Haem Flashcards
Burkitt’s lymphoma translocation
Burkitt’s lymphoma - t(8:14)
c-myc gene translocation
t(8:14)
Burkitt’s lymphoma
the c-myc gene translocation
EBV virus
chronic myeloid leukaemia translocation
Translocation t(9;22) - philadelphia chromosome
follicular lymphoma translocation
The t(14;18) translocation causes increased BCL-2 transcription
warm AIHA antibody
IgG
cold AIHA antibody
IgM
G6PD deficiency drugs
Some drugs causing haemolysis:
anti-malarials: primaquine
ciprofloxacin
sulph- group drugs: sulphonamides, sulphasalazine, sulfonylureas
The presence of DIC without any underlying cause in a young patient suggests what disease
Acute promyelocytic leukaemia - t(15;17)
stain for sideroblastic anaemia
perl’s
Benign ethnic neutropaenia is common in which ethnic group?
black African and Afro-Caribbean ethnicity
- requires no treatment
commonest inherited thrombophilia
factor v leiden
most common bacteria causing neutropenic sepsis
staph epidermis (from lines)
biochemical findings in tumour lysis syndrome
high potassium
high phospate
low calcium
reducing risk of tumour lysis syndrome
high risk - rasburicase
low risk - allopurinol
treatment of tumour lysis syndrome
- IV fluids
- rasburicase
test for hereditary spherocytosis
EMA test
most reliable screening test for hereditary angioedema
Low C4
What is the single most important factor in determining whether cryoprecipitate should be given?
low fibrinogen
inheritance pattern of hereditary angioedema
autosomal dominant
deficient in patients with hereditary angioedema?
C1-INH deficiency
C4 is the best screening test for hereditary angioedema in between attacks. A result of low C4 would support the diagnosis.
Meig’s syndrome
an ovarian fibroma associated with a pleural effusion and ascites
Woman with bone metastases-, most likely to originate from?
breast ca
Direct antiglobulin test
specific for autoimmune haemolytic anaemia
red cell abnormalities in lead poisoning
including basophilic stippling and clover-leaf morphology
Mantle cell lymphoma translocation
t(11;14) translocation
overexpression of cyclin D1, a protein that regulates cell cycle progression, leading to uncontrolled proliferation of B-cells in the mantle zone of lymph nodes.
reed sternberg cells
hodgkin’s lymphoma
Disproportionate microcytic anaemia -
beta-thalassaemia trait
most common symptom of super vena cava obstruction?
dyspnoea
mx of ITP vs TTP
ITP - steroids, IvIg, splenectomy
TTP - plasma exchange, steroids,
Acute promyelocytic leukaemia presentation
young people
DIC
thrombocytopenia
Acute promyelocytic leukaemia translocation
t(15;17) translocation
what is mesna?
given with cyclophosphamide
to decrease its risk of causing haemorrhagic cystitits
gold standard test for paroxysmal nocturnal haemoglobinuria
Flow cytometry for CD59 and CD55
on what chromosome is alpha haemoglobin coded for?
16
how does heparin work?
Heparins generally act by activating antithrombin III. Unfractionated heparin forms a complex which inhibits thrombin, factors Xa, IXa, XIa and XIIa.
LMWH however Forms a complex that only increases the action of antithrombin III on factor Xa
blood film finding in CLL
blood film: smudge cells (also known as smear cells)
most commonn leukaemia in adults?
CLLp
pathophysiology of TTP
1) abnormally large and sticky multimers of von Willebrand’s factor cause platelets to clump within vessels
2) in TTP there is a deficiency of ADAMTS13 (a metalloprotease enzyme) which breakdowns (‘cleaves’) large multimers of von Willebrand’s factor
Features of TTP
rare, typically adult females
fever
fluctuating neuro signs (microemboli)
microangiopathic haemolytic anaemia
thrombocytopenia
renal failure
porphyria cutanea tarda (PCT) is associated with which virus?
hep C
Systemic mastocytosis features
Systemic mastocytosis results from a neoplastic proliferation of mast cells
Features
urticaria pigmentosa - produces a wheal on rubbing (Darier’s sign)
flushing
abdominal pain
monocytosis on the blood film
Diagnosis
raised serum tryptase levels
urinary histamine
reversal for bleeding on dabigatran?
idarucizumab to reverse
reversal of apixaban or rivaroxaban.
Andexanet alfa is used for factor Xa inhibitors
how to reverse heparin
Protamine is used
what causes haemolytic uraemic syndrome?
E.coli O157: H7 is the strain causing haemolytic uraemic syndrome
management PE/DVT in pregnancy?
