237
￭ Sat-363 ￭
ESHAP with Roflumilast as second-line chemotherapy for refractory or relapsed DLBCL
부산대학교병원
*
김철식, 신호진, 김도영
Background/Aims: In patients with primary refractory or relapsed diffuse large B cell lymphoma (DLBCL), disease remission status prior to stem cell transplantation (SCT) influences the future out- come, such as overall survival (OS) and progression free survival (PFS). In several recent studies, Roflumilast (PDE-4 inhibitor) with standard chemotherapy regimen shows better outcome in ag- gressive B-cell neoplasm, such as DLBCL. But the exact mechanism is still unclear According to previous studies, patients who treated with 2nd line ESHAP chemotherapy at refractory or relapsed DLBCL disease status, show 60-70% of OR (Overall Response) rate including 20-40 % of CR (Complete Response) rate. But there are few studies about outcome and prognosis of Roflumilast-ESHAP regimen in refractory & relapsed DLBCL. In this study, we analyze the re- mission rate, OS, PFS and toxicity of roflumilast combined with ESHAP regimen, compare with previous classical ESHAP regimen. Methods: Fourteen patients who had previously received 6 cy- cles of R-CHOP were administered roflumilast (500mcg qd) with classical ESHAP regimen (median 4 cycles, range1~6). This study was conducted from December,2017 to July,2018 in Pusan National University Hospital Results: The overall response rate was 62% (CR 31%, PR 31%). The 3-month OS rate was 84.4% and PFS rate was 76.9%. The 6-month OS rate was 76.9% and PFS rate was 67.3%. In a subgroup analysis, double & triple expressor group (two or more of myc, bcl-2 and bcl-6 positivity) shows shorter OS (3.5 month vs 8.0 month, p=0.007). Median f/u period was 5.6 month and the median survival end points have not been reached. In toxicity analysis, compared with previous studies, roflumilast combined with ESHAP regimen shows higher rate of neutropenia (57%) and thrombocytopenia (71%), but the rate of febrile neutropenia was tolerable (7%).
Conclusions: Compared with previous studies, ESAHP with roflumilast regimen shows similar OR and CR rate in relapsed or refractory DLBCL patients, but shows higher rate of toxicity with toler- able rate of febrile neutropenia
￭ Sat-364 ￭
DHS in a patient who recieved heart transplantation for DCMP: a cae report
서울아산병원
*
황영석, 최은지
Dehydrated hereditary stomatocytosis in a patient who received heart transplantation for dilated cardiomyopathy: A case report Young-Suk Hwang, Eun-Ji Choi Department of Internal Medicine, Department of Hematology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea Dehydrated hereditary stomatocytosis (DHS) is a rare type of inherited hemolytic anemia (HA) caused by increased permeability of red blood cell (RBC) membrane to cations. Patients with DHS typically present with compensated HA and associated complications including splenomegaly, gallstone, and iron overload. Here we report a case of DHS diagnosed after heart transplantation (HTPL). A 26-year-old woman visited hematology outpatient clinic with chronic anemia aggravated after HTPL. She was diagnosed with nonimmune HA and start- ed folic acid supplementation 25 years ago; the osmotic fragility test, hemoglobin electro- phoresis, RBC membrane protein analysis, enzyme deficiency test, anti-globulin test, and CD55 flow cytometry were all negative. Four years later, she was diagnosed with congestive heart fail- ure (CHF) due to dilated cardiomyopathy (DCMP). She had no known family history of hemato- logic or genetic disorders. Routine complete blood count showed normocytic anemia with in- creased reticulocytes. The peripheral blood smear showed anisopoikilocytosis and stomatocy- tosis (Figure 1A). Bone marrow (BM) aspirate and biopsy confirmed a hypercellular marrow with dysplasias (Figure 1B, 1C). Karyotype was normal and Fanconi anemia screening test dem- onstrated increased chromosomal breakage. Diagnostic exome sequencing revealed a pathogenic mutation of PIEZO1 (c.7483_7488dup, E2496ELE) with allele frequency of 0.4, consistent with the diagnosis of DHS. BM dysplasia was considered as consequences of immunosuppressants and sustained hemolysis. The familial genotyping showed no abnormality, determining a de no- vo mutation of the patient. We present a first case of DHS with mutation of PIEZO1 gene.
Although the association between DHS and DCMP seems unclear, PIEZO1 may be involved in the pathogenesis of DCMP which is known as stretch-activated ion channels in cardiac myocytes.
