Haematology Flashcards
What is the process of blood formation called?
Haemopoiesis
The haemopoietic system includes what five components?
Bone marrow, liver, spleen, lymph nodes and thymus
What is the average lifespan of red cells?
120 days
What is the average lifespan of platelets?
7 days
What is the average lifespan of granulocytes?
7 hours
What are reticulocytes?
Young red cells
Reticulocytes represents what percentage of total circulating red blood cells?
0.5-2.5%
What makes up a haem group?
A single molecule of protoporphyrin IX bound to a single ferrous ion (Fe2+)
What symptoms may arise from anaemia?
Fatigue, faintness and breathlessness
In patients with concurrent atheromatous disease, what more serious symptoms can anaemia precipitate?
Angina pectoris and intermittent claudication
What signs might be observable in the anaemic patient? (4)
Skin and mucous membranes may be pale
Tachycardia
Systolic flow murmur
Cardiac failure (in elderly people/ those with compromused cardaicfunction)
What does microcytosis usually represent about the structure of a red blood cell?
Decreased haemoglobin content
What is the most common cause of anaemia worldwide?
Iron deficiency anaemia
What are the causes of iron deficiency anaemia? (4)
Blood loss
Increased demands (such as growth and pregnancy)
Decreased absorption
Poor dietary intake
What signs does iron deficiency produce? (3)
Brittle hair and nails
Atrophic glossitis
Angular stomatitis
Ansiocytosis and poikilocytosis are both features seen on a blood film of an iron-deficient patient; what do these two terms mean?
Ansiocytosis - variation in size
Poikilocytosis - variation in shape
What investigations are indicated in a suspected case of iron deficiency anaemia?
Blood count and blood film
Serum ferritin (reflecting iron stores) are low
Serum iron is low
How is iron deficiency managed?
Find and treat underlying cause
Oral iron supplementation e.g. ferrous sulphate/gluconate
What is sideroblastic anaemia?
A rare disorder of haem synthesis characterised by refractory anaemia in the peripheral circulation and ring sideroblasts in the bone marrow
What are ring sideroblasts?
Erythroblasts with iron deposits in the mitochondria and reflect impaired utilisation of iron within the red cell
What causes sideroblastic anaemia?
It may be inherited or acquired (secondary to myelodysplasia, alcohol excess, lead toxicity)
What is anaemia of chronic disease?
Anaemia occuring in patients with either:
- Chronic inflammatory diseases (such as Crohn’s disease or rheumatoid arthrtitis)
- Chronic infections (e.g. tuberculosis)
- Malignancy
- Chronic kidney disease
As seen on a blood film; what type of anaemia is anaemia of chronic disease?
Normochromic, normocytic anaemia
How is anaemia of chronic disease managed?
Treatment of the underlying chronic disease and sometimes erythropoietin
What is the most important investigation of a macrocytic anemia?
Measurement of serum B12 and red cell folate
What is megaloblastic anaemia?
A macrocytic anaemia charactersed by the presence of megaloblasts in the bone marrow (immature red cells with delayed nucelar maturation relative to that of the cytoplasm)
What is the most common cause of megaloblastic anaemia?
Vitamin B12/folate deficiency
What is pernicious anaemia?
An autoimmune condition in which there is atrophic gastritis with loss of function of the parietal cells and hence a failure to produce intrinsic factor (essential for uptake of vitamin B12)
What are the clinical features of pernicious anaemia?
Insidious onset Glossitits (sore, red tongue) Angular stomatitis Mild jaundice Neurological features (due to B12 deficiency)
What neurological features may be present in B12 deficiency?
Symmetrical damage of the spinal cord leading to progressive weakness, ataxia, and eventual paraplegia
Dementia and visual distrubances may also occur
What is the main difference in the symptom profile between B12 deficiency and folate deficiency?
In folate deficiency there are no neurological features
What might cause folate deficiency? (4) (Non exhsautive list)
Pregnancy, coeliac disease, acohol excess, trimethoprim
What is aplastic anaemia?
Defined as a pancytopenia (deficiency of all blood cells) due to bone marrow failure
What are the clinical features of aplastic anaemia? Why do they occur?
