
Anaemia: Fatigue, lethargy, dyspnoea, faintness, palpitations, headache.
Iron deficiency: brittle hair and nails, atrophic glossitis and angular stomatitis.
6
Q
How does pernicious anaemia ‘B12 deficiency’ clinically present?
A
Anaemia: Fatigue, lethargy, dyspnoea, faintness, palpitations, headache.
B12 deficiency: neurological problems.
7
Q
How does folate-deficiency clinically present?
A
Anaemia: Fatigue, lethargy, dyspnoea, faintness, palpitations, headache
Folate deficiency: Develops over 4 months of deficiency (due to bodily reserves).
Possibly depression.
Glossitis.
8
Q
How does haemolytic anaemia clinically present?
A
Anaemia: Fatigue, lethargy, dyspnoea, faintness, palpitations, headache.
Haemolytic: Jaundice, gall stones, leg ulcers, signs of underlying cause.
9
Q
Iron-deficiency anaemia - note
A
Microcytica.
10
Q
Pernicious anaemia ‘B12 deficiency’ - note
A
Macrocytic,
megaloblastic.
11
Q
Folate-deficiency - note
A
Macrocytic,
megaloblastic.
12
Q
Haemolytic anaemia - note
A
Normocytic.
13
Q
Pathophysiology of Iron-deficiency anaemia
A
Iron is necessary for the formation of haem.
Insufficient iron
- > lack of effective rbc
- > symptoms of anaemia.
14
Q
Pathophysiology of Pernicious anaemia ‘B12 deficiency’
A
Absorption of B12 occurs in the terminal ileum and requires Intrinsic Factor (from gastric parietal cells) for transport across intestinal mucosa.
This IF is deficient in pernicious anaemia.
This causes megaloblastic anemia.
15
Q
Pathophysiology of Folate-deficiency
A
Absorbed in the upper intestine.
Insufficient folate causes megaloblastic anaemia.
This is where erythrocytes are larger and have higher nuclear to cytoplasmic ratios to normal.
16
Q
Pathophysiology of Haemolytic anaemia
A
RBCs are destroyed before their usual 120 day lifespan.
The bone marrow provides compensatory reticulocytes.
RBC destruction can be extra or intra vascular.
Mostly extravascular, where cells are removed from circulation by macrophages, particularly in the spleen.
17
Q
Cause of Iron-deficiency anaemia
A
Blood loss (most common),
increased demands (growth and pregnancy),
decreased absorption (small bowel disease), poor intake.
18
Q
Cause Pernicious anaemia ‘B12 deficiency’
A
Autoimmune destruction of parietal cells/IF.
19
Q
Cause of Folate-deficiency
A
Main cause is poor intake, due to dietary deficiency.
Also possible is an excessive requirement, impaired uptake or antifolate drugs.
20
Q
Cause of Haemolytic anaemia
A
Inherited: Red cell membrane defect (sphereocytosis),
Haemoglobin abnormalities, metabolic defects
Acquired: Autoimmune,
Mechnical destruction,
secondary to systemic disease (liver failure),
infections (malaria).
21
Q
Epidemiology of Iron-deficiency anaemia
A
2-5% of men and post menopausal.
Premenopausal are at higher risk due to menses.
22
Q
Epidemiology of Pernicious anaemia ‘B12 deficiency’
A
1/10,000 in N Europe.
Peak age of 60.
23
Q
Epidemiology of Haemolytic anaemia
A
Depends on underlying cause.
Sickle cell mainly African peoples.
Autoimmune slightly more common in females.
24
Q
Diagnostic tests for Iron-deficiency anaemia
A
FBC: hypochromic microcytic anaemia
Serum ferritin: low.
25
Diagnostic tests for Pernicious anaemia 'B12 deficiency'
Blood film: Macrocytic rbc
Autoantibody: IF antibodies
26
Diagnostic tests for Folate-deficiency
Blood film: Macrocytic
Erythrocyte folate level: Indicated reduced body stores
27
Diagnostic tests for Haemolytic anaemia
Reduced haemoglobin, spherocytes, increased reticulocytosis, increased MCV
28
Treatment for Iron-deficiency anaemia
Iron salts oral.
