Non-Hodgkin's lymphoma

Question 1

definition of non-hodgkin’s lymphoma (NHL)

Answer 1

Lymphomas are malignancies of lymphoid cells originating in lymph nodes or other lymphoid tissues.

NHL are a diverse group consisting of 85% B cell, 15% T cell and NK cell forms, ranging from indolent to aggressive disease and referred to as low, intermediate and high grades.

diffuse large B cell lymphoma is commonest

All lymphomas w/o Reed-Sternberg cells

not all focus on lodes - extranodal tissues generating lymphoma include mucosa-associated lymphoid tissue eg gastric MALT

Question 2

classifications of NHL

Answer 2

may be classified according to the Revised European-American Lymphoma (REAL)classification on the basis of clinical, biological and histological criteria.

The WHO Classification, published in 2001 and updated in 2008, is considered the latest classification of lymphoma

Question 3

aetiology of NHL

Answer 3

accumulation of multiple genetic lesions (activation of oncogenes by chromosomal translocations and inactivation of tumour suppressor genes by chromosomal deletions or mutations)

genome in certain lymphoma subtypes has been altered by introduction of foreign genes by oncogenic viruses

radiotherapy

immunosuppressive agents

chemo

HIV - usually high grade lymophoma from EBV transformed cells

HBV

HCV

H Pylori

connective tissue disease - SLE

inherited and acquired immunodeficiency syndromes

congenital

toxins

Question 4

oncogenic viruses involved in aetiology of NHL

Answer 4

EBV has been detected in cases of Burkitt’s lymphoma and of AIDS-associated lymphomas

HTLV-1 has been implicated in adult T-cell lymphoma/leukaemia(ATLL)

HHV-8 infection is detected in body-cavity-based lymphomas.

Question 5

epidemiology of NHL

Answer 5

increase with age

incidence 2:10000

more common male

more common west

Question 6

sx of NHL

Answer 6

Painless enlarging mass: Often in neck, axilla or groin.

systemic symptoms - less than in Hodgkins, indicates disseminated disease:

• fever
• night sweats
• weight loss >10% 6mo
• symptoms of hypercalcaemia

related to organ involvement

extranodal disease is more common that in Hodgkins

• skin rash
• headache
• sore throat
• abdo discomfort
• testicular swelling

pancytopenia from marrow involvement - anaemia, infection, bleeding (reduced platelets)

can effect bone, CNS and lung

establish performance status of the pt

Question 7

signs of NHL

Answer 7

Painless firm rubbery lymphadenopathy: Cervical, axillary or inguinal. Oropharyngeal (Waldeyer’s ring of lymph nodes) involvement.

skin rashes

• mycosis fungoides
• sezary’s syndrome

abdo mass

hepatosplenomegaly

signs of bone marrow involvement:

• anaemia
• infections
• purpura

Question 8

myocosis fungoides

Answer 8

well defined indurated scaly plaque-like lesions with raised ulcerated nodules caused by cutaneous T cell lymphoma

Question 9

signs of sezary syndrome

Answer 9

slowly developing generalised induration - firmness of the skin

lichenification - increased skin markings due to scratching and rubbing

scaly papules due to follicular prominence

thickened nails

ectropion - drooping of the lower eyelid

diffuse or patchy alopecia - hair loss

erythroderma

Question 10

Ix for NHL

Answer 10

blood FBC

• anaemia, neutropenia, thrombocytopenia if BM involved
• UE
• uric acid
• raised ESR, CRP, LDH (high cell turnover = worse prognosis), LFTs (transaminases if liver involvement)
• Ca may be high
• HIV, HBV, HCV serology - in select pts

blood film - lymphoma cells

bone marrow aspiration and biopsy

imaging

• CXR, CT thorax, abdomen, pelvis.
• CT plus PET scanning is of particular value for evaluation of extranodal involvement.

lymph node biopsy - Histopathologic evaluation, immunophenotyping, cytogenetics.

cytology of effusion

LP for CSF cytology of CNS signs

Question 11

staging for NHL

Answer 11

same as for Hodgkin’s

Question 12

extranodal disease of NHL - gut

Answer 12

gastric MALT

non-malt gastric lymphoma

small bowel lymphomas

Question 13

gastric MALT

Answer 13

caused by H pylori

may regress with eradication

sx - as for gastric ca, systemic features

involves antrum, multifocal and mets late

Question 14

non-MALT gastric lymphomas

Answer 14

usually diffuse large cell B lymphomas

high grade and not responding well to H pylori eradication

Question 15

small bowel lymphomas - NHL

Answer 15

eg IPSID - immunoproliferative small intestine disease

EATCL - enteropathy/coeliac-associated intra-epithelial T cell lymphoma. Sx:

• diarrhoea
• vomiting
• abdo pain
• reduced weight

Question 16

extranodal NHL - oropharynx

Answer 16

Waldeyer’s ring lymphoma = sore throat/obstructed breathing