T-cell-lymphoma presented as a solitary subcutaneous mass in the ventral cervical region of an adult llama- diagnostic and treatment.

BACKGROUND: Neoplasm in South American camelids (SAC) are commonly described. The most frequently reported type of neoplasm are lymphomas and difference in the age suffering from lymphomas of and llamas is seen. This report describes a case of a solitary lymphoma in a 5 years and 9 month old llama mare displaying the approach of diagnostic imaging and successful surgical treatment. CASE PRESENTATION: The llama was referred to the clinic for dyspnoea and inspiratory abnormal respiratory sounds. The clinical examination comprised blood cell count, ultrasonographic and radiographic examinations, endoscopy and fine needle aspiration cytology of a mass detected in the mid cervical region. The mass was surgically removed. Histopathological examination of the surgically removed mass diagnosed a malignant T-cell- lymphoma. According to the results of the clinical, ultrasonographic and radiographic examinations no tumor invasion was apparent in distant organs and the llama was discharged from the clinic seven days after surgery. CONCLUSION: Lymphoma has been reported to be the most common neoplasia in camelids and are more often described in young alpacas and in adult llamas. To the author´s knowledge the case presented here is the first that described a broad panel of diagnostic tools including ultrasound, radiographs, endoscopy, fine needle aspiration cytology and histopathoogical examination as well as a successful surgical treatment of a solitary lymphoma in camelids.

[Patient with Monomorphic Epitheliotropic Intestinal T-Cell Lymphoma with Small Intestinal Perforation Development during Chemotherapy].

Monomorphic epitheliotropic intestinal T-cell lymphoma(MEITL)is classified under type Ⅱ enteropathy-associated T-cell lymphoma(EATL). It is a rare disease with a low incidence rate. This study reports a case of a patient with MEITL who developed small intestinal perforation during chemotherapy. The patient was a 55-year-old woman who presented to a previous clinic with epigastric pain. Enteroscopy results showed a map-like ulcer in the jejunum. Examination of the tissue specimen collected from this site suggested T-cell lymphoma. The patient was referred to our hospital for chemotherapy. Seven days following the initiation of chemotherapy, an abdominal computed tomography(CT)revealed free air, leading to a diagnosis of gastrointestinal perforation. Emergency surgery was performed. Intraoperatively, bowel perforation and a degenerative ulcer were observed at 95 cm and 80 to 115 cm from the Treitz' ligament, respectively. In addition, all-layer intestinal necrosis was noted 150 and 90 cm from the terminal ileum. Total resection and anastomosis were performed. Postoperatively, the patient developed sepsis due to chemotherapy-related pancytopenia but recovered. She was discharged on postoperative day 24. Subsequently, positron emission tomography(PET)-CT revealed residual intestinal tumor cells and peritoneal dissemination. Chemotherapy was initiated, but there was no response. The patient died after 6.5 months. A radical treatment for MEITL has not yet been established. More case reports are needed to improve the prognosis of this disease.

[Primary cutaneous CD4+ small to medium-sized T-cell lymphoma in an adolescent patient]. / Primäres kutanes CD4+ klein- bis mittelgroßzelliges T-Zell-Lymphom bei einem Jugendlichen.

A rare finding of primary cutaneous CD4+ small to medium-sized T­cell lymphoma (SMPTCL) in a fifteen-year-old patient is reported. This is a rare tumor entity for which there is currently no standardized treatment recommendation. At the interdisciplinary tumor board, the decision was made to resect the tumor and reconstruct the defect with a nasolabial advancement flap in a two-stage process. Follow-up examinations, currently over 3 years, have shown the patient to be free of recurrences.

T-cell Thymic Lymphoma With Proventricular Metastasis in a Florida Scrub Jay ( Aphelocoma coerulescens).

An adult, wild-caught, female Florida scrub jay ( Aphelocoma coerulescens) was evaluated because of an observable mass on the ventral neck. Initial physical examination and diagnostic tests were performed, which revealed a subcutaneous mass. Surgical removal of the mass was attempted, but the bird died during surgery. Results of necropsy and histopathologic evaluation identified the mass as thymic lymphoma with proventricular metastasis. Immunohistochemical staining revealed strong cytoplasmic immunoreactivity for CD3 in the thymic mass and within the predominant lymphoid population in the serosal proventricular masses, which confirmed metastasis of T-cell lymphoma. To our knowledge, this is the first report of T-cell thymic lymphoma in a wild Florida scrub jay.

