Presentation Type
Oral Presentation
Discipline Track
Clinical Science
Abstract Type
Case Report
Abstract
Background: Rosai Dorfman Disease (RDD, also known as sinus histiocytosis with massive lymphadenopathy) is a rare non-Langerhans cell histiocytosis, classified into nodal (classic) and extranodal disease. Most patients present with non-tender cervical lymphadenopathy, although other sites including the inguinal and axillary regions have been reported. Extranodal disease (~43% of cases) can involve any organ system. RDD is more common in children, but cases have been reported in patients in their 70s. The prognosis of nodal RDD can correlate with the number of nodal groups involved. Many cases can be managed only with observation, although surgery, chemotherapy, corticosteroids, and immunomodulators have been used in more extensive disease processes or in cases of relapse. However, there is no consensus on the duration and type of treatment due to limited data.
Case Presentation: A 38-year-old male with HIV (CD4 count 754) presented with progressively worsening dyspnea, productive cough, chest pain, multiple painless and indurated palpable lumps on the right side of his neck and right inguinal area, and unintentional 25-pound weight loss over 5 months. Physical exam showed diffuse rhonchi in the right lobe and non-tender indurated right cervical and inguinal lymphadenopathy. CT chest showed multifocal bilateral pneumonia and reactive mediastinal lymph nodes. Right cervical lymph node biopsy revealed Reed-Sternberg cells weakly positive for PAX5, CD20, CD30, CD15, MUM1, findings consistent with mixed cellularitytype Hodgkin lymphoma. Tumor cells also stained positive for EBERish, consistent with EpsteinBarr virus. No infiltration by the lymphoma was seen on bone marrow biopsy. He received one cycle of ABVD chemotherapy and coverage with vancomycin, meropenem, and fluconazole. Right inguinal node excisional biopsy was positive for CD68, BCL1 (cyclin D1), and S100, highlighting sinus histiocytes. No definitive emperipolesis was identified and clinical correlation with additional lymph node sampling was recommended if Rosai-Dorfman disease is clinically suspected. Given his coagulopathic state and limited ambulation, he developed RLE proximal and distal DVT, necessitating IVC filter placement and anticoagulation with rivaroxaban. He also briefly required supplemental oxygen as well as furosemide due to new onset pleural effusions. Dyspnea improved and he did not require oxygen upon discharge. He was discharged on rivaroxaban, Bactrim, and instructions to follow up with his oncologist and HIV clinic.
Conclusion: Our patient did not follow up with his oncologist after discharge. Additionally, the rivaroxaban he needed has a prohibitively high cost, creating a barrier to care for many patients who need treatment for DVT. This is a complex case in a rather young patient whose disease progression will be negatively impacted by a lack of regular follow-up and lack of access to medications due to cost. Additionally, RDD is rare and disease progression is not well known, creating another barrier as this patient requires extensive follow up for monitoring.
Academic/Professional Position
Resident
Mentor/PI Department
Internal Medicine
Recommended Citation
Salloum, Liza; Rojas Huen, Jennifer; Serna, Ricardo; and Valdez, Alisha, "Case of Rosai Dorfman Disease in a Patient with Newly Diagnosed Hodgkin Lymphoma" (2024). Research Symposium. 9.
https://scholarworks.utrgv.edu/somrs/2024/talks/9
Included in
Hematology Commons, Internal Medicine Commons, Neoplasms Commons, Oncology Commons, Pathological Conditions, Signs and Symptoms Commons, Pathology Commons
Case of Rosai Dorfman Disease in a Patient with Newly Diagnosed Hodgkin Lymphoma
Background: Rosai Dorfman Disease (RDD, also known as sinus histiocytosis with massive lymphadenopathy) is a rare non-Langerhans cell histiocytosis, classified into nodal (classic) and extranodal disease. Most patients present with non-tender cervical lymphadenopathy, although other sites including the inguinal and axillary regions have been reported. Extranodal disease (~43% of cases) can involve any organ system. RDD is more common in children, but cases have been reported in patients in their 70s. The prognosis of nodal RDD can correlate with the number of nodal groups involved. Many cases can be managed only with observation, although surgery, chemotherapy, corticosteroids, and immunomodulators have been used in more extensive disease processes or in cases of relapse. However, there is no consensus on the duration and type of treatment due to limited data.
Case Presentation: A 38-year-old male with HIV (CD4 count 754) presented with progressively worsening dyspnea, productive cough, chest pain, multiple painless and indurated palpable lumps on the right side of his neck and right inguinal area, and unintentional 25-pound weight loss over 5 months. Physical exam showed diffuse rhonchi in the right lobe and non-tender indurated right cervical and inguinal lymphadenopathy. CT chest showed multifocal bilateral pneumonia and reactive mediastinal lymph nodes. Right cervical lymph node biopsy revealed Reed-Sternberg cells weakly positive for PAX5, CD20, CD30, CD15, MUM1, findings consistent with mixed cellularitytype Hodgkin lymphoma. Tumor cells also stained positive for EBERish, consistent with EpsteinBarr virus. No infiltration by the lymphoma was seen on bone marrow biopsy. He received one cycle of ABVD chemotherapy and coverage with vancomycin, meropenem, and fluconazole. Right inguinal node excisional biopsy was positive for CD68, BCL1 (cyclin D1), and S100, highlighting sinus histiocytes. No definitive emperipolesis was identified and clinical correlation with additional lymph node sampling was recommended if Rosai-Dorfman disease is clinically suspected. Given his coagulopathic state and limited ambulation, he developed RLE proximal and distal DVT, necessitating IVC filter placement and anticoagulation with rivaroxaban. He also briefly required supplemental oxygen as well as furosemide due to new onset pleural effusions. Dyspnea improved and he did not require oxygen upon discharge. He was discharged on rivaroxaban, Bactrim, and instructions to follow up with his oncologist and HIV clinic.
Conclusion: Our patient did not follow up with his oncologist after discharge. Additionally, the rivaroxaban he needed has a prohibitively high cost, creating a barrier to care for many patients who need treatment for DVT. This is a complex case in a rather young patient whose disease progression will be negatively impacted by a lack of regular follow-up and lack of access to medications due to cost. Additionally, RDD is rare and disease progression is not well known, creating another barrier as this patient requires extensive follow up for monitoring.