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Abstract
RÉSUMÉ
Les auteurs rapportent deux cas de leucémie primitive à plasmocytes. La patiente (A) de 38 ans sans antécédents notables a été adressée pour exploration d’une polyadénopathie cervicale. Le tableau initial clinico-biologique était trompeur associant une otalgie droite, une polyadénopathie cervicale et une polynucléose. L’aspect atypique des cellules au frottis sanguin et à l’examen anatomopathologique de ces adénopathies orientaient le diagnostic vers une adénite réactionnelle à une infection ORL. La blastose sanguine a conduit au diagnostic d’une leucémie à plasmocyte par la présence des cellules CD38+, CD45-, CD138-, CD56-. La patiente (B) de 55 ans, avait un tableau clinique inaugural associant des signes d’infection pulmonaire à une pancytopénie expressive. La plasmocytose sanguine a permis de porter le diagnostic par la présence des cellules CD38+, CD45+, CD138- à l’immunophénotypage. Chez ces deux patientes, le tableau clinique était bruyant et agressif faisant qu’aucun traitement n’a été instauré, avec une évolution vers le décès avant le diagnostic. L’intérêt de ces observations vient d’une part, de la rareté de cette forme de leucémie dont la fréquence est estimée entre 1 à 4% et d’autre part, des difficultés diagnostiques et thérapeutiques avec une évolution fatale à brève échéance. Ceci doit amener les praticiens à y penser devant toute blastose sanguine.
ABSTRACT
We report two cases of primary plasma cell leukemia. The patient (A) was 38-year-old, with no pertinent medical history. She was referred for evaluation of cervical lymp nodes. The initial clinical-biological picture was misleading, associating right ear pain, cervical lymp nodes, and polynucleosis. The atypical appearance of the cells in the blood and the histology of these lymph nodes led to the diagnosis of lymphadenitis post ENT infection. But, in front of plasma cells in the peripheral blood, the diagnosis of primary plasma celle leukemia was made due to the presence of cells expressing CD38 and not expressing CD45, CD138, and CD56. Patient (B), 55 years old, had an inaugural clinical picture associating signs of pulmonary infection and expressive pancytopenia. The immunophenotyping showed cells CD38+, CD45 +, and CD138-. In both patients, the clinical picture was quite aggressive, with progression to death before definitive diagnosis and treatment. Our observations are interesting firstly, because of the rarity of this type of leukemia (the frequency is estimated between 1 to 4%); and secondly, because of the diagnostic, treatment and evolution in our context were challenging. This should incite the practitioners to think about this very severe disease in front of any blood blastosis.
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References
- García-Sanz R, Orfão A, González M, Tabernero MD, Bladé J, Moro MJ et al. Primary plasma cell leukemia: clinical, immunophenotypic, DNA ploidy, and cytogenetic characteristics. Blood 1999 93(3) : 1032–1037.
- Tiedemann RE, Gonzalez-Paz N, Kyle RA, Santana-Davila R, Price-Troska T, Van Wier SA, et al. Genetic aberrations and survival in plasma cell leukemia. Leukemia 2008; 22:1044–1052.
- Lorrea CF, Kyle RA, Durie BGM, Ludwig H, Usmani S, Vesole DH et al . PLASMA CELL LEUKEMIA: consensus statement on diagnostic requirements response criteria, and treatement. Recommandation by the International Myéloma Working Group. National Institute of Health 2013; 27 (4): 780-791.
- Kyle RA, Maldonado JE, Bayrd ED. Plasma cell leukemia. Report on 17 cases. Archives of Internal Medicine 1974 ; 133(5) : 813–818.
- Abarracin F, Fonseca R. Plasma cell leukemia. Blood Rev 2011;25 (3):107-12.
- Muzamil1 J, Aziz SA, Bhat GM, Lone AR, Bhat S, Nabi F. CLINICAL PROFILE OF PLASMA CELL LEUKEMIA AT TERTIARY CARE HOSPITAL IN KASHMIR, INDIA. International Journal Of Advances In Case Reports, 2016; 3(1):33-40.
- Benasconi C, Castelli G, Pagnucco G, brusamolino E. Plasma cell Leukemia. A repport on 15 Patients. Eur J Haematol suppl. 1989;51:76-83.
