Multiple myeloma with Russell bodies and needle-shaped crystalline inclusions

Excerpt

A 69-year-old male was referred to the hematology department for examination of pancytopenia, which worsened during follow-up of HCV-associated hepatocellular carcinoma treated with transarterial chemoembolization. Complete blood count showed moderately decreased WBC (11.0 × 10⁹/L) with normal differentiation and severe anemia (Hb 45 g/L) and thrombocytopenia (platelet 3.0 × 10¹⁰/L). Blood chemistry showed marked increase of IgA (1798 mg/dL) and normal IgG (1381 mg/dL) and IgM (78 mg/dL). Immunoelectrophoresis indicated monoclonal IgA-kappa immunoglobulin. Bone marrow aspiration revealed a nearly normal amount of plasma cells (1.8 %), but featured an increase of abnormal cells containing Russell bodies (17.4 %) and needle-shaped crystalline inclusions (5.6 %) (Fig. 1a–c). These cells were positive for CD138, IgA, and kappa light chain but not for lambda (Fig. 1d–g). The patient was diagnosed with multiple myeloma (MM), IgA kappa type, Durie-Salmon stage IIIA. In bone marrow, dysplasia in myeloid and erythroid series and increase of myeloblasts (4.8 %) were observed. G-banding karyotyping was 45, XY, −7, −18, +r1 in 8 of 20 cells, and monosomy 7 was confirmed by FISH analysis (41 %). Given these findings, she was diagnosed with MM accompanied by myelodysplastic syndrome (MDS); refractory cytopenia with multilineage dysplasia (RCMD). We performed one course of high dose dexamethasone followed by four courses of lenalidomide plus dexamethasone. Marked decrease of myeloma cells (plasma; 1.0 %, Russell body-positive cells: 0.8 %, and needle-shaped crystalline inclusions body-positive cells; 2.8 %) and monosomy 7 signal (41 to 1 % by FISH analysis) in the bone marrow were achieved. Five months later, MDS progressed to RAEB-2 with increase of myeloblasts to 19.2 % in bone marrow and monosomy 7 cells to 60 % in FISH analysis, whilst plasma cells did not increase.

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