[Clinical Characteristics of CD4-CD56+ Blastic Plasmacytoid Dendritic Cell Neoplasm].

OBJECTIVE: To explore the clinical manifestations, pathological features, immunophenotype, as well as diagnosis, treatment and prognosis of patients with CD4-CD56+ blastic plasmacytoid dendritic cell neoplasm (BPDCN), in order to further understand the rare disease. METHODS: The clinical data, laboratory examinations and treatment regimens of two patients with CD4-CD56+ BPDCN in the First Affiliated Hospital of Wannan Medical College were retrospectively analyzed. RESULTS: The two patients were both elderly males with tumor involved in skin, bone marrow, lymph nodes, etc. Immunohistochemical results of skin lesions showed that both CD56 and CD123 were positive, while CD4, CD34, TdT, CD3, CD20, MPO and EBER were negative. Flow cytometry of bone marrow demonstrated that CD56, CD123, and CD304 were all positive, while specific immune markers of myeloid and lymphoid were negative. Two patients were initially very sensitive to acute lymphoblastic leukemia or lymphomatoid chemotherapy regimens, but prone to rapid relapse. The overall survival of both patients was 36 months and 4 months, respectively. CONCLUSION: CD4-CD56+ BPDCN is very rare and easily misdiagnosed as other hematological tumors with poor prognosis. Acute lymphoblastic leukemia or lymphomatoid therapy should be used first to improve the poor prognosis.

[Clinical Characteristics of Aggressive NK-Cell Leukemia].

OBJECTIVE: To explore and summarize the clinical characteristics and treatment of aggressive NK-cell leukemia (ANKL), and provide new insights for clinical diagnosis and treatment of this disease. METHODS: The clinical data of 7 patients with ANKL admitted to the First Affiliated Hospital of Wannan Medical College from March 2014 to July 2021 were retrospectively analyzed, and their clinical characteristics, laboratory and imaging results, treatment and outcomes were analyzed. RESULTS: Among the 7 patients, 5 were males and 2 were females, with a median age of 47 (33-69) years old. The morphology of bone marrow cells in 7 patients showed similar large granular lymphocytes. Immunophenotyping revealed abnormal NK cells in 5 cases. By the end of follow-up, 6 cases died and 1 case survived, with a median survival time of 76.9 (4-347) days. CONCLUSION: ANKL is a rare disease with short course and poor prognosis. If combined with hemophagocytic syndrome (HPS), the prognosis is even worse. There is no unified treatment method at present, and the use of PD-1 inhibitors may prolong the survival in some patients.

[Neutrophil-Lymphocyte Ratio as a Prognostic Predictor in Patients with Newly Diagnosed Angioimmunoblastic T Cell Lymphoma].

OBJECTIVE: To investigate the prognostic evaluation value of neutrophil-lymphocyte ratio (NLR) in patients with newly diagnosed angioimmunoblastic T cell lymphoma (AITL). METHODS: Clinical data of 39 patients with newly diagnosed AITL in our hospital from March 2010 to August 2018 were colleated and retrospective analyzied, and the relationship between NLR before treatment and the prognosis of AITL patients was analyzed. RESULTS: Among 39 AITL patients, the median value of NCR was 5.43. Based on the cut-off value (5.43), all the patients were divided into 2 groups: high NLR group (5.43, n=20) and a low NLR group (＜5.43, n=19). The total effective rate of treatment was lower in the high NLR group as compered with low NLR group (P=0.041). Univariate analysis showed that, age ＞60 years old, extranodal involvement＞1 as well as high NLR were the independent risk factors that affected overall survival (OS) in newly diagnosed AITL patients. Multivariate Cox analysis showed that extranodal involvement＞1 and high NLR were the independent risk factors that affected OS in newly diagnosed AITL patients. CONCLUSION: The NLR may be an independent prognostic factor in patients with newly diagnosed AITL. High NLR associated with poor prognosis.

[Analysis of Clinical Characteristics for Primary Adrenal Lymphoma].

OBJECTIVE: To explore clinical characteristics of the Primary Adrenal Lymphoma(PAL), so as to enhance the understanding of diagnosis, treatment and prognosis of PAL. METHODS: The clinical data of 2 patients with PAL retrospectively analyzed and the clinical characteristics were explored in combination with releted literalures. RESULTS: Adrenal gland neoplasm was found in 2 patients by imaging examination. The pathological type of one case was diffuse large B cell lymphoma, the other one was extranodal NK/T-cell lymphoma. The former refused to hosipitali3t and the other received to be admited in hospital after the definite diagnosis. She died at the 32th day after diagnosis, due to the complication with acute pancreatitis before chemotherapy. The latter accepted the scheme of"Gemox"combining with the scheme"VP-16+DXM"to control hemophagocytic lymphohistiocytosis. The patient's condition deteriorated rapidly after a short period of improvement, then died at the 40th day after chemotherapy because of multiple organ failure. CONCLUSION: PAL is a rare extra-nodal lymphoma with higher malignancy, the combination of chemotherapy and radiotherapy results in the best outcome among all the treatments. The prognosis of patients with different pathological types was diverse, thus it is very important to choose the appropriate treatment according to different pathological types.