Issue :

A 20 year-old man visited RSGM. He revealed that there’s swelling behind the left and right ears.
The lumps appear suddenly and rapidly enlarge within 1 month. Patient is not satisfied with the
tooth that is sick. It has been taken to the dentist and given medicines (antibiotics and anti-
inflammatory drugs), but the swelling never deflates, instead it gets bigger.

Keywords :
1.Swelling behind the right and left ears
2.Lumps grow quickly in 1 month
3.The swelling does not flatten and gets bigger
4.Extraoral : Lumps, normal color, unclear border.
5.HPA : Lymphoblast proliferation, atypical cells, germinal center form, datia cells, reed sternberg
cell
6.RO : Radiolucent lesions

SUPPORTING KEYWORDS :
1. a 20 years old man
2.History of antibiotic and anti-inflammatory treatment
3.No pain and no inflammation
4.There are no teeth that hurt

Patient aged 20 years come to RSGM with complaints, there is a lump behind the left and
right ears. Lumps appear suddenly and quickly enlarge within one month. Patient complain that
there are no teeth diseases. It has been taken to the clinic's dentist and given medicines (antibiotics
and anti-inflammatory drugs), but the swelling never deflates, even enlarges. On extraoral
examination, normal colored capsules were found with unclear boundaries. On HPA examination,
there are many lymphoblast cells, a result of uncontrolled lymphoblast cell proliferation, atypical
cells, the shape of the germinal center, and there are cells in the form of reed-sternberg cells with
characteristic features such as owl eyes. On radiographic examination, the lesion appears
radiolucent.
In this case scenario, a lump that appears suddenly, can be a cyst or a neoplasm. However,
the lump rapidly enlarges within one month and the swelling never deflates even bigger, is a sign
of a malignant neoplasm. Patients complain of no toothache, is a sign of no infection or source of
pain originating from odontogens. The diagnosis is reinforced by HPA and radiographic
examination, on HPA examination found reed sternberg cell formation, which is a typical cell in
hodgkin lymphoma. Lumps are behind the right and left ears, thought to be enlargement of the
lymph glands. This, reinforces the diagnosis of the scenario, that the patient experiences malignant
neoplasm in the form of hodgkin lymphoma.
 Hodgkin's limfoma is a classification of malignant lymphoma. Malignant lymphoma is
cancer that originates from abnormal lymphocyte cells that develop out of control and can spread
to the lymphatic system throughout the body. Malignant lymphoma is often associated with
Epstein Barr Virus (EBV) because it plays a role in the development of limforetic cellular
neoplasms of B cells. The presence of EBV influences the expression of Latent Membrane Protein-
1 (LMP-1). LMP-1 is a latent gene that can change cell lines, change the phenotype of cells, induce
proliferation, and prevent apoptosis in cells infected by inducing BCL-2, A20, and MCI-1 and
activate NF-kB (Setyowati et al ., 2017; Asmara et al., 2018). In 70% or a third of cases of Hodgkin
Lymphoma (LH) that have been reported worldwide, there is involvement of Epstein Barr virus
(EBV) infection in Reed-Sternberg cells (Kumar et al., 2013).
Malignant lymphoma, classified into two types, namely, Hodgkin's Lymphoma and Non-
Hodgkin's Lymphoma. Hodgkin lymphoma is a malignant tumor that affects B lymphocytes
certain characteristics include lymph nodes, especially the cervical lymph node, the majority
regarding young age, tumor tissue composed of large mononuclear and multinucleate tumor cells
scattered in small amounts (Reed-Sternberg cells) and tumor cells that are often circled by T
lymphocytes form a Rossete-like structure (Setyowati et al., 2017).
Hodgkin Limphyoma is most commonly diagnosed in the group ages 20 to 34 years,
accounting for 31% of new cases; however, it can be seen across the age spectrum, from
adolescents to the elderly. Painless lymphadenopathy enlarging over months is a common mode
of presentation. B symptoms—fevers, chills, night sweats, or unexplained weight loss > 10% of
body weight—are frequent in patients with advanced-stage or bulky disease and are prognostic;
therefore, they are included in the staging system. Severe, unremitting pruritus without obvious
skin pathology on examination can be resistant to topical and systemic agents and can be an early
clue to the presence of clinically occult HL (Shanbhag and Ambinder, 2017).
