RESUMEN
The use of steroids is not required in myeloid malignancies and remains controversial in patients with acute lung injury (ALI) or acute respiratory distress syndrome (ARDS). We sought to evaluate dexamethasone in patients with ALI/ARDS caused by acute monocytic leukaemia (AML FAB-M5) via either leukostasis or leukaemic infiltration. Dexamethasone (10 mg every 6 h until neutropenia) was added to chemotherapy and intensive care unit (ICU) management in 20 consecutive patients between 2005 and 2008, whose data were compared with those from 20 historical controls (1994-2002). ICU mortality was the primary criterion. We also compared respiratory deterioration rates, need for ventilation and nosocomial infections. 17 (85%) patients had hyperleukocytosis, 19 (95%) had leukaemic masses, and all 20 had severe pancytopenia. All patients presented with respiratory symptoms and pulmonary infiltrates prior to AML FAB-M5 diagnosis. Compared with historical controls, dexamethasone-treated patients had a significantly lower ICU mortality rate (20% versus 50%; p = 0.04) and a trend for less respiratory deterioration (50% versus 80%; p = 0.07). There were no significant increases in the rates of infections with dexamethasone. In conclusion, in patients with ALI/ARDS related to AML FAB-M5, adding dexamethasone to conventional chemotherapy seemed effective and safe. These results warrant a controlled trial of dexamethasone versus placebo in AML FAB-M5 patients with noninfectious pulmonary infiltrates.
Asunto(s)
Lesión Pulmonar Aguda/tratamiento farmacológico , Lesión Pulmonar Aguda/etiología , Antineoplásicos/uso terapéutico , Dexametasona/uso terapéutico , Leucemia Monocítica Aguda/complicaciones , Leucemia Monocítica Aguda/tratamiento farmacológico , Síndrome de Dificultad Respiratoria/tratamiento farmacológico , Lesión Pulmonar Aguda/mortalidad , Adulto , Anciano , Femenino , Humanos , Unidades de Cuidados Intensivos/estadística & datos numéricos , Leucemia Monocítica Aguda/mortalidad , Infiltración Leucémica/tratamiento farmacológico , Leucostasis/inducido químicamente , Pulmón/efectos de los fármacos , Pulmón/fisiopatología , Masculino , Persona de Mediana Edad , Pancitopenia/tratamiento farmacológico , Respiración Artificial/estadística & datos numéricos , Síndrome de Dificultad Respiratoria/etiología , Síndrome de Dificultad Respiratoria/mortalidad , Pruebas de Función Respiratoria , Índice de Severidad de la Enfermedad , Resultado del TratamientoRESUMEN
BACKGROUND: Cutaneous involvement has been reported in 30-40% of children with the familial form of haemophagocytic syndrome. However, few studies have focused on cutaneous manifestations in patients with reactive haemophagocytic syndrome (RHS). OBJECTIVES: To describe the frequency, clinical features and prognosis of skin involvement in adult patients with RHS. METHODS: We conducted a retrospective study in a French university-based tertiary centre. The medical records of all adult patients with a suspected or confirmed diagnosis of RHS during a 2-year period were reviewed. Demographic, clinical, biological and histological data of patients were compared using nonparametric tests. RESULTS: The medical charts of 151 patients were reviewed, 69 of whom had a definite diagnosis of RHS (35% women; mean +/- SD age 49 +/- 17 years). The aetiology of RHS was mainly B-cell or T-cell lymphoma (n = 33) or herpesvirus infection (n = 19). Cutaneous manifestations were observed in 32 (46%) patients and were of three types: (i) specific to the underlying malignancy (Kaposi sarcoma n = 8, cutaneous lymphoma n = 4), (ii) reflecting the biological consequences of RHS (thrombopenic purpura n = 10, conjunctival jaundice n = 7), and (iii) a generalized, transient, nonpruriginous maculopapular rash (n = 18). None presented with erythroderma, or with eczematiform, ichthyosiform, psoriasiform or bullous lesions. One patient had cytophagic histiocytic panniculitis. Histological features of maculopapular rash biopsies were usually nonspecific. The rate of in-hospital death was not significantly associated with cutaneous involvement. CONCLUSIONS: A generalized, nonpruriginous, transient, maculopapular rash is frequently observed in patients with RHS. Although nonspecific, awareness of this cutaneous involvement may assist physicians in the initial diagnosis of RHS.
Asunto(s)
Exantema/patología , Linfohistiocitosis Hemofagocítica/patología , Adulto , Anciano , Diagnóstico Diferencial , Exantema/epidemiología , Exantema/etiología , Femenino , Francia/epidemiología , Herpes Simple/patología , Humanos , Linfohistiocitosis Hemofagocítica/complicaciones , Linfohistiocitosis Hemofagocítica/epidemiología , Linfoma de Células B/patología , Linfoma Cutáneo de Células T/patología , Masculino , Persona de Mediana Edad , Pronóstico , Estudios Retrospectivos , Neoplasias Cutáneas/patología , Estadística como AsuntoAsunto(s)
Toxina Diftérica/farmacología , Interleucina-3/farmacología , Leucemia-Linfoma de Células T del Adulto/tratamiento farmacológico , Leucemia-Linfoma de Células T del Adulto/metabolismo , Receptores de Interleucina-3/metabolismo , Proteínas Recombinantes de Fusión/farmacología , División Celular , Humanos , Inmunofenotipificación , Subunidad alfa del Receptor de Interleucina-3 , Leucemia-Linfoma de Células T del Adulto/patologíaRESUMEN
Through two consecutive trials, a policy that considered allogeneic stem cell transplantation (SCT) from a sibling donor in second rather than first complete remission (CR) in selected younger patients with acute myeloid leukemia (AML) with t(8;21)/inv(16) (core binding factor (CBF) group) or a normal karyotype (NN group) was followed by Acute Leukemia French Association (ALFA) centers. The outcome of 92 of these patients in first relapse (32 CBF, 60 NN) was reviewed with the aim of validating this strategy. The presence of an FLT3 internal tandem duplication (ITD) was retrospectively assessed in 50 patients. A total of 61 patients (66%) reached a second CR. Donor availability was an independent prognostic factor for survival in the whole patient population as well as in the CBF subset, but not in NN patients, further supporting this strategy for CBF-AMLs. In NN patients, FLT3-ITD was the main bad-prognosis factor for second CR achievement and survival, leading to consider SCT earlier, at least in FLT3-ITD patients with a donor.