Non-Hodgkin's Lymphoma (NHL)

There is a malignant monoclonal proliferation of lymphoid cells ( majority of B-cells and minority of T-cells ).

Working classification

Three grades ( low, intermediate, high ).

Staging by Ann-Arbor classification

Stage 1

Involvement of a single lymph node region or a single extra - lymphatic origin or site.

Stage 2

Involvement of 2 or more lymph node regions on the same side of diaphragm.

Stage 3

Involvement of lymph node regions on both sides of the diaphragm.

Stage 4

Diffuse or disseminated involvement of one or more extra - lymphatic organs.

Stage 5

Localised, solitary involvement of extralymphatic tissue excluding liver and bone marrow.

Clinical features

Patients present with lymph node enlargement, which may be associated with systemic upset: weight loss, sweats, fever and itching.

Hepatosplenomegaly may be present.

Site of extranodal involvement include the bone marrow, gut, thyroid, lung, skin, testis and brain.

Compression syndromes may occur, including gut obstruction, ascites, superior vena cava obstruction and spinal cord compression.

Investgations

Lymph node biopsy under CT guidance
Bone marrow aspiration and trephine.
Immunophenotyping of surface antigens to distinguish T from B cell tumours.
Measurement of uric acid levels: some very aggressive high-grade NHLs are associated with very high urate levels, which can precipitate renal failure when treatment started.
Positive Coombs' test in the presence of complicating hemolytic anaemia
Test for HIV since it may also present with generalised lymphadenopathy.

Mangement

In low grade lymphomas:

Radiotherapy is used for stage 1 and stage 2A.
Chemotherapy for stage 2B, 3 and 4.
CHLORAMBUCIL 20 mg/m2/day for 3 days every month.
Interferons has also been tried.
Autologous bone marrow transplant has been done on experimental basis.
RITUXIMAB along with CYCLOPHOSPHAMIDE, VINCRISTINE and PREDNISOLONE (R-CVP) is commonly used as first-line therapy.

In high grade lymphomas:

Stage 1 disease is treated with radiotherapy.
Stage 2, 3 and 4 diseases are treated with intensive combination chemotherapy.
R-CHOP regimen is used here ( RITUXIMAB, CYCLOPHOSPHAMIDE, ADRIAMYCIN, VINCRISTINE AND PREDNISOLONE ).
HSCT. Autologous HSCT benefits patient with relapsed chemosensitive disease.

Comments

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