Open AccessA Rare Case of Primary Plasma Cell Leukaemia with Monosomy X
Author(s) -
Smita Surendra Masamatti,
Aparrasimha,
Archana Shetty,
C Vijaya
Publication year - 2017
Publication title -
journal of clinical and diagnostic research
Language(s) - English
Resource type - Journals
eISSN - 2249-782X
pISSN - 0973-709X
DOI - 10.7860/jcdr/2017/28512.10367
Subject(s) - dyscrasia , plasma cell leukemia , plasma cell , medicine , pathology , bone marrow , chemotherapy , stem cell , incidence (geometry) , monosomy , karyotype , biology , physics , optics , genetics , biochemistry , gene , chromosome
Primary Plasma Cell Leukaemia (pPCL) is a very rare and aggressive subtype of plasma cell dyscrasias with a very poor outcome and characterized by presence of >2x109/l circulating plasma cells. Overall incidence being 1%-2% of all malignant plasma cell diseases and 0.9% of all acute leukaemias. Patients with primary and secondary PCL have similar clinical features but differences do exist. Here we report a rare and interesting case diagnosed as primary PCL in a 44-year-old female with unusual clinical presentation and complex karyotyping. Her serum electrophoresis showed a monoclonal IgG component and immunohistochemistry of bone marrow plasma cells showed CD 38 positive and CD 20 negative. The patient initially responded partially for combination chemotherapy, but succumbed after 20 days of diagnosis. We are presenting this case to highlight the importance of early diagnosis of such haematological malignancies in settings where treatment options like stem cell transplantation are limited.