LMWH
Ann-Arbour Staging
Stage
I: single lymph node
II: 2 or more lymph nodes/regions on the same side of the diaphragm
III: nodes on both sides of the diaphragm
IV: spread beyond lymph nodes
Each stage may be subdivided into A or B
A = no systemic symptoms other than pruritus
B = weight loss > 10% in last 6 months, fever > 38c, night sweats (poor prognosis
Hodgkin’s lymphoma management
1) chemotherapy is the mainstay of treatment. Two combinations may be used:
- ABVD (doxorubicin, bleomycin, vinblastine, and dacarbazine): considered the standard regime
- BEACOPP (bleomycin, etoposide, doxorubicin, cyclophosphamide, vincristine, procarbazine, and prednisone): alternative regime with better remission rates but higher toxicity
2) radiotherapy
3) combined modality therapy (CMT)
chemotherapy followed by radiotherapy
4) hematopoietic cell transplantation
may be used for relapsed or refractory classic Hodgkin lymphoma
blister cells
G6PD defiency (also bite cells)
Waldenstrom’s macroglobulinaemi features
Features
systemic upset: weight loss, lethargy
hyperviscosity syndrome e.g. visual disturbance
the pentameric configuration of IgM increases serum viscosity
hepatosplenomegaly
lymphadenopathy
cryoglobulinaemia e.g. Raynaud’s
CD markers for hodgkins
Reed-Sternberg cells are classically CD15 and CD30 positive.
low ADAMTS13 level
A low ADAMTS13 level would confirm the diagnosis of TTP.
TRALI is differentiated from TACO by?
hypotension = TRALI
hypertension = TACO
Drug-induced thrombocytopenia
quinine
abciximab
NSAIDs
diuretics: furosemide
antibiotics: penicillins, sulphonamides, rifampicin
anticonvulsants: carbamazepine, valproate
heparin
he development of necrotic skin lesions in this patient after being switched to warfarin
warfarin-induced skin necrosis (WISN). WISN is characteristically associated with Protein C deficiency.
before starting the patient on eculizumab for paroxysmal nocturnal hemoglobinuria, what supportive measure must be given
eculizumab, a monoclonal antibody that inhibits terminal complement activation (C5-C9) which prevents complement-mediated intravascular haemolysis.
C5-9 deficiency predisposes to Neisseria meningitidis infections
give vaccine for Neisseria meningitidis
APML is treated with ?
all-trans retinoic acid (ATRA) to force immature granulocytes into maturation to resolve a blast crisis prior to more definitive chemotherapy.
Dentistry in warfarinised patients -
check INR 72 hours before procedure, proceed if INR < 4.0§
Leukaemoid reaction vs Chronic myeloid leukaemia reaction
Leukaemoid reaction:
high leucocyte alkaline phosphatase score
toxic granulation (Dohle bodies) in the white cells
‘left shift’ of neutrophils i.e. three or fewer segments of the nucleus
Chronic myeloid leukaemia:
low leucocyte alkaline phosphatase score
The most common malignancy associated with acanthosis nigricans
is
gastrointestinal adenocarcinoma
haemophilia types and deficiencies
Haemophilia A is due to a deficiency of factor VIII whilst in haemophilia B (Christmas disease) there is a lack of factor IX
Myelofibrosis - most common presenting symptom -
lethargy
acute vaso-occlusive crisis in sickle cell disease - diagnosis
clinical
Screening for haemochromatosis
general population
transferrin saturation
CLL investigation of choice
immunophenotyping with flow cytometry
with recurrent DVT/PE, when to consider inferior vena caval filters
increasing target INR to 3-4 for long term high-intensity oral anticoagulant therapy
or
switching treatment to LMWH.’
Polycythaemia rubra vera progresses to?
around 5-15% progress to myelofibrosis or AML
Hodgkin’s lymphoma - most common type
nodular sclerosing
mx
Essential thrombocytosis
myeloproliferative disorder
hydroxyurea (hydroxycarbamide) is widely used to reduce the platelet count\
interferon-α is also used in younger patients
low-dose aspirin may be used to reduce the thrombotic risk
inheritance of haemophilia
X-linked recessive condition
CML Indications for treatment
progressive marrow failure: the development or worsening of anaemia and/or thrombocytopenia
massive (>10 cm) or progressive lymphadenopathy
massive (>6 cm) or progressive splenomegaly
progressive lymphocytosis: > 50% increase over 2 months or lymphocyte doubling time < 6 months
systemic symptoms: weight loss > 10% in previous 6 months, fever >38ºC for > 2 weeks, extreme fatigue, night sweats
autoimmune cytopaenias e.g. ITP
basically, if asymptomatic, no need to treat
what kind of autoimmune haemolytic anaemia occurs in with chronic lymphocytic leukaemia
Warm autoimmune haemolytic
Philadelphia translocation, t(9;22) impact on prognosis
Philadelphia translocation, t(9;22) - good prognosis in CML, poor prognosis in AML + ALL
CD Receptor for Epstein-Barr virus
CD 21
differentiating Hodgkin’s lymphoma from non-Hodgkin’s
NonHodgkin’s lymhpoma is more common - extra nodal disease more common
** except in younger females, Hodgkins is more common!
Hodgkin’s lymphoma can experience alcohol-induced pain in the node
‘B’ symptoms typically occur earlier in Hodgkin’s lymphoma and later in non-Hodgkin’s lymphoma
lymphoma in mediastinum is most likely to be which subtype
hodgkins
anti phospholipid syndrome mx
primary thromboprophylaxis
low-dose aspirin
secondary thromboprophylaxis
initial venous thromboembolic events: lifelong warfarin with a target INR of 2-3
recurrent venous thromboembolic events: lifelong warfarin; if occurred whilst taking warfarin then consider adding low-dose aspirin
increase target INR to 3-4
arterial thrombosis should be treated with lifelong warfarin with target INR 2-3
LMWH if planning pregnancy
