Case Presentation: The patient is a previously healthy 23 year old African American female who presented to the hospital with sudden onset lethargy, abdominal pain and profound anemia. Imaging identified hemoperitoneum secondary to hemorrhagic ovarian cyst rupture and given her severe presentation she required salpingoophorectomy. In addition to bicytopenia with a hemoglobin of 4.8 and platelets 59, she was also found to have diffuse cervical lymphadenopathy on exam which prompted further in-hospital investigation. A bone marrow biopsy showed reticular fibrosis in the presence of overall hypercellularity and was therefore inconclusive. An excisional biopsy of a complete left axillary lymph node confirmed Castleman’s disease, plasma cell variant, HHV-8 negative with monotypic lambda restricted plasma cells. Further workup confirmed that the patient met diagnostic criteria for POEMS associated Ideopathic Multicentric Castleman’s disease. Her hemoglobin stabilized after multiple transfusions and she followed up with an outpatient hematologist who started her on a short course of solumedrol with planned initiation of Siltuximab. She is also still being monitored for a complex mass in her remaining ovary that has been found during this hospitalization.
Discussion: Idiopathic multicentric HHV-8 negative Castleman disease is a rare and poorly understood disease that can affect any age group with an estimated median age of 50-65 and a slight male predominance. It is a lymphoproliferative disorder of the angiofollicular cells of to date unknown etiology that is associated with inflammatory hypercytokinemia and other malignancies. Removal of an enlarged lymph node above the diaphragm is essential for diagnosis. Ruptured hemorrhagic ovarian cysts on the other hand, are very common but most cases are uncomplicated with minimal blood loss. This case illustrates the severe presentation of a complicated bleed from a hemorrhagic cyst requiring surgical intervention in a young woman with a previously unrecognized and difficult to diagnose hematologic disease. Prior to this presentation she had three recent episodes of intermittent malaise, subjective fevers, lower extremity numbness, weakness, night sweats and went to outpatient providers where she received empiric antibiotics for SIRS each time. It is likely that her severe presentation on admission was at least in parts due to her underlying lymphoproliferative disorder and thorough in-hospital work-up was essential in identifying her underlying lymphoproliferative disease.
Conclusions: Castleman disease is a relatively uncommon disease that can be difficult to recognize in young and previously healthy patients. Given its potentially deadly course leading to multi-organ failure and its association with malignancy, it needs to be diagnosed early and be distinguished from more common diseases, including lymphoma, in order to initiate treatment accordingly. A severe presentation of an otherwise benign process, in this case a hemorrhagic cyst, should prompt further investigation and provides a unique window for hospitalists to newly identify an underlying hematologic disease process in a timely manner.