Leukemia and Lymphoma

Question 1

Definition of febrile neutropenia

Answer 1

WCC<2 or neutrophils<1

Question 2

Mx of febrile neutropenia (3)

Answer 2

low risk: ceftriaxone+blood cultures

medium: piptaz+gent+cultures

septic shock: vancomycin+meropenem+gent

Question 3

RFs for ALL (2)

Answer 3

down’s

radiation

(does not run in families)

Question 4

Features of ALL (4)

Answer 4

CNS involvement common

period before Dx consists of 2-4 wks of:

• lymphadenopathy
• anaemia, bleeding, infection
• orchidomegaly

Question 5

Classification of ALL (3)

Answer 5

morphological: L1/2/3

surface antigens:

• B-cell precursor: most common
• B Cell (B-lymphoblastic)
• T cell (T-lymphoblastic)

chromosomal analysis:
-philadelphia=poor prognosis

Question 6

Ix for ALL (8)

Answer 6

Raised WCC: lymphoblasts

CXR/CT: lymphadenopathy, lytic bone lesions and pneumonia

LP for CNS involvement:
-raised WCC w. blast forms

low platelets

normocytic anaemia

raised urate and LDH

marrow biopsy: 50-90% of nucleated cells are blasts

Common translocations:

• TEL-AML: 12-21
• philadelphia: 9-22

Question 7

Mx of ALL (6)

Answer 7

supportive: bloods/platelets, fluids, allopurinol

insert SC port/hickman line for IV access

chemo:

• induction: 3 drug induction+intrathecal methotrexate to induce remission
• consolidation and CNS prophylaxis: cranial irradiation if known CNS disease
• maintenance: daily mercaptopurine+weekly intrathecal methotrexate+vincristine/steroids. 3 months of intrathecal drugs. co-trimoxazole prophylaxis
• imatinib if phil. +ve

Question 8

Poor prognostic factors for ALL (4)

Answer 8

male

philadelphia Cr

CNS involvement

<1yr/>10yrs

Question 9

Assoc. of AML (6)

Answer 9

Fanconi anaemia

Down’s

Edward’s

Li-Fraumeni

NF1

Osler-Weber-Rendu

Question 10

RFs for AML (6)

Answer 10

chromosomal abnormalities-Down’s

radiation/chemicals (benzene)

chemotherapy

myelodysplastic syndromes

myeloproliferative syndromes

CML

Question 11

Features and presentation of acute promyelocytic anaemia (APML) (4)

Answer 11

pml-RARA fusion protein

DIC most common

gum hypertrophy

hepatosplenomegaly

Question 12

Ix for AML

Answer 12

marrow biopsy: auer rods

Question 13

Rx of AML (2)

Answer 13

all-trans-retinoic-acid (ATRA) for APML: poorer prognosis than AML

allogenic marrow transplant improves survival; may be offered if suitable donor found

Question 14

Presentation of lymphoma (4)

Answer 14

lymphadenopathy

mediastinal mass: SVC obs

Long Hx

B Sx rare

Question 15

Peak incidence and characteristic features of HL (2)

Answer 15

young and elderly

characterised by Reed-Sternberg cells

Question 16

RFs for HL (5)

Answer 16

sibling affected

EBV

SLE

transplantation

obesity

Question 17

Presentation of HL (3)

Answer 17

painless, enlarged lymph nodes: may fluctuate in size. alcohol may bring pain

B-Sx

Guillan-Barre

Question 18

Ix for HL (2)

Answer 18

LN biopsy (not FNA):

• nodular sclerosing: most common
• lymphocyte predominant: best prognosis
• lymphocyte depleted: worst prognosis
• mixed cellularity

Bloods:

• FBC
• ESR
• raised LDH
• raised urate and Ca

Question 19

Staging of HL (4)

Answer 19

I: confined to single LN
II: two or more LNs on same side of diaphragm
III: two or more LNs on opposite sides of diaphragm
IV: extra-nodal spread

for each stage:
-A/B depending on B-Sx

Question 20

Mx and prognosis of HL (3)

Answer 20

RT+short course chemo

for relapses:
-high dose chemo+autologous SC transplant

prognosis better than NHL

Question 21

RFs for NHL (4)

Answer 21

chronic immune stimulation:

• HIV
• H-pylori
• transplantation
• HTLV-1

environmental carcinogens

congenital

radiation

Question 22

Presentation of NHL (5)

Answer 22

75% w. nodal disease

25% w. extra-nodal disease:

• skin involvement e.g. sezary syndrome
• oropharynx
• gut: gastric MALT(h. pylori), non-MALT gastric lymphomas, small bowel.

B Sx more rare than in HL

Question 23

Ix for NHL (5)

Answer 23

Bloods: FBC, LFT, U+Es, LDH (raised LDH=poor prognosis)

LN/marrow biopsy for classification

XR/CT chest/abdo/pelvis for staging

cytology of any effusions

LP if CNS signs

Question 24

Classification of NHL (2)

Answer 24

low/high grade

Question 25

Features of low-grade NHL (4)

Answer 25

Follicular (18:14; B cell)

marginal zone lymphoma/MALT

lymphocytic lymphoma

lymphoplasmocytoid lymphoma (Waldenstrom’s)

Question 26

Features of high-grade NHL (4)

Answer 26

Diffuse large B cell: most common

lymphoblastic lymphoma

Burkitt’s: (8:14, IgH-Myc) assoc. w. EBV

T cell lymphomas

Question 27

Rx and criteria for Rx of low-grade NHL (5)

Answer 27

Rx if:

• LNs causing blockage/pain
• marrow failure
• B Sx

localised radiotherapy can cure stage I/II

Chemo

Question 28

Rx of high-grad NHL

Answer 28

R-CHOP