Case for diagnosis. Primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorder

Abstract: Primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorder is a rare disease, with an indolent evolution and benign course. The classic presentation is a solitary nodule on the face or trunk. The disorder's rarity and clinical and histopathological characteristics, can make the diagnosis difficult. We present the case of a 36-year-old Caucasian woman with a purplish erythematous nodule, hardened, shiny, asymptomatic, on the left nasal ala, which had grown progressively for 45 days. Histopathological examination and immunohistochemistry panel demonstrated alterations consistent with primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorder. There was complete remission of the condition within 60 days of treatment with potent occlusive corticosteroids.

Biological therapy and development of neoplastic disease in patients with juvenile rheumatic disease: a systematic review / Uso de imunobiológicos e desenvolvimento de doenças neoplásicas em pacientes com doenças reumáticas juvenis: revisão sistemática

Abstract Juvenile rheumatic diseases affect the musculoskeletal system and begin before the age of 18. These conditions have varied, identifiable or unknown etiologies, but those of an autoimmune inflammatory nature have been associated with an increased risk of development of cancer, regardless of treatment. This study aims to assess, through a systematic review of the literature according to Prisma (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) quality criteria, the risk of cancer in patients with juvenile rheumatic disease, and its association with biological agents. The criteria described by the Strengthening the Reporting of Observational Studies in Epidemiology initiative were used in order to assess the methodological quality of those individual items selected in this study. We analyzed nine publications, from a total of 251 papers initially selected. There was an increase in cancer risk in the population with juvenile rheumatic disease versus the general population. Most specified cancers were of a lymphoproliferative nature. Seven studies did not specify the treatment or not defined an association between treatment and cancer risk. Only one study has suggested this association; in it, their authors observed high risk in patients diagnosed in the last 20 years, a period of the advent of new therapies. One study found an increased risk in a population not treated with biological agents, suggesting a disease in its natural course, and not an adverse effect of therapy. Studies have shown an increased risk of malignancy associated with juvenile rheumatic disease, and this may be related to disease activity and not specifically to the treatment with biological agents.

Resumo As doenças reumáticas juvenis afetam o sistema musculoesquelético e se iniciam antes dos 18 anos. Apresentam etiologia variada, identificável ou desconhecida, porém as de natureza inflamatória autoimune têm sido associadas ao maior risco de desenvolvimento de neoplasias, independentemente do tratamento. Este artigo propõe avaliar, por meio de revisão sistemática da literatura de acordo com os critérios de qualidade Prisma (Preferred Reporting Items for Systematic Reviews and Meta- Analyses), o risco de câncer em pacientes com doenças reumáticas juvenis e sua associação com imunobiológicos. Os critérios descritos pela iniciativa Strengthening the Reporting of Observational Studies in Epidemiology foram usados para avaliar a qualidade metodológica individual dos artigos selecionados no presente estudo. Foram analisadas nove publicações, de 251 incialmente selecionadas. Houve aumento no risco de câncer na população com doença reumática juvenil comparada com a população em geral. A maioria dos cânceres especificados foi de natureza linfoproliferativa. Sete estudos não especificaram a terapêutica ou não definiram associação entre ela e o risco de câncer. Apenas um estudo sugeriu essa associação e observou maior risco em pacientes diagnosticados nos últimos 20 anos, período de advento de novas terapias. Um estudo constatou maior risco em uma população não tratada com imunobiológicos, sugeriu tratar-se da evolução natural da doença, e não do efeito adverso da terapêutica. Os estudos demonstram aumento no risco de malignidade associada a doenças reumáticas juvenis que pode estar relacionada à atividade da doença, e não especificamente ao tratamento com imunobiológicos.

Lymphocytoma cutis on the inguinal region: report of a rare case of benign lymphoproliferative disorder

Abstract Lymphocytoma cutis, or benign reactive lymphoid hyperplasia, is an inflammatory skin lesion that mimics clinically and histologically malignant lymphoma. Most cases are idiopathic, but they may also be triggered by multiple factors, such as insect bites, tattoos, injections and herpes zoster. Clinically, the lesions are erythematous, soft papules, plaques or nodules, usually located on the upper limbs and face. The diagnosis is mainly based on histopathology and immunohistochemistry. Corticosteroid injections, cryosurgery, PUVA therapy, radiotherapy and surgery can be therapeutic options in cases requiring immediate treatment. To demonstrate an atypical presentation of this tumor, a case lymphocytoma skin on the groin will be reported, describing its diagnosis and treatment.

