Angioimmunoblastic T-cell lymphoma with intramedullary production of platelet-derived growth factor and possibly complicating myelofibrosis: report of a case with review of the literature

Abstract

A 65-year-old man was diagnosed with angioimmunoblastic T-cell lymphoma (AITL) with bone marrow (BM) infiltration and myelofibrosis (MF). The BM infiltration and the condition of the MF improved following CHOP therapy (cyclophosphamide hydrate, doxorubicin hydrochloride, vincristine sulfate, and prednisolone). After complete remission was achieved, early central nervous system recurrence was noted, with no evidence of BM infiltration or MF. The lymph nodes and BM were examined for cytokines by immunohistochemical staining with monoclonal murine antibodies. The lymphoma cells were positive only for platelet-derived growth factor (PDGF) and negative for basic fibroblast growth factor, fibronectin, vascular endothelial growth factor, transforming growth factor-β (TGF-β), tumor necrosis factor α, interleukin-1β, and interleukin-6. It was thus inferred that the lymphoma cells producing PDGF caused the MF, and that the absence of MF at relapse may have been attributable to the absence of BM infiltration. There have been seven reported cases of AITL with intercurrent MF, although cytokine data (elevations of blood PDGF and TGFβ levels) are available for only one case. The present report is to our knowledge the only report of a case of AITL complicated by MF for which the results of immunohistostaining with anticytokine antibodies are available.

Simultaneous Follicular Lymphoma and Myelofibrosis: Report of a Case with Review of the Literature

“…Meanwhile, the b-FGF produced by the bone marrow megakaryocytes was considered as an irrelevant factor of the development of concurrent MF. 15 On the contrary, another report showed that b-FGF played an important role in the case of MF complicated with peripheral T cell lymphoma (PTCL). 16 In addition, there were a few other case reports consenting to the contribution of cytokines.…”

“…7 , 8 The development of the MF are associated with the production of various cytokines, such as TGF-β, IL-1, PDGF, b-FGF, VEGF. 13 , 15 However, the accurate cause of MF with concurrent lymphoma is not clear yet. It was reported that MF may be attributable to the lymphoma cells producing PDGF in a case of angioimmunoblastic T-cell lymphoma (AITL) with complicating MF.…”

Diffuse Large B-cell Lymphoma during Corticosteroid Therapy for TAFRO Syndrome

“…We first attempted to rule out SLE because while some clinical symptoms (pleural effusion, ascites, proteinuria, hematuria and thrombocytopenia) met the classification criteria for SLE ( 17 ), anemia was not caused by hemolysis and no specific antibodies were detected. AITL also causes various systemic symptoms, such as a fever, effusion and immunological abnormalities, however, myelofibrosis is rarely observed ( 18 , 19 ). Although AITL was not initially completely ruled out, lymphoma cells were not detected in the ascites or bone marrow.…”

Surgical tactics and treatment results at spinal lymphomas

“…Ха-рактерной для лимфом является ремиссия по данным МРТ после назначения стероидных препаратов [7]. Гистологический диагноз лимфомы целесообразно фенотипировать по данным иммуногистохимических исследований для индивидуализации лечения [8]. Удаление очага с последующим облучением, корти-костероидной терапией и системной химиотерапией часто обеспечивает полную ремиссию при наличии первичной неходжкинской лимфомы ЦНС, однако продолжительность жизни больных составляет в среднем от 10 до 18 мес [5,6].…”

“…Стандартная меж-дународная схема лечения предусматривает удаление опухоли, получение адекватного гистологического диагноза, последующее проведение химиотерапии, адъювантной лучевой терапии а также применение кортикостероидов [2,5,6]. Результаты лечения лим-фомы Ходжкина лучше, здесь продолжительность жизни варьирует от 5 месяцев до 15 лет [2,8]. Однако часто поражение ТОСМ возникает после длительного лечения лимфомы Ходжкина, когда химиотерапия уже неэффективна, что обусловливает небольшую продолжительности жизни после возникновения процессов в спинном мозге [4,6].…”