Reduction of immunosuppression combined with whole-brain radiotherapy and concurrent systemic rituximab is an effective yet toxic treatment of primary central nervous system post-transplant lymphoproliferative disorder (pCNS-PTLD): 14 cases from the prospective German PTLD registry.

Post-transplant lymphoproliferative disorders (PTLD) exclusively affecting the central nervous system-primary CNS-PTLD (pCNS-PTLD)-are rare. There is no standard therapy, and previous case series have included heterogeneous treatment approaches. We performed a retrospective, multi-centre analysis of 14 patients with pCNS-PTLD after solid organ transplantation (SOT) treated in the prospective German PTLD registry with reduction of immunosuppression (RI), whole-brain radiotherapy (WBRT), and concurrent systemic rituximab between 2001 and 2018. Twelve of fourteen patients were kidney transplant recipients and median age at diagnosis was 65 years. Thirteen of fourteen cases (93%) were monomorphic PTLD of the diffuse large B-cell lymphoma type, and 12/13 were EBV-associated. The median dose of WBRT administered was 40 Gy with a median fraction of 2 Gy. The median number of administered doses of rituximab (375 mg/m2) IV was four. All ten patients evaluated responded to treatment (100%). Median OS was 2.5 years with a 2-year Kaplan-Meier estimate of 63% (95% confidence interval 30-83%) without any recorded relapses after a median follow-up of 2.6 years. Seven of fourteen patients (50%) suffered grade III/IV infections under therapy (fatal in two cases, 14%). During follow-up, imaging demonstrated grey matter changes interpreted as radiation toxicity in 7/10 evaluated patients (70%). The combination of RI, WBRT, and rituximab was an effective yet toxic treatment of pCNS-PTLD in this series of 14 patients. Future treatment approaches in pCNS-PTLD should take into account the significant risk of infections as well as radiation-induced neurotoxicity.

Low-dose total skin electron beam therapy for refractory cutaneous CD30 positive lymphoproliferative disorders.

We describe a case of a 48-year-old woman with a refractory cutaneous CD30 positive lymphoproliferative disorder treated successfully with total skin electron beam radiotherapy (TSEBT).

Role of Radiotherapy in Post-transplant Lymphoproliferative Disorders: Three Case Reports and Review of the Literature.

Post-transplant lymphoproliferative disorder (PTLD) is an aggressive malignancy that occurs in patients who have undergone solid organ transplantation or hematopoietic stem cell transplantation. It develops as the result of uncontrolled cell proliferations owing to reduced immunological surveillance. PTLD may occur with a various spectrum of clinical presentations, including both localized and extensive disease. Management can be significantly variable according both to the clinical presentation and to the histologic features. The most important systemic treatment strategies are reduction of immunosuppressive therapy, chemotherapy, anti B-cell antibodies, especially rituximab and cytokine-based therapies. The localized form of PTLD could be efficiently treated, and potentially cured, with surgery or radiotherapy (RT). Involved site RT may be a feasible effective option for the treatment of patients with PTLD, given the excellent radio-sensitivity of lymphoid disorders. In this report, we describe 3 adult patients with PTLD treated with moderate-dose RT (24-36 Gy) having a good local control with negligible toxicity. We also review the literature data on the role of radiation therapy in this particular setting.

Indolent T-cell lymphoproliferative disorder of the stomach successfully treated by radiotherapy.

Successful treatment of indolent T-cell lymphoproliferative disorder of the gastrointestinal tract (ITLPDGI) by chemotherapy is rare and watchful waiting is often performed for asymptomatic patients. We report a case of ITLPDGI successfully treated by involved field radiotherapy (IFRT). The patient presented with slow ITLPDGI localised to the stomach with mild symptoms. IFRT (30 Gy/20f) was administered, after which endoscopy revealed resolution of lesions and blood vessel appearance, and absence of proliferating abnormal lymphocytes was confirmed by biopsy. The patient remains lymphoma-free 1 year post-treatment. Although long-term follow-up and additional cases are essential for the evaluation of IFRT as a treatment option for localised ITLPDGL, complete remission after relatively low-dose IFRT is promising, particularly as this has been rarely achieved by chemotherapy.

Experiences in the treatment of refractory chylothorax associated with lymphoproliferative disorders.