29
Treatment for Pernicious anaemia 'B12 deficiency'
(Vitamin B12) Hydroxocobalamin.
If neuro involvement; refer to haematologist.
Do NOT give folic acid instead of B12, this leads to fulminant neurological deficit.
30
Treatment for Folate-deficiency
Folic acid supplement.
Treat any underlying cause.
Advise regarding folate deficiency in pregnancy.
31
Treatment for Haemolytic anaemia
Folate and iron supplement,
immunosuppressive if autoimmune,
splenectomy if hereditary spherocytosis or other approaches fail.
32
Complications of Iron-deficiency anaemia
Side effects of iron salts: black stool,
constipation/diarrhoea,
nausea.
33
Complications of Pernicious anaemia 'B12 deficiency'
Heart failure,
angina,
neuropathy.
34
Complications of Folate-deficiency
Folate required for foetus development.
35
Complications of Haemolytic anaemia
Cardiac failure.
36
Define Bone marrow failure 'Aplastic'
Lack of haemopoiesis as a result of bone marrow failure.
37
Define Sickle cell anaemia
Hereditary deformation of RBC as a result of faulty haemoglobin molecule.
38
Define Thalassaemia
Defective subunit of the haemaglobin complex .
39
Define Glucose-6-phosphate dehydrogenase deficiency
Lack of enzyme that maintains protective protein against oxidant injury.
40
Types of Thalassaemia
Alpha and Beta (for which subunit affected).
41
How does Bone marrow failure 'Aplastic' clinically present?
Anaemia: Fatigue, lethargy, dyspnoea, faintness, palpitations, headache.
Bone marrow failure: Increased suspectability to infection and bleeding.
Bruising, bleeding gums and epistaxis.
42
How does Sickle cell anaemia clinically present?
Presents once Hb F has circulated out at about 6 months.
Vaso-occlusion: Early childhood acute pain in the hands and feet due to occlusion of the small vessels and avascular necrosis of the bone marrow.
In adults, this affects the long bones, ribs, spine and pelvis.
Variable frequency.
Avascular necrosis -> shortened bones in children.
Anaemia: Fatigue, lethargy, dyspnoea, faintness, palpitations, headache. (Often symptoms of anaemia do not appear, since Hb S release oxygen easily.
43
How does Thalassaemia clinically present?
Variable.
Alpha tends to present in utero, whilst beta in infancy.
Can be asymptomatic if heterozygote, or severe anaemia in homozygotes, with failure to thrive and bone deformities (due to hypertrophy of ineffective marrow).
44
How does Glucose-6-phosphate dehydrogenase deficiency clinically present?
Neonatal jaundice,
haemolytic anaemia,
and acute haemolysis (precipitated by fava beans).
45
Thalassaemia - note
Microcytic
46
Pathophysiology of Bone marrow failure 'Aplastic'
Reduction in the number of pluripotential stem cells together with a fault in those remaining or a immune reaction against them, such that they are unable to repopulate.
This can occur in only one cell line, leading to isolated deficiencies.
47
Pathophysiology of Sickle cell anaemia
Changes in a sequence of a haemoglobin subunit causes faulty haemoglobin complex.
This distorts the shape of RBC into sickles when deoxygenated, which are easily destroyed and occlude vessels easily.
This process worsens with repeated oxygenation/deoxygenation
Heterozygotes have minor effect, but protect against malaria.
48
Pathophysiology of Thalassaemia
Defective versions of either alpha or beta subunits of haemoglobin
- > imbalance of subunits available
- > precipitation of globin chains within rbc (or precursor)
- > cell damage,
death of precursor (ineffective haemolysis) and haemolysis.
49
Pathophysiology of Glucose-6-phosphate dehydrogenase deficiency
G6PD is an enzyme in the pentose monophosphate shunt, which maintains glutathione in the reduced state.
This protects against oxidant injury in the RBC.
