HL and NHL

Question 1

What is hodgkins lymphoma?

Answer 1

Lymphomas are neoplasms of lymphoid cells arising in the lymph nodes or other lymph tissues. Hodgkins lymphoma (15%) is characterized by the presence of Reed Steinberg cells (a B cell lineage cell type). And T cell dysfunction

Question 2

What is the aetiology of hodgkins lymphoma?

Answer 2

Unknown. Likely to be linked to EBV infection (50% of HL are found to have EBV genome). Environmental trigger in genetic susceptibility is likely.

Question 3

What is the epidemiology of HL?

Answer 3

Bimodal age distribution> first peak 20-30, second >50. More common in males.

Question 4

What would you find in the history in HL?

Answer 4

Painless enlarging mass in lymph node (neck, axila or groin)

B symptoms: fever >38, weiht loss >10% BW in 6months. Night sweats.

Other: pruritus, cough/dyspnea if intrathoracic disease.

Question 5

What would you find in the exam of HL?

Answer 5

Non tender rubbery lymphadenopathy: cervical, axillary or inguinal.

Splenomegaly and sometimes hepatomegaly, Skin excoriations

Signs of intrathoracic disease (pleural effusion, SVC obstruction)

Question 6

What investigations would you use for HL?

Answer 6

Blood: FBC (anaemia of chronic disease with low MCV, leukocytosis, high neutrophils, high eosinophils, Lymphopaemia with advanced disease. High ESR, CRP). LFT (high LDH, and transaminases may be high.)

REED STERNBERG cells: pale cytoplasm, bilobulated nuclei, OWL EYE eosinophilic nucleoli.

Bone marrow biopsy (involvement only seen in late disease)

Lymph node biopsy

CXR/AXR/PXR etc. Gallium and PET scan

Question 7

What is the Ann Arbour classification?

Answer 7

1. Unilateral single node
2. Unilateral plural node
3. Bulateral nodes
4. Extranodal (ie liver/BM)
5. Absence
6. Presence of B signs
7. Localised extranodal extension
8. Spleen involvement

Question 8

What is the histological subtypes of HL?

Answer 8

· Nodular sclerolising (70%)

· Mixed type (20%)

· Lymphocyte predominant (5%)

· Lymphocyte deprived (5%)

Question 9

What is the management of HL?

Answer 9

Stages I/IIa: radiotherapy (mantle field, Y irradiation) +/- Adjuvant therapy

Stages III/IVAL Cyclical chaemotherapy (ABVD) Adriamycin, Bleomycin, Vincristine, Decarbazine). +/- adjuvant.

Stem cell transplantation if relapsing.

Question 10

What are the complications of HL?

Answer 10

Secondary malignancy after irradiation: AML (1%), NHlymphoma, solid tumors

Inverted Y: infertility, premature menopause and skin cancer

Mantle irradiation: thyroid, cardiac issues, skin cancer, pulmonary fibrosis.

Question 11

What is the prognosis of HL?

Answer 11

Stages I/II: 90% cure

Stages III/IV: 70% cure.

Prognosis less good with B symptoms, older age or lymphocyte depleted type.

Question 12

What is the definition of Non Hodgkins Lymphoma?

Answer 12

Lymphomas are malignancies arising from lymphoid cels in lymph nodes or other lymphoid tissues. Non Hodgkin lymphomas may be divided into B cell derived (85%) T cell (15%) and NK cells (few).

Range between low, intermediate and high grades.

Question 13

What causes NHL?

Answer 13

Complex process involving the accumulation of multiple genetic lesions (activation of oncogenes/deactivation of TSGs).

Genome in certain cases can be altered by viral gene insertion (ie EBV in Burkitts, AIDS associated. HTLV1 in T cell lymphoma, and HHV8 in body cavity based lymphomas

Question 14

What are the RFs for NHL?

Answer 14

· CMV, EBV (Burkitt), HTLV1 (adult T cell), H Pylori (MALT lymphoma)

· Radiotherapy

· Immunosuppression, HIV infection

· Connective tissue disease ie.SLE

· Inherited/acquired immunodeficiency syndromes

Question 15

What is the epidemiology of NHL?

Answer 15

Incidence increases with age. More common in males.

Question 16

What is in the history of someone with NHL?

Answer 16

Painless enlarging mass

Systemic symptoms less common than HL. (weight loss, nightsweat, fever)

Organ involvement (extranodal disease more common than in HL): skin rashes, hepatosplenomegaly, abdominal mass and discomfort. Headache, sore throat, cough.

Specific symptoms:

· Mediastinal T cell tumor: rapid enlargement, SVC obstruction, respiratory

· Large retroperitoneal B cell tumorL vomiting and abdominal pain

· Dissemination stage of NHL: presents as ALL with multiple masses

Question 17

What would you find on examination of someone with NHL?

Answer 17

Painless firm rubbery lymphadenopathy: axillar, inguinal and cervical. Oropharyngeal lymph nodes (Walader/s sign)

Skin rashes: Myocidosis funoides (thickened indurated scaly plaques), Sezary’s syndrome. (erythroderma, peripheral lymphadenopathy, cellular infiltrates of atypical lymphocytes.

Abdominal masses, hepatosplenomegaly

BM involvement: signs of anaemia, bruising infection and purpura.

Question 18

What investigations would you do for someone with NHL?

Answer 18

Blood: FBC (anaemia, thrombocytopenia and neutropaenia if BM involvement). Uric acid, LDH high. ESR and CRP high. Ca may be high. LFT (high transaminase in liver involvement). HIV, HBV, HCV serology.

Blood film: may show lymphoblasts

Bone marrow biopsy

CT/XR/PET scan for metastases

Lymph node biopsy: for immunocytology, immunophenotyping and cytogenetics

Staging: as Hogdkins (M0-7)

Question 19

What is the management of NHL?

Answer 19

The histology and extent of disease dictates treatment

· Stage I/II: 3 cycles of CHOP (cyclophosphamide, hydroxydanorubicin, onovincristine and prednisolone) + Rituximab (anti CD20) if CD20+ disease.

· Stage III/IV: 6-8 cycles as above.

Indolent NHL: watch and wait, single agent chemotherapy may be used.

Relapse: autologous or allogenic stem cell transplant

Baseline echocardiogram must be taken before starting hydroxydanorubicin

Discuss fertility issues, banking is an option.

Question 20

What are the complications and prognosis of NHL?

Answer 20

From treatment: secondary malignancy, BM suppression, N&V, tumor lysis syndrome, mucosiits and infertility.

Depends on histological type. Bad prognosis associated with high LDH, age, stage, and extranodal sites.