Meningeal Involvement in Primary Plasma Cell Leukemia

Excerpt

A 46-year old woman was diagnosed as having primary plasma cell leukemia (pPCL), with 4000/μl circulating plasma cells, massive bone marrow plasma cells infiltration and t(4;14),del 1p,1q gain. She was treated with bortezomib, liposomal doxorubicin and dexamethasone with immunofixation negative complete response. However, just before starting peripheral stem cell mobilization therapy, she experienced right facial nerve palsy. Column and cerebral magnetic resonance imaging (MRI) showed no apparent central nervous system (CNS) involvement. However, lumbar puncture and cerebro-spinal fluid (CSF) cytospin documented meningeal infiltration of CD138+ CD56+ CD38+ plasma cells (Fig. 1). A very small IgG/λ paraprotein in serum and 10% of bone marrow plasma cells were also found. Intrathecal administrations of methotrexate, cytarabine and dexamethasone with cranial-lumbar radiotherapy were given, obtaining CSF plasma cells disappearance and complete resolution of neurological symptoms. The initial therapeutic program was re-started.

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