Case report of adult T-cell lymphoma/leukemia / Relato de caso de linfoma/leucemia de células T do adulto

The adult T-cell leukemia/lymphoma (ATLL) is a rare type of lymphoma caused by human T lymphotropic virus type 1 (HTLV-1). The clinical manifestations include cutaneous lesions, adenopathies, myelopathy/ tropical spastic paraparesis, uveitis, ophthalmological diseases, leukocytosis with lymphocytosis and atypical lymphocytes. The main objective of this study was to report a case of a female patient with ATLL, a farmer with leukocytosis, lymphocytosis, bilateral ocular erythema, cervical lymphadenopathy, in the abdominal visceromegalies and with positive markers for T-cell lymphocytes (CD45, CD2, CD3, CD5, CD4 and CD25). Although ATLL is a rare disease, its delayed diagnosis may lead to serious complications and fatal outcome.

O linfoma/leucemia de células T do adulto (ATLL) é um tipo raro de linfoma causado pelo vírus T-linfotrópico humano tipo I (HTLV-1). O quadro clínico inclui lesões de pele, adenomegalias, mielopatia/paraparesia espástica tropical, uveíte, doença oftalmológica, leucocitose com linfocitose e linfócitos atípicos. O objetivo deste estudo foi relatar o caso de uma paciente com ATLL, agricultora, com leucocitose, linfocitose, eritema ocular bilateral, linfadenopatia cervical, sem visceromegalias abdominais e com marcadores positivos para linfócitos T (CD45, CD2, CD3, CD5, CD4 e CD25). Embora a ATLL seja uma doença rara, a demora no seu diagnóstico pode levar a sérias complicações e ocasionar a morte do paciente.

Estudo da influência dos alelos do HLA classe I e II e do polimorfismo dos genes de citocinas na infecção pelo HTLV-1 / Study of the influence of hla class i and ii alleles and the polymorphism of cytokines genes in htlv-1 infection

O vírus linfotrópico para células T humanas (HTLV-1) é o principal agente causador da Paraparesia Espástica Tropical / Mielopatia associada ao HTLV-1 (PET/MAH) e da Leucemia da célula T do Adulto (LTA). [...]Fatores da interação HTLV-1/ hospedeiro estão envolvidos no risco de desenvolver doença. A lesão neurológica na PET/MAH parece ser consequência de uma reação inflamatória, desencadeada pelo reconhecimento de células infectadas por linfócitos T citotóxicos, com consequente liberação de citocinas e lesão medular. OBJETIVO: Identificar marcadores genéticos, que possam ajudar no prognóstico e tratamento dos pacientes portadores do HTLV-1. MÉTODOS: Nas amostras de 117 portadores do HTLV-1 assintomáticos e 171 pacientes com acometimento neurológico em acompanhamento na cidade do Rio de Janeiro, foram realizadas as tipificações dos genes do HLA Classe I e II, dos polimorfismos dos genes das citocinas -308TNF-α,-174IL-6, +874IFN-γ, códon 10 e 25TGF-β1 e -1082 - 819-592IL-10, e a quantificação da carga proviral. Os dados foram organizados em um banco de dados no programa SPSS. As frequências alélicas e genotípicas foram obtidas por contagem direta. O equilíbrio de Hardy-Weinberg foi avaliado para os polimorfismos das citocinas no sitio http://bioinfo.iconcologia.net/ubbweb/SNPStats_web, em relação ao HLA foram utilizadas as ferramentas disponíveis no sítio “Los Alamos HIV database tools”. As comparações entre os grupos foram realizadas através de tabelas de contingência 2x2 (quiquadrado, exato de Fisher e odds ratios), valores de p≤0,05 foram considerados significantes...

The human T cell lymphotropic vírus (HTLV-1) is the main causingagent of Tropical Spastic Paraparesis/HTLV-1 Associated Myelopathy (HAM/TSP) aswell as of Adult T Cell Leukemia (ATL). [...] Factorsrelated to the HTLV-1/host interaction may be involved in the risk of developing thediseases. The neurological lesion in HAM/TSP may be the consequence of aninflammatory reaction, triggered by the recognition of infected cells by cytotoxic Tlymphocytes, followed by the release of cytokines and central nervous system lesion.OBJECTIVE: This work aims to identify genetic markers, which may help in theprognosis and treatment of HTLV-1 patients. Methods: The polymorphism of the HLAClass I and II genes, as well as the TNF-α, IL6, IFN-γ, TGF-β and IL-10 cytokinegenes, and the proviral load were analysed in 117 asymptomatic HTLV-1 carriersand 171 HTLV-1 symptomatic carriers from Rio de Janeiro city. Data were organizedinto a database using SPSS. The Hardy-Weinberg equilibrium was evaluated forcytokine polimorphisms using the site http://bioinfo.iconcologia.net/ubbweb/SNPStats_web. The tools available in the site “Los Alamos HIV database tools” were used toanalyze the HLA polimorphisms. Comparisons between groups were made using 2x2contingency tables (Fisher Exact test/ χ2 and odds ratios), p values p≤0,05 wereconsidered significant...

