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

Abstract

Plasma cell leukemia (PCL) represents a rare and aggressive form of plasma cell dyscrasia which can be primary (pPCL) or secondary (sPCL). It is diagnosed based on absolute plasma cell count of more than 2.0 9 10 9 /l or a relative proportion of greater than 20% of the peripheral blood leukocyte count. Although pPCL and sPCL share several clinical features, important differences exist. Patients with pPCL are younger; often have extra osseous organ involvement (liver, spleen and other extramedullary sites), increased frequency of renal failure, fast declining performance status and rapid progression to the terminal stage. Patients with sPCL have advanced bone disease. Presented in this article is India data of a short series of five cases of PCL diagnosed at a tertiary care centre from south India over last 5 years. All cases were de novo and had varied spectrum of presentation and so were not suspected to be plasma cell dyscrasia clinically. Detailed hemato-pathological evaluation clinched the diagnosis in all the cases.

Plasma cell leukemia: A case series from South India with emphasis on rarer variants

“…Similar to an earlier series,[ 1 2 3 4 5 6 ] the presence of anemia, thrombocytopenia, renal failure, bleeding tendencies, hepatomegaly, splenomegaly was noted. These features are thought to be more frequent in pPCLs than sPCLs.…”

“…Though most of the data about PCLs is in the form of case reports, there are a few case series published from India. [ 3 4 5 6 ] 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.…”

Primary plasma cell leukemia presenting as a thoracic mass

“…Plasma cell leukaemia is more likely to express CD20, CD45, CD19, CD27 and CD23. [ 1 – 3 , 5 ] In our case besides antigens usually found in plasma cells expressly CD56 and CD19. Our patient didn't have a cytogenetic analysis because of its failure.…”

“…He also found the duration of illness ranged from one week to two months. [ 3 ] Extramedullary presentations such as hepatomegaly, splenomegaly, lymphadenomegaly, leptomeningeal infiltration or extramedullary plasmocytomas are more frequent in pPCL (23%) than in MM (only 4%). Garcia Sanz et al had in her study subcutaneosus nodes, peritoneal plasmocytomas meningeal infiltration and parapleural mass.…”

“…The prognosis of PCL is very poor, with a median overall survival of only 7 months with standard chemotherapy. [ 1 – 3 ] We report the case of a fifty eight year-old smoker male with a diagnosis of PCL which presented as a thoracic mass which was barely treated.…”