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Objective To analyze the clinical characteristics and prognosis of 4 rare types of non -Hodgkin lymphoma(NHL)in children,and to discuss the progress in treatment.Methods Clinical data of 1 4 patients with rare types of NHL at Shanghai Children′s Medical Center,Shanghai Jiaotong University School of Medicine between January 2004 and December 201 4 were retrospectively analyzed,and their clinical features,treatment and prognosis were dis-cussed.Results Fourteen cases were reported including 6 subcutaneous panniculitis -like T -cell lymphoma (SPTCL),3 hydroa vacciniforme -like cutaneous lymphoma(HVLL),2 pediatric follicular lymphoma(PFL)and 3 ex-tranodal NK/T -cell lymphoma,and nasal type(ENKTL).Ten patients (71 .4%)primarily presented with skin lesions and underwent a long course of illness before they were finally diagnosed (the median was 1 0 months),71 .4%(1 0 /1 4 cases)of them associated with fever and 50.0%(7 /1 4 cases)with liver and spleen enlargement,and no evidence of central nervous system (CNS)and bone marrow (BM)involvement was observed,while 28.6% patients (4 /1 4 cases) had more than two lines′abnormalities in peripheral blood examination.Since there were no standard treatment guide-lines,most patients received CHOP (Cyclophosphamide +Adriamycin +Vincristine +Prednisone)and /or mature B -cell NHL -like therapy,and 50.0%(7 /1 4 cases)of them received interferon therapy in addition,while 1 patient re-ceived allogeneic hematopoietic stem cell transplantation after recurrence.The complete remission was achieved in 71 .4%(10 /14 cases)of all the patients.Two of them died,3 lost follow -up,and 1 relapsed.The 3 -year overall survi-val and event free survival rates were 0.84 and 0.57,respectively,after a median follow -up of 26 months (range 12 -64 months).Conclusions Pediatric rare types of NHL show atypical clinical manifestation,low incidence of CNS /BMinfil-tration and long course.It is hard to make pathological diagnosis and differentiation.It is also inappropriate to apply the commonly used staging system to these rare types of NHL.No standard treatment has been found by now.SPTCL,HVLL and PFL have relatively good outcomes when treated with mature B -cell NHL -type therapy plus interferon therapy.
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Background and purpose:Extranodal NK/T-cell lymphoma, nasal type (ENKTL) belongs to a rare type of non-Hodgkin's lymphoma (NHL). Its incidence rate in Asian country is higher than that in Western country. This disease is highly invasive, the pathogenesis of it is still unclear. Resent research shows that epstein-barr virus (EBV) is closely related to the occurrence of it. There is still no standard treatment guidelines of ENKTL, and the prognosis is very bad. Therefore, it is imperative to explore the pathogenesis of ENKTL. This study aimed to investigate the expressions of interleukin-2 (IL-2), interleukin-6 (IL-6) and tumor necrosis factor-α (TNF-α) in the serum of ENKTL patients and their clinical signiifcances. Methods: Luminex liquid chip technology was used to detect the expression levels of IL-2, IL-6 and TNF-αin the serum of 67 ENKTL patients and 26 normal persons. Results:The expression levels of IL-2, IL-6 and TNF-αin the serum of 67 ENKTL patients were (564.1±387.6), (293.3±191.6) and (181.3±91.8)pg/mL, while in the normal persons were (1 097.0±365.7), (417.5±289.6) and (291.3±89.4)pg/mL, respectively. Compared with normal persons, the expression levels of IL-2, IL-6 and TNF-αin ENKTL patients were signiifcantly lower (P<0.05). Further study showed that the expression level of TNF-αin 5 complete remission ENKTL patients [(162.7±10.3)pg/mL] was significantly higher than that in initial treatment patients [(125.2±7.3)pg/mL, P<0.05]. Conclusion:The expressions of IL-2, IL-6 and TNF-αare reduced in the serum of ENKTL patients, and the serum expression level of TNF-αis closely related to the effect of chemotherapy.
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Extranodal NK/T-cell lymphoma,nasal type (ENKTL) is a aggressive lymphoma which arises from NK cells or T cells and has a poor prognosis.This article mainly reviewed and focused on hematological stem cell transplantation in treating ENKTL.