BACKGROUND: Chylothorax is a rare condition which can be associated with malignant lymphoproliferative disorders (LPDs). We retrospectively analyzed the results of the conservative treatment of 10 patients with persistent non-traumatic malignant chylothorax. RESULTS: Conservative treatment lead to a decline of chylothorax after mean of 66 days and consisted of the treatment of the underlying disease and of simultaneous long-term supportive care (drainage of the thoracic cavity, dietary measures and nutrition management). In most cases (80%), chylothorax disappeared only after a successful therapeutic response of the underlying disease. Low-dose radiotherapy had very good effects in two patients. CONCLUSION: Conservative treatment of malignant chylothorax can be considered a suitable method. Based on our results, successful treatment of the lymphoproliferative disorder seems to be a very important factor for the disappearance of chylothorax.

Primary cutaneous post liver transplant lymphoproliferative disorder (PTLD) successfully treated with radiotherapy.

AIM: A case of primary cutaneous post liver transplant lymphoproliferative disorder (PTLD) mimicking squamous cell carcinoma-in-situ is presented. METHODS: Lesions mimicking SCC-in-situ were confirmed to be PTLD on histopathology and immunohistochemistry. Immunosuppression was gradually reduced and the lesions were successfully treated with 50 gray (Gy) in 25 fractions of radiotherapy. RESULTS: There was no recurrence of lesions at 3 months follow-up. CONCLUSION: Radiotherapy is an effective form of cutaneous directed treatment for localized PTLD.

Primary Cutaneous CD4+ Small/Medium Pleomorphic T-Cell Lymphoproliferative Disorder: A Case Series.

BACKGROUND: Primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorder (CD4+ PCSM-LPD) is defined by a predominance of small- to medium-sized CD4+ pleomorphic T cells and a favorable clinical course. OBJECTIVE: We performed a retrospective analysis of 6 patients with CD4+ PCSM-LPD and reviewed the literature to address questions about its diagnosis, treatment, and prognosis. METHODS: Patients were 3 men and 3 women with a median age of 50 years. All patients presented with a single erythematous nodule, localised on the head in 4 patients and the upper trunk in 2 cases. No patients showed extracutaneous disease at any evaluation. Histopathologic features were characterised by nodular, diffuse, or, in 1 case, a superficial dense infiltrate of small/medium-sized pleomorphic CD4+/PD1+ T lymphocytes. T-cell receptor clonality was demonstrated in 5 cases. Treatment was surgical excision in 5 cases and radiotherapy in 1 case. RESULTS: All patients achieved complete resolution without relapses, during a median follow-up of 3 years. A review of the literature confirmed that CD4+ PCSM-LPD presents predominantly with a solitary nodular lesion on the face, neck, or upper trunk in adult patients. Surgical excision is the preferred treatment. Spontaneous resolution after biopsy may occur. CONCLUSIONS: CD4+ PCSM-LPD is a rare disorder with a favorable course.

[Current questions in the radiotherapy of lymphoproliferative tumors]. / A limfoproliferatív daganatok sugárkezelésének kérdéses területei.

Radiation therapy traditionally plays a major role in the treatment of lymphoproliferative diseases. Diagnostics and treatment of these tumors improved tremendously in the recent decades. Molecular diagnostics is able to discriminate its subtypes more precisely than ever and opens the possibility of the introduction of targeted medicines. Imaging, especially functional imaging now has an established role in forming treatment strategy. Radiation therapy showed substantial technical development too. As a consequence of these, the role, dose and technique of radiotherapy changes instantly. In this short review we discuss situations which clinicians, both hemato-oncologists and radiation oncologist may face day by day. These are: the changing role of radiation therapy in early Hodgkin's disease, including dose and filed size reduction and PET-driven radiation therapy; the use of radiation in advanced Hodgkin's disease; the role of radiation therapy of diffuse large B-cell lymphoma in the light of the use of rituximab; and finally the use of modern radiation therapy techniques like intensity-modulated radiation therapy or particle therapy.

Post-transplant lymphoproliferative disorder in the pelvis successfully treated with consolidative radiotherapy.

BACKGROUND: Post-transplant lymphoproliferative disorders (PTLDs) are aggressive malignancies which represent one of the major post-transplant complications. However, treatment options vary significantly and localized disease may be curatively treated with radiotherapy (RT) or surgery. We report a case of recurrent rectal PTLD, which was successfully treated by chemoimmunotherapy followed by RT. CASE PRESENTATION: We describe a patient who developed a rectal lymphoproliferative lesion 11 years after kidney transplant, which was successfully treated with consolidative RT using 25.4 Gy sequential to chemoimmunotherapy (R-CHOP). RT was well tolerated and the patient showed no signs of grade 3 or 4 toxicity. This patient is free of recurrence 52 months after RT, with an overall survival of 62 months since diagnosis. CONCLUSION: Conventionally fractionated moderate-dose RT appears to be a tolerable and effective treatment option for localized PTLD if a sufficient systemic treatment cannot be applied.

