plasma cell leukemia ( pcl ) is a rare form of plasma cell dyscrasia characterized by proliferation of plasma cells that account for more than 20% of the differential wbc count . the primary form presents de novo in patients with no previous history of multiple myeloma ( mm ) and usually features a rapid clinical progression and short survival . it is very important to recognize this entity sufficiently early so that one can offer combination chemotherapy at the earliest followed by stem cell transplant which can prolong survival . here , we report a case of primary pcl who was treated with combination of vincristine , adriamycin , and dexamethasone ( vad ) . a 75-year - old male presented with complaints of loss of weight , anorexia , and generalized body ache of four weeks duration . on examination , he was pale with upper cervical nontender lymphadenopathy , tachycardia , and no organomegaly . hemogram revealed hemoglobin of 7.6 g / dl , total white cell count of 39.610/l , and platelet count was 17110/l . peripheral smear showed hypochromia and anisopoikilocytosis with 46% plasma cells and plasma blasts with roeuleux formation [ figure 1 ] . iu / l , serum creatinine 1.6 mg / dl , and serum calcium 12 mg / dl . coagulation parameters showed prothrombin time ( pt ) 16 s , activated partial thromboplastin time ( aptt ) 42 s , serum fibrinogen 2.2 g / dl , and fdp 30 . serum protein electrophoresis and immunofixation studies showed iga 2 950 mg / dl , igg 628 mg / dl , igm 16.1 mg / dl , and iga kappa paraprotein at a concentration of 2.95 g / dl . after the first cycle of chemotherapy , plasma cells in peripheral smear decreased to less than 10% and patient showed symptomatic improvement . he received one more cycle of chemotherapy with same regimen , however , the disease progressed and he died after one month due to chest infection and respiratory failure . peripheral blood smear showing plasma cells with eccentric nucleus , perinuclear halo with basophilic cytoplasm bone marrow trephine biopsy shows nodular aggregate of neoplastic plasma cells . although plasma cells are occasionally observed in the peripheral blood of patients with myeloma , the term pcl is only used when the number of these circulating cells is significant ( more than 20% ) . because of the low frequency of pcl , most clinical data are collected from case reports and few reviews . phenotypically they originate from proliferation of plasma cells expressing cd38 , minority of cells express cd10 , hla dr , and cd20 . the presence of multiple hemopoietic surface antigens in malignant plasma cells suggests its origin from pluripotent stem cell . primary pcl shows higher expression of cd20 as compared to mm . also , plasma cells from both primary and secondary pcl lack cd56 . among immunoglobulins , the longest survival reported was 28 months . the failure to achieve 50% clearance of blood plasma cells within 10 days after the initiation of treatment is a predictor of no response . treatment with a single alkylating agent plus prednisolone is not appropriate for patients with pcl . combination chemotherapy with vad , cyclophosphamide , and etoposide or other regimens seems to be a better modality . our patient showed no improvement with vad chemotherapy with less than 10% clearance of blood plasma cells within 10 days of starting treatment . because of the poor prognosis of pcl , intensive chemotherapy and subsequent consolidation with autologous or allogenic stem cell transplantation could be a better approach especially in younger patients who are in good clinical condition .
plasma cell leukemia ( pcl ) is a rare entity . 
 there are two presentations of pcl , primary or secondary . 
 the primary or de novo form of pcl presents with an acute and rapidly progressive leukemic phase . 
 this form occurs when the patient has no pre - existing multiple myeloma ( mm ) . 
 the secondary form is the most advanced form of mm . 
 the pcl is a rare disorder representing 12% of the diagnosed cases of mm . 
 median age at presentation is usually above 50 years 
 . the monoclonal protein in patients with pcl may be igg ( 50% ) , iga ( 15% ) , or in rare cases igd or ige ( 6% ) . 
 we report a case of iga primary pcl that is very rare . 
 patient was started on combination therapy with vincristine , adriamycin , and dexamethasone . 
 there was poor response and patient died three months after diagnosis .