Therefore lack of G6PD -> increased haemolysis.
50
Causes of Bone marrow failure 'Aplastic'
Congenital, acquired (mostly),
cytotoxic drugs,
infections etc
51
Cause of Sickle cell anaemia
Autosomal recessive condition affecting haemoglobin B subunits.
52
Cause of Thalassaemia
Genetic.
Homozygotes have severe anaemia,
heterozygotes can vary.
53
Cause of Glucose-6-phosphate dehydrogenase deficiency
X linked recessive
54
Epidemiology of Bone marrow failure 'Aplastic'
2/1,000,000.
More common in Asia.
55
Epidemiology of Sickle cell anaemia
More common in African populations;
sickle cell trait protects against malaria.
56
Epidemiology of Thalassaemia
1% carriers of beta
and 5% carriers of alpha.
57
Epidemiology of Glucose-6-phosphate dehydrogenase deficiency
Most common metabolic RBC disorder.
58
Diagnostic test for Bone marrow failure 'Aplastic'
FBC: Pancytopenia with low reticulocytes.
Bone marrow biopsy: hypocellular marrow with increased fat spaces.
59
Diagnostic test for Sickle cell anaemia
Identified in neonatal screening.
Otherwise, blood film: sickled cells.
60
Diagnostic test for Thalassaemia
Either genetic testing
or haemoglobin electrophoresis (after identifying microcytosis).
61
Diagnostic test for Glucose-6-phosphate dehydrogenase deficiency
Direct measurements of enzymes in RBC.
62
Treatments for Bone marrow failure 'Aplastic'
Removal of causative agent.
Cautious blood and platelet transfusion.
If not spontaneous recovery: BMT or immunosuppressive therapy.
63
Treatment for Sickle cell anaemia
Folic acid.
Pain relief.
BMT in severe disease.
64
Treatments for Thalassaemia
Homozygotes: Blood transfusions to try and avoid complications.
Iron chelating agent for iron overload (desferrioxamine).
Ascorbic acid increases iron excretion in urine, helps offset iron overload.
More severe: BMT
65
Treatment for Glucose-6-phosphate dehydrogenase deficiency
Avoid fava beans.
Transfusion if necessary.
66
Complications of Bone marrow failure 'Aplastic'
Increased infection and bleeding.
67
Complications of Sickle cell anaemia
Chronic pain.
68
Complications of Thalassaemia
Iron overload,
endocrine dysfunction.
69
Define Polycythaemia rubra vera
Genetic condition
-> proportion of blood volume occupied by RBC increases
70
Define Deep vein thrombosis
Thrombus formed in a deep vein.
71
How does Polycythaemia rubra vera clinically present?
Can be asymptomatic.
Pruritus, particularly after exposure to warm water.
Headaches, dizziness and sweating.
Thrombotic complications: MI, stroke, DVT.
Rare but classic: Erythromelalgia (a sudden, severe burning pain in the hands or feet, with red/blue coloration of the skin).
72
How does Deep vein thrombosis clinically present?
Often progresses to pulmonary embolism before presenting.
Classical features: Limb pain and tenderness along the lines of the deep veins,
swelling of the calf,
increase in skin temperature,
pitting oedema.
Resembles cellulitis.
73
Polycythaemia rubra vera - note
Polycythaemia is also used to refer to the state of increased volume of blood composed of RBC.
74
Pathophysiology of Polycythaemia rubra vera
Neoplastic proliferation and maturation of erythroid,
| megakaryocytic and granulocytic elements.
75
Pathophysiology of Deep vein thrombosis
Formation of the thrombus in the deep vein.
This can embolise and often flows up into the pulmonary circulation as a pulmonary embolism.
76
Cause of Polycythaemia rubra vera
Somatic mutation in a single haematopoietic stem cell
77
Cause of Deep vein thrombosis
Thrombotic RF: obesity,
FH,
male,
smoking,
age,
cancer.
78
Epidemiology of Polycythaemia rubra vera
2/100,000 each year
79
Epidemiology of Deep vein thrombosis
1/1000 per year
80
Diagnostic test for Polycythaemia rubra vera
Haematocrit: high.