Cataracts after Low-Dose Radiotherapy for Lymphoproliferative Disease of the Ocular Adnexa.

PURPOSE: To investigate the interval between low-dose radiotherapy and radiation-induced cataracts, and the factors affecting this interval, in patients with lymphoproliferative disease of the ocular adnexa. METHODS: A retrospective case series of 73 eyes of 59 patients who underwent radiotherapy from 1996 to 2005 with total doses ranging from 24 to 30 Gy was conducted. We investigated the relationships between the radiation-associated cataract formation intervals and age, gender, diabetes, and the use of corticosteroids. RESULTS: The mean interval was 36 months. None of the patients with lens shield developed cataracts. Age was inversely and significantly and gender was not significantly associated with the interval from first radiotherapy to cataract occurrence. The intervals did not differ significantly according to the presence of diabetes and corticosteroid therapy. CONCLUSIONS: The mean interval from the start of radiation therapy to radiation-related cataract formation was 36 months and age was a significant factor affecting this interval.

Single-Fraction Radiotherapy for CD30(+) Lymphoproliferative Disorders.

OBJECTIVES: CD30(+) lymphoproliferative disorder is a rare variant of cutaneous T-cell lymphoma. Sustained complete response following first-line treatments is rare. This retrospective review evaluates the response of refractory or recurrent lesions to palliative radiation therapy. METHODS: The records of 6 patients with 12 lesions, treated with radiation therapy, were reviewed. All patients received previous first-line treatments. Patients with clinical and pathological evidence of symptomatic CD30(+) lymphoproliferative disorder, with no history of other cutaneous T-cell lymphoma variants, and with no prior radiation therapy to the index site were included. RESULTS: The median age of patients was 50.5 years (range, 15-83 years). Median size of the treated lesions was 2.5 cm (range, 2-7 cm). Four sites were treated with a single fraction of 750-800 cGy (n = 3) and 8 sites were treated with 4000-4500 cGy in 200-250 cGy fractions (n = 3). Radiation therapy was administered with electrons and bolus. Median follow-up was 113 months (range, 16-147 months). For all sites, there was 100% complete response with acute grade 1-2 dermatitis. CONCLUSIONS: For recurrent and symptomatic radiation-naïve CD30(+) lymphoproliferative disorder lesions, palliative radiation therapy shows excellent response. A single fraction of 750-800 cGy is as effective as a multifractionated course and more convenient.

Successful treatment of monomorphic primary central nervous system post-transplantation lymphoproliferative disorder 5 years after kidney transplantation.

A 31-year-old man underwent living-related kidney transplantation in 2004 as a consequence of primary focal segmental glomerulosclerosis (FSGS). Four years after the transplantation, we confirmed nephrotic syndrome caused by recurrent FSGS. We performed plasmapheresis and low-density lipoprotein adsorption. We also combined steroid therapy with a reduction in the dose of tacrolimus and an increased dose of mycophenolate mofetil. The nephrotic syndrome improved dramatically with this combined therapeutic approach. However, 10 months after these treatments, he revisited our hospital because of altered consciousness. We detected multiple tumor masses in his brain that were ring enhanced on contrast magnetic resonance imaging. Consequently, we suspected primary central nervous system post-transplantation lymphoproliferative disorder (CNS-PTLD). We performed a craniotomy to biopsy the brain tumors. The biopsy specimen showed Epstein-Barr virus-associated diffuse large B-cell lymphoma. There is no definitive treatment for CNS-PTLD. Therefore, we treated the primary CNS-PTLD successfully with whole-brain radiation and discontinuation of immunosuppression therapy.

Patterns of cancer occurrence in different regions of West Bengal--a hospital based study.

The incidence of cancer has been rising steadily in the third world countries including India. The patterns of cancer incidence reflect the racial, cultural and pharmacogenomic diversity within populations and nowhere is this diversity more striking than in the Indian subcontinent. This article shows the diversity in patterns of incidence of major cancers across three medical college hospitals in the state of West Bengal in India. All the data were collected from the period between 2001 and 2005. The results show a striking variation of incidence of major cancers in the urban, semi-urban and rural parts of the same state. Indeed the differences of the patterns are explainable by the cultural and socioeconomic differences within the populations from which the study samples are drawn. This constitutes the largest single hospital based data collected from this part of the world till date and will help in re-evaluation of cancer control programmes promulgated by the health authorities of the region.

Radiotherapy is the best treatment method in post transplant lymphoproliferative disorders localizing in brain: a review of the literature.

