Primary plasma cell leukemia with light chain secretion and multiple chromosomal abnormalities: How successfully treated? - A case report with review of literature

Abstract

Primary plasma cell leukemia is a rare form of plasma cell dyscrasia. We present a case which had leukocytosis with numerous circulating plasma cells in the peripheral blood. Flow cytometry revealed an unusual CD117 expression. Free light chain analysis in the serum showed a markedly elevated level of free lambda light chains. Radiography did not reveal any lytic lesions. Fluorescent in-situ hybridization analysis revealed deletion of 13q14.3 and t(4;14)/t(11;14), while the cytogenetic analysis was normal. The patient was given chemotherapy and was subjected to autologous stem cell transplant, after which she is in complete remission till date.

Clinico-Pathological Spectrum of Primary Plasma Cell Leukemia Diagnosed at a Tertiary Care Centre in South India Over 5 Year Period

“…Recent attempts at genetic and molecular profiling of PCL have shown cytogenetic abnormalities in over 70% of PCL patients [10]. Hypodiploidy and complex karyotypes with multiple numerical and structural abnormalities involving chromosome 1, 13 and 14 are reported in a significant number of PCL cases [4,5,11] including report from India of multiple cytogenetic abnormality in a case of pPCL [12].…”

How I treat plasma cell leukemia

“…In pPCL, the efficacy of combinations of novel agents, such as lenalidomide, bortezomib, and dexamethasone (RVD), 84 bortezomib, thalidomide, and dexamethasone (VTD), 61,64,71 or melphalan, prednisone, bortezomib, and thalidomide (VMPT), 71 appears very promising. Studies describing these regimens invoke only a small numbers of patients but are based on the biologic and clinical features seen.…”

Stringent Complete Remission of Primary Plasma Cell Leukemia with Reduced-dose Bortezomib, Lenalidomide and Dexamethasone: A Case Report and Review of the Literature

“…In fact, bortezomib, lenalidomide and dexamethasone combination therapy (VRD regimen) is highly effective in the treatment of newly diagnosed myeloma (14). There have been only two case reports of PCL patients treated with VRD therapy (15,16) (Table). One case was primary PCL and two cases were secondary PCL.…”

“…One case was primary PCL and two cases were secondary PCL. Goyal et al reported the first primary PCL patient who obtained a complete remission after receiving combination chemotherapy with bortezomib, lenalidomide and dexamethasone (15). However, the details of the treatment regimen and the patient's clinical course were not described.…”