B-cell prolymphocytic leukemia [B-PLL] is a neoplasm of B prolymphocytes affecting the peripheral blood, bone marrow and spleen. The principal disease characteristics are massive splenomegaly with absent or minimal peripheral lymphadenopathy and a rapidly rising lymphocyte count. Here, we report a case of B-PLL in a 42 year old male who had come for routine health check up

Fernandes, H

Jayaprakash, C

Pailoor, K

Swethadri, GK

English

Cognitive Sciences ePrint Archive

 Case Report:
B-cell Prolymphocytic Leukemia in a Young Male.
Kirana Pailoor, GK Swethadri, Jayaprakash, Hilda Fernandes,
Department of Pathology, Father Muller Medical College, Mangalore, India.
Address for Correspondence:
Dr. Kirana Pailoor,
Assistant Professor,
Department of Pathology,
Father Muller Medical College,
Mangalore- 575002,
Karnataka, India. 
E-mail: dockirana@yahoo.co.uk
Citation: Pailoor K, Swethadri GK, Jayaprakash, Fernandes H. B-cell Prolymphocytic Leukemia in a Young Male. Online J Health  
Allied Scs. 2011;10(2):25
URL: http://www.ojhas.org/issue38/2011-2-25.htm
Open Access Archives: http://cogprints.org/view/subjects/OJHAS.html and http://openmed.nic.in/view/subjects/ojhas.html
Submitted: May 19, 2011; Accepted: Jul 16, 2011; Published: Jul 30, 2011
Abstract:  B-cell prolymphocytic leukemia [B-PLL] is a neo-
plasm of B prolymphocytes affecting the peripheral blood, bone 
marrow  and  spleen.  The principal  disease  characteristics  are 
massive  splenomegaly  with  absent  or  minimal  peripheral 
lymphadenopathy and a rapidly rising lymphocyte count. Here, 
we report a case of B-PLL in a 42 year old male who had come 
for routine health check up.
Key Words: B-PLL; Prolymphocyte;  Massive splenomegaly; 
Immunophenotyping.
Introduction:
B-cell  prolyphocytic  leukemia  is  an  extremely  rare  disease, 
comprising  approximately  1%  of  lymphocytic  leukemias.1 It 
needs to be differentiated from T-cell prolymphocytic leukemia 
for theurapetic and prognostic purposes. Differentiation can be 
made  by  a  comprehensive  approach  taking  into  account  the 
clinical  features,  the  cell  morphology  and  the 
immunophenotype  of  leukemic  cells.2 B-cell  usually  affects 
elderly  males  over  50  years  of  age.  The  principal  disease 
characteristics  are  massive  splenomegaly  with  absent  or 
minimal  peripheral  lymphadenopathy  and  a  rapidly  rising 
lymphocyte  count.  Immunological  markers  show  a  B  cell 
phenotype such as strong expression of surface IgM+/- IgD and 
Bcell  antigens  such  as  CD19,CD20,CD22,  CD79a  and 
b.1,2 Here, we present the clinico-pathologic features of a case 
of  B-PLL  with  emphasis  on  the  diagnostic  features  and 
differential diagnosis.
Case Report:
A 42 years old man had come to our centre for routine health 
check up. On physical examination, massive splenomegaly and 
minimal  posterior  cervical  lymphadenopathy  were  observed. 
Laboratory investigation revealed hemoglobin of 12.3g/dl, total 
leucocyte  count  of  12x103/cu.mm  and  platelet  count  of 
71x103/cu.mm.  Blood  biochemistry  was  normal  except  for 
mildly elevated lactate dehydrogenase level.  Peripheral blood 
smear showed 70% atypical lymphoid cells [prolymphocytes] 
which  were  medium  sized  with  regular  round  nucleus, 
moderately condensed nuclear chromatin, a prominent  central 
nucleolus and a relatively small  amount  of  faintly basophilic 
cytoplasm [Figure  1].  Bone  marrow  aspirate  smears  showed 
predominantly atypical lymphoid cells with similar morphology 
as  peripheral  blood  cells  [Figure  2].  There  was  marked 
reduction  of  normal  hematopoietic  cells.  On flow cytometric 
immunophenotyping, the atypical lymphoid cells were positive 
for CD19, CD20, CD22, CD23, CD45 and negative for CD2, 
CD3, CD4, CD5 and ZAP-70. Taking into account the clinical, 
morphological and immunophenotypic features, a diagnosis of 
B-PLL  was  made.  Patient  was  treated  with  Cytoxan, 
Adriamycin,  Vincristine  and Prednisolone  regimen.  A repeat 
