Diagnosis for Cells Case #21

Plasma cell leukemia (PCL) is a rare and aggressive variant of myeloma characterized by the presence of circulating plasma cells. The diagnosis is based upon the percentage (>20%) and absolute number (>2109/l) of plasma cells in the peripheral blood. It is classified as either primary PCL occurring at diagnosis or as secondary PCL in patients with relapsed/refractory myeloma. Primary PCL is a distinct clinic-pathological entity with different cytogenetic and molecular findings.

/ The CellaVision Blog Team

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This blog is created by CellaVision for laboratory professionals with a particular interest in hematology and digital cell morphology. Our aim is to inform, educate and inspire in equal measures – by highlighting interesting articles, sharing interesting patient cases and cell images, and presenting inspiring success stories from our community of CellaVision-users from around the world.

One thought on “Diagnosis for Cells Case #21”

  1. Plasma cell leukemia is a rare variant of multiple myeloma, accounting for 2-3% of all myelomas. It is a highly aggressive multiple myeloma with a short survival, which must meet the following two diagnostic criteria: a) Presence of plasma cells in more than 20% of all peripheral blood leukocytes; B) Presence of plasma cells in numbers greater than 2,000 / ul in peripheral blood.
    Plasma cell leukemia can be classified into two subtypes:
    1. Primary plasma cell leukemia: when leukemy is already present at the time of diagnosis.
    2. Secondary plasma cell leukemia: when it appears as a consequence of the transformation of a previous multiple myeloma.
    Primary plasma cell leukemia is characterized by appearing in younger patients, with a better prognosis than in secondary ones, with a median survival of about 6.8 months. Osteolytic lesions, hypercalcemia, pathological fractures and bone pain are less frequent in primary plasma cell leukemias, with organomegaly (hepatosplenomegaly) and lymphadenopathy more frequently. The serum and / or urinary component M is usually smaller than in typical multiple myelomas, whereas the presence of renal failure is common.
    Circulating plasma cells may adopt a very variable morphology, from mature forms to immature forms similar to myeloblasts, and in some cases have a lymphoid morphology. The immunophenotype of circulating plasma cells is similar to multiple myeloma (cIg +, sIg-, CD19-, CD20-, CD38 +), but CD56 is generally negative; And the cells often express only light chains, IgE or IgD.
    Treatment of plasma cell leukemia includes treatment with VAD, dexamethasone pulses, alkylating agents and even autologous bone marrow transplantation.

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