Primary plasma cell leukemia in a 30-year-old woman

Jain, Deepali; Singh, Tejinder; Akhila, L; Ghosh, Nivedita

doi:10.4103/0377-4929.42533

Cited by 3 publications

(3 citation statements)

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“…To the best of our knowledge, only two case reports of such patients, in whom primary PCL presented at aged 30 and 21 years, have been reported in the literature. 8 , 9 As is often reported, this patient presented with symptoms suggestive of an acute leukaemia. However, some of her presenting symptoms would more commonly be seen in secondary PCL and MM, including the presence of bone pain and lytic lesions.…”

Section: Discussionsupporting

confidence: 59%

Primary plasma cell leukaemia in a 22-year-old woman: A case report

et al. 2015

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Introduction: Primary plasma cell leukaemia is a rare and highly aggressive disease that is commonly diagnosed a decade earlier than multiple myeloma, at a median age of 55 years. However, it has also been described in younger patients, as documented in this case report. It often presents with hepatosplenomegaly and lymphadenopathy, whilst the presence of bony lesions are less-commonly seen when compared to multiple myeloma.Case presentation: This report describes the case of a young woman who presented with symptoms of anaemia and a history of menorrhagia. On further careful examination, she was found to have additional signs and symptoms and was later diagnosed with primary plasma cell leukaemia.Management and outcome: On admission, the patient received supportive care measures, including blood products. At diagnosis, a specific chemotherapy regimen was commenced; however, this failed to induce remission. The decision to continue with supportive care only was made and the patient died seven months later.Discussion: This case study is presented because of its rarity, the young age of the patient at presentation and the unusual clinical and laboratory findings. Persistent anaemia unresponsive to standard treatment should raise the index of suspicion and further investigations directed to exclude malignancies should be considered. (Full article to follow)

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Section: Discussionsupporting

confidence: 59%

Primary plasma cell leukaemia in a 22-year-old woman: A case report

et al. 2015

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“…The inhibition of proteasome, with bortezomib or derivatives, could be even more effective in PCL compared with MM. Indeed, the activity of bortezomib may be independent of the cytogenetic abnormalities, including del [13] and t (4;14), frequently observed in PCL [92]. Furthermore, the high frequency of TP53 inactivation reported in PCL, as a result of 17p13 deletions, could sensitize PCL cells to anthracyclines, as recently reported in breast cancers [25,93].…”

Section: Bortezomibmentioning

confidence: 66%

“…Due to the low frequency of this entity, most publications on PCL are based on case reports, and only a few series exist with more than 20 patients in the medical literature [8,[11][12][13]]. …”

Section: Introductionmentioning

confidence: 99%

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

Jiménez-Zepeda

Dominguez-Martinez

2009

Int J Hematol

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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.

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