ABSTRACT
BACKGROUND: Plasma cell leukemia (PCL) is a rare and aggressive plasma cell neoplasm. In PCL, clonal plasma cells comprise ≥20% of the peripheral blood (PB) leukocytes and/or the absolute clonal PB plasma cell count is ≥2×10(9)/L. Primary PCL (PPCL) originates de novo, whereas, secondary PCL (SPCL) evolves from pre-existing multiple myeloma. METHODS: Clinicohematological features, immunophenotypic profile, and survival of PCL patients were analyzed retrospectively. RESULTS: Between January 2007 and December 2014, ten PPCL and four SPCL patients were investigated (8 PPCLs and 3 SPCLs had complete clinical data). All were North Indians, sharing common geography and ethnicity. Our cohort showed less frequent renal failure, more frequent hepatomegaly, and non-secretory type disease. In contrast to western literature, flow cytometric immunophenotyping of our cohort revealed altered expression of CD138 (67%), CD56 (33%), and CD20 (0%). With novel therapeutic agents, these PPCL patients had a median overall survival of 15 months. CONCLUSION: We highlight that our PPCL patients from North India had distinct clinicohematological and immunophenotypic profiles. The significance of our findings must be tested in a larger patient cohort and must be supported by molecular and cytogenetic investigations to unmask possible significant effects on pathogenesis.
Subject(s)
Humans , Cohort Studies , Cytogenetics , Geography , Hepatomegaly , Immunophenotyping , India , Leukemia, Plasma Cell , Leukocytes , Multiple Myeloma , Neoplasms, Plasma Cell , Plasma Cells , Plasma , Renal Insufficiency , Retrospective Studies , Tertiary Care Centers , Tertiary HealthcareABSTRACT
<p><b>OBJECTIVE</b>To evalueate hepatoprotective effects Feronia elephantum (F. elephantum) correa against thioacetamide (TA) induced liver necrosis in diabetic rats.</p><p><b>METHODS</b>Male wistar rats were made diabetic with alloxan (160 mg/kg) on day 0 of the study. They were intoxicated with hepatotoxicant (thioacetamide, 300 mg/kg, ip) on day 9 of study to produce liver necrosis. Effects of 7 day daily once administration (day 2 to day 9) of EF (400 and 800 mg/kg, po) were evaluated on necorosis of liver in terms of mortality, liver volume, liver weight, serum aspartate aminotransferase (AST) and serum alanine transaminase (ALT), and histopathology of liver sections (for signs of necorosis and inflammation) on day-9 of the study. Separate groups of rats with treated only with alloxan (DA control), thioacetamide (TA control) and both (TA+DA control) were maintained.</p><p><b>RESULTS</b>FE significantly lowered the mortality rate and showed improvement in liver function parameters in TA-induced diabetic rats without change in liver weight, volume and serum glucose levels.</p><p><b>CONCLUSIONS</b>FE showed promising activity against TA-induced liver necorsis in diabetic rats and so might be useful for prevention of liver complications in DM.</p>
Subject(s)
Animals , Male , Rats , Blood Glucose , Chemical and Drug Induced Liver Injury , Drug Therapy , Mortality , Pathology , Diabetes Mellitus, Experimental , Disease Models, Animal , Liver Function Tests , Necrosis , Plant Extracts , Chemistry , Pharmacology , Protective Agents , Rutaceae , Chemistry , ThioacetamideABSTRACT
Essential thrombocythemia [ET] is a rare myeloproliferative disorder occurring predominantly in the elderly population. Its occurence in the pediatric age group is even more rare. We report a 1 3-year-old girl who presented with isolated cerebral venous thrombosis and was diagnosed with essential thrombocythemia. Family screening for any thrombocytosis was 20 negative. With no secondary cause apparent for persistent thrombocytosis, we looked for the JAK2 mutation, but the result was negative. In contrast to linkage of JAK2 mutation positivity with increased thrombotic risk, our case showed that thrombosis can occur in the absence of JAK2 mutation in a case of essential thrombocythaemia. The indications for treatment and the best treatment of children with ET are currently not known, and guidelines for the management of children with ET are needed. Adult patients have near-normal life expectancy because of the low rate of leukemic conversion, but no child has been monitored long enough to assess prognosis