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

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 old...

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Main Authors: Seema Tyagi, Rajat Kumar, Rakhee Kar
Format: Article
Language:English
Published: Galenos Publishing House 2008-09-01
Series:Turkish Journal of Hematology
Subjects:
Online Access:http://www.journalagent.com/z4/download_fulltext.asp?pdir=tjh&plng=eng&un=TJH-54154
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author Seema Tyagi
Rajat Kumar
Rakhee Kar
author_facet Seema Tyagi
Rajat Kumar
Rakhee Kar
author_sort Seema Tyagi
collection DOAJ
description 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.
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spelling doaj.art-b322e731479043b5b1d5a94960ff5ee32023-02-15T16:09:48ZengGalenos Publishing HouseTurkish Journal of Hematology1300-77771308-52632008-09-012503149151De-novo CD5 + B- prolymphocytic leukemia (PLL) presenting at younger age with favourable outcomeSeema TyagiRajat KumarRakhee KarB-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.http://www.journalagent.com/z4/download_fulltext.asp?pdir=tjh&plng=eng&un=TJH-54154CD5+prolymphocytic leukemiasplenomegalyprognosis.
spellingShingle Seema Tyagi
Rajat Kumar
Rakhee Kar
De-novo CD5 + B- prolymphocytic leukemia (PLL) presenting at younger age with favourable outcome
Turkish Journal of Hematology
CD5+
prolymphocytic leukemia
splenomegaly
prognosis.
title De-novo CD5 + B- prolymphocytic leukemia (PLL) presenting at younger age with favourable outcome
title_full De-novo CD5 + B- prolymphocytic leukemia (PLL) presenting at younger age with favourable outcome
title_fullStr De-novo CD5 + B- prolymphocytic leukemia (PLL) presenting at younger age with favourable outcome
title_full_unstemmed De-novo CD5 + B- prolymphocytic leukemia (PLL) presenting at younger age with favourable outcome
title_short De-novo CD5 + B- prolymphocytic leukemia (PLL) presenting at younger age with favourable outcome
title_sort de novo cd5 b prolymphocytic leukemia pll presenting at younger age with favourable outcome
topic CD5+
prolymphocytic leukemia
splenomegaly
prognosis.
url http://www.journalagent.com/z4/download_fulltext.asp?pdir=tjh&plng=eng&un=TJH-54154
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AT rajatkumar denovocd5bprolymphocyticleukemiapllpresentingatyoungeragewithfavourableoutcome
AT rakheekar denovocd5bprolymphocyticleukemiapllpresentingatyoungeragewithfavourableoutcome