- Rodriguez C, Pont JC, Gouin Thibault I, Andrieu AG, Molina T, le Tourneau A, et al. Plasma cell Leukemia Ann Biol Clin (Paris) 2015 ; 63 (5) : 535-9.
- Gluzinski A, Reichentein M. Myeloma und leucaemia lymphatica plasmocellularis. Wien Klin Wochenschr. 1906; 19:336.
- Kyle RA. Multiple myeloma: review of 869 cases. Mayo Clin Proc. 1975; 50:29–40.
- Ramsingh G, Mehan P, Luo J, Vij R, Morgensztern D. Primary plasma cell leukemia: a Surveillance, Epidemiology, and End Results database analysis between 1973 and 2004. Cancer. 2009; 115:5734–5739.
- Chan SM, Georges T, Cherry AM, Meideiros BC. Complete remission of primary plasma cell leukemia with bortezomib, doxorubicin and dexamethasone. A case repport. Cases J 2009; 2(1): 212.
- Tembhare PR, Subramanian PG, Sehgal K, Yajamanam B, Kumar A, Gadge V et al. Immunophenotypic profile of plasma cell leukemia: a retrospective study in a reference cancer center in India and review of literature. Indian J Pathol Microbiol. 2011 Apr-Jun;54(2):294-8.
References
García-Sanz R, Orfão A, González M, Tabernero MD, Bladé J, Moro MJ et al. Primary plasma cell leukemia: clinical, immunophenotypic, DNA ploidy, and cytogenetic characteristics. Blood 1999 93(3) : 1032–1037.
Tiedemann RE, Gonzalez-Paz N, Kyle RA, Santana-Davila R, Price-Troska T, Van Wier SA, et al. Genetic aberrations and survival in plasma cell leukemia. Leukemia 2008; 22:1044–1052.
Lorrea CF, Kyle RA, Durie BGM, Ludwig H, Usmani S, Vesole DH et al . PLASMA CELL LEUKEMIA: consensus statement on diagnostic requirements response criteria, and treatement. Recommandation by the International Myéloma Working Group. National Institute of Health 2013; 27 (4): 780-791.
Kyle RA, Maldonado JE, Bayrd ED. Plasma cell leukemia. Report on 17 cases. Archives of Internal Medicine 1974 ; 133(5) : 813–818.
Abarracin F, Fonseca R. Plasma cell leukemia. Blood Rev 2011;25 (3):107-12.
Muzamil1 J, Aziz SA, Bhat GM, Lone AR, Bhat S, Nabi F. CLINICAL PROFILE OF PLASMA CELL LEUKEMIA AT TERTIARY CARE HOSPITAL IN KASHMIR, INDIA. International Journal Of Advances In Case Reports, 2016; 3(1):33-40.
Benasconi C, Castelli G, Pagnucco G, brusamolino E. Plasma cell Leukemia. A repport on 15 Patients. Eur J Haematol suppl. 1989;51:76-83.
Rodriguez C, Pont JC, Gouin Thibault I, Andrieu AG, Molina T, le Tourneau A, et al. Plasma cell Leukemia Ann Biol Clin (Paris) 2015 ; 63 (5) : 535-9.
Gluzinski A, Reichentein M. Myeloma und leucaemia lymphatica plasmocellularis. Wien Klin Wochenschr. 1906; 19:336.
Kyle RA. Multiple myeloma: review of 869 cases. Mayo Clin Proc. 1975; 50:29–40.
Ramsingh G, Mehan P, Luo J, Vij R, Morgensztern D. Primary plasma cell leukemia: a Surveillance, Epidemiology, and End Results database analysis between 1973 and 2004. Cancer. 2009; 115:5734–5739.
Chan SM, Georges T, Cherry AM, Meideiros BC. Complete remission of primary plasma cell leukemia with bortezomib, doxorubicin and dexamethasone. A case repport. Cases J 2009; 2(1): 212.
Tembhare PR, Subramanian PG, Sehgal K, Yajamanam B, Kumar A, Gadge V et al. Immunophenotypic profile of plasma cell leukemia: a retrospective study in a reference cancer center in India and review of literature. Indian J Pathol Microbiol. 2011 Apr-Jun;54(2):294-8.