Primary plasma cell leukemia: A case report and review of the literature

Ngu, Sam; Asti, Divya; Valecha, Gautam; Thumallapally, Nishitha; Pant, Millie; Bershadskiy, Alexander

doi:10.1002/ccr3.2339

Cited by 11 publications

(9 citation statements)

References 41 publications

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“…3 Untreated multiple myeloma may lead to sPCL within 20-22 months. 4 This case report aims to highlight the need for awareness among clinicians of this disorder and the importance to examine for other associated clinical features to avoid missing such a vital diagnosis.…”

Section: Introductionmentioning

confidence: 99%

Primary plasma cell leukaemia in a 39-year-old man

Kasinathan

2020

BMJ Case Rep

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Plasma cell leukaemia (PCL) is an aggressive haematological malignancy which is classified into primary (pPCL) and secondary PCL. A 39-year-old Indian man presented to the Department of Hematology with a 2-week history of fever and lethargy. Clinically, he was pale and febrile. Haemogram revealed bicytopenia with leucocytosis. The peripheral blood film portrayed rouleax formation with 45% of circulating plasma cells. Serum protein electrophoresis and immunofixation revealed IgG lambda paraproteinaemia of 48 g/L. Bone marrow aspirate, flow cytometry and trephine were consistent with IgG lambda pPCL. He was treated with six cycles of bortezomib, thalidomide and dexamethasone combination chemotherapy followed by high-dose melphalan conditioning and autologous stem cell transplant. Currently, he is in complete remission for the past 18 months and is on oral lenalidomide maintenance therapy. Prognosis is often dismal in pPCL with the median overall survival below 1 year if treatment is delayed.

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Section: Introductionmentioning

confidence: 99%

Primary plasma cell leukaemia in a 39-year-old man

Kasinathan

2020

BMJ Case Rep

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“…Historically prognosis was very poor with a median overall survival of 6 - 11 months for PPCL and 2 - 7 months for SPCL [ 3 , 10 ]. With improvements in chemotherapy and the use of autologous stem cell transplantation, the median overall survival time has improved to 24 months for patients who were diagnosed during 2006 - 2009 compared to earlier decades, although first month mortality is still 15% [ 7 , 11 ].…”

Section: Discussionmentioning

confidence: 99%

“…The authors declare that it has been signed by the patient in-Patel et al J Med Cases. 2020;11 (10):320-323 volved in the case.…”

Section: Financial Disclosurementioning

confidence: 95%

Acute Plasma Cell Leukemia Presenting as Primary Hyperammonemic Encephalopathy

Patel¹,

Mangano²,

Moccia³

et al. 2020

J Med Cases

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“…e current challenge is the short duration of response as demonstrated by a large cohort of 272 pPCL patients in the European Group for Blood and Marrow Transplantation when compared to myeloma [24]. Newer agents have also been used in plasma cell leukemia such as carfilzomib, ixazomib, monoclonal antibodies to CD38 such as daratumumab, and novel therapies such as Bcl-2 inhibitor, venetoclax in those with t (11; 14) [25,26].…”

Section: Managementmentioning

confidence: 99%

Plasma Cell Leukemia: A Review of 3 Cases Managed in Kenya

Ong'ondi

Kagotho

2021

Case Reports in Hematology

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Plasma Cell Leukemia (PCL) is a rare and aggressive form of plasma cell dyscrasia that can arise either de novo (primary plasma cell leukemia) or evolve from previously diagnosed and treated multiple myeloma (secondary PCL). We highlight three clinical cases with very different presentations as a reminder of this diagnosis. The cases also highlight the diversity and variability that cover a patient’s journey that is highly dependent on accessibility based on financial capability and social support. The clinical presentation is more aggressive due to the higher tumour burden and more proliferative tumor cells with cytopenias being profound and more organomegaly. The diagnosis is made based on at least 20% of total white blood cells being circulating plasma cells with a peripheral blood absolute plasma cell count of at least 2 × 109/l. Treatment with novel agents followed by autologous stem cell transplant in those who are transplant eligible leads to better outcomes.

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Primary plasma cell leukemia: A case report and review of the literature

Cited by 11 publications

References 41 publications

Primary plasma cell leukaemia in a 39-year-old man

Primary plasma cell leukaemia in a 39-year-old man

Acute Plasma Cell Leukemia Presenting as Primary Hyperammonemic Encephalopathy

Plasma Cell Leukemia: A Review of 3 Cases Managed in Kenya

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