Haematology/oncology Flashcards
(51 cards)
Hereditary angioedema
Autosomal dominant
Deficiency C1 esterase inhibitor
- treat with C1-INH or fresh frozen plasma
Low C2 and C4
Paroxysmal nocturnal haemoglobinuria
Flow cytometry for Cd59 and CD55
Ham’s test: acid induced haemolysis
Haemolytic of haem cells
- haemolytic anaemia
- May be pancytopenic
- haemoglobinuria : dark coloured urine in mornings
- thrombosis
- May develop aplastic anaemia
Tx blood product replacement, anticoagulant, eculizumab, stem cell transplant
Hodgkin’s lymphoma
Reed Sternberg Cell Ann-Arbor staging 1 single lymph node 2 - 2 or more same side of diaphragm 3 - 2 or more both sides 4 - spread beyond lymp nodes
a - no systemic symptoms other than pruritus
B - wt loss >10% last 6mths, fever > 38, night sweats (poor prognosis)
Imantinib
In CML
Tyrosine kinase inhibitor
CML
Philadelphia Chr >95%
Translocation long arm of Chr 9 and 22 - ABL Chr 9 Porto oncogene
BCR-ABL fusion protein with excess tyrosine kinase activity
- anaemia, wt loss, sweating, splenomegaly
Risk blast transformation - aml 80% all 20%
- Imatinib, hydroxyurea, interferon alpha, allogrnic BM transplant
Cisplatin
Causes cross linking in DNA
SE- ototoxocity, peripheral neuropathy, low Mg
Hydroxyurea
Inhibits ribonucleotide redux tase, decreases DNA synthesis
SE myelosupression
Docetaxel
Prevents microtubule depolymerisation and disassembly, decreasing free tubulin
SE neutropenia
Vincristine/vinblastine
Inhibits formation of microtubules
Vincristine- peripheral neuropathy, paralytic ileum
Vinblastine - myelosupression
Methotrexate
Inhibits dihydrofolate reductase and thymidylate sysnthesis
SE myelosupression, mucositis, liver/lung fibrosis
Flurouracil 5FU
Pyrimidine analoge inducing cell cycle arrest and apoptosis by blocking thymidylate synthase (works during S phase)
SE myelosupression, mucositis, dermatitis
6- mercaptopurine
Purine analogue activates by HGPRTase decreasing purine synthesis
SE myelosupression
Cytarabine
Pyrimidine antagonist
Interferes with DNA synthesis at s phase and inhibits DNA polymerase
SE myelosupression, ataxia
Bleomycin
Degrades preformed DNA
SE lung fibrosis
Doxorubicin
Stabilises DNA topoisomerase II complex
inhibits DNA and RNA synthesis
SE: cardiomyopathy
Cyclophosphamide
Alkylation agent - causes cross linking in DNA
SE haemorrhagic cystitis, myelosupression, transitional cell carcinoma
AML
> 45years
Anaemia, neutropenia, thrombocytopenia, splenomegaly, bone pain
Poor prognostic features: >60yrs, >20% blasts after first course of check, deletions of Chr5 or 7
French- American - British classification
Acute promyelocytic leukaemia
AML
Assoc t(15:17) Fusion PML and RAR-alpha genes
Presents approx 25yrs
Auer Rods
Often DIC or thrombocytopenia at presentation
Good prognosis
G6DP deficiency
X linked recessive
Crisis: ciprofloxacin, fava beans, primaquine
Neonatal jaundice, intravascular haemolysis, gallstones, splenomegaly
- Heinz bodies
CLL
Monoclonal differentiation of B cells
Most common leukaemia in adults
Often asymptomatic, anorexia, wt loss, bleeding, infections, lymphadenopathy
Smudge/smear cells on blood film
Poor prognostic factors: male, >70yrs, lymphocyte >50, CD38, raised LDH, lymphocyte doubling time <12mths, del 17p
Neutropenic sepsis
Gram positive cocci esp staph epidermidis
Neut <0.5
Temp >38 or consistent with clinical sepsis
Consider fungal infections and g-cat
Factor V Leiden
Most common inherited thrombophilia in Europe
Activated protein C resistance. gain of functional mutation
Heterozygous 4-5 VTE risk
Homozygous 10x
Lead poisoning
Abdo pain, peripheral neuropathy, fatigue, constipation, blue lines on gum margin
Microcytic anaemia
Basophilic stippling and clover leaf morphology
Blood level >10 mcg/dl
Urinary coproporphyrin increased
Chelating agents DMSA, EDTA, d-penicillamine
Sideroblastic anaemia
Microcytic
Congential or acquired (alcohol, lead, anti-tb meds’)
*Perl’s stain shows ring sideroblasts
