B-cell neoplasms Precursor B-cell neoplasm
Precursor B-lymphoblastic leukemia/lymphoma Precursor B-cell ALL Mature (peripheral) B-cell neoplasms
B-cell chronic lymphocytic leukemia/small lymphocytic lymphoma B-cell prolymphocytic lymphoma Lymphoplasmacytic lymphoma
Splenic marginal zone B-cell lymphoma (±villous lymphocytes) Hairy cell leukemia
Extranodal marginal zone B-cell lymphoma of MALT type Nodal marginal zone B-cell lymphoma (±monocytoid B-cells) Follicular lymphoma Mantle-cell lymphoma Diffuse large B-cell lymphoma Mediastinal large B-cell lymphoma Primary effusion lymphomas Burkitt's lymphoma/Burkitt cell leukemia T-cell and NK-cell neoplasms Precursor T-cell neoplasm
Precursor T-lymphoblastic leukemia/lymphoma (Precursor T-cell ALL) Mature (peripheral) T-cell neoplasms T-cell prolymphocytic leukemia T-cell granular lymphocytic leukemia Aggressive NK-cell leukemia Adult T-cell leukemia/lymphoma (HTLV-1+) Extranodal NK-/T-cell lymphoma, nasal type Enteropathy-type T-cell lymphoma Hepatosplenic y-S T-cell lymphoma Mycosis fungoides/Sezary syndrome
Anaplastic large-cell lymphoma, T-/null cell, primary cutaneous type Peripheral T-cell lymphoma, not otherwise specified Angioimmunoblastic T-cell lymphoma
Anaplastic large-cell lymphoma, T-/null cell, primary systemic type Posttransplantation lymphoproliferative disorders Early lesions
Reactive plasmacytic hyperplasia Infectious mononucleosis-like PTLD, polymorphic Polyclonal (rare) Monoclonal
PTLD, monomorphic (classify according to lymphoma classification) B-cell lymphomas
Diffuse large B-cell lymphoma (immunoblastic, centroblastic, anaplastic) Burkitt's/Burkitt-like lymphoma Plasma cell myeloma T-cell lymphomas
Peripheral T-cell lymphoma, not otherwise categorized Other types (hepatosplenic, y-S, T/NK) Other types, rare
Hodgkin's disease-like lesions (associated with methotrexate therapy) Plasmacytoma-like lesions
Abbreviations: HTLV1+, human T-cell lymphotropic virus 1; MALT, mucosa-associated lymphoid tissue; PTLD, posttransplantation lymphoproliferative disorders; NK, natural killer; WHO, World Health Organization; ALL, acute lymphoblastic leukemia. Source: From Ref. 2.
with more aggressive disease. Skin involvement resulting in a rash that may be nodular may also occur. T-cell lymphomas have a greater propensity for skin involvement. Neurological involvement can present with focal findings, affecting both the central and the peripheral nervous system. In suspected cases, a magnetic resonance imaging scan in addition to spinal fluid sampling is mandatory (9).
WM, a syndrome that may accompany lymphoplasmacytic lymphoma, results from the presence of monoclonal immunoglobulin (Ig)M (macroglobulinemia). Symptoms can be related to the presence of the lymphoma, due to cytopenias related to marrow infiltration, or due to the monoclonal protein. Hyperviscosity related to the elevated IgM may be present, or the paraprotein may have self-directed antigenic specificity. Fatigue, headache, and weight loss are common. Bleeding is a common manifestation, with gum bleeding and epistaxis of concern in the head and neck. Neurological abnormalities, most commonly peripheral neuropathy, occur and focal cranial nerve abnormalities and mononeuritis multiplex may be seen. The hyperviscosity syndrome can result in confusion, lethargy, visual loss, deafness, ataxia, or diplopia. A sausage-like appearance of the retinal veins has been reported due to venous dilation and segmentation. This is a medical emergency that should be managed with rapidly instituted plasmapheresis.
The signs and symptoms of primary head and neck lymphoma depend on the site(s) of involvement. They are mostly related to either obstruction or mass effect. Systemic or B symptoms are present in the minority of patients (1). These tumors can mimic other neoplasms or can be mistaken for infections or other common head and neck disorders.
Sinonasal tumors typically present with nasal obstruction, which is most often unilateral. Epistaxis, hyposmia, nasal swelling, and mass are other common complaints. Tonsillar lymphomas present with unilateral enlargement, ulceration, dysphagia, and/or odynophagia. Lymphomas of the salivary glands and thyroid gland present as painless masses, and the diagnosis is rarely suspected initially. Laryngeal lymphomas, which are quite rare, present with voice change, airway obstruction, and, occasionally, with hemoptysis. Orbital lymphomas may present with a variety of ocular symptoms, with proptosis and diplopia being most common. Over 80% of all reported cases of extramedullary plasmacytoma occur in the head and neck, mostly in the upper aerodigestive tract. Clinically, they present as a mass and cause symptoms based on their location and by direct effect on adjacent structures, with upper airway obstruction, epistaxis, rhinorrhea, pain, and neck swelling being the most common.
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