[A Case of Angioimmunoblastic T-Cell Lymphoma Merged with Colorectal Cancer That We Were Able to Resect after a Chemotherapy Response].

The patient was 77-year-old man. He visited our hospital with the chief complaint of an abdominal mass in March 2015. We diagnosed the patient with transverse colon cancer and he was suspected of having malignant lymphoma. In March 2016, we attempted to perform right hemicolectomy for the transverse colon cancer, but it was difficult because swollen lymph nodes had formed a large mass with the surrounding tissue, including vessels of the mesentery. We could only complete the surgery after mesenteric lymph node biopsy. On the basis of the results of this biopsy, we diagnosed angioimmunoblastic Tcell lymphoma. At first, we administered THP-COP therapy for malignant lymphoma. However, after 3 courses of this therapy, the patient suddenly developed ileus due to the aforementioned colon cancer. According to enhanced CT performed at the onset of intestinal obstruction, chemotherapy dramatically reduced the size of the intraperitoneal lymph nodes. We therefore concluded that it was able to treat the colon cancer. We performed right hemicolectomy in June 2015. Angioimmunoblastic T-cell lymphoma comprises only 2-3% of all malignant lymphoma cases. We experienced a rare case of complications from angioimmunoblastic T-cell lymphoma and colorectal cancer.

Hematopoietic stem cell transplantation for primary cutaneous γδ T-cell lymphoma and refractory subcutaneous panniculitis-like T-cell lymphoma.

BACKGROUND: The panniculitic T-cell lymphomas (TCLs) comprise 2 distinct entities, αß subcutaneous panniculitis-like TCL (SPTCL) and the γδ cutaneous TCLs with pannicular involvement primary cutaneous γδ (PCGD)-TCL. Although outcomes for most patients with SPTCL are favorable, those with PCGD-TCLs generally have an inferior outcome, and treatment strategies have not been well defined. Allogeneic hematopoietic stem cell transplantation (HSCT) has been shown to be a potentially curative strategy in aggressive TCLs and in refractory and advanced-stage mycosis fungoides. OBJECTIVE: We sought to analyze the outcomes of HSCT for panniculitic cutaneous TCL. RESULTS: Fourteen patients (4 SPTCL, 10 PCGD-TCL) presented with primarily pannicular T-cell infiltrates. Seven patients underwent allogeneic HSCT from matched-related donors and matched-unrelated donors of which 4 (57%) are alive (1 SPTCL, 3 PCGD-TCL) at 7.8, 6.9, 6.2, and 0.25 years. Two patients underwent autologous HSCT (1 SPTCL, 1 PCGD-TCL) and both are alive at a median follow-up of 1.91 years. LIMITATIONS: This study is limited by its retrospective nature and small sample size because of the rarity of SPTCL and PCGD-TCL. CONCLUSION: Aggressive therapy followed by allogeneic HSCT is a promising treatment modality for patients with PCGD-TCL.

Pediatric subcutaneous panniculitis-like T-cell lymphoma with favorable result by immunosuppressive therapy: a report of two cases.

Subcutaneous panniculitis-like T-cell lymphoma (SPTL) is a rare type of skin lymphoma. Histopathology mimicking a lobular panniculitis makes it difficult to distinguish SPTL from benign autoimmune disease. We present cases of a 10-year-old female and an 11-year-old male with SPTL showing recurrent panniculitis and systemic manifestations. Initially, antibiotics and steroids were administered to treat infectious disease and benign panniculitis. However, they experienced recurrent fever and erythema nodosum. Additional immunohistochemistry and T-cell receptor (TCR) gene rearrangement analyses were performed, enabling the establishment of an SPTL diagnosis. The affected patients were given immunosuppressive therapy with favorable results.

Acute abdomen as the first presentation of a malignant lymphoma of the small intestine.

Malignant small intestinal lymphoma is a quite rare disease that often has an atypical presentation with symptoms of abdominal pain, nausea and weight loss. However, as illustrated in the following cases, acute abdomen due to an intestinal perforation can be the first sign of malignant lymphoma.

Allogeneic stem cell transplantation with BEAM and alemtuzumab conditioning immediately after remission induction has curative potential in advanced T-cell non-Hodgkin's lymphoma.

