Evaluation of bone marrow involvement by FDG-PET for refractory Hodgkin lymphoma treated by unrelated allogeneic bone marrow transplantation

A 23-year-old female patient was diagnosed as having Hodgkin lymphoma (mixed cellularity type, clinical stage III B) in September 2000. She underwent ABVD chemotherapy and irradiation of a mediastinal lesion, resulting in complete remission. However, the disease reoccurred three month after the completion of initial treatment. She was admitted to our hospital for allogeneic stem cell transplantation. Thoractic vertebra, lumbar vertebra and iliac bone lesions were detected by FDG-PET, and a diagnosis of bone marrow infiltration was made. She received re-induction chemotherapy but did not achieve complete remission. A residual lesion in her bone marrow was detected by FDG-PET. She underwent unrelated allogeneic bone marrow transplantation in May 2002. Preconditioning was VP-16, CY and TBI. Engraftment of white blood cells was on day 15. Skin GVHD was detected at the same time and she was treated with steroid hormones, resulting in improvement. No residual mass could be detected by FDG-PET on day 60. However, she suffered from fever on day 80. Aggravation of the disease was revealed and she died from progression of the disease on day 120. FDG-PET is useful for the monitoring disease status and for determining the optimal timing of various treatments.     

Treatment of localized extranodal NK/T cell lymphoma,nasal type

Extranodal NK/T cell lymphoma predominantly presents as a localized disease in the upper aerodigestive tract from the nasal cavity to the hypopharynx. Because radiotherapy has better outcomes than chemotherapy with reduced locoregional failure, it should be considered the preferred first-line therapy. However, the addition of chemotherapy is appropriate as part of the initial treatment because of the frequent systemic progression or relapse after radiotherapy. At present, the combination of radiotherapy and chemotherapy can be considered an effective treatment option, and the promising results of recent prospective studies with concurrent chemoradiotherapy support this treatment strategy. In contrast, intensive chemotherapy should be considered as initial treatment for patients with tumors in non-upper-aerodigestive-tract sites, such as skin or intestine because they usually progress to systemic disease. Likewise, for patients with poor prognostic factors, such as a high NK lymphoma prognostic index, autologous stem cell transplantation during remission and additional treatments with central nervous system prophylaxis may be beneficial. However, the precise role of these treatments needs to be clarified further by prospective clinical trials. Thus, a prospective study is warranted to explore a risk-adapted treatment strategy of applying initial chemoradiotherapy and additional consolidation treatments.     

Role of FDG-PET/CT in detecting lymphomatous bone marrow involvement in patients with newly diagnosed diffuse large B-cell lymphoma

To evaluate the role of FDG-PET/CT in detecting bone marrow (BM) involvement, pre-treatment bilateral bone marrow biopsies (BMBs) and FDG-PET/CT scans of 89 patients with diffuse large B-cell lymphoma (DLBCL) treated with rituximab-CHOP were reviewed and analyzed. Fourteen patients (15.7%) had lymphomatous involvement based on BMB (BMB+), and 17 patients (19.1%) had the possibility of BM involvement on FDG-PET/CT (FDG-PET/CT+). Seventy-two patients (80.8%) had concordant results between BMB and FDG-PET/CT (seven patients were positive for both, and 65 patients were negative for both), but 17 patients (19.2%) had a discordant interpretation (seven patients were BMB+ and FDG-PET/CT-, and ten were BMB- and FDG-PET/CT+). Although BMB+ patients had an inferior 2-year EFS (37.0% vs. 79.8%, p?相似文献   

CT of nasal-type T/NK cell lymphoma in the lung

Nasal-type T-cell/natural killer cell lymphoma is a new distinctive clinicopathologic entity with a characteristic immunophenotypic expression of CD56. Most cases show a predilection for the nasopharyngeal region and are referred to as nasal T/NK-cell lymphoma. Few cases occur in areas other than the nose. To the best of our knowledge, nasal type T/NK cell lymphoma with isolated lung involvement has not been reported. We illustrate here the CT findings of this rare tumor occurring primarily in the lung.     

Immunoglobulin D-lambda type multiple myeloma presenting with FDG-PET/CT negative bone marrow involvement

We here report a rare case of a patient with IgD-lambda-positive multiple myeloma presenting with FDG-PET/CT negative bone marrow involvement. A 72-year-old man was admitted to our hospital for evaluation of a paravertebral tumor of the chest. Thoracotomy was performed and a histopathological evaluation of resected intrathoracic tumor demonstrated a plasmacytic neoplasma. Initially we thought that this case was a solitary plasmacytoma because there were no positive findings on postoperative FDG-PET/CT. However, bone marrow aspiration study demonstrated massive infiltration of myeloma cells (72%). It is necessary to recognize that IgD-lambda type myeloma cells may not be sufficiently metabolically active to form high uptake on FDG-PET/CT.     

