Sideroblastic anaemia with reactive thrombocytosis versus myelodysplastic/myeloproliferative disease.

The reported incidence of thrombocytosis among cases of refractory anaemia with ring sideroblasts (RARS) may be as high as 15%. We report four additional cases of this association, which appear to be hematologically heterogeneous. One patient clearly represents a case of RARS with reactive thrombocytosis. Two cases have features suggestive of the coincidental occurrence of essential thrombocythemia and RARS. The fourth case could be best classified as a subtype of myelodysplastic/myeloproliferative disease unclassifiable (MDS/MPD U). Only new biological or molecular markers will allow better differentiation between these disorders.

Chronic clonal proliferative disease of gamma-delta (gammadelta) T-cells in a patient with rheumatoid arthritis and neutropenia: lack of the morphology and the immunophenotype of large granular lymphocytes.

Normal gammadelta T-lymphocytes have the morphology of large granular lymphocytes (LGL) and, as the LGL of alphabeta T-cells, they express pan-T antigens, NK-associated antigens and the cytotoxic molecules, perforin and granzime B. In this report we describe an unusual patient with rheumatoid arthritis and neutropenia who has a chronic gammadelta T-cell proliferation with a chronic, indolent clinical course and atypical lymphocytes, lacking the classical LGL morphology, not expressing NK-associated antigens, and not expressing perforin or granzyme B. In spite of the atypical morphological features of the clonal cells, which were suggestive of a more malignant process, the patient has been followed for 4 years without aggressive therapy. It is important to recognize this entity and to distinguish it from other gammadelta T proliferations such as the hepatosplenic gammadelta T-cell lymphoma.

[A radiological study of the cervical alterations in Down syndrome. New findings on computerized tomography and three dimensional reconstructions]. / Estudio radiológico de las alteraciones cervicales en el síndrome de Down. Nuevos hallazgos mediante tomografía computarizada y reconstrucciones tridimensionales.

OBJECTIVE: We studied a large proportion of the population in our health district who have Down's syndrome to determine the incidence and variety of changes in the spine and to define the guidelines for preventive diagnosis advisable in relation to atlanto-axial instability, a common disorder in these patients. PATIENTS AND METHODS: First phase: a plain X-ray of the cervical spine in a neutral lateral projection and in flexion in 188 patients, measuring the atlanto-odontoid distance. Second phase: computerized tomography (CT) studies and three dimensional reconstructions in 25 patients (13.3%) chosen at random. The axial cuts from the upper portion of C3 to the occiput were 3 mm in thickness with 3 mm intervals and a standard reconstruction algorithm. RESULTS: The incidences of atlanto-axial instability with an atlodontoid distance (3)5 mm were not comparable with the published series. There was a lower incidence (4.2%), with no difference between measurements in flexion and in the neutral lateral views. There was a greater incidence of malformations than in other reports, including a rare case of os odontoideum and also constant asymmetry of the occipital condyles (100%) in the patients of the CT series and consequently instability of the atlas (96%) and off-centered odontoides (84%). CONCLUSIONS: The study showed that there was deficient asymmetrical development of the occipital bone, which caused different heights of the occipital condyles and led to cervico-cranial mal-position. For study of the degree of error of position and congenital anomalies. We recommend replacing plain X-ray studies by CT with three dimensional reconstructions.

[Usefulness of computerized tomography and bronchoscopy in patients with hemoptysis. Analysis of 482 cases]. / Utilidad de la tomografía computarizada y la broncoscopia en los pacientes con hemoptisis. Análisis de 482 casos.

OBJECTIVE: We have examined the computed chest tomography (CT) and bronchoscopy utility in diagnosis and accurately identifying the sites of pulmonary bleeding in patients with hemoptysis. METHODS: We prospectively reviewed the etiology and the site of bleeding of 482 patients with hemoptysis who had undergone bronchoscopy and CT when we suspected a carcinoma, bronchiectasis or the type of bleeding. We analyzed the age, sex, history of tobacco, bleeding, chest radiography, chest computed tomography and bronchoscopy. RESULTS: 62 years (DS 13.6), 84.4% males, 80% smokers and a volume of bleeding of 42.5 ml/day (DS 89) for 15 days (DS 25). Lung cancer was identified in 43%, bronchiectasis in 20%, chronic obstructive lung disease in 14%, pneumonia 8% and an unknown etiology in only 3%. Bronchoscopy located the site of bleeding in 77% and CT in 83%. When we examine only the lung carcinomas, the chest radiography was normal in 11% and the bronchoscopy was diagnostic in 87%, including 6 cases with a normal CT. Simultaneously chest CT or radiography and bronchoscopy detected all the lung cancers. When the chest radiography was inespecific (32%), CT was diagnostic in 43% and bronchoscopy in 14% (p < 0.001) or localized the site of bleeding in 52% with the CT or 23% with the bronchoscopy (< 0.001). CONCLUSIONS: We concluded that bronchoscopy and CT were useful and complementary in etiologic diagnosis and to localize the site of bleeding in patients with hemoptysis. The bronchoscopy was preferable in patients with a lung cancer and the CT when we studied all the etiologies or the patients without a lung cancer, especially when the radiography was normal. When we simultaneously used a CT or a radiography and the bronchoscopy, all the lung cancers were detected.

B-lymphoproliferative disorders in patients with hepatitis C virus infection.

A cohort of 148 consecutive patients with hepatitis C virus infection were studied at the rheumatology out-patient clinic of a tertiary care teaching hospital. The diagnosis of hepatitis C virus infection was supported by detection of HCV RNA in the serum. Cryoglobulin screening was done in all patients and the presence of a monoclonal component was investigated when the cryocrit was higher than 1%. Patients with lymphoproliferative disorders were further investigated. Four patients had a B lymphoproliferative disorder, which represents a prevalence of 2.7% in this cohort of patients with hepatitis C virus infection. Mixed cryoglobulinemia (MC), with cryocrit higher than 1%, was found in 16 of 148 patients (11%). It was type III MC in 13 patients and type II MC in 3. All patients who developed a B lymphoproliferative disorder had mixed cryoglobulinemia, with a monoclonal component (type II MC) in two patients and without a monoclonal component (type III MC) in the other two. The incidence of B-lymphoproliferative disorders among this cohort of patients with hepatitis C virus infection seems to be significantly increased. However, the high frequency of asymptomatic, undiagnosed HCV infection among the apparently healthy general population may decrease the true significance of this association. Systematic screening of cryoglobulin production in patients with hepatitis C virus infection might clarify whether the risk of B lymphoproliferative disorders increases when type II or type III mixed cryoglobulinemia is present.