Patients with relapsed or refractory advanced T cell non-Hodgkin lymphoma have a dismal prognosis and may not even reach allogeneic hematopoietic stem cell transplantation (HSCT) in adequate condition. We present the outcome of 24 consecutive patients (age range 11 to 65 years) treated at a single institution in Kiel within a recent 5.5-year time frame with allogeneic HSCT in a rather uniform approach. Relapsed and refractory T and natural killer cell lymphomas of various subtypes were included. All patients except 1 were in progression or relapse before start of pretransplantation salvage therapy. Five patients had relapsed after autologous HSCT. With intensive remission induction therapy, usually the CLAEG (cladribine, cytosine arabinoside, and etoposide with granulocyte colony-stimulating factor support) protocol, attempts were made to improve disease control and proceed immediately to conditioning with carmustine, etoposide, cytosine arabinoside, melphalan (BEAM), and medium-dose alemtuzumab. Twenty of 21 patients who received CLAEG induction therapy benefited from this protocol and 1 patient appeared to be therapy-resistant. At the time of allogeneic HSCT, 9 patients were in complete remission (CR) (2 in CR1, 5 in CR2, and 2 in CR >2), whereas 50% had never achieved CR. Nineteen transplants were obtained from matched or partially matched unrelated donors and only 5 from siblings. With a median follow-up of 321 days (1252 days for surviving patients), 20 of 22 assessable patients reached CR. Five of these patients had hematologic or molecular relapse. With donor lymphocyte infusions, 1 patient became minimal residual disease MRD negative again and has maintained CR for more than 4 years. The frequency of grades II to IV acute graft-versus-host disease was 25% and chronic graft-versus-host disease, 30%. Intense reinduction therapy followed by reduced-intensity BEAM-alemtuzumab conditioning and allogeneic HSCT is effective and offers curative potential for patients with advanced T cell lymphomas, even for those not in remission.

Diagnostic exercise: Submucosal gastric masses in a cat.

A 14-year-old neutered male cat presenting with chronic vomiting had 2 masses within the submucosa of the stomach that were excised. They presented histologically as circumscribed, submucosal masses consisting of diffusely arranged medium-sized round cells with a moderate amount of cytoplasm and interspersed eosinophils, separated by trabecular fibroblastic stroma. The overlying mucosa was diffusely infiltrated by the same round cells, and marked epitheliotropism was present. Neoplastic cells labelled positive for CD3 and negative for CD79a and CD117. Giemsa staining and silver staining (SNOBA) were also negative. A T-cell lymphoma with reactive fibroplasia was diagnosed, and differential diagnoses including mast cell tumor and feline gastrointestinal eosinophilic sclerosing fibroplasia could be excluded.

[Diagnosis and treatment of primary colorectal non-Hodgkin's lymphoma: analysis of 52 cases].

OBJECTIVE: To summarize and analyze the diagnosis, clinical features and therapy of primary colorectal non-Hodgkin's lymphoma (NHL). METHODS: The clinicopathological data of 52 patients with primary colorectal NHL diagnosed and treated in our department from January 2000 to January 2010 were reviewed and analyzed retrospectively in this study. RESULTS: This group of patients was composed of 45 cases of B cell and 7 T cell lymphomas, including 33 males and 19 females, with a male to female ratio of 1.7:1, and the age at diagnosis was 16 - 74 years old, with a median age of 50 years. The ileocecal region was most frequently involved site, acounted for 48.1%. The common symptoms encountered were abdominal pain (66.7%), diarrhea (15.6%), blood stool (24.4%), and body weight loss (8.9%). All patients were eventually diagnosed by histopathology, and the DLBCL subtype took up 64.4%. Among the 45 cases of B cell subtype, 33 cases (73.3%) were of early stage (IE and IIE confirmed), and the 5-year survival rate was 78.1%, while those of stage IIIE and IVE comprised 26.7%, with a 5-year survival rate of 45.5% (P < 0.05). The 5-year survival rate of all patients was 71.1%. Surgery was employed in 36 cases, and 9 patients received chemotherapy alone. Radical surgery could significantly increase the patients' overall survival rate, as compared with the chemotherapy alone group and palliative surgery group (P < 0.05). CONCLUSIONS: Colorectal non-Hodgkin's lymphoma is a rare malignancy of the gastrointestinal tract. B cell type, male predominance and DLBCL subtype are most encountered manifestations in clinics. Multi-modality management with radical surgical resection of the primary lesion followed by standard chemotherapy, affords better local disease control, and a better survival outcome. Early detection and tailored immunotherapy can obviously prolong the long-term survival time.

