Hemofagositik Sendromla Prezente Olan Nodal EBV + Sitotoksik T-Hücreli Lenfoma Vakası
Özet
Bilinen kronik hastalık öyküsü bulunmayan 31 yaşındaki erkek hasta; yaklaşık 1 aydır mevcut olan ve giderek sıklığı artan ateş, sarılık, halsizlik ve karında şişkinlik şikayeti ile kliniğimize başvurdu. Yapılan fizik muayenesinde ikterik sklera, soluk konjonktiva, boyun ve axilla bölgesinde palpe edilebilen lenfadenopati (LAP) ve hepatosplenomegali saptandı. Yapılan tetkiklerinde pansitopeni, hiperferritinemi, karaciğer fonksiyon testlerinde yükseklik, hiperbilüribinemi, hipofibrinojemi ve koagülasyon parametrelerinde hafif uzama mevcuttu. Hastadan submandibuler LAP ve kemik iliği biyopsisi yapıldı. Patolojik ön değerlendirmesinde hemofagositozis ile uyumlu histiyosit artışı izlenen hastaya hemofagositik sendrom tanısı konulup HLH 2004 tedavi protokolü başlandı. Ön patoloji raporunun: periferik T hücreli lenfoma, nos olarak raporlanması sonrası 2 haftalık HLH 2004 tedavi protokolüne CHOEP protokolü ile devam edildi. EBV VCA Ig M:1.16 (≥11: pozitif), EBV VCA Ig G:49.2 (≥11: pozitif) olması ve klonalite sonucunun raporlanması ile beraber nodal EBV+ sitotoksik t hücreli lenfoma tanısı konuldu. Hastanın takiplerinde genel durumunda tekrar kötüleşme, septik tabloya ilerleme meydana geldi ve CHOEP kemoterapisinin 12. gününde hasta exitus oldu.
Referanslar
Swerdlow SH, Campo E, Pileri SA, Harris NL, Stein H, Siebert R, et al. The 2016 revision of the World Health Organization classification of lymphoid neoplasms. Blood, The Journal of the American Society of Hematology. 2016;127(20):2375-90.
Asano N, Suzuki R, Kagami Y, Ishida F, Kitamura K, Fukutani H, et al. Clinicopathologic and prognostic significance of cytotoxic molecule expression in nodal peripheral T-cell lymphoma, unspecified. The American journal of surgical pathology. 2005;29(10):1284-93.
Kato S, Yamashita D, Nakamura S. Nodal EBV+ cytotoxic T-cell lymphoma: A literature review based on the 2017 WHO classification. Journal of Clinical and Experimental Hematopathology. 2020;60(2):30-6.
Krenacs L, Smyth MJ, Bagdi E, Krenacs T, Kopper L, Rudiger T, et al. The serine protease granzyme M is preferentially expressed in NK-cell, γδ T-cell, and intestinal T-cell lymphomas: evidence of origin from lymphocytes involved in innate immunity. Blood, The Journal of the American Society of Hematology. 2003;101(9):3590-3.
Savage KJ, Ferreri AJ, Zinzani PL, Pileri SA. Peripheral T-cell lymphoma–not otherwise specified. Critical reviews in oncology/hematology. 2011;79(3):321-9.
Zinzani PL, Broccoli A. T‐Cell Lymphoproliferative Disorders. Postgraduate Haematology. 2015:524-36.
Lee S-Y, Stadanlick J, Kappes DJ, Wiest DL, editors. Towards a molecular understanding of the differential signals regulating αβ/γδ T lineage choice. Seminars in immunology; 2010: Elsevier.
Jeon YK, Kim J-H, Sung J-Y, Han JH, Ko Y-H, of the Korean HSG. Epstein-Barr virus–positive nodal T/NK-cell lymphoma: An analysis of 15 cases with distinct clinicopathological features. Human pathology. 2015;46(7):981-90.
Schmitz N, Trümper L, Ziepert M, Nickelsen M, Ho AD, Metzner B, et al. Treatment and prognosis of mature T-cell and NK-cell lymphoma: an analysis of patients with T-cell lymphoma treated in studies of the German High-Grade Non-Hodgkin Lymphoma Study Group. Blood, The Journal of the American Society of Hematology. 2010;116(18):3418-25.
Yamaguchi M, Kwong Y-L, Kim WS, Maeda Y, Hashimoto C, Suh C, et al. Phase II study of SMILE chemotherapy for newly diagnosed stage IV, relapsed, or refractory extranodal natural killer (NK)/T-cell lymphoma, nasal type: the NK-Cell Tumor Study Group study. J Clin Oncol. 2011;29(33):4410-6.
