T-cell lymphoma with rhabdomyolysis: case report and literature review.

Rhabdomyolysis refers to the destruction or disintegration of striated muscles. This syndrome is characterized by muscle breakdown and necrosis, resulting in the leakage of intracellular muscle constituents into the circulation and extracellular fluid. We report a rare case of rhabdomyolysis complicating multi-organ failure caused by T-cell lymphoma in a 32-year-old woman. The final diagnosis was rhabdomyolysis caused by peripheral T-cell lymphoma based on bone marrow aspirate and biopsy.

Collapsing glomerulonephritis with podocyte markers in hemophagocytic syndrome secondary to hepatosplenic T-cell lymphoma / [Glomerulonefritis colapsante con marcadores de desdiferenciación podocitaria en síndrome hemofagocítico secundario a linfoma hepatoesplénico de células T].

The hemophagocytic syndrome is a serious clinical-histological entity secondary to different diseases. Collapsing glomerulonephritis is a proliferative podocytopathy that usually has an unfavorable renal prognosis. We present a case in which both entities were associated, which is an infrequent form of hepatosplenic T-cell lymphoma. In addition, we review the role of the markers of podocyte dedifferentiation in this glomerulopathy and its pathophysiology and treatment.

El síndrome hemofagocítico es una condición clínica e histológica grave, secundaria a diferentes procesos. La glomerulonefritis colapsante es una podocitopatía proliferativa, generalmente de pronóstico desfavorable para la función renal. Se presenta un caso en el que las dos condiciones aparecieron asociadas, lo cual es una forma infrecuente de presentación del linfoma hepatoesplénico de células T. Se discute, asimismo, el papel de los marcadores de desdiferenciación podocitaria en esta glomerulopatía, y se revisan la fisiopatología y el tratamiento.

T-cell lymphoma with rhabdomyolysis: case report and literature review

Rhabdomyolysis refers to the destruction or disintegration of striated muscles. This syndrome is characterized by muscle breakdown and necrosis, resulting in the leakage of intracellular muscle constituents into the circulation and extracellular fluid. We report a rare case of rhabdomyolysis complicating multi-organ failure caused by T-cell lymphoma in a 32-year-old woman. The final diagnosis was rhabdomyolysis caused by peripheral T-cell lymphoma based on bone marrow aspirate and biopsy.

[Paniculitis as manifestation of prolonged febrile syndrome: Case report]. / Paniculitis como manifestación de síndrome febril prolongado en pediatría: Caso clínico.

Fever of unknown origin (FUO) is defined as fever over 7 to 10 days without a diagnosis despite a complete initial study. The most frequent causes are infections, autoimmune and tumors. Even though most cases are self-limited there is a minority that has an underlying etiology with an ominous forecast, encouraging a systematized study. OBJECTIVE: To report a rare case of a boy who presented fever of unknown origin associated to panniculitis and was diagnosed of subcutaneous panniculitis-like-T cell lymphoma and to emphasis the importance of a sequential study of FUO, in order to reach a diagnosis in patients who need a timely intervention. CLINICAL CASE: A ten year old boy, previously healthy, presented subcutaneous nodular lesions of 2 month of evolution, located in abdominal region and extremities, given few symptoms, associated with prolonged fever. He was hospitalized for proper study, in first instance infectious and immune causes were discarded and through lesions biopsy the diagnose of subcutaneous panniculitis-like-T cell lymphoma was reached. CONCLUSION: When FUO is diagnosed, most prevalent causes must be discarded. Then, differential diagnosis, such as immune and neoplasic etiologies, have to be considered. If FUO is associated to elemental nodular lesions, biopsy must be indicated early, in order to find potential malignant cases, avoiding therapeutic delay.

The thyroid status reprograms T cell lymphoma growth and modulates immune cell frequencies.

