|LETTER TO EDITOR
|Year : 2022 | Volume
| Issue : 1 | Page : 90-91
Hardbound indurated plaque and nodules with “m band”: A report of secondary cutaneous diffuse large b-cell lymphoma
Jignaben Krunal Padhiyar, Nayankumar Harshadkumar Patel, Drishti Rajde, Shreya Jadeja
Department of DVL, Gujarat Cancer Society Medical College, Hospital and Research Centre, Ahmedabad, Gujarat, India
|Date of Submission||27-May-2021|
|Date of Decision||26-Jun-2021|
|Date of Acceptance||12-Oct-2021|
|Date of Web Publication||31-Dec-2021|
OPD 35- Ground Floor, Department of DVL, Gujarat Cancer Society Medical College, Hospital and Research Centre, Opp. DRM Office, Naroda Road, Ahmedabad - 380 025, Gujarat
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Padhiyar JK, Patel NH, Rajde D, Jadeja S. Hardbound indurated plaque and nodules with “m band”: A report of secondary cutaneous diffuse large b-cell lymphoma. Indian J Paediatr Dermatol 2022;23:90-1
|How to cite this URL:|
Padhiyar JK, Patel NH, Rajde D, Jadeja S. Hardbound indurated plaque and nodules with “m band”: A report of secondary cutaneous diffuse large b-cell lymphoma. Indian J Paediatr Dermatol [serial online] 2022 [cited 2022 May 20];23:90-1. Available from: https://www.ijpd.in/text.asp?2022/23/1/90/334678
Diffuse large B-cell lymphoma (DLBCL) is the most common type and accounts for up to 40% of all cases of non-Hodgkin's lymphoma (NHL). The incidence of NHL in children <16 years is 0.5−1.2 per 100,000 and DLBCL represents 10%–20% cases of NHL in the pediatric age group., The disease is characterized by heterogeneous presentations and aggressive behavior. Cutaneous involvement in terms of multiplicity of lesions and its time duration in relation to primary site affects the prognosis of the disease.
A 15-year-old girl presented with ill-defined indurated hardbound plaque extending from the neck to intermammary region [Figure 1a along with difficulty in swallowing for 4 months. There were few nodular lesions overlying the indurated plaque. We considered differentials of scleredema adultorum of Buschke, morphea, mycosis fungoides, and lymphoma. Basic and routine investigations revealed only low hemoglobin and raised lactate dehydrogenase. On protein electrophoresis, the patient was found to have “M band” of monoclonal gammopathy. Cutaneous histopathological examination showed infiltrate with “smudge cells” [Figure 1]c leaving a thin narrow Grenz zone [Figure 1]b. Neither increased collagen nor loss of appendages was found ruling out the possibility of morphea. No deposition was found on Alcian blue staining, and also inflammatory cells were positive for immune markers (immunohistochemistry [IHC] study was performed later) ruling out possibility of scleredema adultorum of Buschke. Hence, on suspecting lymphoma involving the skin, a positron emission tomography scan was advised which revealed conglomerated multiple discrete lymph nodes at B/L supraclavicular, B/L cervical, mediastinal, and abdominal stations; lytic destruction of sternum; moderate to severe left pleural effusion; concentric mural wall thickening of cervical and thoracic esophagus showing mild metabolism and enhanced intensity in the subcutaneous tissue in the neck. Trucut biopsy from a neck node in the right posterior triangle revealed diffuse infiltration of the node by large cells with vesicular nuclei, prominent nucleoli, and moderate cytoplasm. Mitosis and increased apoptosis were also seen. Further, IHC study on trucut biopsy specimen from the node showed immunopositivity for CD20, CD10, C-Myc (45%), BCl-2 (focal positive), MUM 1, Ki67 (50%), and immunonegativity for TDT and CD34. For confirmation, IHC study was also done on skin biopsy specimen, which was also positive for CD10, CD20, and Ki67 (90%). [Figure 2].
|Figure 1: (a) A 15-year-old female with ill-defined hardbound plaque over the neck and intermammary area with few overlying nodules diagnosed with secondary cutaneous DLBCL (b) H and E × 100 magnification showing infiltrates of smudge cells in the dermis leaving thin Grenz zone (c) Enlarged view with red arrow pointing at smudge cell|
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|Figure 2: Immunohistochemistry findings of skin biopsy specimen. (a) CD 10 positivity in the infiltrates (×40, magnification). (b) CD 20 positivity in the infiltrates (×100, magnification). (c) Ki 67 proliferation index was 90% (×40, magnification and red arrow pointing at smudge cell)|
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Lee et al. reported that the nodules are the most common manifestation in secondary cutaneous lymphoma followed by papules, plaques, and maculopatches. Our patient had an indurated plaque with few nodules. She had cutaneous involvement at the time of diagnosis and had presented with multiple lesions, both of which are poor prognostic findings. Secondary cutaneous DLBCL demonstrates higher international prognostic index (IPI) when compared to DLBCL leg type. According to revised IPI score, our patient had poor prognosis with probable overall survival of 55% and progression-free survival of 53%.
As per Hans' algorithm, our patient was classified as DLBCL-GCB (germinal center B-cell) type. However, we could not classify according to the new algorithm proposed by William Choi et al. due to unavailability of markers.
Of note, another interesting finding in our case was the presence of “M band” on immunoelectrophoresis. Paraproteinemia has been reported in cases of DLBCL but more commonly with non-GCB types. Further studies on free light chain assay and immunoglobulin type could not be carried out in our patient.
She was diagnosed with DLBCL-GCB stage IV AD+ and was started on rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone regimen. At the time of submission, the patient is in remission post six cycles of chemotherapy, and her lesions have resolved.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Horvat M, Zadnik V, Južnič Šetina T, Boltežar L, Pahole Goličnik J, Novaković S, et al.
Diffuse large B-cell lymphoma: 10 years' real-world clinical experience with rituximab plus cyclophosphamide, doxorubicin, vincristine and prednisolone. Oncol Lett 2018;15:3602-9.
Sandlund JT, Martin MG. Non-Hodgkin lymphoma across the pediatric and adolescent and young adult age spectrum. Hematology Am Soc Hematol Educ Program 2016;2016:589-97.
Allen CE, Kelly KM, Bollard CM. Pediatric lymphomas and histiocytic disorders of childhood. Pediatr Clin North Am 2015;62:139-65.
Lee WJ, Won KH, Won CH, Chang SE, Choi JH, Moon KC, et al.
Secondary cutaneous lymphoma: Comparative clinical features and survival outcome analysis of 106 cases according to lymphoma cell lineage. Br J Dermatol 2015;173:134-45.
Lee WJ, Won KH, Won CH, Chang SE, Choi JH, Moon KC, et al.
Secondary cutaneous diffuse large B-cell lymphoma has a higher international prognostic index score and worse prognosis than diffuse large B-cell lymphoma, leg type. Acta Derm Venereol 2016;96:245-50.
Choi WW, Weisenburger DD, Greiner TC, Piris MA, Banham AH, Delabie J, et al
. A new immunostain algorithm classifies diffuse large B-cell lymphoma into molecular subtypes with high accuracy. Clin Cancer Res 2009;15:5494-502.
Cox MC, Di Napoli A, Fabbri A, Cencini E, Ruco L. The significance of serum immunoglobulin paraprotein in diffuse large B-cell lymphoma. Br J Haematol 2018;182:741-2.
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