Primary peripheral T-cell lymphoma of the cervix with mononeuritis multiplex: an unusual case presentation

Peripheral neuropathy (PN) is characterized by the injury to the peripheral nervous system of varied etiology. Lymphoma is one of the etiologies of PN, presenting various neurological manifestations. Neuropathy associated with peripheral T-cell lymphoma, not otherwise specified (PTCL, NOS) is unusual and fewer cases are documented in the literature. In addition, PTCL, NOS is extremely rare as primary in the female genital tract, especially uterine cervix, and exhibits aggressive clinical course with poor therapy response. We hereby describe a 47-year-old female who presented with fever and chills for 15 days. Clinical examination revealed left-sided lower motor neuron type of facial nerve palsy with Bell's phenomenon. Nerve conduction study of all four limbs illustrated asymmetrical axonal neuropathy (motor > sensory), suggesting mononeuritis multiplex. She developed vaginal bleeding during her hospital stay. Pelvic examination and imaging revealed a 4x3cm polypoidal mass on the posterior lip of the cervix, which was excised and diagnosed as extranodal primary PTCL, NOS based on morphology, immunohistochemistry, and in-situ hybridization findings. Besides, the cerebrospinal fluid (CSF) was infiltrated by the lymphoma cells, detected on cell block preparation. The patient succumbed to her illness within one week despite best efforts and the commencement of chemotherapy. No consent was obtainable for nerve biopsy and autopsy. Thus, we report an extremely rare case of primary extranodal PTCL, NOS of the uterine cervix with unusual presentation of mononeuritis multiplex. Further, we discussed the differentials of PTCL, NOS at this extranodal site.

Angioimmunoblastic T-cell lymphoma with fever, arthritis and skin pigmentation: A case report / 北京大学学报(医学版)

Angioimmunoblastic T-cell lymphoma is a rare T-cell lymphoma. The clinical manifestations are not specific. In addition to the common clinical manifestations of lymphomas such as fever, weight loss, night sweats and lymphadenopathy, it may also have skin rashes, arthritis, multiple serous effusions, eosinophilia and other systemic inflammatory or immune symptoms. The lymphoma cells of angioimmunoblastic T-cell lymphoma originates from follicular helper T cells, and the follicular structure of lymph nodes disappears. In the tumor microenvironment, in addition to tumor cells, there are a large number of over-activated immune cells, such as abnormally activated B cells, which produce a series of systemic inflammation or immune-related symptoms. This disease is rare and difficult to diagnose. This article reports a 36-year-old female. She got fever, joint swelling and pain, skin pigmentation, accompanied by hepatomegaly, splenomegaly, lymphadenopathy, anemia and other multiple-systems manifestations. The clinical manifestations of this patient were similar to autoimmune diseases such as adult onset Still's disease, rheumatoid arthritis, and systemic sclerosis, which made the diagnosis difficult. At the beginning of the disease course, the patient got arthritis and fever. And her white blood cells were significantly increased. Adult onset Still's disease should be considered, but her multiple-systems manifestations could not be explained by adult onset Still's disease. And her arthritis of hands should be distinguished with rheumatoid arthritis. However, the patient's joint swelling could get better within 3-7 days, and there was no synovitis and bone erosion on joint imaging examination. The rheumatoid factor and anti-CCP antibody were negative. The diagnostic evidence for rheumatoid arthritis was insufficient. The patient's skin pigmentation and punctate depigmentation were similar to those of systemic sclerosis. But the patient had no Raynaud's phenomenon, and her sclerosis-related antibody was negative. The diagnostic evidence for systemic sclerosis was also insufficient. After 3 years, she was finally diagnosed with angioimmunoblastic T-cell lymphoma by lymph node biopsy aspiration. This case suggests that the clinical manifestations of angioimmunoblastic T-cell lymphoma are diverse, and some symptoms similar to immune diseases may appear. When the patient's clinical symptoms are atypical and immune diseases cannot explain the patient's condition, and further evidence should be sought to confirm the diagnosis.

Primary nodal peripheral T-cell lymphomas: diagnosis and therapeutic considerations

