plasma cell leukemia ( pcl ) is a rare entity and the diagnosis is made when peripheral blood shows a large number of clonal plasma cells ( absolute count of plasma cells 2  10/l or > 20% of the differential count ) . the diagnosis is still made on the original diagnostic criteria laid down by noel and kyle in 1974 . at that time , both absolute counts and percentages were necessary for diagnosis , however now , any one of these two criteria is considered sufficient . a recent publication by de fernndez de larrea et al . suggests , the incidence of pcl is probably underestimated , and lower diagnostic values be introduced to make the diagnosis early . along with morphology , they suggested incorporating flow cytometry , cytogenetics and molecular markers for detecting patients at the earliest . plasma cell leukemias are classified as primary pcl ( ppcl ) , when they present de novo in leukemic phase and secondary pcl ( spcl ) , when it develops in the background of a diagnosed myeloma . though most of the data about pcls is in the form of case reports , there are a few case series published from india . this work is being presented because it is a rather large series ( 16 cases ) , four young patients , two with  hairy cell  morphology and one case that survived for 4 years on multiple therapeutic regimens . the cases were retrieved from hematopathology records , from 2007 to june 2013 ( 6 years ) . the complete clinical , imageologic details , investigations and follow - up were obtained from the treating department records . the peripheral smear , bone marrow cytology stained with giemsa stain and trephine sections with hematoxylin and eosin , were reviewed . cases with 20% plasma cells or 2000/cmm in peripheral blood samples were included in the study . the panel included cd2 , cd3 , cd5 , cd10 , cd11c , cd19 , cd23 , cd25 , sigm , kappa , lambda , cd38 , cd45 , cd56 , cd103 , cd117 , and cd138 . there were 16 cases diagnosed as pcl during the study period , of which 15 were ppcls , and 1 was spcl . the ages ranged from 25 years to 72 years of age with a median age of 52.5 years . there were four cases under the age of 40 years ( 25 , 36 , 38 , 38 years ) . the patients included 6 males and 10 females giving a male to female ratio of 3:5 . clinical presentation ( n = 16 ) the hematologic , biochemical and immunophenotypic data of the patients were analyzed . all the patients were anemic , with hemoglobin varying from 4.5 to 10.5 g / dl . total leucocyte counts varied from 3500 to 56500/cmm with plasma cells varying from 21% to 70% in the periphery . majority had some degree of thrombocytopenia . due to financial constraints , some of the patients left hospital immediately after the diagnosis was made , hence , all biochemical and immunophenotypic data were not available . the plasma cells had varying morphology . some were classic plasma cells ( 5 ) , some plasmablastic ( 8) and one patient showed cleaved and polymorphus plasma cells . hairy  projections [ figure 1 ] and were sent for immunophenotyping with a suspicion of hairy cell leukemia . however , they were negative for hairy cell markers and positive for plasma cell markers . plasma cells with  hairy cell  morphology giemsa 1000 urea , creatinine levels were available in 14 cases . urine for bence - jones proteins was positive in nine patients ( lambda type-4 , kappa type-5 ) . one of the patients expired immediately after diagnosis , and six left against medical advice , once the dismal prognosis was explained . one patient received various drug combinations including bortezomib + dexamethasone , liposomal doxyrubicin + dexamethasone , melphalan + prednisone and lenolidamide and finally succumbed to her illness 4 years after initial diagnosis . eight of the treated patients expired or were lost to follow - up during therapy , and only one is still coming for treatment . during the study period , 16 cases of pcl were diagnosed , mainly on morphologic grounds . a recent publication suggests that if the diagnostic criteria were reduced to 5% plasma cells and/or an absolute count of 0.5  10/l , while incorporating ancillary techniques , patients could be diagnosed earlier and have a better prognosis . the median age of pcls was 52.5 years , which was comparable to other indian studies [ table 2 ] . comparisons between the present study and other published indian series another odd feature was that there more female patients . similar to an earlier series , the presence of anemia , thrombocytopenia , renal failure , bleeding tendencies , hepatomegaly , splenomegaly was noted . there were two very interesting cases , where the neoplastic cells resembled  hairy cells  . on immunophenotyping , they were negative for b lineage and hairy cell markers , and positive for plasma cell markers cd38 and cd138 . four patients had immunophenotyping by flow cytometry , of which all were positive for cd38 , three for cd138 and negative for cd45 . it has been reported that the malignant plasma cells of pcl ( primary or secondary ) do not or weakly express cd56 . the prognosis in pcls is bad and the response to conventional chemotherapy ( vincristine , adriamycin and dexamethsone ) is poor . hence , novel agents like bortezomib , thalidomide and lenolidomide or their combinations are preferred , followed by transplantation . lenolidomide is less toxic , and its combination with dexamethasone has been effective in newly diagnosed pcls . of the nine who were given specific therapy , eight expired or lost to follow - up and only one patient is still surviving . to conclude , pcls are a rare entity , are aggressive malignancies and patients do poorly in spite of chemotherapy . this series is being presented as it is the second largest series from india with some rare associations ( four cases under 40 years , more females , two cases with
plasma cell leukemia ( pcl ) is a rare and aggressive variant of plasma cell dyscrasia . 
 they occur de novo ( primary ) or as a late manifestation of multiple myeloma ( secondary ) . 
 patients present with anemia , thrombocytopenia , renal failure , organomegaly and extramedullary manifestations . we are presenting this series as it is the second largest series from india ( 16 ) with 4 young cases ( under 40 years of age ) , more number of female patients and two having  hairy cell morphology . 
 it is recommended that techniques like immunophenotyping and protein electrophoresis be performed , whenever the morphology is not characteristic of plasma cells .