UNLABELLED: In spite of considerable evidence on the regulation of immunity by thyroid hormones, the impact of the thyroid status in tumor immunity is poorly understood. Here, we evaluated the antitumor immune responses evoked in mice with different thyroid status (euthyroid, hyperthyroid, and hypothyroid) that developed solid tumors or metastases after inoculation of syngeneic T lymphoma cells. Hyperthyroid mice showed increased tumor growth along with increased expression of cell cycle regulators compared to hypothyroid and control tumor-bearing mice. However, hypothyroid mice showed a higher frequency of metastases than the other groups. Hyperthyroid mice bearing tumors displayed a lower number of tumor-infiltrating T lymphocytes, lower percentage of functional IFN-γ-producing CD8(+) T cells, and higher percentage of CD19(+) B cells than euthyroid tumor-bearing mice. However, no differences were found in the distribution of lymphocyte subpopulations in tumor-draining lymph nodes (TDLNs) or spleens among different experimental groups. Interestingly, hypothyroid TDLN showed an increased percentage of regulatory T (Treg) cells, while hyperthyroid mice displayed increased number and activity of splenic NK cells, which frequency declined in spleens from hypothyroid mice. Moreover, a decreased number of splenic myeloid-derived suppressor cells (MDSCs) were found in tumor-bearing hyperthyroid mice as compared to hypothyroid or euthyroid mice. Additionally, hyperthyroid mice showed increased cytotoxic activity, which declined in hypothyroid mice. Thus, low levels of intratumoral cytotoxic activity would favor tumor local growth in hyperthyroid mice, while regional and systemic antitumor response may contribute to tumor dissemination in hypothyroid animals. Our results highlight the importance of monitoring the thyroid status in patients with T cell lymphomas. KEY MESSAGES: T cell lymphoma phenotype is paradoxically influenced by thyroid status. Hyperthyroidism favors tumor growth and hypothyroidism rises tumor dissemination. Thyroid status affects the distribution of immune cell types in the tumor milieu. Thyroid status also modifies the nature of local and systemic immune responses.

T-cell lymphoma in the tongue of a dog with cutaneous and striated forelimb muscle involvement / Linfoma de células T em língua de cão com envolvimento cutâneo e de musculatura estriada de membro torácico

Background: Primary tongue tumors rarely affect dogs and correspond to 4% of tumors involving the oropharynx. Until now, primary tongue lymphoma had not been reported. However, lymphoma involvement in the skeletal muscle, although quite unusual, was described in the literature in four cases. Cutaneous lymphoma is another rare extranodal manifestation. The objective of this report is to describe a case of T immunophenotype lymphoma occurrence, whose manifestation is atypical, not only because it is situated in the tongue muscle but also because of the subsequent involvement of the striated musculature of the left forelimb and the skin, which showed unfavorable evolution. Case: A female seven-year-old mongrel was seen showing a regular lump in the base of the tongue, 3 cm in diameter, not ulcerated and of firm consistency, with halitosis as the only clinical sign of the disease. Incisional biopsy of the lump was performed and histopathology verified that it was large cell lymphoma. The material was sent for immunohistochemical evaluation and was characterized as T immunophenotype lymphoma by positive CD3 and negative CD79a marking. The CHOP (cyclophosphamide, doxorubicin, vincristine and prednisone) chemotherapy protocol was established as treatment and after the first chemotherapy session there was partial remission of the mass, measuring 2 cm in diameter. The lump, however...(AU)

T-cell lymphoma in the tongue of a dog with cutaneous and striated forelimb muscle involvement / Linfoma de células T em língua de cão com envolvimento cutâneo e de musculatura estriada de membro torácico

Background: Primary tongue tumors rarely affect dogs and correspond to 4% of tumors involving the oropharynx. Until now, primary tongue lymphoma had not been reported. However, lymphoma involvement in the skeletal muscle, although quite unusual, was described in the literature in four cases. Cutaneous lymphoma is another rare extranodal manifestation. The objective of this report is to describe a case of T immunophenotype lymphoma occurrence, whose manifestation is atypical, not only because it is situated in the tongue muscle but also because of the subsequent involvement of the striated musculature of the left forelimb and the skin, which showed unfavorable evolution. Case: A female seven-year-old mongrel was seen showing a regular lump in the base of the tongue, 3 cm in diameter, not ulcerated and of firm consistency, with halitosis as the only clinical sign of the disease. Incisional biopsy of the lump was performed and histopathology verified that it was large cell lymphoma. The material was sent for immunohistochemical evaluation and was characterized as T immunophenotype lymphoma by positive CD3 and negative CD79a marking. The CHOP (cyclophosphamide, doxorubicin, vincristine and prednisone) chemotherapy protocol was established as treatment and after the first chemotherapy session there was partial remission of the mass, measuring 2 cm in diameter. The lump, however...

[Fatal bacteremia related to Capnocytophaga sputigena in a hematological patient with type T non-Hodgkin lymphoma. Diagnosis by 16S rRNA gene sequencing]. / Bacteriemia fulminante asociada a Capnocytophaga sputigena en un paciente con linfoma no Hodgkin tipo T. Diagnóstico por secuenciación genética del ARNr 16S.

