Hägglund Hans, Yavuz Akif Selim, Dreimane Arta, Malm Claes, Sundin Anders, Sander Birgitta, Nilsson Gunnar
Department of Medical Sciences, Uppsala University, Uppsala, Sweden.
Section of Hematology, Uppsala University Hospital, Uppsala, Sweden.
Eur J Haematol. 2021 Feb;106(2):290-293. doi: 10.1111/ejh.13528. Epub 2020 Dec 1.
Advanced systemic mastocytosis is a relatively rare entity where allogeneic stem cell transplantation can lead to the cure of the disease in selected patients. Delayed incomplete responses with graft-versus-mastocytosis effect were published in a few cases. In this particular patient's report, we describe the direct evidence and potency of graft-versus-mastocytosis effect of donor lymphocyte infusions in a patient with systemic mastocytosis with associated hematological neoplasm (SM-AHN). In a 53-year-old female patient, an allogeneic stem cell transplantation after conventional induction treatment was performed for transformed acute myeloid leukemia (AML) during the course of polycythemia vera. After 6 years of remission period of AML and PV, the patient developed aleukemic mast cell leukemia and JAK2-positive myeloproliferative neoplasm (SM-AHN). We were able to achieve a sustained complete remission of SM-AHN lasting for 6 years with only donor lymphocyte infusions in a status of mixed chimerism. The patient is in a good clinical condition and remission. The potent graft-versus-mastocytosis effect in this patient resembles the favorable effect of donor lymphocyte infusions in relapsing chronic myeloid leukemia patients after transplantation. This patient is, to our knowledge, the first case showing the proof of principle of graft-versus-mastocytosis effect.
晚期系统性肥大细胞增多症是一种相对罕见的疾病，在特定患者中，异基因干细胞移植可治愈该疾病。少数病例报道了移植物抗肥大细胞增多症效应导致的延迟不完全缓解。在本病例报告中，我们描述了供体淋巴细胞输注对一名伴有血液系统肿瘤的系统性肥大细胞增多症（SM-AHN）患者产生移植物抗肥大细胞增多症效应的直接证据和效力。一名53岁女性患者，在真性红细胞增多症病程中，因转化型急性髓系白血病（AML）在常规诱导治疗后接受了异基因干细胞移植。在AML和PV缓解6年后，患者发生无白血病性肥大细胞白血病和JAK2阳性骨髓增殖性肿瘤（SM-AHN）。在混合嵌合状态下，仅通过供体淋巴细胞输注，我们使SM-AHN持续完全缓解达6年。患者临床状况良好，处于缓解期。该患者中强大的移植物抗肥大细胞增多症效应类似于移植后复发的慢性髓系白血病患者接受供体淋巴细胞输注后的良好效果。据我们所知，该患者是首例证明移植物抗肥大细胞增多症效应原理的病例。
