• Primary tumors of the lymphoid system
• Cancers involving lymphocytes during maturation or storage in the bone marrow

1. Hodgkin’s lymphoma
2. Non-Hodgkin’s lymphoma
3. Burkitt’s lymphoma

Hodgkin’s Lymphoma

Hodgkin’s disease (HD) is a lymphoid malignancy characterized by the presence of Reed-Sternberg cells of B cell origin. The disease arises usually in single nodal area and successively spreads to contiguous lymph node areas. Extranodal involvement is rare.

Types (Histological)

• Lymphocyte predominant—abundant lymphocytes, few plasma cells, occasional Reed-Sternberg cell, localized involvement of one side of diaphragm and most favorable prognosis.

• Mixed cellularity—lymphocytes, plasma cells, eosino- phils, easily identified Reed-Sternberg cell.

• Nodular sclerosis—sparse lymphocytes, stromal cells, fibrosis and numerous but bizarre Reed-Sternberg cells. It has poor prognosis.

• Lymphocyte depletion—lymphocytes, plasma cells, eosinophils with localized involvement.

ETIOLOGY

• Unkonown
• However both genetic and environmental factors including EBV ( Ebstein Barr Virus) play role in pathogenesis.

CLINICAL FEATURES

• • Age and sex—it is characterized by a bimodal age incidence, peak one in young adults and the second in the 5th decade of life with equal distribution between sexes.
• First sign – asymptomatic enlargement of a supradiaphragmatic lymph node often in the neck
• .Symptoms—theinvolvednodesarepainless.Generalized weakness, loss of weight, cough, dyspnea and anorexia are seen. Pain in back and abdomen owing to splenic enlargement, due to pressure of enlarged nodes or involvement of vertebrae.
• Pel Ebstein fever—characteristic features of this disease are Pel-Ebstein fever, a cyclic spiking of high fever and generalized severe pruritis of unknown etiology.

• Mediastinal lymph node involvement is common

• Night sweats, fever, weight loss

• Rarely produces oral manifestations

0. Radiographic Features
   • Site—it is rarely seen in jaws. The common regions are the posterior maxilla and mandible.
   • Appearance—malignant lymphoma arising in the oral cavity spreads to bone and cause irregular bone loss to the area of the lesion. There are radiolucent areas sepa- rated from each other by normal appearing bone which later become confluent, unless treatment is carried out.
   • Margins—typically,theradiolucentlesionshavediffuse ill defined margins which suggest infiltration of bone.
   • Osteoblastic type—osteoblastic type is uncommon in jaws, but it is seen in the vertebrae and pelvis. In it, there is frank sclerosis with filling of the marrow spaces by bone. It presents as grayness or whiteness which is abnormal. The margins may be well defined and sharp or irregular and trailing off gradually into the normal bone.

Diagnosis

• Clinicaldiagnosis—discreteenlargementoflymphnode which is rubbery in consistency with some systemic signs.
• Radiologicalfeatures—thereisfociofradiolucencyseen in the jaw.
• Laboratorydiagnosis—itischaracterizedbyreplacement of normal lymph node architecture by an admixture of malignant lymphoid cells and non-neoplastic inflammatory cells. Characteristic Reed Sternberg cells are present. Multinucleated giant cells are also present. There is also presence of anemia which is normocytic and normochromic. ESR is raised. There is also raised level of LDH.

MANAGEMENT

Localized disease: The patients with localized disease (IA, IIA) are treated with 3 cycles of chemotherapy (ABVD-doxorubicin, bleomycin, vinblastine and dacarbazine) followed by radiotherapy of involved nodal areas. 

Extensive disease: Patients with B symptoms or extensive disease receive complete course (6-8 cycles) of ABVD chemotherapy.

Long-term cure can be achieved in >90% patients with localized disease and in 50-75% patients with extensive disease.

Non-Hodgkin’s Lymphoma

It is also called as ‘lymphosarcoma’. In this group, there is neoplastic proliferation of lymphoid cells, usually affecting the B-lymphocytes. Unlike Hodgkin’s lymphoma, the disease is frequently widespread at the time of diagnosis, often involving not only the lymph nodes but also bone marrow, spleen and other tissue. Early involvement of bone marrow is typical of this lymphoma.

Types (Histological)

• Nodular—neoplastic cells tend to aggregate in such a way that large clusters of cells are seen.
• Diffuse—thereismonotonousdistributionofcellswith no evidence of nodularity or germinal center pattern.