Deficiency of red cells, white cells and platelets
Causing anaemia, susceptibility to infection and bleeding
Physcial findings include bruising, bleeding gums, epistaxis and mouth infections
How is aplastic anaemia investigated and confirmed?
Trephine bone marrow biopsy for assessment of bone marrow cellularity
What causes aplastic anaemia?
Most commonly encountered due to chemotoxic drugs and radiation
What is the management strategy for aplastic anaemia?
Withdrawal of the offending agent (chemotoxic drug for example) followed by definitive therapy such as blood and platelet transfusions
What medical emergancy may occur in apalstic anaemia?
Neutropenic sepsis
How might neutropenic sepsis present?
Neutropenic patient (neutrophil count <1 x10^9/L) who is pyrexial, or has new-onset confusion, tachycardia, hypotension, dyspnoea or hypothermia
List some blood investigations which are necessary in the case of neutropenic sepsis
FBC Differential white cell count CRP U/Es Liver biochemistry Clotting screen Blood cultures
What radiological investigations might you consider in the neutropenic patient?
- Chest X-ray
- Consider further imaging if there are localising signs e.g. CT abdomen/pelvis
What empirical antibiotics are indicated in the case of suspected neutropenic sepsis?
Piperacillin and aminoglycoside (to cover Gram-negatives and Pseudomonas)
Consider adding vancomycin if clincal deterioration, fever persists or suspected MRSA infection
What is haemolytic anaemia?
Anaemia resulting from increased destruction of red cells with a reduction in lifespan and therefore a compensatory rise in premature reticulocytes
Haemolysis may be either intravascular or extravascualar - which is more common?
Extravascular (within the reticuloendothelial system, mainly the spleen)
What investigations may indicate a intravascular haemolysis?
Raised level of plasma haemoglobin
Positive Schumm’s test (methaemalbumin in plasma)
Low/absent haptoglobins (complex formed from free haemoglobin - removed rapidly by the liver)
Haemosiderinuria - haemosiderin in the urine
Inherited haemolytic anaemias are due to defects in one of three components - what are they?
Defects in either:
- Cell membrane
- Haemoglobin structure
- Metabolic procresses of the red cell
Hereditary spherocytosis is an example of an inherited haemolytic anaemia caused by a defect in which red cell component?
Cell membrane
What inheritance does hereditary spherocytosis show?
Autosomal dominant
What is the pathophysiology of hereditary spherocytosis?
Deficiency in spectrin (most commonly) leading to increase permeability to sodium ions, causing rigid and spherical red cells which are prematurely removed by the spleen (extravascular)
What symptoms may arise in hereditary spherocytosis?
Ranges from asymptomatic to severe haemolysis (anaemia, jaundice, splenomegaly)
What types of crises may occur in any haemolytic anaemia?
Megaloplastic
Aplastic
Haemolytic
What does aplastic crisis usually occur as a result of in the course of normal haemolytic disease?
After infection (particularly erythro(paro)virus)
What does megaloblastic crisis usually occur as a result of in the course of normal haemolytic disease?
Folate deficiency caused by hyperactivity of the bone marrow
What biliary pathology may occur as a result of chronic haemolytic disease?
Pigment gallstones
What investigations may confirm hereditary spherocytosis?
Blood count demonstrates reticulocytosis and anaemia
Blood film shows spherocytes
Evidence of haemolysis (raised serum bilirubin and urinary urobilinogen)
How is hereditary spherocytosis managed?
Splenectomy indicated in adulthood to relieve symtptoms
not in childhood due to risk of fulminant infections
What are thalassaemias?
A group of genetic disorders causing reduced rate of production of one or more globin chains used in the synthesis of haemoglobin (ineffective erythropoiesis)
There are three main forms of beta-thalassaemia; what are they?
- beta-thalassaemia minor (trait) - carrier state due to heterozygous genetic profile
- beta-thalassaemia intermedia - moderate penetrance causing mild anaemia and other thalassaemic features
- beta-thalassaemia major - presents in first year of life with significant thalassaemic features, severe anaemia and hepatosplenomegaly
What are the talassaemic features? Why do they occur?
