Rapidly fatal post-allogeneic stem cell transplant lymphoproliferative disorder presenting with skin and bone marrow involvement

Pathology ◽  
2013 ◽  
Vol 45 (1) ◽  
pp. 81-83 ◽  
Author(s):  
Robin Gasiorowski ◽  
John Gibson ◽  
Geoff Watson ◽  
Judith Trotman ◽  
Stephen Larsen
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.


Cytotherapy ◽  
2013 ◽  
Vol 15 (4) ◽  
pp. S6
Author(s):  
I. McNiece ◽  
S. Sivajothoi ◽  
E. Shpall ◽  
N. Kenyon ◽  
M. Korbling ◽  
...  

2019 ◽  
Vol 37 (15_suppl) ◽  
pp. e18503-e18503
Author(s):  
Brian Pham ◽  
Rasmus Hoeg ◽  
Nisha Hariharan ◽  
Kathyryn Alvarez ◽  
Aaron Seth Rosenberg ◽  
...  

e18503 Background: There is no standard treatment for acute myeloid leukemia (AML) or myelodysplastic syndrome (MDS) relapsing after allogeneic stem cell transplant (SCT). Options include combination chemotherapy, withdrawal of immunosuppression, donor lymphocyte infusion (DLI), and second SCT. Previous studies have used fludarabine, cytarabine, and granulocyte colony stimulating factor (FLAG) or second SCT separately for salvage therapy. Our institution uses FLAG followed by SCT from the same donor (FLAG/SCT) in this setting. We report a retrospective study of FLAG/SCT in MDS and AML patients relapsed after SCT. Methods: Patients who received FLAG/SCT for treatment of relapsed AML or MDS between 2009 and 2018 were identified using the bone marrow transplant database at University of California Davis. Their baseline characteristics and outcomes were determined using the electronic medical record. Descriptive statistics and Kaplan-Meier survival analysis were used to describe patients, rates of graft-versus-host disease (GvHD) and estimate survival times. Results: Nineteen patients received FLAG/SCT for AML (n=18) and MDS (n=1). Median time to relapse from first SCT was 145 days (range 41 to 960 days). Prior to FLAG/SCT, 17 patients had medullary relapse (median bone marrow blasts 27%; range 7-85%). Two patients had extramedullary relapse. Eighteen (94.7%) patients achieved complete remission (CR) after FLAG/SCT. Median follow-up time was 354 days from the first day of FLAG/SCT (range 7 to 2144 days). Six patients (31.6%) relapsed with median time to relapse of 334 days (range 78 to 679 days) after treatment. Overall survival at 2 years was 52.5%. Causes of death were relapsed AML (n=4; 21.1%), infection (n=4; 21.1%) complications of GvHD (n=3,15.8 %), and brain herniation (n=1, 5.3%). Acute GvHD grade I-IV and new onset chronic GvHD occurred in thirteen (68.4%) and eight patients (42.1%), respectively. Conclusions: FLAG/SCT for AML and MDS relapsing after SCT resulted in a high remission rate. The overall survival of over two years suggests that the second SCT augmented the graft versus leukemia effect. The GvHD rate increased after second SCT, but the rate and severity were manageable. FLAG/ SCT is a reasonable option for relapsed AML and MDS after SCT.


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

Blood ◽  
2012 ◽  
Vol 120 (21) ◽  
pp. 1972-1972
Author(s):  
Kitsada Wudhikarn ◽  
Pinyo Rattanaumpawan ◽  
Margarida M Silverman

Abstract Abstract 1972 Introduction: Relapse after allogeneic stem cell transplant (SCT) is a major cause of morbidity and mortality of acute myeloblastic leukemia (AML) patients. Most relapses primarily involve bone marrow whereas extramedullary (EM) relapses are relatively uncommon. Data on natural history, optimal management and treatment outcome in EM relapse following allogeneic SCT are limited. Method: We retrospectively reviewed 130 AML patients who underwent allogeneic SCT at the University of Iowa Hospitals and Clinics between January 2003 and December 2010. Pre-transplant baseline characteristics and post-transplant variables were abstracted. Patterns of relapse after allotransplant, management and outcome were reported. Result: Among 130 AML patients who underwent allogeneic SCT, we identified 61 (47%) relapsed AML after allotransplant. Of 61 relapsed cases, there were 7 (12%) isolated EM, 44 (72%) isolated bone marrow (BM) and 10 (16%) concurrent BM + EM relapses. Among 17 EM relapsed cases, there was a higher prevalence of acute monocytic leukemia subtype (41.5% VS 2.3%, odd ratio [OR] = 4.1, p=0.03), moderate to severe chronic graft versus host disease (GVHD) (35.3% VS 4.6%, OR=2.3, p=0.01) compared to non-EM relapsed patients. Median time to relapse in EM cases was significantly longer than non-EM group (428 days, 95% confidence interval [CI] 203–652 VS 162 days, 95%CI 100–231, p=0.003). EM relapse sites included 1 pulmonary (6%), 3 (18%) genitourinary, 7 (41%) skin/soft tissue, 2 (12%) gastrointestinal and 4 (23%) multisystem involvement. Of 17 EM relapsed cases, 12 (71%) had immunosuppression withdrawn, 13 (76%) received systemic chemotherapy with/without donor lymphocytic infusion and 7 (41%) had radiation therapy to EM relapse sites. The 6-month survival after relapse of EM patients was significantly higher than non-EM group (29.4%, 95%CI 10.7–51.2 VS 6.8%, 95%CI 1.8–16.7, p=0.014). Further subgroup analysis showed that isolated EM relapsed group had significantly higher 6-month survival after relapse than systemic relapse group (isolated BM or concurrent EM + BM) (42.9%, 95%CI 9.8–73.4 VS 9.3%, 95%CI 3.4–18.7, p=0.008). Table 1 compares the management and outcome between isolated EM relapse and concurrent BM + EM relapse. At the time of analysis, three (18%) of 17 EM relapsed cases (all of which were isolated EM patients) achieved disease controlled/remission and remained alive at 10, 13 and 19 months following relapse respectively. Cox proportional hazards analysis identified low hematopoietic cell transplant comorbidity index (HR 0.86, 95%CI 0.74–0.99, p=0.038), relapse after 180 days (HR 0.05, 95%CI 0.01–0.16, p<0.001) and presence of chronic GVHD (HR 3.57, 95%CI 1.02–12.45, p=0.04) to be associated with favorable survival after relapse. Conclusion: EM relapse of AML after alloSCT presents later than bone marrow relapse and occurs despite the presence of GVHD, especially chronic GVHD. As transplant survival has improved, increasing EM relapses have been observed. Although appropriate management is unknown, prognosis of EM relapse is better than systemic relapse and durable remission can be achieved. Disclosures: No relevant conflicts of interest to declare.


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