We described a case of fatal bacteremia related to Capnocytophaga sputigena in a hematological patient. The strain was identified by 16S rRNA gene sequencing.

Common variable immunodeficiency associated with hepatosplenic T-cell lymphoma mimicking juvenile systemic lupus erythematosus.

Common variable immunodeficiency (CVID) is a heterogeneous disorder with susceptibility to infections, autoimmune manifestations, and cancer. To our knowledge, CIVD with T-cell lymphoma mimicking juvenile systemic lupus erythematosus (JSLE) was not described in the literature, and one case was reported herein. An 8-year-old female was admitted in our Pediatric Immunology Unit with a clinical history of hypogammaglobulinemia, recurrent upper respiratory infections, and pneumonias. She had a marked decrease of three serum immunoglobulin isotypes, and the diagnosis of CVID was established. At the age of 17 years, she presented with oral ulceration, nonerosive arthritis, nephritis, serositis, cytopenia, positive antiphospholipid antibodies, and positive antinuclear antibody fulfilling the American College of Rheumatology (ACR) criteria for SLE. She was treated with intravenous methylprednisolone for three consecutive days, and intravenous immunoglobulin, and maintenance therapy of chloroquine, azathioprine and prednisone 40 mg/day. Two months later, she died of septic shock secondary to acute pneumonia. The necropsy showed hepatosplenic T-cell lymphoma with diffuse involvement of bone marrow, spleen, liver, and lungs. The lymphoma cells were positive for CD3 immunostaining and negative for CD20 and lysozyme. In conclusion, the association of CVID and hepatosplenic T-cell lymphoma may simulate JSLE diagnosis.

Infective dermatitis.

Infective dermatitis represents a severe form of recurrent eczematous skin change that presents in childhood. It was first described in 1966. Later, the association of infective dermatitis with an underlying human T-cell lymphotropic virus infection was recognized. Chronic infective dermatitis is associated with an increased risk of malignant transformation and may be an early sign of underlying leukemia or T-cell lymphoma, or both. Infective dermatitis is endemic in parts of South America (Peru, Colombia, Brazil), parts of Africa, the Caribbean, and southwestern Japan. Treatment is difficult, and not infrequently cases, are refractory to therapy.

Necrotizing sialometaplasia of the palate associated with angiocentric T-cell lymphoma.

In this article we present 2 cases of necrotizing sialometaplasia (NS) associated with angiocentric lymphoma of the midline. Immunohistochemical analysis confirmed a T-cell origin, and in situ hybridization in one case revealed its relationship to Epstein-Barr virus. These findings suggest that vascular occlusion by the neoplastic cells produces ischemia, which leads to local infarction contributing to the salivary gland lesion. To our knowledge, the association between angiocentric lymphoma and NS has been previously reported in only one instance, and we suggest that this particular type of lymphoma should be added to the list of related conditions for NS.

Nasal-type NK/T-cell lymphoma presenting as hemophagocytic syndrome in an 11-year-old Mexican boy.

Recently established by the World Health Organization classification, "nasal" and "nasal-type" NK/T-cell lymphoma arise from natural killer (NK) cells. They have distinct clinicopathologic features, specific genotype and phenotype, and a high association with Epstein-Barr virus infection. Nasal-type NK/T-cell lymphoma arise from extranasal sites including skin, soft tissue, gastrointestinal tract, liver, spleen, testes, lung, and central nervous system. Most cases are reported from Asia and South America in adults. There are very few cases reported in the pediatric age group. We report an 11-year-old child with extranodal nasal-type NK/T-cell lymphoma presenting as hemophagocytic syndrome and multiorgan system failure with fatal outcome.

Anaplastic T-cell lymphoma presenting as fatal acute liver failure

Acute liver failure (ALF) is an uncommon manifestation of liver disease and constitutes a medical emergency for which early identification is necessary. Hepatic involvement by hematologic malignancies although frequent, rarely causes severe hepatic dysfunction. Even more, acute hepatic failure as the first manifestation of a hematologic malignancy is extremely uncommon, although some cases have been reported in the literature. We describe the case of a 61 y/o puertorrican veteran who developed acute hepatic failure secondary to massive infiltration of the liver by a recurrent non-Hodgkin's lymphoma.

Infection by Rhodotorula sp. in children receiving treatment for malignant diseases.

