A 37 year old male with T cell acute lymphoblastic leukemia with normal cytogenetics 46XY in second complete remission (CR2) after 8 cycles of HyperCVAD and 4 cycles of salvage nelarabine underwent myeloablative conditioning with cyclophosphamide TBI for a matched related donor allogeneic stem cell transplant from his brother. His day+100 bone marrow biopsy showed CR2 and was 100% donor. His post transplant course was unremarkable until he relapsed d+162. The immunosuppression was tapered off rapidly and 2 cycles of nelarabine and a donor lymphocyte infusion (1×107 CD3 cells/kg) were completed by d+254 and CR3 was achieved. He did well until d+608 when he presented with a month long history of dyspnea and recurrent bilateral large pleural effusions (despite multiple large volume thoracenteses) and anasarca including facial edema. He had gained 15 kilograms in 2 weeks. He had no fever, skin rash, sicca syndrome or lichen planus. Chest and abdomen CT showed massive bilateral pleural effusions, but no lung infiltrate, pericardial effusion or ascites or lymphadenopathy. The complete blood count, renal function and liver function tests were normal and there was normal hepatopedal flow in the main portal vein and its branches. The echocardiogram showed a left ventricular ejection fraction of 60% (normal) and there were no vegetations. The serum ANA, anti-Ro and anti-La were negative. The bone marrow biopsy showed no evidence of relapse of T cell ALL. The peripheral blood STR was 100% donor. The pleural fluid was transudative and cytology showed no malignancy. Pleural fluid flow cytometry showed no CD20+ B cells but did show polyclonal T cells with a CD4:CD8 ratio of 0.22. The pleural biopsy showed reactive mesothelial cells with mixed acute and chronic inflammation and no malignancy. Ebstein Barr virus in-situ hybridization and HHV-8 immunohistochemistry was negative on the pleural biopsy. All bacterial, fungal and viral (including CMV, EBV, HSV, adenovirus, VZV, HIV, BK) of the blood, urine and pleural fluid were unremarkable. A random skin biopsy of the forearm showed scleroderma. Methylprednisolone 2 mg/kg iv daily and therapeutic tacrolimus with extracorporeal photoporesis was begun with resolution of the pleural effusions and anasarca two months later. On d+903 he remains in CR3 and is on tapering courses of prednisone and tacrolimus without recurrence of the pleural effusions or anasarca. Conclusion: Isolated sterile pleural effusions with anasarca should be recognized as a rare feature of late onset chronic graft versus host disease so as to allow prompt initiation of immunosuppression. The patient shared the HLA antigens HLA DR15 and HLA DQW6 which are associated with autoimmune disease such as systemic lupus erythematosus suggesting that patients with this HLA combination may be more prone to this presentation.
No relevant conflicts of interest to declare.
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