Primary diffuse choroid plexus T-cell lymphoma: case report.

We present here a case of primary choroid plexus T-cell lymphoma with no evidence of immunodeficiency or immunological disease. As ventricular T-cell lymphoma is extremely rare, there is only limited information on the radiological findings of ventricular T-cell lymphoma. In this report, we also include some unusual MRI findings in this case that have never been described before.

Long-term salivary function after conditioning with busulfan, fractionated or single-dose TBI.

OBJECTIVES: Does conditioning with fractionated total body irradiation (fTBI) or busulfan (Bu) causes less salivary dysfunction compared with single dose (sTBI) after hematopoietic stem cell transplantation (HSCT). PATIENTS AND METHODS: A total of 74 adolescents below 13 years of age received allogeneic HSCT and conditioning with either: sTBI, fTBI or Bu. The unstimulated (USSR) and stimulated (SSSR) whole salivary secretion rates were measured at 15 years of age. RESULTS: Irrespective of conditioning type, there were no significant differences in USSR or SSSR between groups. Girls had a significantly lower SSSR, 0.7 ± 0.3 ml per min compared with 1.1 ± 0.4 ml per min in boys (P < 0.001). A significant correlation between age at HSCT and SSSR at 15 years of age (P = 0.02) in children conditioned with sTBI was found as well as an inverse correlation between the plasma area under curve (AUC) of Bu and SSSR. In the multivariate model, only female sex was significantly correlated with low SSSR at 15 years of age (OR 3.93, 95% CI 1.21-12.79; P = 0.021). CONCLUSION: No differences in long-term whole salivary function after HSCT in adolescents receiving conditioning with sTBI, fTBI or Bu were found. Total systemic exposure to Bu was negatively correlated with stimulated salivary secretion.

Nasal NK/T cell lymphoma mimicking an latrogenic lateral nasal wall infection: a diagnostic dilemma.

Extra nodal NK/T cell lymphoma, 'nasal type' is a rare clinicopathological entity. The prevalence of nasal lymphoma is estimated at 0.17-1.5% for all non -Hodgkin's lymphomas (NHL), of which 45% originate from the NK/T cell. It is more commonly encountered in Asian countries. The main clinical features are nasal congestion and epistaxis due to local aggressive destruction. It has a distinct immunophenotypic profile of CD2+, CD56+ and CD3-. The tumor often shows polymorphic lymphoreticular infiltrates and necrosis. We present a case of a 50-year-old male who presented with lateral nasal wall infection following endoscopic sinonasal surgery and later proven to be extranodal NK/T cell 'nasal type' lymphoma with immunophenotypic features.

Successful treatment of a primary gastric T-cell lymphoma lacking the human T-cell leukemia virus type 1.

Primary gastric lymphomas generally are of B-cell lineage. Among the previously reported cases of exceptional primary gastric T-cell lymphomas, most demonstrate evidence of human T-cell leukemia virus type 1 (HTLV-1) infection with a poor prognosis. The present study is a report of a rare case of primary gastric T-cell lymphoma without HTLV-1 which could be successfully treated with surgical resection and adjuvant chemotherapy. The patient was a 50-year-old Japanese male who presented with epigastric pain. Clinical examination showed no lymphadenopathy or skin changes. Radiographic and endoscopic examinations revealed an ill-demarcated ulcerative lesion in the stomach, and a biopsy specimen confirmed high-grade lymphoma. Total gastrectomy with regional lymph node dissection was performed. Histological examination of the gastric lesion revealed a malignant lymphoma, diffuse large cell type, with lymph nodal involvement. On immunohistochemistry, tumor cells were positive for CD3, CD4 and CD30, but negative for CD8, CD20 and CD56, implying a T-cell nature. Following surgery, the patient received 8 cycles of chemotherapy (cyclophosphamide, doxorubicin, vincristine, and prednisolone/CHOP). To date the patient has been free of recurrence for 86 months without further treatment. Review of previously reported cases of primary gastric T-cell lymphoma indicates that the prognosis is better without than with HTLV-1. We conclude that primary gastric T-cell lymphomas without HTLV-1 should be managed in the same way as the more common diffuse large B-cell type gastric lymphomas.

[Primary subcutaneous panniculitis-like T-cell cutaneous lymphoma: clinical presentation, treatment and prognosis].

