Haem Flashcards
What are the meyloid cells?
Eosinphils, neutrophils and basophils
What is CML?
Proliferation of myeloid cells 15% of leukaemias Rare in childhood Mostly 40-60 years old Slight male predominance more than 80 percent have the philadelphia chromosome
What is the philadelphia chromosome
t(9,22).
Forms fusion gene BCR/ABL in chromosome 22 that has tyrosine kinase. the prognosis is worse without this
Signs of CML
raised WCC, fatigue, tiredness and weight loss, splenomegaly, positive cytogenetics (florescent in situ hybridisation) , philadelphia chromosome
How do u treat CML?
BCR-ABL Tyrosine Kinase Inhibitors
1st line - Imatinib
2nd line (more potent) – dasatinib,
nilotinib, bosutinib.
Name 1st line tyrosine kinase inhibitor?
imatinib
What would u suspect if male patient in 50s presents with fatigue, unexplained wightloss?
check cytogeneics, WCC levels (raised) and treat with imatinib. it is CML
How do u find the philadelphia chromosome?
from cytogenetics (karyotype, FISH or PCR)
What chemo drug is used for CML?
Hydroxycarbimide
When would you do a stem cell transplant in CML?
in children. this is the only cure but presents rare in children.
What is CLL?
chronic lymphobastic leukemia. twice as commonn in females. due to B cells that aggregrate because they’ve escaped apoptosis. most commonest leukemia
what is the most common leuemia?
CLL
what staging is used in CLL?
Rai staging (based on FBCs)
How does CLL present and what are the complications?
sweats, asymptomatic., splenomegaly, hepatomegaly, ENGLARGED RUBBBERY NON TENDER NODES, raised lymphocytes,
What is a common CLL complication
presenting with decreased Hb - indication of autoimmune haemolysis. others:Infection (due to hypogammaglobulinaemia)
Marrow failure (causes anaemia with decreased neutrophils and platlets)
Treatment for CLL?
retuximab for later stages. chemo, BM tranplant, stem cell tranplant, radiotherapy for lymphadenopathy and splenomegaly
What are the lymph nodes like in CLL
englarged, rubbery nontender
What is the prognosis of newly diagnosed CLL?
1/3 never progress
1/3 progress slowly
1/3 progress actively
which leukemia is most common in childhood?
ALL. malignancy of B/T cells
what does ALL have a strong association with?
Down’s syndrome
What is thalasaemia?
autoimmune, hereditary destruction of RBC. leads to haematuria, bone deformities, yellow skin
Which leukemia do you treat with retuximab in later stages
CLL
How do you distinguish between AML and ALL?
AML: in adults, gum hypertrophy
ALL: enlarged lymph nodes, childhoon, bone pain, CNS involvement
What do all leukemia;s have
Bone pain, intfileration, hepatosplenomegaly. lymphoblastic have enlarged lymph nodes
Which leukemia has a higher mortality ?
AML - 10 percent. 60 percent relapse rates. adults. gun hypertrophy.
What investigations do u perform if u suspect acute leukemias?
FBcs, blood film, clotting screen, lumbar puncture
what may you see in clotting screens of acute leukemias?
DIC - disseminated intravascular coagulation
What is DIC?
disseminated intravascular coagulation. too much clotting and too much bleeding at the same time.
generation of fibrin within the blood vessels and also consumption of platelets/coagulation factors causing secondary activation of fibrinolysis.
what is DIC caused by?
Malignancy, septicaemia, obstetric causes, trauma, infections, haemolytic transfusion reactions, liver disease etc
how do you treat DIC?
Treat underlying cause. Maintain blood volume and tissue perfusion.
May need transfusions
Activated protein C
What is thrombocytopenia?
Reduced platelet production in bone marrow
Excessive peripheral destruction of platelets
Problems of in enlarged spleen
What are the clinical features of immune thrombocytopenic purpura?
purpura, increased bruising, epsitaxis, menorrhagia
How do you treat ITP?
CS - prednisolone, splenctomy, IV IG, ANti D
Why do you do a lumbar puncture for ALL?
there is CNS involving symptoms
How do you treat AML and ALL?
Supportive (blood/platelets/fluids)
Treat infection
Chemotherapy (remission induction (regenerate) ➔ consolidation (intensification) ➔ maintenance: steroids
Bone Marrow Transplantation (during 1st remission)
If there is a deficiency in platlet, what symptoms do you get?
Thrombocytopenia (nose/gum bleeds, bruising
What is a lymphoma?
malignancy of mature lymphocytes that arises in the lymphatic system
What is the difference between leukemia and lymphona?
leukemia is a malinancy of the lymphotcyte precursors. Lymphoma is a mal. of mature lymphocytes
Charactaristic feature of Hodgkin’s lypmhoma?
Reed-Sternberg cells
painless non tender, rubbery lymph node enlargment
hepatosplenomelagy
alcohol induced pain?
Symptoms of Hodgkin’s lymphoma?