• Etiology

• Viral—the etiology is unclear but herpes virus and Epstein barr etiology has been suggested.
• Immunological—there may be induced immunologic effect permitting a malignant clone to proliferate.

0. Clinical Features
   • Age and sex—it affects persons of all ages from infants to the elderly. But is commonest in middle age group. Males are affected more commonly than the females.
   • Onset—the onset of symptoms may be insidious. Painless lymph node enlargement of abdominal and mediastinal region are the most common finding. Very often the first group of lymph nodes affected may be cervical, axillary or inguinal.
   • Symptoms—the patient complains of tiredness, loss of weight, fever and sweating. Pain is the main symptom of bone involvement which may present as a patho- logical fracture. Patient may complain of abdominal pain, nausea, vomiting, diarrhea or intestinal obstruc- tion which may occur due to involvement of gastro- intestinal tract. Pressure effect of lymphoma may cause dysphagia, breathlessness, vomiting, intestinal obstruction or ascites and paraplegia.
   • Signs—if liver and spleen are involved, hepato- splenomegaly is present. The growth is fleshy and is prone to ulceration.

The matted, nontender lymph node enlargement in the lateral cervical region represents a common presentation of lymphoma.

Oral manifestations

• Gingival or mucosal tissue swelling or masses
• Rapid growth of tumour from non-healing tooth extraction site.
• Paresthesia or anaesthesia due to nerve invasion

Radiographic Features

• Appearance—asthediseaseprogressessmallradiolucent foci scattered throughout the area may be seen. Subsequent radiographs of the expanding lesion will show that these small foci have coalesced to form large multilocular moth eaten radiolucency with poorly defined margins.

• Margins—lesion blends imperceptibly with adjacent normal bone, in most of the cases.

• Effect on surrounding structures—lesions may cause marked expansion of bone. Erosion and perforation of cortex may occur.

• Maxillary sinus involvement—if the lesion involves maxillary sinus, possible opacification with breached cortical walls and associated paracentral or intracentral mass.

• Teeth—cortices of unerupted tooth buds and lamina dura of adjacent teeth are lost. Teeth may be resorbed.

Diagnosis

• Clinical diagnosis—bluish color mass of the palate with multiple lymph node involvement may suspect the diagnosis of non-Hodgkin’s lymphoma.
• Radiological features—expansion of bone with radio- lucency is present.
• Laboratory diagnosis—blood count usually shows hypersplenism or hemolytic anemia. The reduced WBC and RBC counts are seen along with reduced hemo- globin levels and reticulocytosis. In some cases, there may be slight increase in lymphocytes and thrombo- cytopenia. Moderate degree of anemia will also present when there is considerable bone marrow involvement. Some very aggressive high grade non-Hodgkin’s lymphomas are associated with very high urate levels which can precipitate renal failure.

Treatment

The disease stage is determined after clinical evaluation and investigations.
Treatment of low grade lymphoma: It depends on the stage of the disease and clinical status.

• Asymptomatic patients generally do not require therapy.
• Symptomatic localized (stage I) disease can be treatedwith radiotherapy.
• Symptomatic patients with extensive disease (stage III,IV) are treated with chlorambucil or combination chemotherapy (CVP; cyclophosphamide, vincristine, and prednisolone or CHOP; cyclophosphamide, doxorubicin, vincristine and prednisolone).

• Other newer effective agents are fludarabine, monoclonal antibody (anti-CD20) with or without radionuclides, and lymphoma vaccine.
• The relapse following therapy is common. Hence, low grade NHL is generally not curable. However, the cure is now possible in some cases with the use of radio- immunotherapy.
• Treatment of high grade lymphoma:

• Localized high grade NHL (stage I and non-bulky stage II) is treated with three cycles of chemotherapy (CHOP with monoclonal antibody, anti-CD20) followed by radiotherapy.
• Extensive disease (bulky stage II, stage III, and stage IV) is treated with six to eight cycles of chemotherapy.
• Autologous bone marrow transplantation is indicatedin relapsed cases.
• The cure rate is 60-90% in stage I and II and 30-40% instage III and IV.

REFERENCE- SHAFER’S TEXTBOOK OF ORAL PATHOLOGY, ANIL GHOM TEXTBOOK OF ORAL MEDICINE AND GOGLE SLIDE SHARE