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Mrcp > Haematology/oncology > Flashcards

Haematology/oncology Flashcards

1
Q

Hereditary angioedema

A

Autosomal dominant

Deficiency C1 esterase inhibitor
- treat with C1-INH or fresh frozen plasma

Low C2 and C4

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2
Q

Paroxysmal nocturnal haemoglobinuria

A

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

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3
Q

Hodgkin’s lymphoma

A 
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)

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4
Q

Imantinib

A

In CML

Tyrosine kinase inhibitor

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5
Q

CML

A

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
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6
Q

Cisplatin

A

Causes cross linking in DNA

SE- ototoxocity, peripheral neuropathy, low Mg

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7
Q

Hydroxyurea

A

Inhibits ribonucleotide redux tase, decreases DNA synthesis

SE myelosupression

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8
Q

Docetaxel

A

Prevents microtubule depolymerisation and disassembly, decreasing free tubulin

SE neutropenia

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9
Q

Vincristine/vinblastine

A

Inhibits formation of microtubules

Vincristine- peripheral neuropathy, paralytic ileum

Vinblastine - myelosupression

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10
Q

Methotrexate

A

Inhibits dihydrofolate reductase and thymidylate sysnthesis

SE myelosupression, mucositis, liver/lung fibrosis

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11
Q

Flurouracil 5FU

A

Pyrimidine analoge inducing cell cycle arrest and apoptosis by blocking thymidylate synthase (works during S phase)

SE myelosupression, mucositis, dermatitis

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12
Q

6- mercaptopurine

A

Purine analogue activates by HGPRTase decreasing purine synthesis

SE myelosupression

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13
Q

Cytarabine

A

Pyrimidine antagonist
Interferes with DNA synthesis at s phase and inhibits DNA polymerase

SE myelosupression, ataxia

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14
Q

Bleomycin

A

Degrades preformed DNA

SE lung fibrosis

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15
Q

Doxorubicin

A

Stabilises DNA topoisomerase II complex
inhibits DNA and RNA synthesis

SE: cardiomyopathy

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16
Q

Cyclophosphamide

A

Alkylation agent - causes cross linking in DNA

SE haemorrhagic cystitis, myelosupression, transitional cell carcinoma

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17
Q

AML

A

> 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

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18
Q

Acute promyelocytic leukaemia

A

AML

Assoc t(15:17)
Fusion PML and RAR-alpha genes

Presents approx 25yrs
Auer Rods
Often DIC or thrombocytopenia at presentation
Good prognosis

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19
Q

G6DP deficiency

A

X linked recessive

Crisis: ciprofloxacin, fava beans, primaquine

Neonatal jaundice, intravascular haemolysis, gallstones, splenomegaly

  • Heinz bodies
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20
Q

CLL

A

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

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21
Q

Neutropenic sepsis

A

Gram positive cocci esp staph epidermidis

Neut <0.5
Temp >38 or consistent with clinical sepsis

Consider fungal infections and g-cat

22
Q

Factor V Leiden

A

Most common inherited thrombophilia in Europe

Activated protein C resistance. gain of functional mutation

Heterozygous 4-5 VTE risk
Homozygous 10x

23
Q

Lead poisoning

A

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

24
Q

Sideroblastic anaemia

A

Microcytic

Congential or acquired (alcohol, lead, anti-tb meds’)

*Perl’s stain shows ring sideroblasts

25
Q

Leukaemia reaction

A

Presence of immature cells

Causes: severe infection or haemolysis, massive haemorrhage, metastatic cancer with BM infiltration

High leukocyte alkaline phosphates score
Dohle bodies in white cells
Left shift of neutrophils ie three or less segments of nucleus

26
Q

Von Willebrand’s disease

A

Usually autodominant inheritance

Epistaxis and menorrhagia common
Prolonged bleeding time, APTT prolonged, reduced factor VIII

Type 1 partial reduction vWF, type 2 abnormal form, type 3 total lack (autosomal recessive)

Tx: tranexamic acjd, desmopressin, factor VIII concentrate

27
Q

Warm autoimmune haemolytic anaemia

A

AI disease eg SLE
Neoplasm eg CLL, lymphoma
Drugs eg methyldola

Mx steroids, immunosuppressive, splenectomy

28
Q

Cold AI haemolytic anaemia

A

Usually IgM at 4degrees

Neoplasia eg lymphoma
Infections eg EBV mycoplasma

29
Q

Meng’s syndrome

A

Ovarian fibroma
Pleural effusion
Ascites

30
Q

Multiple myeloma

A

Bone disease
Hypercalcemia (osteoclasts bone resorption)
Renal failure

Major and minor criteria

Bence Jones proteins
Rain drop skull

31
Q

Acute intermittent pophyria

A

Deficiency porphobilinogen deaminase

Urine red on standing
Females 20-40
HTN and tachy

Autosomal dominant

32
Q

Pophyria cutanea tarda

A

Defect uroporphyrinogen decarboxylase

Photosensitive rash with Bullae

Caused by hepatic damage

Urine pink fluorescent under Wood’s lamp

Mx chloroquine

33
Q

Variegated porphyria

A

Autosomal dominant
Defect in protoporphyrinogen oxidase

Phoyosensitive blistering rash

South Africans

34
Q

Pernicious anaemia

A

B12 deficiency
Macrocyclic anaemia
Anti Gastric parietal cell abs 90%
Anti intrinsic abs 50% (more specific)

35
Q

Autoimmune haemolytic anaemia

A

Positive direct antiglobulin test (Coombs)

36
Q

IDA

A

Koilonychia
Atrophic glossitis
Post cricoid webs
Angular stomatitis

Pencil poikilocytes
Target cells

37
Q

Aniline dye

A

Bladder TCC

38
Q

Aflatoxin

A

Liver HCC

39
Q

Asbestos

A

Mesothelioma and bronchial Ca

40
Q

Nitrosamines

A

Oesophageal and gastric Ca

41
Q

Vinyl Chloride

A

Hepatic angiosarcoma

42
Q

Burkitt’s lymphoma

A

MYC oncogene translocated to immunoglobulin gene

T(8;14)

43
Q

Mantle cell lymphoma

A

T(11;14)

Deregulation of cyclin D1 (BCL-1) gene

44
Q

Cryoglobinaemia

A

Low complement

High ESR

45
Q

Thrombocytosis

A

JAK2 mutation 50%
CALR
MPL

Mx hydroxyurea, interferon alpha in younger pts

46
Q

T9:22

A

CML abl/bcr

47
Q

T15:17

A

Acute pro myelocytic leukaemia

48
Q

T8:14

A

Burkitt’s lymphoma

8 c-myc oncogene

49
Q

T14:18

A

Follicular lymphoma
14 Ig heavy constant region
18 Bcl2 anti apoptosis gene

50
Q

T11:14

A

Mantle cell lymphoma

11 cyclin D oncogene

51
Q

Cervical Ca

A

HPV 16.18.33
16-> e6 which inhibits p53
17 -> e7 inhibits RB suppressor gene

SCC 80%
Adenocarcinoma 20%

Mrcp (22 decks)

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