OBJECTIVE: To explore the clinical presentation, treatment and prognosis study of primary subcutaneous panniculitis-like T-cell cutaneous lymphoma (SCPTCL). METHODS: Ten cases of SCPTCL, treated in our hospital from January 1999 to January 2009, were included in this study. Their clinicopathological data were reviewed and analyzed retrospectively. RESULTS: the median age was 50.5 years (range: 10 - 58), 4 males and 6 females. There were seven CD56 positive, two negative cases and 1 unclear case. Four cases had repeatedly nodules regressed spontaneously without treatment before diagnosis and new nodules appeared at different sites. Seven patients presented with multiple subcutaneous nodules or deeply seated plaques, most commonly on the extremities and trunk. Ulceration of nodules occurred in 3 cases, and the lesions were painful in five cases. The lesions appeared nodules at the beginning, and then gradually grew into tumors. Four patients had abnormal liver function and one patient had hemophagocytic syndrome (HPS), four patients had lymphadenopathy or visceral involvement. Three cases with single lesion underwent surgical excision in combination with chemotherapy or chemotherapy/radiotherapy. One case lost follow up, and two cases live without disease. Among the seven patients with multiple lesions, lymphadenopathy or visceral involvement, one underwent local surgical excision and is alive without disease, six of them received chemotherapy or multi-modality treatment mainly with chemotherapy. Three of these 6 cases are alive without progression, one used histone deacetylase inhibitors after progression and obtained partial regression, and 2 died. The median follow-up for all the 10 patients was 44 months (range: 14 - 99). The progression free survival was 66.7% (6/9), and overall survival was 77.8% (7/9). CONCLUSION: SPTCL has an indolent course, some lesions can regress spontaneously and relapse again. Patients with single lesion may live long-term without disease after multimodality therapy. Patients with multiple lesions or extracutaneous involvement are sensitive to CHOP-like regimen, but the duration of remission is short. Histone deacetylase inhibitors may be a promising drug in the treatment for SCPTCL relapse.

[Clinicopathologic study of 7 cases of gastric T-cell lymphoma].

OBJECTIVE: To study the clinicopathologic characteristics of gastric T-cell lymphoma. METHODS: The clinicopathologic features of 7 cases of gastric T-cell lymphoma were retrospectively reviewed. Immunohistochemical study, T-cell receptor gene rearrangement analysis and evaluation of Epstein Barr virus (EBV) status were also performed. RESULTS: The median age at onset of gastric T-cell lymphoma was 45 years. The male-to-female ratio was 6 to 1. The clinical information was available in 6 cases; and one of them had history of persistent diarrhea and 5 had hypoproteinemia. Histologically, 5 cases consisted of large lymphoma cells and the remaining 2 cases showed mainly medium-sized cells. Intraepithelial lymphoma cell infiltration was found in one case. The lymphoma cells of all cases were negative for CD20 and CD79a. CD3 and TIA-1 expression was noted in 6 of the 7 cases. CD5, ßF-1 and CD30 were positive in 4 cases and CD4 was positive in 3 cases. In-situ hybridization for Epstein-Barr virus-encoded RNA was negative. Clonal T-cell receptor gene rearrangement was demonstrated in all cases. CONCLUSION: Gastric T-cell lymphoma is a rare type of malignant lymphoma, with distinctive clinicopathologic characteristics.

T Cell Primary Effusion Lymphoma in an HIV-Negative Man with Liver Cirrhosis.

BACKGROUND Primary effusion lymphoma (PEL) is a rare and aggressive non-Hodgkin lymphoma (NHL) that is responsible for 1% of all lymphomas not related to human immunodeficiency virus (HIV). PEL is characterized by human herpesvirus-8 (HHV-8) positivity in the absence of overt tumor burden that does not exhibit typical B cell or T cell immunophenotype characteristics. The exact mechanism of development is unknown, but it is hypothesized to develop from post-germinal B cell origin. Although it is most common in HIV patients, other immunocompromising comorbidities can be seen in conjunction with PEL, including liver cirrhosis. CASE REPORT We present the case of a 73-year-old HIV-seronegative man with alcohol-induced liver cirrhosis who was found to have T cell PEL of the pleural space diagnosed by thoracentesis. CONCLUSIONS Little is known regarding oncogenesis of T cell PEL, and few studies exist regarding appropriate treatment regimens for PEL as a whole, prompting need for further investigation and discussion to improve survival rates. Even in the absence of active HIV infection, PEL should be considered as a potential cause of pleural effusion in cirrhotic patients in order to prompt earlier treatment for the best chance of survival.