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1991 77: 1617-1619
CD5+, CD11c+, CD20+ hairy cell leukemia [letter; comment]
PS Heimann, JW Vardiman, W Stock, LC Platanias and HM Golomb
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CORRESPONDENCE
1617
CD5+, CDllc’, CD20’ HAIRY CELL LEUKEMIA
To the Editor:
In their recent report in Bloorl, Wormsley et a1 concluded that
CDllc+,CD5+ B-cell chronic leukemias are subgroups of chronic
lymphocytic leukemia (CLL) and prolymphocytic leukemia (PLL)
rather than of hairy cell leukemia (HCL).’ We do not disagree that
chronic B-cell lymphoproliferative disorders other than HCL may
demonstrate CDllc’, and have previouslyreported on such cases;
but we do have reservations regarding the implication that HCL
should be excluded as a diagnostic possibility when the leukemic
cells possess the CD5 antigen in addition to CDllc and B-cell
markers. We have recently encountered a case of HCL with CD5+,
CDllc+, CD20+ leukemic cells, which points out the need for
cautious interpretation of immune surface markers in the context
of clinical, morphologic, and cytochemicalfindings.
The patient is an 85-year-old retired physician who had been in
excellent health until 1985, when moderate splenomegaly was
discovered on a routine physical examination at another institution. A CBC performed at that time showed a white blood cell
count (wbc) of 6,70O/kL with 9% neutrophils and 37% “atypical
lymphocytes,” a hemoglobin (Hgb) level of 12.3 g/dL, and a
platelet count of 177,OOO/pL. A bone marrow biopsy was performed and a tentative diagnosis of HCL was made. The patient
was followed with no medical intervention, but has had routine
physical examinationsevery 6 months.
The patient was referred to the University of Chicago for
additional evaluation in July 1990. Moderate splenomegaly was
present, but he had no lymphadenopathy or hepatomegaly. The
wbc was 9,10O/p,L with 52% “hairy cells,” 33% small lymphocytes,
12% neutrophils, and 3% monocytes. The Hgb was 11.8 g/dL and
the platelet count was 11O,OOO/pL. The “hairy cells” were large (12
to 15 p”, and had round to oval, eccentrically placed nuclei with
delicate chromatin and inconspicuous nucleoli, as well as abundant
cytoplasm with villous margins (Fig 1). More than one half of the
Fig 1. This photomicrographillustratesthe featuresof the neoplastic cells in the peripheral blood smear of the patient (Wright‘s stain;
original magnification x 1,000).
hairy cells had tartrate-resistant acid phosphatase reactivity. A
bone marrow biopsy specimen was only 10% cellular, but nearly
one half of the marrow cells had histologic features consistent with
those of HCL. Immunophenotypic studies of peripheral-blood
mononuclear cells were performed by flow cytometry as well as by
the alkaline-phosphatase-anti-alkalinephosphatase (APAAP)technique on peripheral blood smears. The immunophenotype of the
neoplastic cells was CD5+, CDllc+, CD19+, CD20+, and CD25+.
This immunophenotypewas confirmed by examination of the hairy
cells on APAAP-stained peripheral blood smears. Dual staining by
flow cytometry showed that CD5 and CD20 as well as CD19 and
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CORRESPONDENCE
1618
C D l l c were coexpressed on the neoplastic cells. Therefore, the
leukemic cells from our patient were CD5+, CDllc', CD20+, but
the clinical, morphologic, and cytochemical findings were those of
HCL.
Although we agree with Wormsely et a1 that additional prospective studies of CDllc', CD5+ B-cell leukemias are required to
show whether they are a clinically significant subgroup of lymphocytic neoplasms, we consider our case as demonstrating that this
phenotype is not necessarily confined to B-CLL or B-PLL, but may,
in fact, be found in HCL as well. Until additional information
regarding such unusual cases is acquired, we believe that it is
prudent to interpret monoclonal antibody studies cautiously, and
to rely on current clinical and morphologic findings for diagnostic
purposes.
