Mehmet Can Ugur1, Alp Ozguzer2, Dudu Kahraman Solakoglu2, Gulden Diniz2 and Cengiz Ceylan3
1Izmir Tepecik Education and Training Hospital, Department of Internal Medicine, Turkey
2Izmir Tepecik Education and Training Hospital, Department of Pathology, Turkey
3Izmir TepecikEducation and Training Hospital, Hematology, Turkey
*Corresponding author: Mehmet Can Ugur, Department of Internal Medicine,Izmir Tepecik Education and Training Hospital, Turkey, Tel:+90 5058861126; E-mail:email@example.com
Received Date: 03 October, 2016; Accepted Date: 25 October, 2016; Published Date: 31 October, 2016
Isolated cutaneous follicular lymphoma as are common; cases with isolated skin involvement are rare in the literature. We presented a relapsed follicular lymphoma case, initially diagnosed in cervical lymphnode, with skin involvement following the treatment.
A 70 year woman presented with cervical lymphadenopathy and she was diagnosed FollicularLymphoma. A diagnosis of Follicular Lymphoma was made and the patient underwent 6cycles of CHOP chemotherapy. The patient was admitted with abdominal lesions occurred in the preceding month. There were 2 lesions on the abdomen skin. Biopsy specimens obtained from skin lesions showed the similar results with the previous biopsy specimens obtained from lymph nodes. All these results pointed out the skin involvement of follicular lymphoma. The patient underwent 6 cycles of CHOP chemotherapy for the second course. Complete remission was obtained and the lesions disappeared. She has gone into complete remission for 5 months.
Relapsing FL involves usually lymphnodes and bone marrow while skin involvement is very rare. Our case is quite different from other cases in the literature by means of site of involvement and lack of CD20 expression. AlthoughRCHOP is recommended for the therapy, wegave 6cycles of CHOP chemotherapy due to financial reasons and lack of CD20 expression. Lesions completely disappeared and this was a favorable prognosis.
Follicular lymphoma (FL), classified under the headline of B-cellneoplasm by World Health Organization, is these condmost frequent neoplasm among Non-Hodgin Lymphomas (NHL) in America and West European countries. It accounts for 20-25% of NHL and 70% of indolent Lymphomas. It is also the most common form of extra-nodal B-cell Lymphomas with a rate of 60% [1,2]. Although isolated cutaneous follicular lymphomas are common, cases with isolated skin involvement are rare in the literature. We presented are lapsed follicular lymphoma case, initially diagnosed in cervical lymphnode, with skin involvement following the treatment.
A 70 year old woman admitted to our clinic with cervical lymphadenopathy in 2012. In Lymph node biopsy, neoplastic cells were negative for CD3,CD20,CD30,CD45 and Row here as neoplastic cells were strongly positive for CD79 a and Bcl2 (Figure 1A and 1B). A diagnosis of Follicular Lymphoma was made and the patient underwent 6cycles of chemotherapy of cyclophosphamide, vincristin, adria blastin and methyl prednisolone (CHOP). Bone marrow biopsy did not reveal any involvement.
The patient was admitted to our clinic with abdominal lesions occurred in the preceding month. On examination, the vital findings of the patient were normal. In the physical examination, there were 2 pale red colored slightly elevated lesions with a diameter of 1-2 cm on the surface of the abdomen skin (Figure 2). Her complete blood cell count (CBC) showed leucocyte: 3500/ uL, hemoglobin: 12.1 g/dL platelet: 194000/uL. In biochemical assessment, all the tests were within normal limits except for lactate dehydrogenase (LDH) with a level of 361 U/L.
Abdominal, thoracic and cervical computerized tomography with contrast revealed multiple, solid, subcutaneous, ovoid and round shaped nodular lesions with the largest one of 26mm in diameter. These images are in concordance with soft-tissue involvement in a patient with lymphoma. Biopsy specimens obtained from skin lesions showed the similar results with the previous biopsy specimens obtained from lymphnodes; neoplastic cells were negative for CD3,CD20,CD30,CD45 and Row here as neoplastic cells were
Follicular lymphomas, account for the majority of indolen tlymphomas, is slightly more likely to be diagnosed in women than men . FL might be presented as primary skin lesions but to our knowledge, only one case of relapsed follicular lymphoma with skin involvement was reported in the literature.
