Category: Clinical Sciences/Health Conditions
HHV-8-Negative/Idiopathic Multicentric Castleman Disease (TAFRO variant)
A 34-year-old female was admitted for acute inpatient rehabilitation following prolonged hospitalization for systemic inflammation of unknown etiology. She initially presented to acute care with fever and malaise, followed by rapid clinical deterioration and undifferentiated shock. An extensive workup (including bone marrow and core needle biopsies) was unremarkable. Treatment with high-dose steroids resulted in improvement. During subsequent inpatient rehabilitation, she made functional gains in the areas of mobility, transfers, and self-care. However, functional decline prior to planned discharge home was noted, along with recurrence of fevers. She was re-admitted to acute care. Further investigation revealed progression of diffuse lymphadenopathy, elevated interleukin-6, and renal failure along with anasarca. Excisional lymph node biopsy was contraindicated due to thrombocytopenia. The decision was made to treat for presumed HHV-8-Negative/Idiopathic Multicentric Castleman Disease (TAFRO variant) with humanized monoclonal antibody against the interleukin-6 receptor. Due to favorable response, patient was ultimately able to resume rehabilitation.
Castleman disease is a rare lymphoproliferative disorder affecting single or multiple lymph nodes. Approximately 6500-7700 new cases are diagnosed in the U.S. annually. The TAFRO variant is differentiated by thrombocytopenia, anasarca, systemic inflammation, renal dysfunction and organomegaly. In addition, the disease can result in a myriad of functional impairments. Initial presentation is non-specific, and the diagnosis is difficult as a result.
Addressing the root cause(s) for plateau or decline in function is one of the cornerstones of a comprehensive rehabilitation program. Physiatrists are attune to subtle changes in function and symptomatology over time. In our case, this unique skill set resulted in the patient receiving appropriate diagnosis and treatment for HHV-8-Negative/Idiopathic Multicentric Castleman Disease (TAFRO variant.)