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Primary nasal type natural killer (NK)/T cell (NKTC) lymphoma, a specific form of malignant lymphoma, has a higher geographic incidence in Oriental, Mexican, and South American populations than the Western population. In Koreans, it comprises 9-12% of all cases of non-Hodgkin's lymphoma. This type of lymphoma has also been named as angiocentic lymphoma and lethal midline granuloma because the most common site is the upper airway area and its clinical aggressiveness presents with a necrotic and destructive pattern. NKTC lymphoma can also be detected in different organs (testis, spleen, parotid gland, skin, gastroinstinal tract, central nervous system, lungs, bone marrow, etc.) other than the upper airway including the oral cavity. The lymphoma detected in the oral cavity shows various destructive and inflammatory changes, similar to the signs of inflammation and infection from periodontitis and pulpal disease, making a diagnosis difficult with just the clinical signs. For early detection, clinical, radiological, and pathological examinations are required. This report describes the clinical, radiological and histological characteristics with a case report for the early detection of NKTC lymphoma in the oral cavity.
Subject(s)
Bone Marrow , Central Nervous System , Early Diagnosis , Granuloma, Lethal Midline , Incidence , Inflammation , Lung , Lymphoma , Lymphoma, Non-Hodgkin , Mouth , Parotid Gland , Periodontitis , Skin , SpleenABSTRACT
NK/T-cell lymphoma is rare type of lymphoproliferative disorder, but it is clinically aggressive with a poor prognosis. The World Health Organization (WHO) classified mature NK cell neoplasms into extranodal NK/T-cell lymphoma nasal type and aggressive NK cell leukemia. NK/T-cell lymphoma is characterized by a strong association with Epstein-Barr virus (EBV), and when it involved the skin, the lesions clinically appear as deep nodules, infiltrative plaques and ulcerative plaques. We herein report on a case of the nasal type of extranodal NK/T-cell lymphoma that showed the immunophenotype of CD20-, CD56+ and CD45RO+, and it was positive on in situ hybridization for EBV. Our case was characterized by the clinical resemblance with pyoderma gangrenosum and this malady generally displays chronic painful ulcer and necrotic lesions.
Subject(s)
Chronic Pain , Herpesvirus 4, Human , In Situ Hybridization , Killer Cells, Natural , Leukemia , Lymphoma , Lymphoproliferative Disorders , Prognosis , Pyoderma , Pyoderma Gangrenosum , Skin , Ulcer , World Health OrganizationABSTRACT
We report a case of a 38-year-old male with extranodal NK/T-cell lymphoma, nasal type, showing unusual clinical and pathological features. The patient was admitted for soft tissue swelling and tenderness in both legs. The patient had been treated intermittently 8 months prior for repeated muco-cutaneous ulcers. A muscle biopsy showed medium-sized atypical lymphoid cells with bizarre nuclei and plump cytoplasm, infiltrating to the skeletal muscle fibers with angiocentricity. The immunoresults were Ki-1+, CD56+, cytoplasmic CD3+, with EBV-in situ hybridization +. The patient rapidly deteriorated and died of sepsis and respiratory failure shortly after initiation of low-dose chemotherapy. A careful review of previous biopsies revealed scarce atypical lymphoid cells around vessels with similar immunoprofiles without the presence of Ki-1 positive cells. This case emphasizes that an extranodal NK/T-cell lymphoma may have a dermatomyositis-like diffuse presentation. Ki-1 co-expression can be an unexpected event in a process of the disease course; however, this should be validated with future studies.
Subject(s)
Adult , Humans , Male , Biopsy , Cytoplasm , Dermatomyositis , Drug Therapy , Leg , Lymphocytes , Lymphoma , Muscle Fibers, Skeletal , Respiratory Insufficiency , Sepsis , UlcerABSTRACT
Extranodal NK/T cell lymphoma(nasal type) is a common,high malignant degree and poor prognosis entity of the nasal lymphoma.It is the most important to understand its genesis,development,clinical manifestation,pathologic diagnosis,therapy and prognosis.