Nodal peripheral T-cell lymphomas are a rare group of neoplasms derived from post-thymic and activated T lymphocytes. A review of scientific articles listed in PubMed, Lilacs, and the Cochrane Library databases was performed using the term "peripheral T-cell lymphomas". According to the World Health Organization classification of hematopoietic tissue tumors, this group of neoplasms consists of peripheral T-cell lymphoma not otherwise specified (PTCL-NOS), angioimmunoblastic T-cell lymphoma (AITL), anaplastic large cell lymphoma-anaplastic lymphoma kinase positive (ALCL-ALK+), and a provisional entity called anaplastic large cell lymphoma-anaplastic lymphoma kinase negative (ALCL-ALK-). Because the treatment and prognoses of these neoplasms involve different principles, it is essential to distinguish each one by its clinical, immunophenotypic, genetic, and molecular features. Except for anaplastic large cell lymphoma-anaplastic lymphoma kinase positive, which has no adverse international prognostic index, the prognosis of nodal peripheral T-cell lymphomas is worse than that of aggressive B-cell lymphomas. Chemotherapy based on anthracyclines provides poor outcomes because these neoplasms frequently have multidrug-resistant phenotypes. Based on this, the current tendency is to use intensified cyclophosphamide, doxorubicin, vincristine, prednisolone (CHOP) regimens with the addition of new drugs, and autologous hematopoietic stem cell transplantation. This paper describes the clinical features and diagnostic methods, and proposes a therapeutic algorithm for nodal peripheral T-cell lymphoma patients...

Three Cases of Candidiasis Misidentified as Candida famata by the Vitek 2 System

No abstract available.

Linfoma primario de células T del páncreas: presentación de un caso clínico / Primary T cell lymphoma of the pancreas: clinical case report

El Linfoma Primario de Páncreas es una enfermedad rara, representando el 1 % de todos los linfomas de presentación extranodal y el 0,5% de todas las masas pancreáticas. Menos de 150 casos han sido reportados en la literatura en ingles, los cuales son generalmente linfomas de células tipo B. Los linfomas de celulas T, representan aproximadamente el 4% de los linfomas primarios de pancreas y la supervivencia a los 5 años es del 0%. Reportamos el caso de una mujer de 28 años de edad que se presento a la emergencia con una colangitis aguda severa y una historia insidiosa de baja de peso e icteria obstructiva. La tomografía revelo una lesión tumoral heterogénea, difusa en cabeza de páncreas asi como dilatación leve del conducto pancreático, dilatación de vía biliar intra y extra-hepatica, no adenopatías retroperitoneales y sin infiltración hepática ni esplénica. Se realizo una autopsia dirigida y los estudios histopatologicos confirmaron un linfoma no Hodgkin de células T, CD3 + CD20-.

Primary pancreatic lymphomas (PPL) are rare tumors, comprising 1% of extra-nodal lymphomas and 0.5% of all pancreatic masses. Fewer than 150 cases have been reported worldwide, which most commonly are large B cell lymphomas. T cell lymphomas comprise 4% of all PPL and present a 5-year survival rate of 0%. We report the case of a 28 year-old peruvian woman who presented with a fatal acute cholangitis and a history of insidious weight loss and obstructive jaundice. The CT scan revaled a diffuse heterogeneus mass in the head of the pancreas along with a mildly dilated pancreatic duct and dilated intra and extra-hepatic bile ducts, no liver, splenic involvement, or retroperitoneal adenopathies were evident. An autopsy was performed and the histopathologic investigation confirmed a T cell non-Hodgkin lymphoma, CD3+ CD20-.

HTLV 1 associated adult T cell lymphoma/leukemia a clinicopathologic, immunophenotypic tale of three cases from non-endemic region of south India.

Adult T cell lymphoma/leukemia is a peripheral T-cell neoplasm caused by human T-cell lymphotrophic virus-1, affects mostly adults with systemic involvement and poor prognosis. Diagnosis of adult T-Cell leukemia/Lymphoma is challenging. The clinico-pathologic and immuno-phenotypic features of the three cases will be presented.

Mainly Adrenal Gland Involving NK/T-Cell Nasal Type Lymphoma Diagnosed with Delay due to Mimicking Adrenal Hemorrhage

A 29-yr-old man, presented with abdominal pain and fever, had an initial computed tomography (CT) scan revealing low attenuation of both adrenal glands. The initial concern was for tuberculous adrenalitis or autoimmune adrenalitis combined with adrenal hemorrhage. The patient started empirical anti-tuberculous medication, but there was no improvement. Enlargement of cervical lymph nodes were developed after that and excisional biopsy of cervical lymph nodes was performed. Pathological finding of excised lymph nodes was compatible to NK/T-cell lymphoma. The patient died due to the progression of the disease even after undergoing therapeutic trials including chemotherapy. Lymphoma mainly involving adrenal gland in the early stage of the disease is rare and the vast majority of cases that have been reported were of B-cell origin. From this case it is suggested that extra-nodal NK/T-cell lymphoma should be considered as a cause of bilateral adrenal masses although it is rare.

Immunophenotyping of mature T/NK cell neoplasm presenting as leukemia.

