[A case of acute lymphoblastic leukemia with an unusual presentation and slow development]. / Leucemia Linfoblastica Acuta (LLA). Descrizione di un caso ad esordio inusuale e lento decorso.

The authors describe the case of a 3-year-old girl with acute lymphoblastic leukemia which was diagnosed several months after the appearance of the first symptoms. The delay can be attributed to the vague symptoms at onset in the form of a single laterocervical adenopathy which responded to antibiotic and antiphlogistic therapy, the total absence of any indicative hematological symptoms and the patient s persistent excellent general conditions. In the light of this unusual case, the authors stress the need to carry out invasive diagnostic tests on lymph node lesions that are often defined as aspecific given that they may occasionally disguise more severe lymphoproliferative diseases.

[Infantile dermatomyositis. Clinical aspects and prospective treatments]. / Dermatomiosite infantile. Aspetti clinici e prospettive terapeutiche.

Juvenile dermatomyositis (DMI) is a rare illness of multisystem inflammatory disease of unknown etiology. It is usually considered as a part of the spectrum of polymyositis/dermatomyositis (PM/DM), and is distinguished from the adult form in having a number of different clinical features as well as distinctive pathologic findings. Although the clinical findings and course of the disease, laboratory evaluation and therapy have been reviewed, many of these studies are old and may not accurately reflect the current status of the disease as it is now defined and treated. Generally it is first diagnosed by the appearance of typical signs in both the skin and muscles. The clinical picture shows early signs of heterogeneity and is confirmed by these latter developments. Its classification falls into three groups: acute single cycle, chronic polycycle and continued chronic; this sickness invariably evolves into one or another of these three. Our current work involves 15 cases of DMI; 8 have acute monocycle, 4 chronic polycycle and 3 continued chronic. This illustrates the clinical and biological heterogenousness of this illness which in itself presents extreme difficulties when considering possible treatment. From our experience, high dose of corticosteroida intraveins have had the best result. Where cases have been acute at the beginning of the sickness and where there has been complications, particularly those of a bronco-pulmonary nature (we treated 3 such cases) it has proven to be beneficial to use corticosteroid together with the immunosuppresant: ciclofosfamide. The use of ciclosporina in one case who had resisted other forms of therapy has appeared to be effective, though this has yet to be verified.