Manifestations of Lymphoma

Lymphomas are the most prevalent haematological malignancies. They are highly curable particularly in the early stage. As is the case with all cancers, there are no symptoms typical of lymphoma. The manifestations of lymphoma include:

Lymphadenopathy

Lymph node may be classified into superficial or deep group.

  1. Superficial Nodes: Superficial nodes include occupital, cervical, supraclavicular, epitrochlear, axillary, inguinal node.
    1. Cervical Nodes: Enlargement of cervical nodes is common. Children may have palpable cervical nodes that regress with age. These nodes are usually soft and small. Large, firm to hard, matted or enlarging cervical nodes need evaluation. The commonest cause of enlargement of cervical nodes is infection of upper repiratory tract. Patients with cervical lymphadenopathy associated with an upper respiratory tract infection need to be followed up till the lymph node regress. Nodes that do not regress after the infection subsides need evaluation.
    2. Occipital Nodes: Occipital nodes may be enlarged in patients with lice infestations. It is unusual to find occipital node engagement in disease.
    3. Axillary Nodes: Enlargement of axillary nodes usually indicates a disease in the draining areas – upper limbs, breast, lungs. Patients with recurrent trauma of the upper limbs may have enlarged axillary nodes.
    4. Inguinal Nodes: Inguinal lymphadenopathy is a feature of disease of the lower limb and the anogenital region. Recurrent trauma to the lower limbs can cause inguinal lymphadenopathy.
    5. Epitrochlear Nodes: Enlarged epitrochlear or popleteal nodes always indicates a disease, usually one causing a diffuse lymphadenopathy.
  2. Deep Nodes: The deep nodes include the mediastinal, hilar, intra-abdominal and pelvic nodes. Enlargement of these nodes causes pressure symptoms and is . Enlargement of  these nodes indicates disease.

Almost all Hodgkin’s lymphomas and majority of non-Hodgkin’s lymphoma present with enlargement of lymph nodes. Lymphadenopathy is a common symptom. It may be a result of  infection, inflammation or malignancy.

  1. Lymphadenopathy of Acute Infection/Inflammation: Patients with acute inflammation can be differentiated from lymphoma as they have painful and tender nodes often with erythema of the overlying skin. These node can suppurate. The area drained by the node has a inflammatory lesion, usually an infection.
  2. Lymphadenopathy of Chronic Infection/Inflammation: Lymphadenopathy of chronic inflammation has lacks erythema, pain and do not suppurate. The node enlarge at a slower rate. Differentiation of lymphadenopathy caused by chronic inflammatory disorders and malignancy can only be made by pathological examination of the nodes. The differentiation between tuberculous lymphadenitis and lymphoma (particularly Hodgkin’s lymphoma) can be challenging as regions of the world that have high prevalence of tuberculosis also have a resource constrain.

 

Tuberculosis lymphadenitis is common in resource constrained regions of the world. It is the practice in these regions to initiate anti-tuberculous therapy for patients with matted cervical nodes either on clinical grounds or following a fine needle aspiration cytology. While this can not be justified scientifically, proponents of this practice point to the lack of healthcare infrastructure to justify this practice. If one is choosing this path one must follow the patient to confirm regression and be aware of the existence of paradoxical response in tuberculosis. Paradoxical response is characterised by increase in constitutional symptoms and lymph node size on initiation of anti-tuberculous treatment. It is seen in about 10-15% patients.

Splenomegaly and Hepatomegaly

Spleen is a lymphoid organ. Lymphomas can originate in the spleen of the spleen can be secondarily involved by lymphoma. Primary splenic lymphomas, defined as splenic involvement without lymph node involvement forms about 6% of all lymphomas (Blood 2010; 117:2585). The symptoms of splenic lymphomas include abdominal discomfort and early satiety due to splenomegaly, constitutional symptoms like fever, weight loss and night sweats due to underlying lymphomatous process.  Patients with splenic lymphomas may have alterations in blood counts. Important history includes that of infections hepatitis C, hepatitis B, autoimmune disorders, treatment with anti-TNF agents.

Bone Marrow and Peripheral Blood

All lymphomas may involve the bone marrow and spread into the blood. Bone marrow and peripheral involvement is common in two groups of lymphoma the very high grade lymphomas like lymphoblastic lymphoma and Burkitt’s lymphoma and low grade lymphomas like small lymphocytic lymphoma. Bone marrow involvement in lymphoblastic lymphoma and Burkitt’s lymphoma manifests as acute lymphoblastic leukaemia is destructive. It manifests as leucocytosis and bone marrow failure (anaemia, neutropenia, thrombocytopenia). Leucocytosis is also a feature of bone marrow involvement with low grade lymphomas. These cells are mature. The growth is accommodative and cytopenias occurs late in the disease, if at all.

Extranodal Lymphomas

It is exceptional to have an extra-nodal Hodgkin’s lymphoma. The proportion of extra nodal non-Hodgkin lymphoma varies across the world.

  1. Gastrointestinal Tract: The commonest site of extranodal involvement is the gastrointestinal tract. Within the gastrointestinal tract, stomach is the commonest site for extranodal lymphomas of the gastrointestinal tract followed by small intestine, colon and oesophagus. The most common manifestation of gastrointestinal lymphoma is nonspecific symptoms like abdominal dyscomfort and pain. Anaemia is a feature of gastric and colonic lymphoma. High grade small intestinal lymphoma may present with intussusception.
  2. Skin Involvement: Cutaneous lymphomas are a distinct entity. Unlike other sites many cutaneous lymphomas are of T cell origin. They present with macules, papules and nodules.
  3. Central Nervous System: Central nervous system may manifest with global symptoms like comfusion, headache and altered consciousness, may have foaly neurolohical deficiit, seizures or multiple cranial nerve involvement because lymphomatous meningitis
  4. Primary testicular lymphoma presents as a unilateral painless swelling of the testis in an elderly male (see Primary Testicular Lymphoma). Hydrocele is present in about 40% of the patients.

Presentation Peculiar to Some Lymphomas

  1. Lymphoplasmacytic lymphoma is unlike other lymphomas in that symptoms of fatigue, weakness and breathlessness dominate. Splenomegaly and lymphoadenopathy are uncommon. Patinets may develop symptoms of hyper viscosity including headache, blurring, eipstasix.
  2. Nasal NK/T cell lymphoma presents with obstructive and destructive mass in the upper aerodigestive tract. It is reported most commonly in patients from the far east.
  3. Hepatosplenic γδ-T cell lymphoma presents with hepatosplenomegaly, no adenopathy, B symptoms and cytopenias. Patinets may have history of anti-TNF therapy for Crohn’s disease.
  4. Intravascular diffuse Large B Cell lymphoma is characterized by disseminated intravascular proliferations of B cells most commonly in the vessels of the CNS, kidney and lungs. Patinets present with symptoms secondary to vascular occlusion. The diagnosis is usually difficult.

Paraneoplastic Syndromes

Paraneoplastic syndromes associated with lymphomas include hypercalcaemia, syndrome of inappropriate ADH secretion, paraneoplastic ceribellar degeneration, motor neuron disease, acute polyradiculopathy, polyneuropathy of paraproteinaemia, neuropathy due to paraneoplastic vasculitis, neuromuscular junction disorders, sweets syndrome and minimal change nephrotic syndrome (see paraneoplastic syndromes associated with lymphoma for more conditions and a detailed discussion).

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