Symptoms and signs
1. Indolent lymphomas
As a group, low-grade lymphomas are characterized by indolent
clinical behavior and comparatively prolonged survival (median
survival, 6-10 years). Most patients have advanced- stage disease at
diagnosis, and only about 10%-20% have stage I or II disease.
- They usually appear above the age of 50
and present with generalized lymphadenopathy.
- They commonly involve the bone marrow and spleen.
- Despite the relative incurability of the disease in its advanced
stages, follicular lymphoma patients tend to experience a long
overall survival (8-12 years).
- Lymphoplasmacytic Lymphoma is an indolent lymphoma associated with
a monoclonal paraprotein of immunoglobulin M type.
- They usually
present with generalized lymph node, splenic and bone marrow involvement.
- Some patients may develop hyperviscosity syndrome (which
may lead to retinopathy, congestive heart failure and CNS dysfunction)
due to excessive paraprotein secretion.
- Patients may also experience
chronic cold agglutinin disease.
Marginal zone lymphomas
- Nodal marginal zone lymphomas involve the nodes and are called monocytoid B-cell lymphomas
- Extranodal marginal zone lymphoma involves the extranodal sites
(gastrointestinal tract, thyroid, lung, breast, skin), they are called
mucosa-associated lymphatic tissue (MALT) lymphomas.
Splenic marginal zone lymphoma
- Splenic marginal zone lymphoma is an indolent lymphoma that is
marked by massive splenomegaly and peripheral blood and bone marrow
involvement, usually without adenopathy.
- Splenectomy may result in
prolonged remission, otherwise it is treated as low grade lymphoma.
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2. Intermediate to high grade lymphoma
The clinical manifestations of intermediate- and high-grade NHLs
are diverse and depend on the site of disease involvement. These
tumors have a rapid growth rate and present as painless masses that
cause symptoms when they infiltrate tissues or obstruct organs.
Lymphomas more frequently affects the lymph nodes, spleen, liver,
and bone marrow, but any extranodal site may also be primarily
- As with Hodgkin's disease, NHL also presents with systemic B
symptoms, including fever, which may or may not have the Pel-Ebstein
relapsing pattern; drenching night sweats; and more than 10% weight
loss. Generalized pruritus may also be present.
- Nonparathyroid hormone
induced hypercalcemia occurs in approximately 10% of patients.