Clonality assessment of lymphoproliferative lesions using the polymerase chain reaction: An analysis of two methods.

Background: Lymphoid malignancies are a heterogeneous group of disorders which may be difficult to differentiate from reactive proliferations even after immunohistochemistry. Polymerase chain reaction (PCR) is believed to be a good adjunct tool for diagnosis. Materials and Methods: We examined 24 cases of neoplastic and non-neoplastic lymphoproliferative lesions in this study and evaluated the PCR as an additional tool in the confirmation of the diagnosis. Two different PCR methodologies were evaluated. Results: In the evaluation of the T-cell PCR, it was seen that the correlation using both the commercial kits and the custom-synthesized primers was highly significant at a P value of <0.05. In the evaluation of the B-cell PCR, it was seen that the correlation using both the commercial kits and the custom-synthesized primers was not significant using either method (P > 0.05). Conclusions: Both the methods showed an excellent concordance for T-cell γ gene rearrangements, However, the same was not seen in the B-cell receptor rearrangements. This may be because of the small sample size or the inability of consensus V primers to recognize complementary DNA sequences in all of the V segments.

Papulosis linfomatoide / Lymphomatoid papulosis

La papulosis linfomatoide (PL) es considerada en la actualidad una forma indolente de linfoma cutáneo CD30+. Su presentación es más frecuente entre la 4º y 5º décadas de la vida, con un discreto predominio en el sexo masculino (1,5/1). Su mecanismo etiopatogénico es complejo y se ha vinculado principalmente con factores genéticos e inmunitarios. Aunque exhibe características clínicas de benignidad, se manifiesta histológicamente con rasgos de malignidad. Las técnicas inmunohistoquímicas resultan de utilidad a los fines diagnósticos y recientemente han permitido la identificación de un nuevo tipo de PL que remeda un linfoma cutáneo primario agresivo a células T epidermotrópico CD8+ (propuesto como PL tipo D). Puede hallarse asociada a otros trastornos linfoproliferativos y a entidades inflamatorias con perfil de citocinas Th2, entre otros. El diagnóstico diferencial con otros linfomas cutáneos, y especialmente con los casos que presentan el antígeno CD30, puede a veces resultar muy dificultoso. Si bien la PL tiene un curso clínico benigno, quienes la padecen tienen un mayor riesgo de desarrollar una segunda neoplasia. Las opciones terapéuticas disponibles son múltiples; sin embargo, ninguna de ellas ha resultado hasta ahora completamente eficaz.

Posttransplantation lymphoproliferative disorder involving liver after renal transplantation / 대한간학회지

No abstract available.

Dynamic Contrast Enhanced Magnetic Resonance Imaging of Diffuse Spinal Bone Marrow Infiltration in Patients with Hematological Malignancies

OBJECTIVE: To investigate the significance of the dynamic contrast enhanced magnetic resonance imaging (DCE-MRI) parameters of diffuse spinal bone marrow infiltration in patients with hematological malignancies. MATERIALS AND METHODS: Dynamic gadolinium-enhanced MR imaging of the lumbar spine was performed in 26 patients with histologically proven diffuse bone marrow infiltration, including multiple myeloma (n = 6), acute lymphoblastic leukemia (n = 6), acute myeloid leukemia (n = 5), chronic myeloid leukemia (n = 7), and non-Hodgkin lymphoma (n = 2). Twenty subjects whose spinal MRI was normal, made up the control group. Peak enhancement percentage (Emax), enhancement slope (ES), and time to peak (TTP) were determined from a time-intensity curve (TIC) of lumbar vertebral bone marrow. A comparison between baseline and follow-up MR images and its histological correlation were evaluated in 10 patients. The infiltration grade of hematopoietic marrow with plasma cells was evaluated by a histological assessment of bone marrow. RESULTS: Differences in Emax, ES, and TTP values between the control group and the patients with diffuse bone marrow infiltration were significant (t = -11.51, -9.81 and 3.91, respectively, p 0.05). A positive correlation was found between Emax, ES values and the histological grade of bone marrow infiltration (r = 0.86 and 0.84 respectively, p < 0.01). A negative correlation was found between the TTP values and bone marrow infiltration histological grade (r = -0.54, p < 0.01). A decrease in the Emax and ES values was observed with increased TTP values after treatment in all of the 10 patients who responded to treatment (t = -7.92, -4.55, and 5.12, respectively, p < 0.01). CONCLUSION: DCE-MRI of spine can be a useful tool in detecting diffuse marrow infiltration of hematological malignancies, while its parameters including Emax, ES, and TTP can reflect the malignancies' histological grade.

