Cancer + bleeding disorders Flashcards
Chronic myeloid leukaemia summary card
Uncontrolled proliferation of granulocyte precursors in BM but slower than AML caused by Philadelphia chromsome in 80% pts (t(9,22) - BCR-ABL fusion gene)
- asymptomatic, may present with gout, hyperviscosity, (headache, visual disturbance, thrombotic event), hypermetabolic (wt loss, sweaty, malaise), BM failure
- increased WCC (>100x1o^9)
- massive splenomegaly in 90% cases
Acute myeloid leukaemia summary card
Blast cells do not go on to form promyelocyte -> myelocyte -> neutrophils etc
- present with bone failure
- accumulation of myeloblast cells (>20%) on blood film; AUER RODS present
- RFs: irradiation, Downs’, anti-cancer drugs, increased incidence with age
- promyelotic, aggressive subtype due to t(15;17), associated with DIC
Chronic lymphocytic leukaemia summary card
Accumulation of mature incompetent lymphocytes as they are unable to undergo apoptosis
- associated with autoimmune thrombocytopenia + anaemia = Evan’s syndrome
- asymptomatic largerly, may present with bone marrow failure, enlarged non-tender lymphadenopathy, hepatosplenomegaly
- Blood film shows SMEAR/SMUDGE cells (cells are so fragile)
Acute lymphoblastic leukaemia summary card
Uncontrolled proliferation of lymphoblasts, most commonest cancer of childhood (75% under 6yo)
- 75% are B cell ALL
- present with bone failure (low Hb + plts but high WCC)
- T cell enlargers thymus leading to mediastinal compression thus wheeze
- hepatosplenomegaly, enlarged lymph nodes, swollen testes
What do you see in bone marrow failure?
Anaemia - fatigue, pallor, SOB Neutropenia - recurrent infections Thrombocytopenia - bleeding and easy bruising
If the pt has the following, which leukaemia do they have?
a) Auer rods on blood film
b) Smear cells on blood film
c) Child with easy bruising, splenomegaly and a wheeze
d) t(15;17) mutation
e) t(9;22) mutation
a) AML
b) CLL
c) ALL
d) AML myelotic subtype
e) CML
Buzzword differences between the lymphomas
Hodgkin's - Reed Sternberg cells (Owl's eyes) - painful after alcohol ingestion Non-Hodgkin's - HIV, SLE, Sjorgen's association (EBV assoc. w/ both HL and NHL) Burkitt's (subtype of NHL, B cell) - African child - starry sky appearance under microscope
Hodgkin’s lymphoma summary card
Malignant proliferation of lymphocytes occuring in 20-30 or >50 yo (bimodal)
- association with EBV (50% cases)
- painless enlarging mass in neck/axilla/groin, becomes painful after alcohol
- non-tender firm rubbery lymphadenopathy, fever, night sweats, wt loss, +/- hepatosplenomegaly
- Lymph node biopsy with Ann Arbour staging shows Reed Sternberg cells (Owl’s eyes)
Non-Hodgkin’s lymphoma summary card
Malignancy of lymphoid cells in lymph nodes WITHOUT Reed-Sternberg cells
- 85% are B cells, 15% are T/NK cells
- associated with EBV, HIV, SLE, Sjorgen’s syndrome
- increased incidence with age
- painless, enlarging mass in neck/axilla/groin, bone marrow failure, hepatosplenomegaly, sore throat, headache, shin rashes (more common to have organ involvement than B sx)
- lymph node biospy and Ann Arbour staging
Burkitt’s summary card
Subtype of NHL B cell
- occurs in large lymph node in the jaw, fast-growing
- buzzword: African child
- under microscope a starry sky appearance is seen
What is multiple myeloma?
Haematological malignancy characterised by proliferation of plasma cells and production of monoclonal immunoglobulin (IgG/IgA)
What results in multiple myeloma?
Unknown cause, potential viral trigger
RFs: ionising radiation, HIV, agricultural work, occupational chemical exposure (benzene, herbicides)
How may multiple myeloma present and why?
> 70yo from Afro-Carribean > Caucausian > Asian
C alcium
- tired, thirsty, polyuria, nausea, constipation
R enal impairment
- Ig deposits in kidney, present in 20% cases
A naemia
- neutropenia, thrombocytopenia as BM infiltrated by plasma cells
B one pain/lesions
- increased osteoclast activation due to myeloma cell signalling in the back/ribs
What is MGUS?