Hypertrophy of inept bone marrow causes:
- Bony abnormalities (enlarged maxilla, prominent frontal and parietal bones)
- Recurrent leg ulcers
- Gallstones
How is thalassaemia diagnosed?
Haemoglobin electrophoresis
How is thalassaemia treated?
Regular blood transfusions (keeping Hb >100g/L) - suppressing ineffective erythropoiesis and bony abnormalities
Aduvant chelation therapy required to prevent iron overload and folate supplementation.
Describe the clincal spectrum of alpha-thalassaemia
Ranging from mild anaemia with microcytosis to severe condition incompatible with life resulting in stillbirht (hydrops fetalis)
What are sickle syndromes?
A family of haemoglobin disorders in which the sickle beta-globin gene is inherited.
Why does sickle cell anaemia not present and features until around 6 months of age?
Production of foetal haemoglobin (HbF) is unaffected and so sickle disease doesnt manifest until HbF decreases to adult levels (at 6 months of age)
Sickle cell anaemia has marked phenotypic variation. Outline some of the clinical features which may present.
Vaso-occlusion - acute dactylitis in children is more likely. In adults, long bone, rib, spinal and pelvic pain more likely.
Other vaso-occulsive complications: avascular necrosis of bones, retinal iscahemia, cerebral infarction, priapism
Anaemia - usually exists in a steady state Hb 60-80g/L with a high reticulocyte count (10-20%)
Other complications: Chronic kidney disease, leg ulcers, osteomyelitis, pulmonary hypertension, acute chest syndrome
What events may precipitate a rapid destabilisation of haemoglobin levels in a patient with sickle cell disease? (2)
Splenic sequestration - spleen becomes engorged with red cells leading to rapid enlargement of spleen (can also occur in the liver)
Aplatic anaemia - commonly due to eythrovirus B19 infection
What is acute chest syndrome?
Medical emergancy characterised by fever, cough, dyspnoea and pulmonary infiltrates on chest X-ray
How are painful crises managed?
Morphine 0.1mg/kg IV or SC every 20 mins with aduvants
Also prescribe: laxatives (lactulose 10ml BD), anti-pruritics, antiemetics, anxiolytics, antibiotics
Oxygen, rehydration
Check for acute chest or liver/splenic sequestration
In patients with recurrent painful crises, what drug is indicated to help prevent them?
Hydroxycarbamide (raises concentration of foetal haemoglobin)
What is the most common red cell enzyme deficiency causing haemolytic anaemia?
Glucose-6-phosphate dehydrogenase deficiency (G6PD)
What is the inheritance pattern of G6PD?
X-linked
What are the clinical features of G6PD?
Neonatal jaundice, chronic haemolytic anaemia, acute haemolysis (precipitated by fava beans, nitrofurantoin and other drugs)
How are autoimmune haemolyses categorised?
Depending on whether the antibody reacts best at body temperature (warm antibodies) or at lower temperatures (cold)
What is mechanical haemolytic anaemia?
Red cells are injured by physical trauma in the circulation. Can be due to a number of things:
- Leaking prosthetic valves
- March haemoglobinuria ( damage to red cells from prolonged marching)
- Microangiopathic haemolysis (e.g. in DIC)
What are myeloproliferative disorders?
Disorders of uncontrolled clonal proliferation of one or more cell lines in the bone marrow.
Name the myeloproliferative disorders?
Polycythaemia vera
Essential thrombocytopaenia
Myelofibrosis
Chronic myeloid leukaemia
What are the symptoms of polycythaemia vera?
All as a result of both hypervolaemia and hyperviscocity
Headache, vertigo, tinnitus, visual disturbance, angina pectoris, intermittent claudication, pruritis, venous thrombosis
What signs may be found in polycythaema vera?
Plethoric complexion, hepatosplenomegaly (distinguishes primary from secondary polycythaemia)
What other conditions are people with polycythaemia vera at risk of?
Gout - due to increased uric acid production
Haemorrhage - due to friable haemostatic plugs
How is polycythaemia vera treated?
