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Acute Plasma Cell Leukemia Presenting as Primary Hyperammonemic Encephalopathy
Dveet Patel1,2, Andrew Mangano2, David Moccia2
1Department of Internal Medicine, Grand Strand Health, Myrtle Beach, SC, USA.
Abstract:
Primary plasma cell leukemia (PPCL) is a rare form of multiple myeloma (MM) and is a rare aggressive disease with a median overall survival of 6 - 11 months. We present a case of acute hyperammonemic encephalopathy as the initial presentation of PPCL in a 78-year-old woman to highlight an atypical presentation of this disorder.
Insights
Primary plasma cell leukemia (PPCL), a rare aggressive multiple myeloma, can present atypically. This case highlights acute hyperammonemic encephalopathy as an unusual initial symptom of PPCL.
Area of Science:
- Hematology
- Oncology
- Neurology
Background:
- Primary plasma cell leukemia (PPCL) is an aggressive hematologic malignancy, a rare subtype of multiple myeloma (MM).
- PPCL is characterized by a poor prognosis, with a median overall survival typically ranging from 6 to 11 months.
- Early diagnosis and treatment are crucial for improving patient outcomes in aggressive cancers.
Observation:
- A 78-year-old woman presented with acute hyperammonemic encephalopathy.
- This neurological presentation was the first indication of an underlying disease process.
- Diagnostic workup revealed the cause to be an undiagnosed case of primary plasma cell leukemia.
Findings:
- The patient's acute hyperammonemic encephalopathy served as an atypical initial manifestation of PPCL.
- This case underscores that neurological symptoms can be the presenting feature of this rare plasma cell disorder.
- The findings emphasize the importance of considering hematologic malignancies in the differential diagnosis of unexplained encephalopathy.
Implications:
- Recognizing atypical presentations of PPCL, such as hyperammonemic encephalopathy, can lead to earlier diagnosis and intervention.
- This case expands the understanding of the clinical spectrum of primary plasma cell leukemia.
- Highlighting rare initial symptoms can improve diagnostic strategies for aggressive hematologic cancers.
