Haematology Flashcards
What is the diagnosis of hereditary spherocytosis?
EMA binding test
How is hereditary spherocytosis managed? (of both acute haemolytic crises and longer term treatment)
Acute haemolytic crisis:
treatment is generally supportive
transfusion if necessary
Longer term treatment:
folate replacement
splenectomy
What is seen on blood film in G6PD? (2)
Heinz bodies
Bite/blister cells
What medications should be avoided in G6PD? (3)
Sulf drugs
Anti-malarials
Ciprofloxacin
How does iron overload present?
Same as haemochromatosis DM Impotence Joint pain Liver cirrhosis
What are features of beta thalassaemia major?
Severe symptomatic anaemia
Frontal bossing
Maxillary overgrowth
Extramedullary haematopoiesis
What is the management of a sickle cell crisis?
Red cell exchange transfusion Oxygen Morphine IV fluids IV Abx
What are features of Fanconi anaemia?
Café au lait spots
Aplastic anaemia
Increased risk of AML
Short stature and thumb abnomarlities
What is the inheritance pattern of Fanconi anaemia?
Autosomal recessive
What is sideroblastic anaemia?
Type of microcytic anaemia - can be congenital or ACQUIRED
High ferritin and transferrin saturation
How is sideroblastic anaemia seen on blood film?
Basophilic stippling
What is seen on bone marrow biopsy in sideroblastic anaemia?
Ringed sideroblasts
What is the most common type of Hodgkin’s lymphoma?
Which one has the best prognosis?
Nodular sclerosing
Best prognosis = Lymphocyte predominant
What is the management of non-Hodgkin’s lymphoma?
R-CHOP
Which marker is raised in lymphoma?
LDH- Lactate dehydrogenase
Which marker is decreased in CML?
Leukocyte alkaline phosphatase
What is Richter’s transformation?
CLL –> High grade lymphoma
Which type of autoimmune haemolytic anaemia does CLL predispose to?
Warm
What is tumour lysis syndrome and what are seen on lab results?
Complication of chemo treatment to leukaemia/lymphoma
High creatinine
Abdominal pain
AKI
High uric acid
High potassium
High phosphate
Low calcium
What is given for prophylaxis of tumour lysis syndrome?
IV Allopurinol
What are the 4 investigations done in multiple myeloma?
BLIP
B= Bence Jones (urine electrophoresis) L= serum light chain assay I = Serum immunoglobulins - raised P= serum protein electrophoresis --> increased monoclonal IgG
What are x-ray signs of multiple myeloma?
Lytic lesions
Punched out lesions
Raindrop skull
What is myelodysplastic syndrome?
Acquired disorder - blood cells do not develop properly
usually due to chemo or radiotherapy
Low Hb
Low WCC
Low platelets
How does essential thrombocytosis present?
Headaches
Dizziness
Burning sensation in hands
Increased risk of thrombosis
How is essential thrombocytosis managed?
Aspirin
Hydroxycarbamide
What are causes of polycythaemia?
Relative – dehydration
Primary – Polycythaemia rubra vera
Secondary – COPD, high altitude, obstructive sleep apnoea
How can you differentiate between primary and secondary polycythaemia?
Red cell mass studies
How does polycythaemia present?
Plethoric appearance
Itching esp in bath/heat
Increased risk of thrombosis
High ESR
Low leukocyte alkaline phosphatase
How is polycythaemia rubra vera managed?
Low dose aspirin
Venesuction
What clotting results are seen in DIC?
Low platelets Low fibrinogen High D-dimer High Prothrombin time High APTT High bleeding time
How does Graft vs. Host disease present?
Painful maculopapular rash
Jaundice
Watery/bloody diarrhoea
N+V
What is hereditary angioedema and how is it managed?
Autosomal dominant condition - low plasma levels of C1
Leads to attacks where there is painless non-pruritic swelling
No urticaria
Management = IV C1 inhibitor concentrate
Which DOAC is preferred in renal impairment?
Apixaban
How does chronic steroid use show on FBC?
Neutrophilia
Raised ESR and osteoporosis?