Venesection (maintain PCV <0.45L/L) - may be sufficient
Chemotherapy:
1. Hydroxycarbamide to reduce platelets
2. Low dose aspirin - reduce recurrence of thromboses
3. Anagrelide - useful for thrombolysis
4. Radioactive phosphorus - to combat risk of leukaemic conversion
5. Allopurinol - decrease uric acid levels
What is essential thombocythaemia?
Conditon characterised by normal haemoglobin levels and white cell counts but elevated platelets
How may essential thombocythaemia present?
Either with bleeding or thromboses
How is essential thrombocythaemia diagnosed?
An otherwise well with a platelet count of over 1000 x10^9/L is generally considered to be sufficient for diagnosis
How is essential thrombocythaemia treated?
Hydroxycarabamide and/or anagrelide if required
List some differnetial diagnoses for essential thrombocythaemia
Reactive thrombocytosis Autoimmune rheumatic disorders Malignancy Polycythaemia vera Myelodysplasia
What is myelofibrosis?
A condition characterised by haemopoietic stem cell proliferation associated with marrow fibrosis
How does myelofibrosis present?
Insidious onset of weakness, weight loss and lethargy
Bleeding may occur in thrombocytopaenic patient
Hepatomegaly and massive splenomegaly
What are the four most common causes of death in myelofibrosis?
Transformation into acute myeloid leukaeia
Progression of myelofibrosis
Cardiovascular disease
Infection
What investigations might support your diagnosis of myelofibrosis?
- Blood count (showing anaemia)
- Trephine bone marrow biospy (increased fibrosis)
- Absent philidelphia chromosome (distiinguishes myelofibrosis from chronic myeloid leukaemia)
- JAK2 mutation (present in 50% of cases)
How is myelofibrosis managed?
Transfusions (correct anaemia)
Allopurinol (prevent gout)
JAK2 inhibitors (e.g. rutixumab)
Allogenic stem cell transplant in young patients (offers only hope to cure)
What is myelodysplasia?
A group of acquired marrow disorders caused by defects in stem cells; resulting in progressive bone marrow failure and potential evolution into acute myeloid leukaemia
How is myelodysplasia diagnosed?
Paradoxical appearance of peripheral pancytopenia on blood film and hypercellular bone marrow (due to prematuer loss of cells by apoptosis)
What blood constituents are present in fresh frozen plasma?
All coagulation factors
What blood constituents are present in cryoprecipitate?
3
Factor VIII
Von Willebrand factor
Fibrinogen
What complications may occur after a transfusion (excluding massive transfusion)?
(5)
- ABO mismatch (most severe)
- Febrile reactions (due to activation of anti-leucocyte antibodies in the recipient)
- Anaphylactic reactions (in IgA deficient patients)
- Infection
- Heart failure (particularly in elderly)
What complications may occur during/after massive transfusion?
Hypocalcaemia
Hyperkalaemia
Hypothermia
What are the five types of white cells found in the peripheral blood?
Neutrophils, eosinophils, basophils (all called granulocytes) and lymphocytes and monocytes
What are monocytes?
Precursors to tissue macrophages
What pathological processes do eosinophils play a part in?
Allergic reactions Helminth infections and protozoa Asthma Malignancy Hyper-eosinophilic syndrome
What is hyper-eosinophilic syndrome?
Restrictive cardiomyopathy, hepatosplenomegaly and very high eosinophils (>0.4 x10^9/L)
What is immune thrombocytopenic purpura (ITP)?
Immune destruction of platelets
Describe the different occurrences of immune thrombocytopenic purpura in adults and children
In children - usually follows viral infection rapidly and is self-limiting
In adults - less acutely and often seen concurrently with other immune conditions e.g. SLE
How is immune thrombocytopenic purpura diagnosed?
Diagnosis of exclusion
Platelet antibodies not essenstial for diagnosis but may be seen in 60-70% of patients
How is immune thrombocytopenic purpura managed?
