ABSTRACT
Background: Primary plasma cell leukemia (pPCL) is uncommon, aggressive and has a different biology than multiple myeloma (MM). Aim: To report the features of patients with pPCL. Material and Methods: Review of databases of the Hematology Department and the Hematology laboratory. Results: Of 178 patients with monoclonal gammopathies, five (2.8%) patients aged 33 to 64 years (three females) had a pPCL. The mean hemoglobin was 7.3 g/dL, the mean white blood cell count was 52,500/mm3, with 58% plasma cells, and the mean platelet count was 83,600/mm3. The mean bone marrow infiltration was 89%, LDH was 2,003 IU/L, serum calcium was 13 mg/dL, and creatinine 1.5 mg/dL. Two patients had bone lesions. Three were IgG, one IgA lambda and one lambda light chain. CD20 was positive in one, CD56 was negative in all and CD117 was negative in 3 cases. By conventional cytogenetic analysis, two had a complex karyotype. By Fluorescence in situ Hybridization, one was positive for TP53 and another for t (11; 14). One patient did not receive any treatment, three patients received VTD PACE and one CTD. None underwent transplant. Three patients are alive. The mean survival was 14 months. Conclusions: These patients with pPCL were younger and had a more aggressive clinical outcome than in multiple myeloma.
Subject(s)
Humans , Male , Female , Adult , Middle Aged , Leukemia, Plasma Cell/genetics , Leukemia, Plasma Cell/epidemiology , Paraproteinemias/genetics , Paraproteinemias/pathology , Paraproteinemias/epidemiology , Blood Cell Count , Leukemia, Plasma Cell/pathology , Leukemia, Plasma Cell/therapy , Survival Analysis , Chile/epidemiology , Calcium/blood , Retrospective Studies , Treatment Outcome , In Situ Hybridization, Fluorescence , Creatinine/blood , Cytogenetic Analysis , Flow Cytometry/methodsABSTRACT
Plasma cell leukemia is a rare, aggressive form of multiple myeloma. A 35-year-old male presented with backache, generalized weakness, and facial puffiness. His complete blood count showed anemia and a high WBC count with atypical cells on peripheral smear. Bone marrow examination showed more than 90% of atypical plasma cells, confirming a diagnosis of plasma cell leukemia. Patient also had azotemia, hypercalcemia, and hyperuricemia. The patient was started on chemotherapy along with supportive care. Patient improved dramatically and he was discharged on regular follow-up.
Subject(s)
Adult , Antineoplastic Agents/therapeutic use , Blood Cell Count , Hospice Care , Humans , Leukemia, Plasma Cell/drug therapy , Leukemia, Plasma Cell/epidemiology , Male , Melphalan/therapeutic use , Prednisolone/therapeutic use , Thalidomide/therapeutic useABSTRACT
Plasma cell leukemia is considered as the leukemic variant of multiple myeloma. It is a rare entity. There are two forms: a secondary one following a known myeloma, the diagnosis of which is easy, and a primary one arising without a preceding phase of multiple myeloma. The diagnosis of the latter form is more difficult, a differential diagnosis has often to be discussed with other lymphoproliferative diseases. Prognosis is poor. We report 2 cases of secondary plasma cell leukaemia diagnosed over ten years, among 59 of multiple myeloma cases. We describe the epidemiologic, clinical, biological and evolutionary characteristics