ISSN: 1300-7777 E-ISSN: 1308-5263
De-novo CD5 + B- prolymphocytic leukemia (PLL) presenting at younger age with favourable outcome [Turk J Hematol]
Turk J Hematol. 2008; 25(3): 149-151

De-novo CD5 + B- prolymphocytic leukemia (PLL) presenting at younger age with favourable outcome

Rakhee Kar, Rajat Kumar, Seema Tyagi
Department Of Hematology, All India Institute Of Medical Sciences, New Delhi, India

B-cell prolymphocytic leukemia (B-PLL) comprises 1% of chronic lymphocytic leukemias. CD5 positivity is seen in 1/3rd of cases which generally arise from pre existing CLL. They have longer median survival compared with de novo B-PLL which are commonly CD5 negative and are more aggressive with an older age of presentation. Herewith, we describe a 48-year-old male of de-novo CD5+ B-PLL presenting with minimal lymphadenopathy and massive splenomegaly with 90% atypical lymphoid cells in the peripheral smear and bone marrow. Immunophenotyping was strongly positive for CD5, CD45, CD19, CD22, FMC-7, S-Ig and CD38, moderately positive for CD 11c, weakly positive for CD23 and negative for CD-103 and ZAP 70. The patient responded well to fludarabine and cyclophosphamide and had an uneventful hospital course. Our case illustrates a de-novo B-PLL with aberrant CD5 positivity who had a short duration of illness, younger age at presentation and favourable treatment outcome.

Keywords: CD5+, Prolymphocytic leukemia, Splenomegaly, Prognosis


Küçük yaşta olumlu prognoz ile seyreden de-novo CD5 + B- prolenfositik lösemi (PLL)

Rakhee Kar, Rajat Kumar, Seema Tyagi
Department Of Hematology, All India Institute Of Medical Sciences, New Delhi, India

B-hücreli prolenfositik lösemi (B-PLL), kronik lenfositik lösemilerin %1’ini oluşturur. CD5 pozitifliği vakaların 1/3’ünde görünmekte olup genellikle önceden var olan CLL’den ileri gelmektedir. Genellikle CD5 negatif olan ve ileri yaşta göründüğünde daha agresif olan de novo B-PLL’e nazaran daha uzun ortalama sağkalımları bulunmaktadır. Burada, de novo CD5+B-PLL hastası olan, minimal lenfadenopati ve masif splenomegaliye sahip, periferik yaymasında ve kemik iliğinde %90 atipik lenfoid hücre olan 48 yaşındaki bir erkeği sunuyoruz. İmmünofenotipleme,CD5, CD45, CD19, CD22, FMC-7, S-Ig ve CD38 için güçlü derecede, CD 11c için orta derecede ve CD23 için zayıf derecede pozitifti ve CD-103 ve ZAP 70 için de negatifti. Hasta fludarabin ve siklofosfamide iyi yanıt vermişti ve hastanede kaldığı sürede herhangi bir olay ortaya çıkmamıştı. Aberan CD5 pozitifliği olan de novo B-PLL olgumuz, kısa hastalık süresi, genç yaşta başvuru ve tedaviye iyi cevap göstermektedir.

Anahtar Kelimeler: CD5+, prolymphocytic leukemia, splenomegaly, prognosis.


Rakhee Kar, Rajat Kumar, Seema Tyagi. De-novo CD5 + B- prolymphocytic leukemia (PLL) presenting at younger age with favourable outcome. Turk J Hematol. 2008; 25(3): 149-151

Corresponding Author: Rakhee Kar, India


TOOLS
Full Text PDF
Print
Download citation
RIS
EndNote
BibTex
Medlars
Procite
Reference Manager
Share with email
Share
Send email to author

Similar articles
PubMed
Google Scholar


 



Impact Factor (2016) = 0.686