First-line therapy - oral corticosteroids or IV IgG where rapid rise in platelets is optimal (pre-surgery)
Second-line therapy - splenectomy and thrombopoietin receptor agonists (e.g. romiplostim)
What is thrombotic thrombocytopenic purpura?
Widespead adhesion and agglutination of platelets leading to microvascular thrombosis and profound thombocytopenia (<150 x10^9/L)
Deficiency of what protease causes thrombotic thrombocytopenic purpura
ADAMTS-13
What can cause thrombotic thrombocytopenic purpura?
Congenital or sporadic or autoantibody-associated (pregnancy, infection etc.)
What are the symptoms of thrombotic thrombocytopenic purpura?
Florid purpura, renal failure, fluctuant cerebral dysfunction and haemolytic anaemia
How is thrombotic thrombocytopenic purpura treated? What is counter-intuitively contraindicated?
Plasmapheresis
High dose steroids
Platelet concentrates are contraindicated
What is Haemophilia A caused by? What is the inheritance pattern?
X-linked recessive disorder resulting in a deficiency of factor VIII
Haemophiliac is categorised into mild, moderate and severe entities. Describe each.
- Mild (>5U/dL) - bleeding only with trauma
- Moderate (1-5U/dL) - severe bleeding following injury and occaisional spontaneous bleeding
- Severe (<1U/dL) - severe spontaneous bleeding into muscles/joints causing crippling arthropathy
What investigations would help confirm the diagnosis of Haemophilia A?
Prolonged APTT
Normal PT and vWF
How is haemophilia A treated?
- IV infusion of recombinant factor VIII (with supplies at home too)
- Synthetic vasopressin (used to increase production in patients with mild haemophilia)
- Advice about lifestyle e.g. avoiding contact sports
What is the difference between haemophilia A and B? What treatment options differ in haemophilia B?
Haemophilia B is a deficiency of factor IX
Vasopressin is ineffective and therefore not used in treatment of haemophilia B
What is von Willebrand’s disease?
hereditary deficiency of von Willebrand Factor (vWF)
What is the function of von Willebrand factor?
Contributes to platelet adhesion to damaged subendothelium and stabilisaton of factor VIII
Outline the three different types of von Willebrand’s disease
Type 1 (quantitative) - decreased vWF Type 2 (qualitative) - poor functioning vWF Type 3 (absolute deficiency) - absence of vWF
Outline the inheritance pattern of the types of von Willebrand’s disease
Type 1 and 2 are autosomal dominant
Type 3 is autosomal recessive
What investigations might help support a diagnosis of von Willebrand’s disease?
- Prolonged bleeding time (indicating poor platelet adhesion)
- Prolonged APTT
- Normal PT
Vitamin K is reuqired for the formation of which coagulation factors?
Factors II, VII, IX and X
What may cause vitamin K deficiency?
Malnutrition, malabsorption or warfarin use
How is vitamin K deficiency treated?
IM Vitamin K supplementation
What is disseminated intravascaulr coagulation (DIC)?
Widespread generation of fibrin within blood vessels. There is massive consumption of platelets and coagulation factors
What are the most comon causes of disseminated intravascaulr coagulation (DIC)? (4)
- Sepsis
- Major trauma (surgery, burn etc.)
- Advanced cancer
- Obstetric complications (amniotic embolism, abruptio placentae)
What is the clinical presentation of disseminated intravascaulr coagulation (DIC)?
Varies from no bleeding at all to complete haemostatic failure
Thrombotic events may occur too
What investigations are indicated in the susepcted case of disseminated intravascaulr coagulation (DIC)?
Blood count - showing severe thrombocytopenia
Prolonged APTT, PT and bleeding time
Blood film - fragmented red cells
How is disseminated intravascaulr coagulation (DIC) treated?
Treat the underlyig cause Platelet concentrates (maintain platelets above 50 x10^9/L
Cryoprecipitate and red cells are also indicated in bleeding patients
Aterial thrombi are formed of what blood constituent and arise due to what underlying chronic pathology?
Platelets adhere to atheroma (atherosclerosis)
Where do arterial thrombi occur?
Areas of turbulent blood flow e.g. bifurcating arteries
What are venous thrombi formed of?
