A 62-year-old man with stage IV follicular lymphoma presented with acute left-sided abdominal pain, acute-on-chronic anaemia and thrombocytopenia. An urgent contrast-enhanced CT scan of the abdomen demonstrated the presence of haemoperitoneum. Irregular linear-branching hypodensities distorting the
Abstract Natural killer (NK)/T-cell lymphomas are aggressive malignancies with a predilection for Asian and South American populations. Epstein–Barr virus (EBV) infection in lymphoma cells is universal. Predominantly extranodal, NK/T-cell lymphomas are divided clinically into nasal (involving the nose and upper aerodigestive tract), non-nasal (involving the skin, gastrointestinal tract, testes, and other organs), and aggressive leukaemia/lymphoma (involving the marrow and multiple organs) subtypes. Initial assessment should include imaging with positron emission tomography computed tomography (PET/CT), quantification of plasma EBV DNA as a surrogate marker of lymphoma load, and bone marrow examination with in situ hybridization for EBV-encoded small RNA. Prognostication can be based on presentation parameters (age, stage, lymph node involvement, clinical subtypes, and EBV DNA), which represent patient factors and lymphoma load; and dynamic parameters during treatment (serial plasma EBV DNA and interim/end-of-treatment PET/CT), which reflect response to therapy. Therapeutic goals are to achieve undetectable plasma EBV DNA and normal PET/CT (Deauville score ≤ 3). NK/T-cell lymphomas express the multidrug resistance phenotype, rendering anthracycline-containing regimens ineffective. Stage I/II nasal cases are treated with non-anthracycline asparaginase-based regimens plus sequential/concurrent radiotherapy. Stage III/IV nasal, and non-nasal and aggressive leukaemia/lymphoma cases are treated with asparaginase-containing regimens and consolidated by allogeneic haematopoietic stem cell transplantation (HSCT) in suitable patients. Autologous HSCT does not improve outcome. In relapsed/refractory cases, novel approaches comprise immune checkpoint blockade of PD1/PD-L1, EBV-specific cytotoxic T-cells, monoclonal antibodies, and histone deacetylase inhibitors. Future strategies may include inhibition of signalling pathways and driver mutations, and immunotherapy targeting the lymphoma and its microenvironment.
To report our experience managing a large series of Chinese patients with primary nasal lymphoma.From January 1975 to December 1993, 100 patients (median age, 50 years) with newly diagnosed primary nasal lymphoma were studied. There were four low-grade, 62 intermediate-grade, nine high-grade, and 25 unclassifiable lymphomas. Immunophenotyping was performed in 45 patients: eight B cell, 35 T cell, and two uncertain. All cases of angiocentric lymphoma that were typed were T cell. Fifty-two patients had stage I disease, 15 had stage II, four had stage III, and 29 had stage IV. Only 15 patients had B symptoms (weight loss, night sweats, and/or fever), and 11 had bulky disease. Thirty-nine patients with clinically localized stage I and II disease received local radiotherapy alone (before 1980), and the remaining 28 stage I and II patients received combination chemotherapy followed by local radiotherapy. The 33 patients with advanced stage III and IV disease were given combination chemotherapy, and additional radiotherapy was given to five of them who had bulky local disease.Significantly higher complete remission rates were observed in patients with early stages of disease and those without B symptoms. Superior disease-free survival after complete remission was observed in patients with stage I/II disease. Univariate factors associated with a better overall survival included age less than 60 years, stage I disease, and absence of B symptoms. Survival was significantly better in the subgroup of patients with stage I disease.Patients with nasal lymphoma, especially those with advanced disease, seemed to have a poor prognosis, and their clinical outcome was not improved significantly by the use of chemotherapy instead of radiotherapy or the use of doxorubicin-containing chemotherapeutic regimens.
To the Editor: Recently, the use of hormonal supplementation in healthy elderly subjects to prevent the effects of aging has generated much interest in the lay and medical literature.1, 2 During normal aging, there is progressive decline in growth hormone (GH) secretion (somatopause), impairment of ovarian and testicular function, and reduced adrenal androgen secretion. GH supplementation increases lean body mass, skin thickness, and bone density and decreases adipose tissue in normal elderly people.1 Testosterone administration in elderly men might improve lean body mass and sexual function.3 Dehydroepiandrosterone (DHEA) administration might also have beneficial effects on some age-related bodily changes.4 Nevertheless, the side effects and long-term safety of hormonal supplementation remain unknown. A healthy 65-year-old physician practicing family medicine decided to self-administer a combination of GH, testosterone, and DHEA to maintain vigor and prevent aging. Before commencement of treatment, his blood counts were hemoglobin (Hb) 16.8 g/dL, white blood cell (WBC) 7.7 × 109/L, and platelet (plt) 238 × 109/L. He had not undergone endocrinological evaluations. The treatment consisted of four units of recombinant human GH subcutaneously every week, testosterone 250 mg intramuscularly every month, and oral DHEA 50 mg every day. After 3 months of hormonal administration, a blood count showed Hb=19.8 g/dL, WBC=5.9 × 109/L, and plt=189 × 109/L. The treatment was stopped and venesection of two units of blood performed. Physical examination was normal. Ultrasonogram of the abdomen and kidneys were normal. Plasma erythropoietin was suppressed to an undetectable level. A diagnosis of hormone-induced polycythemia was made. He was reassured but advised to stop the medication. Two months after cessation of treatment, his blood counts had returned to normal (Hb=15.4 g/dL, WBC=6.6 × 109/L, and plt=182 × 109/L). Plasma erythropoietin level had risen to 5.9 mU/mL (reference range=2.9–24.5). His latest blood counts and erythropoietin levels have remained normal, nearly 1 year afterward. None of the hormones used in this case has been reported to give rise to polycythemia when administered at the dose described. Therefore, the development of polycythemia was likely due to the synergistic interactions of the three hormonal supplements. Neither the benefits nor the long-term safety of hormonal supplementation are known. In elderly subjects, concerns have been raised about the possibility of increased cancer risks with GH administration.2 This case showed that even physicians might find it difficult to determine the risk and benefits from the existing literature. The medical profession and the lay public should wait for future clinical and laboratory research to determine the role of hormonal supplementation in the elderly population.
'/%3e%3cuse xlink:href='%23a' transform='rotate(144 450 300)'/%3e%3cuse xlink:href='%23a' transform='rotate(216 450 300)'/%3e%3cuse xlink:href='%23a' transform='rotate(288 450 300)'/%3e%3c/svg%3e)
'%3e%3cpath fill='%23012169' d='M0 0v30h60V0z'/%3e%3cpath stroke='%23fff' stroke-width='6' d='m0 0 60 30m0-30L0 30'/%3e%3cpath stroke='%23C8102E' stroke-width='4' d='m0 0 60 30m0-30L0 30' clip-path='url(%23b)'/%3e%3cpath stroke='%23fff' stroke-width='10' d='M30 0v30M0 15h60'/%3e%3cpath stroke='%23C8102E' stroke-width='6' d='M30 0v30M0 15h60'/%3e%3c/g%3e%3c/svg%3e)
'/%3e%3cuse xlink:href='%23a' transform='rotate(23.036 .093 25.536)'/%3e%3cuse xlink:href='%23a' transform='rotate(45.87 1.273 16.18)'/%3e%3cuse xlink:href='%23a' transform='rotate(69.945 .996 12.078)'/%3e%3cuse xlink:href='%23a' transform='rotate(20.66 -19.689 31.932)'/%3e%3c/svg%3e)