haematology to work on COPY Flashcards
Give 3 signs of anaemia
- Conjunctival pallor
- Hyper-dynamic circulation
- Tachycardia
- Systolic flow murmur
What are the specific signs of B12 deficiency/pernicious anaemia?
Inflamed tongue (glossitis), angular stomatitis, mild jaundice
Neurological - paraesthesia, psychological problems, ataxia
- dementia and visual disturbances
Give 3 acquired causes of haemolytic anaemia
- Autoimmune
- Drug induced
- Secondary to systemic disease
- Malaria
Give 3 chronic complications of sickle cell disease
- Renal impairment
- Pulmonary hypertension
- Joint damage
- growth and development problems
- cardiomegaly, MI
- hepatomegaly
- retinopathy
- impaired placental blood flow
Describe the treatment for sickle cell disease
- Precipitating factors should be avoided - e.g. infection, dehydration, cold
- Folic acid
- Acute attacks:
- IV fluids
- Analgesia
- Oxygen - Possible blood transfusions
- Oral hydroxycarbamide to reduce frequency of crises
- Stem cell transplant
long term management
- hydroxyurea - increases HbF levels so is used as prophylaxis
- pneumococcal vaccine
what are the clinical features of G6PD deficiency?
Most are asymptomatic Pallor Fatigue Palpitations Shortness of breath Jaundice Exacerbated by ingesting fava beans
Name 3 things that can precipitate G6PD deficiency
- Broad beans / fava beans
- Infection
- Drugs - Primaquine, sulphonamide, quinolones
what are the secondary causes of absolute polycythaemia?
hypoxia
- Smoking - Lung disease - Cyanotic heart disease - High altitude - obstructive sleep apnoea
Excess EPO - renal disease
alcohol
what are the clinical features of polycythaemia?
Headaches and dizziness Itching Fatigue Tinnitus Erythromelalgia – burning sensation in fingers and toes Hypertension Angina Hepatosplenomegaly (distinguishes 1o from 2o)
What is the treatment for polycythaemia?
No cure Treatment aim is to maintain normal blood count Venesection - relieves symptoms Low does aspirin Radioactive phosphorus in those over 70
What can cause decreased platelet production?
- Congenital causes (e.g. malfunctioning megakaryocytes)
- Infiltration of bone marrow (e.g. leukaemia)
- Alcohol
- Infection (e.g. HIV/TB)
- Reduced TPO
- Aplastic anaemia
What can cause increased platelet destruction?
- Autoimmune (e.g. ITP)
- Hypersplenism
- Drug related (e.g. heparin induced)
- DIC and TTP –> increased consumption
What can cause reduced platelet function?
- Congenital abnormality
- Medications - aspirin
- VWF disease
- Uraemia
Give 3 things that can cause coagulation disorders
- Vitamin K deficiency
- Liver disease
- Congenital - haemophilia
What is thrombotic thrombocytopenia (TTP)?
- Widespread adhesion and aggregation of platelets leads to microvascular thrombosis and consumption of platelets
- Occurs due to a reduction in ADAMTS-13 – a protease that is normally responsible for the degradation of vWF
What is the treatment for thrombotic thrombocytopenia (TTP)?
- Plasma exchange (removed ADAMTS13 autoantibodies)
- Splenectomy
- IV steroids
- IV methylprednisolone
- IV rituximab
- folic acid
- platelet transfusion = contraindicated
Give 3 causes of immune thrombocytopenia (ITP)
Primary = viral infection (children) Secondary (chronic, adults) - Autoimmune conditions - Malignancies - CLL - Infections - HIV/Hep C
What is the management for immune thrombocytopenia (ITP)?
- first line = Corticosteroid (prednisolone) or IV immunoglobulin - IV IgG
- second line = Splenectomy or immunosuppression (rituximab)
Give 3 signs of haemolytic anaemia
- Pallor
- Jaundice
- Splenomegaly
Give 2 specific symptoms of iron deficiency anaemia
- Koilonychia - spoon nails
- Brittle hair and nails
- Atrophic glossitis
Give 3 causes of neutrophilia
- Infection
- Inflammation
- Cancer
- CML = primary cause
Give 3 causes of lymphocytosis
- Viral infection
- Inflammation
- Cancer
- CLL = primary cause
Give 3 causes of thrombocytosis
- Infection
- Inflammation
- Cancer
- Essential thrombocythaemia = primary cause
Give 3 causes of neutropenia
- Underproduction = marrow failure, marrow infiltration, marrow toxicity (e.g. drugs)
- Increased removal = autoimmune, Felty’s syndrome, cyclical
Give 2 causes of thrombocytopenia
- Production failure - marrow suppression, marrow failure
2. Increased removal - immune response (ITP), consumption (DIC), splenomegaly
Give 4 risk factors for febrile neutropenia
- If the patient had chemotherapy <6 weeks ago
- Any patient who has had a stem cell transplant <1 year ago
- Any haematological condition causing neutropenia
- Bone marrow infiltration
- those on methotrexate, carbimazole and clozapine
What is hyper viscosity syndrome?
Increase in blood viscosity usually due to high levels of immunoglobulins
What 3 blood test values would be increased in someone with polycythaemia?
- Hb
- RCC
- PCV
what are the clinical features of chronic lymphoblastic leukaemia (CLL)?
- often no symptoms
- may be anaemic
- weight loss, sweats, anorexia
- hepatosplenomegaly
- enlarged, rubbery, non-tender nodes
what are the risk factors for folate deficiency?
- elderly
- poverty
- alcoholic
- pregnant
- crohn’s/coeliac
what are the risk factors for pernicious anaemia?
- female
- elderly
- fair hair and blue eyes
- blood group A
- thyroid and addison’s disease
what is the clinical presentation of folate deficiency?
