The aggressive peripheral T-cell lymphomas: 2015.

BACKGROUND: T-cell lymphomas make up approximately 10%-15% of lymphoid malignancies. The frequency of these lymphomas varies geographically, with the highest incidence in parts of Asia. DIAGNOSIS: The diagnosis of aggressive peripheral T-cell lymphoma (PTCL) is usually made using the World Health Organization classification. The ability of hematopathologists to reproducibly diagnosis aggressive PTCL is lower than that for aggressive B-cell lymphomas, with a range of 72%-97% for the aggressive PTCLs. RISK STRATIFICATION: Patients with aggressive PTCL are staged using the Ann Arbor Classification. Although somewhat controversial, positron emission tomography scans seem to be useful as they are in aggressive B-cell lymphomas. The most commonly used prognostic index is the International Prognostic Index. The specific subtype of aggressive PTCL is an important risk factor, with the best survival seen in anaplastic large-cell lymphoma-particularly young patients with the anaplastic lymphoma kinase positive subtype. RISK-ADAPTED THERAPY: Anaplastic large-cell lymphoma is the only subgroup to have a good response to a CHOP-like regimen. Angioimmunoblastic T-cell lymphoma has a prolonged disease-free survival in only ~20% of patients, but younger patients who have an autotransplant in remission seem to do better. PTCL-not otherwise specified is not one disease. Anthracycline-containing regimens have disappointing results, and a new approach is needed. Natural killer/T-cell lymphoma localized to the nose and nasal sinuses seems to be best treated with radiotherapy-containing regimens. Enteropathy-associated PTCL and hepatosplenic PTCL are rare disorders with a generally poor response to therapy, although selected patients with enteropathy-associated PTCL seem to benefit from intensive therapy.

Angioimmunoblastic T-cell lymphoma masquerading as granulomatous lymphadenitis: Fine needle aspiration cytology, clinical and radiology correlation.

Fine needle aspiration (FNA) is a minimally invasive technique used in the initial diagnosis of superficial lesions, including lymphadenopathy. Its benefit in lymph node pathology, however, is highly variable, especially in heterogeneous lymphoproliferative disorders like angioimmunoblastic T-cell lymphoma (AITL). AITL is an aggressive hematopoietic malignancy, histologically characterized by medium-sized neoplastic cells, high endothelial venule proliferations, and a heterogeneous hematolymphoid background. Diagnostic difficulty arises at lymph node FNA, where cytology yields nonspecific polymorphous collections of medium-sized lymphocytes, hematolymphoid cells, dendritic cell-lymphoid complexes, and lymphoid tissue fragments with transgressing blood vessels; findings mimicking reactive lymphadenopathy. We present a case of a 62-year-old male who presented with cervical lymphadenopathy. Neck level II lymph node FNA revealed granulomatous inflammation. A cell block was prepared for additional infectious studies but was non-contributory due to lack of material. Flow cytometry showed no evidence of non-Hodgkin lymphoma. Excisional biopsy revealed lymph node effacement by a T-cell lymphoproliferative disorder consistent with AITL. This case contributes to the paucity of literature regarding the cytologic features of AITL observed at FNA, and becomes the premier case to emphasize the addition of granulomatous features. Despite the aggressive nature of this entity, cases are frequently misdiagnosed as reactive on initial evaluation resulting in delay of treatment. This report serves to raise suspicion of AITL and other polymorphic cellular lymphomas in the setting of reactive granulomatous cytomorphology, thus prompting histological examination of tissue biopsy, expediting treatment, and ultimately providing potential improvement to the current prognosis.

Chemotherapy combined with radiotherapy for successful treatment of angioimmunoblastic T-cell lymphoma: a case report.