Introduction : Mature T/NK cell lymphomas (MTNKL) presenting as leukemia are rare and show considerable overlapping of clinical, morphological and immunophenotypic features. AIM: Critical analysis of the morphology and immunophenotypic profile of MTNKL. Materials and Methods : We reviewed 380 consecutive cases of mature lymphoid neoplasm that presented as leukemia and were diagnosed on morphology and immunophenotyping of bone marrow and/or peripheral blood samples. Results : Peripheral blood and bone marrow involvement was seen in all cases. MTNKL constituted 4% (nine cases) of all mature lymphoid neoplasms presenting as leukemia. It included four cases of T-large granular leukemia (T-LGL), two of T-cell prolymphocytic leukemia small cell variant (T-PLL), two of adult T-cell leukemia/lymphoma (ATLL) and one of primary cutaneous gamma delta T-cell lymphoma (PCGDTCL). T-LGL revealed CD4-/CD8+ phenotype in three, and CD4+/CD8+ phenotype in one case. CD56 was absent in all the cases of T-LGL. One case of T- PLL small cell variant showed CD4+/CD8- phenotype, while the other revealed CD4-/CD8+ phenotype. Both cases of ATLL showed CD4+/CD8+/CD25+ phenotype. The single case of PCGDTCL showed CD4-/CD8- phenotype pattern. CD3 and CD5 were expressed in all MTNKL. CD7 was absent in three cases of T-LGL. TCRα/β was performed in three cases of T-LGL and was positive in all. TCRα/β was also seen in both the cases of T-PLL small variant. However, TCRα/β was seen in the single case of PCGDTCL. Conclusion : Mature nodal T/NK cell neoplasms are rare and MTNKL presenting as leukemia are even rarer. There is an overlap between the immunophenotypic profiles of different MTNKL subtypes and elaborate T/NK cell panels are required for their evaluation.

Linfoma não-Hodgkin de células T em cavidade oral: relato de caso / T-cell non-Hodgkin's lymphoma in oral cavity: a case report

Paciente se apresentou ao serviço de Diagnóstico e Cirurgia Buco-Maxilo-Facial queixando-se de uma úlcera dolorosa no palato associada a disfagia. Ao exame intrabucal pôde-se notar úlcera gigantiforme em palato duro e mole exibindo bordas elevadas com presença periférica de tecido granulomatoso e área central de necrose coberta por exsudato fibrinoso. Os exames laboratoriais revelaram sorologia negativa para HIV e HTLV-1 e 2 e o diagnóstico histopatológico foi de linfoma maligno difuso de células T pleomorfo. A proposta deste trabalho, portanto, é apresentar um caso clínico de linfoma não-Hodgkin de células T, em cavidade oral, enfatizando a sua importância clínica e a necessidade do diagnóstico precoce.

Malignant lymphoma without lymphadenopathy.

Hepatosplenic T-Cell lymphoma (HSTCL) is a rare form of extra-nodal post-thymic T-cell non-Hodgkin's lymphoma that primarily involves liver and spleen with B symptoms, with a characteristic absence of lymphadenopathy. We report such an entity in a 65-year-old man who was diagnosed to have multiple myeloma and treated for the same for two years. A clinical diagnosis of secondary myelofibrosis was suspected and was investigated, when he developed pancytopenia and massive hepatosplenomegaly at one of his follow-up visits. The patient underwent therapeutic splenectomy with a simultaneous wedge biopsy of the liver and with their corresponding histopathological and immunohistochemical features, the diagnosis of HSTCL was clinched.

Nasal NK/T cell lymphoma presenting as a lethal midline granuloma.

Nasal NK/T cell lymphomas are aggressive, locally destructive, midfacial, necrotizing lesions. The nonspecific clinical symptoms constitute a major stumbling block in the early diagnosis and management of these lymphomas. We report here a case of probable nasal NK/T cell lymphoma in an apparently healthy male that progressed rapidly in a short span of time and was managed subsequently with chemotherapy and external beam irradiation with which the lesion regressed.

Aspectos da tomografia computadorizada no linfoma em pacientes abaixo de 20 anos de idade / Computed tomography findings in patients less than 20 years old with lymphoma