Typical signs of Hodgkin's lymphoma are obtained by HPA examination characterized by
the presence of Reed Sternberg cells against the background of pleomorphic inflammatory cells
(lymphocytes, eosinophils, neutrophils, plasma cells, and histiocytes). Whereas on HPA Non-
Hodgkin Lymphoma examination there is an increase in heterogeneity of malignant cells with
variations in cell size, number of cytoplasm, and nucleoli that are very prominent. Neoplastic
lymphoid cells are large and have twice the size of normal lymphocytes without any description
of Reed-Sternberg cells (Gobbi, 2013; Kumar et al., 2013)
Reed-Sternberg cells are very large cells with a diameter of about 15-45 micrometers,
multi-lobular large nuclei with many protruding nucleus children and a slightly eucinophilic
cytoplasm. The main characteristic of the Reed-Sternberg cell is that there are two core children
side by side, the picture looks like an owl-eye (Kumar et al., 2013).
The expression of the gene from EBV is thought to trigger transformation and
reprogramming of lymphocyte B cells towards one of the LH phenotypes. At the time of primary
infection, EBV will enter the latent phase in the memory of B-lymphocyte cells so that EBV can
survive throughout the lifetime of lymphocyte B cells. EBV then codes for the products of the
EBNA-1 and LMP-1 genes which are thought to play a role in the memory transformation of
lymphocyte B cells. These gene products work on intracellular signaling pathways where EBNA-
1 works directly by giving a negative feed on the expression of tumor suppressor genes and
enhancing tumor development through positive feed on CCL22 which then promotes lymphocyte
B-cell activation. At the same time, the LMP-1 gene product mimics the signal produced by CD40
which works to activate the NF-kB, p38, PI3K, AP1 and JAK-STAT signal pathways in promoting
the survival of lymphocyte B cells. EBV infection is also thought to be the cause of genetic
mutations in Ig genes that encode B-cell lymphocyte receptors where EBV then encodes the LMP-
2 gene which is able to reprogram mature lymphocyte B cells towards one of the LH phenotypes
and prevent the occurrence of apoptosis through activation Rescue signal at the germinal center of
B-lymphocyte cells. As a result of the series of processes mentioned above causing uncontrolled
clonal expansion of lymphocyte B cells which will then secrete various cytokines, such as IL-5
which will attract and activate eosinophils and IL-13 which can stimulate Reed-Sternberg cells
further to express CD30 (Ki-1) and CD15 (Leu-M1). CD30 is a lymphocyte activation marker
expressed by reactive and malignant lymphoid tissue cells, whereas CD15 is a marker of
granulocytes, monocytes and T-cells activated lymphocytes that are not normally expressed by B
lymphocyte cells. People with a family history have had LH, especially twins and people with
immune system disorders, such as people with HIV / AIDS also have a high risk of suffering from
LH (McDade, 2015).
Patient diagnosed with IA and IIA Hodgkin Lymphoma because the tumor in the patient is not
large, the tumor is in less than 3 different lymph node areas (only in the back of the ear), does not
cause any symptoms, and ESR (erythrocyte sedimentation rate) does not increase .
Treatment for patients diagnosed with Hodgkin Stage IA and IIA lymphoma is chemotherapy
(usually 2 to 4 cycles), followed by radiation to the initial location of the disease (ISRT or location
radiation therapy involved 20 to 35 Gy). PET / CT scans can be done after several times
chemotherapy to see the results of treatment and to determine how much treatment is needed next.
If treatment does not produce results, chemotherapy can be done using different drugs or high-
dose chemotherapy and radiation followed by stem cell transplantation (stem cells). If
chemotherapy does not help, treatment can be done with Corticosteroids. If the patient cannot
undergo chemotherapy because of other health problems, radiation therapy can be done without
chemotherapy. Treatment with monoclonal antibody (Adcetris®) can be another option.

Sumber :
McDade L. Classical Hodgkin’s Lymphoma: Pathogenesis and Future Treatment Directions. Res
Medica. 2015;23(1):47-57p.
Kumar V, Abbas AK, Aster JC. Robbins Basic Pathology. Edisi 9. Philadelphia. W. B. Saunders
Company. 2013. 440-442p.
Shanbhag, S. and Ambinder, R. (2017). Hodgkin lymphoma: A review and update on recent
progress. CA: A Cancer Journal for Clinicians, 68(2), pp.116-132.