JAK2 testing: can be negative (JAK2 negative prv).
81
Diagnostic test for Deep vein thrombosis
Ultrasound,
D-dimers,
contrast venography.
82
Treatment for Polycythaemia rubra vera
Seek to prevent thrombosis (low dose aspirin).
Intermittent long term phlebotomy.
Possibly myelosuppression.
83
Treatment for Deep vein thrombosis
Low molecular weight heparin,
other anticoagulation therapy possible.
84
Complications of Polycythaemia rubra vera
Can transform into acute myeloid leukaemia.
Thrombotic events.
85
Complications of Deep vein thrombosis
Pulmonary embolism quite likely.
86
Define Over-anticoagulation
Excess use of anticoagulation therapy.
87
Define Disseminated intravascular coagulation
Coagulation systems (thrombin) activated inappropriately.
88
Define Platelet disorders
Disorder affecting the function of the platelets.
89
How does Over-anticoagulation clinically present?
Bleeding.
90
How does Disseminated intravascular coagulation clinically present?
Underlying condition.
Bleeding from unrelated sites (ENT, GI, resp, site of venepuncture).
Confusion,
fever.
Skin: Purpura, petechiae, localised infarction.
91
Types of platelet disorders
Immune thrombocytopenic purpura.
Thrombotic thrombocytopenic purpura.
92
How does Immune thrombocytopenic purpura (platelet disorder) clinically present?
Rapid onset of purpura (usually self limiting in children).
Easy bruising,
epistaxis and
menorrhagia.
Major haemorrhage is rare.
93
How does Thrombotic thrombocytopenic purpura (platelet disorder) clinically present?
Florid purpura,
fever,
fluctuating cerebral dysfunction
and haemolytic anaemia.
Often renal failure.
94
Immune thrombocytopenic purpura (platelet disorder) - note
Immune destruction of platelet cells.
95
Pathophysiology of Over-anticoagulation
Anticoagulation causes the blood to inappropriately avoid clotting
-> Bleeding.
(Warfarin knocks out factors II, VII, IX and X).
96
Pathophysiology of Disseminated intravascular coagulation
Clotting occurs inappropriately and diffusely, until clotting factors are exhausted.
At this point uncontrolled bleeding occurs.
97
Pathophysiology of Immune thrombocytopenic purpura (platelet disorder)
Formation of antibodies against platelets
- > autoimmune platelet destruction
- > thrombocytopenia.
The spleen is the site of autoantibody production and the site of platelet phagocytosis.
Therefore splenectomy has a role as a treatment.
98
Pathophysiology of Thrombotic thrombocytopenic purpura (platelet disorder)
Deficiency of ADAMTS 13 (protease which degrades vWF)
- > Widespread adhesion and aggregation of platelets
- > microvascular thrombosis
- > profound thrombocytopenia.
99
Cause of Over-anticoagulation
Iatrogenic (inappropriate prescription of warfarin / heparin).
100
Cause of Disseminated intravascular coagulation
Secondary to infection,
malignancy,
major trauma.
101
Cause of Immune thrombocytopenic purpura (platelet disorder)
In children, a result of viral infection.
In adults, occurs in combination with another autoimmune condition (SLE etc).
102
Cause of Thrombotic thrombocytopenic purpura (platelet disorder)
Congenital (genetic absence of ADAMTS 13)
or autoantibody mediated (autoantibody against ADAMTS 13).
103
Epidemiology of Immune thrombocytopenic purpura (platelet disorder)
5/100,000 in children,
2/100,000 in adults
104
Diagnostic test for Disseminated intravascular coagulation
Prothrombin time elevated,
activated partial thromboplastin time elevated,
platelets low,
fibrinogen low.
105
Diagnostic test for Immune thrombocytopenic purpura (platelet disorder)
FBC: Isolated thrombocytopenia.
106
Diagnostic test for Thrombotic thrombocytopenic purpura (platelet disorder)
Raised lactate dehydrogenase levels (from ischaemic or necrotic cells).
