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Simultaneous Diagnosis of Hairy Cell Leukemia and Chronic Lymphocytic Leukemia/Small Lymphocytic Lymphoma

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Simultaneous Diagnosis of Hairy Cell Leukemia and Chronic Lymphocytic Leukemia/Small Lymphocytic Lymphoma Leukemia (2002) 16, 1454–1459 2002 Nature Publishing Group All rights reserved 0887-6924/02 $25.00 www.nature.com/leu Simultaneous diagnosis of hairy cell leukemia and chronic lymphocytic leukemia/small lymphocytic lymphoma: a frequent association? E Gine´1, F Bosch1, N Villamor2, M Rozman2, D Colomer2,ALo´pez-Guillermo1, E Campo2 andE Montserrat 1 1Institute of Hematology and Oncology, Department of Hematology, Hospital Cli´nic, Institut d’Investigacions Biome`diques August Pi i Sunyer (IDIBAPS), Barcelona, Spain; and 2Hematopathology Unit, Hospital Clı´nic, Institut d’Investigacions, Biome`diques August Pi i Sunyer (IDIBAPS), Barcelona, Spain The association of hairy cell leukemia (HCL) with other neo- phomas are the most frequently hematological malignancies plasms, mainly non-Hodgkin’s lymphomas, is well known. reported.10,15,16 The association of HCL andchronic lympho- However, the simultaneous diagnosis of HCL and chronic lym- phocytic leukemia/small lymphocytic lymphoma (CLL/SLL) is cytic leukemia/small lymphocytic lymphoma (CLL/SLL) is con- rare, with only few cases of such an association having been sidered to be exceedingly rare. Thus, in an extensive com- reported. We describe three patients with a well-characterized puter-assistedsearch of the literature, only one case of HCL in whom a CLL/SLL population was detected. Of note, transformation of CLL/SLL into HCL,17 one case of HCL diag- these cases represent a significant proportion (11.5%; 95% CI: nosedyears after the diagnosisof CLL/SLL, 18 andsome cases 0% to 24%) of the total number of HCL cases diagnosed in our of lymphoproliferative disorders with intermediate features institution during the same period of time. All three patients 19–21 were treated with deoxycoformycin. They achieved a complete between HCL andCLL/SLL have been reported. More- response of the HCL, whereas the CLL/SLL population per- over, in the four largest studies assessing the incidence of sisted in all cases. The immunoglobulin gene rearrangement secondneoplasms in patients with HCL, includinga total of analysis, in two informative cases, suggested that the HCL and 1433 patients, no case of CLL/SLL has been described.12–15 CLL/SLL populations arose from different B cell clones. This Since 1991, all the cases of HCL diagnosed in our center study indicates that the association of HCL and CLL/SLL might were systematically screened for the presence of additional be much more frequent than previously recognized. Therefore, a large panel of monoclonal antibodies, including those neces- B cell populations using an extensive panel of monoclonal sary to detect CLL/SLL, should be employed when studying antibodies. Using this approach, three patients with the simul- patients with HCL. taneous existence of a HCL and CLL/SLL were identified. The Leukemia (2002) 16, 1454–1459. doi:10.1038/sj.leu.2402553 aim of the present study is to describe the clinical, morpho- Keywords: hairy cell leukemia; chronic lymphocytic leukemia; logic, immunophenotypic andmolecular features of these small lymphocytic lymphoma; immunoglobulin gene rearrangement cases andto comment on the association between HCL and CLL/SLL. Introduction Patients and methods Hairy cell leukemia (HCL) is an infrequent chronic lympho- proliferative disorder (2% of all adult leukemias), initially Patients and treatment described by Bouroncle et al,1 which is characterizedby the infiltration of the bone marrow andthe spleen by mature lym- Between 1991 and1997, 26 consecutive patients were diag- phocytes with typical cytoplasmic projections. Pancytopenia, nosed with HCL in our institution according to standard clini- splenomegaly, peripheral involvement andopportunistic cal, morphological andphenotypic criteria. 22 During the same infections are common clinical manifestations of HCL. In most periodof time, 270 patients were diagnosedwith CLL/SLL. 23,24 instances the prognosis of patients with HCL is good, with an In all cases the immunophenotype of the neoplasic cells was overall survival of around85% at 8 years from diagnosis. 2–4 studied by cytofluorometry as described below. Phenotypic Hairy cells strongly express CD20, CD22 andmonoclonal andmolecular analysis from peripheral bloodandbone mar- surface immunoglobulins, together with the distinctive row samples were performed at diagnosis and during the expression of CD25, CD11c, FMC7 andCD103. Trisomy 5, course of the disease. Most patients with HCL were treated structural abnormalities involving the pericentromeric regions with deoxycoformycin (DCF) at a dose of 4 mg/m2 every 2 of chromosomes 5 and2, 1q42 abnormalities, and14q alter- weeks, until maximal response or for a minimum of six cycles ations are frequently foundin patients with HCL, 5 but there is in case of treatment failure. Response was evaluatedaccord- no recurrent chromosomal aberration typical of this disease. ing to conventional criteria.3,25 Cyclin D1 gene overexpression, in absence of t(11;14), is foundin the majority of cases. 