Fibrin and red cells
Where do venous thrombi occur?
Originate around the valves of veins; partiularly in the deep veins of the leg
What sort of drugs target venous thombi?
Heparin and warfairn (anti-coagulants)
What sort of drugs target arterial thrombi?
Aspirin and clopidegrel (anti-platelets)
Fondapariunux is an anti-coagulant; what coagulation factor does it inhibit?
Factor X (similar in action to herapin)
What is dabigatran and what is it prescribed for?
Direct thrombi inhibitor; given as prophylaxis for venous thromboembolism following hip or knee replacement surgery
What is the target INR of a paitent to prevent thromboembolism?
2.5 (3.5 if recurrent venous thromboembolism despite monotherapy with warfarin)
What are the two most common side effects of iron supplementation?
Constipation and diarrhoea
What are common side effects of B12 supplementation? What is the potential severe side effect on resolution of severe B12 deficiency anaemia?
Itching, fever, nausea and diziness
Potential for severe hypokalaemia due to intracellular shift on anaemia resolution
What does NOAC stand for?
Novel Oral Anti-Coagulants
What is alpha-fetoprotein?
Serum tumour biomarker for hepatocellular carcinoma and non-seminomatous germ cell tumour of the testes
What is beta-human chorionic gonadotropin (beta-HCG) ?
A serum tumour biomarker for choriocarcinoma, germ cell tumours of the testes and lung cancer
What is the tumour biomarker for prostate cancer?
Prostate specific antigen (PSA)
Chorioembryonic antigen (CEA) may be raised in what cancers?
Colorectal cancer
Some gastrointestinal malignancies
What is the tumour biomarker for ovarian cancer?
CA-125
What is CA19-9?
Serum tumour biomarker for upper gastrointestinal malignancies
What is CA15-3?
Serum tumour biomarker for breast cancer
What is osteopontin?
Serum tumour biomarker for mesothelioma
Outline the four sources of haemopoietic stem cell transplant
Allogenic - bone marrow from another individual
Autologous - bone marrow harvested from self
Syngenic - bone marrow from identical twin
Umbilical cord
What is superior vena cava syndrome?
Arising from any upper mediastinal mass (most commonly lung cancer or lymphoma) - characterised by compression of the superior vena cava
What is the presentation of superior vena cava syndrome?
Difficulty breathing or swallowing
Odematous face
Venous congestion in the neck (dilated veins)
How is superior vena cava syndrome treated?
Immediate steroids and vascular stenting
Potentially radiotherapy/chemotherapy to shrink mass
What is acute tumour lysis syndrome?
A condition characterised by rapid breakdown of tumour following commencement of treatment.
What biochemical abnormalities arise in acute tumour lysis syndrome?
Hyperphosphataemia (and secondary hypercalcaemia)
Hyperkalaemia
Hyperuraemia
What is the main complication of acute tumour lysis syndrome?
Acute kidney injury due to urate and calcium phosphate deposition in renal tubules
Acute Lymphoblastic Leukaemia has a propensity to involve the CNS. For this reason, what extra treatment measure is taken?
Intrathecal chemotherapy (prophylaxis)
Sometimes cranial irratiation is used in high risk patients
What are the clinical features of acute leuakemia?
Bone marrow failure (anaemia, thormbocytopenia and prepensity to infection)
Sometimes there is lymphadenopathy and heaptosplenomegaly
What genetic funding is characteristic of chronic myeloid leukaemia?
Philidelphia chromosome mutation
What is the presentation of chornic myeloid leukaemia?
Insidious onset of fever, weight loss, sweating and symptoms of anaemia
Massive splenomegaly is characeristic
Outline the phases of chronic myeloid leukaemia
Chronic-phase - lasts 3-4 years
Blastic transformtion - usually acute myeloid leukaemia (causing rapid death)
What is the first-line treatment for chronic-phase chronic myeloid leukaemia?
Imatinib (tyrosine kinase inhibitors)
Not effective after blastic transformation
What is chronic lymphocytic leukaemia?
