Cuesta, B. (Braulia)

Panizo, C. (Carlos)

Rocha, E. (Eduardo)

Universidad de Navarra

Primary plasma cell leukemia with unusual morphology and complex karyotype

Dadun, University of Navarra

Primary plasma cell leukemia with unusual morphology and complexkaryotypeCARLOS PANIZO, BRAULIA CUESTA, EDUARDO ROCHADepartment of Hematology, University Clinic of Navarra, School of Medicine, University of Navarra, SpainHaematologica 1998; 83:849-850 the irreplaceable imageCorrespondence: Eduardo Rocha, M.D., Department of Hematology,University Clinic, Avda. Pío XII s/n, Pamplona 31008, Spain.Phone: international +34-948-296397 • Fax: international +34-948-296500 • E-mail: erocha@cun.esistics seem to be related to a worse prognosis thanthe usual well-differentiated myeloma and give rise toa differential diagnosis of myeloid or monocyticleukemia, lymphoma or nonhematologic malignan-cy.2,5,6 Although the expression of CD13 and CD33antigens on normal plasma cells has been reported,the presence of the myeloid antigen CD13 on myelo-ma cells is an uncommon finding. Since CD13 anti-gen is expressed by cells of the granulocytic andmonocytic lineages, its presence on plasma cells sup-ports the hypothesis of an early stem cell disorder inthe development of PCL.7 Morphological features ofthe atypical cells in this patient initially suggestedundifferentiated blast cells rather than plasmablastA58-year-old woman was hospitalized becauseof weakness, edema of the extremities andascites. Physical examination revealed pallorand liver enlargement. Biological findings were asfollows: hemoglobin 9.3 g/dL, leukocyte count72.83109/L, with 85% atypical cells, platelet count1313109/L, increased lactic dehydrogenase (780U/L) and b2-microglobulin (16.9 mg/L). Serum pro-tein electrophoresis revealed severe hypogamma-globulinemia, and monoclonal kappa light chain(800 mg/dL) was found in the urine. No liver or kid-ney dysfunction was detected by serum biochemicaldeterminations and no abnormalities were seen onthe X-ray study.Bone marrow aspirate smears revealed almostcomplete replacement of the bone marrow by a dif-fuse infiltration of cells showing similar features tothose observed in the peripheral blood smears. Manyof these cells were large, with abundant, moderate-ly basophilic cytoplasm, and contained two to threedistinct nuclear lobes. Other cells had cleaved nuclei,giving them a monocytoid appearance. The nucleiwith lobulation were often of different size and occa-sionally had prominent nucleoli (Figure 1). Neo-plastic cells were peroxidase, PAS and Sudan nega-tive, and acid phosphatase positive, with the reac-tivity distributed in granules in the cytoplasm. A mas-sive infiltration of cells with cleaved nuclei was alsonoted in the sinusoids of a liver biopsy that was per-formed to exclude hepatic disease as being the ori-gin of the ascites (Figure 2). Flow cytometry of bonemarrow cells showed an intense reactivity with CD38and CD13 antibodies and the presence of cytoplas-matic k light chains. The stem cell antigen CD34,and the myelomonocytic antigens CD33, CD14 andCD15 were negative.Plasma cell leukemia has been defined as a clonalproliferation in which the number of plasma cells inthe blood is greater than 20 percent of the totalleukocytes or the absolute plasma cell count exceeds23109/L.1 The neoplastic cells in most cases closelyresemble normal plasma cells, although sometimesplasma cell leukemia with aberrant morphologic fea-tures (cleaved, monocytoid or multilobated nuclei)has been reported.2-4 These morphologic character-Figure 2. Liver biopsy with sinusoidal infiltrate of monocy-toid cells (Hematoxylin-eosin, original magnification 3400).Figure 1. Bone marrow aspirate smear showing blasts cellswith cleaved and multilobated nuclei (May-Grünwald-Giem-sa, original magnification 31200).C. Panizo et al.850or typical plasma cells, however the immunopheno-type findings allowed us to make the final diagnosisof plasma cell leukemia.The cytogenetic study performed on bone marrowcells showed an abnormal cellular clone with complexkaryotype in all 26 mitoses examined: 46,XX, del(1)(q21), +der(1), -5,-8,t(12;?) (q22;?), -14, add(14)(q32), +3mar. Although these abnormalities are notspecific to PCL, abnormalities of chromosome 14, pri-marily 14q+, are present in 50% of the patients suffer-ing the PCL with abnormal karyotype.8 Besides, dupli-cations and deletions of chromosomes 1 and 8 are alsocommonly involved in PCL. Therefore, karyotype find-ings were very useful in order to confirm this patient’sdiagnosis.The patient was treated monthly with VAD (vin-cristine, doxorubicin and dexamethasone) cycles.After receiving two cycles both clinical and biochem-ical data improved but the patient suddenly diedfrom cerebral hemorrhage. We conclude that recog-nition of aberrant features of plasma cells is helpfulin the differential diagnosis of plasma cell leukemiaand myeloma from other hematopoietic neoplasmsand occasionally from metastatic tumors.References1. Kosmo MA, Gale RP. Plasma cell leukemia. SeminHematol 1987; 24:202-8.2. Zukerberg LR, Ferry JA, Conlon M, Harris N. Plasmacell myeloma with cleaved, multilobated and mono-cytoid nuclei. Am J Clin Pathol 1990; 93:657-61.3. Reed M, McKenna RW, Bridges R, Parkin J, Frizzera G,Brunning RD. Morphologic  manifestations of mono-clonal gammopathies. Am J Clin Pathol 1981; 76:8-23.4. Zawadzki ZA, Kapadia S, Barnes AE. Leukemic myelo-matosis (plasma cell leukemia). Am J Clin Pathol1978; 70:605-11.5. Fritz E, Ludwig H, Kundi M. Prognostic relevance ofcellular morphology in multiple myeloma. Blood1984; 63:1072-9.6. Eclache V, Lusina D, Lejeune F, Cassaus P, Smadja N,Lortholary P. Plasma cell leukemia mimicking acutemonocytic leukaemia in the course of multiple myelo-ma. Nouv Rev Fr Hematol 1993; 35:419-22.7. Barlogie B, Epstein J, Selvanayagam P, Alexanian R.Plasma cell myeloma. New biological insights andadvances in therapy. Blood 1989; 73:865-79.8. Heim S, Mitelman F. Cancer Cytogenetics. Chromo-somal and molecular genetic aberrations of tumorcells. 2nd ed. New York: John Wiley & Sons; 1995.

Primary plasma cell leukemia with unusual morphology and complex karyotype

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Dadun, University of Navarra