6 Although the normal counter- part of the hairy cells has not yet been identified, this disease Immunophenotypic analysis is considered to derive from clonal post-germinal center B cells, basedon the assessment of the immunoglobulin gene Peripheral bloodor bone marrow samples were usedto assess rearrangements7 andthe analysis of somatic hypermutation of the membrane phenotype of lymphocytes. Briefly, 5 × 105 VH genes.8 cells were incubated(15 min) with the appropriate amounts The occurrence of secondneoplasms in patients with HCL of monoclonal antibodies using double or triple color combi- is a well-known fact.9–15 Among them, non-Hodgkin’s lym- nations, followedby redcell lyses using FACS lysing solution. Stainedsamples were acquiredin a FACScan flow cytometer (Becton Dickinson Immunocytometry Systems, San Jose, CA, Correspondence: F Bosch, Department of Hematology, Hospital Clı´nic Villarroel no. 170, 08036-Barcelona, Spain; Fax: 34-93- USA), andanalyzedusing the Paint-a Gate Pro software 2275428 (Becton Dickinson). The reactivity against T cell antigens (at Received4 September 2001; accepted27 February 2002 least CD3, CD4, CD5, CD8), B cell antigens (at least CD19, HCL and CLL E Gine´ et al 1455 CD22, CD20, kappa andlambdalight chain), andother anti- Patient 1 gens (at least CD11c, CD23, CD25, CD103) was analyzed. When required, immunohistochemical analysis was perfor- A 59-year-oldman was referredto our center with a palpable medin bone marrow biopsies using CD79a andCD5 anti- splenomegaly andretroperitoneal lymph nodes.The WBC bodies in order to ascertain CLL/SLL infiltrates. count was 9.53 × 109/l (60% lymphocytes, the great majority with a hairy cell appearance). The bone marrow aspirate showedthe presence of 54% hairy cells. A bone marrow Immunoglobulin gene rearrangement biopsy revealedinterstitial infiltration by two distinctlympho- cyte subtypes: (1) medium-sized lymphocytes, with a round High molecular weight DNA was extractedfrom mononuclear nuclear profile andfine chromatin, widelyseparatedfrom cells isolated by Ficoll gradient according to standard pro- each other by their cytoplasm, (2) small lymphocytes, with cedures.26 Consensus primers for the framework 3 (FR3) por- roundnucleus andmature chromatin; there was a marked tion of the variable region andof the 3 Ј portion of the joining increase in reticulin fibers andthe small lymphocyte infiltrates region of the immunoglobulin heavy chain gene, basedon were positive for CD79a andCD5 (Figure 1). The immuno- 26 phenotype of peripheral bloodshowed40% of lymphocytes previously publishedstudies, were usedto amplify the + + + CDR3 region. The reaction mixture of PCR contained30 with a HCL phenotype (CD19 ,CD25, CD11c , andlambda chain restriction), whereas 12% of lymphocytes hada CLL/SLL pmols of each primer, 200 mM of each dNTPs, 2.5 mM MgCl2 + + + +/− and1.25 U of High ExpandFidelity(Roche Diagnostics, phenotype (CD19 ,CD5,CD23 ,CD22 , andlambdachain Mannheim, Germany) and1 ␮g of template. The reaction con- restriction) (Figure 2-1). sistedof 40 cycles of a denaturationstep at 94 °C for 1 min The patient was treatedwith DCF. After treatment, a com- (10 min first cycle), an annealing step at 55°C for 2 min and plete response was documented. However, both hairy cell an extension step for 1 min at 72°C (10 min last cycle). Ampli- andCLL/SLL immunophenotypic markers were observedin fiedDNA was electrophoresedon an acrylamide-bis(19:1) the peripheral blood. Six years after diagnosis the patient gel 5% and was stained using ethidium bromide and vis- presentedwith clinical manifestations relatedto the CLL/SLL, × 9 ualizedunderUV. lymphocytosis (20 10 /l), thrombocytopenia anda palpable splenomegaly. The presence of both B cell lymphocytic popu- lations was again confirmedby morphologic andimmuno- phenotyping methods (53% of CLL/SLL lymphocytes and 2% Literature search of hairy cells in the peripheral blood). After completing treat- ment with 2-chlorodeoxyadenosine, the hematological para- A literature search of the periodJanuary 1966 to July 2001 meters normalizedandthe immunophenotypic analysis was performedusing the Pub MedService, National Library detected only the presence of the CLL/SLL population. The of Medicine – National Center for Biotechnology information PCR analysis performedat that time showeda single heavy (www.ncbi.nlm.nih.gov/) USA. Key words consulted were: chain immunoglobulin gene (JH) band. hairy cell leukemia andchronic lymphocytic leukemia, hairy cell leukemia, hairy cell leukemia andtreatment. Patient 2 Results A 49-year-oldman was
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