Cancer and Bleeding Disorders Flashcards
Which cells are granulocytes (3)
Basophils, eosinophils, neutrophils
Which cells are myeloid cells (5)
Granulocytes (basophils, eosinophils, neutrophils) platelets and RBC
What triad do acute leukaemias cause
Anaemia (↓ Hb): fatigue, pallor, breathlessness
Neutropenia (↓ neutrophils): recurrent infections
Thrombocytopenia (↓ platelets): bleeding and easy bruising
What are the effects of bone marrow failure
Anaemia (↓ Hb): fatigue, pallor, breathlessness
Neutropenia (↓ neutrophils): recurrent infections
Thrombocytopenia (↓ platelets): bleeding and easy bruising
RF AML (4)
Down’s, irradiation, anti-cancer drugs, age (incidence ↑ with age)
Which disease shows Auer rods on a blood film
AML
What is seen on blood film in AML (2)
Auer rods and accumulation of myeloblast cells
What is promyelocytic leukaemia a subtype of
Subtype of AML
What is promyelocytic leukaemia associated with
DIC
What is the most common type of ALL
¾ are B cell ALL
Which cells are particularly deficient in AML
Granulocytes (neutrophils, basophils, eosinophils)
Which cells are particularly deficient in ALL (4)
B lymphocytes (and plasma cells), T lymphocytes and NK cells but most ALL’s are B lymphocyte form
Which is the commonest cancer of childhood
ALL
What does ALL present with
Bone marrow failure symptoms
Organ infiltration:
hepatosplenomegaly, enlarged lymph notes, swollen testes
When does ALL present with a thymus mass
T cell ALL
What are the blood results of ALL (3)
↑↑ WCC, ↓ Hb, ↓ plts
What is seen in the BM film of ALL
BM film: >20% lymphoblasts
What accumulates in CLL
Accumulation of mature incompetent lymphocytes (unable to undergo apoptosis)
What happens in the bone marrow of CLL patients
BM becomes infiltrated by small, nomoformic B cells, meaning patients will start to develop symptoms and signs of BM failure
Symptoms of CLL
Symptoms of BM failure: recurrent infections (including herpes zoster), sx of anaemia, easy bruising/bleeding
Examination of CLL (2)
O/E: enlarged non-tender lymphadenopathy, hepatosplenomegaly
Blood results of CLL (4)
Bloods: ↑ lymphocytes, ↓ Hb, ↓ neutrophils, ↓ platelets
Blood film of CLL
smear/smudge cells
In which disease is smear/smudge cells seen in
CLL
Associations of CLL
AI thrombocytopenia and anaemia (Evan’s syndrome)
What is Evan’s syndrome
AI thrombocytopenia and anaemia
What can CLL become
aggressive NHL = Richter’s syndrome
What happens in CML
Uncontrolled proliferation of granulocyte precursors in BM but slower progression than AML
Symptoms of CML (think about the ways it manifests (3))
Up to 50% are asymptomatic
Hypermetabolic symptoms: weight loss, malaise, sweating
Hyperviscosity symptoms: visual disturbance, headaches, thrombotic event
Gout
Same triad: anaemia, thrombocytopenia, neutropenia
MASSIVE splenomegaly in 90%
Which cancer is the Philadelphia chromosome associated with
CML
Do you see splenomegaly in acute leukaemias and why
Splenomegaly does not have time to form in acute leukaemias
What is seen in the FBC of CML
↑↑ WCC (often >100 x109)
What happens in Hodgkin’s lymphoma
Malignant proliferation of lymphocytes, accumulate in LNs lymphadenopathy
Age of presentation of Hodgkin’s lymphoma
Bimodal age distribution peaks in 20-30 and >50y
What is Hodgkins lymphoma associated with
50% of cases associated with EBV infection
Presentation of Hodgkin’s lymphoma (5)
Painless enlarging mass (most often in neck, occasionally axilla or groin)
May become painful after alcohol ingestion
B symptoms
What are B symptoms (3)
fevers >38, night sweats, weight loss (>10% in last 6 months)
What is seen on examination of Hodgkins lymphoma (3)
non tender firm rubbery lymphadenopathy, splenomegaly +/- hepatomegaly
Ix for Hodgkins lymphoma
Lymph node biopsy under microscopy = Reed Sternberg cells
What type of cell is seen in Hodgkins lymphoma
Reed-Sternberg
What are Reed-Sternberg cells seen in
Hodgkins lymphoma
What is non-Hodgkins lymphoma
Malignancy of lymphoid cells originating in LNs without Reed Sternberg cells
Which cells are involved in non-Hodgkins lymphoma
85% are B cell
15% are T cell or NK cell
Associations of non-Hodgkins’ lymphoma (5)
Associated with EBV, HIV, SLE, Sjogren’s
↑ with age
Age of non-Hogkins lymphoma
Increases with age
Presentation of non-Hodgkins lymphoma
Painless enlarging mass in neck, axilla or groin
Systemic symptoms (less common than in HL): weight loss, night sweats, fever
