RESUMEN
BACKGROUND: Sezary syndrome (SS) presents clinically as erythroderma, which may be pigmented, and pruritic, associated with peripheral lymphadenopathies. Erythroderma may also occur in a broad range of reactive and malignant conditions including T-cell prolymphocytic leukemia (T-PLL). We report a case initially diagnosed as SS but ultimately diagnosed as T-PLL based upon skin involvement. CASE REPORT: A 70-year-old man was referred by his hematologist for management of SS. Physical examination revealed lymphadenopathies and mild diffuse erythema without infiltration. His WBC count was elevated at 8.3 G/L. A peripheral blood smear showed Sezary-like cells. Flow cytometry of peripheral blood revealed prolymphocytic T-cells staining positively for CD2, CD3, CD4 and CD7. Cytogenetic studies showed chromosomal abnormalities in terms of number and structure with missing chromosomes 6 and13, as well as deletion of chromosome 17. Finally, a diagnosis of T-PLL was made. Pentostatin was initiated pending treatment with alemtuzumab, but the patient's overall condition deteriorated rapidly and he died 10 days later. DISCUSSION: Diagnosis of LPLT is based upon a number of factors. In the case presented herein, the clinically atypical nature of the skin lesions prompted the dermatologist to review the diagnosis. The morphology of the circulating T-lymphocytes and their immunologic and phenotypic characteristics finally ruled out the diagnosis of Sezary syndrome, while their association with compatible cytogenetic anomalies enabled a diagnosis of prolymphocytic leukemia to be made instead. CONCLUSION: Prolymphocytic leukemia involves complex differential diagnosis with regard to Sezary syndrome, posing potential pitfalls for hematologists and dermatologists.
Asunto(s)
Errores Diagnósticos , Leucemia Prolinfocítica de Células T/diagnóstico , Neoplasias Cutáneas/diagnóstico , Linfocitos T/patología , Adenocarcinoma/terapia , Anciano , Antimetabolitos Antineoplásicos/uso terapéutico , Capecitabina/uso terapéutico , Deleción Cromosómica , Terapia Combinada , Diagnóstico Tardío , Resultado Fatal , Humanos , Inmunofenotipificación , Leucemia Prolinfocítica de Células T/tratamiento farmacológico , Leucemia Prolinfocítica de Células T/genética , Leucemia Prolinfocítica de Células T/patología , Masculino , Neoplasias Primarias Secundarias , Pentostatina/uso terapéutico , Neoplasias del Recto/terapia , Síndrome de Sézary/diagnóstico , Neoplasias Cutáneas/tratamiento farmacológico , Neoplasias Cutáneas/genética , Neoplasias Cutáneas/patologíaRESUMEN
INTRODUCTION: The hemophagocytic syndrome is rare and sometimes associated with tuberculosis. OBSERVATION: We report the case of a 30-year-old migrant from Congo presenting with a recurrent right pleuropneumonia, cachexia and night sweat. Blood tests revealed bicytopenia with a normal myelogram. Thorax CT-scan showed large mediastinal lymph nodes and pleuritis. Mediastinal lymph node biopsy concluded to granulomatosis lymphadenopathy with necrosis and bone marrow biopsy suggested hemophagocytic syndrome. The outcome was favorable with antibiotics and corticosteroids.
Asunto(s)
Linfohistiocitosis Hemofagocítica/etiología , Tuberculosis/complicaciones , Adulto , Humanos , MasculinoRESUMEN
Scarlet fever is a rare disease in adult patients. We report a patient in whom scarlet fever was associated with hypertrophic gastritis and multiple organ failure. A 62-year-old woman presented with septic shock and multiple organ failure. Bacteriological survey was negative. Abdominal tomodensitometry showed an hypertrophic gastritis. Histological analysis demonstrated a non specific gastritis without any tumoral sign. Cefotaxime and amoxicillin led to improvement and hypertrophic gastritis progressively resolved. A sandpaper rash over the body with finger desquamation, elevation of antistreptolysin O and a recent contact with an infected grandson led to the diagnosis of scarlet fever. Due to antibiotic prescription, scarlet fever is now uncommon. Although classical, ENT or gastroenteritis presentations may be puzzling for the diagnosis of scarlet fever. As 150 years ago, diagnosis of scarlet fever is still a clinical challenge.
Asunto(s)
Gastritis Hipertrófica/microbiología , Insuficiencia Multiorgánica/microbiología , Escarlatina/diagnóstico , Escarlatina/microbiología , Streptococcus pyogenes , Amoxicilina/uso terapéutico , Antibacterianos/uso terapéutico , Antiestreptolisina/sangre , Biomarcadores/sangre , Cefotaxima/uso terapéutico , Diagnóstico Diferencial , Quimioterapia Combinada , Exantema/microbiología , Femenino , Gastritis Hipertrófica/diagnóstico , Gastritis Hipertrófica/tratamiento farmacológico , Gastritis Hipertrófica/inmunología , Humanos , Factores Inmunológicos/sangre , Persona de Mediana Edad , Insuficiencia Multiorgánica/diagnóstico , Insuficiencia Multiorgánica/tratamiento farmacológico , Insuficiencia Multiorgánica/inmunología , Escarlatina/tratamiento farmacológico , Escarlatina/inmunología , Choque Séptico/microbiología , Streptococcus pyogenes/aislamiento & purificación , Resultado del TratamientoRESUMEN
Microcystins are usually the predominant cyanotoxins present in both drinking and recreational waters after cyanobacterial blooms. Their classic toxic effect is hepatotoxicity through inhibition of serine/threonine phosphatases. However, recent studies also reported oxidative stress generation and disruption of ion regulation in aquatic organisms after microcystins exposure. In the present study, aqueous extracts of Microcystis aeruginosa were administered to the estuarine crab Chasmagnathus granulatus (Decapoda, Brachyura) by gavage in variable doses (from 34 to 860 microg kg(-1)) and exposure times (6, 12, and 72 h). A control group was exposed to saline solution. Analyzed variables included oxygen consumption, lipid peroxidation (LPO), enzyme activities (glutathione S-transferases or GST; alanine aminotransferase or ALT; aspartate aminotransferase or AST; and lactate dehydrogenase or LDH), glycogen, and microcystins content. Oxygen consumption increased in organisms exposed for 12h to 860 microg kg(-1) of microcystins and a similar result was observed after 72 h at doses equal to or higher than 34 microg kg(-1). LPO levels increased in doses equal to or higher than 34 microg kg(-1) after 72 h. GST and LDH activities increased after 12 h (at a dose of 860 microg kg(-1)), but ALT and AST activities remained unaltered in all experimental conditions. Glycogen content decreased after 72 h exposure at doses equal to or higher than 172 microg kg(-1). After 12h of exposure to 860 microg kg(-1) of microcystins, the concentration found in the hepatopancreas of C. granulatus was 13.17+/-0.56 microg kg(-1). In crabs exposed to doses higher than 172 microg kg(-1) during 72 h this value raised to 32.14+/-4.12 microg kg(-1). The obtained results indicated that microcystins exposure led the tissue to an oxidative stress condition (high LPO levels), at least in part favored by the augment of oxygen consumption, altering the glycogen metabolism. GST responses were only observed in the short-term experiment (12 h) and no effect on classical markers of vertebrate liver damage (ALT and AST) was observed. Although the hepatopancreas from C. granulatus accumulated a relatively low concentration of toxins, it was enough to induce physiological and biochemical disturbances.