RESUMO
Background: Primary plasma cell leukemia (pPCL) is uncommon, aggressive and has a different biology than multiple myeloma (MM). Aim: To report the features of patients with pPCL. Material and Methods: Review of databases of the Hematology Department and the Hematology laboratory. Results: Of 178 patients with monoclonal gammopathies, five (2.8%) patients aged 33 to 64 years (three females) had a pPCL. The mean hemoglobin was 7.3 g/dL, the mean white blood cell count was 52,500/mm3, with 58% plasma cells, and the mean platelet count was 83,600/mm3. The mean bone marrow infiltration was 89%, LDH was 2,003 IU/L, serum calcium was 13 mg/dL, and creatinine 1.5 mg/dL. Two patients had bone lesions. Three were IgG, one IgA lambda and one lambda light chain. CD20 was positive in one, CD56 was negative in all and CD117 was negative in 3 cases. By conventional cytogenetic analysis, two had a complex karyotype. By Fluorescence in situ Hybridization, one was positive for TP53 and another for t (11; 14). One patient did not receive any treatment, three patients received VTD PACE and one CTD. None underwent transplant. Three patients are alive. The mean survival was 14 months. Conclusions: These patients with pPCL were younger and had a more aggressive clinical outcome than in multiple myeloma.
Assuntos
Humanos , Masculino , Feminino , Adulto , Pessoa de Meia-Idade , Leucemia Plasmocitária/genética , Leucemia Plasmocitária/epidemiologia , Paraproteinemias/genética , Paraproteinemias/patologia , Paraproteinemias/epidemiologia , Contagem de Células Sanguíneas , Leucemia Plasmocitária/patologia , Leucemia Plasmocitária/terapia , Análise de Sobrevida , Chile/epidemiologia , Cálcio/sangue , Estudos Retrospectivos , Resultado do Tratamento , Hibridização in Situ Fluorescente , Creatinina/sangue , Análise Citogenética , Citometria de Fluxo/métodosRESUMO
To determine the prevalence of monoclonal gammopathies in Kuwait and its association with chronic hepatitis C virus [HCV] infection. Methodology: Serum protein electrophoresis and measurement of immunoglobulin levels were carried out prospectively in 100 consecutive patients with chronic HCV infection. Among the 100 patients tested [82 males, 18 females; median age 45 years], 59 had polyclonal band in serum protein electrophoresis while the other 41 had a normal pattern. None of the patients had monoclonal gammopathy. The mean serum immunoglobulin levels were: IgG 18.4 g/l [range 4-39], IgA 2.9 g/l [range 0.09-8] and IgM 1.6 g/l [range 0.14-6.08]. There was no evidence of monoclonal gammopathies in patients with chronic HCV infection in Kuwait