107
Treatments for Over-anticoagulation
Warfarin: Phytomenadione
(Vitamin K)
Heparin: protamine sulfate.
108
Treatment for Disseminated intravascular coagulation
Consider transfusion of platelets.
109
Treatments for Immune thrombocytopenic purpura (platelet disorder)
Oral corticosteroids (prednisolone).
Second line: splenectomy.
110
Treatments for Thrombotic thrombocytopenic purpura (platelet disorder)
Plasma exchange to remove autoantibodies to ADAMTS 13.
Splenectomy as a last resort.
111
Complications of Over-anticoagulation
Bleeding out
-> Death
112
Complications of Disseminated intravascular coagulation
Organ failure by infarction,
death.
113
Complications of Immune thrombocytopenic purpura (platelet disorder)
Bleeding.
114
Define Acute lymphoblastic leukemia (ALL)
Acute malignant transformation of a clone of lymphoid progenitor cells.
115
Define Chronic lymphocytic leukemia (CLL)
Chronic malignant transformation of a clone of mature lymphoid cells.
116
Define Acute myeloid leukemia (AML)
Acute malignant transformation of a clone of myeloid progenitor cells.
117
Define Chronic myeloid leukemia (CML)
Chronic malignant transformation of a clone of myeloid cells.
118
How does Acute lymphoblastic leukemia (ALL) clinically present?
Fatigue, dizziness and palpitations.
Anaemia, bleeding and infection due to bone marrow failure.
Bone pain.
Hepatosplenomegaly.
119
How does Chronic lymphocytic leukemia (CLL) clinically present?
Early: Asymptomatic (indolent), but isolated lymphocytosis is frequent.
When symptomatic: Anaemia, bleeding and infection due to bone marrow failure.
Rubbery lymph nodes.
120
How does Acute myeloid leukemia (AML) clinically present?
Fatigue, dizziness and palpitations.
Anaemia, bleeding and infection due to bone marrow failure.
Hepatosplenomegaly.
121
How does Chronic myeloid leukemia (CML) clinically present?
Insidious onset, fever, weight loss, sweating, anaemia.
Massive splenomegaly.
Untreated, this lasts 3-4 years, before progressing.
122
Acute lymphoblastic leukemia (ALL) - note
Prospensity to involve the CNS.
123
Chronic lymphocytic leukemia (CLL) - note
Histology: Smudge (smear?) cells.
124
Acute myeloid leukemia (AML) - note
Histology: Auer rods.
125
Pathophysiology of Acute lymphoblastic leukemia (ALL)
Uncontrolled proliferation of precursor B or T cells
-> Accumulation of leukaemic cells in bone marrow, peripheral blood and other tissues.
Additionally a reduction in red cells, platelets and neutrophils.
126
Pathophysiology of Chronic lymphocytic leukemia (CLL)
Uncontrolled proliferation and accumulation of mature B (or T) cells.
Autoimmune haemolysis can occur
-> anaemia.
127
Pathophysiology of Acute myeloid leukemia (AML)
Accumulation of leukaemic cells in bone marrow, peripheral blood and other tissues.
Additionally a reduction in red cells, platelets and neutrophils
128
Pathophysiology of Chronic myeloid leukemia (CML)
Insidious onset lasts 3-4 years
- > Blast formation
- > Acute myeloid leukaemia
- > rapid death.
129
Causes of Acute lymphoblastic leukemia (ALL)
Genetic: Genetic risk.
Down's Syndrome -> 20x risk
Environmental: Chemicals (benzene compounds) drugs (alkylating agents)
Radiation exposure.
130
Cause of Chronic lymphocytic leukemia (CLL)
Genetic: Mutation (11q or 17p deletions for example).
131
Causes of Acute myeloid leukemia (AML)
Can transform from myelodysplasia.
Genetic: Can be due to mutation such as translocations.