Trastorno linfoproliferativo post trasplante renal en un niño / Lymphoproliferative disorders after renal transplantation in a child

Post transplant lymphoproliferative disorder (PTLD) is the commonest form of post transplant malignancy in children. The incidence in renal transplant recipients varies between 2 percent-4 percent. They are characterized by uncontrolled B lymphocyte proliferation, in most cases driven by Epstein Barr virus (EBV). They are more common in younger children, EBV seronegative patients and those who receive aggressive immunosuppression. PTLD commonly presents in an unspecific form and it requires high suspicion rate for its diagnosis, especially in children with risk factors. We report a twelve year-old girl who developed fever, sore throat and lymph node enlargement, six months after receiving a renal allograft. Laboratory assessment and imaging studies were compatible with PTLD, which was confirmed by biopsy. Treatment was reduction of immunosuppression and surveillance. The patient had a favorable evolution.

Hairy cell leukaemia in Oman four cases

Hairy cell leukaemia [HCL] is a rare, clonal, chronic lymphoproliferative disorder commonly seen in males in the middle years of life. Pancytopaenia with moderate to massive splenomegaly is the most common clinical presentation. Diagnosis is made on detecting the lymphocytes with abundant cytoplasm which spread into hair-like processes on peripheral blood and bone marrow smears, thus giving the name, 'hairy cell leukaemia'. The bone marrow aspirate is frequently a dry tap. The trephine biopsy has the characteristic features of a honey comb appearance and flow cytometry is typically CD[103], CD[25], FMC[7], CD[11c], gamma or kappa light chain positive with the classic B lymphocyte markers CD[19], CD[20], CD7[9a]. Purine analogues followed by granulocyte-colony stimulating factor [G-CSF] to manage the febrile neutropenia is currently the treatment of choice. A 10 year disease free survival is recorded with these management strategies. Experimental use of anti CD[20] and CD[22] has also shown promising results in the treatment of this disease. We report four cases of HCL diagnosed in a span of two years at the Royal Hospital, Muscat, Oman

Desordem linfoproliferativa pós-transplante em paciente pediátrico / Post-transplantation lymphoproliferative disorder in pediatric patient / Post-transplantation lymphoproliferative disorder in pediatric patient

Terapias de imunossupressão, a que pacientes transplantados devem ser submetidos, os expõe a um alto risco de desenvolver desordens linfoproliferativas pós-transplante (PTLD). Descrevemos o caso de uma criança submetida a transplante cardíaco aos sete meses de idade e que acabou desenvolvendo PTLD, aos nove anos, diagnosticada por meio de retirada de nódulo pulmonar.

Immunosuppressive therapy for transplanted patients exposes them to a high risk of developing posttransplantation lymphoproliferative disorders (PTLD). We report the case of a child undergoing heart transplantation at seven months of age who developed PTLD at nine years of age, diagnosed by resection of a pulmonary nodule.

Enfermedades linfoproliferativas CD30 (+) de la piel / CD30-positive lymphoproliferative disorders of the skin

Las enfermedades linfoproliferativas CD30 (+) de la piel corresponden al grupo más común dentro de los linfomas cutáneos primarios de células T, después de la micosis fungoides. Este grupo incluye: papulosis linfomatoide, linfoma cutáneo primario de células grandes T CD30(+) (anaplástico), linfoma de células T CD30(+) con compromiso nodal regional y el linfoma cutáneo de células T CD30(+) secundario. Entre estas condiciones existe una sobreposición clínica, histológica e inmunofenotípica. La mayoría de los pacientes con esta condición tienen excelente pronóstico. Este artículo revisa los conocimientos actuales sobre estas condiciones con énfasis en diagnóstico y tratamiento.

Lymphoproliferative responses of splenocytes before and after challenge with schistosoma haematobium in C57BL/6 mice vaccinated with human anti-idiotypes

