Pediatric Hematology and Oncology, 31:372–374, 2014Copyright C© Informa Healthcare USA, Inc.ISSN: 0888-0018 print / 1521-0669 onlineDOI: 10.3109/08880018.2014.903448

LETTER

Peripheral T-Cell Lymphoma in A Childwith Cutaneous Involvement

Begul Yagcı-Kupeli,1 Harun Gezer,2 Fatma Levent Istifli,3 Umit Celik,2

and Serhan Kupeli4

1Pediatric Oncology Unit, Numune Education and Research Hospital, Adana, Turkey;2Department of Pediatrics, Numune Education and Research Hospital, Adana, Turkey;3Pediatric Infectious Disease Unit, Numune Education and Research Hospital, Adana,Turkey; 4Department of Pediatric Oncology/Pediatric Bone Marrow Transplantation Unit,Faculty of Medicine, Cukurova University, Adana, Turkey

Keywords child, peripheral T-cell lymphoma, skin involvement

Dear Editor,

Non-Hodgkin Lymphomas (NHL) comprise 60% of all childhood lymphomas [1,2]. Inchildren at least 90% of NHL are high grade lymphomas and they typically present withextranodal disease, rapidly progressive course, bone marrow, and central nervous sys-tem involvement [3]. Peripheral T-cell lymphomas (PTCL) are heterogenous subgroupof NHL that comprise nearly 1% of all childhood NHLs [4,5]. A patient with generalizedlymphadenopathy, and maculopapular rash diagnosed with PTCL with skin involve-ment is presented here.

An 8-year-old-male presented with fever and rash that had begun 1 week before hisadmission. Physical examination revealed pink-colored maculopapular rash, perior-bital edema, facial erythema (Figure 1A), dyspnea, generalized lymphadenopathy withthe largest (2.0 × 2.5 cm) located at right lower cervical region, hepatosplenomegaly,and fever of 38.5◦C. His WBC count was 20.340/μL and there were atypical lympho-cytes on peripheral blood smear. L1 and L2 types of lymphoblasts accounting for 20%of all cells were seen on bone marrow aspiration smears. Flow cytometric analysisof bone marrow revealed CD2 and CD7 positivity (86% and 78%, respectively). BFMT-cell ALL-type therapy was started after skin and lymph node biopsies which con-firmed the PTCL. Cerebrospinal fluid (CSF) was negative for blasts at time of diagnosis.Evaluation of response to treatment at the 8th day of treatment with peripheral bloodsmear revealed 6% lymphoblasts (absolute blast count was 228/mm3). At the 28th dayof treatment, bilateral restriction of lateral gaze developed and central nervous syteminvolvement was detected with lymphoblasts seen on CSF (Figure 1B). The involve-ment of bone marrow was 60% at the time of progression. Chemotherapy protocolwas intensified and triple intrathecal therapy with prednisolone, methotrexate, and

Received 9 February 2014; accepted 8 March 2014.Address correspondence to Serhan Kupeli, MD, MSc, Department of Pediatric Oncology/PediatricBone Marrow Transplantation Unit, Faculty of Medicine, Cukurova University, Adana, Turkey.E-mail: serhankupeli@cu.edu.tr

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FIGURE 1A Pink-colored maculopapular rash, periorbital edema, facial erythema.

cytarabine was administered every other day until the CSF was cleared from lym-phoblasts. HSCT was planned for the time remission was achieved. HLA-matched sib-ling donor was found. However, preperative regimen for HSCT could not be startedbecause of severe bone marrow supression and fever. The patient died due to neu-tropenic sepsis at the end of third month of treatment.

Involvement of extranodal sites such as skin, lung, liver, and bone is common inPTCL [5,6]. Maculopapular rash resembling viral infection was one of the most strik-ing finding in our patient. Most probable diagnoses in such a condition are lymphoma,leukemia, and Langerhans cell histiocytosis. Reich et al. [7] reported manifestationsof skin involvement of NHL as tumor, hard infiltration, edema of subcutaneous tis-sue, maculopapular lesions, generalized erythroderma, and generalized ichthyosis.Skin involvement in NHL can be seen as primary cutaneous NHL or as part of sys-temic disease [8]. In our patient, skin involvement was one component of the ad-vanced stage PTCL along with mediastinal, peripheral lymph node, and bone marrowinvolvement. Windsor et al. [5] recommended T-cell type therapy rather than B-cell

FIGURE 1B Lymphoblasts on cerebrospinal fluid smear.

Copyright C© Informa Healthcare USA, Inc.

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B. Yagcı-Kupeli et al.

lymphoma protocols in treatment of childhood PTCL. Our patient was treated withan ALL-type BFM protocol [9]. Even though using an intensive protocol, progressioncould not be avoided in our case.

In conclusion, diagnosis and management of PTCLs bear many problems regard-ing its rarity, highly aggressive clinical behavior, and absence of adequate evidence ontreatment outcome. Intensified ALL type therapies should be used and HSCT shouldbe planned if there is a HLA-matched donor.

Declaration of Interest

The authors report no conflicts of interest. The authors alone are responsible for thecontent and writing of the paper.

REFERENCES

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[2] Izarzugaza MI, Steliarova-Foucher E, Martos MC, Zivkovic S. Non-Hodking’s lymphoma incidenceand survival in European children and adolescents(1978–1997): report from the Automated Child-hood Cancer Information. Eur J Cancer. 2006;42:2050–2063.

[3] Sanlund JT, Downing JR, Crist WM. Non-Hodking’s lymphoma in childhood. N Engl J Med.1996;334(19):1238–1248.

[4] Reiter A, Schrabbe M, Parwaresch R, et al. Non-Hodking’s lymohomas of childhood and adoles-cence: results of a treatment stratified for biologic subtypes and stage-a report of the Berlin-Frankfurt-Munster group. J Clin Oncol. 1995;13:359–372.

[5] Windsor R, Stiller C, Webb D. Peripheral T-cell lymphoma in childhood: population-based experiencein the United Kingdom over 20 years. Pediatr Blood Cancer. 2008;50(4):784–787.

[6] Arrowsmith ER, Macon WR, Kinney MC, et al. Peripheral T-cell lymphomas: clinical features and prog-nostic factors of 92 cases defined by the Revised European-American Lymphoma Classi- fication. LeukLymphoma 2003;44:241–249.

[7] Reich A, Wrobel G, Kazanowska B, et al. Skin involvement in highly malignant non-Hodgkinlymphomas of childhood and adolescence. Acta Dermatovenerol Alp Panonica Adriat.2006;15(4):158–168.

[8] Daar G, Kupeli S, Yalcın B, et al. Primary cutaneous anaplastic large cell lymphoma. Pediatr HematolOncol. 2010;27(7):558–563.

[9] Reiter A, Schrappe M, Ludwig WD, et al. Intensive ALL-type therapy without local radiotherapy pro-vides a 90% event-free survival for children withT-cell lymphoblastic lymphoma: a BFM Group report.Blood. 2000;95:416–421.

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