Treatment of advanced extranodal NK/T cell lymphoma,nasal-type and aggressive NK-cell leukemia

Extranodal NK/T cell lymphoma, nasal type (ENKL) with advanced stage and aggressive NK-cell leukemia (ANKL) are highly aggressive neoplasms with a dismal clinical outcome. It is well known that P-glycoprotein, which is a product of MDR1 gene and related to multi-drug resistance, is expressed on tumor cells of ENKL or ANKL. This is a major reason for the refractoriness to conventional chemotherapeutic regimens for malignant lymphoma containing anthracycline. However, recent studies have identified that several drugs including l-asparaginase, methotrexate and alkylators show excellent effect for these tumors. The SMILE (steroid, methotrexate, ifosfamide, l-asparaginase and etoposide) regimen is one of the promising regimens for advanced or relapsed/refractory ENKL, but its myelotoxicity is strong. ANKL needs another treatment strategy because of a systemic disease progression and extensive organ insufficiency. Optimal treatment scheme using such effective agents for these unfavorable NK-cell tumors should further be explored.     

Lymphomatoidgastropathy mimicking extranodal NK/T cell lymphoma, nasal type: a case report

Extranodal natural killer (NK)/T-cell lymphoma, nasal type, exhibits aggressive tumor behavior and carries a poor prognosis. Recently, lymphomatoid gastropathy with NK/T cell infiltration into gastric mucosa has been recognized as a pseudo-malignant disease which regresses without treatment. Because the conventional immunohistochemical criteria of lymphomatoid gastropathy is similar to that of extranodal NK/T-cell lymphoma nasal type, it is difficult to distinguish between the two conditions by histopathological evaluation only. Here, we report a rare case of lymphomatoid gastropathy in a 57-year-old female. Gastroendoscopy on routine check-up revealed elevated reddish lesions < 1 cm in diameter in the gastric fornix and body. Although repeat endoscopies at 1 and 6 mo later revealed no gastric lesions at any locations without any treatments, at 12 mo later gastric lymphomatoid lesions recurred at gastric fornix and body. Histological examination of endoscopic biopsy specimens at 12 mo showed atypical NK cell infiltration with CD3⁺, CD4^-, CD5^-, CD7⁺, CD8^-, CD20^-, CD30^-, CD56⁺, CD79a^- and T-cell-restricted intracellular antigen-1⁺ into gastric mucosa. After treatment for Helicobacter pylori (H. pylori) eradication, the lesions disappeared in all locations of the gastric fornix and body over the subsequent 12 mo. Here, we report a case of H. pylori-positive lymphomatoid gastropathy with massive NK-cell proliferation, and also review the literature concerning newly identified lymphomatoid gastropathy based on comparison of extra nodal NK/T-cell lymphoma nasal type. In any case, these lesions are evaluated with biopsy specimens, the possibility of this benign entity should be considered, and excessive treatment should be carefully avoided. Close follow-up for this case of lymphomatoid gastropathy is necessary to exclude any underlying malignancy.     

Detection of extranodal and spleen involvement by FDG-PET imaging predicts adverse survival in untreated follicular lymphoma

Predicting early clinical failure in patients with untreated follicular lymphoma (FL) is important but difficult. This study aimed to determine the incidence and patterns of extranodal (EN) and spleen disease using PET/CT, and assess their utility in predicting early clinical failure. PET/CT images from 613 cases of untreated FL (2003-2016) were reviewed. The location and number of EN sites, patterns of bone involvement, and splenic involvement were recorded. Outcomes were assessed using event-free survival (EFS), overall survival (OS), and early clinical failure at 24 months (EFS24). So, 49% (301/613) of patients had PET/CT-detected EN involvement, and 28% (171/613) had spleen involvement. The presence of ≥2 EN sites, spleen, bone or soft tissue involvement all predicted failure to achieve EFS24. Presence of ≥2 EN sites and bone involvement pattern were also predictive of OS in a univariate analysis. In a multivariate analysis with FLIPI-2 factors, spleen involvement, pattern of bone involvement, and soft tissue involvement independently predicted a lower EFS (HR 1.49 (1.11-2.00), P = .007; HR 1.71 (1.10-2.65), P = .017; and HR 1.67 (1.06-2.62), P = .026, respectively). When the multivariate analysis was performed using PRIMA-PI factors (marrow and B2M), the number of EN sites was an independent prognostic factor for inferior OS (HR 2.28; P = .05). Baseline PET/CT identifies EN involvement in nearly half of patients with untreated FL. The presence of ≥2 EN sites, bone, soft tissue, or splenic involvement predicts early clinical failure. These results, when combined with other factors, may better identify high-risk patients and guide therapy.     