BACKGROUND: The incidence of angioimmunoblastic T-cell lymphoma is rare worldwide, and it has a poor prognosis. There is no proven or standard first-line therapy that works for the majority of patients with angioimmunoblastic T-cell lymphoma because of the rarity of this disease. The treatment and management are challenging for clinicians. CASE PRESENTATION: This report presents the diagnosis and treatment of a 65-year-old Chinese man who presented with cough and lymph node swellings in the left axillary region. The patient was diagnosed with angioimmunoblastic T-cell lymphoma. He underwent eight cycles of chemotherapy with CHOP (cyclophosphamide, hydroxydaunorubicin, oncovin, prednisone) followed by TOMO radiotherapy (helical tomotherapy, a kind of radiotherapy for cancer treatment using spiral computed tomographic scanning). After treatment, the therapeutic effects were evaluated by magnetic resonance imaging and computed tomography about every 3 months. The patient recovered well with no sign of tumor recurrence and no obvious severe treatment-related adverse effects. CONCLUSION: This treatment experience indicates an essential role for the combination of radiation therapy with CHOP, which may have a better prognosis than treatments without radiation therapy. But challenges warrant further validation in prospective studies.

CT and PET/CT findings of T-cell lymphoma.

OBJECTIVE: The purpose of this study was to describe the extranodal features of T-cell lymphoma at CT and PET/CT. CONCLUSION: The extranodal features of T-cell lymphoma are not specific and usually cannot be used to differentiate T-cell lymphoma from other aggressive types of lymphoma. Noncutaneous subtypes frequently manifest with visceral involvement. The goal of CT in initial staging is to exclude visceral involvement. Evidence on the utility of PET/CT is promising, showing high diagnostic value in evaluation of occult disease and treatment response, but the role of PET/CT is evolving.

Discussion of 18F-FDG PET/CT imaging characteristics and diagnostic values of angioimmunoblastic T-cell lymphoma.

The aim of this study was to discuss 18F-FDG PET/CT imaging characteristics and diagnostic values of angioimmunoblastic T-cell lymphoma (AITL). The PET/CT features of 24 cases of pathologically confirmed AITL were analyzed. The individual and total diagnostic values of 13 sites using computed tomography (CT) and positron emission tomography (PET)/CT were calculated. The maximum standard uptake value (SUVmax) of lesions with lymph node infiltration and extranodal organ infiltration were 5.4-25.1 (median, 9.7) and 1.5-12.5 (median, 5.5), respectively. The diagnostic sensitivity, specificity and accuracy of AITL using CT were 78.3%, 100%, and 87.8%, respectively; using PET/CT, these values were 98.9%, 100%, and 99.4%, respectively. One patient undergoing PET/CT staging was upstaged from stage II to III, and two patients were upstaged from stage III to IV. PET/CT is very valuable in the diagnosis and staging of AITL.

Value of interim FDG PET/CT for predicting outcome of patients with angioimmunoblastic T-cell lymphoma.

Subjects were 45 patients with angioimmunoblastic T-cell lymphoma (AITL) who underwent 2-[18F]-fluoro-2-deoxy-d-glucose (FDG) positron-emission tomography/computed tomography (PET/CT) at baseline and interim after 2-4 cycles. Predictors of progression-free survival (PFS) and overall survival (OS) were assessed. Positive interim PET/CT (Deauville score ≥3) was a significant independent predictor of poor PFS (Hazard ratio, 4.42; p=.028), and showed marginal significance to predict OS (p=.065). Less than 60% decrease in the average change of maximum standardized uptake value normalized by lean body mass (SULmax) also was a significant independent predictor of poor PFS (Hazard ratio, 12.96; p=.001) and poor OS (Hazard ratio, 24.11; p=.006). Interim PET/CT has a significant prognostic value for predicting PFS and OS in patients with AITL. Deauville score and percent decrease of SULmax have the potential to be useful parameter in classifying patients into good and poor responders.

[A case of angioimmunoblastic T-cell lymphoma with interstitial shadow which disappeared after injection of hydrocortisone].