Monoglonal gammopathy of unknown significance
- pre-malignant condition
- accumulation of some monoclonal plasma cells
- absent CRAB features
Ix for multiple myeloma
Bloods => increased ESR, CRP, urea/Cr, calcium, normal ALP Blood film => rouleax formation Serum/urine electrophoresis => Bence Jone proteins* Serum monoclonal protein => >30g/L BM aspirate => increased plasma cella (>10%)
Compare the haemophilias
X-linked genetic disorder
Haemophilia A = FVIII (more common)
Haemophilia B = FIX
Both lead to a prolonged APTT and factor assays confirm the diagnosis
How do haemophilias present?
Presents in children after surgery/trauma
- excessive bleeding/bruising
- haematuria, haemarthrosis, haematomas (increased risk of compartment syndrome/nerve palsies)
- O/E signs of IDA, joint deformity, pallor
What are the types of von Willebrand disease?
Type 1 (AD): reduced levels of normal vWF Type 2 (AD): defective vWF Type 3 (AR): complete lack of vWF and decreased FVIII - most severed; bleeding into joints + soft tissues
How may vWD present?
More superficial bleeding compared to haemophiliacs
Bruising, epistaxis, menorrhagia
Increased gum bleeding post tooth extraction
Prolonged bleeding from minor wounds
Ix for vWD
APTT = increased PT = normal Bleeding time = increased vWF levels = low platelets = normal FVIII = low
What is DIC?
Disseminated intravascular coagulation is when there is a systemic activation of coagulation, which leads to:
1) intravascular deposition of fibrin => thrombosis of small and mid-size vessels with organic failure (ischaemic organ damage)
2) depletion of platelets and coagulation factors => bleeding
What type of anaemia is caused by DIC and how?
Microangiopathic haemolytic anaemia (MAHA)
- the fibrin deposits in mircocirculation cause RBCs to flow against pressure thus they become fragmented
- peripheral blood film would show schistocytes
How might DIC present?
Acute, onset => sepsis, trauma, obstetric complication, malignancy
Pt is severely unwell with underlying disease sx and evidence of bleeding:
a) ACUTE => petechiae, purpura, ecchymores, epistaxis, mucosal bleeding, haemorrhage, respiratory distress
b) CHRONIC => deep venous/arterial thrombosis or embolism
Ix for DIC show
FBC = low platelets, low Hb
Clotting = low fibrinogen, increased PT/APTT, increased fibrin degredation products
Peripheral blood film = schistocytes (MAHA)
A 5 year old boy of Indian ethnic origin presented with lymphadenopathy and a mediastinal mass on CXR. WBC: 180 x 109/L Hb: 93 g/L Plts: 43 x 109/L Blood film shows blast cells
What is the most likely cause of the mediastinal mass?
a) Thymoma
b) Acute myeloid leukaemia
c) Acute lymphoblastic leukaemia
d) Haemorrhage into the mediastinum
e) Pneumonia with leukaemoid reaction
c) Acute lymphoblastic leukaemia
An 83 year old man with no abnormal physical findings is found to have a high white cell count and high lymphocyte count on a blood test.
A blood film is requested and it is found to have smear cells.
What is the most likely diagnosis?
a) Acute lymphoblastic leukaemia
b) Chronic lymphocytic leukaemia
c) HIV infection
d) Infectious mononucleosis
e) Whooping cough
b) Chronic lymphocytic leukaemia
A 1 year old boy presented to A&E department with a swollen right elbow following minor trauma. On examination and radiology there was no evidence of bony injury. He was sent home.
3 days later he was brought back with increased pain and swelling. Joint aspiration yielded haemorrhagic fluid.
A coagulation screen was performed which showed a normal PT and a prolonged APTT of 96 seconds (NR 24-35s). The prolonged APTT was corrected by mixing the infant’s plasma with normal plasma.
What is the most likely diagnosis?
a) Disseminated intravascular coagulation
b) Von Willebrand’s disease
c) Haemophilia
d) Autoimmune thrombocytopenia
e) Fracture of the elbow
c) Haemophilia
A 16 year old boy presents to his GP complaining of nosebleeds and bleeding after brushing his teeth. He is unsure of how long it has been going on for but decided to seek advice after having to continually excuse himself from lessons. On examination you notice he has some skin bruises. A blood test shows a prolonged bleeding time and APTT. Platelet count and PT are normal.
What is the most likely diagnosis?
a) Von Willebrand disease
b) Liver disease
c) Disseminated intravascular coagulation
d) Congenital afribrinogenaemia
e) Haemophilia
a) von Willebrand disease
