A 13-year-old girl presented with multiple recurrent cutaneous plaques for more than six months,which had been aggravated with intermittent fever for five months.No obvious systemic abnormality was found.Dermatological examination revealed multiple,non-ulcerative,painless,infiltrative,indurated,poorly marginated,purple subcutaneous plaques measuring 3-1 1 cm in diameter with slight squamation in bilateral buttocks and lower limbs.Laboratory investigations showed bicytopenia with the white blood cell count being (0.03-3.7) × 109/L and red blood cell count being (2.8-4.4) × 1012/L,a normal platelet count,hypofibrinogenemia (1.79 g/L) and low proportion of natural killer cells (4.6％).Bone marrow smear showed active proliferation of cells,decreased proportion of granulocytes,presence of a few indefinitely classified cells,and phagocytosis.Reticulocytes were easily seen in the bone marrow smear.Pathologically,no obvious abnormality was observed in the epidermis or dermis,while the subcutaneous adipose tissue,especially fat lobules and some interlobular septa,was extensively infiltrated by large-to medium-sized lymphoid cells with pleomorphic and twisted nuclei as well as a small amount of cytoplasm; necrosis and phagocytosis of nuclear debris and lymphocytes were visible.The atypical lymphoid cells stained positive for CD3,T-cell intracellular antigen-1,granzyme B and TCRγδ with partial loss of CD5 and CD7,but negative for CD56,CD4,CD8 and TCRαβ.No Epstein-Barr virus-encoded RNA (EBER) was detected by in situ hybridization.Based on these findings,a diagnosis of primary cutaneous γδ-T cell lymphoma with hemophagocytic syndrome was made.

Objective To study the expressions of anaplastic lymphoma kinase (ALK-1) and cytotoxic proteins in primary systemic anaplastic large cell lymphoma (S-ALCL) and their relationship with clinical outcome. Methods 51 S-ALCL cases were collected from Lymphoma Lab of Peking University Health Science Centre & Peking Children's Hospital. The morphologic characteristics were studied under routine microscope, and essential immunohistochemical stainings were performed and reviewed to confirm the diagnosis of S-ALCL. Immunohistochemical stainings for ALK-1 and cytotoxic proteins (TIA-1 & granzyme B) were performed using standard SP method. Patients related clinical data including follow-up materials were collected. Results Survival time of 44 cases with completely clinical follow up materials ranged from 0.5～66months. 36 out of 51 cases(37 %) was positive for ALK-1 protein. While 20 cases out of 47 S-ALCL cases ( 42.55 % ) positive for granzyme B and 22 out of 28 cases (81.48 %) were positive for TIA-1. The prognosis of patients with ALK-1 protein positive and granzyme B negative expression was better, but TIA-1 expression might have nothing to do with clinical outcome (P＞0.05). In addition, multivariate analysis confirmed that ALK-1 protein expression, granzyme B protein expression and Ann-Arbor stage system were possible for prognosis(P＜0.05), Conclusion Expression of ALK-1 and granzyme B protein expression may serve as two independent prognostic predictors in S-ALCL patients.

Esophageal cancer is a malignant tumor with high incidence and mortality rate in the world and its pathogenic factors are complex and diverse.There are no obvious symptoms in the early stage,and most patients are in the middle to late stage at the initial diagnosis.The prognosis of esophageal cancer is poor.The treatment mode of conventional surgical resection combined with chemoradiotherapy can no longer meet the current treatment needs of disease,and new treatment strategies are urgently needed.Molecular targeted therapy and immunotherapy are new treatment methods that have emerged in recent years,which have broken the therapeutic bottleneck and have been proven to play important roles in the treatment of esophageal cancer.The current research progress of the main targets and their related targeted drugs in molecular targeted therapy and immunotherapy for esophageal cancer were reviewed in this article,which provided reference for the application of precision medicine in the field of esophageal cancer.