Haematology Flashcards
How does hereditary spherocytosis present?
- Anaemia
- Jaundice
- Splenomegaly
- Pigment gallstones
Describe the management of hereditary spherocytosis
- Folic acid
- Transfusion
- Splenectomy (if very severe anaemia)
How does G6PD present?
- Anaemia
- Neonatal jaundice
- Splenomegaly
- Pigment gallstones
- Drug, broad bean or infection precipitated jaundice and anaemia
How does sickle cell disease present?
- Bone crisis
- Chest crisis
- Stroke
- Increased infection risk (hyposplenism)
- Gallstones
- Aplastic crisis
- Sequestration crisis: spleen and liver
Describe the management of a sickle cell crisis
- Analgesia (opiates and NSAIDs)
- Hydration
- Oxygen
- Blood transfusion
- Consider antibiotics
Name some of the treatment options for sickle cell disease
- Vaccinations
- Penicillin
- Folic acid
- Blood transfusion
- DMARDs: hydroxycarbamide
- Bone marrow transplantation
- Gene therapy
What is beta thalassaemia major?
- No beta chains
- Transfusion dependent anaemia
What is thalassaemia minor?
- Carrier state
- Hypochromic microcytic red cell indices
How does beta thalassaemia major present?
- Severe anaemia
- Ineffective bone marrow
- Bony deformities
- Splenomegaly
- Growth retardation
How is beta thalassaemia major managed?
- Chronic transfusion support
- Iron chelation therapy (s/c desferrioxamine)
- MRI scans
- Bone marrow transplantation
What investigations might be done for the different types of anaemia?
- Microcytic: ferritin
- Normocytic: reticulocyte count
- Macrocytic: B12, folate and bone marrow
Name the causes of a microcytic anaemia with a normal ferritin
- Thalassaemia
- Secondary anaemia
- Sideroblastic anaemia
Name the causes of a normocytic anaemia with an increased reticulocyte count
- Acute blood loss
- Haemolysis
Name the causes of a normocytic anaemia with a normal or low reticulocyte count
- Secondary anaemia
- Hypoplasia
- Marrow infiltration
Name some of the causes of a haemolytic anaemia
- Congenital
- Autoimmune
- Mechanical e.g. artificial valve
- Severe infection/DIC
- PET/HUS/TTP
Describe the management of haemolytic anaemia
- Folic acid
- Immunosuppression
- Splenectomy
- Treat causes
- Transfusion
Name the causes of megaloblastic anaemia
- B12 deficiency
- Folate deficiency
Name the causes of non-megaloblastic macrocytic anaemia
- Myelodysplasia
- Marrow infiltration
- Drugs
Which drugs are given as prophylaxis for neutropenic sepsis in haematological malignancy?
- Ciprofloxacin
- Fluconazole
- Aciclovir
- Co-trimoxazole
How does neutropenic sepsis present?
- Fever
- Rigor
- Chest infection/pneumonia
- Cellulitis
- UTI
- Septic shock
How can neutropenic sepsis be managed?
- Resuscitate
- Tazocin + Gentamicin +/-vancomycin or tecoplanin
- Antifungal if no response within 72 hours
- CT CAP
Name the types of transfusion reactions
- Transfusion associated dyspnoea
- Transfusion related lung injury
- Transfusion associated circulatory overload
- Transfusion associated graft vs host
What is Factor V Leiden ?
- A autosomal dominant mutation in the gene for Clotting factor V
- Causes a hypercoagulable state
How can Factor V Leiden be managed?
Anticoagulation after VTE
How can DIC be managed?
- Platelet +/- plasma transfusion +/- cryoprecipitate
- Heparin
What type of bleeding is found in platelet disorders?
- Epistaxis
- Purpura
- Menorrhagia
- GI
What type of bleeding is found in coagulation factor disorders?
- Articular
- Muscle
How do Haemophilia A and B present?
- X linked (majority boys)
- Haemarthrosis
- Muscle haematoma
- CNS bleeding
- Retroperitoneal bleeding
- Post surgical bleeding
- Reduced Factor VIII or FIX
How can Von Willebrand disease be managed?
- VWF concentrate
- DDAVP (desmopressin)
How can ITP be managed?
- Steroids
- IV IgG
- Splenectomy
- Thrombopoietin analogues (romiplostim)
How does AML present?
- Bone marrow failure
- Hepatomegaly and splenomegaly
- Petechiae
How does ALL present?
- Fatigue
- Malaise
- Headache
- Fever
- Bone marrow failure
- Recurrent infections
- Bone pain
How does CLL present?
- Immune deficiency
- Lymphadenopathy
- Splenomegaly
- Petechiae
- Fatigue
How does CML present?
- Fatigue
- Night sweats
- Weight loss
- Early satiety or abdominal distension
- LUQ pain (splenic infarction)
- Hepatosplenomegaly
- Lymphadenopathy
- Easy bruising
- Gout
Name the B symptoms of Lymphomas
- Night sweats
- Weight loss
- Fever
How are lymphomas staged?
- Stage 1: lymphoma in one group of nodes
- Stage 2: 2 or more groups of nodes on the same side of the diaphragm
- Stage 3: lymph nodes on both sides of the diaphragm
- Stage 4: lymphomas outside the lymphatic system or in thebone marrow
- B for any B symptoms
What is a myeloma?
Neoplastic disorder of plasma cells resulting in excessive production of a single type of immunoglobulin
How does myeloma present?
- Bone lesions, pathological fractures and cord compression
- Hypercalcaemia
- Bone marrow failure
- Infections
- Renal failure
How can haemachromatosis be managed?
- Venesection
- Desferrioxamine mesilate
- Vitamin C
How can hyposplemism/splenectomy be managed?
- Immunisations
- Phenoxymethylpenicillin/ amoxicillin/ erythromycin
Name three examples of myeloproliferative disorders
- Chronic idiopathic myelofibrosis (scar tissue in the bone marrow)
- Polycythaemia Vera (excessive production of RBCs)
- Essential thrombocythaemia (raised platelets)
What is pancytopenia?
A reduction in red cells, white cells and platelets
What is polycythaemia?
A increased concentration of red blood cells
