Haem Problems Flashcards
Aplastic anaemia
Severe hematologic condition in which the body fails to make blood cells in sufficient numbers
example: aplastic crisis in a patient with sickle cell disease due to the Parovirus B19
Von Willebrand disease
Inherited bleeding disorder characterized by a reduced quantity or function of von Willebrand factor (VWF).
This protein normally links platelets to the exposed endothelium and stabilises clotting factor VIII, and its deficiency or dysfunction leads to an increased risk of bleeding.
normal PT time, prolonged APTT
Disseminated intravascular coagulation (DIC)
A severe, complex condition that describes the inappropriate activation of the clotting cascades, resulting in thrombus formation (in microvasculature) and subsequently leading to the depletion of clotting factors and platelets => bleeding
- Low platelets and fibrinogen (being used up quickly)
- Increased PT, APTT and d-dimer (fibrin degradation product)
Polcythaemia rubra vera
Polycythaemia is characterised by an increase in haematocrit, red cell count, and haemoglobin concentration.
- Relative = due to low plasma volume
- Absolute = the red cell mass is genuinely increased
Key signs and symptoms include fatigue, headache, visual disturbances, pruritus, and increased risk of thrombosis and haemorrhage
NOTE:
- assoc. with itching after a hot bath/shower
- Raised red cell mass, low serum erythropoetin
- > 95% have JAK2 gene
Haemophilia A and B
Both X-linked recessive inherited bleeding disorders, caused by deficiencies in clotting factors VIII and IX respectively, both of which are integral components of the instrinsic pathway of the coagulation cascade.
Tumour lysis syndrome
Characterised by metabolic and electrolyte abnormalities that occur when a large number of cancer cells die within a short period.
These abnormalities include hyperkalaemia, hyperphosphataemia and hyperuricaemia. Hyperuricaemia associated with chemotherapy can be prevented by taking allopurinol
Pernicious anaemia
An autoimmune condition associated with a deficiency of intrinsic factors and, subsequently, vitamin B12 => megaloblastic anaemia
Multiple myeloma
A malignant plasma cell dyscrasia characterized by the proliferation of abnormal plasma cells (post-germinal B cells) in the bone marrow. These plasma cells secrete monoclonal antibodies (most commonly of the Ig subtype) and antibody fragments into the serum and the urine. There is also a relative deficiency of functional antibodies, resulting in a relative hypogammaglobulinaemia. This condition leads to bone destruction, kidney dysfunction, anaemia, and a range of systemic symptoms
- serum electrophoresis = an IgG paraprotein spike
- Urinary bence jones protein (monoclonal globulin protein or immunoglobulin light chain found in urine)
Transfusion-related acute lung injury (TRALI)
Severe lung disease within 6 hours of the transfusion, featuring severe hypoxaemia, which does not respond to oxygenation, caused by anti-leukocyte antibodies. This is very often related to the use of fresh frozen plasma. Treatment is primarily supportive, requiring aggressive oxygenation and often admission to intensive care.
Deep Vein Thrombosis (DVT)
The intra-luminal occlusion of deep veins with a clot, in a limb or the pelvis.
Hodgkin’s lymphoma
Hodgkin lymphoma is malignant lymphoma characterised by the presence of Reed–Sternberg cells.
Peak incidence is in the third decade of life.
often presents in young adults with cervical or supraclavicular non-tender lymphadenopathy
G6PD haemolytic anaemia
Glucose-6-phosphate dehydrogenase (G6PD) deficiency is an inherited condition in which patients are excessively susceptible to the development of haemolytic anaemia
Triggers include: henna, fava beans
Haemolytic anaemia
The premature destruction of red blood cells (RBCs), resulting in a reduced RBC lifespan.
It can be categorised into hereditary and acquired forms, and further divided into intravascular or extravascular and autoimmune or non-autoimmune subtypes
Subacute combined degeneration of the spinal cord
(Just for year 2 knowledge - will probs need to know more about in third year)
This is a a complication of replacing folate before B12 or giving them together so ALWAYS give B12 before folate
Haemosiderosis
Iron overload disorder resulting in accumulation of hemosiderin
