Cardiac sarcoidosis.

Sarcoidosis is a systemic disease which affects many organs, including the heart. Cardiac sarcoidosis has a reported incidence of about 25 % and carries a poor prognosis. It can occur in the form of conduction abnormalities, pericardial and valvular heart disease, congestive heart failure, arrhythmias and sudden cardiac death. The diagnosis of cardiac sarcoidosis is difficult, requiring a high index of suspicion and the use of electrocardiography, echocardiography, nuclear medicine imaging, myocardial biopsy and magnetic resonance imaging. Corticosteroids have been the cornerstone of treatment of cardiac sarcoidosis, but other immunosuppressives have also been used, along with standard heart failure therapy, antiarrhythmic medications, pacemakers and implantable defibrillators. Cardiac transplantation is an option for patients who do not respond to medical treatment. We briefly review the current armamentarium for the diagnosis and treatment of cardiac sarcoidosis.

Primary lung involvement in Waldenström's macroglobulinaemia: report of two cases and review of the literature.

Pulmonary involvement in Waldenström's macroglobulinaemia (WM) occurs in 3-5% of cases, but lung involvement without bone marrow infiltration is extremely rare. We report 2 patients who presented with bilateral consolidations on chest X-ray and non-specific symptoms and were treated for a long period of time for pulmonary infections until the diagnosis was made by open lung biopsy. Both patients presented high monoclonal IgM in the serum and one also had blood lymphoplasmacytosis. Trephine bone biopsy and bone marrow smears were normal and there was no other site of involvement. Along with the presentation of our patients, we review the literature, discuss some of the possible underlying mechanisms and raise the attention of clinicians to this rare manifestation of the disease.

Waldenström's macroglobulinemia: clinical course and prognostic factors in 60 patients. Experience from a single hematology unit.

Waldenström's macroglobulinemia (WM) is a lymphoplasmacytic lymphoma characterized by the presence in patients' serum of an IgM monoclonal component. We report on our experience with 60 WM patients, focusing on their clinical findings, response to treatment, and the possible identification of prognostic factors. Of these patients, 70% presented with fatigue, and lymphadenopathy was observed in 22%, splenomegaly in 18%, hepatomegaly in 13%, and extranodal site of involvement in 6%. Bleeding tendency was seen in 17%, infections in 17%, hyperviscosity syndrome in 12%, and cardiac failure in 25% of the patients. The median of IgM levels was 30 g/l with hypoalbuminemia in 20% of cases, hypogammaglobulinemia in 27%, polyclonal hypergammaglobulinemia in 15%, kappa light-chain restriction in 78%, and Bence-Jones proteinuria in 54%. Anemia was frequent (85%), followed by leukocytosis (18%), lymphocytosis (12%), leukopenia (10%), and thrombocytopenia (10%). Cryoglobulinemia and autoimmune hemolytic anemia were encountered in 5%. In all cases but two, bone marrow was involved. Of 50 patients initially treated with intermittent oral chlorambucil, 46 (92%) responded. Median overall survival was 108 months. Factors associated with adverse prognosis were age > or =65 years (p=0.06), presence of lymphadenopathy (p=0.06), bone marrow infiltration > or =50% (p=0.007), international prognostic index (IPI) > or =3 (p=0.0001), and Morel's scoring system (p=0.04). Concluding, we found in this series of WM patients that chlorambucil is an effective treatment and that the parameters of age, lymphadenopathy, percentage of bone marrow infiltration, IPI, and Morel's scoring system carry prognostic significance.

Vascular manifestations of Behçet's disease.

The purpose of this article is to present the spectrum of vascular disease accompanying Behçet's disease. Four cases of the disease with various vascular manifestations are presented. In the first case superior vena cava syndrome due to thrombosis was the presenting symptom. In the second case foot ischemia with toe necrosis was prevalent. In the third case the patient developed recurrent aneurysmal disease in large and medium size arteries requiring successive vascular surgery, and in the fourth case the patient had SVC thrombosis and ruptured femoral aneurysm. All four patients were young males. Although vascular complications of Behçet disease are uncommon they may pose serious therapeutic problems.