Anti-idiotypic vaccines [anti-Id or antibody 2; Ab2] in experimental schistosomiasis engender varying degrees of resistance to challenge infection. To further characterize the mechanisms involved in the induction of protective immunity associated with such a vaccine model, spleen cells of mice vaccinated with human Ab2 [HAb2] were investigated for their lymphoproliferative responses before and after challenge infection with normal S. haematobium cercariae. HAb2 was purified from sera of chronically infected patients using protective rabbit antibodies [RAb1] isolated from sera of rabbits multiply immunized with UV-irradiated cercariae by affinity chromatography over soluble worm antigenic preparation [SWAP]. Vaccination of C57BL/6 [C57] mice with HAb2 resulted in - 31% and - 36% protection in two experiments of resistance to infection. Splenocytes were collected prior to challenge at week 6-post initial immunization and after challenge at days 6, 10, 28 and 90. Prior to challenge, in vitro splenic responses of HAb2-vaccinated animals [HAb2-group] to phytohaemagglutinin [PHA] declined while both SWAP and HAb2-driven responses increased, all compared to naive control. After challenge, PHA responses increased in the two test groups on day 6 then significantly decreased to lower levels. On the other hand, SWAP- and HAb2-driven responses of HAb2 group increased by day 6 then declined while the same responses in infected control mice increased on days 10 through 28 and decreased by day 90. Generally, proliferation obtained following in vitro stimulation with HAb2 was greater than that with SWAP in the HAb2-group after challenge. These results suggested that human anti-Id antibodies could mimic at the T cell level the properties of a protective antigenic epitopes of the irradiated-cercariae vaccine

Biopsia por Aspiración con Aguja fina en el Síndrome Adénico Cervical Crónico / Fine needle aspiration biopsy in the chronic adenic cervical syndrome

Con el objetivo de determinar en nuestro medio el valor de la citología aspirativa con aguja fina, al evaluar una linfadenopatía cervical asintomática, como procedimiento diagnóstico preliminar, se realizó un estudio de 40 pacientes que acudieron a la consulta de otorrinolaringología del Hospital Clinicoquirúrgico Docente "Joaquín Albarrán", durante el año 1994, en los cuales se practicó primero biopsia aspirativa con aguja fina y posteriormente la extirpación quirúrica de los ganglios afectados para evaluar los diagnósticos citopatológicos realizados. El 72,5 por ciento de los pacientes tenían más de 50 años y el 62,5 por ciento era del sexo masculino. Se diagnosticaron lesiones metastásicas en el 45 por ciento, tumores primarios en el 20 por ciento, adenitis aguda en 7,5 por ciento. En la correlación se encontraron 2 falsos positivos (5 por ciento) e igual número de falsos negativos. La eficacia del método fue de 90 por ciento, sensibilidad de 92,3 por ciento y especialidad de 85,75 por ciento. Los índices predictivos positivos y negativos fueron 92,3 y 85,7 por ciento

Lethal midline granuloma and lymphoproliferative disorders.

Seventeen patients, who presented with unhealing ulcers or destructive lesions of the upper aero-digestive tract at Ramathibodi hospital from 1977 to 1985 were reported. Lesions caused by infection, Wegener's granulomatosis or non-hematopoietic malignancy were excluded. A spectrum of histopathologic findings were evident in our patients, ranging from acute and chronic inflammatory changes with or without necrosis, polymorphic reticulosis or lymphamatoid granulomatosis, and malignant lymphoma of the non-Hodgkin's type (NHL). Although some initial histopathologic findings were non-specific, evidence of lymphoproliferative disorders finally emerged. These malignant lymphoid cells had a predilection for the GI tract and skin. Lymphoma staging should thus be done. Bleeding from the lesion, treatment-induced leucopenia, and sepsis were common in these patients. Early aggressive treatment including adequate antibiotic coverage for superimposed infection, improved nutritional status, and early radiation to the primary lesion are suggested for those diseases.

Estudo das leucemias mieloides agudas e síndromes linfoproliferativas à microscopia eletrônica / Acute myeloid leukemias and lymphoproliferative syndromes by electron microscopy

Foram estudados o sangue periférico e ou a medula óssea de 24 portadores de hemopatias malignas por meio da microscopia eletrônica, sendo 12 casos de leucemia mielóide aguda e 12 casos de síndromes linfoproliferativas. Nos 15 casos de leucemias agudas fizemos uma correlaçäo com a classificaçäo FAB (Frech-American British Group), 1978, baseada na morfologia e citoquímica na microscopia de luz, com o estudo morfológico e a reaçäo citoquímica da mieloperoxidase, através do microscópio eletrónico. Assim, dois casos de LMA, que apresentavam dúvidas no microscópio de luz, com o estudo ao microscópio eletrônico confirmamos os tipos morfológicos: um caso de LMA que apresentava dúvidas do ML, com o estudo ao ME foi reclassificado. Todos os outros casos de leucemias agudas e todos de síndromes linfoproliferativas foram confirmados à microscopia eletrônica, corroborando no diagnóstico final. Os nossos resultados, de um modo geral, foram concordantes com a literatura