HEMATO-ONCOLOGY

Imagini în hematologie: leucemia cu plasmocite

 Images in hematology: plasma cell leukemia

First published: 24 aprilie 2021

Editorial Group: MEDICHUB MEDIA

DOI: 10.26416/OnHe.55.2.2021.5020

Abstract

We present the images of peripheral blood smears of a patient diagnosed with plasma cell leukemia. Plasma cell leukemia is a rare and aggressive disease, characterized by more than 20% plasma cells in the peripheral blood or/and an absolute count of more than 2000/mm3.

Keywords
plasma cell leukemia, peripheral blood smear, erythrocytic rouleaux

Rezumat

Prezentăm imaginile cu frotiurile de sânge periferic ale unui pacient diagnosticat cu leucemie cu plasmocite. Leucemia cu plasmocite este o boală rară şi agresivă, caracterizată prin prezenţa a mai mult de 20% de plasmocite în sângele periferic şi/sau peste 2000/mm3 în valoare absolută.

A 66-year-old patient was admitted in the intensive care unit with neurological impairment, with 6 points on Glasgow Coma Scale. The patient had a history of chronic subdural hematoma, with recent hemorrhage and surgical intervention.

  • Automated complete blood count: severe anemia (hemoglobin 6.7 g/dl), moderate thrombocytopenia (platelet count 66,000/mm3), and leukocytosis (11,800/mm3) with monocytosis (4600/mm3).

  • Biochemistry: acute kidney injury (creatinine 7.08 mg/dl, urea 129.7 mg/dl), hyperbilirubinemia (total bilirubin 1.79 mg/dl; direct bilirubin 1.07 mg/dl), and hyperglycemia (161 mg/dl).

  • Computed tomography: multiple areas of osteolysis located in the thoracic and lumbar vertebrae, and pelvis.

The peripheral blood smear unexpectedly revealed the presence of small-medium sized cells with high nuclear/cytoplasmic ratio, round eccentric nuclei, some with nucleoli, basophilic cytoplasm, some with vacuoles, resembling plasmacytoid lymphocytes, and also erythrocytic rouleaux, suggesting plasma cell leukemia. Bone marrow aspirate confirmed the diagnosis, describing 72% plasma cells, with plasmablastic morphology (Figures 1, 2 and 3).
 

Figure 1. Blood smear showing normal neutro­phils and monocytes, small plasma cells with high nuclear/cytoplasmic ratio, round nuclei and eccentric cytoplasm, resembling plasmacytoid lymphocytes. A) Large malignant plasma cells with abundant cytoplasm, with proeminent nucleoli. B) May-Grünwald-Giemsa stain, OM 100x. Note the presence of red blood cell rouleaux in the background
Figure 1. Blood smear showing normal neutro­phils and monocytes, small plasma cells with high nuclear/cytoplasmic ratio, round nuclei and eccentric cytoplasm, resembling plasmacytoid lymphocytes. A) Large malignant plasma cells with abundant cytoplasm, with proeminent nucleoli. B) May-Grünwald-Giemsa stain, OM 100x. Note the presence of red blood cell rouleaux in the background
Figure 2. Blood smear reveals multiple red blood cell rouleaux, which can be linked to high blood protein levels. A) Atypical plasma cell with plasmablastic features, with visible nucleoli and vacuoles.  B) May-Grünwald-Giemsa stain, OM 100x
Figure 2. Blood smear reveals multiple red blood cell rouleaux, which can be linked to high blood protein levels. A) Atypical plasma cell with plasmablastic features, with visible nucleoli and vacuoles. B) May-Grünwald-Giemsa stain, OM 100x
Figure 3. Hypercellular bone marrow aspirate that highlights multiple plasma cells with atypical features. A) May-Grünwald-Giemsa stain, OM 40x. Rare case of plasma cell leukemia (PCL) showing various atypical morphologic features, large malignant plasma cells with vast cytoplasm that exhibits a proeminent pale nucleus described as “plasmablastic morphology”.  B), C), D) May-Grünwald-Giemsa stain, OM 100x
Figure 3. Hypercellular bone marrow aspirate that highlights multiple plasma cells with atypical features. A) May-Grünwald-Giemsa stain, OM 40x. Rare case of plasma cell leukemia (PCL) showing various atypical morphologic features, large malignant plasma cells with vast cytoplasm that exhibits a proeminent pale nucleus described as “plasmablastic morphology”. B), C), D) May-Grünwald-Giemsa stain, OM 100x

Plasma cell leukemia (PCL) is a rare and aggressive variant of myeloma, characterized by the presence of circulating plasma cells. It is classified in primary or secondary PCL, the later being found in patients already diagnosed with relapsed/refractory myeloma(1).

Initially, the plasma cells are predominantly confined in the bone marrow, and only rarely enter the blood stream; the mechanisms are poorly understood. In fact, the plasma cells can be detected in the peripheral blood in only a small proportion of plasma cell dyscrasias. A major role is played by the bone marrow microenvironment(1).

According to Kyle’s criteria, the diagnosis requires circulating plasma cells (≥20% of peripheral blood leukocytes and/or an absolute count >2000/mm3 in the peri­phe­ral blood).

The diagnosis of PCL requires a thorough physical examination, along with laboratory and ra­diological investigations. The disease is characterized by a progressive evolution, with short survival(2-4)

Bibliografie

  1. Rich R, Fleisher T, Shearer W, Schroeder H, Frew A, Weyard C. Monoclonal Gammopathies, Clinical Immunology: Principles and Practice. Fifth Edition, Elsevier, 2019. doi: 10.1016/B978-0-7020-6896-6.00108-3.

  2. Ravi P, Kumar SK, Roeker L, Gonsalves W, Buadi F, Lacy MQ, Go RS, et al. Revised diagnostic criteria for plasma cell leukemia: results of a Mayo Clinic study with comparison of outcomes to multiple myeloma. Blood Cancer J. 2018;8(12):116. doi: 10.1038/s41408-018-0140-1. 

  3. Fernández de Larrea C, Kyle RA, Durie BG, Ludwig H, Usmani S, Vesole DH, et al. International Myeloma Working Group. Plasma cell leukemia: consensus statement on diagnostic requirements, response criteria and treatment recommendations by the International Myeloma Working Group. Leukemia. 2013;27(4):780-791. doi: 10.1038/leu.2012.336.

  4. Vlădăreanu AM. Diagnosticul hemopatiilor maligne. In: Note şi imagini de atlas. Editura Universitară „Carol Davila”, Bucureşti, 2007.