132
Cause of Chronic myeloid leukemia (CML)
Genetic: 95% of cases:
Philadelphia chromosome: reciprocal translocation (t(9;22)),
creating fusion gene (BCR-ABL) with tyrosine kinase activity which alters cell growth.
133
Epidemiology of Acute lymphoblastic leukemia (ALL)
Most common cancer in children.
134
Epidemiology of Chronic lymphocytic leukemia (CLL)
Most common leukaemia in the western world.
Usually of older people.
135
Epidemiology of Acute myeloid leukemia (AML)
Most common in older adults and elderly.
136
Epidemiology of Chronic myeloid leukemia (CML)
Most common in elderly.
137
Diagnostic tests for Acute lymphoblastic leukemia (ALL)
Peripheral blood film: anaemia and thrombocytopenia
Bone marrow aspirate: Leukaemic blast cells.
138
Diagnostic tests for Chronic lymphocytic leukemia (CLL)
FBC: Anaemia, raised white cell
Blood film: Smudge cells.
139
Diagnostic tests for Acute myeloid leukemia (AML)
Peripheral blood film: anaemia, thrombocytopenia, auer rods
Bone marrow aspirate: Leukaemic blast cells.
140
Diagnostic tests for Chronic myeloid leukemia (CML)
FBC: Anaemia, raised white cells.
Cytogenetics: Philadelphia chromosome.
141
Treatment for Acute lymphoblastic leukemia (ALL)
Chemotherapy with vincristine, dexamethasone, asparaginase and daunorubicin.
Intrathecal: methotrexate.
Follow up maintenance.
142
Treatment for Chronic lymphocytic leukemia (CLL)
Incurable.
Chlorambucil (with or without prednisolone)
- > Decreases blood count
- > Decreases lymphadenopathy and splenomegaly.
143
Treatment for Acute myeloid leukemia (AML)
Low risk of treatment failure: Chemotherapy, in intervals to allow marrow recovery.
Intermediate: Chemotherapy followed by allogenic bone marrow transplant.
High risk: Only curable with allogenic transplantation.
144
Treatment for Chronic myeloid leukemia (CML)
Imatinib (tyrosine kinase inhibitor).
If in acute stage: Chemotherapy.
145
Complications of Acute lymphoblastic leukemia (ALL)
Children: excellent prognosis (1/5 die).
Adults: Poorer prognosis, only 30% cured.
146
Complications of Chronic lymphocytic leukemia (CLL)
Death
147
Complications of Acute myeloid leukemia (AML)
Death
148
Complications of Chronic myeloid leukemia (CML)
Death
149
Sequelae of Acute lymphoblastic leukemia (ALL)
Recurrence (if in remission).
Secondary cancer.
150
Sequelae of Chronic myeloid leukemia (CML)
Acute myeloid leukemia.
Myelofibrosis.
151
Define lymphoma
Malignant tumour of the lymphatic system
152
Types of lymphoma
Hodgkin's
Non-Hodgkin's
153
How does Hodgkin's lymphoma clinically present?
Painless rubbery lymph node enlargement,
hepatosplenomegaly,
Systemic 'B symptoms': (fever, drenching night sweats, weight loss).
Pruritus, fatigue, anorexia,
and alcohol-induced pain in affected nodes.
Extranodal involvement is possible.
154
How does Non-Hodgkin's lymphoma clinically present?
As Hodgkin's.
Can be indolent or aggressive (depending on origin cell).
Extranodal involvement more common than in Hodgkin's.
155
Pathophysiology of Hodgkin's lymphoma
Malignant transformation of normal T or B cell in lymph nodes.
Reed-Sternberg cells seen on histology.
Staging: 1: Single lymph node or site
2: Two or more nodes/sites, same side of the diaphragm
3: Lymph nodes either side of the diaphragm
4: Diffuse or disseminated of one or more extralymphatic tissue
156
Pathophysiology of Non-Hodgkin's lymphoma
70% B cell, 30% T cell by origin.
Clonal expansion of lymphocytes.
Mature lymphocyte transformation: Indolent course.
Proliferating cell: Aggressive.
