We describe a series of 5 patients in whom the application of consensus criteria for the diagnosis of chronic lymphocytic leukemia (CLL) led to a revised diagnosis. These patients had been recruited in a wider study describing the occurrence of  CLL in Kenya. This case series highlights some of the pitfalls of making a diagnosis of CLL without incorporating all criteria

Malkit, Riyat

Mulwa-Babu, E.

Parech, D.

E. Mulwa-Babu

M. Riyat

D. Paresh

Crossref

Journal africain du cancer / African Journal of Cancer

Applying consensus criteria to the evaluation of patients with suspected chronic lymphocytic leukemia/small lymphocytic lymphoma

eCommons@AKU

eCommons@AKUPathology, East Africa Medical College, East AfricaJanuary 2014Applying consensus criteria to the evaluation ofpatients with suspected chronic lymphocyticleukemia/small lymphocytic lymphomaE. Mulwa-BabuAga Khan UniversityRiyat MalkitAga Khan University, riyat.malkit@aku.eduD. ParechUniversity of NairobiFollow this and additional works at: http://ecommons.aku.edu/eastafrica_fhs_mc_patholPart of the Pathology CommonsRecommended CitationMulwa-Babu, E., Malkit, R., Parech, D. (2014). Applying consensus criteria to the evaluation of patients with suspected chroniclymphocytic leukemia/small lymphocytic lymphoma. African Journal of Cancer, 6(4), 215-217.Available at: http://ecommons.aku.edu/eastafrica_fhs_mc_pathol/127ORIGINAL ARTICLE / ARTICLE ORIGINALApplying Consensus Criteria to the Evaluation of Patients withSuspected Chronic Lymphocytic Leukemia/Small LymphocyticLymphomaApplication des critères de consensus pour le diagnostic de la leucémie lymphoïde chroniqueE. Mulwa-Babu · D. Paresh · M. RiyatReceived: 27 March 2014; Accepted: 12 May 2014© Lavoisier SAS 2014Abstract We describe a series of 5 patients in whom theapplication of consensus criteria for the diagnosis of chroniclymphocytic leukemia (CLL) led to a revised diagnosis.These patients had been recruited in a wider study describingthe occurrence of CLL in Kenya. This case series highlightssome of the pitfalls of making a diagnosis of CLL withoutincorporating all criteria.Keywords Pitfalls · CLL · DiagnosisRésumé Nous décrivons une série de cinq patients chez les-quels l’application des critères de consensus pour le diagnos-tic de la leucémie lymphoïde chronique (LLC) a conduit àune révision du diagnostic. Ces patients ont été recrutés dansune étude plus large décrivant la LLC au Kenya. Cette sériede cas met en lumière les pièges dans le diagnostic de la LLCquand tous les critères n’y sont pas intégrés.Mots clés Pièges · LLC · DiagnosticIntroductionThe laboratory diagnosis of lymphoma has evolved over theyears from being based on morphology alone to the inclu-sion of immunophenotyping and molecular data [1–3]. Spe-cifically, the diagnosis of chronic lymphocytic leukemia(CLL) according to the World Health Organization (WHO)and the International Workshop on CLL (IWCLL) requiresthe following [3,4]:• Duration: Persistent lymphocytosis ≥3 months;• Counts: Monoclonal B cell count ≥5 × 109/L when thereis lymphocytosis only;• Morphology: Consistent morphology as defined by theFrench-American-British (FAB) classification [5];• Immunophenotype: The prototype is co-expression of CD5and CD23 in monoclonal B cells with dim expression ofsurface immunoglobulin (sIg) and absent/dim CD79b (orCD22), and the absence of expression of FMC7. The scor-ing system described by Matutes et al. can help distinguishCLL from other non-Hodgkin lymphomas (NHL) on thebasis of immunophenotype [6,7].Small lymphocytic lymphoma (SLL) and CLL are differ-ent presentations of the same disease [4].Materials and methodsPatients were recruited as part of a wider study describingthe occurrence of CLL in Kenya [8]. Morphological evalua-tion was by FAB criteria. Results of immunophenotypingwere interpreted based on the scoring system described byMatutes et al. [6,7].ResultsCriteria: Duration and countsCase 1: Case of essential thrombocythemiaA 62-year-old man was referred to Aga Khan UniversityHospital, Nairobi (AKUH,N) for bone marrow evaluationwith a new diagnosis of CLL. The basis for this diagnosisE. Mulwa-Babu (*) · M. RiyatDepartment of pathology, Aga Khan University Hospital,Nairobi, Kenyae-mail : evenduku@yahoo.co.ukD. PareshUniversity of Nairobi, Uhuru Highway, Nairobi 00100, KenyaJ. Afr. Cancer (2014) 6:215-217DOI 10.1007/s12558-014-0332-ywas uncertain as records were not accessible. However,history was significant for erythromelalgia and persistentthrombocytosis, and the patient had not received any therapyfor CLL. Physical examination was noteworthy for the pres-ence of moderate splenomegaly. Interestingly, the patient didnot have lymphocytosis. Subsequent blood counts were alsonegative for lymphocytosis. There was no evidence of reac-tive thrombocytosis and bone marrow evaluation done wasconsistent with essential thrombocythemia (ET).Criterion: MorphologyCase 2: Case of HCLA 61-year-old man was seen at AKUH,N seeking a secondmedical opinion. He had been on follow-up as a case ofCLL. Clinical history was remarkable for recurrent anemia,thrombocytopenia, and non-response to chlorambucil ther-apy. Physical examination was notable for pallor and mas-sive