Coexistence of Essential Thrombocythemia and Waldenström Macroglobulinemia: A Case Report

Abstract

Waldenström macroglobulinemia (WM) and essential thrombocythemia (ET) are distinct hematologic malignancies that have only been reported to co-occur in one previous patient. We present a 64-year-old man with a significant family history for WM who was found to have both ET and WM. He had symptomatic ET, diagnosed by elevated platelets and a positive JAK2 V617F mutation, and asymptomatic WM was found on serum electrophoresis done for screening due to family history. Genomic evaluation of the myeloid and lymphoid cells suggested independent neoplastic transformation. This is the second reported case of a patient with both WM and ET. There was no evidence for a shared mechanism in these dual malignancies.

Figure 1

Images from the third and most recent marrow study from our patient. (a) A Wright–Giemsa–stained marrow aspirate featuring a small hyperlobulated micromegakaryocyte with scattered plasma cells (image taken at 40 ×). (b) Four select flow cytometry panels that demonstrate kappa monoclonal B cells and plasma cell populations. (c) A PAS stain particle preparation with a marrow particle featuring increased small hypolobated megakaryocytes which are clustering (image taken at 20 ×). (d) Immunohistochemistry staining for CD138 highlighting a lymphoid aggregate in the center comprised of CD138 negative small lymphocytes being surrounded by CD138 positive plasma cells; note the percentage of plasma cells in this field is increased although the overall plasma cell percentage was ∼10%–20% (image taken at 10 ×).