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A 84-year-old female noticed erythema over her whole body for several months and was referred to our department for evaluation of her skin eruption. A physical examination revealed millet-sized erythematous papules and macules all over her body, a high body temperature, and a decreased level of consciousness. A laboratory examination showed an elevated white blood cell count (8200/μl), atypical lymphocytes (3%) and sIL-2R (4030U / ml). Computed Tomography showed systemic lymphadenopathy. A lymph node biopsy taken from the left inguinal lymph node revealed destruction of the lymph nodes, enlargement of the high endothelial venules, and atypical lymphocyte infiltration. Based on the clinical findings and laboratory examination, we diagnosed angioimmunoblastic Tcell lymphoma (AITL). AITL is a relatively rare peripheral T-cell lymphoma with severe systemic symptoms such as fever and lymph node swelling. While approximately half of all cases experience skin symptoms, which are one of the initial symptoms, it is difficult to determine the diagnosis due to the various clinical features or many non-specific rashes. We should keep in mind a differential diagnosis of lymphoma in cases of the presence of persistent eruption, systemic symptoms, and the existence of atypical lymphocytes in peripheral blood.

A 69-year-old man was diagnosed with diffuse large B-cell lymphoma (DLBCL) negative for Epstein-Barr virus-encoded small nuclear RNA 1 (EBER-1) in October 2011, when he was also diagnosed as having a human T-cell leukemia virus type-I (HTLV-1) carrier. He achieved complete response after six courses of R-CHOP therapy. In February 2015, the patient had high fever and markedly elevated serum lactate dehydrogenase (LDH) level. Bone marrow examination revealed infiltration of CD4-positive T-cell malignancy. Based on the tentative diagnosis of adult T-cell leukemia/lymphoma, modified LSG15 therapy was initiated. His symptoms and serum LDH level quickly improved after the start of treatment. During the treatment, HTLV-1 proviral DNA integration was reported negative, allowing his final diagnosis to be peripheral T-cell lymphoma, not otherwise specified (PTCL-NOS). Despite discontinuation of chemotherapy in the middle of the second course due to the patient's preference, complete remission was reached. He remains in clinical remission at 28 months after the treatment discontinuation. Discordant lymphoma of DLBCL and PTCL-NOS in HTLV-1 carrier has not been well characterized and will be discussed with a literature review.

Peripheral T-cell lymphoma (PTCL) is a heterogeneous group of aggressive non-Hodgkin lymphomas (NHLs) with poor prognoses as compared with those of B-cell NHLs. CHOP or CHOP-like regimen has been considered to be the standard treatment for almost all pathological subtypes of PTCL; however, these regimens result in low complete response rate and short progression-free survival (PFS). Due to these insufficient results with CHOP-based chemotherapy, there is an urgent need for more effective and newer therapeutic strategies. The positive results of the ECHELON-2 study, which demonstrated significantly longer PFS with brentuximab vedotin plus CHP therapy in the frontline treatment for CD30-positive PTCLs, have a great impact on our clinical practice. At present, translational research is being actively conducted to elucidate molecular biology in PTCLs, and deep understanding of the underlying molecular mechanism of PTCLs would produce changes in disease classification. Until now, clinical development of novel agents based on the clinical classification has been carried out for all PTCL subtypes, but from now, treatment development based on molecular biology will be strongly required.

The diagnosis of malignant tumors during pregnancy is not uncommon; the incidence is one per six thousand pregnancies. However, the diagnosis of malignant lymphoma-especially T-cell lymphoma-during pregnancy is extremely rare. Thus, the early detection and management of T-cell lymphoma necessitates difficult decision-making. A 30-year-old woman developed consciousness disturbance on postpartum day three. Because brain MRI showed multiple edematous lesions in both hemispheres, vasculitis or encephalitis was initially suspected, and diagnostic therapy was initiated with the administration of steroids. One month later, the patient suddenly developed a subarachnoid hemorrhage followed by acute hydrocephalus. Emergent ventricular drainage and lesion biopsy were simultaneously performed. Based on the findings, the patient was diagnosed with peripheral T-cell lymphoma not otherwise specified(PTCL-NOS). Laboratory findings indicated Epstein-Barr virus(EBV)infection. Moreover, the same diagnosis was supported by breast and bone marrow biopsies. Thus, the brain lesions were presumed to be metastatic in nature. The prognosis of PTCL-NOS is severely poor in pregnant women as diagnosis is delayed owing to limitations of radiological examinations and because symptoms can be confused with those of other diseases or hyperemesis gravidarum. Additionally, the alteration of immunotolerance in association with pregnancy and EBV infection might have influenced the aggressive features of this case. When a pregnant woman presents with neurological symptoms, malignant lymphoma should be considered when making a differential diagnosis.

