Haematology Flashcards
What are the causes of microcytic anaemia?
- Thalassaemia
- Anaemia of chronic disease
- Iron deficiency
- Lead poisoning
- Sideroblastic
What are the causes of normocytic anaemia?
- Anaplastic anaemia
- Anaemia of chronic disease
- Acute blood loss
- Haemolytic anaemia
- Hypothyroidism
What are the causes of macrocytic anaemia?
Megaloblastic:
- B12 and folate deficiency
Normoblastic
- alcohol
- reticulocytosis
- hypothyroidism
- liver disease
- drugs e.g. azathioprine
What are the generic symptoms of anaemia?
- dizziness
- palpitation
- fatigue
- headaches
- shortness of breath
What are the generic signs of anaemia?
- Pale skin
- conjunctival pallor
- tachycardia
- raised resp rate
What are the causes of iron deficiency anaemia?
- dietary intake not sufficient
- malabsorption e.g. cystic fibrosis
- pregnancy
- growth spurt
- blood loss e.g. menorrhagia, GI loss
Bloods iron deficiency
- microcytic
- hypochromic
- low iron
- low ferritin (may be raised or normal if inflammation)
- low transferrin
- high total iron binding capacity
Bloods anaemia of chronic disease
- normocytic normochromic or microcytic hypochromic
- decreased iron
- increased ferritin
- normal or low total iron binding capacity
- low transferrin
What is sideroblastic anaemia?
Deposition of iron in the mitochondria, forming a ring around the nucleus of the cell
What are the causes of sideroblastic anaemia?
- congenital
- myelodysplasia
- alcohol
- lead
- anti TB drugs
bloods sideroblastic anaemia
- hypochromic
- microcytic
- high ferritin, iron and transferrin
- basophillic stipping
- sideoblast prussian blue staining
What are the inherited types of hameolytic anaemia?
- hereditary spherocytosis
- G6PD deficiency
- Hereditary Elliptocytosis
- Sickle cell anaemia
What are the acquired forms of haemolytic anaemia?
- warm type autoimmune
- cold type autoimmune
- prosthetic valve haemolysis
- alloimmune: blood transfusion reaction or heamolytic disease of newborn
- microangiopathic haemolytic anaemia
Presentation of hereditary spherocytosis
- jaundice
- gallstones
- splenomegaly
- aplastic crisis if parovirus
bloods hereditary spherocytosis
- sphere shaped RBC
- raised MCHC
- raised reticulocytes
What is the management of hereditary spherocytosis?
- folate supplements
- splenectomy
What is the inheritance of hereditary spherocytosis?
Autosomal dominant
What is the inheritance of G6PD deficiency?
X linked recessive
What are the triggers for G6PD deficiency?
- fava beans
- infection
- primaquine (anti-malarial)
- ciprofloxacin
- sulfonylurea
- sulfasalazine
What is the management of warm type autoimmune haemolytic anaemia?
Steroids ± rituximab
immunoglobulin warm type AIHA?
IgG
immunoglobulin cold type AIHA
IgM
What are the causes of warm type AIHA?
- Idiopathic
- Autoimmune disease e.g. SLE
- Neoplasia (lymphoma, leukaemia)
What are the causes of cold type AIHA?
- neoplasia e.g. lymphoma
- Infection: mycoplasma, EBV
What is the cause of haemolytic anaemia with prosthetic heart valves?
Turbulence around the valve causing collision of the red blood cells with the implanted valve
What is microangiopathic haemolytic anaemia?
Structural deformities of the blood vessels results in haemolysis
What are the causes of microangiopathic haemolytic anaemia
- Haemolytic uraemic syndrome
- disseminated intravascular dissemination
- thrombotic thrombocytopenia purpura
- SLE
- cancer
What are the causes of vitamin B12 deficiency?
- pernicious anaemia
- post gastrectomy
- vegan diet
- disorders of the terminal ileum (Crohn’s or ileoceacal resection)
What is the management of vitamin B12 deficiency anaemia?
- IM hydroxocobalamin 3 times a week for two weeks then once every 3 months
- Must correct B12 before folate due to degeneration of the cord
What chromosome alpha thalassaemia?
16
What chromosome beta thalassaemia?
11
Describe alpha thalassaemia
- if one or two alleles: hypochromic, microcytic and normal Hb
- if 3: HbH disease: hypochromic, microcytic, splenomegaly
- if 4: death in utero
Describe beta thalassaemia
- trait/minor: hypochromic, microcytic, target cell, Hb normal
- Major (both genes): severe anaemia, splenomegaly, hepatomegaly and bone expansion
Bone expansion beta thalassaemia major
- hair on end appearance in skull
- fragility fracture
What is the management of beta thalassaemia major?
- regular red blood cell transfusions
- consider splenectomy
- monitor iron, iron chelation (desferrioxamine)
- folic acid
Schistocytes
Microangiopathic haemolytic anaemia
Why may the urine appear dark in haemolytic anaemia?
Haemoglobinuria in severe haemolysis, usually intravascular
Differentials of petechiae
- Leukaemia
- Meningococcal septicaemia
- Vasculitis
- Henoch-Schonlein Purpura (HSP)
- Idiopathic Thrombocytopenia Purpura (ITP)
- Non-accidental injury
What is the presentation of leukaemia?
- fatigue
- petechiae and abnormal bruising
- fever
- abnormal bleeding
- hepatosplenomegaly
- lymphadenopathy
What are the types of leukaemia?