A case of angioimmunoblastic T-cell lymphoma (AITL) was reported. A 56-year-old woman was admitted because of high fever and systemic lymphademopathy. Her chest X-ray on admission showed mediastinal and bilateral hilar lymphademopathy (BHL) and interstitial shadows in both lower lung fields. Chest CT scan revealed thickening of interlobular septum and bronchovascular bundles. There was a remarkable elevation in serum soluble interleukin-2 receptor. A biopsy of cervical lymph nodes for histopathological examination revealed AITL. After intravenous injection of 200mg of hydrocortisone for 7 days, the interstitial shadows and BHL disappeared. It was suspected that interstitial shadow was caused by pulmonary infiltration of AITL.

Angioimmunoblastic lymphadenopathy presenting as superior vena caval obstruction.

A 60-year-old man presented with features of superior vena cava (SVC) obstruction. On evaluation, he was diagnosed as having angioimmunoblastic lymphadenopathy with dysproteinemia (AILD). SVC obstruction due to AILD, to our knowledge, has not been described.

The lung in immunoblastic lymphadenopathy.

Immunoblastic lymphadenopathy presents with fever, malaise, cough, dyspnea, lymphadenopathy, hepatosplenomegaly, hypergammaglobulinemia, interstitial pulmonary infiltrates, mediastinal adenopathy, and effusions. The diagnosis is made on the basis of lymph node biopsy. Based on the course in our patients and the cases presented in previous reports, a suggested program of treatment for immunoblastic lymphadenopathy includes administration of moderate doses of steroids initially, with a subsequent increase to a higher dosage if desired improvement does not occur. Chemotherapy with three drugs, cyclophosphamide, vincristine, and prednisone, is indicated if remission using steroids fails. The diagnostic dilemma of whether pulmonary infiltrates are due to the disease itself or to pulmonary infection or to cytotoxic changes from chemotherapy always exists and often requires specimens from either transbronchoscopic or open-lung biopsy for definitive diagnosis and treatment.

Immunoblastic lymphadenopathy-like T-cell lymphoma complicated by multiple gastrointestinal involvement.

We report a rare case of immunoblastic lymphadenopathy (IBL)-like T-cell lymphoma complicated by multiple gastrointestinal involvement, which appeared to be ameliorated by chemotherapy but resulted in perforative peritonitis. A 66-year-old Japanese woman who had generalized lymphadenopathy and eruptions was admitted to our hospital because of bloody stool. Colonoscopic examination revealed hemorrhagic ulcers in the terminal ileum and a saucer-like ulcer in the cecum. Gastrointestinal endoscopy revealed several ulcerative or elevated lesions in stomach and duodenum. Biopsy specimens of these lesions and of a lymph node showed characteristic histological features of IBL-like T-cell lymphoma. The initial treatment with prednisolone (PSL) and cyclophosphamide (CPA) was effective. Six months after the treatment, however, she developed bloody stool again caused by multiple ulcerative lesions in the large intestine. The recurrence of the disease was determined histologically, and four courses of CPA, PSL, vinblastine sulfate and doxorubicin hydrochloride (CHOP) therapy were administered. One month after completing the CHOP therapy, she developed intestinal obstruction and then acute peritonitis resulting from perforation at an ulcer scar in the jejunum. Surgical treatment was successful, and histological examination demonstrated no lymphoma cells in the resected specimen. A gastrointestinal perforation should be recognized as a potential complication of IBL-like T-cell lymphoma, even during remission.

Lymphography in angio-immunoblastic lymphadenopathy.

Angio-immunoblastic lymphadenopathy (AIL) produces a characteristic lymphoproliferative syndrome, with characteristic lymphographic appearances. The authors studied the lymphographic manifestations of 18 cases of AIL. In 15 out of 18 cases, enlargement is observed in all the lymph nodes with a lacy or reticular storage pattern and sharp, well delimited margins. This lymphographic appearance reflects the histologic changes; only the peripheral sinuses, which are not involved, can be observed on lymphography.

Pulmonary involvement in angio-immunoblastic lymphadenopathy (A.I.L.D.). Case report and review of literature.