OBJETIVO: Descrever os achados gerais do linfoma em pacientes abaixo de 20 anos de idade e por subtipo histológico. MATERIAIS E MÉTODOS: Estudo retrospectivo do arquivo digital de tomografia computadorizada do Centro de Controle do Câncer do Hospital Universitário Pedro Ernesto da Universidade do Estado do Rio de Janeiro, no período de março de 2003 a julho de 2005. Dos 22 casos - 16 do sexo masculino e 6 do sexo feminino, com média de idade de 11,5 anos -, 12 eram do subtipo Hodgkin e 10 eram não-Hodgkin. RESULTADOS: Dos achados gerais, verificamos as linfonodomegalias mediastinais como o mais freqüente (59 por cento), com predomínio no grupo Hodgkin (75 por cento), seguido por hepatoesplenomegalia (50 por cento) e linfonodomegalias cervicais e retroperitoneais (27,3 por cento). No subtipo Hodgkin houve predomínio do acometimento linfonodal, em sucessivas cadeias, seguido pela hepatoesplenomegalia (50 por cento). Verificamos um caso de massa tonsilar unilateral, opacidade pulmonar em "vidro-fosco" e nódulos renais. No subtipo não-Hodgkin houve predomínio extranodal caracterizado por hepatoesplenomegalia (50 por cento), espessamento de alça intestinal (40 por cento), derrame pleural (30 por cento), nódulo pulmonar (20 por cento), ascite (10 por cento), derrame pericárdico (10 por cento) e lesões ósseas mistas (10 por cento). CONCLUSÃO: A tomografia computadorizada é de grande valia no diagnóstico, estadiamento e seguimento do linfoma, com achados de alerta como massa linfonodal, notadamente mediastinal, hepatoesplenomegalia, massa unilateral na tonsila e espessamento parietal de alça intestinal.

OBJECTIVE: To describe the general findings of lymphoma and their histological patterns in patients less than 20 years old. MATERIALS AND METHODS: Twenty-two cases (16 male and 6 female, mean age 11.5 years) from the digital archive of computed tomography at the Cancer Control Center of "Hospital Universitário Pedro Ernesto - Universidade do Estado do Rio de Janeiro", Rio de Janeiro, RJ, Brazil, were retrospectively analyzed in the period between March 2003 and July 2005. Of these 22 cases, 12 were Hodgkin's and 10 were non-Hodgkin's. RESULTS: Overall, mediastinal lymphadenomegaly was the most frequent finding (59 percent), with predominance in the Hodgkin's subgroup (75 percent), followed by hepatosplenomegaly (50 percent) and cervical and retroperitoneal lymphadenomegaly (27.3 percent). The Hodgkin's subgroup presented a prevalence of lymphadenopathy, in many lymph node chains, followed by hepatosplenomegaly (50 percent). One case was found with unilateral tonsillar mass, pulmonary ground-glass opacities, and renal nodules. In the non-Hodgkin's subgroup, the disease was predominantly extranodal, characterized by hepatosplenomegaly (50 percent), thickening of the intestinal wall (40 percent), pleural effusion (30 percent), pulmonary nodule (20 percent), ascites (10 percent), pericardial effusion (10 percent) and mixed bone lesions (10 percent). CONCLUSION: Computed tomography is an extremely useful method for detection, staging and follow-up of lymphomas, with alert findings like mediastinal lymphadenopathy, hepatosplenomegaly, unilateral tonsillar mass and thickening of intestinal wall.

NK/T-cell lymphoma in AIDS.

A 42-year-old man diagnosed to be HIV positive and on highly active antiretroviral treatment (HAART), presented with double vision and gradual drooping of the left eyelid. He had left 3rd cranial nerve palsy and partial right lower-motor-neuron facial palsy. CT of the PNS revealed soft tissue filling the right maxillary sinus antruin. Further workup showed the mass to be an NK/T cell lymphoma.

Nasal extranodal NK/T-cell lymphoma presenting as a perforating palatal ulcer: a diagnostic challenge.

A 40-year-old man presented with chronic nasal stuffiness and bloodstained discharge of 3 years' duration, along with a non-healing palatal ulcer since 2 months. Examination revealed a perforation in the midline on the hard palate and a superficial ulcer on the soft palate. Histopathology and immunohistochemistry suggested a diagnosis of extranodal nasal/nasal-type T-cell lymphoma. The patient was started on multiagent chemotherapy in the form of cyclophosphamide, doxorubicin, vincristine and prednisolone but succumbed after two cycles. Only one case of nasal T cell lymphoma presenting as nasal septal perforation, oronasal fistula and a concomitant palatal ulcer has been described. We report this case of a perforating palatal ulcer as a rare presentation of nasal lymphoma.

Panniculitis-like T-cell lymphoma with fatal termination.

A 58-year-old male presented with three weeks history of multiple subcutaneous nodules and fever. Histopathological feature of the nodule was characteristic of panniculitis. Three months later, the patient presented with carvical lymphadenopathy and compressive thoracic myelopathy and a diagnosis of diffuse mixed- cell lymphoma was established. Immunohistochemical study of subcutaneous lesions confirmed their T-cell origin. Chemotherapy was started but patient succumbed to his disease.

Subcutaneous panniculitis-like T cell lymphoma in a HIV positive patient.

Immunocompromised patients are at increased risk for developing certain malignant tumors, particularly aggressive B cell lymphomas and extranodal lymphomas like primary central nervous system lymphoma and primary effusion lymphoma. T cell lymphomas are uncommon in these patients. We report a rare case of subcutaneous panniculitis-like T cell lymphoma in a HIV positive patient who presented with multiple subcutaneous nodules.