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
What are symptoms of primary myelofibrosis?
Weight loss
Night sweats
Low grade fever
Fatigue
Cachexia
What causes acute haemolytic reaction?
ABO/ rhesus incompatibility
What are the symptoms of acute haemolytic transfusion reaction?
Agitation
Pyrexia
Hypotension
Flushing
Abdo /chest pain
DIC
What is pathophysiology o von willebrands?
Von willerbrand protein helps platelets stick to damaged area and stabilises factor 8. Therefore is there is deficiency in VWP then there is lack of this clotting and a greater bleeding time
What is treatment of vitamin b12 deficiency?
IM 1mg hydroxocobalamin on alternate days until improved then maintenance therapy of 1mg every two months
In anaemia of chronic disease what happens to the total iron binding capacity and the ferritin levels?
TIBC decrease
Ferritin levels increase. This is opposite to iron deficiency anaemia
What type of anaemia is b12 deficiency cause?
Macrocytic
In a patient on warfrin with INR between 5-8 what is the management?
Hold warfarin for a few days
What is heparin induced thrombocytopenia and how does it present?
Immune mediated reaction causing thrombocytopenia - presents 5-14 days after starting heparin therapy (e.g. dalteparin)
What cells can be present in IDC?
Schistocytes - fragmented RBC due to excess fibrin strands haemolysing passing RBC
What is the difference between VWB type 1 , 2 and 3?
1 is most common and least severe, 2 and 3 are more severe. Type 3 is caused by almost complete deficiency of VWF
Which medication suppresses the production of BRC by controlling erythocytosis?
Hydroxycarbamide
What is target INR for patients with recurrent VTE and on warfarin?
3-4
What is INR target for mechanical mitral valves?
3.5
What is target INR for Antiphospholipid syndrome?
2.5
What are some symptoms of idiopathic thrombocytopenia purpura?
Prolonged gum bleeding
Menorrhagia
Epistaxis
What cells are precursors to mature RBC?
Nucleated RBC
When are nucleated RBC seen?
Bone marrow is working hard to produce RBC quickly e.g. in a haemolytic crisis
What is medical treatment for von willebrand?
Desmopressin which stimulates release of VWF
What is treatment for beta thalassaemia major?
2-4 weeks blood transfusions
Folate deficiency can cause pancytopenia
Which one of the following structures contains white and red pulp, and functions to trap foreign substances carried in the blood?
Spleen
Which blood vessel in the spleen is most responsible for monitoring the quality of red blood cells and removing aged ones from circulation?
Splenic sinusoid
What is transfusion haemosiderosis?
Repeated blood transfusions lead to iron deposition in endocrine organs and heart - indication for chelation. If irreversible then heart failure and needs a transplant. Can be a complication of thalassaemia major treatment
What is first line for DVT treatment? unless?
LMWH unless bad renal function - if so give them apixaban
What is a paraproteinaemia?
Overproduction of a monoclonal immunoglobulin or fragment of the antibody which are present in serum or urine - can be pre-malignant or malignant
What is definition of partial remission in acute myeloid leukaemia?
Blasts greater than 5% otherwise all good
What is significance of large number of baasophils in CML?
Means there is accelerated phase of disease
When are rouleaux found?
Multiple myeloma
What is a common cause of AKI in sickle cell patients?
Renal papillary necrosis - sickled RBC cause infarction and necrosis of renal papillae
Which antimalarial should be avoided in g6PD deficiency?
Primaquine
How should iron tablets be taken with levothyroxine?
Iron when taken with levothyroxine can reduce levothyroxine absorption so the levothyroxine should be taken 4 hours before or after levothyroxine
What is sideroblastic anaemia?
Sufficient iron but infective erythropoiesis causing microcytic anaemia and deposition of iron in organs
What will be seen in bone marrow in sideroblastic anaemia?
Ring sideroblasts
What is management of sideroblastic anaemia?
Avoid alcohol
Chelation therapy to reduce iron overload
B6
Blood transfusion
Stem cell transplant
What level must platelets be below to give them to a non bleeding patient?
Below 10
For hyperkalaemia treatment what should be given first then next?
Calcium gluconate
then… insulin and dextrose
The calcium gluconate protects the heart as it stabilises the myocardial membranes
Which substance regulates clotting by breaking down fibrin and fibrinogen - inhibiting thrombin activity?
Plasmin
Why does ciprofloxacin lead to an increased levels of warfarin?
It’s a cytochrome p450 inhibitor - thus increasing warfarin’s affect
What is anisocytosis?
High red cell distribution width - lots of variation in size of RBCs
Which antibodies are usually found with intrinsic factor antibodies?
Parietal cell antibodies
Which translocation is Philadelphia chromosome?
Reciprocal translocation 22 and 11
Which virus is associated with aplastic crisis in sickles?
Parovirus b19
What is splenic sequestration crisis?
Acute splenomegaly
Drop in haemoglobin
Raised reticulocyte count
What is the most common cause of inherited aplastic anaemia?
Fanconi anaemia
How many days bed rest is a risk factor for VTE?
More than 5
Surgery within how many weeks is a risk factor for VTE?
8 weeks
What initiates the intrinsic pathway?
When blood comes into contact with collagen on an injured vessel wall
What are symptoms of pernicious anaemia?
Cognitive impairement
jaundice
neurological symptoms: neuropathy , balance, sensation and coordination
Which clotting factors are inhibited production by warfarin?
ii, vii, ix, x
2 + 7 = 9 and 10
Which test is useful in diagnosis of autoimmune haemolytic anaemia?
Coombs test
What does coombs test test for?
Presence of antibodies against circulating RBC which can induce hemolysis
What is characteristic presentation of idiopathic autoimmune disorder?
Isolated thrombocytopenia
What is management of idiopathic immune thrombocytopenia?
Usually watch and wait if fine but oral pred if platelet count is less than 30
In which non hodgkin lymphoma are IgM papaproteinaemia found?
Waldenstorm macroglobulinaemia
Which clotting disorder is assocaited with turner’s?
Haemophilia A
What is first line for primary autoimmune haemolytic anaemia?
Corticosteroids
Why would you get a dry tap from bone marrow?
If patient has primary myelofibrosis as bone marrow has fibrosed
Which UTI drug is common to trigger a haemolytic crisis in G6PD deficiency?
Nitrofurantoin
Which cellular bodies are found in haemolytic crisis in g6pd deficiency?
Heinz bodies
If the question prompt mentions abnormal proliferation of megakaryocytes what should you start thinking?
Myeofibrosis
What can henoch schonlein prupura present with?
After an URTI
Abdo pain
Arthritis
Glumerulonephritis
How can anabolic steroid use affect RBC?
Increase erythropoiesis - causing secondary polycythaemia
What is first line diagnostic test for non hodgkin lymphoma?
Excisional biopsy
What do smudge cells indicate?
Chronic lymphocytic leukaemia
How is aplastic crisis in sickles managed?
Conservatively unless symptomatic anaemia in which case blood transfusion is needed
Who should hydroxycarbamide (hydroxyurea) be given to sickle cell patients?
Two or more crises
Which immune cell accumulate abnormally in multiple myeloma?
Monocytes