Fever, weight loss, night sweas, lethargy, pururitis, alcohol induced pain in nodes.
what are RF for HL
being young/elderly
EBV, SLE, post-transplantation, obesity
What is the ann arbor system?
stage 1 - one lymph node
2 - lymph node and lungs
3 - both sides of diaphram and spleen
4 - liver bone and nodules
RF for non hodgkins?
h. pylori, HIV, toxins
What Sx of non-hodgkin’s
GI symptoms - abdo pain, diarrhoea and vomitting and skin involvement as well as fever, weight loss, lymph pain, etc and alcohol induced
tx for non HL?
Chemo and radiotherapy, steroids and rituximab
tx for HL?
chemo and radio
Which lymphoma has a worse prognosis
non hodgkins - no reed sternberg cells
What is myeloma?
Myeloma is a malignant bone tumour due to malignant proliferation of plasma cells (B lymphocytes) accumulating in the bone marrow. renal and bone dysfuncton This leads to overproduction of Ig or an Ig fragment causing dysfunction of many organs (esp kidney). The Ig is seen as a monoclonal band, or paraprotein, on serum or urine electrophoresis.
What is there an overproduction in myeloma?
Ig causing dysfunction to many orgams. seen as a monclonal band or a paraprotioin on a serum or urine electrophoresis
What does the CRAB acronym stand for?
Myeloma symptoms. increase in Ca Renal failure Aenemia Bone lesions
What is a common x-ray finding in myeloma?
pepperpot skull. (bone lesions)
why do you get bone lesions in myeloma?
plasma cells activate osteoclasts. (over proliferation of plasma cells)
Typical presentation of myeloma?
MEn old, back pain, punched out lesions on x-ray and hypercalcaemia. Bence Jones proteins in urine
How would you investigate myeloma?
Bloods - calcaemia
CXR
renal output reduced?
What are the complications of myeloma?
AKI
Hypercalcaemia - fluids
spinalcord compression? - dexamethasom
hyperviscocity? - plasmapheresis
what treatments are available for myeloma?
supportive - analgesia and bisphosponates for bone pain
transfusion/EPO for anaemia
regular IV immunoglobulin/anx for infections
chemotherapy
What is microcytic anaemia?
Iron deficiency
Anaemia or chronic disease
reduced Hb and reduced MCV
thalassaemia
What is Macrocytic anaemia?
B12 deficiency, metabolic diseases or alvohol or liver disease
what does B12 deficienty cause?
reduced RBC formation. reduction in reticulocyes
what is pernicious anaemia?
autimmune attack on parietal cells so decreased IF produced and B12 absorption decreased RBC formation.
Sickle cell anaemia RBC count?
raised. sick cells last 10-20 days. normal last 120 days. raised reticulocute count to kee up with degradation
When would ferritin levels increase?
as a response to imflammation and malignancy
Where is B12 absorbed?
TERMINAL ILEUM BOUND TO INTRINSIC FACTOR
Cause of iron deficiency>?
Hookworm.
Breastfeeding.
Bleeding.
Poor diet.
What is someone with anaemia more prone to?
high altitude sickness
What would RBC look like in iron deficiency anaemia?
hypochromic microcytic
Why would methotrexate cause folate deficiency?
It is a folate antagonist.
what is usually the treatment for rheumatoid arthiritis?
Methotrexate and folate acid
What does methotrexate inhibit
dihydrofolate reductase which converts folic acid to tetrahydrofolate (FH4).
What is prescribed with methotrexate to prevent folate deficiency ?
folate supplements
What is a disadvantage of using NOACS?
there is no reversing agent. vit k can be given to reverse warfarin
what drug contains vit k?
Beriplex (contains vitamin K dependent coagulation factors)
What is the order of hemostasis?
blood vessel spasm, platelet plug formation, blood coagulation
What is MCV?
Mean corpuscular volume (small RBCs on the blood film). term-
microcytic
What is MCHC?
Mean corpuscular hemoglobin concentration (pale RBCs on the film). term - hypochromatic
What anaemia is MCV <80?
microcytic - result of Fe definiceny and thalassaemia or chonic disease eg CKD
What anaemia is MCV >100
Macrocytic - alcohol and liver disease
What anaemia is 80-100 MCV
Normicytic
What are symptoms of iroon definiciency?
Angular stomatitis (rash around mouth) Atrophic glossitis (enlarged tongue) Brittle hair and nails - koilonychia
What is absolute polycythaemia
too many RBCs due to more EPO produced
What is apparent polycythaemia?
not enough plasma volume in the blood. same blood cell count. so increased blood viscocity
Which polycythaemia occurs as a secondary result of connection with respiratory/ circulatory disease or cancer.
Absolute polycythaemia.
Causes of apparent polyctyhaemia?
Being overweight, smoking, alcohol and diuretics.
Dehydration,
Stress: Gaisbock syndrome
What does the JACK2 mutation cause?
increased sensitivity of bone marrow cells to cause EPO to increase RBC production
causes of absolute polycythaemia?
Chronic hypoxia (COPD and sleep apnoea)
- Poor oxygen delivery (e.g. high altitude)
- Abnormal RBC structure
- Tumours releasing high levels of EPO
- Problem with the kidneys eg kidney tumour or narrowing of the arteries supplying blood to the kidneys.
