Zeitschrift für Zytologie und Histologie

Acute Abdominal Pain Revealing a Primary Plasma Cell Leukemia: A Rare and Aggressive Case of Plasma Cell Dyscrasia

Abstract

Luiz Arthur Calheiros Leite, Alessandra Gabrielly Magalhães, Jailson Ferreira Silva, Alexssando Pedro Silva, Paloma Lys Medeiros, Jeymesson Raphael Vieira, Cintia Machado, Renata Brandão, Claudia Wanderley Barros Correia, Tómas Jelínek and Roman Hájek

Primary plasma cell leukemia is a rare and aggressive form of plasma cell neoplasm characterized by the presence of circulating plasma cells in peripheral blood. Here, we report an interesting case of a patient with primary plasma cell leukemia, who was diagnosed during postoperative period after surgery for acute appendicitis. After the appendectomy, the patient developed leukocytosis of 41.2×109/L with a high number of circulating plasma cells (32.5×109/L). The immunophenotypic analysis of leukemic plasma cells indicated the following antigen profile: CD138�?¢�?�?º�?¢�?�?º�?¢�?�?º, CD38�?¢�?�?º�?¢�?�?º�?¢�?�?º, IgMc�?¢�?�?º, CD56¯ and CD20�?¢�?�?». Electrophoresis showed a monoclonal IgG component of 5810 mg/dL, and diagnosis of primary plasma cell leukemia was established. After the induction chemotherapy with cyclophosphamide, bortezomib and dexamethasone, the patient achieved partial response with regression of hepatomegaly and decreased pleural effusion. Nevertheless, the patient died of sepsis. Therapy with proteasome inhibitors and stem cell transplantation may also provide increased survival and improve the prognosis of patients with primary plasma cell leukemia.