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Han L.,Zhengzhou University | Han L.,Lymphoma Diagnosis and Treatment Center | Zhang M.,Zhengzhou University | Zhang M.,Lymphoma Diagnosis and Treatment Center | And 23 more authors.
Oncology Letters | Year: 2014

Natural killer (NK)/T-cell lymphoma-associated hemophagocytic syndrome (HPS) is a rare and fatal disease with no optimal treatment. The present study reports the clinical features, diagnosis and treatment process of three patients with relapsed NK/T-cell lymphoma-associated HPS. All of the patients were classified as Ann Arbor stage IV and presented with a poor performance status. Two patients were successfully treated with a pegaspargase-containing combination regimen and one patient succumbed due to serious complications. These cases indicate that for patients with a history of lymphoma, the diagnosis of HPS should be considered when patients present with progressive high fever, pancytopenia and liver dysfunction. Early identification and effective treatments, including pegaspargase-based regimens are essential for an enhanced prognosis.

Hu T.,Lymphoma Diagnosis and Treatment Center | Wang X.,Lymphoma Diagnosis and Treatment Center | Yu C.,Lymphoma Diagnosis and Treatment Center | Yan J.,Lymphoma Diagnosis and Treatment Center | And 12 more authors.
Oncology Letters | Year: 2013

Follicular dendritic cell sarcoma (FDCS) is a rare neoplasm arising most commonly from follicular dendritic cells in the lymph nodes. It is exceedingly rare in extranodal sites, particularly in the pharyngeal region. The present study reports 3 cases occurring in the pharyngeal region. Case 1 had tonsil and cervical lymph node involvement, while case 3 also had tonsil involvement. Cases 1 and 3 relapsed locally at 3 and 17 months after surgery, respectively. Case 2 was diagnosed with a tumor in the parapharyngeal space and the patient succumbed to the disease 5 months after treatment with combined surgery and chemotherapy. All 3 cases were misdiagnosed initially. Pathological biopsy examination, including histopathology and immunohistochemistry, was essential for diagnosis. The data for 52 cases, including cases from the literature and the present cases, were analyzed. The results indicated that 57% (26/46) of the initial diagnoses were inaccurate, while the recurrence, metastasis and mortality rates were 40, 16 and 10%, respectively. The statistics supported the theory that FDCS of the pharyngeal region is a low-grade sarcoma. Involvement of the tonsils (52%, 27/52) and parapharyngeal space (19%, 10/52) were observed most commonly, while FDCS at various sites showed different prognoses. The various survival rates were calculated in the present study. The large tumors (≥4 cm) had a poorer prognosis than the small tumors (<4 cm; P<0.05). Among the 50 cases with available follow-up data, 46% (23/50) were treated with surgery alone, 52% (26/50) with combination therapy (surgery followed by chemotherapy and/or radiotherapy) and 2% (1/50) with surveillance. There was no statistically significant evidence (P>0.05) that combination therapy improves survival rates, compared with surgery alone.

Han L.,Zhengzhou University | Han L.,Lymphoma Diagnosis and Treatment Center | Li L.,Zhengzhou University | Li L.,Lymphoma Diagnosis and Treatment Center | And 21 more authors.
Leukemia and Lymphoma | Year: 2014

Natural killer (NK)/T cell lymphoma associated with hemophagocytic syndrome (NK/T-LAHS) is a rare and life-threatening disease. The clinical features and overall survival of NK/T cell and other T cell lymphomas associated with hemophagocytic syndrome remain uncertain. We retrospectively reviewed the clinical records of 15 patients with NK/T-LAHS and 14 patients with other T-LAHS from December 2008 to June 2013. Patients with NK/T cell lymphoma had a higher International Prognostic Index (p = 0.045) and were more likely to be positive for Epstein-Barr virus (p = 0.025) than those with T cell lymphoma. The level of aspartate aminotransferase (AST) was significantly higher than that of alanine aminotransferase (ALT) in the NK/T-LAHS group (p = 0.005), as well as in the T-LAHS group (p = 0.019). The level of direct bilirubin (DBIL) was more likely to be elevated than that of indirect bilirubin (IBIL) in patients with NK/ T-LAHS (p = 0.047), while there was no significant difference in the T-LAHS group (p = 0.124). The median survival time for patients with NK/T and T cell lymphoma was 28 and 33 days, respectively (p = 0.726). However, in the NK/T-LAHS group, the median survival time of patients treated with and without pegaspargase was 116 and 15 days, respectively (p = 0.003). Our results suggest that patients with NK/T-LAHS are at higher risk and suffer a worse prognosis. However, the use of pegaspargase could benefit patients and lead to long-term overall survival. © 2014 Informa UK, Ltd.

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