RESUMO
BACKGROUND: Multiple myeloma is the most common malignant plasma cell dyscrasia and ranks second among primary haematological malignancies. This study describes the epidemiologic, clinical and pathologic profile of monoclonal gammopathies seen in the University Hospital of the West Indies (UHWI), a tertiary care referral centre. MATERIALS AND METHOD: A retrospective analysis of 85 cases diagnosed at UHWI over the 5-year period 2003-2007 was conducted. The cases were identified from the bone marrow records as well as the computerized database of the Medical Records Department. Clinical presentation, family and personal history and demographic data were retrieved. Haematological and biochemical results were also analyzed. RESULTS: There were 85 patients diagnosed with monoclonal gammopathies. The M:F ratio was 1.2:1 and the mean age was 65.7±1.3 years. Eighty percent of the patients had skeletal pain and 40% experienced weight loss. Of the patients experiencing bone pain 56.7% had multiple lytic lesions, 26.7% had pathological fractures and 26.7% had compression fractures. Seventy-four patients (87.1%) had a haemoglobin level <12.0 g/dL with 52.9% having values <8.0 g/dL. Renal impairment was evident at diagnosis in 36.5% . Hypercalcemia was seen in 26.5% and hyperuricemia in 45.9%. Of the 79 patients who had serum protein electrophoresis performed, 77.2% had at least one monoclonal band and of these 24.6% had a monoclonal protein also present on urine protein electrophoresis. CONCLUSIONS: The demographic profile in this group of patients is largely similar to other studies in predominantly Caucasian populations; however there was a notable increase in prevalence of severe disease at presentation, with the majority of patients presenting at the most advanced stage. It is probable that these differences reflect socioeconomic factors and not merely inherent ethnic variation in disease biology.
Assuntos
Paraproteinemias/patologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Medula Óssea/patologia , Região do Caribe , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos RetrospectivosRESUMO
A 38-year-old female of African-Caribbean origin presented with symptomatic anemia and was found to have hypoplastic thumbs and patchy hypopigmentation. Peripheral blood examination revealed pancytopenia and the bone marrow biopsy confirmed marrow hypoplasia. Fanconi anemia was later confirmed by flow cytometry and diepoxybutane testing. Treatment was limited to transfusions after development of toxicity with cyclosporine and androgen therapy. She manifested classical features of transfusion-related hemosiderosis and died 12 years after initial presentation.
Assuntos
Anemia de Fanconi/diagnóstico , Adulto , Idade de Início , Transfusão de Sangue , Anemia de Fanconi/epidemiologia , Anemia de Fanconi/terapia , Feminino , Humanos , Quelantes de Ferro/uso terapêutico , Jamaica , Polegar/anormalidadesRESUMO
A 16-year-old boy presented with cervical lymphadenopathy and histological features of a plasmacytoma. He was found to have lytic bone lesions, a serum IgA M-protein, and elevated beta 2-microglobulin. There was absence of anemia, hypercalcemia, and marrow plasmacytosis. He received local irradiation and 6 courses of chemotherapy comprising vincristine, adriamycin, and dexamethasone given at 4 weekly intervals. This was followed by complete resolution of his disease, and he remains clinically well 5 years after completion of therapy.