Raised ESR + Osteoporosis = Multiple myeloma until proven otherwise
Management of DIC
- Resus measures
2. Fresh frozen plasma + Cryoprecipitate
What are signs on examination of lymphoma?
Nodule
Splenomegaly
Hepatomegaly
What are general signs on examination of anaemia?
Tachypnoea
Tachycardia
Conjunctival pallor
What are iron-deficiency anaemia specific signs?
Angular Cheilitis
Koinionychia
Atrophic glossitis
What are side effects of ferrous sulphate?
Black stools
Constipation
Diarrhoea
Nausea
What are acute complications of multiple myeloma?
Hypercalcaemia
Spinal cord compression
Acute renal failure
Hyperviscosity
What should be given between every unit of packed red cells?
Stat dose of furosemide - can lead to fluid overload.
Multiple Myeloma vs. Waldenstrom’s Macroglobulinaemia
MM - usually IgG
Waldenstroms - usually IgM
Waldenstorms can still cause systemic upset - weight loss, ltheragy, hyperviscosciy syndrome, hepatomegaly, lymphadenopathy
No raised calcium
DIC blood results
- ↓ platelets
- ↓ fibrinogen
- ↑ PT & APTT
- ↑ fibrinogen degradation products (D-dimer)
Patient with hereditary spherocystosis + Abdominal pain?
Gallstones
Why does haemolytic anaemia lead to gallstones?
Haemolysis –> Increased bilirubin –> Excreted into the bile
What is seen on blood film in DIC?
Schisocytes
What are causes of sideroblastic anaemia?
Congenital Myelodysplasia anti-TB meds Alcohol Lead
Beta thalassaemia trait vs. Beta thalassaemia major?
Beta thalassaemia trait - presents with mild microcytic anaemia
Beta thalassaemia major - presents in first year of life with failure to thrive and hepatosplenomegaly
Macrocytic anaemia + hyper-segmented neutrophil polymorphs on blood film?
Megaloblastic anaemia - either folate or B12
Platelet transfusion thresholds?
Not bleeding = <10
Minor bleeding = <30
Prior to surgery = <50
Surgery at major site/bleeding at major site = <100
Do inherited haematological conditions cause early jaundice or prolonged jaundice?
Early jaundice - presents prior to 24 hours.
What is seen on blood film in thalassaemia?
Heinz bodies
Target cells
Basophilic stippling
What is seen on blood film in hyposplenism?
Target cells Howell-Joly bodies Siderotic granules Acanthocytes Pappenheimer bodies
What is Graft versus Host disease and how does it present?
Often after a bone marrow transplant but can also occur after organ transplant or blood transfusion in those who are immunosuppressed
Painful maculopapular rash
Jaundice
Watery/bloody diarrhoea
N+V
How can you prevent Graft versus Host disease from a blood transfusion?
Need to give irradiated RBCs to minimise risk of graft versus host disease
What clotting results are seen in vitamin K deficiency?
Raised APTT
Raised prothrombin time
Normal bleeding time
What is the inheritance pattern of haemophilia?
X-linked recessive
How is Von Willebrand’s disease managed?
Desmopressin - stimulates release of vWF
Tranexamic acid for menorrhagia
VWF infusion
Which protein is deficient in thrombotic thrombocytopenic purpura?
ADAMTS13
How is polycythaemia vera managed?
Venesection to reduce Hb
Aspirin to reduce risk of thrombotic events
What is given to reduce the risk of tumour lysis syndrome?
Allopurinol or Rasburicase
What does lead poisoning do to the blood?
Causes sideroblastic anaemia
Basophilic stippling
Microcytic anaemia
Raised ferritin and iron
What is seen on blood film in myelofibrosis?
Tear drop poikilocytes
Due to RBCs trying to get through sclerosed bone marrow
What is seen on blood film in Multiple Myeloma?
Rouleaux formation
What prophylactic antibiotics are someone with a splenectomy given?
Penicillin V
How is sideroblastic anaemia treated?
Supportive - transfusions may be needed (+Iron chelation with Desferrioxamine)
Pyirodixine
Why does chemotherapy increase the risk of gout?
Due to increased production of uric acid
