Plasma cell leukemia: a highly aggressive monoclonal gammopathy with a very poor prognosis

Abstract

Plasma cell leukemia (PCL) is an aggressive variant of multiple myeloma and is characterized by the presence of >20% and/or an absolute number of greater 2 x 10(9)/L plasma cells circulating in the peripheral blood. PCL represents approximately 2-4% of all MM diagnosis and exists in two forms: primary PCL (PPCL, 60% of cases) presents de novo, whereas secondary PCL (SPCL, accounts for the remaining 40%) consists of a leukemic transformation in patients with a previously diagnosed MM. Because the mechanisms contributing to the pathogenesis of PCL are not fully understood, immunophenotyping, genetic evaluation (conventional karyotype, FISH, GEP and array-CGH), and immunohistochemistry are really important tools to investigate why plasma cells escape from bone marrow and become highly aggressive. Since treatment with standard agents and steroids is poorly effective, a combination of new drugs as part of the induction regimens and bone marrow transplant (autologous and allogeneic approaches) could nearly overcome the poor prognosis exhibited by PCL patients.

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

“…Genomic and clinical differences between PCL and myeloma have been recognized. The presence of p53 deletion, 13q deletions, karyotypic complexity, hypodiploidy and 1q gains could confer an advanced stage on PC disease progression characterized by therapy resistance and a dismal prognosis [17]. There are older sporadic reports from India of treatment of primary PCL with single agent ifosfamide [18] and more recent reports of autologous stem cell transplantation in patients with pPCL who relapsed after initial remission [19,20].…”

Kemoterapotik ajan olarak dosetakselin ARH 77 Ig G plazma hücreli lösemi hücre serisi üzerindeki sitotoksik etkisi

“…ARH-77 hücre hattı agresif seyreden ve sağ kalım oranlarının düşük olduğu bir lösemi türü olan plazma hücreli lösemiye ait hücrelerden meydana gelmiştir. Metastaz oranı yüksek olması sebebiyle bu kanserde öncelikli olarak metastazın önlenmesini sağlayan tedavi protokollerinin kullanılması uygundur [16]. Bu sebeple dosetakselin kombinasyon tedavileri ile birlikte kullanılması tedaviye alınan cevabın başarısını etkileyecektir.…”

Hemoglobin disorders in the developing world: A perspective from Sri Lanka

“…To the editor: Primary plasma cell leukemia (PPCL) is an aggressive and unusual form of multiple myeloma defined as the presence of at least 20% of circulating leukocytes representing plasma cells [1]. To date no treatment regimen has been found to be clearly efficacious, and survival is generally reported to be short [2].…”

“…It is estimated that 2-3% of disability adjusted life years among children in Asia are attributable to the disorders of hemoglobin [1]. Similar statistics are likely to be present in Africa, although adequate epidemiological studies are lacking [1,2].…”

“…It is estimated that 2-3% of disability adjusted life years among children in Asia are attributable to the disorders of hemoglobin [1]. Similar statistics are likely to be present in Africa, although adequate epidemiological studies are lacking [1,2]. These numbers are undoubtedly going to increase in the ensuing years as many countries make an epidemiologic transition with a concomitant reduction in morbidity and mortality due to the nutritional and infectious diseases that commonly claim the lives of infants with these disorders [3].…”