[Primary plasma cell leukemia. Report of five cases].
Leucemia de células plasmáticas primaria: experiencia en un hospital público chileno.
Adult
Blood Cell Count
Calcium
/ blood
Chile
/ epidemiology
Creatinine
/ blood
Cytogenetic Analysis
Female
Flow Cytometry
/ methods
Humans
In Situ Hybridization, Fluorescence
Leukemia, Plasma Cell
/ epidemiology
Male
Middle Aged
Paraproteinemias
/ epidemiology
Retrospective Studies
Survival Analysis
Treatment Outcome
Journal
Revista medica de Chile
ISSN: 0717-6163
Titre abrégé: Rev Med Chil
Pays: Chile
ID NLM: 0404312
Informations de publication
Date de publication:
2019
2019
Historique:
received:
11
09
2018
accepted:
12
12
2018
entrez:
9
3
2019
pubmed:
9
3
2019
medline:
19
3
2019
Statut:
ppublish
Résumé
Primary plasma cell leukemia (pPCL) is uncommon, aggressive and has a different biology than multiple myeloma (MM). To report the features of patients with pPCL. Review of databases of the Hematology Department and the Hematology laboratory. 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. These patients with pPCL were younger and had a more aggressive clinical outcome than in multiple myeloma.
Sections du résumé
BACKGROUND
BACKGROUND
Primary plasma cell leukemia (pPCL) is uncommon, aggressive and has a different biology than multiple myeloma (MM).
AIM
OBJECTIVE
To report the features of patients with pPCL.
MATERIAL AND METHODS
METHODS
Review of databases of the Hematology Department and the Hematology laboratory.
RESULTS
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
CONCLUSIONS
These patients with pPCL were younger and had a more aggressive clinical outcome than in multiple myeloma.
Identifiants
pubmed: 30848760
pii: S0034-98872019000100018
doi: 10.4067/S0034-98872019000100018
pii:
doi:
Substances chimiques
Creatinine
AYI8EX34EU
Calcium
SY7Q814VUP
Types de publication
Journal Article
Langues
spa
Sous-ensembles de citation
IM