Presentation of polycythaemia?
asymp/easy bleeding bruising fatigue, itchy skin from histamine.
signs of polycythaemia?
splenomegaly, HTN,red hands and feets, thrombocytosis with raised Hb
What are you at risk of with polycythaemia?
BLood clots, Mis, PEs, DVTs, Stroke.
Diagnosis for polycythaemia?
EPO serum level, FBC/film: raised haemocrit and neutrophils, platelets and basophils.
Treatment for Polycythaemia vera?
venesection, hydrocardamide, interferon
Types of polycythaemia?
Absolute, apparent, polycythaemia vera
Polycythaemia - Diagnosis, presentation, pathophys, treatment?
DX - raised EPO, Raised neutrophils, basophils, platelets, JACK2 gene testing
Px - easy bruising, bleeding, itching from histamine,
Path - genetic rare causes an increase in RBC due to increased EPO due to JACK2 mutation.
Tx - venesection, low dose aspirin to reduce blood clots, rituximab
What is pancytopenia?
Reduction in all three of: RBCs, WBCs and platelets.
diagnosed by leukopenia, thrombocytopenia, anaemia. treat cause.
caused by chemo/abx/ infective causes/cancer/folate or b12 deficiency/ SLE
AML summary ?
Adults 50-70, cancer of myleloid precursor. present with anaemia, weight loss, infections, bleeding, bruising.
ix - increase WCC and reduced platelets, neutrophils, Hb.
GUM HYPERATROPHY
AUER RODS on staining
treated with pallitive chemoo
ALL summary?
affects children. lymphoid precursors. increased WCC, decreased Hb and platelets.
Px - bone pain, marrow failure, CNS INVOLVEMENT, infectoion, bruising and bleeding
blood film - LARGE BLAST CELLS
DOWN SYNDROM LINK
treat with pallitive chemo
CML summary?
adults. myloid cells. philadelphia chromosom (9,22). increasedWCC and INCREASED/variable platelets, low Hb or normal
Bone aspirate - increased cells.
TREAT WITH IMATINAB. - tyrosine kinase inhibitor.
Sx - weight loss, fever, anaemia, night sweats
HEPATOSPLENOMEGALY AND LYMPHADENOPATHY
BLOOD FILM - increased immature cells and basophilia
CLL summary?
affects children, B cells. increased WCC and reduced platelets.
sx- weight loss, anaemia, fever, night sweats.
ENLARGED RUBBERY NON-TENDER LYMPH NODES
BONE ASP - INREASED LYMH
TX - PREDNISOLONE AND CHEMO/ RITUXIMAB, stem cell transplant
most common.
SMUDGE CELLS
complications - infection, bleeding, autoimmune haemolysis
prognosis - 1/3 never progress/progress/progress slowly
Chemo options?
methotrexate and hydroxycarbimide
what is rituximab?
CD20 monoclonal antibody/ anti-CD20
Myleoma summary
CRAB, pepper pot skull
tx - IVIG, EPo transfusion
IX - raised esr, ca, rolaux, raised creatinie, bence jones protein,
complications - hypercalcaemia, spinal cord compression (MRI and dexamethason), AKI
Hodgkin’s lymphoma
malignancy of mature lymphocytes that arises in the lymphatic system.
Rubbery, non-tender, swollen lymph nodes - painful when drinking, B symptoms. reed sternberg
ix - BFilm, LN biopsy, FISH, BM biopsy,
causes - h. pyrlori, transplant, autimmune,
ANn arbor
tx - chemo. meth and hydrox.
what are the B symptoms
weight loss, pruritus, night sweats, fever. B symptoms imply poor prognosis.
Non-hodgkins
paraprotein present, h pylori, , BM tranplant and radiotherapy. lymphoadenopathy
Hyperviscocity syndrome
Malignant plasma cells churn out lots of immunoglobulin which increases viscosity of the blood.
The bigger the Ig, the less you need to get the syndrome:
- IgM > IgA > IgG
causes - myleoma, leukemia, thrombocytosis, sickle cell
sx - mucosal bleeding, ataxia, confusion, visual changes, papillodeama, peripheral oedema,
Rissk - congestive failure, IHD, pulmonary oedema with multiple organ failure
Malaria relation to haematology?
The plasmodium protozoa are injected by female mozzies and multiply in RBCs.
This causes haemolysis, RBC sequestration and cytokine release. Can get thrombocytopaenia.
what trait makes RBC lifespan shorter and protects from plasmidium protazoa?
sickle cell trait
What is the correct mechanism of action for the anti-emetic drug ondansetron?
5HT3 antagonist eg ondansetron.Centrally acting on the chemo receptor trigger zone in the brain. Pro-kinetics for gastrostasis. Used in chemo
approach to the management of a patient with suspected febrile neutropaenia
Perform cultures and start broad spectrum IV Abx
What complication of CLL had decreased Hb and raised WCC and normal platelets?
Autoimmune haemolysis