PRISCILLA S. HEIMANN
JAMES W. VARDIMAN
Department ofPathology
WENDY STOCK
LEONIDAS C. PLATANIAS
HARVEY M. GOLOMB
Department of Medicine
University of Chicago Hospitals
Pntzker School of Medicine
Chicago, IL
REFERENCES
1. Wormsley SB, Baird SM, Gadol N, Rai KR, Sobol RE:
Characteristics of CDllc' CD5' B-cell leukemias and the identification of novel peripheral blood B-cell subsets with chronic
lymphoid leukemia immunophenotypes. Blood 76:123,1990
2. Vardiman JW, Gilewski TA, Ratain MJ, Bitter MA, Bradlow
BA, Golomb HM: Evaluation of Leu-M5 (CDllc) in hairy cell
leukemia by the alkaline phosphatase anti-alkaline phosphatase
technique. Am J Clin Pathol90:250,1988
RESPONSE
We agree that the results of immunophenotypic analyses should
be interpreted in the context of conventional morphologic, cytochemical, and clinical diagnostic criteria. However, it is well
recognized that the diagnosis of a small proportion of chronic
B-cell leukemias will be problematic owing to the considerable
heterogeneity of these diseases and their overlap of morphologic,
immunophenotypic, and clinical features. These difficulties are
reflected by the case of CDS+, CDllc+ hairy cell leukemia (HCL)
described by Heimann et al, our series of CD5+, CDllc' chronic
lymphocytic leukemias (CLL),' and the recent report by Hanson et
al' describing CDllc+ B-cell leukemias with CLL morphology and
clinical characteristics associated with HCL.
It should be noted that the case described by Heimann et a1 is
uncommon, as CD5 is rarely expressed by leukemias with HCL
m~rphology.~
In our series of 119 patients with CD5+ B-cell
immunophenotypes, 26 (22%) were CDllc'and all CDllc' cases
with peripheral blood smears available for review (n = 14) had
CLL morphology.' Many of our cases with CD5+, CDllc' B-cell
immunophenotypes were characterized by the presence of large
lymphocytes with abundant cytoplasm.' Similar large lymphocyte
morphologic features were reported by Hanson et alzin their study
of CDllc' B-cell leukemias with CLL morphology. While our
CDllc+ cases shared morphologic characteristics with those reported by Hanson et al, the presenting clinical features of our cases
were different in some respects. In our patients, lymphadenopathy
was common (9 of 14 cases) and splenomegaly was present in 6 of
14 patients.' In the patients studied by Hanson, et al: generalized
lymphadenopathy was uncommon (1 of 14 cases) and splenomegaly
was frequently obselved (11 of 14 cases). All of the patients in our
study had CD5' immunophenotypes,' whereas only 7 of 14 patients
evaluated by Hanson et a1 were CDS'? As the number of patients
evaluated in these studies was small, definition of the morphologic,
immunophenotypic, and clinical characteristics of CDllc' B-cell
leukemias with CLL morphology will require assessment of a larger
number of patients. Nevertheless, our results and those of others1.2,4.S
indicate
'
that CDllc is not restricted to cases with HCL
morphology and that CDllc may be expressed by B-cell leukemias
with CLL morphology.',' Prospective studies of larger numbers of
patients will be necessary to determine whether CDllc' B-cell
leukemias with CLL morphology represent a distinct pathologic
entity of clinical significance.
SUSAN B. WORMSLEY
Cytometncs, Inc
Division of Specialty Laboratories, Inc.
San Diego, CA
STEPHEN M. BAIRD
Departments of Pathology and the Cancer Center
University of Califomia
VeteransAdministration Medical Center
San Diego, CA
NANCY GADOL
Becton Dickinson
Mountain Wew, CA
KANTI R. RAI
Long Island Jewish Hospital
New York, NY
ROBERT E. SOBOL
Cancer Center
University of Califomia
Mercy Hospital Medical Center
San Diego, CA
REFERENCES
1. Wormsley SB, Baird SM, Gadol N, Rai KR, Sobol RE:
Characteristics of CDllc'CDS' chronic B-cell leukemias and the
identification of novel peripheral blood B-cells subsets with chronic
lymphoid leukemia immunophenotypes. Blood 76:123,1990
2. Hanson CA, Gribbin TE, Schnitzer B, Schlegelmilch JA,
Mitchell BS, Stoolman LM: C D l l c (LEU-M5) expression charac-
terizes a B-cell chronic lymphoproliferative disorder with features
of both chronic lymphocytic leukemia and hairy cell leukemia.
Blood 76:2360,1990
3. Foon KA, Todd R F 111: Immunologic classification of leukemia and lymphoma. Blood 68:1,1987
4. Vardiman JW, Gilewski TA, Ratain MJ, Bitter MA, Bradlow
From www.bloodjournal.org by guest on December 4, 2014. For personal use only.
CORRESPONDENCE
BA, Golomb HM: Evaluation of Leu-M5 (CDllc) in hairy cell
leukemia by the alkaline phosphatase antialkaline phosphatase
technique. Am J Clin Pathol90:250,1988
1619
5. Sheibani K, Sohn CC, Burke JS, Winberg CD, Wu AM,
Rappaport H Monocytoid B-cell lymphoma: A novel B-cell
neoplasm. Am J PathoI 124:310,1986