Froin's syndrome is characterized by a combination of marked coagulation, elevated protein levels, and xanthochromia of the cerebrospinal fluid (CSF). It is due to blockage of CSF flow by a spinal cord mass or results from meningeal irritation during meningitis. However, Froin's syndrome has not been reported in hematological malignancies. Herein, we present two cases of lymphomatous leptomeningitis with Froin's syndrome. A 66-year-old man suffered consciousness disturbance during chemotherapy for a relapse of peripheral T-cell lymphoma, not otherwise specified. An 84-year-old man complained of pain and paralysis in both legs during chemotherapy for diffuse large B-cell lymphoma. In both cases, CSF analysis showed indications of Froin's syndrome, and cytology revealed lymphoma cells. In contrast to the highly elevated CSF protein levels, normal to only mildly elevated cell counts were not associated with advanced leptomeningeal disease. This is the first case report of leptomeningeal involvement in hematological malignancy associated with Froin's syndrome. As there are potentially undiagnosed cases including mild forms, Froin's syndrome should be kept in mind during CSF examination of patients with hematological malignancies.

We retrospectively analyzed clinical and pathological features, treatments, and prognoses in 28 patients with newly diagnosed peripheral T-cell lymphoma, not otherwise specified (PTCL-NOS) in Niigata University Medical and Dental Hospital. Of them, 16 were males and 12 were females, and their median age was 62.5 (range, 26-88) years. The International Prognostic Index was high-intermediate/high in 68% of patients. Twelve patients were treated with CHOP/THP-COP and nine with third-generation chemotherapy regimens. At a median follow-up period of 30 (range: 1-164) months, the 2-year overall survival and progression-free survival rates were 61% and 44%, respectively. Further investigation of novel agents for treating PTCL-NOS is warranted.

A 50-year-old woman was referred to our hospital because a mass lesion had been palpable through the vaginal wall during a cervical cancer screening examination. A contrast-enhanced computed tomography (CT) scan and magnetic resonance imaging (MRI) revealed marked thickening of the vaginal wall, constituting a mass 96 mm in diameter. Abnormal FDG uptake was observed in the vaginal mass, but no other lesions were detected by positron emission tomography (PET/CT). A transvaginal biopsy from the tumor revealed peripheral T-cell lymphoma, not otherwise specified (PTCL-NOS). Although endoscopic examinations revealed no signs of infiltration in either the bladder or the rectum, the MRI findings suggested invasion into the adjacent rectal wall. She achieved complete remission after six courses of biweekly THP-COP therapy, to which field radiation (39.6 Gy) was added. PTCL of the vagina is rare and this case therefore merits description in the literature.

A 77-year-old-man was diagnosed with follicular lymphoma (FL), grade 3A. After six courses of R-THP-COP (rituximab, pirarubicin, cyclophosphamide, vincristine and prednisolone) therapy, he achieved complete remission (CR). He achieved a second CR after radiotherapy, a third CR after six courses of bendamustine /rituximab (BR) therapy, and a fourth CR after six courses of BR therapy. However 2 months after the last chemotherapy, his tumor progressed rapidly and he died. Autopsy results showed medium and large lymphoid cells with pleomorphic, irregular nuclei and prominent nucleoli infiltrated in multiple lymph nodes, the liver, the lung, and the spleen. The lymphoid cells were positive for CD3, CD8, granzymeB, TIA-1 and negative for CD4, CD20, CD79a, CD10, and CD56. Autopsy diagnosis was peripheral T-cell lymphoma, not otherwise specified. Occurrence of lymphoma in T-cell lineage should be considered, if the course of low-grade B-cell lymphomas, such as FL rapidly progresses.

We report a 69-year-old male with CD3-positive peripheral T-cell lymphoma, not otherwise specified (PTCL-nos). Interestingly, tumor cells slightly expressed CD20 as well. Southern analyses of the tumor cells showed rearrangement for only the T cell receptor gene but not the immunoglobulin genes. This patient achieved partial remission with a treatment regimen of THP-COP excluding prednisolone, but died of pneumonia. Although CD20-positive PTCL is rare, a review of the reported cases suggests that CD20-positive PTCL has a poor prognosis and that bone marrow infiltration of tumor cells results in a poorer prognosis in CD20-positive PTCL than in usual PTCL. By accumulating cases of this rare entity of lymphoma, we need to clarify the biological nature of the tumor cells and usefulness of rituximab combined with standard chemotherapy.