Onset IgA-Type Multiple Myeloma with Flame cells

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Multiple myeloma (MM) represents 1% of all neoplasms and 10% of all malignant hematological diseases. The monoclonal immunoglobulin (M protein) is predominantly IgG (50%), with IgA (20%) or free chains (20%) being less frequent. The prevalence of IgA biclonality is 2%.

The flame cells have a characteristic bright purple-red cytoplasm (Wright-Giemsa staining), related to the high carbohydrate content of IgA molecules, present at their endoplasmic reticulum and cytoplasmic projections. Although usually associated with IgA MM, this polymorphic cells with eosinophilic cytoplasm can also be present in non-IgA MM, MGUS and reactive plasmacytosis.

A 68-year-old male consults his physician, due to unrelated symptoms. The analytical evaluation revealed normochromic normocytic anemia (hemoglobin 113g/L) and acute kidney injury (AKI) KDIGO stage I (sCr 150μmol/L). Follow-up reevaluation, after 1 month, showed increased IgA (22.3g/dL), protein electrophoresis with 2 Beta region peaks (0.3 and 0.7g/dL), and Lambda Bence-Jones protein, with the patient being referred to Hematology.

At the hospital, an in-depth analytical study showed worsening of anemia (hemoglobin 76g/L), AKI KDIGO stage 3 (sCr 645µmol/L), increased lambda free light chains (3550mg/dL) and ratio (0.006). Immunofixation identified biclonal IgA Lambda. Bone marrow aspirate revealed 92% plasma cell infiltration (flame cells). Flow cytometry shown 63.8% plasma cells with CD138+|CD38+|CD56+|CD81+|CD19-|CD45-|β2-microglobulin+|CD27-|CD28-|CD117-|Lambda cytoplasmic chains. Cytogenetic analysis revealed 1q+ in 35% nuclei and a CT scan identified multiple lytic lesions in the spine and pelvis. The patient was diagnosed with MM, initiated bortezomib, cyclophosphamide plus dexamethasone, and due to a good clinical response after 5 cycles underwent autologous stem cell transplant.