- may be asymptomatic
- may present with anaemia symptoms
- glossitis
- no neuropathy
what investigations should be undertaken in folate deficiency?
- blood count and film = megaloblastic, RBCs macrocytic
- low serum and red cell folate low
- serum bilirubin may be raised
what is the management for acute attacks in sickle cell anaemia?
IV fluids
analgesia
oxygen
antibiotics
what is the treatment for G6PD?
- blood transfusion
- stop exacerbating drugs
what investigations should be undertaken for polycythaemia?
- blood count = raised WBC and platelets
- raised Hb
- JAK2 mutation on genetic screen
- bone marrow biopsy - proliferation of granulocytes and megakaryocytes
- low serum EPO
which investigations should be undertaken for immune thrombocytopenia purpura (ITP)?
- bone marrow examination = thrombocytopenia with normal/increased megakaryocytes
- platelet autoantibodies
what are the causes of thrombotic thrombocytopenic purpura (TTP)?
idiopathic autoimmune e.g. SLE cancer pregnancy drug related
what is the management for von Willebrand’s disease?
- Education on bleeding risks
- Stop any antiplatelet drugs and NSAIDs
- Tranexamic acid - used for minor bleeds
- Combined oral contraceptive to control menorrhagia in women
- Desmopressin -> increases vWF levels
- Platelet transfusions can be helpful in some
- Family screening should be offered
what are the causes of aplastic anaemia?
- inherited
- idiopathic
- benzene
- chemotherapeutic drugs
- antibiotics
- infections - EBV, HIV and TB
what investigations are undertaken for aplastic anaemia?
- blood cell count
- pancytopenia and low reticulocyte count
bone marrow examination - hypocellular with increased fat spaces
- pancytopenia and low reticulocyte count
what is the treatment for aplastic anaemia?
- treat cause
- antibiotics for neutropenia
- RBC transfusion and platelets
- bone marrow transplant
- immunosuppressive therapy
- ATG and ciclosporin if under 40
what are the investigations for von Willebrand’s disease?
- FBC
- Fibrinogen level
- Platelet count - normal
- Clotting screen- Plasma vWF decreased
- Factor VIII levels - can be decreased as vWF is not present to protect it
what are the investigations for haemophilia B?
- Normal prothrombin time
- Prolonged activated partial thromboplastin time
- Low factor IX levels
- Endoscopy if GI bleed suspected
what is the treatment for malaria?
- Chloroquine for non-falciparum malaria
- Oral quinine sulphate for falciparum
- Add IV quinine dihydrochloride for severe disease
Do not treat those with G6PD deficiency
Need specialist help
what is the pathophysiology of anaemia of chronic disease?
● Decreased release of iron from bone marrow to developing erythroblasts
● Inadequate erythropoietin response to anaemia – decreased RBC survival
what are the side effects of erythropoietin treatment?
flu-like symptoms,
hypertension,
raised platelets,
thromboembolism
what are the main causes of aplastic anaemia?
- inherited - Fanconi’s anaemia
- idiopathic- benzene, toulene, glue sniffing
- chemotherapeutic drugs
- antibiotics
- infections - EBV, HIV, TB
what is the long term management for sickle cell anaemia?
- hydroxyurea - increases the HbF levels and is used in the prophylactic management of sickle cell anaemia to prevent painful episodes
- pneumococcal vaccine
how is heparin treatment reversed?
- proteamine
Describe the treatment for symptomatic myeloma
Bone pain = analgesia Bisphosphonates (zolendronate) Blood transfusion - for anaemia Fluids and dialysis - renal failure Chemotherapy Stem cell transplant
what are the clinical features of AML?
- Anaemia -> breathlessness, fatigue, pallor
- low WCC = Infection
- low platelets = bleeding/bruising
- Hepatosplenomegaly
- Peripheral lymphadenopathy
- Gum hypertrophy
- Bone marrow failure and bone pain
what are the clinical features of CML?
- Symptomatic anaemia
- Abdominal pain = splenomegaly
- Weight loss, tiredness, palor
- Gout - due to purine breakdown
- Bleeding - due to platelet dysfunction
- fever and sweats in absence of infection
What is the treatment for CML?
- Chemotherapy
- Tyrosine kinase inhibitors, e.g. Imatinib - Given orally
- Stem cell transplant
what are the clinical features of ALL?
- Anaemia = breathlessness, fatigue, pallor
- Low WCC = Infection
- low platelets = bleeding and bruising
- Hepatosplenomegaly
- Peripheral lymphadenopathy
- CNS involvement - headache, cranial nerve palsies
- SVC obstruction, dilated superficial chest veins
- Bone marrow failure and bone pain
What is the treatment for ALL?
- Blood and platelet transfusion
- Chemotherapy
- Steroids
- Allopurinol to prevent tumour lysis syndrome
- Intrathecal drugs, e.g. methotrexate
- Acute control of infections with IV antibiotics
- Neutropenia makes this high risk
- Stem cell transplant
Describe the treatment for AML
- Blood and platelet transfusions
- IV fluids
- Allopurinol to prevent tumour lysis
- Infection control with IV antibiotics
- Chemotherapy
- Steroids
- Sibling matched allogenic bone marrow transplant
What is the diagnostic test for multiple myeloma
Serum/urine electrophoresis
what is DIC?
pathological activation of coagulation cascade
fibrin in vessel walls
there is platelet and coagulation factor consumption
give 3 causes of DIC?
sepsis
trauma
malignancy
what is the affect of DIC on blood results
TT, PTT, APTT and fibrinogen
TT, PTT, APTT all increase
fibrinogen decreases
what is the most common trigger of sickle cell crisis?
parvovirus B19
