CASE REPORT
Concurrent occurrence of two B chronic lymphoproliferative
disorders—a rare phenomenon posing diagnostic
dilemma for hematopathologist
Ashish Gupta
1
&
Shashi Bansal
1
&
Richa Sharma
2
Received: 22 June 2017 /Accepted: 8 November 2017 /Published online: 17 November 2017
#
Springer-Verlag GmbH Germany, part of Springer Nature 2017
Abstract CLL and HCL are distinct mature B cell neoplasm with
characteristic clinical, haematological and immunophenotypic
profiles. Their synchronous or metachronous occurrence has been
reported in the past but with a paucity of literature. These cases
present a challenge from diagnostic as well as therapeutic point of
view for hematopathologist and treating physician in common.
We present an interesting case with synchronous presentation of
HCL with CLL.
Keywords Chronic lymphocyticleukaemia(CLL)
.
Hairy cell
leukaemia (HCL),
.
Chronic lymphoproliferative disorder
(CLPD)
Introduction
CLL and HCL are distinct chronic B cell lymphoproliferative
disorders with specific clinical presentation, morphology and
immunophenotypic expression.
CLL is usually diagnosed with peripheral blood presenting
with monoclonal B cell lymphocytosis (> 5 × 10
9
/L) in a
background of smudge cells [1]. The lymphocytes usually pre-
sents with a monotonous population of small mature lympho-
cytes with clumped chromatin and scanty cytoplasm in peripher-
al blood admixed with prolymphocytes(usually < 10%). The
cells typically express CD5, CD23, CD200, dim CD20, dim
monotypic surface immunoglobulin with negative expression
of FMC 7 and CD10.There is a co-expression of CD5 and
CD23 by CLL cells. Clinically, patients usually present at a me-
dian age of 65 years with variable symptoms as fatigue, infec-
tions, autoimmune hemolytic anaemia, frequent lymphadenop-
athy with only a minority of cases presenting with
hepatosplenomegaly. According to international workshop on
CLL (IWCLL), lymphocytosis should be present for at least
3 months and permits diagnosis of CLL with lower lymphocyte
counts in patients presenting with cytopoenia or disease associ-
ated symptoms [1]. The minimal criterion for bone marrow in-
volvement as described by IWCLL includes > 30% lymphoid
cells to be present at time of presentation [1]. The non-leukemic
phase of CLL is termed as SLL(small lymphocytic leukaemia)
which according to IWCLL is defined as patients presenting with
lymphadenopathy, absence of cytopoenias due to marrow infil-
tration by CLL/SLL and < 5 × 10
9
peripheral blood monoclonal
B lymphocytes [1]. SLL cases have tissue morphology and
immunophenotype of CLL.
On the other hand, HCL is an indolent B cell neoplasm with a
median age of presentation being 50 years. The clinical spectrum
of HCL differs from CLL with most patients presenting with
pancytopoenia (monocytopoenia) and splenomegaly [2]. The
other presenting symptoms include fatigue, left upper quadrant
pain, fever and bleeding. Lymphadenopathy is unusual in HCL
cases at presentation however advanced cases may present with
lymph node infiltration with variable interfollicular/ paracortical
infiltration [2]. Most cases present with circulating neoplastic
lymphoid cells having typical Bhairy^ cytoplasmic projections
(hairy cells). The hairy cells are small to medium sized have oval
* Richa Sharma
rrichasharmaa@gmail.com
Ashish Gupta
ashishgupta.path@gmail.com
Shashi Bansal
drshashi12@gmail.com
1
Department of Hematopathology and Molecular Biology, Bhagwan
Mahveer Cancer Hospital, Adress for correspondence: Flat no
203,Plot no C 103,Sona Avinash Enclave, Bapu Nagar,
Jaipur, Rajasthan, India
2
Department of Hematopathology, Bhagwan Mahveer Cancer
Hospital, Jaipur, Rajasthan, India
J Hematopathol (2017) 10:129–132
https://doi.org/10.1007/s12308-017-0308-9
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