Ng S-B, Chung T-H, Kato S, Nakamura S, Takahashi E, Ko Y-H, et al. Epstein-Barr virus-associated primary nodal T/NK-cell lymphoma shows a distinct molecular signature and copy number changes. Haematologica. 2018;103(2):278.
Scott RB, Robb-Smith A. Histiocytic medullary reticulosis. The Lancet. 1939;234(6047):194-8.
Hale Ö. Hemofagositik Sendrom.thd.org.tr
Soy M, Atagündüz P, Atagündüz I, Sucak GT. Hemophagocytic lymphohistiocytosis: a review inspired by the COVID-19 pandemic. Rheumatology International. 2021;41(1):7-18.
Canna SW, Marsh RA. Pediatric hemophagocytic lymphohistiocytosis. Blood. 2020;135(16):1332-
Referanslar
Swerdlow SH, Campo E, Pileri SA, Harris NL, Stein H, Siebert R, et al. The 2016 revision of the World Health Organization classification of lymphoid neoplasms. Blood, The Journal of the American Society of Hematology. 2016;127(20):2375-90.
Asano N, Suzuki R, Kagami Y, Ishida F, Kitamura K, Fukutani H, et al. Clinicopathologic and prognostic significance of cytotoxic molecule expression in nodal peripheral T-cell lymphoma, unspecified. The American journal of surgical pathology. 2005;29(10):1284-93.
Kato S, Yamashita D, Nakamura S. Nodal EBV+ cytotoxic T-cell lymphoma: A literature review based on the 2017 WHO classification. Journal of Clinical and Experimental Hematopathology. 2020;60(2):30-6.
Krenacs L, Smyth MJ, Bagdi E, Krenacs T, Kopper L, Rudiger T, et al. The serine protease granzyme M is preferentially expressed in NK-cell, γδ T-cell, and intestinal T-cell lymphomas: evidence of origin from lymphocytes involved in innate immunity. Blood, The Journal of the American Society of Hematology. 2003;101(9):3590-3.
Savage KJ, Ferreri AJ, Zinzani PL, Pileri SA. Peripheral T-cell lymphoma–not otherwise specified. Critical reviews in oncology/hematology. 2011;79(3):321-9.
Zinzani PL, Broccoli A. T‐Cell Lymphoproliferative Disorders. Postgraduate Haematology. 2015:524-36.
Lee S-Y, Stadanlick J, Kappes DJ, Wiest DL, editors. Towards a molecular understanding of the differential signals regulating αβ/γδ T lineage choice. Seminars in immunology; 2010: Elsevier.
Jeon YK, Kim J-H, Sung J-Y, Han JH, Ko Y-H, of the Korean HSG. Epstein-Barr virus–positive nodal T/NK-cell lymphoma: An analysis of 15 cases with distinct clinicopathological features. Human pathology. 2015;46(7):981-90.
Schmitz N, Trümper L, Ziepert M, Nickelsen M, Ho AD, Metzner B, et al. Treatment and prognosis of mature T-cell and NK-cell lymphoma: an analysis of patients with T-cell lymphoma treated in studies of the German High-Grade Non-Hodgkin Lymphoma Study Group. Blood, The Journal of the American Society of Hematology. 2010;116(18):3418-25.
Yamaguchi M, Kwong Y-L, Kim WS, Maeda Y, Hashimoto C, Suh C, et al. Phase II study of SMILE chemotherapy for newly diagnosed stage IV, relapsed, or refractory extranodal natural killer (NK)/T-cell lymphoma, nasal type: the NK-Cell Tumor Study Group study. J Clin Oncol. 2011;29(33):4410-6.
Ng S-B, Chung T-H, Kato S, Nakamura S, Takahashi E, Ko Y-H, et al. Epstein-Barr virus-associated primary nodal T/NK-cell lymphoma shows a distinct molecular signature and copy number changes. Haematologica. 2018;103(2):278.
Scott RB, Robb-Smith A. Histiocytic medullary reticulosis. The Lancet. 1939;234(6047):194-8.
Hale Ö. Hemofagositik Sendrom.thd.org.tr
Soy M, Atagündüz P, Atagündüz I, Sucak GT. Hemophagocytic lymphohistiocytosis: a review inspired by the COVID-19 pandemic. Rheumatology International. 2021;41(1):7-18.
Canna SW, Marsh RA. Pediatric hemophagocytic lymphohistiocytosis. Blood. 2020;135(16):1332-