splenomegaly. Morphology was, however, inconsistentwith CLL as per the FAB criteria (Fig. 1). Immunophenotyp-ing confirmed the suspected diagnosis of hairy cell leukemia(HCL). The immunophenotype was CD5−, CD23 border-line, bright sIg expression with lambda restriction, CD11c/CD22 co-expression with bright CD22 expression, FMC7positive, and CD103+.Criterion: ImmunophenotypeCase 3–5: Cases of diagnostic uncertaintyCase 3 was a 50-year-old female patient who had initiallybeen diagnosed as CLL on morphological evaluation of abone marrow aspirate. However, on immunophenotypingof a peripheral blood sample with leukocytosis, most cellswere weak CD45 positive and suspicious for blasts. Thecells were CD19 positive and lacked surface immunoglobu-lin expression. Further immunophenotyping confirmed thediagnosis of acute lymphoblastic leukemia (ALL) and anamended report was issued (Fig. 2).Cases 4 and 5 were elderly male patients with sustainedlymphocytosis. However, morphological evaluation was notconclusive due to the presence of cleaved cells and moderateamounts of cytoplasm (Fig. 3). A diagnosis of a chroniclymphoproliferative disorder (CLPD) was given and CLL/SLL and follicular lymphoma were suggested as possibili-ties. The immunophenotype for both, however, scored 5/5which is highly specific for CLL [6]. CD 10 was negativefor both.DiscussionThe incorporation of relevant criteria for diagnosis of varioushematological malignancies is not merely of academicFig. 1 Peripheral blood smear for Case 2 showing hairy cellsFig. 2 Peripheral blood smear for Case 3Fig. 3 Representative field of peripheral smear findings in Cases4 and 5216 J. Afr. Cancer (2014) 6:215-217interest. It results in appropriate classification, prognostica-tion, and management of patients. The management princi-ples of ALL, HLL, and ET differ from those of CLL/SLL[4,9]. Altering the diagnosis for the patients described in thisseries, therefore, optimized their management.While sub-Saharan Africa is plagued by resource limita-tions [10], incorporation of requisite criteria is vital whencircumstances permit an optimal evaluation. However,even in the absence of access to more specialized tests likeimmunophenotyping, clinical correlation using simple crite-ria like the presence and persistence of lymphocytosis can goa long way in distinguishing transient from clonal causes oflymphocytosis. Building expertise in microscopy would alsogo a long way in the recognition of classic features such asthose seen in Case 2.ConclusionThe application of the WHO criteria for the diagnosis oflymphomas including CLL/SLL results in better definitionof disease entities. Where resources allow, morphologicalevaluation should be supported by the relevant diagnostictests.Acknowledgments Dr. Etabale Mbayah for his assistancewith translating the abstract into French.Conflict of interest: E. Mulwa-Babu, D. Paresh, andM. Riyat have no conflicts of interest to declare.Ethical standards: Approval for the study was obtainedfrom the institution’s research and ethics committees; con-sent was obtained from the patients; results were conveyedto attending physicians.References1. Gall EA, Mallory TB (1942) Malignant lymphoma: A clinico-pathologic survey of 618 cases. Am J Pathol 18:381–4292. Hicks EB, Rappaport H, Winter WJ (1956) Follicular lymphoma;a re-evaluation of its position in the scheme of malignant lym-phoma, based on a survey of 253 cases. Cancer 9:792–8213. Swerdlow SH, Campo E, Harris NL, et al (2008) WHO classifi-cation of tumours of haematopoietic and lymphoid tissues, 4thed. IARC, Lyon, pp 180-24. Hallek M, Cheson BD, Catovsky D, et al (2008) Guidelines forthe diagnosis and treatment of chronic lymphocytic leukemia: areport from the International Workshop on Chronic LymphocyticLeukemia updating the National Cancer Institute-Working Group1996 guidelines. Blood 111:5446–565. Bennett JM, Catovsky D, Daniel MT, et al (1989) Proposals forthe classification of chronic (mature) B and T lymphoid leukae-mias. French-American-British (FAB) Cooperative Group. J ClinPathol 42:567–846. Matutes E, Owusu-Ankomah K, Morilla R, et al (1994) Theimmunological profile of B-cell disorders and proposal of a scor-ing system for the diagnosis of CLL. Leukemia 8:1640–57. Moreau EJ, Matutes E, A’Hern RP, et al (1997) Improvement ofthe chronic lymphocytic leukemia scoring system with the mono-clonal antibody SN8 (CD79b). Am J Clin Pathol 108:378–828. Mulwa-Babu E, Paresh D, Riyat M (2013) Chronic lymphocyticleukemia in Kenya: an immunophenotypic and clinicopathologicstudy. Afr J Cancer 5:192–79. Beer P, Erber WN, Campbell PJ, Green AR (2011) How I treatessential thrombocythemia. Blood 117:1472–8210. Naresh KN, Raphael M, Ayers L, et al (2011) Lymphomas insub-Saharan Africa - what can we learn and how can we help inimproving diagnosis, managing patients and fostering transla-tional research? Br J Haematol 154:696–703J. Afr. Cancer (2014) 6:215-217 217

http://ecommons.aku.edu/cgi/viewcontent.cgi?article=1141&context=eastafrica_fhs_mc_pathol

Applying consensus criteria to the evaluation of patients with suspected chronic lymphocytic leukemia/small lymphocytic lymphoma

Abstract

Similar works

Full text

Available Versions

Crossref

eCommons@AKU