SESSION TYPE: Critical Care Student/Resident Cases
PRESENTED ON: Monday, October 22, 2012 at 01:45 PM - 03:00 PM
INTRODUCTION: Sinus histiocytosis with massive lymphadenopathy is a rare histiocytic disorder (1969). The etiology is unknown although suspected occult infection or exaggerated immune response to infectious agent is possible.
CASE PRESENTATION: A 27 yo AA female presenting with 1 week of worsening dyspnea & chest pressure. She reports having similar episodes for 1year. She reports palpitations, nausea and cough. CT Chest demonstrated a pericardial effusion, right pleural effusion, and extensive bilateral air space opacity in a tree and bud configuration and enlargement of the medisastinal and bihilar soft tissue mass impinging on the pulmonary artery. TTE showed markedly dilated RA/RV & reduced systolic function. PA systolic pressure was 85-90mmHg. She was treated with IV steroids and required high concentration oxygen. VAT with lymph node and lung biopsy was performed before pulmonary artery stenting and radiation therapy without significant therapeutic response. Pathology concluded “sinus histiocytosis with massive lymphadenopathy”. Histiocytes showed emperipolesis and were CD68+, variably S100+, and CD1a negative.
DISCUSSION: SHML occurs in any age group but most frequently in young children/adults, Males> Females, in those of African or Caribbean decent. Potential causative agents include Post-viral (HHV6 or EBV), Hodgkins/non-hodgkins, and auto-immune disease although exact etiology is unclear. Typically SHML presents as massive bilateral cervical lyphadenopathy with fever, night sweats, & weight loss. Common extranodal sites include skin, upper respiratory tract, and bone but rarely include lung, spleen and bone marrow. Kidney, lower respiratory tract and liver involvement are poor prognostic factors. These patients typically have leukocytosis, elevated ESR, and polyclonal hypergammaglobulinemia. On pathology lymph nodes show pericapsular fibrosis with dilated sinuses, heavily infiltrated with large histiocytes, lymphocytes and plasma cells. Emperipolesis is the engulfment of lymphocytes and erythrocytes by histiocytes that express S100 and is considered the hallmark of SHML/RDD. Cells stain positive for S100, CD68 and negative for CD1a. We report a rare presentation of sinus histiocytosis with massive lymphadenopathy which presents as a mediastinal mass with lung involvement. Majority of patients have a benign course and do not require treatment. Therapy is reserved for patients with extranodal disease with vital organ involvement causing life-threatening complications. Systemic corticosteroids can decrease nodal size and symptoms. Radiotherapy and chemotherapy have limited efficacy.
CONCLUSIONS: We report an aytpical presentation on a rare histiocytic disorder of biopsy proven Rosai-Dorfman disease (SHLM) resulting in hypoxic respiratory failure and death.
1) Y. Shi et al. Sinus histiocytosis with Massive Lymphadenopathy (Rosai-Dorfman Disease): A case report and review of 49 cases with fine needle aspiration cytology. Cytojournal 2011:8:3
DISCLOSURE: The following authors have nothing to disclose: Sajeet Sawhney, Edison Gavilanes, George Haralambou, Melvin Hochman
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