Haematology Flashcards
What would presence of spherocytes on blood smear indicate?
Hemolytic anaemia
What antiglobulin test is positive in hemolytic anaemia?
Direct antiglobulin testing
IgG antibodies present
What condition produces bite cells in blood smear?
G6PD
When are howell jolly bodies present?
Splenic dysfunction
When are rouleaux cells present?
Malignant lymphoma and multiple myeloma
Which cells are seen in hodgkin’s lymphoma?
Reed sternberg
What chromosome is important in development in chronic myeloid leukaemia?
Philadelphia chromosome
What is wrong with the Philadelphia chromosome in CML?
Reciprocal translocation between chromosomes 9 and 22
What is light chain deposition?
are blood cell disorder that occurs when the body deposits too many light chains in organs, damaging them and causing disease. Light chains are fragments of immunoglobulins that the body uses to fight infection. The kidneys normally clear light chains, but in LCDD, they build up and cause disease.
Which disease is associated with light chain deposition and why can it cause renal failure?
Multiple myeloma
Causes renal failure by causing tubular toxicity
What is non-haemolytic febrile transfusion reaction?
Due to acute reaction to plasma proteins - as long as temp is below 38.5 and patient is asymptomatic transfusion ca be temp stopped then continue with regular observations
Which factor is haemophilia A vs B caused by?
A - Factor Viii
B - Factor ix
What is haemophilia?
Deficiencies in clotting factors of viii and ix which are part of coagulation cascade
What is diagnositc method for haemophilias?
Factor assays
How can minor bleeds in haemophilia A be managed medically? and how does it work?
Desmopressin - increases factor Viii
For major bleeds in haemophilia A and B what is treatment?
Recombinant factor viii and vi respectively
What complication in haemophilia A can arise following factor viii treatment?
Boy can develop inhibitor to factor viii
What is inheritance pattern of haemophilia?
X linked recessive
What causes beta thalassaemia major?
Defect causing a ceasing in beta chain production - therefore no HbA is produced so HbF is continued to be produced to compensate for this
When does beta thalassaemia major present?
First year of life
When does HbF normally stop being produced?
At 6 months
What symptoms can present with beta thalassaemia major?
RBC being produced in abnromal locations like spleen - causing splenomegaly and skull - causes frontal bossing
Which ethnicity of patients are usually affected by beta thalassaemia major?
Mediterranean or far eastern
What is inheritance of thalassaemia?
Autosomal recessive
What is cause of alpha thalassaemia?
Non functioning copies of four alpha globin gene on chromosome 16
Inheritance of 2 - is asymptomatic
3- symptomatic : haemoglobin H disease - microcytic anaemia , splenomegaly
4: hydrops fetalis - incompatible with life
What puts a patient at risk of vitamin K deficiency?
Alcoholism
Poor nutrition
GI disease
What is disseminated intravascular coagulation?
Widespread activation of coagulation pathways and subsequent depletion of platelets and coagulation factors
What can cause disseminated intravascular coagulation?
Sepsis
Haematological malignancies
Organ dysfunction
What happens to the PT in DIC?
Prolonged
What is polycythaemia rubra vera?
chronic blood cancer that causes the bone marrow to produce too many red blood cells
What symptoms would present with polycythaemia rubra vera?
Red cell mass
Fatigue
Headache
Visual disturbance
Increased risk of thrombosis and haemorrhage
Tinnitus
Itching post hot bath
Facial plethora
What gene is mutated in polycythaemia rubra vera?
JAK-2 v617F
What is management principles of polycythaemia rubra vera?
Venesection
Cytoreductive therapy - suppresses erythropoiesis
Aspirin daily
What symptoms is classic post bath in polycythaemia rubra vera?
Itching after a hot bath
What antibodies can be developed with phenytoin use?
Antibodies to factor Viii
What would be expected of the APTT with mixing studies in congenital factor viii deficiency?
Corrected APTT if it does not correct it means the bleedig disorder is due to antibody action rather than failure to produce clotting factors
What is serum erythropoietin levels in primary vs secondary polycythaemia rubra vera?
Primary: low serum erythropoietin because through feedback loop body tries to lessen erythropoietin so no more further erythrocytosis.
In secondary polycythaemia the erythrocytosis is not driven by erythropoietin so the serum erythropoietin is high
What is plummer vinson syndrome?
Triad of oesophageal webs
Microcytic anaemia
Glossitis
Who does Plummer Vinson usually affect?
Middle aged women
What are ferritin levels like in Plummer Vinson?
Reduced
What is treatment of Plummer Vinson?
Iron replacement
Endoscopic dilation of oesophagus
Which proteins are found in urine electrophoresis in multiple myeloma?
Bence jones proteins - M proteins
What is primary myelofibrosis?
Chronic progressive myeloproliferative disorder - low production of RBC , WBC and platelets
What are risk factors for primary myelofibrosis?
Old age
Industrial solvents
What cells are seen on peripheral blood film in myelofibrosis?
Tear drop shaped RBC