Rhodotorula sp. are commensal yeasts that may cause opportunistic infections. There have been only a few case reports of Rhodotorula fungemia in children with cancer, and in all of them the patients had a central venous catheter inserted. The authors report three nonfatal cases of fungemia by Rhodotorula in patients with post-chemotherapy neutropenia. Two of three patients required catheter removal, and a response was achieved with systemic antifungal therapy. Aggressive therapy may be required for selected high-risk patients.

Anaplastic T-cell lymphoma presenting as fatal acute liver failure.

Acute liver failure (ALF) is an uncommon manifestation of liver disease and constitutes a medical emergency for which early identification is necessary. Hepatic involvement by hematologic malignancies although frequent, rarely causes severe hepatic dysfunction. Even more, acute hepatic failure as the first manifestation of a hematologic malignancy is extremely uncommon, although some cases have been reported in the literature. We describe the case of a 61 y/o puertorrican veteran who developed acute hepatic failure secondary to massive infiltration of the liver by a recurrent non-Hodgkin's lymphoma.

Linfomas No-Hodgkin de células B y T primarias intestinales e infección por virus de Epstein Barr en la población uruguaya / B-cell and Primary T-cell intestinal no-Hodgkin lymphomas and Epstein Barr virus infection in the Uruguayan population

El propósito del presente trabajo es establecer si existe asociación entre los linfomas No-Hodgkin (LNH), a células B y T, y la infección por el virus de Epstein Barr (VEB) en la población uruguaya. El VEB se encuentra universalmente asociado con linfoma tipo Burkitt, periférico de células T, carcinoma nasofaríngeo indiferenciado y linfomas "Natural Killer", mostrando importante variación geográfica. La Enfermedad de Hodgkin (EH), y los linfomas B esporádicos procedentes de América Latina, han evidenciado mayores tasas de asociación con la infección por VEB que los originados en los países occidentales. En los LNH de células T de origen gastrointestinal, la frecuencia en la positividad para el VEB está influida por el sitio anatómico del tumor primario y el fenotipo de la célula neoplásica, siendo independiente de la presencia o ausencia de enteropatia. Analizamos 14 casos de linfomas a células B y T primarios intestinales en pacientes HIV negativos, diagnosticados por microscopía óptica y e inmunomarcación, evaluando la coexistencia con el VEB con técnicas de inmunohistoquimica (IHQ) con EBV LMP-1. En dos (14,3 por ciento) de los casos se comprobó esta asociación, siendo los restantes negativos para la infección por VEB. Los dos linfomas con evidencia de infección por el VEB fueron: uno, en mujeres y, el otro, en hombres, ambos de fenotipo a células B y ambos del subtipo difuso a grandes células, el subtipo que representó 72 por ciento de los linfomas estudiados.

Linfomas No-Hodgkin de células B y T primarias intestinales e infección por virus de Epstein Barr en la población uruguaya / B-cell and Primary T-cell intestinal no-Hodgkin lymphomas and Epstein Barr virus infection in the Uruguayan population

El propósito del presente trabajo es establecer si existe asociación entre los linfomas No-Hodgkin (LNH), a células B y T, y la infección por el virus de Epstein Barr (VEB) en la población uruguaya. El VEB se encuentra universalmente asociado con linfoma tipo Burkitt, periférico de células T, carcinoma nasofaríngeo indiferenciado y linfomas Natural Killer, mostrando importante variación geográfica. La Enfermedad de Hodgkin (EH), y los linfomas B esporádicos procedentes de América Latina, han evidenciado mayores tasas de asociación con la infección por VEB que los originados en los países occidentales. En los LNH de células T de origen gastrointestinal, la frecuencia en la positividad para el VEB está influida por el sitio anatómico del tumor primario y el fenotipo de la célula neoplásica, siendo independiente de la presencia o ausencia de enteropatia. Analizamos 14 casos de linfomas a células B y T primarios intestinales en pacientes HIV negativos, diagnosticados por microscopía óptica y e inmunomarcación, evaluando la coexistencia con el VEB con técnicas de inmunohistoquimica (IHQ) con EBV LMP-1. En dos (14,3 por ciento) de los casos se comprobó esta asociación, siendo los restantes negativos para la infección por VEB. Los dos linfomas con evidencia de infección por el VEB fueron: uno, en mujeres y, el otro, en hombres, ambos de fenotipo a células B y ambos del subtipo difuso a grandes células, el subtipo que representó 72 por ciento de los linfomas estudiados. (AU)