# An Unusual Case of Primary Plasma Cell Leukemia Presenting As Pericardial Effusion

## TL;DR

A rare case of plasma cell leukemia presented as pericardial effusion in a 58-year-old woman, highlighting the disease's varied symptoms and poor prognosis.

## Contribution

This paper presents a unique clinical case of primary plasma cell leukemia manifesting as pericardial effusion.

## Key findings

- Plasma cell leukemia can present with pericardial effusion as a primary symptom.
- The patient showed initial response to treatment but later became refractory.
- The case underscores the aggressive nature and poor survival outcomes of plasma cell leukemia.

## Abstract

Plasma cell leukemia represents the rarest form of plasma cell dyscrasias arising from monoclonal proliferation of plasma cells in peripheral blood. Its aggressive natural history raises a significant therapeutic challenge. We present a case of a 58-year-old female who presented with a cough accompanied by exertional dyspnea. Further radiological investigations revealed a circumferential pericardial effusion. A pericardiocentesis was performed, resulting in the drainage of 650 cc of hemorrhagic pericardial fluid. Cytology revealed monoclonal plasma cells expressing CD38 and CD138 with cytoplasmic lambda light chain restriction. A bone marrow biopsy disclosed a hypercellular marrow with a monoclonal plasma cell population of 33%. Treatment was initiated with intravenous dexamethasone and weekly subcutaneous injections of bortezomib, followed by outpatient cycles consisting of bortezomib, lenalidomide, daratumumab, and dexamethasone, with a good initial response. However, five months later, the plasma cell leukemia became refractory to treatment, and the patient passed away from septic shock secondary to community-acquired pneumonia. This case illustrates the variable presentations of plasma cell leukemia, as well as its poor prognosis. Further research is warranted to optimize therapeutic regimens and enhance overall survival.