LAUSR

A 61-year-old female, with lower-extremity paresthesias and weakness of 9-month duration, was diagnosed with chronic inflammatory demyelinating polyneuropathy (CIDP). Initial evaluation revealed bilateral foot drop, areflexia, sensorimotor neuropathy with a demyelinating component, an elevated cerebrospinal fluid protein, leukocytosis, polycythemia and thrombocytosis, without JAK2 V617F mutation. CT scans identified sclerotic bone lesions (Figure 1A) and marrow biopsy demonstrated clusters of atypical megakaryocytes (Figure 1B). Karyotyping revealed translocation (9;22) in 1/50 cells, without additional structural abnormalities. Morphological features of chronic myelogenous leukemia (CML) or acute lymphoblastic leukemia were absent. Serum immunofixation was not performed, but the other monoclonal protein studies were unremarkable. Although a diagnosis of CML was entertained, no CML-directed treatment was initiated. Despite 3 months of FIGURE 1 Characteristic bone marrow histopathologic and skeletal radiographic findings associated with POEMS syndrome. (A) Widespread sclerotic lesions throughout the spine and pelvis (arrow). (B) Megakaryocytic hyperplasia (arrows, [more than 6 megakaryocytes in HPF]), atypia with mild hyperchromia. (C) Lambda restricted plasma cells with partial rimming around lymphoid aggregates (arrows). [Color figure can be viewed at wileyonlinelibrary.com]