An extranodal NK/T cell lymphoma,nasal type,with specific immunophenotypic and genotypic features

Extranodal NK/T cell lymphoma, 'nasal type,' is a rare clinicopathological entity in Europe. The main clinical features are nasal congestion, sore throat, dysphagia and epistaxis, due to a destructive mass involving the midline facial tissues. Pathologically, lymphoma cells exhibit angioinvasion, angiodestruction and coagulative necrosis. We report the case of a patient who presented with fever, dyspnea, nasal congestion, headache, distention of right nasal turbinates and exophytic lower leg ulcerating lesions. A CT scan of visceral scull demonstrated a filling mass of right frontal, ethmoidal and maxillary sinuses with erosion of the wall of right maxillary sinus and ventral portion of the diaphragm. A biopsy was performed in the skin lesion and showed an angioinvasive NK/T cell lymphoma CD56 negative with clonal rearrangement of the T-cell-receptor gamma gene. Up to our knowledge, this is a rare immunophenotype for NK/T-cell, 'nasal type,' lymphomas. However, the lymphoma may be classified as extranodal NK/T cell lymphoma, 'nasal type,' due to typical clinical presentation, radiologic findings and pathological characteristics of polymorphism, angioinvasion, angiodestruction and coagulative necrosis.     

Prognostic factors for classifying extranodal NK/T cell lymphoma, nasal type, as lymphoid neoplasia

This study evaluated the applicability of prognostic factors commonly used for diagnosis of classical lymphoma outcomes to extranodal NK/T cell lymphoma, nasal type (NTCL). Clinical features and their associations with lactate dehydrogenase (LDH) were evaluated in 70 patients. RLDH was defined as the ratio of LDH to the upper normal limit. RLDH was associated with stage (I-II vs. III-IV), lymph node involvement (LNI), and International Prognostic Index score (<2 vs. > or =2). Poor performance status and advanced stage were common in patients with local tumor invasiveness (LTI). LDH level, classified into three levels (low, high, and very high) was associated with survival (P < 0.001). In multivariate analysis, the predictive values of LDH level, B symptom, performance status, and stage remained significant whereas those of LTI and LNI did not. Scoring was performed by weighting each factor with 0.5 or 1.0 according to its hazard ratio. Scores were classified into four groups. Groups with high scores were associated with unfavorable outcomes (P < 0.001). Current study suggests that prognostic factors for NHL may be useful to predict the outcome of NTCL but the model should take LDH level and the prognostic weight of each factor into account.     

Survival benefit with salvage radiotherapy for patients with locoregionally recurrent extranodal NK/T cell lymphoma, nasal type

The purposes of this study are to evaluate prognosis in patients with locoregionally recurrent extranodal nasal-type NK/T cell lymphoma (NKTCL) and to determine the value of salvage radiotherapy. Forty-two patients with NKTCL who developed first locoregional recurrence with (n?=?13) or without (n?=?29) systemic failure were reviewed. Retreatment included chemotherapy (n?=?20), radiotherapy (n?=?13), and radiotherapy plus chemotherapy (n?=?9). Fifteen patients were reirradiated for localized recurrent disease. The 5-year overall survival (OS) rate after recurrence was 40 %, with a median survival of 26 months. The 2-year OS rate and median OS were 68 % and 36 months for locoregional recurrence only, compared with 31 % and 14 months for both locoregional and systemic recurrence, respectively (p?=?0.034). Subgroup analysis for patients with localized recurrent disease revealed an improved OS with radiotherapy. The 2-year and 5-year OS rates were 77 and 69 % for radiotherapy, respectively, compared with a 2-year OS rate of 50 % and median OS of 16 months for chemotherapy alone (p?=?0.006). Patients with localized recurrence had a better prognosis than those with systemic recurrence. Salvage radiotherapy or reirradiation resulted in a favorable prognosis for patients with localized recurrent disease.     

Monoclonal gammopathy in extranodal marginal zone lymphoma (ENMZL) correlates with advanced disease and bone marrow involvement

Monoclonal gammopathy is a well-recognized occurrence in splenic marginal zone lymphoma (MZL); however, its prevalence has never been reported in extranodal MZL (ENMZL). We present results of a retrospective analysis of 26 patients with newly diagnosed as well as previously treated ENMZL. Monoclonal gammopathy was detected in 7 out of 26 (27%) patients. All patients with a monoclonal gammopathy had stage IV disease, and most of them had involvement of lymph nodes and/or bone marrow (n = 6). Only 1 out of 7 patients with a monoclonal spike had stage IV disease diagnosed based on multifocal mucosal involvement. There was also a strong correlation between the involvement of bone marrow and the presence of a monoclonal spike (Fisher's exact test, P = 0.0007). Prevalence of monoclonal gammopathy is higher than previously recognized and indicates advanced disease. However, the prognostic significance of the presence of monoclonal gammopathy in this population is unknown.