count was done 6 months after chemotherapy. It showed a total  
leucocyte  count  of  9,000  cells/cu.mm  with  the  absence  of 
prolymphocytes.  The  patient  is  doing  well,  20  months  after 
diagnosis and therapy.
Figure 1: Peripheral blood smear showing prolymphocytes 
which have moderately condensed nuclear chromatin and a 
prominent nucleolus [Leishmanx100X]
Figure 2: Bone marrow aspirate smear showing numerous 
prolymphocytes in diffuse sheets [Leishmanx 100X].
1
This work is licensed under a
Creative Commons Attribution-
No Derivative Works 2.5 India License
Online Journal of Health and Allied Sciences
Peer Reviewed, Open Access, Free Online Journal
Published Quarterly :  Mangalore, South India : ISSN 0972-5997
Volume 10, Issue 2; April-June 2011
Figure 3: Dot plots showing immunoreactivity pattern of 
prolymphocytes in B-cell prolymphocytic leukemia
Discussion:
Prolymphocytic  leukemia  was  first  described  in  1974  by 
Galton, as a rare variant of chronic lymphocytic leukemia. 3-6 It 
is a rare chronic chronic lymphoproliferative disorder that in-
cludes two subtypes, B cell and T cell, each with its own dis-
tinct  clinical,  laboratory  and  pathological  features.4 Most  pa-
tients are over  60-year  old,  with a median age of  65-69 and 
similar male: female distribution.1
The key features of B-PLL are massive splenomegaly with ab-
sent  or  minimal  peripheral  lymphadenopathy  and  a  rapidly 
rising lymphocyte count, usually over 100x109/L. Anemia and 
thrombocytopenia  are  seen  in  about  half  the  number  of 
cases.1,3,7 T-PLL  patients  usually  present  with  generalized 
lymphadenopathy and skin lesions.1 Morphologically,  majority 
(>55%  and  usually  >90%)  of  the  circulating  cells  are  pro-
lymphocytes;  that is,  medium sized cells (twice the size of a 
small  lymphocyte),  with  a  round  nucleus,  moderately  con-
densed nuclear chromatin, a prominent central nucleolus and a 
relatively small amount of faintly basophilic cytoplasm. In con-
trast, the cells of T-PLL are small to medium-sized lymphoid 
cells with agranular basophilic cytoplasm,and have a markedly 
irregular  nuclei.1,2,8  Typically,  B-PLL  is  differentiated  from 
chronic  lymphocytic  leukemia  [CLL] and CLL/PL with  55% 
prolymphocytes  being  a  key  criteria.  B  prolymphocytes  are 
more  uniform and  have  a  more  regular  nuclear  outline  than 
those of CLL/PL. Also, CLL/PL have 10-55% prolymphocytes 
and a variable number of plasmacytoid lymphocytes.4 The dis-
tinction  from  Hairy  cell  leukemia  [HCL]  variant  is  based 
mainly on the appearances of the cytoplasm. In HCL variant, 
the cytoplasm is more abundant and distinctly villous, whereas 
in B-PLL it is generally smooth.1,2
The bone marrow shows interstitial or nodular infiltrate of nuc-
leolated  cells  with  an  intertrabecular  distribution.1,8 In  the 
present case, the bone marrow was diffusely infiltrated by pro-
lymphocytes with marked suppression of normal hematopoietic 
elements. The cells of B-PLL strongly express B-cell antigens 
such as CD19, CD20, CD22, CD79a and CD79b. In this case, 
the patient strongly expressed CD19, CD20, CD22, CD23 and 
CD45. The cells of T-PLL express CD2, CD3 and CD7. The re-
ported median survival  is 3 to 4 years for patients with pro-
lymphocytic leukemia and 8 years for those with CLL. Patients 
with  T-PLL have  even  poorer  prognosis  than those  with  B-
PLL.1,2,8 B-PLL responds  poorly  o  therapies  for  CLL and  T-
PLL.  Hence,  typing  PLL is  very critical  for  both  therapeut-
ic and prognostic purposes.
In conclusion, a precise diagnosis by means of a comprehensive 
approach involving clinical, morphological and immunopheno-
typic features are extremely critical in this era for patient man-
agement and prognostication. Also, this case illustrates the im-
portance of immunophenotyping as an adjunct to morphology 
in the diagnosis of chronic lymphoproliferative disorders.
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