Palacios Abufón A et al. reported subcutaneous lesions located on the face, chest and backin apatient with a previously diagnosis ofFL following splenectomy. In the biopsy specimen detection of CD20, Bcl2 and Bcl6 positivity was accepted as relapse . It is similar with our case except for CD20 negativity. Generally, 20% of patients with FL suffer from B symptoms at the time of diagnosis. Although it is characterized by nodal disorder, bone marrow involvement is present in 70% of patients
at diagnosis and the other sites of involvements are not common [5,6]. In our case, being female gender and lack of extra nodal involvement except for skin are in accordance with the literature. In follicular lymphomas there are not any characteristic laboratory findings but elevated LDH levels and cytopenia are common as in our case .
Follicular Lymphoma International Prognostic Index (FLIPI) was developed by international cooperative studies in 1985 and 1992 to collect data on initial characteristics of a large number of patients with FL and to propose the long term survival of the patient. In this scoring system, the prognostic factors are age, stage, number of lymph node areas involved, serum hemoglobin and LDH levels . The FLIPI score placed our patient into the high risk group.
As for the other lymphomas, diagnosis of FL is done by excisional biopsy. Bone marrow biopsy does not have diagnostic priority although it is an important part of the staging procedure . While these tumor tissues express characteristically CD20 (or- CD19), CD10 and BCL-6, they are negative for CD5 and CD23. Thet (14;18) translocation that inhibits apoptosis and bcl-2 gene over expression as seen in our case, are both pathognomonic for FL and can be detected in 89% of the cases [10,11]. The differential diagnosis has to be performed with cutaneous follicle center lym phoma (PK-FML). PK-FMLsareal ways negative for bcl-2 . Loss of CD20 expression in B-celllymphomas after Rituximab (R) Chimeric Anti-CD20 Monoclonal Antibody treatment is observed in some cases . CD20 negative de novo DLBCL cases are rare in the literature (14). On the other hand, CD20 negative de novo FL cases are strikingly rare . The case reported by Orla H et al. shows similarity with our caseby means of lacking CD20 expression.
Generally,15-30% of FL cases are diagnose dinearly stages (Stage1-2) . According to the general management recommendations for FL, patients with Stage I/II disease who are curable are under taken treatment . Combinations with rituximab have been recommended for the initial chemotherapy. CHOP or R-CHOP treatments are recommended in patients observing loss of CD20 expression after Rituximab therapy . ThecaseofOrlaHetal. responded well to dexamethasone, cytarabine, cisplatin (DHAP) combination . Due to Turkey’s health finance policies, it is not possible for us to prescribe the semedicines for stage- IB and IIB disease. For this reason, we initiated CHOP chemotherapy in our patient with Stage 1 disease according to Ann Arbor classification.
Relapsing FL involve susually lymphnodes and bone marrow while skin involvement is very rare. Our case is quite different from other cases in the literature by means of site of involve mentand lack of CD20 expression. Differentiation of FL from cut aneousfollicle center lymphoma might be confusing. Systemic involve ment and CD20 negativity are important factors for the differential diagnosis of PK-FML. Although R-CHOP is recommended for the therapy, we gave 6cycles of CHOP chemotherapy due to financial reasons and lack of CD20 expression. Lesions completely disappeare dand this was a favorable prognosis.
Figures 1A and 1B: Microscopic image of lymphnode with H&E and lymphoid cell infiltration with immuno-histochemistry and Bcl-2 positivity. DAB,x200.
Figure2: Pale red colored slightly elevated lesion with a diameter of 1-2 cm and the incision scar of the biopsy procedure
Figure3: Microscopic image: Patchynodular lymphoid infiltrates in dermis. H&E,x40
Figure 4:Centrositicneo plastic lymphoid cells together with small numbers of centroblastic neoplastic lymphoidcells.H&E,x200
Figure 5: Immuno histochemistry image of FLFL: Bcl-2 positivity in neoplastic cells. DAB,x100
Citation: Ugur MC, Ozguzer A, Solakoglu DK, Diniz G, Ceylan C (2016) A Rare Follicular Lymphoma Case: Relapsed with Skin Involvement. Ann Case Rep: ACRT-117. DOI: 10.29011/2574-7754/100017