Incurable disease of the elderly characterised by uncontrolled proliferation of mature B lymphocytes
Outline the course of chronic lymphocytic leukaemia
Indolent course and generally asymptomatic
Later on, symptoms of bone marrow failure and hepatosplenomegaly
What investigations would support a diagnosis of chronic lymphocytic leukaemia?
Blood count - lymphocytosis, anaemia, thrombocytopenia
Blood film - smudge cells (artefact of small mature lymphocytes)
Immunophenotyping - essential to exclude reactive lymphocytosis
Cytogenetics - identify specific mutation
What is the standard first-line therapy for chronic lymphocytic leukaemia?
Combination of fludarabine, cyclophosphamide, rutixumab
What is lymphoma?
B and T cell malignancies of the lymphoid system
How are lymphomas classified?
Classified as either Hodgkin (specific entity) or Non-Hodgkin (over 50 subtypes)
What virus is most strongly associated with Hodgkin lymphoma?
Epstein-Barr Virus
What demographic is Hodgkin lymphoma most likely to present in?
It is typically a disease of young adults
What are the clinical features of Hodgkin lymphoma?
Lymphadenopathy (often cervical) with rubbery consistency
Potentially hepatosplenomegaly
Systemic “B” symptoms
Constitutional symptoms
What are systemic “B” symptoms often seen in Hodgkin lymphoma?
Fever
Drenching night sweats
Weight loss (>10% of body weight in 6 months)
What constitutional symptoms may be featured in Hodgkin lymphoma?
Pruritus
Fatigue
Anorexia
Alcohol-induced pain at site of lymphadenopathy
What investigations would help you confirm the diagnosis of Hodgkin lymphoma?
Blood count - anaemia (normochromic and normocytic)
ESR - raised (used as indicator of disease activity)
Serum lactate dehydrogenase (poor prognostic marker)
Chest X-ray - mediatinal widening
Diagnosis by lymph node biospy (seeing Reed-Steinberg cells)
Staging by CT and PET scans
How is early-stage Hodgkin lymphoma treated?
Breif chemotherapy followed by field irradiation
How is late-stage Hodgkin lymphoma treated?
Cyclical combination of chemotherapy and field irradiation
Non-hodgkin lymphoma (NHL) is comprised of over 50 subtypes. The majority of NHLs are formed from which cell lineage?
80% are of B cell origin
Some Non-Hodgkin Lymphomas are associated with specific infections; what lymphoma is associated with helicobacter pylori?
Gastric mucosal assocaited lymphoid tissue (MALT) lymphoma
What is the classical presenation of Non-Hodgkin Lymphoma?
Presentation of a painless peripheral lymph node enlargement
Systemic featues and/or bone marrow infilatration may also occur
What investigations are indicated in the case of Non-Hodgkin Lymphoma?
Blood count - anaemia, leucocytosis, thrombocytopenia
Abnormal LFTs
Serum lactate dehydrogenase (prognostic factor)
Bone marrow biospy (confirms marrow involvement)
Lymph node biopspy for definitive diagnosis
What is multiple myeloma?
Malignant proliferation of plasma cells in the bone marrow producing paraproteins (immunoglobulins); most commonly IgA or IgG
What are the clinical features of multiple myeloma?
Bone destruction - increased osteoclastic activity causing lytic lesions and pathological fractures
Bone marrow infiltration - causing anaemia, infections and bleeding
Acute kidney injury - due to light chain deposits in tubules, hypercalcaemia and hyperuraemia.
Paraprotein aggregates in blood - hyperviscocity causing blurred vision and gangrene
What investigations allow the diagnosis of multiple myeloma?
Two of three of the following must be present:
- Paraproteinaemia/ urinary Bence-Jones protein
- Radiological evidence of bony features
- Plasma cell proliferation on bone marrow aspirate
How is multiple myeloma managed?
Good supportive care - including transfusion/erythropoietin, prompt infection treatment, treatment of acute kidney injury, analgesia, plasmapheresis for hyperviscocity
Chemotherapy and autologous stem cell transplant
A patient preents with paraproteinaemia but has no other signs of myeloma. What is this called?
Monoclonal Gammopathy of Unknown Significance