157
Cause of Hodgkin's lymphoma
Previous Epstein-Barr virus in some cases.
158
Cause of Non-Hodgkin's lymphoma
Usually unknown. H. pylori infection in MALT lymphoma.
EBV infection in Burkitt's lymphoma.
159
Epidemiology of Hodgkin's lymphoma
More common in young adults.
160
Epidemiology of Non-Hodgkin's lymphoma
Rare before 40.
Specific varieties more common in children.
161
Diagnostic tests for Hodgkin's lymphoma
Lymph node biopsy: showing Reed-Sternberg cells.
CT: Staging.
ESR: raised.
162
Diagnostic tests for Non-Hodgkin's lymphoma
FBC: Possible anaemia
Lymph node biopsy: Needed for diagnosis.
CT: staging.
163
Treatment for Hodgkin's lymphoma
Early stage disease: Brief chemo (ABVD) and irradiation
Advanced: Cyclical chemo (ABVD) and irradiation.
164
Treatment for Non-Hodgkin's lymphoma
Diffuse large b cell lymphoma: Cyclical combination chemo and field irradiation.
Primary gastric lymphoma: H. pylori eradication
Burkitt's: Cyclical combo chemo.
165
Complications of Hodgkin's lymphoma
Death.
Worse prognosis with B symptoms.
166
Complications of Non-Hodgkin's lymphoma
Death.
167
Define Multiple Myeloma
Malignant disease of bone marrow plasma cells.
168
Define Malaria
Infection by Plasmodium transmitted by mosquito.
169
How does multiple myeloma clinically present?
Bone destruction: Back pain, pathological fractures
Bone marrow infiltration: Anaemia, infections and bleeding
Paraprotein aggregates: Blurred vision, gangrene and bleeding.
170
How does malaria clinically present?
Most with falciparum present in the first month, others can incubate for months.
No specific symptoms; take effective history.
Fever, chills, rigors, cough, myalgia, splenomegaly, hepatomegaly
Severe: Impaired consciousness, shortness of breath, bleeding, fits, hypovolaemia.
171
Pathophysiology of multiple myeloma
Clonal proliferation of bone marrow cells, usually capable of monoclonal antibodies (usually IgA or IgG).
Can be associated with the excretion of light chains in the urine (Bence Jones protein).
Bone destruction: increased osteoclastic activity
-> bone pain and osteolytic lesions
Infiltration of bone marrow -> reduced function
AKI: due to light chain and amyloid deposition, hypercalcaemia and hyperuricaemia.
Paraproteins: Can aggregate in the blood to greatly increase viscosity
172
Pathophysiology of malaria
Bite by infected mosquito
- > sporozoites in the saliva travel to the liver to mature
- > rupture to release merozoites into the blood
- > invade RBC and undergo asexual reproduction to create sporozoites a mosquito can pick up (hence cycle)
173
Cause of multiple myeloma
Mutation.
About half of cases; translocation placing oncogene into immunoglobulin heavy chain gene on chromosome 14.
174
Cause of malaria
Infection by Plasmodium (usually falciparum).
175
Epidemiology of multiple myeloma
Peak presentation at 60 years.
176
Epidemiology of malaria
Poor, young, pregnant, elderly all more at risk.
Consider if recently travelled abroad.
177
Diagnostic tests for multiple myeloma
Paraproteinaemia,
Bence Jones protein in urine,
CT,
Bone marrow aspirate.
178
Diagnostic test for malaria
Blood smear with giemsa stain.
179
Treatments for multiple myeloma
Chemotherapy with autologous stem cell transplant.
Paraprotein: plasmapheresis.
Blood transfusion, for anaemia.
Treatment of infection.
AKI can be treated by hydration.
180
Treatments for malaria
Non-falciparum: Chloroquine
Falciparum: Quinine sulfate, atovaquone-proguanil and artemether with lumefantrine.
Severe Falciparum: IV quinine dihydrochloride.
181
Complications of multiple myeloma
Terminal.
With treatment, median survival is 5 years.
182
Complications of malaria
Multiple organ failure and death.