A.I.L.D. is a hyperimmune disorder of the B-cell system of unknown etiology. Although it is distinguished morphologically from Hodgkin's disease, the clinical manifestations may be similar. Pulmonary localisation of A.I.L.D. is infrequently seen. We report the radiologic and pathologic findings of a pulmonary localisation of A.I.L.D. The infiltrates were first localised in the interstitium, developing into complete consolidation of the lung bases. It is important to remember that although superimposed infection is a frequent complication, specific interstitial infiltration may occur and is often fatal.

Polyarthritis, rash and lymphadenopathy: case reports of two patients with angioimmunoblastic lymphadenopathy presenting to a rheumatology clinic.

Two patients presented with a symmetrical inflammatory polyarthropathy. Both patients fulfilled the diagnostic criteria for angioimmunoblastic lymphadenopathy. We present the two case histories and review the current literature. Although an uncommon disease, the diagnosis of angioimmunoblastic lymphadenopathy should be considered in a patient presenting with polyarthritis and skin rash.

The role of chest computed tomography in the diagnosis of drug-related reactions.

Computed tomography (CT) of the chest provides important information toward the diagnosis of drug-induced lung disease. CT's ability to demonstrate subtle parenchymal and pleural changes, small nodules, and adenopathy is valuable in the early detection of drug-related reactions. CT is also of value in monitoring the appearance, progression, and resolution of pulmonary damage in patients receiving potentially toxic drugs. The CT appearances of specific drug reactions are reviewed, including the spectrum of CT findings in bleomycin toxicity and amiodarone-induced lung disease.

["Transparent circles" lymph glands: an unusual appearance on lymphography in certain proliferative lymphoid syndromes: prognostic value]. / Les ganglions "cerclés transparents". Aspect inhabituel de la lymphographie dans certains syndromes lympho-prolifératifs. Intêrêt pronostique.

The authors describe 27 cases in which a very particular type of lymphographic image was observed, associating disseminated lesions in all the retroperitoneal lymph glands, and an appearance of "circled" glands of very weak density. This image may be rarely observed in Hodgkin's disease and non-Hodgkin type hematosarcomas. It is frequently observed, however, during the course of angio-immunoblastic lymphadenopathy. Whatever the etiology, this image is of particularly poor prognostic significance.

Observation versus neck dissection for positron-emission tomography-negative lymphadenopathy after chemoradiotherapy.

OBJECTIVES/HYPOTHESIS: To analyze outcomes among patients with residual positron-emission tomography (PET)-negative lymphadenopathy after chemoradiotherapy for head and neck cancer based on whether or not they underwent neck dissection. STUDY DESIGN: Retrospective review. METHODS: Fifty-five patients with stage III/IV squamous cell carcinoma of the head and neck were identified with residual PET-negative lymphadenopathy based on standardized uptake value of <3. All patients had been treated with chemoradiotherapy to a median dose of 70 Gy (range, 60-4 Gy). RESULTS: With a median follow-up of 30 months (range, 6-67 months), the 3-year overall survival (85% vs. 81%, P = .57), progression-free survival (88% vs. 88%, P = .42), and local-regional control (96% vs. 100%, P = .68), did not differ between patients treated by neck dissection or observation. CONCLUSIONS: Omission of neck dissection appears to be reasonable for patients with residual lymphadenopathy but negative PET after chemoradiotherapy for head and neck cancer. LEVEL OF EVIDENCE: 4.

Cyclosporine A and reduced-intensity conditioning allogeneic stem cell transplantation for relapsed angioimmunoblastic T cell lymphoma with hemophagocytic syndrome.

No standard therapeutic approaches have so far been established for the treatment of relapsed angioimmunoblastic T-cell lymphoma (AITL), a subtype of non-Hodgkin lymphoma. This case report describes an AITL patient who relapsed with hemophagocytic syndrome (HPS) two months after receiving high-dose chemotherapy (HDCT) supported by autologous peripheral blood stem cell transplantation (PBSCT). The patient was successfully treated with cyclosporine A (CsA) and subsequent allogeneic PBSCT with reduced intensity conditioning regimen (RIST). RIST may deserve consideration for treatment of AITL patients with severe complications such as HPS. Additionally, CsA could be a less-toxic therapeutic option for pre-RIST induction therapy against AITL.