037 Post-transplant lymphoproliferative disorder in skeletal muscle arising seven years after allogeneic stem cell transplant

2018 ◽  
Vol 89 (6) ◽  
pp. A15.3-A16
Author(s):  
Alice Powell ◽  
Joanne Sy ◽  
Yael Barnett ◽  
Simon Mosalski ◽  
Bruce Brew ◽  
...  

IntroductionPost-transplant lymphoproliferative disorder (PTLD) is a well-recognised serious complication of transplantation. However, it rarely affects muscle.MethodsA case report detailing presentation, evaluation, management and outcome of a patient with acute on chronic proximal myopathy on a background of allogeneic stem cell transplant is described. The patient was diagnosed with PTLD affecting skeletal muscle.ResultsA 54 year old man presented with painful proximal myopathy seven years after successful allogeneic stem cell transplant for acute myeloid leukaemia. He had been managed with immunosuppression for extensive cutaneous graft versus host disease (GVHD) from the time of transplant. Initial quadriceps muscle biopsy showed changes in keeping with GVHD and features suggestive of drug-related necrotising myositis. The painful myalgia evolved to include bulbar muscles with dysphagia despite pulse steroid and intravenous immunoglobulin therapy. A positron emission tomography (PET) scan demonstrated intense and extensive hyper-metabolism in multiple muscle groups in keeping with diffuse myositis with the most prominent activity involving neck muscles, the left shoulder girdle and left arm musculature. A second biopsy of the sternocleidomastoid demonstrated infiltration with haematolymphoid cells consistent with post-transplant lymphoproliferative disorder. Treatment with reduction in immunosuppression, rituximab and rehabilitation saw improvement in myalgia and weakness. Unfortunately, exacerbation of chronic GVHD ensued following reduction in immunosuppression and contributed to oesophageal stricturing eventually requiring a percutaneous endoscopic gastrostomy tube for feeding.ConclusionThis case highlights the complex morbidity of allogeneic stem cell transplant and the need to consider PTLD in the differential for patients who present with neurological symptoms.

2018 ◽  
Vol 22 (8) ◽  
pp. e13302 ◽  
Author(s):  
Csaba Kassa ◽  
Péter Reményi ◽  
János Sinkó ◽  
Krisztián Kállay ◽  
Gabriella Kertész ◽  
...  

2020 ◽  
Vol 4 (02) ◽  
pp. 49-51
Author(s):  
Tangia Muquith

Epstein –Barr virus related post-transplant lymphoproliferative disorder is a fatal and life-threatening complication because of immunocompromised state.1 Haemophagocytic lymphohistiocytosis is a sign of poor outcome in EBV associated PTLD after allogeneic stem cell transplantation. It is particularly common when in vivo T cell depletion strategies have been applied.2 In both situations, post-transplant lymphoproliferative disorder and Haemophagocytic lymphohistiocytosis, infection with EBV is the key mechanism. Here I present a case of 29 years old female with acute myeloid leukaemia after second allogeneic stem cell transplant, who developed PTLD complicated with Haemophagocytic lymphohistiocytosis secondary to Epstein – Barr virus (EBV) infection. Patient was treated with chemo immunotherapy and responded but ultimately died after 100 days of transplant. The association of HLH and EBV related PTLD is rare and data on outcome of these patients are limited with very high mortality.


2018 ◽  
Vol 11 (1) ◽  
pp. e226666 ◽  
Author(s):  
Brianne J Sullivan ◽  
Grace J Kim ◽  
Gabriel Sara

Post-transplant lymphoproliferative disorder (PTLD) is a recognised complication of solid and haematopoietic stem cell transplant. It consists of a heterogeneous group of lymphoid neoplasms that arises secondary to post-transplant immunosuppression. Although there is no definite standard of care for the optimal treatment for PTLD, rituximab, a monoclonal antibody, with and/or without chemotherapy (usually CHOP=cytoxan, doxorubicin, vincristine, prednisone) has become a routine part of the treatment of any CD20 (+) PTLD, with response rates similar to chemotherapy with decreased toxicity. A rare and often lethal, complication of rituximab therapy for PTLD is bowel perforation secondary to tumour lysis of lymphoma involving the intestine. A small number of cases of bowel perforation have been reported, with very few documented survivors. The risk for recurrent perforation in the setting of ongoing rituximab treatment is unknown. There is sparse data supporting how to best treat the survivors.


Blood ◽  
2007 ◽  
Vol 110 (11) ◽  
pp. 4719-4719
Author(s):  
John Quinn ◽  
Sajir Mohamedbhai ◽  
Marilyn Treacey ◽  
Shirley D’Sa ◽  
Amit Nathwani

Abstract High-dose methlyprednislone (HDMP) is active in refractory chronic lymphocytic leukaemia (CLL), and rituximab, although showing limited efficacy as a single agent, is effective when used in combination with other cytotoxic agents. Early relapse (<12 months) after purine-analogue based treatment poses a difficult management problem as older patients may be unable to tolerate treatment intensification. This retrospective audit examines the outcome in patients with advanced, refractory/relapsed CLL treated with a combination of high-dose steroids and rituximab. Eleven patients with CLL were treated with rituximab (375mg/m2) and high-dose steroids between 2003 and 2007. Ten patients had advanced and/or refractory disease and one patient had severe autoimmune haemolytic anaemia complicating early-stage CLL. Median age was 70 (range 54–82) and there were 7 male and 4 female patients. Nine patients had Binet stage C disease. Six of the 7 patients for whom results were available had germ-line variable heavy chain immunoglobuliun genes. Median number of prior treatments was 2 (range 1–6) with 9 patients having already received a fludarabine based-regimen. Six patients received a combination of rituximab and high-dose methylprednisolone (1gm/m2 on days 1–5) and 5 patients received a combination of rituximab and high-dose dexamethasone (40mg daily on days 1–4) and. Cycles were repeated every 28 days and the median number of cycles received was 4 (3–6). Antiviral and anti-PCP prophlaxis with aciclovir and co-trimoxazole was routinely prescribed. Response was assessed according to the NCI working group criteria. There was one complete response (CR) and 7 partial responses (PR). Furthermore, 2 of the patients who achieved a PR were successfully salvaged prior to reduced-intensity allogeneic stem-cell transplant and both remain in complete remission (CR) post-transplant. The other 3 patients had minor responses that did not meet NCI response criteria. Median duration of response was 13 months (6–35), excluding the 2 patients who received allogeneic transplants. Three patients required hospital admission with infective complications during treatment. No other significant toxicity was observed. 3 patients have died, none of whom had attained a PR. In conclusion, we found that rituximab in combination with high-dose steroids is a safe and well-tolerated combination in patients with advanced refractory CLL. One patient achieved a CR and we observed a PR in 7/11 patients. Importantly, 2 of these patients were successfully salvaged prior to reduced-intensity conditioning allogeneic stem-cell transplant and both remain in CR at 4 and 12 months respectively post-transplant.


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