BACKGROUND: Localization of post transplant lymphoproliferative disorders (PTLD) in the central nervous system (CNS) is a rare but life-threatening complication of transplantation. In the current study, we sought to aggregate data of PTLD in the series existing in the literatures on brain localization of PTLD and to concentrate on the management methods of the disease to compare and find the best treatment strategies in these patients. MATERIAL/METHODS: We conducted a thorough search of the literature to find treatment strategies employed to manage CNS involvement by PTLD. Data were pooled and standardized and reanalyzed. RESULTS: Overall 79 patients with CNS PTLD were entered into analysis. Patients undergone radiotherapy represented a significant superior outcome compared to that of patients who had not received radiotherapy (p<0.05). other treatment strategies had no significant impact on the survival (p>0.2 for all). One and five years survival rates for CNS involved PTLD patients who underwent radiotherapy were 71% and 37%, respectively; compared to 41% and 28%, respectively, for the control group. CONCLUSIONS: In the current study, we found that radiotherapy is an effective method of treatment for organ recipients who develop PTLD within central nervous system during their post transplant period. No beneficial effect for chemotherapy, interferon alfa, or a combination of them with radiotherapy was detected. We recommend using radiotherapy in all transplant patients developing CNS PTLD.

Novel therapeutic option for orbital atypical lymphoid hyperplasia.

Ocular lymphoid tumours represent a spectrum of lymphoproliferative disease and can be subdivided into benign or reactive lymphoid hyperplasia, indeterminate or atypical lymphoid proliferations and malignant lymphoma. Treatment options include a wait and watch approach, systemic steroids, local radiotherapy or systemic chemotherapy. We describe a case of bilateral atypical lymphoid hyperplasia treated successfully with combination immunotherapy and radiotherapy. A 60-year-old lady presented with proptosis and left supra-orbital mass and was diagnosed to have bilateral atypical lymphoid hyperplasia. She had extensive extraocular facial infiltrates but no other sites of involvement on staging investigations. She was treated with eight doses of rituximab 375 mg/m² at weekly intervals with a good partial response, followed by consolidative radiotherapy. Rituximab may be an effective treatment adjunct/alternative for patients with atypical lymphoid hyperplasia of the orbit, particularly where widespread lesions preclude the use of initial radiotherapy.

Radiation therapy for B-cell cutaneous lymphoid hyperplasia.

Recent work has demonstrated that B-cell cutaneous lymphoid hyperplasia (BCCLH) lies in a spectrum of B-cell lymphoproliferative disorders that can progress to primary cutaneous B-cell lymphoma (CBCL). In light of this work, definitive therapy with methods such as radiotherapy is an important part of the treatment strategy. Few outcome data exist for patients with treatment-resistant BCCLH. We present a case study of a 63-year-old woman with BCCLH who failed immunomodulatory treatment but responded well to an aggressive course of radiotherapy. After 18 fractions of 6 MeV electron beam therapy with 200 cGy per fraction, the patient has been recurrence-free for 3 years. Acute toxicity was limited to Radiation Therapy Oncology Group grade II skin toxicity, which resolved within 1 month of treatment.

[Radiotherapy of inguinal lymphorrhea after vascular surgery. A retrospective analysis]. / Die Strahlentherapie inguinaler Lymphfisteln nach gefässchirurgischen Eingriffen. Eine retrospektive Analyse.

BACKGROUND AND PURPOSE: The formation of inguinal lymphorrhea following vascular surgery is a rare but potentially serious problem with an incidence of about 2%. There is no consensus on the most effective treatment for groin lymphorrhea. In a retrospective analysis the usefulness of irradiation in the treatment of inguinal lymph fistulas was investigated. PATIENTS AND METHODS: From 08/1997 to 12/2000, 28 patients with inguinal lymph fistulas were irradiated postoperatively (4th-19th day) with a single dose of 3 Gy up to a total dose of 9 Gy on 3 consecutive days using 120- to 300-kV photons. Three further patients received 2 x 4 Gy and 3 x 5 Gy, respectively, due to an interposed weekend. RESULTS: Secretion volume at the beginning of radiotherapy varied between 50 and 650 ml daily (mean 203 ml, median 175 ml), at the end of radiotherapy between 0 and 350 ml (mean 126 ml, median 120 ml). 3/28 lymph fistulas had resolved during radiotherapy. In 17/28 patients (60.7%) the drains could be removed within 10 days, in further 10/28 patients (35.7%) within 10-20 days after the end of radiotherapy. CONCLUSION: Overall, irradiation of inguinal lymph fistulas proved to be an effective and well-tolerated treatment, facilitating removal of fistula drains within 10-20 days (mean 10.5, median 7 days) after the completion of radiotherapy, thus appearing a good alternative to other conservative treatment modalities.