- acute myeloid leukaemia
- acute lymphoblastic leukaemia
- chronic myeloid leukaemia
- chronic lymphocytic leukaemia
Blood acute myeloid leukaemia
- blast cells
- auer rods
Blood acute lymphoblastic leukaemia
- blast cells
- no auer rods
Blood chronic lymphocytic lymphoma
- smear/smudge cells on blood film
Which leukaemias are associated with the philadelphia chromosome?
- acute lymphoblastic leukaemia
- chronic myeloid leukaemia
What is Richter’s transformation?
The transformation of chronic lymphocytic leukaemia into a high grade lymphoma
What is the presentation of Hodgkin’s lymphoma?
- lympadenopathy: rubbery, non tender
- B symtpoms: fever, weight loss, night sweats
- Fatigue
- itch
- cough
- shortness of breath
- abdominal pain
What are the risk factors for Hodgkin’s lymphoma?
- HIV
- EBV
- Family history
- Autoimmune conditions e.g. rheumatoid arthritis
Hodgkin’s lymphoma biopsy
Reed Sternberg cells
Explain the staging of hodgkin’s lymphoma
- I: confined to one region of lymph nodes
- II: more than one region but one side of the diaphragm
- III: lymph nodes above and below the diaphragm
- IV: widespread involvement including non lymphatic organs
What are the non-Hodgkin lymphomas?
- Burkitt
- MALT
- Diffuse large B cell
What is monoclonal gammopathy of undetermind significance?
Excess of a single type of antibody or antibody component without any other features of myeloma or cancer
What is smouldering myeloma?
Progression of MGUS, high levels of antibodies or antibody components. Premalignant
What is multiple myeloma?
Myeloma affecting multiple areas of the body
Bloods myeloma
- low WCC (neutropenia)
- anaemia
- thrombocytopenia
- Rasied ESR
- Raised calcium
- Raised plasma viscosity
Specific tests for myeloma
- Bence jones proteins
- serum free light chain assay
- serum immunoglobulin
- serum protein electrophoresis
- bone marrow biopsy
Investigation for spread of myeloma
- MRI
- CT if MRI not suitable, X ray if CT not suitable
What are the signs of myeloma on X-Ray?
- punched out lesions
- lytic lesions
- raindrop skull
What is the presentation of myeloma?
- Dehydration ( myeloma renal disease)
- Easy bruising
- reduced sight
- Heart failure
- Bone pain (pathological fracture, increased activity of osteoclasts)
What are the myeloproliferative neoplasms?
- Essential thrombocytopenia
- Myelofibrosis
- polycythaemia vera
What is the presentation of essential thrombocytopenia?
- anaemia
- splenomegaly
- portal hypertension
- thrombosis
- decreased WCC
RAISED PLATELETS
What is the presentation of polycythaemia vera?
- splenomegaly
- portal hypertension
- thrombosis
- Low WCC
RAISED HAEMOGLOBIN
What is the presentation of myelofibrosis?
- anaemia
- splenomegaly
- portal HTN
- low platelets and WCC
blood film myelofibrosis
- Teardrop RBCs
- Poikilocytosis
- blasts
What is the management of essential thrombocytopenia?
- Hydroxyurea
- Aspirin
- Chemotherapy
What is the management of polycythaemia vera?
- venesection
- aspirin
- chemotherapy
What is the management of myelofibrosis
- Allogenic stem cell transplant
- chemotherapy
What are the genetic mutations associated with myeloproliferative disorders?
- JAK2
- MPL
- CALR
What is Von Willebrand Disease?
Autosomal dominant bleeding disorder
What are the types of von willebrand disease?
- Type 1: decreased factor
- Type 2: abnormal VWF
- Type 3: total lack of VMF (type 3)
What are the symptoms of von willebrand disease?
- increased bleeding
- monorrhagia
- nose bleeds (epistaxis)
What are the investigations for von willebrand disease?
- Prolonged bleeding time
- APTT may be prolonged
- VWF function assay
What is the management of von willebrand disease?
- tranexamic acid
- desmopression
- factor VIII
What is the deficiency in haemophilia A?
Factor VIII
What is the deficiency in haemophilia B?
Factor IX
What is the inheritance of haemophilia A and B?
Autosomal recessive
What are the symptoms of haemophilia?
- excessive bruising
- fatigue
- recurrent or severe bleeding
- musculoskeletal bleeding - presents with the swelling of joints
- cutaneous purpura in acquired
What are the risk factors for acquired haemophilia?
- Age >60
- Autoimmune conditions
- IBD
- Diabetes
- Malignancy
Investigations haemophilia
- Prolonged APTT
- Plasma factor VIII and IX assay: decreased or absent
- Mixing study: APTT corrected
- FBC: Hb may be low
- Prothrombin time, platelet aggregation studies should be normal
What is the treatment of haemophilia?
- Replace factor VIII or IX
- If acquired: immunosuppression with prednisolone and cyclophosphamide
What does a low haemoglobin with a high reticulocyte count suggest?
Haemolysis or bleeding
Coagulation screen DIC
- ↓ platelets
- ↓ fibrinogen
- ↑ PT & APTT
- ↑ fibrinogen degradation products
What is the diagnostic test for hereditary spherocytosis?
EMA binding test
What is the empirical antibiotic of choice for neutropenic sepsis?
Piperacillin with tazobactam (Tazocin)
Why are blood products irradiated?
To avoid transfusion related graft vs host disease
What is the presentation of idiopathic Thrombocytopenic purpura?
- petechiae, purpura
- bleeding (e.g. epistaxis)
- catastrophic bleed (uncommon)
What is the first line treatment for ITP?
Oral prednisolone
How long should the oral contraceptive pill be stopped before surgery?
4 weeks
What is the reversal agent for dabigatran?
Idarucizumab