Isolated relapse in the oral cavity of a child with t-lineage acute lymphoblastic leukemia

Despite high cure rates, approximately 20% of patients with acute lymphoblastic leukemia (ALL) have disease relapse. Isolated recurrence in oral cavity is extremely unusual. The aim of this paper is to report a case of an isolated relapse occurred in a child with T-lineage ALL. Clinical picture included swelling and pain in the right upper gingiva of the oral cavity, with no other clinical or hematological alterations. Diagnosis was confirmed by biopsy and immunohistochemical staining. Bone marrow aspiration was normal. Five months later leukemic infiltration of the bone marrow was detected and systemic chemotherapy was reintroduced. This case report highlights the relevance of dental care during and after chemotherapy, not only to treat lesions in the oral cavity resulting from the disease itself or from treatment side effects, but also to detect unusual sites of ALL relapse.

Apesar dos altos índices de cura, cerca de 20% dos pacientes com leucemia linfóide aguda (LLA) apresentam recidiva da doença. Recidiva isolada na cavidade oral é extremamente incomum. O objetivo deste trabalho é relatar um caso de recidiva isolada em criança com LLA de linhagem T. A apresentação clínica foi quadro de edema e dor na cavidade oral, na região superior da gengiva à direita, sem outras alterações clínicas ou hematológicas. O diagnóstico foi confirmado por meio de biópsia e imuno-histoquímica. O mielograma era normal. Cinco meses após a manifestação inicial na cavidade oral, foi detectada infiltração leucêmica na medula óssea. O tratamento com quimioterapia sistêmica foi reintroduzido. Este relato de caso ressalta a importância do acompanhamento clínico e odontológico durante e após o tratamento quimioterápico, não somente com o objetivo de tratar as alterações na cavidade oral decorrentes da própria doença ou dos efeitos adversos do tratamento, mas para que sejam detectadas apresentações incomuns de recidiva na LLA.

Immunophenotypic patterns in precursor T-cell neoplasm.

T-cell lymphoproliferative disorders are a heterogeneous group of lymphoid neoplasm that can mimic both benign conditions and non-hematopoietic tumors. In routine clinical practice, morphology and immunophenotyping forms the basis of their diagnosis. In this retrospective analysis, we evaluate the utility of flow cytometric immunophenotyping patterns in diagnosis of precursor T-cell neoplasm. Aberrant expression of T-cell antigens was found in all the cases of precursor T-cell neoplasm. The residual normal T-lymphocytes, identifiable in majority of cases, were found to be useful in evaluation of quantitative differences in antigen expression by leukemic cells. A careful analysis of flow cytometric immunophenotyping data can provide additional information which is useful for diagnosis of precursor T-cell neoplasm. This information can be further utilized for analysis of minimal residual disease in these tumors.

[Specific cutaneous manifestations in adult Tcell leukemia/lymphoma]

Adult T cell leukemia/lymphoma [ATLL] is an aggresive malignancy which may occur in human T lymphotropic virus [HTLVl] infected persons. HTLV1 is endemic in Khorasan with prevalence of 2.3% in general population. Since specific cutaneous manifestations of lymphoma may occur in a significant number of patients, we studied ATLL patients in Mashhad In this descriptive study the demographic and clinical data of 23 patient suffering from ATLL who were admitted in hematology and dermatology departments of Ghaem Hospital, during 1995-2004 and had specific cutaneous lesions confirmed histopathologically with atypical lymphocytes in skin lesions, were recorded and analyzed. Eleven patients were male and 12 were female. The mean age of patients was 48.17 +/- 14.1 years. Birth places of over 85% of cases were north of Khorasan. The most common types of specific skin lesions were maculopapular rash in 11 cases [47.8%] and papular lesions in 4 cases [17.4%]. Other lesions included plaque, icthyosis like, erythroderma, tumor, and nodular lesions. In most of patients [56.5%] the skin lesions were generalized The most common type of specific skin lesions in ATLL was maculopapular rash especially with generalized distribution. Other types of specific skin lesions in order of frequency were papule, plaque, icthyosis like, nodule, tumor and erythroderma

Sarna noruega en un paciente con linfoma/leucemia de celulas T del adulto / Norwegian escabies in a patient with lymphoma/adult T cells leukemia

Se informa un caso de sarna noruega asociado con moniliasis oral, tuberculosis pulmonar y ganglionar en un paciente que sufria linfoma/leucemia de celulas T del adulto y cuyo diagnostico se establecio con criterios clinicos e histopatologicos