Organ involvement (more common than in HL): skin rashes, headache hepatosplenomegaly, sore throat, cough
Signs of BM failure: anaemia, neutropenia, thrombocytopenia
Differences between HL and NHL
No Reed-Sternberg cells in NHL
Hodgkins - pain after drinking
NHL is also associated with HIV, SLE and Sjogrens as well as EBV (HL is only EBV)
Systemic symptoms (less common in NHL than in HL):
Organ involvement (more common NHL than in HL)
What is Burkitt’s lymphoma
Subtype of NHL (B cell):
African child
Large LN in the jaw (fast-growing)
Under microscopy: starry sky appearance
What happens in MM
Haematological malignancy characterised by proliferation of plasma cells + production of a monoclonal immunoglobulin (usually IgG or IgA)
MM epidemiology - age and race
> 70 years. Afrocarribeans > Caucasians > Asians
RFs of MM (4)
ionizing radiation, HIV, agricultural work, occupational chemical exposure e.g. benzene, herbicides
S/s symptoms of MM (4)
↑ Ca tired, thirsty, polyuria, nausea, constipation
Renal impairment Ig and its fragments (light chains) deposit in the kidney – present in 20% at diagnosis (associated with worse prognosis)
Anaemia (+ neutropenia, thrombocytopenia) due to marrow infiltration by plasma cells. Also get recurrent bacterial infections due to low levels of other Ig
Bone pain/lesions: increased osteoclast activation due to myeloma cell signaling back/rib pain
What is MGUS
Monoglonal gammopathy of unknown significance:
Pre-malignant condition – accumulation of some monoclonal plasma cells
1% acquire additional mutations MM
Ix for MM (5)
Serum/urine electrophoresis
Bence Jones proteins
Bloods
↑ ESR, ↑CRP, ↑ urea/Cr, ↑ Ca, ALP normal
Blood film: rouleax formation
Serum MONOCLONAL protein >30g/L
BM aspirate = ↑ plasma cells (>10%)
What is seen in the blood film of MM
Rouleaux
What disease is rouleaux seen in in the blood film
MM
What is seen in the bloods of MM (5)
ESR, ↑CRP, ↑ urea/Cr, ↑ Ca, ALP normal
What is pathognomic of MM
Bence Jones serum/urine electrophoresis
What is the difference in bloods between MM and malignancy
In malignancy you get high Ca with HIGH ALP
MM high calcium with NORMAL ALP
Which factor is deficient in Haemophilia A
Factor 8
Which factor is deficient in Haemophilia B
Factor 9
What form of inheritance are haemophilias
X-linked recessive - typically affects boys
When do haemophilias present
Early in life or after surgery trauma
What is a haematoma
painful bleeding into muscles (increased pressure can lead to compartment syndrome or nerve palsies)
What can haematomas lead to
increased pressure can lead to compartment syndrome or nerve palsies
What is a haemarthrosis
after minimal trauma swollen painful joints
S/s of haemophilias
Haemarthrosis Haematomas Haematuria Excessive bruising/bleeding Bruises Joint deformity from haemarthrosis Signs of IDA
Is the APTT checking the intrinsic or extrinsic pathway
Intrinsic
Is the PT checking the intrinsic or extrinsic pathway
Extrinsic
Ix for haemophilias
prolonged APTT (intrinsic pathway); factor assay confirms diagnosis
What does vWF do (3)
vWF is a protein involved in blood clotting:
Forms the bridge between the damaged subendothelium and the platelets (via the GP1b receptor)
Makes platelets bind to each other
Binds to factor 8 and prevents its degradation
What receptor does vWF attach to platelets through
Gp1b
Which factor does vWF bind to and what does it do to it
Binds to factor 8 and prevents its degradation
What are 3 types of vWD and what are the inheritance types
Type 1: reduced levels of normal vWF (AD)
Type 2: defective vWF (AD)
Type 3: complete lack of vWF and highly reduced factor 8 (AR)
Ix for wVD (6)
↑ APTT, ↑ bleeding time, ↓ vWF levels, N plts, normal PT, (↓ factor 8)
Presentation of vWD
More superficial bleeding compared to haemophilias
Bruising, epistaxis, menorrhagia
Increased gum bleeding post tooth extraction
Prolonged bleeding from minor wounds
When does DIC occur (4)
Occurs in sepsis (esp children with meningococcal septicaemia), trauma, obstetric complications, malignancy
What should you be careful of in children with meningococcal septicaemia
DIC
Signs of chronic DIC
signs of deep venous or arterial thrombosis or embolism
Signs of acute DIC (7)
petechiae, purpura, ecchymoses, epistaxis, mucosal bleeding, hemorrhage, respiratory distress
Ix for DIC (6)
FBC (↓ plts, ↓ Hb)
Clotting (↓ fibrinogen, ↑ PT/APTT, ↑ fibrin degradation products)
Peripheral blood film: schistocytes (MAHA)
What is seen in the blood film of DIC
schistocytes
