2021EBMT Lu Daopei Medical Team Director Zhao Yanli reports the latest research results

Method: This is a retrospective report
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Number of patients: All 3 patients were diagnosed with HSTCL
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Diagnosis method: Three patients were subjected to bone marrow biopsy and bone marrow aspiration analysis using morphology, immunology, cytogenetics and molecular biology technology (MICM)
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If conditions permit, perform a spleen biopsy to confirm the diagnosis
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Observe the enlargement of lymph nodes, spleen and liver by CT and ultrasound
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Transplant donors: All 3 patients used haploidentical donors
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Pretreatment plan: The intensive MAC treatment plan is as follows: Two adult patients: CLAG (G-CSF 300ug/d×6, Cladribine 5mg/m2/d×5, Cytarabine 2g/m2/d×5) + TBI (2Gy×6) + cyclophosphamide (Cy) + antithymocyte globulin (ATG) [VP-16 (15mg/kg) + TBI (2Gy×6)+Cy + ATG], pediatric patients: CLAG + white Shufei (1.
2mg/kg×16)+Cy + ATG; GVHD prevention: CSA+short-range MTX+MMF

Results: Age at diagnosis: 2.
5, 21 and 29 years old, morphology: 3 cases had bone marrow invasion, and 1 case had central nervous system involvement
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One case was confirmed pathologically after splenectomy
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T cell immunophenotype: two cases of γδ type and 1 case of αβ type
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Chromosomal abnormalities: 2 patients with i(7)/r(7), 2 cases +8
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Molecular biology examination: STAT5B was detected in 2 patients
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Complications before transplantation: One patient was accompanied by hemophagocytic lymphohistiocytosis (HLH).
Before transplantation, HLH94 regimen + Rucotinib was used to control the condition
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Disease duration before transplantation: 2, 6, and 8 months respectively
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Disease status at the time of transplantation: Two adult patients achieved complete remission of bone marrow morphology (CR), but both multicolor flow cytometry monitoring of minimal residual disease (MRD) were positive
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Children with primary drug resistance have not been relieved after 5 courses of chemotherapy
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Spleen and liver size during transplantation: The spleens of two adult patients were significantly retracted
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The liver is significantly enlarged after spleenectomy in children
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Hematopoietic implantation: No implantation failure was found after transplantation
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Acute GVHD: Acute GVHD IV (skin and gastrointestinal tract) occurred in children on the 19th day after transplantation, which was controlled after combined treatment
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Chronic GVHD: All 3 patients developed localized chronic GVHD
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Hepatic venule occlusive disease (VOD): Adult patients with HLH before transplantation were diagnosed with VOD on the 57th day after transplantation, and controlled by defibrillation and other treatments
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Follow-up time: As of December 12, 2020, the follow-up time after transplantation is 3, 7 and 9 months respectively
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Status at the last follow-up: All patients were disease-free and fully donor mosaic
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Conclusion: Considering the high-risk and poor prognostic characteristics of HSTCL, the encouraging clinical results of this study indicate that enhanced MAC with haplo-HSCT is a safe and effective treatment for HSTCL
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references:

Vose, J.
, J.
Armitage, and D.
Weisenburger.
2008.
'International peripheral T-cell and natural killer/T-cell lymphoma study: pathology findings and clinical outcomes', J Clin Oncol, 26: 4124-30

Foss, FM, SM Horwitz, M.
Civallero, et al.
2020.
'Incidence and outcomes of rare T cell lymphomas from the T Cell Project: hepatosplenic, enteropathy associated and peripheral gamma delta T cell lymphomas', Am J Hematol, 95: 151-55

Acknowledgement: Thanks to the head nurse Duan Jianchun for guiding nursing work
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Thank our patients and family members for their cooperation and understanding
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Poster display 02

Haploidentical stem cell transplantation in AML with t(6;9)(p23; q34); DEK-NUP214 shows a favourable outcome: single center experience (haploid hematopoietic stem cell transplantation t(6;9)(p23; q34); DEK-NUP214 Acute Myeloid Leukemia has a good prognosis: single center experience)

First author: Liu Lihui, corresponding author: Liu Hongxing, abstract number: P093

Background: In 2008, the WHO classification of leukemia classified t(6;9)(p23;q34); DEK-NUP214 AML into a separate type
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t(6;9)(p23;q34) AML can be manifested as various morphological subtypes of AML, often manifested as multi-lineage pathological hematopoiesis, increased basophils in peripheral blood or bone marrow (defined as ≥2% ), most of them merge FLT3-ITD
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This type of leukemia has a poor response to standard chemotherapy and a high recurrence rate.
Compared with chemotherapy, allogeneic hematopoietic stem cell transplantation can significantly improve the prognosis
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We report the clinical and molecular characteristics of 14 patients with t(6;9)(p23;q34) AML from Hebei Yanda Ludaopei Hospital.
These patients received haploid hematopoietic stem cell transplantation from September 2013 to October 2019 , Observed its curative effect
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Research methods: We collected the clinical data, karyotype analysis, immunophenotyping, and 58 common gene mutations in hematological tumors of 14 patients with t(6;9) (p23;q34) AML
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In addition, the DEK-NUP214 fusion gene was identified and verified by methods such as total RNA sequencing, RT-PCR, and first-generation sequencing
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The patient underwent haploid hematopoietic stem cell transplantation according to the routine of Hebei Yanda Ludaopei Hospital, mainly based on Bu/Cy/ATG-based myeloablative pretreatment.
The transplant used bone marrow and peripheral blood hematopoietic stem cells, and GVHD was prevented by CSA+ MMF+Short-range MTX
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Results of the study: There were 8 males and 6 females, with a median age of 23 years.
There were 8 AML-M2, 5 AML-M4, and 1 ML-M5
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Multi-line pathological hematopoiesis is common (12/14 cases), and immunotypes are all myeloid phenotypes (CD13, CD33, CD38, HLA-DR).
Increased basophils is only seen in 3/14 patients, which is not common
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All patients had gene mutations, 64.
29% (9/14) of the patients had FLT3-ITD
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All patients received haploid hematopoietic stem cell transplantation, and most of them were in complete remission at the time of transplantation (7 cases of CR1, 1 case of CR2, 3 cases of CR3, 3 cases of NR).
The patients were followed up for 8 to 95 months and all patients were alive
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Research conclusions: Our report confirms that t (6;9) (p23;q34) AML has unique clinical characteristics, often manifested as multi-line pathological hematopoiesis, immunophenotyping is the expression of myeloid antigens, and often combined with FLT3-ITD mutations
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Haploid hematopoietic stem cell transplantation can significantly improve the prognosis, which seems to be better than the previously reported results of allogeneic hematopoietic stem cell transplantation (2-year OS is 61%)
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It may be because most of our patients are in complete remission after transplantation, and haploid hematopoietic stem cell transplantation has a stronger transplant anti-leukemia effect
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Because the number of patients is small, further research is needed
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The EBMT annual meeting is one of the most influential international conferences in the field of blood disease treatment
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At present, China's research on hematopoietic stem cell transplantation and cellular immunotherapy is at the world's leading level
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With the advancement and innovation of medical technology, China is also playing an increasingly important role in the world's medical field
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Lu Daopei's medical team collaborated in clinical and laboratory tests to continuously explore cellular immunotherapy and targeted therapy, as well as hematopoietic stem cell transplantation.
In particular, it has achieved important breakthroughs in clinical research on refractory and recurrent hematological tumors
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